Split (1)

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In case 1, the immunohistochemical analyses on the two surgical specimens showed a moderately differentiated adenocarcinoma infiltrating through the muscularis propria into the tissues surrounding the colon and an invasive ductal carcinoma of the breast, which suggests this as a synchronous occurrence of tumors. The reported overall incidence of DPM has ranged from 3% to 20% among colorectal cancer patients; this frequency is comparatively higher among the general population . The incidence of synchronous breast and colon cancers in women is reported to be 3.85% . Uncertainty persists over the association between synchronous breast and colon cancers, which have not been thoroughly studied . Synchronous tumors have a high disposition for family history. In individuals with hereditary breast and colorectal cancers, genetic mutation of CHEK2*1100delC (CHEK2) has been observed . An increased risk of breast cancer by three to five times has been attributed to this low-penetrance breast cancer-predisposing gene . Numerous epidemiological studies have examined the relationship between breast and colorectal cancer, examining the risk of developing a second primary non-colorectal cancer following colorectal adenocarcinoma, with the majority of those secondary malignancies occurring within three years of the primary colorectal cancer . According to Ueno et al., when colon cancer was correlated with the existence of other tumors in women, the most commonly seen tumor was gastric cancer, followed by breast cancer . The outcome of synchronous cancers relies on each cancer stage individually. Compared with single cancer, synchronous cancers have no worse prognosis with appropriate therapy. In our multidisciplinary team (MDT) cancer meeting, it was decided to do an open extended left hemicolectomy with primary colo-colonic anastomosis followed by bilateral mastectomy and bilateral sentinel lymph node biopsies of both axillae. The patient had received four cycles of neoadjuvant chemotherapy, and post-operatively the patient received adjuvant chemotherapy, radiotherapy, and hormonal therapy. If both tumors demand adjuvant chemotherapy, it is critical to think about what chemotherapy regimen should be administered post-operatively. However, the challenge is to find an anticancer therapy approach that incorporates both cancer types without increasing toxicity or appropriate pharmacological reactions and without having a negative impact on the overall prognosis. In case 2, the patient has a poorly differentiated adenocarcinoma (grade 3) of the colon with focal mucinous differentiation and papillary renal cell carcinoma (RCC) and oncocytoma synchronously. The coexistence of RCC with other neoplasms has already been reported in the literature. According to data from the Norwegian Cancer Registry, 18% of 1425 RCC patients who were studied over the course of seven years had at least one additional primary tumor . A retrospective investigation conducted at a Japanese university hospital discovered a synchronous malignant tumor in 5.9% of patients who underwent RCC resection, and the presence of other primary tumors at the time of nephrectomy was found to be an independent predictor of post-operative survival . Czene and Hemminki's study demonstrates unequivocally that individuals with RCC face an increased risk of developing additional malignancies not only during the first year following the main diagnosis but also after > 10 years . A rare case of coexistence of three neoplasms (colonic carcinoma, renal cell carcinoma, and gastrointestinal stromal tumor) in a single patient has been reported . It is suggested that microsatellite instability testing may be used in all patients presenting with colorectal and urological cancers to detect a common genetic aberration between malignancies . Both RCC and colon cancer can have a genetic susceptibility caused by mutations in the c-MET and c-KIT proto-oncogenes, where both proto-oncogenes encode receptor tyrosine kinases . However, this does not prove an etiological link between the two tumor types. With careful patient selection, therapeutic goals like improving symptoms, quality of life, and palliation can be achieved . However, the optimal management of such cases remains unclear. In our case, a right hemicolectomy with right partial nephrectomy in the same session was performed. The use of en bloc resection has been found to have a significant role in locally advanced disease, although there is a lack of evidence supporting radical resection. In case 3 of our report, the MPMTs were diagnosed as a non-invasive papillary urothelial carcinoma of the renal pelvis, low grade (G1) and rectal tumor invasive adenocarcinoma, and low grade (G1) with an invasion of muscularis propria. Urothelial cancer (UC) is the fourth most common malignancy. The upper urinary tract (renal pelvis and ureter) urothelial cancer is rare, being only 5-10% of all UC. Upper tract UC (UTUC) is three times more common in males than in females and it peaks at the age of 70-90 years. The development of UTUC is linked to both hereditary and environmental factors . A strong association has been found between upper urinary tract urothelial cell carcinomas (UUT-UCC) and hereditary non-polyposis colorectal cancer (HNPCC), as HNPCC carriers have a 22-fold higher risk of developing UUT-UCC than the general population . HNPCC, also known as Lynch syndrome (LS), is an autosomal dominant cancer syndrome that is caused by a mutation in one of the DNA mismatch repair (MMR) genes (MLH1, MSH2, MSH6, and PMS2) that causes microsatellite instability (MSI). Among HNPCC-associated cancers, the prevalence of UC is 5%, which makes it the third most common cancer after colon cancer (63%) and endometrial cancer (9%). HNPCC is the most common hereditary form of colon cancer, responsible for 3% of all new colon cancer diagnoses and it carries a 70-90% lifetime risk of colorectal cancer . A study that aimed to determine the association between colorectal cancer and urologic cancers by quantifying colorectal cancer (CRC) risk after urologic cancer, as well as the risk for urologic cancer after CRC in patients without a known genetic syndrome, concluded that a history of renal pelvis and ureteral cancers, especially when diagnosed before the age of 60, increase the risk for subsequent CRC by 80% and 44%, respectively. Likewise, a previous CRC, particularly in cases with MPMT, increases the risk of developing renal pelvis cancer by 59% and ureteral cancer by 100%. These findings suggest a possible common pathogenetic mechanism between urologic cancers and CRC .	4.402344	0.612793	sec[2]/p[1]	en	0.999998	PMC9867554	https://doi.org/10.7759/cureus.32839	[ "cancer", "colorectal", "cancers", "colon", "risk", "breast", "synchronous", "that", "tumor", "both" ]	[ { "code": "2D4Z", "title": "Unspecified malignant neoplasms of unspecified sites" }, { "code": "2C0Z", "title": "Malignant neoplasms of intestine, unspecified" }, { "code": "2B5Z", "title": "Malignant mesenchymal neoplasm of unspecified type" }, { "code": "2E2Z", "title": "Malignant neoplasm metastasis, unspecified" }, { "code": "2D42", "title": "Malignant neoplasms of ill-defined sites" }, { "code": "DB33.Z", "title": "Noninfectious colitis or proctitis, unspecified" }, { "code": "DB35.Z", "title": "Polyp of large intestine, unspecified" }, { "code": "2B91.Y", "title": "Other specified malignant neoplasms of rectosigmoid junction" }, { "code": "2B91.Z", "title": "Malignant neoplasms of rectosigmoid junction, unspecified" }, { "code": "2B90.Y", "title": "Other specified malignant neoplasms of colon" } ]	=== ICD-11 CODES FOUND === [2D4Z] Unspecified malignant neoplasms of unspecified sites Also known as: Unspecified malignant neoplasms of unspecified sites \| malignancy of unspecified site \| malignancy unspecified primary site \| malignant growth of unspecified site \| malignant mass of unspecified site [2C0Z] Malignant neoplasms of intestine, unspecified Also known as: Malignant neoplasms of intestine, unspecified \| cancer of intestine \| malignant neoplasm of intestine NOS \| malignant tumour of intestine NOS \| intestinal cancer NOS [2B5Z] Malignant mesenchymal neoplasm of unspecified type Also known as: Malignant mesenchymal neoplasm of unspecified type \| calvarium cancer \| ethmoid bone cancer \| facial bone cancer \| frontal bone cancer [2E2Z] Malignant neoplasm metastasis, unspecified Also known as: Malignant neoplasm metastasis, unspecified \| secondary malignant neoplasm \| metastasis \| metastases \| disseminated metastases [2D42] Malignant neoplasms of ill-defined sites Definition: Malignant neoplasms of ill defined sites is used for cases where the documentation refers to a site that includes multiple organ systems and tissue types that should be coded separately. Also known as: Malignant neoplasms of ill-defined sites \| Malignant neoplasm of ill-defined site of head, face or neck \| Malignant neoplasm of nose NOS \| Primary malignant neoplasm of cheek \| malignant neoplasm of cheek NOS [DB33.Z] Noninfectious colitis or proctitis, unspecified Also known as: Noninfectious colitis or proctitis, unspecified \| Certain noninfectious colitis or proctitis \| colorectal inflammation NOS \| proctitis NOS [DB35.Z] Polyp of large intestine, unspecified Also known as: Polyp of large intestine, unspecified \| Polyp of large intestine \| Colorectal polyp \| Polyp of rectum \| rectal polyp [2B91.Y] Other specified malignant neoplasms of rectosigmoid junction Also known as: Other specified malignant neoplasms of rectosigmoid junction \| Carcinoma of rectosigmoid junction \| carcinoma of rectum with sigmoid \| colorectal carcinoma [2B91.Z] Malignant neoplasms of rectosigmoid junction, unspecified Also known as: Malignant neoplasms of rectosigmoid junction, unspecified \| Malignant neoplasms of rectosigmoid junction \| rectosigmoid junction cancer \| primary malignant neoplasm of rectosigmoid junction \| rectosigmoid colon cancer [2B90.Y] Other specified malignant neoplasms of colon Also known as: Other specified malignant neoplasms of colon \| Neuroendocrine neoplasm of colon \| Colon endocrine neoplasm \| Neuroendocrine carcinoma of colon \| NEC - [neuroendocrine carcinoma] of colon === GRAPH WALKS === --- Walk 1 --- [2D4Z] Unspecified malignant neoplasms of unspecified sites --PARENT--> [?] Malignant neoplasms of ill-defined or unspecified primary sites --CHILD--> [2D42] Malignant neoplasms of ill-defined sites Def: Malignant neoplasms of ill defined sites is used for cases where the documentation refers to a site that includes multiple organ systems and tissue types that should be coded separately.... --- Walk 2 --- [2D4Z] Unspecified malignant neoplasms of unspecified sites --PARENT--> [?] Malignant neoplasms of ill-defined or unspecified primary sites --EXCLUDES--> [?] Malignant mesenchymal neoplasms Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn... --- Walk 3 --- [2C0Z] Malignant neoplasms of intestine, unspecified --PARENT--> [?] Malignant neoplasms of intestine --EXCLUDES--> [?] Malignant mesenchymal neoplasms Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn... --- Walk 4 --- [2C0Z] Malignant neoplasms of intestine, unspecified --PARENT--> [?] Malignant neoplasms of intestine --PARENT--> [?] Malignant neoplasms of digestive organs Def: A primary malignant neoplasm involving any part of the gastrointestinal system.... --- Walk 5 --- [2B5Z] Malignant mesenchymal neoplasm of unspecified type --PARENT--> [?] Malignant mesenchymal neoplasms Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn... --CHILD--> [2B52] Ewing sarcoma, primary site Def: A small round cell tumour that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It represents one of the two ends of the spectrum called Ew... --- Walk 6 --- [2B5Z] Malignant mesenchymal neoplasm of unspecified type --PARENT--> [?] Malignant mesenchymal neoplasms Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn... --EXCLUDES--> [?] Mesenchymal tumours of meninges	[ "[2D4Z] Unspecified malignant neoplasms of unspecified sites\n --PARENT--> [?] Malignant neoplasms of ill-defined or unspecified primary sites\n --CHILD--> [2D42] Malignant neoplasms of ill-defined sites\n Def: Malignant neoplasms of ill defined sites is used for cases where the documentation refers to a site that includes multiple organ systems and tissue types that should be coded separately....", "[2D4Z] Unspecified malignant neoplasms of unspecified sites\n --PARENT--> [?] Malignant neoplasms of ill-defined or unspecified primary sites\n --EXCLUDES--> [?] Malignant mesenchymal neoplasms\n Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn...", "[2C0Z] Malignant neoplasms of intestine, unspecified\n --PARENT--> [?] Malignant neoplasms of intestine\n --EXCLUDES--> [?] Malignant mesenchymal neoplasms\n Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn...", "[2C0Z] Malignant neoplasms of intestine, unspecified\n --PARENT--> [?] Malignant neoplasms of intestine\n --PARENT--> [?] Malignant neoplasms of digestive organs\n Def: A primary malignant neoplasm involving any part of the gastrointestinal system....", "[2B5Z] Malignant mesenchymal neoplasm of unspecified type\n --PARENT--> [?] Malignant mesenchymal neoplasms\n Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn...\n --CHILD--> [2B52] Ewing sarcoma, primary site\n Def: A small round cell tumour that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It represents one of the two ends of the spectrum called Ew...", "[2B5Z] Malignant mesenchymal neoplasm of unspecified type\n --PARENT--> [?] Malignant mesenchymal neoplasms\n Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn...\n --EXCLUDES--> [?] Mesenchymal tumours of meninges" ]	2D4Z	Unspecified malignant neoplasms of unspecified sites	[ { "from_icd11": "2D4Z", "icd10_code": "C802", "icd10_title": "Malignant neoplasm associated with transplanted organ" }, { "from_icd11": "2D4Z", "icd10_code": "C7650", "icd10_title": "Malignant neoplasm of unspecified lower limb" }, { "from_icd11": "2D4Z", "icd10_code": "C7642", "icd10_title": "Malignant neoplasm of left upper limb" }, { "from_icd11": "2D4Z", "icd10_code": "C7640", "icd10_title": "Malignant neoplasm of unspecified upper limb" }, { "from_icd11": "2D4Z", "icd10_code": "C7652", "icd10_title": "Malignant neoplasm of left lower limb" }, { "from_icd11": "2D4Z", "icd10_code": "C7651", "icd10_title": "Malignant neoplasm of right lower limb" }, { "from_icd11": "2D4Z", "icd10_code": "C7641", "icd10_title": "Malignant neoplasm of right upper limb" }, { "from_icd11": "2D4Z", "icd10_code": "C801", "icd10_title": "Malignant (primary) neoplasm, unspecified" }, { "from_icd11": "2D4Z", "icd10_code": "C768", "icd10_title": "Malignant neoplasm of other specified ill-defined sites" }, { "from_icd11": "2D4Z", "icd10_code": "C761", "icd10_title": "Malignant neoplasm of thorax" }, { "from_icd11": "2D4Z", "icd10_code": "C762", "icd10_title": "Malignant neoplasm of abdomen" }, { "from_icd11": "2D4Z", "icd10_code": "C763", "icd10_title": "Malignant neoplasm of pelvis" }, { "from_icd11": "2D4Z", "icd10_code": "C800", "icd10_title": "Disseminated malignant neoplasm, unspecified" }, { "from_icd11": "2D4Z", "icd10_code": "C76-C80", "icd10_title": "" }, { "from_icd11": "2D4Z", "icd10_code": "C76", "icd10_title": "Malignant neoplasm of other and ill-defined sites" } ]	C802	Malignant neoplasm associated with transplanted organ
A 77-year-old male patient was referred to our hospital after a computed tomography (CT) scan performed during a follow-up after prostate cancer surgery at another hospital revealed a gradually enlarging mediastinal tumor. He had a history of hypertension, no recurrence after prostate cancer surgery, right inguinal hernia surgery, no medications, occasional drinking, and smoking 20 cigarettes per day. Blood test results revealed the following: total cholesterol, 283 mg/dL; low-density lipoprotein cholesterol, 191 mg/dL; triglyceride (TG), 44 mg/dL, tumor-related marker carcinoembryonic antigen, 6.2 ng/mL; alpha-fetoprotein, 3.9 ng/mL; squamous cell carcinoma antigen, 1.9 ng/mL; cytokeratin 19 fragment, 4.5 ng/mL; soluble interleukin-2 receptor, 429 U/mL; pro-gastrin releasing peptide, 63.9 pg/mL; thyroid stimulating hormone, 1.770 μIU/mL; and human chorionic gonadotropin beta subunit, ≤ 0.1 ng/mL. Contrast-enhanced CT revealed a cephalocaudally continuous multifocal cystic lesion from the posterior mediastinum to the retroperitoneum at the level of the 8th thoracic vertebra to the 2nd lumbar vertebra. The tumor was approximately 20 mm in maximum size, tortuous throughout, but about 180 mm in linear distance, with a predominantly fatty component inside, septal and partly contrast-enhanced components . Magnetic resonance imaging revealed a high-intensity T2-weighted image, which led to a radiological diagnosis of lipoma or well-differentiated liposarcoma . In addition, CT in the prone position showed that gravity widened the thoracic cavity, allowing resection from the thoracic cavity to the caudal side of the tumor; therefore, we decided to perform bilateral thoracoscopic surgery in the prone position . The patient underwent surgery in the prone position, with surgical monitors placed on the left, right, and caudal sides. Because the head end of the tumor was on the right side, a right thoracoscopic surgery was initiated. We placed a 12-mm port in the 7th intercostal space above the posterior axillary line, a 5-mm port in the 9th intercostal space, and a 12-mm port (camera) in the 8th intercostal space above the inferior scapular angle line. The tumor was continuous from the caudal side of the tracheal bifurcation beyond the diaphragm. The intraoperative diagnosis of thoracic duct cyst was made based on the presence of a thicker membrane structure than that of lipoma and hyperdifferentiated liposarcoma, firmer palpation with forceps, and the fact that it coincided with the location of the thoracic duct often seen during esophageal surgery. To prevent chyle leakage, a vessel-sealing system was used to dissect the area around the tumor, and inflow vessels were clipped as needed. All intercostal arteries adjacent to the tumor were preserved to maintain the blood flow to the Adamkiewicz artery. The right side of the diaphragmatic leg aortic hiatus was opened, and the tumor was dissected circumferentially to the point, where it continued to the left side of the aorta, completing the approach from the right thoracic cavity. The tumor was mostly located on the right side of the mediastinum; however, the most caudal side of the tumor in the abdomen was on the left side of the abdominal aorta. The right-sided approach had a poor view of the most caudal side, and we thought that it was necessary to add a left-sided approach due to ensure a better view of the most caudal side of the tumor for reliable clipping. Next, we performed a left thoracoscopic surgery. A 5-mm port was placed in the 7th intercostal space above the left midaxillary line, a 12-mm port in the 9th intercostal space, and a 12-mm port (camera) in the 8th intercostal space above the inferior angle of the scapula. The left side of the diaphragmatic leg aortic hiatus was opened, and the tumor was detached from the vertebral body and aorta to reach the caudal margin. At the caudal margin, the patient was clipped to the contiguous left cisterna chyli, and the dissected specimen was removed. A drain was placed at the midaxillary line of the 9th intercostal space on the patient's right side, and the surgery was completed . The operation lasted 4 h 30 min, and there was minimal blood loss. The excised specimen was a segmental tumor approximately 20 × 180 mm in size, and the surgical findings were suggestive of a thoracic duct cyst rather than a lipoma . Histopathological examination revealed a cystic structure with a fibrous wall and smooth muscle stained with alpha-smooth muscle actin around the cystic structure . The tumor appears to be segmented; however, the interior of the tumor was highly degenerated and no valves inherent to the thoracic duct were histopathologically observed. The cystic fluid was milky white with a high TG level of 1310 mg/dL. Based on these findings, a diagnosis of a thoracic duct cyst was made. The patient's postoperative course was uneventful, and he was discharged 4 days postoperatively. A CT performed 13 months after surgery showed no recurrence. Fig. 1 Computed tomography scan showing a continuous multifocal cystic lesion in the cephalocaudal direction from the posterior mediastinum to the retroperitoneum at the 8th thoracic to the 2nd lumbar levels. The white arrowhead (top left) indicates the most cephalad, and the black arrowhead (bottom right) indicates the most caudal Fig. 2 Magnetic resonance imaging showing high intensity on T2-weighted image Fig. 3 Contrast-enhanced computed tomography in the supine position in the upper row and in the prone position in the lower row. Comparison of a , A the most cephalic side of the tumor, b , B confluence of the hepatic vein and inferior vena cava, and c , C level at which the celiac artery branches off the aorta (white arrow: thoracic duct cyst) Fig. 4 a In the right thoracic cavity, the tumor is continuous from the caudal side of the tracheal bifurcation to the diaphragm. b To prevent chyle leakage, a vessel-sealing system is used to dissect the tumor perimeter, and the cord-like material is securely clipped and detached. c Tumor is multifocal and connected in a bead-like pattern. d Left side of the aortic hiatus is opened from the left thoracic cavity, the tumor is dissected from the aorta, and the caudal margin of the tumor is reached. At the caudal margin, clipping is performed, and the dissected specimen is removed Fig. 5 Segmental tumor approximately 20 × 180 mm in size Fig. 6 a Loupe image of hematoxylin and eosin staining. Cystic structures with fibrous walls are present. b Loupe image of α-smooth muscle actin (α-SMA) staining. Smooth muscle stained with α-SMA around the cystic structures	4.019531	0.973633	sec[1]/p[0]	en	0.999996	37747542	https://doi.org/10.1186/s40792-023-01740-6	[ "tumor", "side", "thoracic", "intercostal", "cystic", "space", "port", "duct", "position", "cavity" ]	[ { "code": "2F9Z", "title": "Neoplasms of unknown behaviour of unspecified site" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "2E6Z", "title": "Carcinoma in situ of unspecified site" }, { "code": "2F91.1", "title": "Neoplasms of unknown behaviour of trachea, bronchus or lung" }, { "code": "2F92", "title": "Neoplasms of unknown behaviour of skin" }, { "code": "NE60", "title": "Harmful effects of drugs, medicaments or biological substances, not elsewhere classified" }, { "code": "LA8B.2Y", "title": "Other specified congenital anomaly of aorta or its branches" }, { "code": "LB9A.6", "title": "Split foot" }, { "code": "LA80.0", "title": "Laevocardia" }, { "code": "LA80.1", "title": "Dextrocardia" } ]	=== ICD-11 CODES FOUND === [2F9Z] Neoplasms of unknown behaviour of unspecified site Also known as: Neoplasms of unknown behaviour of unspecified site \| neoplasia \| neoplasm growth NOS \| tumour of unspecified site \| tumourlet of unspecified site [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [2E6Z] Carcinoma in situ of unspecified site Also known as: Carcinoma in situ of unspecified site \| carcinoma in situ of unspecified site \| carcinoma in situ NOS \| carcinoma in situ \| in situ neoplasm [2F91.1] Neoplasms of unknown behaviour of trachea, bronchus or lung Also known as: Neoplasms of unknown behaviour of trachea, bronchus or lung \| trachea, bronchus or lung tumour NOS \| Intravascular bronchial alveolar tumour of unspecified site \| Bronchial adenoma of unknown behaviour of unspecified site \| Intravascular bronchial alveolar tumour unknown behaviour of unspecified site [2F92] Neoplasms of unknown behaviour of skin Also known as: Neoplasms of unknown behaviour of skin \| skin tumour NOS [NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified Also known as: Harmful effects of drugs, medicaments or biological substances, not elsewhere classified \| drugs, medicaments or biological substances, toxicity not elsewhere classified \| adverse drug effects \| drug reaction NOS \| drug allergy NOS Excludes: Alcohol intoxication \| pathological drug intoxication \| hypersensitivity reaction to correctly administered drug [LA8B.2Y] Other specified congenital anomaly of aorta or its branches Also known as: Other specified congenital anomaly of aorta or its branches \| Congenital anomaly of ascending aorta \| Hypoplasia of ascending aorta \| Congenital ascending aorta aneurysm or dilation \| congenital ascending aortic aneurysm or dilation [LB9A.6] Split foot Definition: A condition caused by malformation of the foot during the antenatal period. This condition is characterised by a deep median cleft of the foot due to the absence of the central rays. Also known as: Split foot \| lobster claw foot \| split foot, unspecified side \| cleft of foot \| Split foot, unilateral [LA80.0] Laevocardia Definition: A congenital cardiovascular finding in which the heart is predominantly to the left of the thoracic midline. Also known as: Laevocardia \| Left-sided heart \| Levocardia [LA80.1] Dextrocardia Definition: A congenital cardiovascular malformation in which the heart is predominantly to the right of the thoracic midline. This is independent of the orientation of the cardiac apex. Also known as: Dextrocardia \| heart in right chest \| right-sided heart \| congenital dextrocardia of heart \| transposition of heart Excludes: Isomerism of left atrial appendages \| Isomerism of right atrial appendages \| Total mirror imagery === GRAPH WALKS === --- Walk 1 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2F92] Neoplasms of unknown behaviour of skin --- Walk 2 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --PARENT--> [02] Neoplasms Def: An abnormal or uncontrolled cellular proliferation which is not coordinated with an organism's requirements for normal tissue growth, replacement or repair.... --- Walk 3 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Breast lump or mass female --PARENT--> [?] Symptoms, signs or clinical findings involving the female genital system --- Walk 4 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Breast lump or mass female --PARENT--> [?] Symptoms, signs or clinical findings involving the female genital system --- Walk 5 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E62] Carcinoma in situ of middle ear or respiratory system --- Walk 6 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs	[ "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2F92] Neoplasms of unknown behaviour of skin", "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --PARENT--> [02] Neoplasms\n Def: An abnormal or uncontrolled cellular proliferation which is not coordinated with an organism's requirements for normal tissue growth, replacement or repair....", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Breast lump or mass female\n --PARENT--> [?] Symptoms, signs or clinical findings involving the female genital system", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Breast lump or mass female\n --PARENT--> [?] Symptoms, signs or clinical findings involving the female genital system", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E62] Carcinoma in situ of middle ear or respiratory system", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs" ]	2F9Z	Neoplasms of unknown behaviour of unspecified site	[ { "from_icd11": "2F9Z", "icd10_code": "D487", "icd10_title": "Neoplasm of uncertain behavior of other specified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D482", "icd10_title": "Neoplasm of uncertain behavior of peripheral nerves and autonomic nervous system" }, { "from_icd11": "2F9Z", "icd10_code": "D37-D48", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D377", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D48", "icd10_title": "Neoplasm of uncertain behavior of other and unspecified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D489", "icd10_title": "Neoplasm of uncertain behavior, unspecified" }, { "from_icd11": "ME61", "icd10_code": "R2240", "icd10_title": "Localized swelling, mass and lump, unspecified lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2232", "icd10_title": "Localized swelling, mass and lump, left upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2242", "icd10_title": "Localized swelling, mass and lump, left lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2231", "icd10_title": "Localized swelling, mass and lump, right upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2241", "icd10_title": "Localized swelling, mass and lump, right lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2233", "icd10_title": "Localized swelling, mass and lump, upper limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2243", "icd10_title": "Localized swelling, mass and lump, lower limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2230", "icd10_title": "Localized swelling, mass and lump, unspecified upper limb" }, { "from_icd11": "ME61", "icd10_code": "R220", "icd10_title": "Localized swelling, mass and lump, head" } ]	D487	Neoplasm of uncertain behavior of other specified sites
A European male, long-hair cat, approximately 9 months old, was brought to the veterinary clinic. Since adoption, the cat exhibited rapid breathing, at an average rate of 45/min at rest and up to 70/min after exertion. The patient had previously undergone an echocardiographic examination, revealing an interventricular septal defect. Treatment with 2 mg/kg of sildenafil (Viagra, Pfizer, Sandwich, UK) twice daily and 2 mg/kg of furosemide (Furosoral, Le Vet. Beheer B.V., Oudewater, The Netherlands) twice daily was initiated. On clinical examination, tachypnoea was noted, but no heart murmurs were detected. Other parameters were within reference intervals. Transthoracic echocardiography revealed an atrial septal defect (ASD) of type I and type II , interventricular septal defect filled with thick, short tendinous cords, and a common atrioventricular valve with regurgitation . Significant enlargement of the right ventricle and right atrium was observed. A diagnosis of AVC defect type A—transitional form, with equal valve distribution between both ventricles, was made. An additional muscular defect in the IVS was visualized. The right-to-left shunt velocity through the ventricular septal defect (VSD) was 0.8 m/s, as visualized by color flow mapping (CFM), indicating equalized pressures in both ventricles, which suggests PH. Sildenafil dose was increased to 3 mg/kg twice daily, and furosemide was changed to torasemide (Upcard, Vétoquinol SA, Paris, France) at 0.3 mg/kg once daily due to difficulties in drug administration. Despite pharmacotherapy, the patient’s condition deteriorated—tachypnoea intensified and ascites developed. The decision was made to proceed with surgical pulmonary artery banding (PAB). On the day of surgery, the patient was in critical condition, with dyspnea and ascites. Before surgery, approximately 500 mL of hemorrhagic fluid was evacuated through abdominocentesis. Furosemide (Furosemidum, Polpharma, Starogard Gdański, Poland) at 4 mg/kg was administered intravenously; oxygen therapy was initiated in an oxygen chamber with a fraction of inspired oxygen (FiO 2 ) of 70%. After stabilizing the patient’s condition, surgery was performed. The anesthetic protocol included fentanyl (Fentadon, Dechra, Shrewsbury, UK) at 3 µg/kg IV in a single bolus, midazolam (Dormazolam, Dechra, Shrewsbury, UK) at 0.2 mg/kg IV, alfaxalone (Alfaxan Multidose, Zoetis, Parsippany, New York, USA) at 1.5 mg/kg IV for induction, propofol (Propomitor, Orion Pharma, Warsaw, Poland) at 5 mg/kg IV for induction, and isoflurane (Isoflurin, Geulincx, Tanowo, Poland) for maintenance anesthesia. The percentage of isoflurane vaporized in oxygen during the maintenance of anesthesia ranged from 1.0% to 2.0%, and local anesthesia was used with a serratus plane block using bupivacaine (Bupivacainum Hydrochloricum WZF 0.5%, Polpharma, Starogard Gdański, Poland) at 1.75 mg/kg under ultrasound guidance. A surgical approach was performed in the fifth left intercostal space. After retracting the left cranial lung lobe, the phrenic nerve was isolated. An incision was made in the pericardial sac along the visible pulmonary arterial trunk. The isolation of the trunk from ascending aorta was conducted using angled vascular forceps and Metzenbaum scissors. A Gore-Tex band, approximately 4 mm wide, prepared from a vascular patch (W. L. Gore & Associates, Inc., Newark, NJ, USA), was placed around the trunk . The vessel lumen was gradually reduced by applying sequential titanium vascular clips. The Gore-Tex band was then sutured to the vessel adventitia to prevent it from migrating towards the bifurcation of the pulmonary arterial trunk. The resulting vessel lumen was estimated to be approximately 50% of the original diameter of the pulmonary arterial trunk. The pericardial sac was not sutured. The lung was repositioned, and the chest wall closure was performed. The blood flow velocity in the pulmonary artery (v) was measured using continuous-wave Doppler, yielding a result of 3.8 m/s. This indicates a pressure gradient of 57 mmHg across the banding, i.e., between the right ventricular cavity and peripheral pulmonary arteries (according to the simplified Bernoulli equation: delta ∆P = 4 × v2). This level of increase of the central pulmonary pressure effectively decreases the pressure difference between both ventricles resulting in a decrease in shunting. The echocardiographic parameters before and after PAB are summarized in Table 1 . The patient was hospitalized for 3 days after the procedure. The cat’s condition was stable, with a maintained appetite and normal body temperature; dyspnea resolved. A continuous rate infusion of fentanyl and ketamine (Vetaketam, Vet-agro, Lublin, Poland) was continued for the first day, then the dose was tapered by half, followed by switching to oral transmucosal buprenorphine (Bunondol, Polfa Warszawa S.A, Warsaw, Poland) every 8 h. Pain management also included the administration of meloxicam (Metacam, Boehringer Ingelheim Vetmedica GmbH, Ingelheim/Rhein, Germany) at a dose of 0.05 mg/kg subcutaneously (SC) and gabapentin (Neurontin, Pfizer, Sanwich, UK) at a dose of 15 mg/kg per os (PO). Sildenafil was continued at a dose of 2 mg/kg 3 times per day. On the second day post-surgery, the patient required abdominocentesis—400 mL of hemorrhagic fluid was evacuated. The patient was discharged from the hospital on the third day after the surgery. An echocardiographic follow-up 3 weeks post-operation revealed stenosis of the pulmonary trunk with an unchanged blood flow velocity of 3.8 m/s , as in the postoperative control immediately after the surgery, with a significant reduction in the size of the right atrium and right ventricle and a visible VSD with bidirectional low-velocity flow . As of the manuscript publication, the patient is thirteen months post-surgery. No pharmacotherapy is necessary. Fluid does not accumulate in body cavities. The cat has a normal appetite and exhibits behavior typical of a healthy individual—it runs and jumps. In four follow-up echocardiographic studies conducted on the day of surgery, 3 weeks, 2 months, and 9 months after surgery, the results were consistent with those obtained immediately after the procedure. Specifically, the blood flow through the pulmonary artery remained at 3.5–3.8 m/s, and there was a noticeable reduction in the size of the right atrium and right ventricle, as well as a decrease in the defect in the muscular part. The cat has remained in stable condition without the need for pharmacotherapy for 12 months post-surgery and is still alive at the time of reporting.	4.039063	0.96875	sec[1]/p[0]	en	0.999997	PMC11988142	https://doi.org/10.3390/ani15071044	[ "pulmonary", "defect", "poland", "trunk", "flow", "approximately", "echocardiographic", "septal", "daily", "velocity" ]	[ { "code": "CB40.Y", "title": "Other specified diseases of the respiratory system" }, { "code": "LA75.1", "title": "Agenesis of lung" }, { "code": "CA40.Z", "title": "Pneumonia, organism unspecified" }, { "code": "CB41", "title": "Respiratory failure" }, { "code": "NB32.3Y", "title": "Other injury of lung" }, { "code": "BE2Z", "title": "Diseases of the circulatory system, unspecified" }, { "code": "LD9Z", "title": "Developmental anomalies, unspecified" }, { "code": "5D2Z", "title": "Metabolic disorders, unspecified" }, { "code": "MC1Y", "title": "Other specified symptoms or signs involving the visual system" }, { "code": "AB52", "title": "Deafness not otherwise specified" } ]	=== ICD-11 CODES FOUND === [CB40.Y] Other specified diseases of the respiratory system Also known as: Other specified diseases of the respiratory system \| Secondary respiratory disorders \| Respiratory disorders in diseases classified elsewhere \| Diseases of bronchus, not elsewhere classified \| Acquired bronchial diverticulum [LA75.1] Agenesis of lung Definition: This refers to the absence or rudimentary residua of an undeveloped lung. Also known as: Agenesis of lung \| Pulmonary agenesis \| absence of lung \| aplasia of lung \| apulmonism [CA40.Z] Pneumonia, organism unspecified Also known as: Pneumonia, organism unspecified \| Pneumonia \| infectious pneumonia \| PN - [pneumonia] \| lobar pneumonia NOS [CB41] Respiratory failure Definition: Respiratory failure is a life-threatening impairment of oxygenation or carbon dioxide (CO2) elimination. Respiratory failure may occur because of impaired gas exchange, decreased ventilation, or both. The level of oxygen in the blood becomes dangerously low or the level of carbon dioxide becomes dangerously high. Also known as: Respiratory failure \| lung failure NOS \| pulmonary failure Excludes: Acute respiratory distress syndrome \| Respiratory arrest \| Respiratory distress of newborn [NB32.3Y] Other injury of lung Also known as: Other injury of lung \| Haematoma of lung \| Traumatic hydropneumothorax \| Acute traumatic lung congestion \| Rupture of lung [BE2Z] Diseases of the circulatory system, unspecified Also known as: Diseases of the circulatory system, unspecified \| circulatory disease NOS \| cardiovascular disease NOS \| cardiovascular system disease NOS \| CVS - [cardiovascular system] disease [LD9Z] Developmental anomalies, unspecified Also known as: Developmental anomalies, unspecified \| congenital malformations, deformations and chromosomal abnormalities \| congenital malformation NOS \| developmental abnormality NOS \| fetal abnormality NOS [5D2Z] Metabolic disorders, unspecified Also known as: Metabolic disorders, unspecified \| metabolic abnormality \| metabolic debility \| metabolic defect \| metabolic disruption [MC1Y] Other specified symptoms or signs involving the visual system Also known as: Other specified symptoms or signs involving the visual system \| Erythema of eyelid \| Visual disturbances \| disturbances of vision \| difficulty seeing [AB52] Deafness not otherwise specified Also known as: Deafness not otherwise specified \| deaf \| hearing loss NOS \| hearing impairment \| hearing loss === GRAPH WALKS === --- Walk 1 --- [CB40.Y] Other specified diseases of the respiratory system --PARENT--> [CB40] Certain diseases of the respiratory system --RELATED_TO--> [?] Airway obstruction in the neonate due to airway abnormality --- Walk 2 --- [CB40.Y] Other specified diseases of the respiratory system --PARENT--> [CB40] Certain diseases of the respiratory system --CHILD--> [CB40.1] Young syndrome Def: Young syndrome is characterised by the association of obstructive azoospermia with recurrent sinobronchial infections.... --- Walk 3 --- [LA75.1] Agenesis of lung Def: This refers to the absence or rudimentary residua of an undeveloped lung.... --PARENT--> [LA75] Structural developmental anomalies of lungs Def: Any condition caused by failure of the lungs to correctly develop during the antenatal period.... --CHILD--> [LA75.1] Agenesis of lung Def: This refers to the absence or rudimentary residua of an undeveloped lung.... --- Walk 4 --- [LA75.1] Agenesis of lung Def: This refers to the absence or rudimentary residua of an undeveloped lung.... --PARENT--> [LA75] Structural developmental anomalies of lungs Def: Any condition caused by failure of the lungs to correctly develop during the antenatal period.... --PARENT--> [?] Structural developmental anomalies of the respiratory system --- Walk 5 --- [CA40.Z] Pneumonia, organism unspecified --PARENT--> [CA40] Pneumonia Def: A disease of the lungs, frequently but not always caused by an infection with bacteria, virus, fungus, or parasite. This disease is characterised by fever, chills, cough with sputum production, chest ... --EXCLUDES--> [?] Pneumonitis Def: Pneumonitis is a general term that refers to inflammation of lung tissue. Pneumonitis includes the non-infectious lung diseases that cause inflammation of the interstitium of the lung tissue mainly.... --- Walk 6 --- [CA40.Z] Pneumonia, organism unspecified --PARENT--> [CA40] Pneumonia Def: A disease of the lungs, frequently but not always caused by an infection with bacteria, virus, fungus, or parasite. This disease is characterised by fever, chills, cough with sputum production, chest ... --RELATED_TO--> [?] Severe acute respiratory syndrome Def: A disease of the respiratory system, caused by an infection with coronavirus. This disease is characterised by fever, headache, cough, myalgia, tachycardia, or diarrhoea. This disease may also lead to...	[ "[CB40.Y] Other specified diseases of the respiratory system\n --PARENT--> [CB40] Certain diseases of the respiratory system\n --RELATED_TO--> [?] Airway obstruction in the neonate due to airway abnormality", "[CB40.Y] Other specified diseases of the respiratory system\n --PARENT--> [CB40] Certain diseases of the respiratory system\n --CHILD--> [CB40.1] Young syndrome\n Def: Young syndrome is characterised by the association of obstructive azoospermia with recurrent sinobronchial infections....", "[LA75.1] Agenesis of lung\n Def: This refers to the absence or rudimentary residua of an undeveloped lung....\n --PARENT--> [LA75] Structural developmental anomalies of lungs\n Def: Any condition caused by failure of the lungs to correctly develop during the antenatal period....\n --CHILD--> [LA75.1] Agenesis of lung\n Def: This refers to the absence or rudimentary residua of an undeveloped lung....", "[LA75.1] Agenesis of lung\n Def: This refers to the absence or rudimentary residua of an undeveloped lung....\n --PARENT--> [LA75] Structural developmental anomalies of lungs\n Def: Any condition caused by failure of the lungs to correctly develop during the antenatal period....\n --PARENT--> [?] Structural developmental anomalies of the respiratory system", "[CA40.Z] Pneumonia, organism unspecified\n --PARENT--> [CA40] Pneumonia\n Def: A disease of the lungs, frequently but not always caused by an infection with bacteria, virus, fungus, or parasite. This disease is characterised by fever, chills, cough with sputum production, chest ...\n --EXCLUDES--> [?] Pneumonitis\n Def: Pneumonitis is a general term that refers to inflammation of lung tissue. Pneumonitis includes the non-infectious lung diseases that cause inflammation of the interstitium of the lung tissue mainly....", "[CA40.Z] Pneumonia, organism unspecified\n --PARENT--> [CA40] Pneumonia\n Def: A disease of the lungs, frequently but not always caused by an infection with bacteria, virus, fungus, or parasite. This disease is characterised by fever, chills, cough with sputum production, chest ...\n --RELATED_TO--> [?] Severe acute respiratory syndrome\n Def: A disease of the respiratory system, caused by an infection with coronavirus. This disease is characterised by fever, headache, cough, myalgia, tachycardia, or diarrhoea. This disease may also lead to..." ]	CB40.Y	Other specified diseases of the respiratory system	[ { "from_icd11": "LA75.1", "icd10_code": "Q333", "icd10_title": "Agenesis of lung" }, { "from_icd11": "CA40.Z", "icd10_code": "J189", "icd10_title": "Pneumonia, unspecified organism" }, { "from_icd11": "CA40.Z", "icd10_code": "J181", "icd10_title": "Lobar pneumonia, unspecified organism" }, { "from_icd11": "CA40.Z", "icd10_code": "J188", "icd10_title": "Other pneumonia, unspecified organism" }, { "from_icd11": "CA40.Z", "icd10_code": "J168", "icd10_title": "Pneumonia due to other specified infectious organisms" }, { "from_icd11": "CA40.Z", "icd10_code": "J180", "icd10_title": "Bronchopneumonia, unspecified organism" }, { "from_icd11": "CA40.Z", "icd10_code": "J17", "icd10_title": "Pneumonia in diseases classified elsewhere" }, { "from_icd11": "CA40.Z", "icd10_code": "J182", "icd10_title": "Hypostatic pneumonia, unspecified organism" }, { "from_icd11": "CA40.Z", "icd10_code": "J16", "icd10_title": "Pneumonia due to other infectious organisms, not elsewhere classified" }, { "from_icd11": "CA40.Z", "icd10_code": "J171", "icd10_title": "" }, { "from_icd11": "CA40.Z", "icd10_code": "J173", "icd10_title": "" }, { "from_icd11": "CA40.Z", "icd10_code": "J178", "icd10_title": "" }, { "from_icd11": "CA40.Z", "icd10_code": "J18", "icd10_title": "Pneumonia, unspecified organism" }, { "from_icd11": "CB41", "icd10_code": "J9622", "icd10_title": "Acute and chronic respiratory failure with hypercapnia" }, { "from_icd11": "CB41", "icd10_code": "J9620", "icd10_title": "Acute and chronic respiratory failure, unspecified whether with hypoxia or hypercapnia" } ]	Q333	Agenesis of lung
A 45-year-old female presented to surgical oncology outpatient with a chief complaint of abdominal mass and per vaginal bleed for 4 months. As stated by the patient, she had undergone an abdominal surgical exploration before 7 months to remove pelvic mass. The patient also gave history of total abdominal hysterectomy before 3 years in view of abnormal uterine bleeding. However, the patient did not have any documentation regarding the abovementioned surgeries. The patient did not have any significant personal and family history. Clinical examination general condition was fair; vitals were stable; pallor was present; on per abdominal examination, a large, soft abdominopelvic mass of 15 x 15 cm in size was found, which was fixed, non-tender, not moving with respiration and was more deviated towards the left side. On per speculum examination, the vault was found replaced by a big smooth growth occupying upper 2/3rd of the vagina, but no vaginal invasion was present. On per vaginum, a smooth lobular abdominopelvic mass of around 15 x 15 cm was felt arising from vault. Routine biochemical investigations were within normal limit, except for hemoglobin level, which was 9gm%. An MRI was done which showed ill-defined heterogeneously enhancing mass lesion noted involving the uterus and cervix, measuring 9.3 x 9.2 x 16 cm (AP X TR X CC). The lesion was invading adjacent myometrium, reaching up to serosa in the fundal region. It was also infiltrating the bilateral adnexa, but bilateral ovaries were not separately visualized. Inferiorly involvement extended till the upper 1/3 of the vagina. Anteriorly, the lesion was closely abutting the urinary bladder with suspicious loss of fat planes at places. Enlarged and necrotic common iliac lymph nodes were noted, largest measuring 1.1 cm. Multiple omental and peritoneal deposits were noted, largest measuring 4.2 x 2.9 cm. There was no ascites and blood inside the peritoneal cavity. MRI suggested a probable diagnosis of endometrial carcinoma with extensions . An USG-guided biopsy was taken from the pelvic mass, which on histopathological examination, showed cores of fibrous tissue along with the presence of cells in sheets, micro-papillae, perivascularly arranged, and were polygonal with well-defined cytoplasmic borders, granular eosinophilic cytoplasm, central to eccentric round nuclei . Few cells showed nuclear inclusion, occasional psammomatous calcification which suggest of neoplastic etiology. An immunohistochemistry (IHC) panel was requested which revealed TFE-3 score 3+, H Caldesmon score 1+, GATA-3 score 1+, Melan A-score 4+, and HMB-45, score 4 +. Ki 67-positivity was seen in 30–35% of tumor cells . On the basis of histopathological and IHC analysis, the diagnosis of perivascular tumor with melanocytic differentiation was made and the possibility of TFE 3 mutation associated with PEComa was favoured. In view of advanced nature of disease, and due to rarity of cases with no standard guidelines for the management of uterine pecoma, decision for chemotherapy was taken and the patient was started on mTOR inhibitor, everolimus 10 mg OD. She was kept on monthly follow-up, in every visit, she was symptomatically improved. After 3 months of therapy, she was again examined, and there was no mass/lump palpable, on per abdomen and per vaginum examination. Repeat MRI pelvis was done which showed nearly resolution of pre-existing mass with decreased signal intensity showing nonviable remnants . As per RECIST (Response Evaluation Criteria in Solid Tumors) criteria, tumor shows partial response with mTOR inhibitors within 3 months of therapy. She was again followed after 6 months of therapy, there was again no mass/lump palpable on examination, MRI was repeated again showing no evidence of any solid mass lesion or altered enhancement noted, and urinary bladder (straight long arrow) and rectum (straight short arrow) can now be clearly seen without compression in the pelvis , suggesting a complete response as per RECIST criteria. Multiple ROI (region of interest) values were acquired at anatomically distinct areas of the tumor before and after CT on MRI. The correlation of ROI over serial scans was done by an approximate visual assessment. It was noted that there was elevation of ADC (apparent diffusion coefficient) in pre- and post-chemotherapies MRI (819.2mm 2 /s-951 mm 2 /s), indicating towards good response (viz. facilitation due to apoptosis and increased vasogenic edema; however, the last scan showed reduction in mean ADC values (789mm 2 /s), most probably due to overall tumor volume on ROI becomes negligible and probably due to post CT fibrosis. Till now date patient is following with us, and she is continuing her everolimus, with no recurrence of lesion after 1 year of therapy. Fig. 1 (1) Pre-chemotherapy ( A ) and post-chemotherapy ( B ) axial MRI sections of the pelvis at the level of the third sacral vertebrae. Sequences have been taken with high TE and TR values (viz. T2 weighting). The pretherapy scan ( A ) shows the bulky multilobulated mass (straight white arrow) with multiple satellite lesions (straight black arrow). On post therapy scan after 3 months ( B ), the main lesion shows near complete regression (straight white arrow) while the satellite lesions which were showing cystic degeneration (straight black arrow) have converted to much smaller hemorrhagic cysts (note the fluid-fluid level within the cyst). Also note that the urinary bladder (curved white arrow) and the rectum (curved black arrow) which were grossly compressed by the mass in the pre-therapy scan could be well visualized in the post therapy scan, confirming the regression of the tumor. Also note the reduction of signal intensity in after treatment signifying that the remaining tissue may just be the non-viable fibrotic tumoral remnant. (2) On post therapy scan after 6 months, there was no evidence of any solid mass lesion or altered enhancement noted, urinary bladder( straight long arrow) and rectum(straight short arrow) can now be clearly seen without compression. Fig. 2 On histopathological examination. A The cores of the fibrous tissue along with the presence of cells in sheets; B 3 the polygonal cells with well-defined cytoplasmic borders, granular eosinophilic cytoplasm, and central to eccentric round nuclei; and 3 C the presence of cells in sheets, micro-papillae, and perivascularly arrangement Fig. 3 A Strong expression of HMB-45, Score 4+ (40x); B strong expression of MELAN A Score 4 + (40x); C Strong expression of TFE-3, Score 3 + (40x); and D -Ki-67 positivity seen in 30–35% of tumour cells	4.039063	0.977539	sec[1]/p[0]	en	0.999997	35232443	https://doi.org/10.1186/s12957-021-02462-5	[ "arrow", "which", "score", "straight", "lesion", "cells", "tumor", "scan", "abdominal", "urinary" ]	[ { "code": "BD50.41", "title": "Abdominal aortic aneurysm with rupture" }, { "code": "EK91", "title": "Dermatoses which may presage cutaneous lymphoma" }, { "code": "MH12.1", "title": "Death occurring less than 24 hours from onset of symptoms, not otherwise explained" }, { "code": "8A44.3", "title": "Certain specified leukodystrophies" }, { "code": "MB20.1&XC87", "title": "Glasgow Coma Scale, eyes opening, never" }, { "code": "KD30.0", "title": "Birth depression with 5 minute Apgar score 0-3" }, { "code": "KD30.1", "title": "Birth depression with 5 minute Apgar score 4-6" }, { "code": "MB20.1", "title": "Coma" }, { "code": "KB21.0", "title": "Severe birth asphyxia" }, { "code": "8B22.1&XA03D2", "title": "Cerebral venous thrombosis of straight sinus" } ]	=== ICD-11 CODES FOUND === [BD50.41] Abdominal aortic aneurysm with rupture Also known as: Abdominal aortic aneurysm with rupture \| abdominal aorta aneurysm rupture \| abdominal aorta aneurysm ruptured \| abdominal aortic aneurysm which has ruptured \| ruptured AAA [EK91] Dermatoses which may presage cutaneous lymphoma Definition: Dermatoses which may represent the earliest stages of cutaneous lymphoma but where it is not possible to confirm their neoplastic nature. Also known as: Dermatoses which may presage cutaneous lymphoma [MH12.1] Death occurring less than 24 hours from onset of symptoms, not otherwise explained Also known as: Death occurring less than 24 hours from onset of symptoms, not otherwise explained \| died without sign of disease \| Death known not to be violent or instantaneous for which no cause can be discovered \| death known not to be violent or instantaneous, cause unknown \| Death without sign of disease Includes: Death known not to be violent or instantaneous for which no cause can be discovered \| Death without sign of disease [8A44.3] Certain specified leukodystrophies Also known as: Certain specified leukodystrophies \| CACH syndrome \| Vanishing white matter disease \| Childhood ataxia with central nervous system hypomyelination \| Congenital or early infantile CACH syndrome [KD30.0] Birth depression with 5 minute Apgar score 0-3 Definition: A condition characterised by cardiorespiratory and neurological depression, defined as an Apgar score between 0 to 3 at 5 minutes following birth. Also known as: Birth depression with 5 minute Apgar score 0-3 [KD30.1] Birth depression with 5 minute Apgar score 4-6 Definition: A condition characterised by cardiorespiratory and neurological depression, defined as an Apgar score between 4 and 6 at 5 minutes following birth. Also known as: Birth depression with 5 minute Apgar score 4-6 [MB20.1] Coma Definition: Acute state lasting more than one hour and usually less than a month. The comatose patient is unresponsive, lying with their eyes closed and cannot be aroused even by vigorous and noxious stimuli. Motor responses to noxious stimulation are limited to reflexive behaviour. Etiologies include but are not limited to traumatic, anoxic, infectious, neoplastic, vascular, inflammatory and metabolic brain injuries. Also known as: Coma \| comatose \| exanimation \| Coma, NOS \| Unconsciousness, NOS Excludes: Diabetic coma \| Hepatic coma \| Neonatal coma [KB21.0] Severe birth asphyxia Definition: Pulse less than 100 per minute at birth and falling or steady, respiration absent or gasping, colour poor, tone absent. Also known as: Severe birth asphyxia \| severe perinatal hypoxia \| asphyxia pallida of newborn \| Asphyxia with 5-minute Apgar score 0-3 \| newborn severe asphyxia === GRAPH WALKS === --- Walk 1 --- [BD50.41] Abdominal aortic aneurysm with rupture --PARENT--> [BD50.4] Abdominal aortic aneurysm --CHILD--> [BD50.41] Abdominal aortic aneurysm with rupture --- Walk 2 --- [BD50.41] Abdominal aortic aneurysm with rupture --PARENT--> [BD50.4] Abdominal aortic aneurysm --CHILD--> [BD50.4Z] Abdominal aortic aneurysm, without mention of perforation or rupture --- Walk 3 --- [EK91] Dermatoses which may presage cutaneous lymphoma Def: Dermatoses which may represent the earliest stages of cutaneous lymphoma but where it is not possible to confirm their neoplastic nature.... --CHILD--> [EK91.0] Large plaque parapsoriasis Def: Large plaque parapsoriasis is a chronic skin disorder characterised by the indolent development over years or decades of scaly patches or slightly elevated plaques which may be clinically indistinguis... --PARENT--> [EK91] Dermatoses which may presage cutaneous lymphoma Def: Dermatoses which may represent the earliest stages of cutaneous lymphoma but where it is not possible to confirm their neoplastic nature.... --- Walk 4 --- [EK91] Dermatoses which may presage cutaneous lymphoma Def: Dermatoses which may represent the earliest stages of cutaneous lymphoma but where it is not possible to confirm their neoplastic nature.... --CHILD--> [EK91.1] Poikiloderma vasculare atrophicans Def: Poikiloderma vasculare atrophicans is a cutaneous reaction pattern characterised by mottled hyper- and hypomelanosis, telangiectasia and progressive dermal and epidermal atrophy. It may manifest as a ... --PARENT--> [EK91] Dermatoses which may presage cutaneous lymphoma Def: Dermatoses which may represent the earliest stages of cutaneous lymphoma but where it is not possible to confirm their neoplastic nature.... --- Walk 5 --- [MH12.1] Death occurring less than 24 hours from onset of symptoms, not otherwise explained --PARENT--> [MH12] Other sudden death, cause unknown --EXCLUDES--> [?] Sudden infant death syndrome Def: Sudden infant death syndrome is the abrupt and unexplained death of an apparently healthy infant under one year of age, remaining unexplained after a thorough case investigation, including performance... --- Walk 6 --- [MH12.1] Death occurring less than 24 hours from onset of symptoms, not otherwise explained --PARENT--> [MH12] Other sudden death, cause unknown --CHILD--> [MH12.0] Instantaneous death	[ "[BD50.41] Abdominal aortic aneurysm with rupture\n --PARENT--> [BD50.4] Abdominal aortic aneurysm\n --CHILD--> [BD50.41] Abdominal aortic aneurysm with rupture", "[BD50.41] Abdominal aortic aneurysm with rupture\n --PARENT--> [BD50.4] Abdominal aortic aneurysm\n --CHILD--> [BD50.4Z] Abdominal aortic aneurysm, without mention of perforation or rupture", "[EK91] Dermatoses which may presage cutaneous lymphoma\n Def: Dermatoses which may represent the earliest stages of cutaneous lymphoma but where it is not possible to confirm their neoplastic nature....\n --CHILD--> [EK91.0] Large plaque parapsoriasis\n Def: Large plaque parapsoriasis is a chronic skin disorder characterised by the indolent development over years or decades of scaly patches or slightly elevated plaques which may be clinically indistinguis...\n --PARENT--> [EK91] Dermatoses which may presage cutaneous lymphoma\n Def: Dermatoses which may represent the earliest stages of cutaneous lymphoma but where it is not possible to confirm their neoplastic nature....", "[EK91] Dermatoses which may presage cutaneous lymphoma\n Def: Dermatoses which may represent the earliest stages of cutaneous lymphoma but where it is not possible to confirm their neoplastic nature....\n --CHILD--> [EK91.1] Poikiloderma vasculare atrophicans\n Def: Poikiloderma vasculare atrophicans is a cutaneous reaction pattern characterised by mottled hyper- and hypomelanosis, telangiectasia and progressive dermal and epidermal atrophy. It may manifest as a ...\n --PARENT--> [EK91] Dermatoses which may presage cutaneous lymphoma\n Def: Dermatoses which may represent the earliest stages of cutaneous lymphoma but where it is not possible to confirm their neoplastic nature....", "[MH12.1] Death occurring less than 24 hours from onset of symptoms, not otherwise explained\n --PARENT--> [MH12] Other sudden death, cause unknown\n --EXCLUDES--> [?] Sudden infant death syndrome\n Def: Sudden infant death syndrome is the abrupt and unexplained death of an apparently healthy infant under one year of age, remaining unexplained after a thorough case investigation, including performance...", "[MH12.1] Death occurring less than 24 hours from onset of symptoms, not otherwise explained\n --PARENT--> [MH12] Other sudden death, cause unknown\n --CHILD--> [MH12.0] Instantaneous death" ]	BD50.41	Abdominal aortic aneurysm with rupture	[ { "from_icd11": "BD50.41", "icd10_code": "I713", "icd10_title": "Abdominal aortic aneurysm, ruptured" }, { "from_icd11": "EK91", "icd10_code": "L989", "icd10_title": "Disorder of the skin and subcutaneous tissue, unspecified" }, { "from_icd11": "MH12.1", "icd10_code": "R961", "icd10_title": "" }, { "from_icd11": "KD30.0", "icd10_code": "P210", "icd10_title": "" }, { "from_icd11": "KD30.1", "icd10_code": "P211", "icd10_title": "" }, { "from_icd11": "MB20.1", "icd10_code": "R402142", "icd10_title": "Coma scale, eyes open, spontaneous, at arrival to emergency department" }, { "from_icd11": "MB20.1", "icd10_code": "R402362", "icd10_title": "Coma scale, best motor response, obeys commands, at arrival to emergency department" }, { "from_icd11": "MB20.1", "icd10_code": "R402252", "icd10_title": "Coma scale, best verbal response, oriented, at arrival to emergency department" }, { "from_icd11": "MB20.1", "icd10_code": "R402412", "icd10_title": "Glasgow coma scale score 13-15, at arrival to emergency department" }, { "from_icd11": "MB20.1", "icd10_code": "R4020", "icd10_title": "Unspecified coma" }, { "from_icd11": "MB20.1", "icd10_code": "R402141", "icd10_title": "Coma scale, eyes open, spontaneous, in the field [EMT or ambulance]" }, { "from_icd11": "MB20.1", "icd10_code": "R402361", "icd10_title": "Coma scale, best motor response, obeys commands, in the field [EMT or ambulance]" }, { "from_icd11": "MB20.1", "icd10_code": "R402251", "icd10_title": "Coma scale, best verbal response, oriented, in the field [EMT or ambulance]" }, { "from_icd11": "MB20.1", "icd10_code": "R402413", "icd10_title": "Glasgow coma scale score 13-15, at hospital admission" }, { "from_icd11": "MB20.1", "icd10_code": "R402143", "icd10_title": "Coma scale, eyes open, spontaneous, at hospital admission" } ]	I713	Abdominal aortic aneurysm, ruptured
Table 1 demonstrates cases of jejunal diverticulosis with diagnosis and management reported in the literature over the last 10 years. Comparing this case to others reported in literature, our patient was younger in age (mean age = 74). She presented with an acute abdomen, whereas most small bowel diverticula are asymptomatic. The diverticula were found on the mesenteric side of the bowel, the most common location for jejunal diverticulosis. In cases published in the last decade, only one report stated finding anti-mesenteric small bowel diverticula . Our patient was treated with an open surgical resection, as reported in the majority of the cases. Of note, there were reports of conservative treatment and laparoscopic resection , but not in patients with perforated diverticulitis presenting with an acute abdomen. Table 1 Characteristics and treatment of jejunal diverticulosis in cases published between 2010 and 2020 Paper Year Age Gender Preoperative diagnosis Distance from Trietz’s lig. (cm) Location No. of diverticula No. of perforations Type Diverticulitis Treatment Prough H 2019 65 M Diverticulitis NA Mesentery 1 0 NA Yes Open surgery with resection Gurala D 2019 70 F Small intestine diverticulitis NA NA 2 1 NA Yes Failed conservative, laparoscopic surgery with resection Mazahreh TS 2019 68 M Gastrointestinal bleed 10 NA Multiple 0 True and false No Open surgery with resection Fleres F 2018 88 F Unknown NA NA 3 1 NA Yes Open surgery with resection Fleres F 2018 86 F Volvulus 150 Mesentery Multiple 0 NA Yes Open surgery with resection Abdelbaki A 2018 65 F Small intestine diverticulitis 45 NA Multiple 1 NA Yes Open surgery with resection Abdelbaki A 2018 74 F Small intestine diverticulitis NA NA NA 0 NA Yes Conservative Abdelbaki A 2018 87 M Small intestine diverticulitis NA NA NA 0 NA Yes Conservative Syllaios A 2018 75 M Small intestine diverticulitis NA NA 6 0 False Yes Open surgery with resection Kagolanu DC 2018 91 M Small intestine diverticulitis NA NA Multiple 0 NA Yes Conservative Ejaz S 2017 76 M Small intestine diverticulitis NA NA Multiple NA NA Yes Conservative Ejaz S 2017 78 F Small intestine diverticulitis NA NA Multiple 0 NA Yes Conservative Ejaz S 2017 87 M Small intestine diverticulitis NA Mesentery Multiple NA NA Yes Conservative Grubbs J 2017 90 M Sigmoid diverticulitis NA NA Multiple 1 NA Yes Open surgery with resection Kumar D 2017 60 F Small intestine diverticulitis NA Mesentery Multiple 0 NA Yes Open surgery with resection Kumar D 2017 68 M Small intestine diverticulitis NA Mesentery Multiple 1 NA Yes Open surgery with resection Cui J 2017 65 F Small intestine diverticulitis NA Mesentery Multiple 0 NA Yes Failed conservative, laparoscopic surgery with resection Malghan L 2017 91 F Small intestine diverticulitis NA NA Multiple 0 NA Yes Open surgery with resection Karas L 2017 82 F Intestinal mass NA NA Multiple 0 NA Yes Open surgery with resection Karas L 2017 80 F Small intestine diverticulosis NA NA Multiple 0 NA Yes Laparoscopic surgery with resection Mohi RS 2016 62 M Volvulus NA Mesentery Multiple 0 NA Yes Open surgery with resection Aydin E 2016 69 M Small intestine diverticulitis 20 Mesentery Multiple 0 NA Yes Open surgery with resection Tenreiro N 2016 81 M Diverticulitis NA NA Multiple 1 NA Yes Failed conservative, open surgery with resection Ghrissi R 2016 72 M Small bowel obstruction NA NA Multiple 0 NA No Open surgery with resection Harbi H 2016 31 M Unknown NA NA Multiple 1 False Yes Open surgery with resection De Minicis S 2015 60 M Jejunal diverticula NA NA Multiple 0 NA Yes Conservative Natarajan K 2015 56 M Small intestine diverticulitis 8 Mesentery Multiple 3 False Yes Open surgery with resection Kassir R 2015 79 M Small intestine diverticulitis NA Mesentery Multiple 1 NA Yes Open surgery with resection Fidan N 2015 67 M Small intestine diverticulitis NA NA Multiple 0 NA Yes Conservative Levack MM 2014 77 M Small intestine diverticulum NA NA 1 1 NA No Conservative Xu XQ 2014 86 M Small intestine diverticulitis 50 NA Multiple NA NA Yes Open surgery with resection Fresow R 2014 73 M Small intestine diverticulitis 0 NA 3 0 NA Yes Open surgery, no resection Corcelles R 2014 63 F Intestinal perforation NA NA Multiple NA NA Yes Laparoscopic resection Ojili V 2014 75 M Small intestine diverticulitis NA NA NA NA NA Yes Open surgery with resection Zamani A 2013 63 F Small intestine diverticulitis 12 NA Multiple 1 NA Yes Open surgery with resection Aydin I 2013 74 F Small intestine diverticulitis 40–100 Mesentery Multiple 1 False Yes Open surgery with resection Singal R 2012 63 M Unknown NA NA Multiple 0 NA No Open surgery, no resection Ferreira-Aparicio FE 2012 65 F Appendicitis 0 NA Multiple Multiple NA Yes Open surgery with resection and ileostomy Ferrarese A 2012 92 F Intestinal perforation NA NA NA 1 NA Yes Open surgery with resection Garnet DJ 2011 80 M Small intestine diverticulitis NA NA Multiple 1 NA Yes Laparoscopic converted open surgery, with resection Tan KK 2011 88 M Gastrointestinal hemorrhage NA NA NA 0 NA No Open surgery with resection Tan KK 2011 72 M Gastrointestinal hemorrhage NA NA NA 0 NA No Open surgery with resection Tan KK 2011 84 M Gastrointestinal hemorrhage NA NA Multiple 0 NA No Open surgery with resection Tan KK 2011 70 M Intestinal inflammation NA NA Multiple 1 NA Yes Open surgery with resection Tan KK 2011 84 M Intestinal inflammation NA NA 1 1 NA Yes Open surgery with resection Tan KK 2011 75 M Intestinal inflammation NA NA Multiple 1 NA Yes Open surgery with resection Nonose R 2011 86 F Intestinal perforation 15–50 Anti-mesenteric Multiple 1 NA Yes Open surgery with resection Falidas E 2011 55 M Small intestine diverticulum and bowel obstruction NA NA Multiple 0 NA Yes Failed conservative, open surgery with resection Sakpal SV 2010 25 F Enteritis NA Mesentery 1 1 NA Yes Open surgery with resection França M 2010 75 M Small intestine diverticulitis NA Mesentery 3 1 NA Yes Open surgery with resection Vanrykel F 2010 79 F Small intestine diverticulitis NA NA Multiple 1 NA Yes Laparoscopic converted open surgery, with resection Chugay P 2010 89 F Small intestine diverticula NA NA Multiple NA NA No Failed conservative, open surgery with resection Chugay P 2010 79 M Small intestine perforation NA NA Multiple 1 NA Yes Open surgery with resection PubMed database was queried for studies published from January 1, 2010 to April 31, 2020, with English language restriction. Search strategy included the term “jejunal diverticulitis.” Case series lacking patient-specific data were excluded Lig ligament, No. number, NA not available, F female, M male	4.082031	0.895508	sec[2]/p[1]	en	0.999996	32632508	https://doi.org/10.1186/s40792-020-00929-3	[ "resection", "open", "multiple", "small", "diverticulitis", "intestine", "conservative", "mesentery", "laparoscopic", "intestinal" ]	[ { "code": "DA0E.5Y", "title": "Other specified malocclusion" }, { "code": "ME05.0", "title": "Constipation" }, { "code": "QB61.Z", "title": "Presence of artificial opening, unspecified" }, { "code": "LA8B.4", "title": "Patent arterial duct" }, { "code": "NA81.4", "title": "Open bite of thorax" }, { "code": "6B64", "title": "Dissociative identity disorder" }, { "code": "JA80.Z", "title": "Maternal care related to unspecified multiple gestation" }, { "code": "QA46.Z", "title": "Outcome of delivery, unspecified" }, { "code": "8A40.Z", "title": "Multiple sclerosis, unspecified" }, { "code": "ND31", "title": "Open wounds involving multiple body regions" } ]	=== ICD-11 CODES FOUND === [DA0E.5Y] Other specified malocclusion Also known as: Other specified malocclusion \| Crossbite \| overbite \| openbite \| excessive overbite Includes: Crossbite [ME05.0] Constipation Definition: Constipation is an acute or chronic condition in which bowel movements occur less often than usual or consist of hard, dry stools that are often painful or difficult to pass. Here constipation other than specifically described elsewhere, such as in motility disorders of intestine or in functional bowel diseases, is described. Also known as: Constipation \| faecal impaction \| constipated \| chronic constipation with overflow \| difficult passing motion Includes: faecal impaction Excludes: Functional constipation \| Functional constipation of infants, toddlers or children \| Atonic constipation [QB61.Z] Presence of artificial opening, unspecified Also known as: Presence of artificial opening, unspecified \| Presence of artificial opening \| artificial opening status \| Artificial opening status, unspecified [LA8B.4] Patent arterial duct Definition: A congenital cardiovascular finding in which the arterial duct (ductus arteriosus) is open beyond the normal age of spontaneous closure. Also known as: Patent arterial duct \| patent ductus Botalli \| patent ductus arteriosus NOS \| open ductus arteriosus \| persistent ductus arteriosus Includes: bilateral arterial ducts [NA81.4] Open bite of thorax Also known as: Open bite of thorax \| Open bite of front wall of thorax \| Open bite of back wall of thorax [6B64] Dissociative identity disorder Definition: Dissociative identity disorder is characterised by disruption of identity in which there are two or more distinct personality states (dissociative identities) associated with marked discontinuities in the sense of self and agency. Each personality state includes its own pattern of experiencing, perceiving, conceiving, and relating to self, the body, and the environment. At least two distinct personality states recurrently take executive control of the individual’s consciousness and functioning i Also known as: Dissociative identity disorder \| Multiple personality \| Multiple personality disorder [JA80.Z] Maternal care related to unspecified multiple gestation Also known as: Maternal care related to unspecified multiple gestation \| Maternal care related to multiple gestation \| multiple gestation, unspecified, unspecified trimester \| multiple pregnancy \| Multiple pregnancy NOS [QA46.Z] Outcome of delivery, unspecified Also known as: Outcome of delivery, unspecified \| Outcome of delivery \| Multiple birth, unspecified \| Single birth, unspecified [8A40.Z] Multiple sclerosis, unspecified Also known as: Multiple sclerosis, unspecified \| Multiple sclerosis \| cerebrospinal sclerosis \| disseminated sclerosis \| generalised multiple sclerosis [ND31] Open wounds involving multiple body regions Also known as: Open wounds involving multiple body regions \| Open wounds involving head with neck \| Open wounds of sites classifiable as open wounds to the head or open wounds of the neck \| Nasopharyngeal laceration \| Open wounds involving thorax with abdomen, lower back or pelvis Excludes: Traumatic amputations involving multiple body regions === GRAPH WALKS === --- Walk 1 --- [DA0E.5Y] Other specified malocclusion --PARENT--> [DA0E.5] Malocclusion Def: Malocclusion is the atypical relationship between maxillary and mandibular teeth which may interfere with the efficiency of excursive movements of the mandible that are essential for the effective mas... --PARENT--> [DA0E] Dentofacial anomalies Def: A congenital or acquired abnormality in which the dental and oral structures deviate from normal form, function, or position.... --- Walk 2 --- [DA0E.5Y] Other specified malocclusion --PARENT--> [DA0E.5] Malocclusion Def: Malocclusion is the atypical relationship between maxillary and mandibular teeth which may interfere with the efficiency of excursive movements of the mandible that are essential for the effective mas... --PARENT--> [DA0E] Dentofacial anomalies Def: A congenital or acquired abnormality in which the dental and oral structures deviate from normal form, function, or position.... --- Walk 3 --- [ME05.0] Constipation Def: Constipation is an acute or chronic condition in which bowel movements occur less often than usual or consist of hard, dry stools that are often painful or difficult to pass. Here constipation other t... --EXCLUDES--> [?] Functional constipation Def: Functional constipation is a functional bowel disorder that presents as persistently difficult, infrequent, or seemingly incomplete defecation, which do not meet IBS criteria.... --EXCLUDES--> [?] Constipation Def: Constipation is an acute or chronic condition in which bowel movements occur less often than usual or consist of hard, dry stools that are often painful or difficult to pass. Here constipation other t... --- Walk 4 --- [ME05.0] Constipation Def: Constipation is an acute or chronic condition in which bowel movements occur less often than usual or consist of hard, dry stools that are often painful or difficult to pass. Here constipation other t... --PARENT--> [ME05] Change in bowel habit Def: Bowel habits are the time, size, amount, consistency and frequency of bowel movements throughout the day. A change in bowel habits is any alteration in regular bowel habits.... --CHILD--> [ME05.Y] Other specified change in bowel habit --- Walk 5 --- [QB61.Z] Presence of artificial opening, unspecified --PARENT--> [QB61] Presence of artificial opening --CHILD--> [QB61.0] Presence of tracheostomy --- Walk 6 --- [QB61.Z] Presence of artificial opening, unspecified --PARENT--> [QB61] Presence of artificial opening --EXCLUDES--> [?] Attention to artificial openings	[ "[DA0E.5Y] Other specified malocclusion\n --PARENT--> [DA0E.5] Malocclusion\n Def: Malocclusion is the atypical relationship between maxillary and mandibular teeth which may interfere with the efficiency of excursive movements of the mandible that are essential for the effective mas...\n --PARENT--> [DA0E] Dentofacial anomalies\n Def: A congenital or acquired abnormality in which the dental and oral structures deviate from normal form, function, or position....", "[DA0E.5Y] Other specified malocclusion\n --PARENT--> [DA0E.5] Malocclusion\n Def: Malocclusion is the atypical relationship between maxillary and mandibular teeth which may interfere with the efficiency of excursive movements of the mandible that are essential for the effective mas...\n --PARENT--> [DA0E] Dentofacial anomalies\n Def: A congenital or acquired abnormality in which the dental and oral structures deviate from normal form, function, or position....", "[ME05.0] Constipation\n Def: Constipation is an acute or chronic condition in which bowel movements occur less often than usual or consist of hard, dry stools that are often painful or difficult to pass. Here constipation other t...\n --EXCLUDES--> [?] Functional constipation\n Def: Functional constipation is a functional bowel disorder that presents as persistently difficult, infrequent, or seemingly incomplete defecation, which do not meet IBS criteria....\n --EXCLUDES--> [?] Constipation\n Def: Constipation is an acute or chronic condition in which bowel movements occur less often than usual or consist of hard, dry stools that are often painful or difficult to pass. Here constipation other t...", "[ME05.0] Constipation\n Def: Constipation is an acute or chronic condition in which bowel movements occur less often than usual or consist of hard, dry stools that are often painful or difficult to pass. Here constipation other t...\n --PARENT--> [ME05] Change in bowel habit\n Def: Bowel habits are the time, size, amount, consistency and frequency of bowel movements throughout the day. A change in bowel habits is any alteration in regular bowel habits....\n --CHILD--> [ME05.Y] Other specified change in bowel habit", "[QB61.Z] Presence of artificial opening, unspecified\n --PARENT--> [QB61] Presence of artificial opening\n --CHILD--> [QB61.0] Presence of tracheostomy", "[QB61.Z] Presence of artificial opening, unspecified\n --PARENT--> [QB61] Presence of artificial opening\n --EXCLUDES--> [?] Attention to artificial openings" ]	DA0E.5Y	Other specified malocclusion	[ { "from_icd11": "ME05.0", "icd10_code": "K5900", "icd10_title": "Constipation, unspecified" }, { "from_icd11": "ME05.0", "icd10_code": "K5909", "icd10_title": "Other constipation" }, { "from_icd11": "QB61.Z", "icd10_code": "Z936", "icd10_title": "Other artificial openings of urinary tract status" }, { "from_icd11": "QB61.Z", "icd10_code": "Z934", "icd10_title": "Other artificial openings of gastrointestinal tract status" }, { "from_icd11": "QB61.Z", "icd10_code": "Z938", "icd10_title": "Other artificial opening status" }, { "from_icd11": "QB61.Z", "icd10_code": "Z939", "icd10_title": "Artificial opening status, unspecified" }, { "from_icd11": "QB61.Z", "icd10_code": "Z93", "icd10_title": "Artificial opening status" }, { "from_icd11": "LA8B.4", "icd10_code": "Q250", "icd10_title": "Patent ductus arteriosus" }, { "from_icd11": "6B64", "icd10_code": "F449", "icd10_title": "Dissociative and conversion disorder, unspecified" }, { "from_icd11": "6B64", "icd10_code": "F44", "icd10_title": "Dissociative and conversion disorders" }, { "from_icd11": "JA80.Z", "icd10_code": "O30", "icd10_title": "Multiple gestation" }, { "from_icd11": "JA80.Z", "icd10_code": "O308", "icd10_title": "Other specified multiple gestation" }, { "from_icd11": "JA80.Z", "icd10_code": "O309", "icd10_title": "Multiple gestation, unspecified" }, { "from_icd11": "QA46.Z", "icd10_code": "Z379", "icd10_title": "Outcome of delivery, unspecified" }, { "from_icd11": "QA46.Z", "icd10_code": "Z37", "icd10_title": "Outcome of delivery" } ]	K5900	Constipation, unspecified
A 65-year-old woman was referred to our endocrinology center for evaluation of diabetes mellitus, hyperlipidemia, and bilateral adrenal masses, which were detected for the first time prior to a scheduled operation for sigmoid-colon cancer. She previously underwent total hysterectomy for uterine fibroids at the age of 44. She was on anti-hypertensive medication from approximately 40 years of age, and had experienced aortic dissection at the age of 56. She showed normal stature and a body mass index of 24.7 kg/m 2 . She did not show any Cushingoid signs. Fasting morning serum cortisol and urinary free cortisol levels (measured by immune radio metric assay method, SRL, Tokyo) were normal (Table 1 ). However, midnight levels of serum cortisol were high, and both overnight dexamethasone suppression tests, using 1 mg and 8 mg dexamethasone, did not suppress serum cortisol or dehydroepiandrosterone (DHEA) -sulfate levels. Plasma ACTH levels were low and did not respond to 100 μg of intravenous corticotropin-releasing hormone. Furthermore, a dexamethasone suppression test using Liddle’s method showed a paradoxical increase in the levels of urinary cortisol (Table 2 ). The ratio of plasma aldosterone concentration (PAC) to plasma renin activity (PRA) was significantly high, although PAC was within the normal range. Based on the results of endocrinological examinations, the patient was diagnosed with idiopathic hyperaldosteronism (Tables 1 and 2 ). Adrenal venous sampling indicated bilateral aldosterone hypersecretion (Table 1 ). Bilateral adrenal tumors, 25 × 13 mm and 18 × 15 mm, in the right and left gland respectively, had the appearance of adrenocortical adenoma on computed tomography and magnetic resonance imaging . Accumulations of 131 I-adosterol in adrenal tumors were observed on both sides, though predominantly on the left . Various extra-adrenal masses were detected in several imaging modalities, and patchy brown skin pigmentations were observed systemically . Table 1 Laboratory data of the present patient Variable, unit Value Reference range Variable, unit Value Reference range <Blood examination> <Urine analysis> Fasting plasma glucose, mg/dL 111 73–109 Free cortisol, μg/day 73.5 11.2–80.3 Hemoglobin A 1 c, % 7.7 4.9–6.0 Androsterone, mg/day 0.31 0.4–3.00 Na, mmol/L 143 138–145 Etiocholanolone, mg/day 0.56 0.30–2.50 K, mmol/L 3.3 3.6–4.8 Dehydroepiandrosterone, mg/day 0.02 0.04–2.60 Cl, mmol/L 105 101–108 11-OH-Androsterone, mg/day 0.4 0.22–1.60 ACTH , pg/mL 3.1 7.2–63.3 11-OH-Etiocholanorone, mg/day 0.27 0.02–0.65 ACTH , pg/mL 2.5 N/A 11-keto-Androsterone, mg/day 0.01 < 0.07 Cortisol , μg/dL 10.6 4.0–18.3 11-keto-Etiocholanolone, mg/day 0.1 0.03–0.50 Cortisol , μg/dL 11.4 < 5.0 Dehydroepiandrosterone sulfate, μg/dL 129 12–133 <Adrenal venous sampling> Total testosterone, ng/mL 0.26 0.11–0.47 Cortisol (rt. adrenal vein), μg/dL 361 Corticosterone, ng/mL 2.36 0.12–8.48 Cortisol (lt. adrenal vein), μg/dL 380 Deoxycorticosterone, ng/mL 0.09 0.03–0.33 Cortisol (inferior vena cava), μg/dL 33.6 PRA, ng/mL/h 0.2 0.3–2.9 PAC (rt. adrenal vein), pg/mL 10,900 PAC, pg/mL 88.3 29.9–159 PAC (lt. adrenal vein), pg/mL 13,000 PAC (inferior vena cava), pg/mL 328 Adrenal venous sampling was performed while administering continuous intravenous infusion of ACTH (50 μg/hour). Text in parentheses indicate the location of blood sampling ACTH, adrenocorticotropic hormone; PRA, plasma renin activity; PAC, plasma aldosterone concentration Table 2 Endocrinological data of loading test Loading agent Variable, unit Value, pre-operation Value, post-operation Reference range 1 mg of DEX, overnight Serum cortisol, μg/dL 11.8 4.6 < 1.8 8 mg of DEX, overnight Serum cortisol, μg/dL 13.1 8.7 < 1.0 100 μg of CRH intravenously Basal ACTH, pg/mL 2.7 2.6 7.2–63.3 Peak ACTH, pg/mL (time, min) 5.2 (90) 9.1 (90) > 2× basal ACTH 2 L of saline intravenously PAC (before loading), pg/mL 78 73 29.9–159 PAC (after loading), pg/mL 92 81 < 60 50 mg of captopril orally PAC (0 min), pg/mL 56 114 29.9–159 PAC (60 min), pg/mL 59 102 N/A PAC (90 min), pg/mL 50 82 N/A ARR (0 min) 140 285 < 200 ARR (60 min) 590 102 < 200 ARR (90 min) 496 74 < 200 40 mg of furosemide intravenously with keeping upright PRA (0 min), ng/mL/h 0.3 0.4 0.3–2.9 PRA (60 min), ng/mL/h 0.3 0.5 > 2.0 PRA (120 min), ng/mL/h 0.4 0.8 > 2.0 <Dexamethasone suppression test, Liddle’s method> No DEX Urinary- free cortisol, μg/day 74 14 11.2–80.3 2 mg/day of DEX, the first day Urinary- free cortisol, μg/day 538 32 2 mg/day of DEX, the second day Urinary- free cortisol, μg/day 284 28 8 mg/day of DEX, the first day Urinary- free cortisol, μg/day 141 21 8 mg/day of DEX, the second day Urinary- free cortisol, μg/day 136 20 Overnight suppression test with 1 mg and 8 mg of DEX did not suppress cortisol levels, but paradoxically resulted in an increase in urinary cortisol levels DEX, dexamethasone; CRH, corticotropin-releasing hormone; ACTH, adrenocorticotropic hormone; PAC, plasma aldosterone concentration; ARR, plasma aldosterone / plasma renin activity ratio; PRA, plasma renin activity; N/A, not applicable Fig. 1 Imaging study of bilateral adrenal masses. Computed tomography scan shows bilateral adrenal tumors. ( a ) right side, 25 × 13 mm and ( b ) left side, 18 × 15 mm. Magnetic resonance imaging showed low intensity loci in adrenal tumors in “out phase” compared with “in phase” of chemical shift imaging on T1-weighted image. ( c ) right, in-phase; ( d ) right, out-phase; ( e ) left, in-phase; ( f ) left, out-phase. ( g ) 131 I-adosterol scintigraphy shows bilateral adrenal accumulation. Arrows in each image designate the adrenal tumors or more accurately where the adosterol is accumulating in the adrenal tumors Fig. 2 Imaging studies of extra-adrenal tumors in this patient. a A convexity meningioma 21 × 13 × 18 mm in size on enhanced T1WI MRI of the Head (arrow). The pituitary tumor is not marked. b A hyperechoic lesion about 14 mm in size in the interventricular septum on echocardiography (arrow). c Cervical ultra-sonography revealed four tumors in both thyroid lobes (arrow). d Colonoscopy shows tubular adenocarcinoma in the colon. This tumor was later resected, but the status of GNAS mutation was not assessed. e Enhanced abdominal computed tomography showed cystic lesions compatible with intraductal papillary mucinous neoplasm in the pancreatic body (arrow). f Brown patchy pigmentations were observed systemically. Skin appearance with patchy pigmentations of bilateral lower limbs is shown. g X-ray imaging and bone scintigraphy with 99m Tc-scintigraphy did not indicate fibrous dysplasia	4.1875	0.906738	sec[1]/p[0]	en	0.999997	30670014	https://doi.org/10.1186/s12902-019-0345-8	[ "cortisol", "adrenal", "plasma", "acth", "urinary", "tumors", "free", "imaging", "phase", "serum" ]	[ { "code": "5A70.Y", "title": "Other specified Cushing syndrome" }, { "code": "5A70.Z", "title": "Cushing syndrome, unspecified" }, { "code": "5A76.Y", "title": "Other specified disorders of adrenal gland" }, { "code": "5A74.0", "title": "Acquired adrenocortical insufficiency" }, { "code": "5A7Z", "title": "Disorders of the adrenal glands or adrenal hormone system, unspecified" }, { "code": "5A74.Z", "title": "Adrenocortical insufficiency, unspecified" }, { "code": "5A74.Y", "title": "Other specified adrenocortical insufficiency" }, { "code": "LC8Z", "title": "Structural developmental anomalies of the adrenal glands, unspecified" }, { "code": "2A83.2", "title": "Solitary plasmacytoma" }, { "code": "2A83.Z", "title": "Plasma cell neoplasm, unspecified" } ]	=== ICD-11 CODES FOUND === [5A70.Y] Other specified Cushing syndrome Also known as: Other specified Cushing syndrome \| ACTH-dependent Cushing syndrome \| ACTH-independent Cushing syndrome \| ACTH-independent Cushing syndrome due to bilateral adrenocortical hyperplasia \| ACTH-independent macronodular adrenal hyperplasia [5A70.Z] Cushing syndrome, unspecified Also known as: Cushing syndrome, unspecified \| Cushing syndrome \| Hyperadrenocorticism \| Hypercortisolism \| Cushing syndrome NOS [5A76.Y] Other specified disorders of adrenal gland Also known as: Other specified disorders of adrenal gland \| Suprarenal gland abscess \| Suprarenal abscess \| Adrenal gland inflammation \| adrenal glandular inflammation [5A74.0] Acquired adrenocortical insufficiency Definition: This is a acquired condition in which the adrenal glands do not produce adequate amounts of steroid hormones, primarily cortisol; but may also include impaired production of aldosterone (a mineralocorticoid), which regulates sodium conservation, potassium secretion, and water retention. Also known as: Acquired adrenocortical insufficiency \| Addison disease \| Chronic acquired adrenal insufficiency \| Autoimmune Addison disease \| Primary Addison disease Excludes: Amyloidosis [5A7Z] Disorders of the adrenal glands or adrenal hormone system, unspecified Also known as: Disorders of the adrenal glands or adrenal hormone system, unspecified \| Adrenal gland disease, not elsewhere classified \| adrenal cortex disease \| adrenal cortical disease \| adrenal glandular disease [5A74.Z] Adrenocortical insufficiency, unspecified Also known as: Adrenocortical insufficiency, unspecified \| Adrenocortical insufficiency \| adrenal failure NOS \| Hypoadrenocorticism \| adrenocortical hypofunction [5A74.Y] Other specified adrenocortical insufficiency Also known as: Other specified adrenocortical insufficiency \| Congenital adrenocortical insufficiency \| Congenital isolated ACTH deficiency \| Familial adrenal hypoplasia \| Familial hypoadrenocorticism [LC8Z] Structural developmental anomalies of the adrenal glands, unspecified Also known as: Structural developmental anomalies of the adrenal glands, unspecified \| adrenal anomaly \| adrenal gland anomaly \| congenital anomaly of adrenal gland \| congenital malformation of adrenal gland [2A83.2] Solitary plasmacytoma Definition: A single focus of clonal (malignant) plasma cells either in the bone or in another anatomic site without peripheral blood involvement. Also known as: Solitary plasmacytoma \| solitary plasmacytoma without mention of remission \| solitary myeloma \| localised malignant plasma cell tumour NOS \| plasmacytoma NOS Includes: solitary myeloma [2A83.Z] Plasma cell neoplasm, unspecified Also known as: Plasma cell neoplasm, unspecified \| Plasma cell neoplasms \| plasma cell tumours \| plasma cells dyscrasia \| plasma cell neoplasm NOS === GRAPH WALKS === --- Walk 1 --- [5A70.Y] Other specified Cushing syndrome --PARENT--> [5A70] Cushing syndrome Def: Cushing syndrome results from excess of corticosteroid hormones in the body due to overstimulation of the adrenal glands by excessive amounts of the hormone ACTH, secreted either by a tumuor of the pi... --CHILD--> [5A70.2] Pseudo-Cushing syndrome Def: This is a condition in which patients display the signs, symptoms, and abnormal hormone levels seen in Cushing's syndrome. However, pseudo-Cushing's syndrome is not caused by a problem with the hypoth... --- Walk 2 --- [5A70.Y] Other specified Cushing syndrome --PARENT--> [5A70] Cushing syndrome Def: Cushing syndrome results from excess of corticosteroid hormones in the body due to overstimulation of the adrenal glands by excessive amounts of the hormone ACTH, secreted either by a tumuor of the pi... --CHILD--> [5A70.2] Pseudo-Cushing syndrome Def: This is a condition in which patients display the signs, symptoms, and abnormal hormone levels seen in Cushing's syndrome. However, pseudo-Cushing's syndrome is not caused by a problem with the hypoth... --- Walk 3 --- [5A70.Z] Cushing syndrome, unspecified --PARENT--> [5A70] Cushing syndrome Def: Cushing syndrome results from excess of corticosteroid hormones in the body due to overstimulation of the adrenal glands by excessive amounts of the hormone ACTH, secreted either by a tumuor of the pi... --CHILD--> [5A70.0] Pituitary-dependent Cushing disease Def: Pituitary-dependent Cushing disease is caused by a pituitary tumour, generally benign (adenoma) but rarely malignant (carcinoma), which secretes adrenocorticotropin (ACTH) autonomously, leading to hyp... --- Walk 4 --- [5A70.Z] Cushing syndrome, unspecified --PARENT--> [5A70] Cushing syndrome Def: Cushing syndrome results from excess of corticosteroid hormones in the body due to overstimulation of the adrenal glands by excessive amounts of the hormone ACTH, secreted either by a tumuor of the pi... --CHILD--> [5A70.2] Pseudo-Cushing syndrome Def: This is a condition in which patients display the signs, symptoms, and abnormal hormone levels seen in Cushing's syndrome. However, pseudo-Cushing's syndrome is not caused by a problem with the hypoth... --- Walk 5 --- [5A76.Y] Other specified disorders of adrenal gland --PARENT--> [5A76] Certain specified disorders of adrenal gland --CHILD--> [5A76.Y] Other specified disorders of adrenal gland --- Walk 6 --- [5A76.Y] Other specified disorders of adrenal gland --PARENT--> [5A76] Certain specified disorders of adrenal gland --CHILD--> [5A76.0] Premature adrenarche Def: Premature development of pubic and/or axillary hair without central or peripheral precocious puberty. Children show premature clinical and/or laboratory signs of androgen action without estrogen actio...	[ "[5A70.Y] Other specified Cushing syndrome\n --PARENT--> [5A70] Cushing syndrome\n Def: Cushing syndrome results from excess of corticosteroid hormones in the body due to overstimulation of the adrenal glands by excessive amounts of the hormone ACTH, secreted either by a tumuor of the pi...\n --CHILD--> [5A70.2] Pseudo-Cushing syndrome\n Def: This is a condition in which patients display the signs, symptoms, and abnormal hormone levels seen in Cushing's syndrome. However, pseudo-Cushing's syndrome is not caused by a problem with the hypoth...", "[5A70.Y] Other specified Cushing syndrome\n --PARENT--> [5A70] Cushing syndrome\n Def: Cushing syndrome results from excess of corticosteroid hormones in the body due to overstimulation of the adrenal glands by excessive amounts of the hormone ACTH, secreted either by a tumuor of the pi...\n --CHILD--> [5A70.2] Pseudo-Cushing syndrome\n Def: This is a condition in which patients display the signs, symptoms, and abnormal hormone levels seen in Cushing's syndrome. However, pseudo-Cushing's syndrome is not caused by a problem with the hypoth...", "[5A70.Z] Cushing syndrome, unspecified\n --PARENT--> [5A70] Cushing syndrome\n Def: Cushing syndrome results from excess of corticosteroid hormones in the body due to overstimulation of the adrenal glands by excessive amounts of the hormone ACTH, secreted either by a tumuor of the pi...\n --CHILD--> [5A70.0] Pituitary-dependent Cushing disease\n Def: Pituitary-dependent Cushing disease is caused by a pituitary tumour, generally benign (adenoma) but rarely malignant (carcinoma), which secretes adrenocorticotropin (ACTH) autonomously, leading to hyp...", "[5A70.Z] Cushing syndrome, unspecified\n --PARENT--> [5A70] Cushing syndrome\n Def: Cushing syndrome results from excess of corticosteroid hormones in the body due to overstimulation of the adrenal glands by excessive amounts of the hormone ACTH, secreted either by a tumuor of the pi...\n --CHILD--> [5A70.2] Pseudo-Cushing syndrome\n Def: This is a condition in which patients display the signs, symptoms, and abnormal hormone levels seen in Cushing's syndrome. However, pseudo-Cushing's syndrome is not caused by a problem with the hypoth...", "[5A76.Y] Other specified disorders of adrenal gland\n --PARENT--> [5A76] Certain specified disorders of adrenal gland\n --CHILD--> [5A76.Y] Other specified disorders of adrenal gland", "[5A76.Y] Other specified disorders of adrenal gland\n --PARENT--> [5A76] Certain specified disorders of adrenal gland\n --CHILD--> [5A76.0] Premature adrenarche\n Def: Premature development of pubic and/or axillary hair without central or peripheral precocious puberty. Children show premature clinical and/or laboratory signs of androgen action without estrogen actio..." ]	5A70.Y	Other specified Cushing syndrome	[ { "from_icd11": "5A70.Z", "icd10_code": "E242", "icd10_title": "Drug-induced Cushing's syndrome" }, { "from_icd11": "5A70.Z", "icd10_code": "E249", "icd10_title": "Cushing's syndrome, unspecified" }, { "from_icd11": "5A70.Z", "icd10_code": "E248", "icd10_title": "Other Cushing's syndrome" }, { "from_icd11": "5A70.Z", "icd10_code": "E24", "icd10_title": "Cushing's syndrome" }, { "from_icd11": "5A74.0", "icd10_code": "E273", "icd10_title": "Drug-induced adrenocortical insufficiency" }, { "from_icd11": "5A74.0", "icd10_code": "E271", "icd10_title": "Primary adrenocortical insufficiency" }, { "from_icd11": "5A74.0", "icd10_code": "E35", "icd10_title": "Disorders of endocrine glands in diseases classified elsewhere" }, { "from_icd11": "5A7Z", "icd10_code": "E2740", "icd10_title": "Unspecified adrenocortical insufficiency" }, { "from_icd11": "5A7Z", "icd10_code": "E2749", "icd10_title": "Other adrenocortical insufficiency" }, { "from_icd11": "5A7Z", "icd10_code": "E279", "icd10_title": "Disorder of adrenal gland, unspecified" }, { "from_icd11": "5A7Z", "icd10_code": "E27", "icd10_title": "Other disorders of adrenal gland" }, { "from_icd11": "5A7Z", "icd10_code": "E274", "icd10_title": "Other and unspecified adrenocortical insufficiency" }, { "from_icd11": "LC8Z", "icd10_code": "Q891", "icd10_title": "Congenital malformations of adrenal gland" }, { "from_icd11": "2A83.2", "icd10_code": "C9030", "icd10_title": "Solitary plasmacytoma not having achieved remission" }, { "from_icd11": "2A83.2", "icd10_code": "C9032", "icd10_title": "Solitary plasmacytoma in relapse" } ]	E242	Drug-induced Cushing's syndrome
In June 2021, a 59-year-old Japanese male with a history of lumbar compression fracture visited a local clinic complaining of fever lasting for five days. A systemic computed tomography (CT) scan was immediately taken, but did not locate any origins of fever. The complete blood counts showed white blood cells (WBC) of 6,700 /μL (13% neutrophils) and platelets of 1.02 × 10 5 /μL, with normal hemoglobin levels of 11.4 g/dL. Detailed examination of the peripheral blood identified emergence of neutrophils with decreased cytoplasmic granules or with pseudo-Pelger-Huёt nuclear anomaly, and giant platelets. Analysis of the bone marrow aspiration showed hypercellular bone marrow with increased number of myeloblasts (4.6%) and ring sideroblasts (77%). Multilineage dysplasia of the hematopoietic cells were also evident, such as nuclear multilobation and nuclear budding erythroblasts, and megakaryocytes with separated nuclear lobes. The patient was thus diagnosed as MDS with low blasts and ring sideroblasts. In September 2021, the patient was referred to our hospital. The bone marrow examination was repeated, which detected increased number of myeloblasts to 7.8%. Cytogenetic analysis of the bone marrow showed complex karyotype: 45, XY, add(5) (q11.2), add(7) (p13), + 8, − 9, − 17, add(19) (p13.3), − 20, + r, 2 ~ 6dim [20/20] . The fluorescence in situ hybridization (FISH) resulted positive for probes detecting deletion of 5q, but negative for probes detecting monosomy 7. The patient decided to receive an allogenic hematopoietic stem cell transplantation (HSCT) as a curative therapy for MDS, intermediate-2 risk according to the International Prognostic Scoring System (IPSS), and we started coordination of bone marrow donors. In January 2022, the bone marrow aspiration was repeated to evaluate the progression of the disease. Chromosomal analysis showed complex karyotype containing + 22 that had not been recognized in September 2021: 40, X, -Y, − 5, add(7)(p13), − 9, add(15)(p11.2), -16, -17, -18, add(19)(p13.3), − 20, + r, 2dmin [8/20]/45, XY, − 3, add(5)(q11.2), − 7, − 9, add(12)(p11.2), − 17, add(19)(p13.3), − 20, + 22, + r, + 2mar, 2dmin [4/20]/44, XY, add(5)(q11.2), del(7)(p13), − 9, add(11)(p11.2), − 16, − 17, add(19)(p13.3), − 20, + r, + mar, 2dimn [3/20]/karyotypes similar to above (including add(5)(q11.2), add(19)(p13.3), and dmin) [5/20] . In February 2022, the patient was admitted to the hospital to receive a bridging chemotherapy for HSCT. At presentation, the patient complained of modest pain in the right thigh that started three weeks prior to admission. Because of persisting pain, a bone X-ray was taken, which showed presence of an osteolytic lesion in the right femur. Magnetic resonance imaging (MRI) showed a 45 mm mass in the right distal femur with moderate enhancement on post-contrast fat-suppressed T1-weighted images, which invades the tissue outside of the bone . Similar pathological lesions were also recognized in the right proximal femur and the right pelvis. The Hematoxylin and Eosin (H & E) stain of the fine needle-biopsied samples from the right femur showed proliferation of immature myeloid cells with atypical nucleus . The immunohistochemistry analysis of the fine needle-biopsied samples from the right femur showed infiltrating myeloblasts that were positive for vimentin, leukocyte common antigen (LCA), c-kit and CD34 , while negative for cytokeratin AE1/AE3, S100, MPO (Myeloperoxidase), CD3 and CD20. These myeloblasts were morphologically and immunohistochemically considered identical to those in the bone marrow. The first course of azacitidine treatment was subsequently administered. After the diagnosis of the myeloid sarcoma, the weight bearing on the right leg was immediately restricted, and the patient was instructed to walk with double crutches. However, in March 2022, the patient complained of severe pain and swelling in the right thigh just after he twisted the right leg while changing body position on the bed. A plain X-ray picture was immediately taken, which showed pathological fracture of the right femur . The complete blood counts of the peripheral blood showed increased number of myeloblasts to 24.0%. Chromosomal analysis of the bone marrow cells revealed expansion of the clones that harbor trisomy 22 . With the diagnosis of MDS-overt AML, the patient underwent intensive chemotherapy with cytarabine and idarubicin. The pathological fracture of the right femur was managed conservatively without surgical intervention. This was because the surgical fixation of the fracture was considered to be at high risk for infectious complications, given the immunosuppression in uncontrolled AML. The induction chemotherapy with cytarabine and idarubicin achieved complete remission with incomplete count recovery (CRi). The patient then underwent consolidative chemotherapy with high-dose cytarabine in April 2022. The bone marrow examination in the next month, however, showed increased number of myeloblasts and proerythroblasts. Reinduction chemotherapy with azacitidine and venetoclax was initiated, yet was not effective enough to control the rapidly proliferating AML cells. The patient succumbed to death in June 2022 due to the progression of the disease. Fig. 1 Myeloid sarcoma-associated pathological fracture. A Clonal evolution of MDS/AML cells with trisomy 22 in the bone marrow. Chromosomal analysis of the bone marrow cells showed additional chromosomal alterations including + 22 that had been observed just before the development of myeloid sarcoma in the right femur. (A-1) 45, XY, − 3, add (5)(q11.2), − 7, − 9, add(12)(p11.2), − 17, add(19)(p13.3), -20, + 22, + r, + 2mar, 2dmin. (A-2) 46, XY, add(5)(q11.2), − 7, + add(8)(q24.1), − 9, + 11, add(15)(p11.2), − 17, − 19, der(20)t(7;20), (q11.2;q13.1), + 22, + r, 2dmin. (A-3) 45, XY, − 1, add(1)(q21), − 3, add(5)(q11.2), − 7, − 9, − 12, − 17, add(19)(p13.3), − 20, + 22, + r, + 4mar, 3dmin. B Post-contrast fat-suppressed T1-weighted magnetic resonance image (MRI) of the myeloid sarcoma in the right femur presenting as an osteolytic lesion. C – E Histopathological analysis of the sample obtained from the fine needle biopsy of the myeloid sarcoma in the right femur. The Hematoxylin and Eosin (H & E)-staining revealed dense proliferation of immature myeloid cells with nuclear enlargement and high nuclear to cytoplasmic (N/C) ratio ( D ). The immature myeloid cells were stained positive for c-kit ( E ) and partially positive for CD34 ( F ). F Plain X-ray image of the myeloid sarcoma in the right femur presenting as a pathological fracture	4.191406	0.962891	sec[1]/p[0]	en	0.999996	37707761	https://doi.org/10.1007/s12185-023-03656-1	[ "bone", "marrow", "femur", "cells", "myeloid", "fracture", "nuclear", "myeloblasts", "sarcoma", "blood" ]	[ { "code": "FC0Z", "title": "Diseases of the musculoskeletal system or connective tissue, unspecified" }, { "code": "FB84.Z", "title": "Osteomyelitis or osteitis, unspecified" }, { "code": "FB80.Z", "title": "Disorder of bone density or structure, unspecified" }, { "code": "FB86.11", "title": "Hypertrophy of bone" }, { "code": "FB86.1Z", "title": "Bone hyperplasias, unspecified" }, { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "3A70.Z", "title": "Aplastic anaemia, unspecified" }, { "code": "3C0Y", "title": "Other specified diseases of the blood or blood-forming organs" }, { "code": "3A70.12", "title": "Idiopathic aplastic anaemia" }, { "code": "NE84", "title": "Failure or rejection of transplanted organs or tissues" } ]	=== ICD-11 CODES FOUND === [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified Also known as: Diseases of the musculoskeletal system or connective tissue, unspecified \| bone disease NOS \| bone disorder NOS \| bone lesion NOS \| musculoskeletal complications NOS [FB84.Z] Osteomyelitis or osteitis, unspecified Also known as: Osteomyelitis or osteitis, unspecified \| Osteomyelitis or osteitis \| bone inflammation \| bone ulcer \| bone inflammatory disease [FB80.Z] Disorder of bone density or structure, unspecified Also known as: Disorder of bone density or structure, unspecified \| Certain specified disorders of bone density or structure [FB86.11] Hypertrophy of bone Also known as: Hypertrophy of bone \| bone thickening \| periosteum thickening \| Infantile cortical hyperostoses \| Post traumatic subperiosteal ossification [FB86.1Z] Bone hyperplasias, unspecified Also known as: Bone hyperplasias, unspecified \| Bone hyperplasias [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [3A70.Z] Aplastic anaemia, unspecified Also known as: Aplastic anaemia, unspecified \| Aplastic anaemia \| erythroid aplasia \| AA - [aplastic anaemia] \| haematopoietic aplasia [3C0Y] Other specified diseases of the blood or blood-forming organs Also known as: Other specified diseases of the blood or blood-forming organs \| Congenital anomaly blood or lymph other \| Blood dyscrasia \| blood dyscrasia NOS \| Bone marrow hyperplasia [3A70.12] Idiopathic aplastic anaemia Also known as: Idiopathic aplastic anaemia \| Idiopathic bone marrow failure \| idiopathic aplastic anaemia NOS [NE84] Failure or rejection of transplanted organs or tissues Also known as: Failure or rejection of transplanted organs or tissues \| organ transplant rejection \| transplant failure \| transplant rejection \| Bone-marrow transplant rejection === GRAPH WALKS === --- Walk 1 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --EXCLUDES--> [?] Pregnancy, childbirth or the puerperium Def: A group of conditions characterised as occurring during the period of time from conception to delivery (pregnancy), during labour and delivery (childbirth) or during the approximately six weeks after ... --- Walk 2 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --EXCLUDES--> [?] Endocrine, nutritional or metabolic diseases Def: This chapter includes endocrine diseases, nutritional diseases as well as metabolic diseases.... --- Walk 3 --- [FB84.Z] Osteomyelitis or osteitis, unspecified --PARENT--> [FB84] Osteomyelitis or osteitis --EXCLUDES--> [?] Infection of vertebra Def: A condition of the vertebrae, caused by an infection with a bacterial, viral, fungal, or parasitic source. This condition commonly presents with fever, chills, headache, weight loss, or may be asympto... --- Walk 4 --- [FB84.Z] Osteomyelitis or osteitis, unspecified --PARENT--> [FB84] Osteomyelitis or osteitis --CHILD--> [FB84.0] Acute haematogenous osteomyelitis --- Walk 5 --- [FB80.Z] Disorder of bone density or structure, unspecified --PARENT--> [FB80] Certain specified disorders of bone density or structure --RELATED_TO--> [?] Osteogenesis imperfecta Def: Osteogenesis imperfecta (OI) comprises a heterogeneous group of genetic disorders characterised by increased bone fragility, low bone mass, and susceptibility to bone fractures with variable severity.... --- Walk 6 --- [FB80.Z] Disorder of bone density or structure, unspecified --PARENT--> [FB80] Certain specified disorders of bone density or structure --EXCLUDES--> [?] Osteopoikilosis	[ "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --EXCLUDES--> [?] Pregnancy, childbirth or the puerperium\n Def: A group of conditions characterised as occurring during the period of time from conception to delivery (pregnancy), during labour and delivery (childbirth) or during the approximately six weeks after ...", "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --EXCLUDES--> [?] Endocrine, nutritional or metabolic diseases\n Def: This chapter includes endocrine diseases, nutritional diseases as well as metabolic diseases....", "[FB84.Z] Osteomyelitis or osteitis, unspecified\n --PARENT--> [FB84] Osteomyelitis or osteitis\n --EXCLUDES--> [?] Infection of vertebra\n Def: A condition of the vertebrae, caused by an infection with a bacterial, viral, fungal, or parasitic source. This condition commonly presents with fever, chills, headache, weight loss, or may be asympto...", "[FB84.Z] Osteomyelitis or osteitis, unspecified\n --PARENT--> [FB84] Osteomyelitis or osteitis\n --CHILD--> [FB84.0] Acute haematogenous osteomyelitis", "[FB80.Z] Disorder of bone density or structure, unspecified\n --PARENT--> [FB80] Certain specified disorders of bone density or structure\n --RELATED_TO--> [?] Osteogenesis imperfecta\n Def: Osteogenesis imperfecta (OI) comprises a heterogeneous group of genetic disorders characterised by increased bone fragility, low bone mass, and susceptibility to bone fractures with variable severity....", "[FB80.Z] Disorder of bone density or structure, unspecified\n --PARENT--> [FB80] Certain specified disorders of bone density or structure\n --EXCLUDES--> [?] Osteopoikilosis" ]	FC0Z	Diseases of the musculoskeletal system or connective tissue, unspecified	[ { "from_icd11": "FC0Z", "icd10_code": "XIII", "icd10_title": "" }, { "from_icd11": "FB84.Z", "icd10_code": "M86672", "icd10_title": "Other chronic osteomyelitis, left ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86172", "icd10_title": "Other acute osteomyelitis, left ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86171", "icd10_title": "Other acute osteomyelitis, right ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86671", "icd10_title": "Other chronic osteomyelitis, right ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X7", "icd10_title": "Other osteomyelitis, ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X8", "icd10_title": "Other osteomyelitis, other site" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X6", "icd10_title": "Other osteomyelitis, lower leg" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X9", "icd10_title": "Other osteomyelitis, unspecified sites" }, { "from_icd11": "FB84.Z", "icd10_code": "M8668", "icd10_title": "Other chronic osteomyelitis, other site" }, { "from_icd11": "FB84.Z", "icd10_code": "M86662", "icd10_title": "Other chronic osteomyelitis, left tibia and fibula" }, { "from_icd11": "FB84.Z", "icd10_code": "M86151", "icd10_title": "Other acute osteomyelitis, right femur" }, { "from_icd11": "FB84.Z", "icd10_code": "M86141", "icd10_title": "Other acute osteomyelitis, right hand" }, { "from_icd11": "FB84.Z", "icd10_code": "M86641", "icd10_title": "Other chronic osteomyelitis, right hand" }, { "from_icd11": "FB84.Z", "icd10_code": "M8669", "icd10_title": "Other chronic osteomyelitis, multiple sites" } ]	XIII
In 2015, a then 52-year-old man was referred to our radiation oncology department for evaluation of hemostatic treatment. He presented with chest pain, dyspnea, fatigue, loss of appetite, weight loss, recurrent respiratory infections, and episodes of significant hemoptysis. Medical history included pulmonary TB in 1995 , which was adequately treated with antitubercular medications for 6 months, resulting in a residual cavity in the left lower lobe. Hemoptysis first occurred a couple of years after initial TB treatment and symptoms had become aggravated since then. In 2007, the patient presented with a new episode of hemoptysis. His medical history and a CT scan of the chest lead to the differential diagnosis of pulmonary aspergillosis. The patient was hemodynamically stable and a hemoglobin of 155 g/L (reference value 135–168 g/L) was measured. Since bronchoscopy confirmed endobronchial bleeding from the segments where the lesion was located, partial resection of the left inferior lobe was performed in the same year. Aspergilloma was confirmed by histopathological examination of the resected lung tissue. A post-interventional CT scan of the chest, however, showed persistence of the aspergilloma. In addition, persistence was suggested microbiologically by positive Aspergillus cultures from bronchoalveolar lavage fluid and sputum at different timepoints. In the following years, three attempts at endovascular embolization of pulmonary arteries, the first in 2008, were performed due to recurring hemoptysis, without achieving lasting hemostasis. Therefore, long-term antifungal therapy with itraconazole was initiated in 2009 and continued for 2.5 years. In addition to the aspergillosis, the patient was diagnosed with a pulmonary actinomycosis in 2015, a rare opportunistic bacterial infection of the lung. Actinomyces were also isolated from a bronchoscopic biopsy in the left lower lobe. Although Aspergillus cultures and Aspergillus precipitin could not reaffirm aspergilloma persistence at that time, these results did not definitely rule out a concomitant fungal and bacterial infection. The patient’s pulmonary function was further reduced after suffering multiple pulmonary emboli. The last complete pulmonary function testing prior to radiotherapy was performed in 2013. It revealed a vital capacity (VC) of 2.15 L (44% of reference value), forced expiratory volume in one second (FEV 1 ) of 1.55 L (41%), and a diffusing capacity of the lungs for carbon monoxide (DLCO) of 7.4 mmol/min/kPa (71%), so that the patient was considered unfit for further surgical interventions. During the first consultation in our clinic, the patient reported recurrent episodes of hemoptysis causing blood loss of more than 100 ml per day. A CT of the chest showed a persistent, spiculated, partly cavernous lesion in the left lower lobe with a diameter of approximately 5 cm . Treatment planning also included an 18 F‑FDG-PET/CT scan to localize the fungal manifestation and identified the metabolically active inflamed vascular lining of the cavity as the most likely cause of bleeding . SBRT with a total dose of 16 Gy was applied in two fractions of 8 Gy on consecutive days with a robotic arm-mounted linear accelerator equipped with an iris collimator . Dose was prescribed to the 80% isodose line and the ray-tracing algorithm was used for dose calculation. After acquisition of a 4D-planning CT to account for respiratory motion, the planning target volume (PTV) was generated from an internal target volume (ITV), adding a 2-mm margin. Despite an irregularly shaped PTV, the chosen irradiation technique allowed us to achieve a conformal dose distribution of the target volume and tolerable doses for relevant organs at risk (OAR) as shown in the dose–volume histogram . The treatment was well tolerated, and no side effects were reported by the patient. During the 6 years of follow-up at our department, the patient has reported a significant decrease in hemoptysis frequency and volume, and no new long-term medication or invasive treatments have been necessary since then. When he presented at the hospital’s emergency unit with dyspnea and small-volume hemoptysis in 2016, there were no signs of an active or older bleeding evident in CT or bronchoscopy. Hemoglobin level remained stable at around 140 g/L over the years and fell below 120 g/L only once during an episode of community-acquired pneumonia in 2016 not accompanied by hemoptysis. Regular CT scans of the chest confirmed a stable size of the pulmonary lesion after an initial pseudoprogression, which is often observed after SBRT for large target volumes . Fig. 1 Timeline of important diagnostic and therapeutic steps. The upper part graphically shows onset and end of hemoptysis. The upward arrows indicate the most severe episodes of hemoptysis measured by bronchoscopic findings and patient-reported frequency and quantity. BAE bronchial artery embolization , CT computed tomography , SBRT stereotactic body radiotherapy Fig. 2 Pre- and postradiotherapy (RT) imaging. a Pre-RT thoracic CT scan 8 years after partial surgical resection of the left inferior lobe. The aspergilloma is localized adjacent to the great pulmonary vessels, showing the characteristic radiographic feature of the “air-crescent sign” ( white arrow ). b Aspergilloma decreased in size at 3‑year follow-up and no further interventions were necessary. After mild episodes of hemoptysis initially, no further episodes have been reported since then. CT computed tomography , RT radiotherapy Fig. 3 Treatment planning and modalities including dose–volume histogram (DVH). a Treatment planning included an 18 F‑FDG-PET/CT scan to precisely localize the fungal manifestation and the primarily inflamed and eroded vascular lining of the cavity, most likely causing the bleeding. b 16 Gy was applied in two fractions of 8 Gy on consecutive days with a robotic arm-mounted linear accelerator. Dose was prescribed to the 80% isodose line. PTV ( red ) was generated from an ITV ( green ) by adding a 2-mm margin. The ITV accounts for motion of the target lesion during respiration registered by a 4D planning CT. The PTV on the other hand accounts for setup and planning uncertainties. c The DVH represents the exact radiation dose (shown as percentage of the prescribed dose) delivered to the target volumes and organs at risk (OAR; also shown as percentage of total volume). CT computed tomography , CTV clinical target volume , FDG fluorodeoxyglucose , ITV internal target volume , PET positron emission tomography , PTV planning target volume , RT radiotherapy	4.078125	0.970703	sec[1]/sec[0]/p[0]	en	0.999997	36264357	https://doi.org/10.1007/s00066-022-02013-1	[ "hemoptysis", "pulmonary", "target", "planning", "chest", "episodes", "lobe", "scan", "aspergilloma", "since" ]	[ { "code": "MD22", "title": "Haemoptysis" }, { "code": "1F85", "title": "Paragonimiasis" }, { "code": "1B10.Z", "title": "Respiratory tuberculosis, without mention of bacteriological or histological confirmation" }, { "code": "KB28.Z", "title": "Pulmonary haemorrhage originating in the perinatal period, unspecified" }, { "code": "CB40.Y", "title": "Other specified diseases of the respiratory system" }, { "code": "LA75.1", "title": "Agenesis of lung" }, { "code": "CA40.Z", "title": "Pneumonia, organism unspecified" }, { "code": "CB41", "title": "Respiratory failure" }, { "code": "NB32.3Y", "title": "Other injury of lung" }, { "code": "QE04", "title": "Target of perceived adverse discrimination or persecution" } ]	=== ICD-11 CODES FOUND === [MD22] Haemoptysis Definition: Expectoration or spitting of blood originating from any part of the respiratory tract, usually from haemorrhage in the lung parenchyma and the bronchial arteries. Also known as: Haemoptysis \| blood streaked sputum \| coughing up blood \| Blood-stained sputum \| Cough with haemorrhage Includes: Blood-stained sputum \| Cough with haemorrhage [1F85] Paragonimiasis Definition: A disease caused by an infection with the parasitic worm Paragonimus. This disease is characterised by cough or haemoptysis, or may be asymptomatic. This disease may present with other symptoms depending on the site where the parasite migrates to. Transmission is commonly by ingestion of undercooked contaminated crustaceans (crab or crayfish). Confirmation is commonly by identification of Paragonimus eggs in a sputum or faecal sample. Also known as: Paragonimiasis \| Pulmonary distomiasis \| Parasitic haemoptysis \| Oriental lung fluke disease \| Endemic haemoptysis Includes: lung fluke disease \| infection due to paragonimus species \| Infestation due to Paragonimus species [1B10.Z] Respiratory tuberculosis, without mention of bacteriological or histological confirmation Also known as: Respiratory tuberculosis, without mention of bacteriological or histological confirmation \| Tuberculosis of the respiratory system \| respiratory tuberculosis \| pulmonary tuberculosis \| pulmonary TB [KB28.Z] Pulmonary haemorrhage originating in the perinatal period, unspecified Also known as: Pulmonary haemorrhage originating in the perinatal period, unspecified \| Pulmonary haemorrhage originating in the perinatal period \| neonatal pulmonary haemorrhage \| newborn lung haemorrhage \| pulmonary haemorrhage in newborn [CB40.Y] Other specified diseases of the respiratory system Also known as: Other specified diseases of the respiratory system \| Secondary respiratory disorders \| Respiratory disorders in diseases classified elsewhere \| Diseases of bronchus, not elsewhere classified \| Acquired bronchial diverticulum [LA75.1] Agenesis of lung Definition: This refers to the absence or rudimentary residua of an undeveloped lung. Also known as: Agenesis of lung \| Pulmonary agenesis \| absence of lung \| aplasia of lung \| apulmonism [CA40.Z] Pneumonia, organism unspecified Also known as: Pneumonia, organism unspecified \| Pneumonia \| infectious pneumonia \| PN - [pneumonia] \| lobar pneumonia NOS [CB41] Respiratory failure Definition: Respiratory failure is a life-threatening impairment of oxygenation or carbon dioxide (CO2) elimination. Respiratory failure may occur because of impaired gas exchange, decreased ventilation, or both. The level of oxygen in the blood becomes dangerously low or the level of carbon dioxide becomes dangerously high. Also known as: Respiratory failure \| lung failure NOS \| pulmonary failure Excludes: Acute respiratory distress syndrome \| Respiratory arrest \| Respiratory distress of newborn [NB32.3Y] Other injury of lung Also known as: Other injury of lung \| Haematoma of lung \| Traumatic hydropneumothorax \| Acute traumatic lung congestion \| Rupture of lung [QE04] Target of perceived adverse discrimination or persecution Definition: Persecution or discrimination, perceived as reality by an individual or real, on the basis of membership in some group (such as defined by skin colour, religion, ethnic origin, sexual orientation, gender identity and expression, etc.) rather than personal characteristics Also known as: Target of perceived adverse discrimination or persecution \| Exposure to discrimination \| Ethnic discrimination \| Political discrimination \| Racial discrimination Excludes: Social exclusion or rejection === GRAPH WALKS === --- Walk 1 --- [MD22] Haemoptysis Def: Expectoration or spitting of blood originating from any part of the respiratory tract, usually from haemorrhage in the lung parenchyma and the bronchial arteries.... --PARENT--> [?] Haemorrhage from respiratory passages Def: Haemorrhage from respiratory passages is the bleeding from upper respiratory tract or lower respiratory tract. The major passages and structures of the upper respiratory tract include the nose or nost... --CHILD--> [MD20] Epistaxis Def: Bleeding from the nose... --- Walk 2 --- [MD22] Haemoptysis Def: Expectoration or spitting of blood originating from any part of the respiratory tract, usually from haemorrhage in the lung parenchyma and the bronchial arteries.... --PARENT--> [?] Haemorrhage from respiratory passages Def: Haemorrhage from respiratory passages is the bleeding from upper respiratory tract or lower respiratory tract. The major passages and structures of the upper respiratory tract include the nose or nost... --EXCLUDES--> [?] Pulmonary haemorrhage originating in the perinatal period Def: A condition characterised by bleeding from the lung which begins during the period of time around childbirth, especially the five months before and one month after birth.... --- Walk 3 --- [1F85] Paragonimiasis Def: A disease caused by an infection with the parasitic worm Paragonimus. This disease is characterised by cough or haemoptysis, or may be asymptomatic. This disease may present with other symptoms depend... --PARENT--> [?] Diseases due to trematodes --CHILD--> [1F80] Clonorchiasis Def: A condition caused by an infection with the parasitic worm Clonorchis sinensis. This condition commonly presents with inflammation and obstruction of the biliary ducts. This condition may also present... --- Walk 4 --- [1F85] Paragonimiasis Def: A disease caused by an infection with the parasitic worm Paragonimus. This disease is characterised by cough or haemoptysis, or may be asymptomatic. This disease may present with other symptoms depend... --PARENT--> [?] Diseases due to trematodes --CHILD--> [1F80] Clonorchiasis Def: A condition caused by an infection with the parasitic worm Clonorchis sinensis. This condition commonly presents with inflammation and obstruction of the biliary ducts. This condition may also present... --- Walk 5 --- [1B10.Z] Respiratory tuberculosis, without mention of bacteriological or histological confirmation --PARENT--> [1B10] Tuberculosis of the respiratory system Def: This is a progressive or chronic disease resulting from infection with the bacterium Mycobacterium tuberculosis or other bacteria in the M. tuberculosis complex: M. bovis, M. africanum, M. canetti, M.... --PARENT--> [?] Tuberculosis Def: A disease caused by an infection with bacteria of the Mycobacterium tuberculosis complex. This disease presents with symptoms depending on the site of infection. Transmission is commonly by inhalation... --- Walk 6 --- [1B10.Z] Respiratory tuberculosis, without mention of bacteriological or histological confirmation --PARENT--> [1B10] Tuberculosis of the respiratory system Def: This is a progressive or chronic disease resulting from infection with the bacterium Mycobacterium tuberculosis or other bacteria in the M. tuberculosis complex: M. bovis, M. africanum, M. canetti, M.... --CHILD--> [1B10.Z] Respiratory tuberculosis, without mention of bacteriological or histological confirmation	[ "[MD22] Haemoptysis\n Def: Expectoration or spitting of blood originating from any part of the respiratory tract, usually from haemorrhage in the lung parenchyma and the bronchial arteries....\n --PARENT--> [?] Haemorrhage from respiratory passages\n Def: Haemorrhage from respiratory passages is the bleeding from upper respiratory tract or lower respiratory tract. The major passages and structures of the upper respiratory tract include the nose or nost...\n --CHILD--> [MD20] Epistaxis\n Def: Bleeding from the nose...", "[MD22] Haemoptysis\n Def: Expectoration or spitting of blood originating from any part of the respiratory tract, usually from haemorrhage in the lung parenchyma and the bronchial arteries....\n --PARENT--> [?] Haemorrhage from respiratory passages\n Def: Haemorrhage from respiratory passages is the bleeding from upper respiratory tract or lower respiratory tract. The major passages and structures of the upper respiratory tract include the nose or nost...\n --EXCLUDES--> [?] Pulmonary haemorrhage originating in the perinatal period\n Def: A condition characterised by bleeding from the lung which begins during the period of time around childbirth, especially the five months before and one month after birth....", "[1F85] Paragonimiasis\n Def: A disease caused by an infection with the parasitic worm Paragonimus. This disease is characterised by cough or haemoptysis, or may be asymptomatic. This disease may present with other symptoms depend...\n --PARENT--> [?] Diseases due to trematodes\n --CHILD--> [1F80] Clonorchiasis\n Def: A condition caused by an infection with the parasitic worm Clonorchis sinensis. This condition commonly presents with inflammation and obstruction of the biliary ducts. This condition may also present...", "[1F85] Paragonimiasis\n Def: A disease caused by an infection with the parasitic worm Paragonimus. This disease is characterised by cough or haemoptysis, or may be asymptomatic. This disease may present with other symptoms depend...\n --PARENT--> [?] Diseases due to trematodes\n --CHILD--> [1F80] Clonorchiasis\n Def: A condition caused by an infection with the parasitic worm Clonorchis sinensis. This condition commonly presents with inflammation and obstruction of the biliary ducts. This condition may also present...", "[1B10.Z] Respiratory tuberculosis, without mention of bacteriological or histological confirmation\n --PARENT--> [1B10] Tuberculosis of the respiratory system\n Def: This is a progressive or chronic disease resulting from infection with the bacterium Mycobacterium tuberculosis or other bacteria in the M. tuberculosis complex: M. bovis, M. africanum, M. canetti, M....\n --PARENT--> [?] Tuberculosis\n Def: A disease caused by an infection with bacteria of the Mycobacterium tuberculosis complex. This disease presents with symptoms depending on the site of infection. Transmission is commonly by inhalation...", "[1B10.Z] Respiratory tuberculosis, without mention of bacteriological or histological confirmation\n --PARENT--> [1B10] Tuberculosis of the respiratory system\n Def: This is a progressive or chronic disease resulting from infection with the bacterium Mycobacterium tuberculosis or other bacteria in the M. tuberculosis complex: M. bovis, M. africanum, M. canetti, M....\n --CHILD--> [1B10.Z] Respiratory tuberculosis, without mention of bacteriological or histological confirmation" ]	MD22	Haemoptysis	[ { "from_icd11": "MD22", "icd10_code": "R042", "icd10_title": "Hemoptysis" }, { "from_icd11": "1F85", "icd10_code": "B664", "icd10_title": "Paragonimiasis" }, { "from_icd11": "1B10.Z", "icd10_code": "A162", "icd10_title": "" }, { "from_icd11": "1B10.Z", "icd10_code": "A163", "icd10_title": "" }, { "from_icd11": "1B10.Z", "icd10_code": "A164", "icd10_title": "" }, { "from_icd11": "1B10.Z", "icd10_code": "A165", "icd10_title": "" }, { "from_icd11": "1B10.Z", "icd10_code": "A167", "icd10_title": "" }, { "from_icd11": "1B10.Z", "icd10_code": "A168", "icd10_title": "" }, { "from_icd11": "1B10.Z", "icd10_code": "A169", "icd10_title": "" }, { "from_icd11": "KB28.Z", "icd10_code": "P26", "icd10_title": "Pulmonary hemorrhage originating in the perinatal period" }, { "from_icd11": "KB28.Z", "icd10_code": "P261", "icd10_title": "Massive pulmonary hemorrhage originating in the perinatal period" }, { "from_icd11": "KB28.Z", "icd10_code": "P268", "icd10_title": "Other pulmonary hemorrhages originating in the perinatal period" }, { "from_icd11": "KB28.Z", "icd10_code": "P269", "icd10_title": "Unspecified pulmonary hemorrhage originating in the perinatal period" }, { "from_icd11": "LA75.1", "icd10_code": "Q333", "icd10_title": "Agenesis of lung" }, { "from_icd11": "CA40.Z", "icd10_code": "J189", "icd10_title": "Pneumonia, unspecified organism" } ]	R042	Hemoptysis
A 73-year-old man underwent periodic surveillance for abdominal aortic aneurysm (AAA) and asymptomatic PAD at the Division of Cardiovascular Surgery in our hospital. The patient was a skilled, active dentist. His resting ankle–brachial index (ABI) was 0.55 and 0.52 for the right and left sides, respectively. Computed tomography (CT) indicated an AAA of 38 mm, PAD, the possibility of rectal arteriovenous malformation, and the increased wall thickness of the sigmoid colon with regional lymph node swelling . The IMA was well developed and measured more than 5 mm in diameter on CT angiography. Colonoscopy revealed advanced sigmoid colon cancer , and our initial diagnosis was cT4aN1bM0, cStage IIIB sigmoid colon cancer according to the 8th edition of the Union for Cancer Control TNM classification. The patient also had dilated cardiomyopathy. Echocardiograms revealed a left ventricular ejection fraction of 35–40% with regular administration of β-blockers, angiotensin-converting-enzyme inhibitors, and diuretics. Two major concerns were related to successful cancer treatment. First, ligation of the IMA or superior rectal artery (SRA), which is a vital procedure in CME for advanced sigmoid colon cancer, could result in insufficient blood flow and ischemia of the lower limbs. Second, the procedure could result in insufficient blood supply to the remaining rectum and increase the risk of leakage in the remaining rectosigmoid anastomosis. In the treatment of symptomatic PAD, CT angiography is a potentially less invasive and adequate technique to plan for the provision of additional blood supply during surgery. However, evaluation of blood distribution after shutting off the IMA blood flow was not sufficient, and further angiographic work-up using a balloon-occlusion catheter by interventional radiologists revealed that his lower limbs were receiving collateral blood flow from the internal iliac branches, with the flow being worse in the left limb. The flows of the internal iliac arteries were sustained by the prominent IMA blood flow through the collateral mid-rectal arteries and collateral lumbar arteries . In a multidisciplinary conference with interventional radiologists and cardiovascular surgeons, a left axillofemoral bypass graft was preferred to avoid ischemia of the lower limbs. Because of the low patency rate of an axillofemoral bypass graft , a “Y-shaped” connection to the bilateral femoral artery was not proposed to preserve the right femoral artery in case of graft occlusion. Additionally, the possibility of conversion from laparoscopic surgery to laparotomy requiring a median hypogastric incision traversing the Y-shaped bypass could not be ruled out. The patient underwent surgical angioplasty of the left common to the superficial femoral artery and axillofemoral artery bypass . Another multidisciplinary conference was held among cardiovascular physicians, anesthetists, perioperative nurses, and gastrointestinal surgeons. Retrograde blood flow through the obturator artery to the remaining rectum can be expected after IMA ligation. However, taking the patient’s comorbidities into consideration, the cardiovascular physicians warned that his heart would not overcome the pan-peritonitis if an anastomotic leak occurred. Although indocyanine green fluorescence imaging (ICG-FI) was used intraoperatively to confirm blood supply to the remaining rectum, we proposed Hartmann’s procedure and obtained the patient’s consent. A month after the bypass, the patient underwent laparoscopic sigmoid colectomy and D3 lymph node dissection with IMA preservation, composed of preservation of the IMA to the left colic artery and ligation of the SRA . Anesthetists and perioperative nurses proposed oxygen saturation monitoring of both feet during laparoscopic surgery, which required extended lithotomy in the Trendelenburg position . The mobilization of the rectum was limited down to the peritoneal reflection so as not to damage the middle rectal artery (MRA), which provided collateral blood flow. The MRA fed the patient’s lower limbs until ligation of the SRA and provided retrograde flow to the remaining rectum after the sigmoid colectomy. Although ICG-FI revealed good perfusion of the remaining rectum, anastomosis was omitted as planned. The cardiovascular surgeon evaluated the blood flow at both feet by Doppler echo immediately after surgery in the operating room and confirmed sufficient blood flow to the lower limbs. The patient’s postoperative course was uneventful, and he was discharged 10 days postoperatively. Pathological examination of the specimen showed a pT3N0M0, pStage IIA tumor. The patient resumed work a month after the resection and was followed up for a year with no evidence of tumor recurrence. Fig. 1 Computed tomography images from periodic surveillance for abdominal aortic aneurysm and asymptomatic peripheral artery disease. a Severe calcification was observed between the abdominal aorta and both femoral arteries. Both external iliac arteries were disrupted. b Well-demarcated wall thickening was found in the sigmoid colon. c The inferior mesenteric artery was well developed and measured more than 5 mm in diameter. A dilated inferior mesenteric vein was winding along the inferior mesenteric artery Fig. 2 Colonoscopic image of advanced sigmoid colon cancer. The tumor was categorized as type 2 and occupied four-fifths of the circumference. The scope did not pass through the cancer canal. Well-differentiated adenocarcinoma was evident from the biopsy specimen Fig. 3 Images from angiographic evaluation by interventional radiology. a Abdominal aortography with inferior mesenteric artery (IMA) balloon occlusion showed developed collateral lumber arteries feeding the lower extremities. b The early phase of IMA selective angiography showed a prominent IMA flow. c In IMA selective angiography, retrograde blood flow of the internal iliac arteries was sustained by IMA blood flow through the collateral mid-rectal arteries Fig. 4 Computed tomography angiography taken after the surgeries. a After the axillofemoral bypass. Excellent blood flow to the left extremity through the bypass was proven. b After the laparoscopic sigmoid colectomy with inferior mesenteric artery preservation, composed of preservation of the inferior mesenteric artery to left colic artery and ligation of the superior rectal artery Fig. 5 To visualize sufficient blood flow to the lower extremities, oxygen saturation was monitored in both feet during laparoscopic sigmoid colectomy, which required extended lithotomy in the Trendelenburg position	4.199219	0.942383	sec[1]/p[0]	en	0.999997	34417902	https://doi.org/10.1186/s40792-021-01274-9	[ "artery", "blood", "flow", "sigmoid", "arteries", "cancer", "bypass", "rectal", "colon", "remaining" ]	[ { "code": "BD5Z", "title": "Diseases of arteries or arterioles, unspecified" }, { "code": "BD52", "title": "Certain specified disorders of arteries or arterioles" }, { "code": "BD52.3", "title": "Rupture of artery" }, { "code": "BD52.2", "title": "Stricture of artery" }, { "code": "BD40.Z", "title": "Atherosclerotic chronic arterial occlusive disease, unspecified" }, { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" }, { "code": "MA12.1", "title": "Finding of cocaine in blood" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "MA12.2", "title": "Finding of hallucinogen in blood" } ]	=== ICD-11 CODES FOUND === [BD5Z] Diseases of arteries or arterioles, unspecified Also known as: Diseases of arteries or arterioles, unspecified \| artery disease NOS \| arterial disease NOS \| arteriolar disease NOS \| disorder of artery NOS [BD52] Certain specified disorders of arteries or arterioles Also known as: Certain specified disorders of arteries or arterioles \| Aortic dilatation - joint hypermobility - arterial tortuosity \| Generalised arterial calcification of infancy \| Median arcuate ligament syndrome \| Aortic root abscess Excludes: collagen (vascular) diseases \| Hypersensitivity angiitis \| Acute arterial occlusion [BD52.3] Rupture of artery Also known as: Rupture of artery \| ruptured artery \| artery fistula \| Aortic duodenal fistula \| Aortic colon fistula Excludes: traumatic rupture of artery - see injury of blood vessel by body region [BD52.2] Stricture of artery Also known as: Stricture of artery \| arterial stenosis \| arterial stricture \| artery stricture \| stenosis of artery [BD40.Z] Atherosclerotic chronic arterial occlusive disease, unspecified Also known as: Atherosclerotic chronic arterial occlusive disease, unspecified \| Atherosclerotic chronic arterial occlusive disease \| arteriosclerosis, NOS \| generalised atherosclerosis \| atherosclerosis NOS [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS [MA12.1] Finding of cocaine in blood Also known as: Finding of cocaine in blood \| cocaine in blood [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [MA12.2] Finding of hallucinogen in blood Also known as: Finding of hallucinogen in blood \| hallucinogen in blood === GRAPH WALKS === --- Walk 1 --- [BD5Z] Diseases of arteries or arterioles, unspecified --PARENT--> [?] Diseases of arteries or arterioles --CHILD--> [?] Chronic arterial occlusive disease --- Walk 2 --- [BD5Z] Diseases of arteries or arterioles, unspecified --PARENT--> [?] Diseases of arteries or arterioles --CHILD--> [?] Chronic arterial occlusive disease --- Walk 3 --- [BD52] Certain specified disorders of arteries or arterioles --CHILD--> [BD52.2] Stricture of artery --PARENT--> [BD52] Certain specified disorders of arteries or arterioles --- Walk 4 --- [BD52] Certain specified disorders of arteries or arterioles --CHILD--> [BD52.2] Stricture of artery --PARENT--> [BD52] Certain specified disorders of arteries or arterioles --- Walk 5 --- [BD52.3] Rupture of artery --PARENT--> [BD52] Certain specified disorders of arteries or arterioles --EXCLUDES--> [?] Nonorgan specific systemic autoimmune disorders --- Walk 6 --- [BD52.3] Rupture of artery --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes Def: !markdown In the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre... --EXCLUDES--> [?] Stress fracture, not elsewhere classified	[ "[BD5Z] Diseases of arteries or arterioles, unspecified\n --PARENT--> [?] Diseases of arteries or arterioles\n --CHILD--> [?] Chronic arterial occlusive disease", "[BD5Z] Diseases of arteries or arterioles, unspecified\n --PARENT--> [?] Diseases of arteries or arterioles\n --CHILD--> [?] Chronic arterial occlusive disease", "[BD52] Certain specified disorders of arteries or arterioles\n --CHILD--> [BD52.2] Stricture of artery\n --PARENT--> [BD52] Certain specified disorders of arteries or arterioles", "[BD52] Certain specified disorders of arteries or arterioles\n --CHILD--> [BD52.2] Stricture of artery\n --PARENT--> [BD52] Certain specified disorders of arteries or arterioles", "[BD52.3] Rupture of artery\n --PARENT--> [BD52] Certain specified disorders of arteries or arterioles\n --EXCLUDES--> [?] Nonorgan specific systemic autoimmune disorders", "[BD52.3] Rupture of artery\n --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes\n Def: !markdown\nIn the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre...\n --EXCLUDES--> [?] Stress fracture, not elsewhere classified" ]	BD5Z	Diseases of arteries or arterioles, unspecified	[ { "from_icd11": "BD5Z", "icd10_code": "I7389", "icd10_title": "Other specified peripheral vascular diseases" }, { "from_icd11": "BD5Z", "icd10_code": "I7419", "icd10_title": "Embolism and thrombosis of other parts of aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7411", "icd10_title": "Embolism and thrombosis of thoracic aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7410", "icd10_title": "Embolism and thrombosis of unspecified parts of aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7381", "icd10_title": "Erythromelalgia" }, { "from_icd11": "BD5Z", "icd10_code": "I745", "icd10_title": "Embolism and thrombosis of iliac artery" }, { "from_icd11": "BD5Z", "icd10_code": "I789", "icd10_title": "Disease of capillaries, unspecified" }, { "from_icd11": "BD5Z", "icd10_code": "I748", "icd10_title": "Embolism and thrombosis of other arteries" }, { "from_icd11": "BD5Z", "icd10_code": "I749", "icd10_title": "Embolism and thrombosis of unspecified artery" }, { "from_icd11": "BD5Z", "icd10_code": "I781", "icd10_title": "Nevus, non-neoplastic" }, { "from_icd11": "BD5Z", "icd10_code": "I788", "icd10_title": "Other diseases of capillaries" }, { "from_icd11": "BD5Z", "icd10_code": "I744", "icd10_title": "Embolism and thrombosis of arteries of extremities, unspecified" }, { "from_icd11": "BD5Z", "icd10_code": "I70-I79", "icd10_title": "" }, { "from_icd11": "BD5Z", "icd10_code": "I74", "icd10_title": "Arterial embolism and thrombosis" }, { "from_icd11": "BD5Z", "icd10_code": "I73", "icd10_title": "Other peripheral vascular diseases" } ]	I7389	Other specified peripheral vascular diseases
A young female, 30 years old, presented in our clinic suffering a right side unilateral vestibular hypofunction (UVH) due to vestibular neuritis (VN) . At the time of diagnosis, she reported sudden and severe rotatory vertigo with associated autonomic symptoms over the previous 48 hours. Pure tone audiometry (PTA) in both ears was 5 dBHL; she had no tinnitus. The patient denied any previous history of related problems, although her mother was suffering from migraine headache. The patient was hospitalized three days, where she received intravenous steroids and antiemetics. Inner ear and cerebellar magnetic resonance imaging (MRI) were normal. She began vestibular rehabilitation treatment, with a progressive exercise regimen. Initially, she performed gaze stability and balance exercises 3–5 times a day in her home, for a total stimulus time of 20–40 min daily. The gaze stability exercises included the x 1 and x 2 paradigms for both near and far target distances. The balance exercises were provided to improve her use of vestibular information to maintain balance. We progressed these exercises by reducing the base of support, altering vision, and proprioceptive input (eyes open or closed; standing on a firm or soft surface). The gait exercises included walking in tandem, with eyes closed, with cephalic movement in the sagittal and horizontal planes. Habituation exercises were indicated based on the result of the 16 movements from the Motion Sensitivity Quotient . The habituation movements included 4 repetitions, 4 times a day, until the exercises did not generate any symptoms for 48 hours, at which time the patient suspended them. Videonystagmography (VNG) showed unilateral weakness of the right ear at 78%. At the time of discharge, her Dizziness Handicap Inventory (DHI) was 66. The patient continued with this rehabilitation treatment for 9 months, after which the final DHI improved to 36. Although she said that she did all the prescribed exercises, she reported blurred vision and a permanent dizzy sensation. She decided to discontinue treatment but returned to the clinic after three months with the same symptoms. Additionally, she now reported a new onset of periodic headaches that did not fulfill migraine or vestibular migraine criteria . A repeated VNG exam showed spontaneous nystagmus towards the left, with a slow phase velocity (SPV) of 7°/sec. At that moment DHI was 54; the Motion Sensitivity Quotient (MSQ) was 11.81 points; Functional Gait Assessment (FGA) was normal; and modified Clinical Test of Sensory Interaction and Balance (mCTSIB) was 120/120. She restarted vestibular rehabilitation and after 10 sessions her DHI was not better (64 points). Laboratory studies showed normal levels for FAN (Antinuclear Factor), folic acid, Anti-DNA, ionogram, magnesium, calcium, proteinogram, VDRL, and vitamin B12. However, she reported that her headaches had become premenstrual and they now fulfilled the criteria for a migraine diagnosis. She was started on 12.5 mg oral amitriptyline daily and prescribed dietary measures. Subsequently this patient reported less blurred vision and dizziness and no headaches for two months. A new MRI and an angiocerebral MRI were normal. Nevertheless, the patient returned two months later complaining of persistent dizziness, blurred vision, and no benefits from the amitriptyline in spite of the fact that the dosage had been raised to 50 mg daily. We modified her treatment to start vestibular rehabilitation again (her initial DHI was 40) and she was started on 25 mg oral topiramate daily. The patient did not tolerate this medication and discontinued its use. Next, she was prescribed 10 mg of flunarizine daily with good tolerance. Repeated VNG showed spontaneous left nystagmus with a SPV of 3°/sec. Her DHI was now 34, but her clinical dynamic visual acuity was abnormal showing a 6-line difference from static visual acuity. During the next 4 months, the patient did not come to the clinic, after which period of time she came in and was treated with 25 mg of oral venlafaxine daily (provided by another clinic). At this point, we tested with the vHIT , which showed a gain of 0.57 in the right horizontal canal with overt and covert saccades . The other semicircular canals had normal gains. Electrocochleography was normal, Brainstem Auditory Evoked Response was normal, a new MRI was normal, and PTA was normal. A 4th VNG showed spontaneous horizontal nystagmus to the right, with a SPV of −3°/sec (was interpreted as a recovery nystagmus), and an orthoptic evaluation was normal. Though she said she still suffered from blurred vision and dizziness, she reported fewer headaches. We prescribed 24 mg daily of betahistine (8 mg every 8 hours) to improve compensation. While the patient experienced an improvement, she reported that the dizziness persisted throughout the day. She began working again but had to stop 1 month later due to blurred vision, headaches, and dizziness. Once again, she began vestibular rehabilitation plus psychological therapy. This combination of therapies resulted in some improvement, though her symptoms persisted during head movement, especially in the dark. Her constant blurred vision made it difficult for her to read. Her 5th VNG showed that a spontaneous nystagmus to the left was 1.8°/sec, while the vHIT was the same as before. Based on persistent symptoms and lack of improvement with traditional vestibular rehabilitation, we elected to treat her using passive and predictive, yaw head impulses towards the affected side only. This was done for five consecutive days. Video HIT was used to ensure that the velocity of impulse was correct to stimulate her right horizontal canal in the field of fast movements. When the patient did the head impulse exercises, she sat in front of a solid black circle of 10 mm in diameter on a white background placed at one meter. This circle was positioned at the same level or height as her occipitonasal axis. Stimulation of the affected semicircular canal was done with 10 series of 15 passive head impulses (done by the therapist) with 30 seconds of rest between each series. The initial head position was such that the patient's gaze was centered on the point in front of her with ±2° between the horizontal and vertical planes . The head impulses were small and fast, with a peak amplitude of 15 degrees, a peak velocity of 150°/sec, and peak acceleration of 3000°/sec; the return to the initial position was slow. Video HIT equipment was used to monitor the velocity and amplitude of movements, which were corrected when necessary.	4.09375	0.972656	sec[1]/p[0]	en	0.999997	28243476	https://doi.org/10.1155/2017/2145173	[ "vestibular", "exercises", "that", "daily", "vision", "this", "rehabilitation", "dizziness", "blurred", "head" ]	[ { "code": "AB34.Z", "title": "Disorders of vestibular function, unspecified" }, { "code": "LA22.4", "title": "Structural developmental anomalies of inner ear" }, { "code": "AB30.Z", "title": "Acute vestibular syndrome, unspecified" }, { "code": "AB32.Z", "title": "Chronic vestibular syndrome, unspecified" }, { "code": "AB31.Z", "title": "Episodic vestibular syndrome, unspecified" }, { "code": "QE20", "title": "Lack of physical exercise" }, { "code": "QB95.1", "title": "Physical rehabilitation" }, { "code": "NF06.Z", "title": "Effects of strenuous physical exercise, unspecified" }, { "code": "NF06.Y", "title": "Other specified effects of strenuous physical exercise" }, { "code": "8A83", "title": "Other primary headache disorder" } ]	=== ICD-11 CODES FOUND === [AB34.Z] Disorders of vestibular function, unspecified Also known as: Disorders of vestibular function, unspecified \| Disorders of vestibular function \| Vestibulopathies \| vestibular disorder \| disease or disorder of labyrinth of ear [LA22.4] Structural developmental anomalies of inner ear Definition: Any condition caused by failure of the inner ear to correctly develop during the antenatal period. Also known as: Structural developmental anomalies of inner ear \| congenital abnormal shape of inner ear \| congenital abnormality of inner ear \| congenital anomaly of inner ear \| congenital malformation of inner ear [AB30.Z] Acute vestibular syndrome, unspecified Also known as: Acute vestibular syndrome, unspecified \| Acute vestibular syndrome [AB32.Z] Chronic vestibular syndrome, unspecified Also known as: Chronic vestibular syndrome, unspecified \| Chronic vestibular syndrome [AB31.Z] Episodic vestibular syndrome, unspecified Also known as: Episodic vestibular syndrome, unspecified \| Episodic vestibular syndrome [QE20] Lack of physical exercise Also known as: Lack of physical exercise \| sedentary lifestyle [QB95.1] Physical rehabilitation Also known as: Physical rehabilitation \| admission for physical rehabilitation \| admission for physiotherapy \| physio \| Physical rehabilitation other than cardiac rehabilitation Excludes: Cardiac rehabilitation [NF06.Z] Effects of strenuous physical exercise, unspecified Also known as: Effects of strenuous physical exercise, unspecified \| Effects of strenuous physical exercise [NF06.Y] Other specified effects of strenuous physical exercise Also known as: Other specified effects of strenuous physical exercise [8A83] Other primary headache disorder Definition: A group of clinically heterogeneous headache disorders, believed to be primary. Although largely unrelated, they fall into four categories: headaches associated with physical exertion; headaches attributed to direct physical but innocuous stimuli; epicranial headaches; and other miscellaneous primary headache disorders. Also known as: Other primary headache disorder \| Primary cough headache \| Primary exercise headache \| Primary headache associated with sexual activity \| Preorgasmic headache === GRAPH WALKS === --- Walk 1 --- [AB34.Z] Disorders of vestibular function, unspecified --PARENT--> [AB34] Disorders of vestibular function --CHILD--> [AB34.Y] Other specified disorders of vestibular function --- Walk 2 --- [AB34.Z] Disorders of vestibular function, unspecified --PARENT--> [AB34] Disorders of vestibular function --CHILD--> [AB34.Y] Other specified disorders of vestibular function --- Walk 3 --- [LA22.4] Structural developmental anomalies of inner ear Def: Any condition caused by failure of the inner ear to correctly develop during the antenatal period.... --PARENT--> [LA22] Structural developmental anomalies of ear causing hearing impairment Def: Any condition caused by the failure of the ear to correctly develop during the antenatal period. These conditions are characterised by hearing impairment.... --CHILD--> [LA22.1] Anotia Def: Complete absence of any auricular structures.... --- Walk 4 --- [LA22.4] Structural developmental anomalies of inner ear Def: Any condition caused by failure of the inner ear to correctly develop during the antenatal period.... --PARENT--> [LA22] Structural developmental anomalies of ear causing hearing impairment Def: Any condition caused by the failure of the ear to correctly develop during the antenatal period. These conditions are characterised by hearing impairment.... --CHILD--> [LA22.0] Microtia Def: Microtia is a congenital malformation of variable severity of the external and middle ear. Both hereditary factors (evidence for familial craniofacial microsomia and patterns suggestive of multifactor... --- Walk 5 --- [AB30.Z] Acute vestibular syndrome, unspecified --PARENT--> [AB30] Acute vestibular syndrome Def: A clinical syndrome of acute-onset, continuous vertigo, dizziness, or unsteadiness lasting days to weeks, and generally including features suggestive of new, ongoing vestibular system dysfunction (e.g... --CHILD--> [AB30.Y] Other specified acute vestibular syndrome --- Walk 6 --- [AB30.Z] Acute vestibular syndrome, unspecified --PARENT--> [AB30] Acute vestibular syndrome Def: A clinical syndrome of acute-onset, continuous vertigo, dizziness, or unsteadiness lasting days to weeks, and generally including features suggestive of new, ongoing vestibular system dysfunction (e.g... --CHILD--> [AB30.1] Labyrinthitis Def: Labyrinthitis is an inflammatory disorder of the inner ear (labyrinth) producing disturbances of balance and hearing to varying degrees. It can be caused by bacterial or viral infections and autoimmun...	[ "[AB34.Z] Disorders of vestibular function, unspecified\n --PARENT--> [AB34] Disorders of vestibular function\n --CHILD--> [AB34.Y] Other specified disorders of vestibular function", "[AB34.Z] Disorders of vestibular function, unspecified\n --PARENT--> [AB34] Disorders of vestibular function\n --CHILD--> [AB34.Y] Other specified disorders of vestibular function", "[LA22.4] Structural developmental anomalies of inner ear\n Def: Any condition caused by failure of the inner ear to correctly develop during the antenatal period....\n --PARENT--> [LA22] Structural developmental anomalies of ear causing hearing impairment\n Def: Any condition caused by the failure of the ear to correctly develop during the antenatal period. These conditions are characterised by hearing impairment....\n --CHILD--> [LA22.1] Anotia\n Def: Complete absence of any auricular structures....", "[LA22.4] Structural developmental anomalies of inner ear\n Def: Any condition caused by failure of the inner ear to correctly develop during the antenatal period....\n --PARENT--> [LA22] Structural developmental anomalies of ear causing hearing impairment\n Def: Any condition caused by the failure of the ear to correctly develop during the antenatal period. These conditions are characterised by hearing impairment....\n --CHILD--> [LA22.0] Microtia\n Def: Microtia is a congenital malformation of variable severity of the external and middle ear. Both hereditary factors (evidence for familial craniofacial microsomia and patterns suggestive of multifactor...", "[AB30.Z] Acute vestibular syndrome, unspecified\n --PARENT--> [AB30] Acute vestibular syndrome\n Def: A clinical syndrome of acute-onset, continuous vertigo, dizziness, or unsteadiness lasting days to weeks, and generally including features suggestive of new, ongoing vestibular system dysfunction (e.g...\n --CHILD--> [AB30.Y] Other specified acute vestibular syndrome", "[AB30.Z] Acute vestibular syndrome, unspecified\n --PARENT--> [AB30] Acute vestibular syndrome\n Def: A clinical syndrome of acute-onset, continuous vertigo, dizziness, or unsteadiness lasting days to weeks, and generally including features suggestive of new, ongoing vestibular system dysfunction (e.g...\n --CHILD--> [AB30.1] Labyrinthitis\n Def: Labyrinthitis is an inflammatory disorder of the inner ear (labyrinth) producing disturbances of balance and hearing to varying degrees. It can be caused by bacterial or viral infections and autoimmun..." ]	AB34.Z	Disorders of vestibular function, unspecified	[ { "from_icd11": "AB34.Z", "icd10_code": "H829", "icd10_title": "Vertiginous syndromes in diseases classified elsewhere, unspecified ear" }, { "from_icd11": "AB34.Z", "icd10_code": "H818X9", "icd10_title": "Other disorders of vestibular function, unspecified ear" }, { "from_icd11": "AB34.Z", "icd10_code": "H8190", "icd10_title": "Unspecified disorder of vestibular function, unspecified ear" }, { "from_icd11": "AB34.Z", "icd10_code": "H818X2", "icd10_title": "Other disorders of vestibular function, left ear" }, { "from_icd11": "AB34.Z", "icd10_code": "H8191", "icd10_title": "Unspecified disorder of vestibular function, right ear" }, { "from_icd11": "AB34.Z", "icd10_code": "H818X3", "icd10_title": "Other disorders of vestibular function, bilateral" }, { "from_icd11": "AB34.Z", "icd10_code": "H8192", "icd10_title": "Unspecified disorder of vestibular function, left ear" }, { "from_icd11": "AB34.Z", "icd10_code": "H8193", "icd10_title": "Unspecified disorder of vestibular function, bilateral" }, { "from_icd11": "AB34.Z", "icd10_code": "H81", "icd10_title": "Disorders of vestibular function" }, { "from_icd11": "AB34.Z", "icd10_code": "H818", "icd10_title": "Other disorders of vestibular function" }, { "from_icd11": "AB34.Z", "icd10_code": "H819", "icd10_title": "Unspecified disorder of vestibular function" }, { "from_icd11": "AB34.Z", "icd10_code": "H82", "icd10_title": "Vertiginous syndromes in diseases classified elsewhere" }, { "from_icd11": "LA22.4", "icd10_code": "Q165", "icd10_title": "Congenital malformation of inner ear" }, { "from_icd11": "QE20", "icd10_code": "Z723", "icd10_title": "Lack of physical exercise" }, { "from_icd11": "QE20", "icd10_code": "Z642", "icd10_title": "" } ]	H829	Vertiginous syndromes in diseases classified elsewhere, unspecified ear
A 48-year-old female patient presented in December 2022 with a complaint of acute painless diminution of vision OS that occurred approximately 2 months earlier. History taking revealed that visual symptoms started four days after coronary artery bypass grafting (CABG) which was performed two months earlier for an acute myocardial ischemic (MI) event. Furthermore, the patient reported undergoing percutaneous coronary intervention (PCI) one year before for another myocardial ischemic event. The rather late presentation after nearly two months from the onset of visual symptoms was explained by the patient being admitted in the surgical intensive care unit (ICU) and inpatient ward for two weeks following CABG. At the time of presentation, BCVA was 6/6 OD and 3/60 OS. Anterior segment examination was unremarkable OU. Conversely, fundus examination OS revealed multiple yellowish-white lesions i.e. cotton-wool spots exclusively in the posterior pole. Fundoscopic examination OD was completely normal. FFA was delayed until 6 weeks after the onset of visual symptoms because the patient was admitted in the surgical ICU and inpatient ward for two weeks after CABG. Also, the cardiothoracic surgeon recommended that FFA be deferred for a few more weeks until the patient’s general condition stabilized which further contributed to the late presentation. FFA OS showed delayed arterial and venous filling, prolonged arterio-venous transit (A-V transit), patchy choroidal filling with choroidal dropouts, mid-peripheral retinal microaneurysms, peripheral retinal ischemia, widened irregular foveal avascular zone (FAZ) and late perivascular staining . SD-OCT and OCTA OS were performed at presentation which revealed multiple inner retinal hyperreflectivities that corresponded to the cotton-wool spots . It also showed diffuse macular thinning predominantly affecting inner retinal layers which, we hypothesize, was the sequelae of Purtscher flecken that had already resolved given the late presentation of the patient 2 months after the onset of symptoms . Furthermore, OCTA showed decreased macular vascular density, optic nerve head (ONH) and radial peripapillary capillary density (RPCD) OS compared to OD . Our initial differential diagnosis included central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO), cotton-wool spots caused by surgery-related anemia or bacteremia, retinitis and Purtscher-like retinopathy. However, CRAO and BRAO were discarded on basis of patchy, multifocal, discrete yellowish-white inner retinal lesions as opposed to the diffuse or sectoral retinal whitening characteristic of CRAO and BRAO respectively. Anemia and bacteremia were excluded because complete blood count (CBC) showed only mild microcytic hypochromic anemia with blood hemoglobin level of 10.2 gm/dl not sufficient to cause extensive cotton-wool spots. Also, CBC was negative for leukocytosis and bandemia and there was no history of fever which are important markers of bacteremia. In addition, retinitis was excluded due to the relatively poor vision that was inconsistent with the foveal-sparing lesions, were they to be retinitis and also on the basis of absence of inflammatory signs in both the posterior and anterior segments. Moreover, the angiographic findings of delayed arterial and venous filling and prolonged arteriovenous (A-V) transit time highly suggested a vascular rather than an inflammatory etiology particularly in a patient with history of two episodes of acute myocardial ischemia. Therefore, a diagnosis of Purtscher-like retinopathy was made based on the multiple cotton-wool spots with the exclusive posterior pole distribution, the supporting FFA and SD-OCT findings elaborated earlier as well as the presence of a suggestive concurrent systemic cause i.e. APS which was diagnosed later. This is consistent with the diagnostic criteria of Purtscher retinopathy proposed by Miguel et al. where a diagnosis of Purtscher retinopathy is made when a patient has at least three of the five stated criteria which include Purtscher flecken, retinal hemorrhages in low to moderate number, cotton-wool spots confined to the posterior pole, a probable explanatory association and complementary investigations compatible with the diagnosis . Our case fulfills three of these criteria including the cotton-wool spots confined to the posterior pole, the probable cause which is APS and the compatible investigative findings in FFA, SD-OCT and OCTA . We also hypothesize that the patient probably had Purtscher flecken, that had nonetheless resolved at the rather late time of presentation 2 months after the onset of symptoms. However, the marked inner retinal thinning highly suggested that Purtscher flecken may have indeed developed at an earlier stage of the disease. A diagnosis of Purtscher-like retinopathy in a young female patient with systemic manifestations of occlusive vasculopathy necessitated further evaluation for an underlying systemic cause that, we suggest, was the causative factor of both the myocardial ischemia and the Purtscher-like retinopathy. So, the patient was referred to a rheumatologist who diagnosed her with primary APS based on the arterial thrombotic events i.e. myocardial infarctions, retinal microvascular thrombosis i.e. Purtscher-like retinopathy, thrombosed segments of radial, ulnar, anterior tibial, posterior tibial and peroneal arteries in arterial duplex and CT angiography and positive anticardiolipin antibodies (aCL) according to the updated Sapporo classification of APS . Other laboratory investigations, performed by the rheumatologist, included CBC, liver enzymes i.e. alanine transaminase (ALT) and aspartate transaminase (AST), kidney function tests i.e. serum creatinine and blood urea, rheumatoid factor (RF), antinuclear antibodies (ANA), anti-double-stranded DNA antibodies (dsDNA Abs), β2GP and LA. RF, ANA and dsDNA Abs were negative excluding SLE which is a common cause of secondary APS. CBC was positive for mild microcytic hypochromic anemia with other parameters being within normal ranges. Also, liver enzymes and kidney function tests were normal. Despite β2GP and LA being negative, persistently high aCL together with the aforementioned clinical signs supported a diagnosis of primary APS according to the updated Sapporo classification of APS . Consequently, the patient was hospitalized and received anticoagulants, pulsed steroid therapy and cyclophosphamide. In conclusion, the patient was diagnosed as Purtscher-like retinopathy secondary to APS which may have been precipitated by CABG.	4.128906	0.974121	sec[1]/p[0]	en	0.999997	PMC10157585	https://doi.org/10.1186/s12886-023-02935-z	[ "purtscher", "retinal", "that", "which", "retinopathy", "cotton", "wool", "spots", "like", "myocardial" ]	[ { "code": "9B7Z", "title": "Disorders of the retina, unspecified" }, { "code": "9B74.Z", "title": "Retinal vascular occlusions, unspecified" }, { "code": "9B73.4", "title": "Retinal breaks without detachment" }, { "code": "9B78.9", "title": "Retinal atrophy" }, { "code": "9B7Y", "title": "Other specified disorders of the retina" }, { "code": "8A80.Z", "title": "Migraine, unspecified" }, { "code": "QA76", "title": "Medication or substance that is known to be an allergen without injury or harm" }, { "code": "PL13.6", "title": "Medication or substance that is known to be an allergen, as mode of injury or harm" }, { "code": "9C40.A0", "title": "Papilloedema" }, { "code": "PA6Z", "title": "Unintentional fall from unspecified height" } ]	=== ICD-11 CODES FOUND === [9B7Z] Disorders of the retina, unspecified Also known as: Disorders of the retina, unspecified \| retinal disease \| retinal lesion NOS [9B74.Z] Retinal vascular occlusions, unspecified Also known as: Retinal vascular occlusions, unspecified \| Retinal vascular occlusions \| occlusion of retinal vessels \| retinal obstruction [9B73.4] Retinal breaks without detachment Also known as: Retinal breaks without detachment \| Retinal break NOS \| ruptured retina \| Horseshoe tear of retina without detachment \| Round hole of retina without detachment Includes: Horseshoe tear of retina without detachment \| Round hole of retina without detachment Excludes: Chorioretinal scars after surgery for detachment \| peripheral retinal degeneration without break [9B78.9] Retinal atrophy Definition: This is a group of genetic diseases and is characterised by the bilateral degeneration of the retina, causing progressive vision loss culminating in blindness. Also known as: Retinal atrophy \| atrophic retina [9B7Y] Other specified disorders of the retina Also known as: Other specified disorders of the retina \| Åland Island eye disease \| Cone dystrophy with supernormal rod response \| Familial retinal arterial macroaneurysm \| IRVAN syndrome [8A80.Z] Migraine, unspecified Also known as: Migraine, unspecified \| Migraine [QA76] Medication or substance that is known to be an allergen without injury or harm Definition: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm. Also known as: Medication or substance that is known to be an allergen without injury or harm Excludes: Medication or substance that is known to be an allergen, as mode of injury or harm [PL13.6] Medication or substance that is known to be an allergen, as mode of injury or harm Also known as: Medication or substance that is known to be an allergen, as mode of injury or harm \| medication error relating to known allergy to drug or substance \| adverse drug event relating to known allergy to drug or substance [9C40.A0] Papilloedema Definition: Optic disc swelling that results from increased intracranial pressure Also known as: Papilloedema \| Optic disc swelling that results from increased intracranial pressure Includes: Optic disc swelling that results from increased intracranial pressure [PA6Z] Unintentional fall from unspecified height Also known as: Unintentional fall from unspecified height \| fall NOS \| accidental fall \| fall causing injury that resulted in death \| fell NOS === GRAPH WALKS === --- Walk 1 --- [9B7Z] Disorders of the retina, unspecified --PARENT--> [?] Disorders of the retina --RELATED_TO--> [?] Certain congenital malformations of posterior segment of eye --- Walk 2 --- [9B7Z] Disorders of the retina, unspecified --PARENT--> [?] Disorders of the retina --RELATED_TO--> [?] Neoplasms of retina Def: This refers to tumours of the light-sensitive layer of tissue, lining the inner surface of the eye. The optics of the eye create an image of the visual world on the retina, which serves much the same ... --- Walk 3 --- [9B74.Z] Retinal vascular occlusions, unspecified --PARENT--> [9B74] Retinal vascular occlusions Def: These are obstruction or closure of retinal vascular structures.... --PARENT--> [?] Disorders of the retina --- Walk 4 --- [9B74.Z] Retinal vascular occlusions, unspecified --PARENT--> [9B74] Retinal vascular occlusions Def: These are obstruction or closure of retinal vascular structures.... --CHILD--> [9B74.1] Retinal venous occlusions --- Walk 5 --- [9B73.4] Retinal breaks without detachment --EXCLUDES--> [?] Chorioretinal scars after surgery for detachment --PARENT--> [?] Postprocedural disorders of eye or ocular adnexa --- Walk 6 --- [9B73.4] Retinal breaks without detachment --EXCLUDES--> [?] Peripheral retinal degeneration --CHILD--> [?] Microcystoid degeneration of retina	[ "[9B7Z] Disorders of the retina, unspecified\n --PARENT--> [?] Disorders of the retina\n --RELATED_TO--> [?] Certain congenital malformations of posterior segment of eye", "[9B7Z] Disorders of the retina, unspecified\n --PARENT--> [?] Disorders of the retina\n --RELATED_TO--> [?] Neoplasms of retina\n Def: This refers to tumours of the light-sensitive layer of tissue, lining the inner surface of the eye. The optics of the eye create an image of the visual world on the retina, which serves much the same ...", "[9B74.Z] Retinal vascular occlusions, unspecified\n --PARENT--> [9B74] Retinal vascular occlusions\n Def: These are obstruction or closure of retinal vascular structures....\n --PARENT--> [?] Disorders of the retina", "[9B74.Z] Retinal vascular occlusions, unspecified\n --PARENT--> [9B74] Retinal vascular occlusions\n Def: These are obstruction or closure of retinal vascular structures....\n --CHILD--> [9B74.1] Retinal venous occlusions", "[9B73.4] Retinal breaks without detachment\n --EXCLUDES--> [?] Chorioretinal scars after surgery for detachment\n --PARENT--> [?] Postprocedural disorders of eye or ocular adnexa", "[9B73.4] Retinal breaks without detachment\n --EXCLUDES--> [?] Peripheral retinal degeneration\n --CHILD--> [?] Microcystoid degeneration of retina" ]	9B7Z	Disorders of the retina, unspecified	[ { "from_icd11": "9B7Z", "icd10_code": "H30-H36", "icd10_title": "" }, { "from_icd11": "9B7Z", "icd10_code": "H32", "icd10_title": "Chorioretinal disorders in diseases classified elsewhere" }, { "from_icd11": "9B7Z", "icd10_code": "H320", "icd10_title": "" }, { "from_icd11": "9B7Z", "icd10_code": "H328", "icd10_title": "" }, { "from_icd11": "9B74.Z", "icd10_code": "H348192", "icd10_title": "Central retinal vein occlusion, unspecified eye, stable" }, { "from_icd11": "9B74.Z", "icd10_code": "H348310", "icd10_title": "Tributary (branch) retinal vein occlusion, right eye, with macular edema" }, { "from_icd11": "9B74.Z", "icd10_code": "H348112", "icd10_title": "Central retinal vein occlusion, right eye, stable" }, { "from_icd11": "9B74.Z", "icd10_code": "H348122", "icd10_title": "Central retinal vein occlusion, left eye, stable" }, { "from_icd11": "9B74.Z", "icd10_code": "H34819", "icd10_title": "Central retinal vein occlusion, unspecified eye" }, { "from_icd11": "9B74.Z", "icd10_code": "H348392", "icd10_title": "Tributary (branch) retinal vein occlusion, unspecified eye, stable" }, { "from_icd11": "9B74.Z", "icd10_code": "H34812", "icd10_title": "Central retinal vein occlusion, left eye" }, { "from_icd11": "9B74.Z", "icd10_code": "H34813", "icd10_title": "Central retinal vein occlusion, bilateral" }, { "from_icd11": "9B74.Z", "icd10_code": "H348120", "icd10_title": "Central retinal vein occlusion, left eye, with macular edema" }, { "from_icd11": "9B74.Z", "icd10_code": "H348320", "icd10_title": "Tributary (branch) retinal vein occlusion, left eye, with macular edema" }, { "from_icd11": "9B74.Z", "icd10_code": "H34811", "icd10_title": "Central retinal vein occlusion, right eye" } ]	H30-H36
IUP is considered as the rarest type of ectopic pregnancy, with a rate of less than 1% , while the rate of IUP is expected to increase in the future due to the growing number of caesarean deliveries. Here we report a case of IUP implanted into the fundal myometrium after caesarean section and B-Lynch uterine compression suture. The etiologies and pathogenesis of IUP are unknown, possible causes include caesarean section, in vitro fertilization/embryo transfer, myomectomy, adenomyosis, or pelvic infection . B-Lynch suture is a surgical technique to control postpartum hemorrhage caused by uterine atony. It involves placing a compressive suture around the uterus to reduce its size and blood flow. In this case, B-Lynch suture was applied in a sling-like fashion on the right side of the fundus, which caused myometrial damage . The blastocyst implanted into the myometrial defect through a microscopic dehiscent tract. Moreover, the mechanical effect and the interruption of uterine blood supply by B-Lynch suture resulted in intrauterine adhesions . Because of the intrauterine adhesions and the absence of overlying endometrium, the fertilized ovum could not implant in the uterine cavity. In addition, the formation of the microscopic dehiscent tract in the myometrium made B-Lynch suture a risk factor for IUP. The diagnostic algorithm includes ultrasound, serum β-HCG levels, and a clinical examination. Early diagnosis of IUP can avoid serious complications. However, early diagnosis is sometimes difficult due to its various manifestations, and it might be mistaken for interstitial or cornual pregnancy. The diagnosis of IUP requires clear visualization of the endometrial–myometrial junction to delineate that the gestational sac is completely surrounded by the myometrium. TVS is generally the first imaging examination to be used , however, MRI may be a better way to evaluate the complete extent of this disease , and may help to delineate the uterine cavity better. In this case, the ultrasound scan showed that the gestational sac was located at the right side of the uterine fundus, and the myometrium near the lower endometrial cavity was thin, suggesting that the gestational sac was not completely surrounded by the uterine muscle. Therefore, the sonographer misdiagnosed it as a right cornual pregnancy. However, due to the endometrial fibrosis caused by B-Lynch, the MRI could not provide a full view of the endometrial–myometrial junction and endometrial lining, and thus could not distinguish the relationship between the gestational sac and the myometrium. So we did not recognize it as an IUP. It is worth mentioning that we hypothesized that there was a severe intrauterine adhesion because of the absence of endometrial lining and clinical symptoms of oligomenorrhea, until we proceeded with exploratory hysteroscopy to verify the conjecture. There is no universal treatment modality because of the few cases reported. Management of intramural pregnancies should be individualized. It depends on the location of the conception, degree of myometrial involvement, gestational age and desire to maintain pregnancy. Commonly available management options include expectant, medical (methotrexate ± vasopressin) and surgical (laparoscopy, hysteroscopy or laparotomy) treatment . Women need to be informed that IUP can lead to massive bleeding and high maternal mortality rates if they continue with the pregnancy. Ramkrishna pointed out that for patients with gestational age less than 8 weeks and no uterine rupture, medical treatment can be considered, but it has disadvantages such as long treatment duration, slow decline of serum β-HCG, and delayed surgical timing in case of uterine rupture. Surgical treatment includes laparoscopy, laparotomy, and hysteroscopy combined with laparoscopy , and the principle is to preserve the patient’s fertility function as much as possible. For cases where the gestational tissue is far from the serosal layer and difficult to locate by naked eye, hysteroscopy combined with TVS can accurately locate the implantation site of the gestational sac and clarify the relationship between the gestational sac and the endometrial cavity. In this case, considering that the IUP tissue has rich blood supply and the surrounding myometrium is thin, if conservative medical treatment is chosen, the risk of uterine rupture and massive bleeding is high. The patient has a strong desire for fertility, and surgical treatment can not only completely remove the lesion, shorten the treatment duration, but also repair the poorly healed scar after surgery. However, for patients with stable condition, small lesion, and clear diagnosis of IUP, ultrasound-guided puncture aspiration and local injection of MTX can be performed, which can preserve the integrity of the uterus and offer a new treatment option for IUP patients who have fertility requirements. LI et al . first reported the use of uterine artery embolization and medication to treat IUP which reduced the risk of uterine rupture and massive bleeding, and provided technical support for preserving the fertility of the patients. B-Lynch suture is very successful in avoiding the need for hysterectomy during refractory postpartum hemorrhage that does not respond to medical treatment . However, the impact of B-Lynch suture on future fertility potential needs further study. From this case, we learned that intrauterine adhesions are one of the important causes of IUP induced by B-Lynch, and also one of the difficulties in treating patients who have fertility requirements. In B-Lynch surgery, we should pay attention to reducing the damage to the uterine muscle layer and endometrium. If intrauterine adhesions occur after surgery, we need to remove the adhesions with hysteroscopy as soon as possible to restore the shape of the uterine cavity, and improve the endometrial growth with estrogen replacement therapy and reduce the impact on future fertility potential. After treatment, the β-hCG level should be closely monitored, and vaginal ultrasound and MRI should be performed if necessary, to be aware of the possibility of persistent ectopic pregnancy, or even more severe trophoblastic disease caused by IUP. We find this case quite unique for two reasons. One is that the gestational sac implanted into the microscopic dehiscent tract at the fundus caused by the B-Lynch uterine compression suture, rather than the caesarean section incision. The other is the fact that hysteroscopy revealed a severe intrauterine adhesion, which could be another reason for the result of IUP.	4.230469	0.915039	sec[2]/p[0]	en	0.999996	PMC10986008	https://doi.org/10.1186/s12905-024-03027-w	[ "uterine", "that", "lynch", "gestational", "suture", "endometrial", "fertility", "pregnancy", "myometrium", "this" ]	[ { "code": "GA1Z&XA99N3", "title": "Noninflammatory disorders of uterus, except cervix" }, { "code": "GA01.Z", "title": "Inflammatory disorders of the uterus, except cervix, unspecified" }, { "code": "GA16.Y", "title": "Other specified acquired abnormalities of uterus, except cervix" }, { "code": "NB92.6", "title": "Injury of uterus" }, { "code": "GC04.1Y", "title": "Other specified fistulae involving female genital tract" }, { "code": "8A80.Z", "title": "Migraine, unspecified" }, { "code": "QA76", "title": "Medication or substance that is known to be an allergen without injury or harm" }, { "code": "PL13.6", "title": "Medication or substance that is known to be an allergen, as mode of injury or harm" }, { "code": "9C40.A0", "title": "Papilloedema" }, { "code": "PA6Z", "title": "Unintentional fall from unspecified height" } ]	=== ICD-11 CODES FOUND === [GA01.Z] Inflammatory disorders of the uterus, except cervix, unspecified Also known as: Inflammatory disorders of the uterus, except cervix, unspecified \| Inflammatory disorders of the uterus, except cervix \| inflammatory disease of the uterus \| uterine inflammatory disease \| uterus inflammation [GA16.Y] Other specified acquired abnormalities of uterus, except cervix Also known as: Other specified acquired abnormalities of uterus, except cervix \| Polyp of corpus uteri \| intrauterine polyp \| polyp of body of uterus \| polyp of uterus [NB92.6] Injury of uterus Also known as: Injury of uterus \| uterine injury \| intrauterine injury NOS \| Injury of uterus without open wound into cavity \| Injury of uterus with open wound into cavity [GC04.1Y] Other specified fistulae involving female genital tract Also known as: Other specified fistulae involving female genital tract \| Other female intestinal-genital tract fistulae \| Intestinouterine fistula \| enterouterine fistula \| Cervicosigmoidal fistula [8A80.Z] Migraine, unspecified Also known as: Migraine, unspecified \| Migraine [QA76] Medication or substance that is known to be an allergen without injury or harm Definition: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm. Also known as: Medication or substance that is known to be an allergen without injury or harm Excludes: Medication or substance that is known to be an allergen, as mode of injury or harm [PL13.6] Medication or substance that is known to be an allergen, as mode of injury or harm Also known as: Medication or substance that is known to be an allergen, as mode of injury or harm \| medication error relating to known allergy to drug or substance \| adverse drug event relating to known allergy to drug or substance [9C40.A0] Papilloedema Definition: Optic disc swelling that results from increased intracranial pressure Also known as: Papilloedema \| Optic disc swelling that results from increased intracranial pressure Includes: Optic disc swelling that results from increased intracranial pressure [PA6Z] Unintentional fall from unspecified height Also known as: Unintentional fall from unspecified height \| fall NOS \| accidental fall \| fall causing injury that resulted in death \| fell NOS === GRAPH WALKS === --- Walk 1 --- [GA01.Z] Inflammatory disorders of the uterus, except cervix, unspecified --PARENT--> [GA01] Inflammatory disorders of the uterus, except cervix Def: A spectrum of inflammations involving the Uterus and the supporting tissues. It is usually caused by an ascending infection of organisms from the endocervix. Such inflammation can lead to functional i... --PARENT--> [?] Inflammatory disorders of the female genital tract --- Walk 2 --- [GA01.Z] Inflammatory disorders of the uterus, except cervix, unspecified --PARENT--> [GA01] Inflammatory disorders of the uterus, except cervix Def: A spectrum of inflammations involving the Uterus and the supporting tissues. It is usually caused by an ascending infection of organisms from the endocervix. Such inflammation can lead to functional i... --CHILD--> [GA01.0] Acute inflammatory disease of uterus --- Walk 3 --- [GA16.Y] Other specified acquired abnormalities of uterus, except cervix --PARENT--> [GA16] Acquired abnormalities of uterus, except cervix Def: Any condition of the uterus, caused by determinants arising after birth. These conditions are characterised by a malfunction, malformation, or another anomaly of the uterus (excluding the cervix).... --CHILD--> [GA16.1] Malposition of uterus Def: A condition of the uterus, caused by weakened pelvic ligaments, enlargement of the uterus, scarred pelvic tissue from pregnancy, tumour, menopause, endometriosis, inflammation, or salpingitis. This co... --- Walk 4 --- [GA16.Y] Other specified acquired abnormalities of uterus, except cervix --PARENT--> [GA16] Acquired abnormalities of uterus, except cervix Def: Any condition of the uterus, caused by determinants arising after birth. These conditions are characterised by a malfunction, malformation, or another anomaly of the uterus (excluding the cervix).... --PARENT--> [?] Noninflammatory disorders of female genital tract Def: Any disorder of the female genital tract, characterised by pathological changes, leading to noninflammatory effects.... --- Walk 5 --- [NB92.6] Injury of uterus --PARENT--> [NB92] Injury of urinary or pelvic organs --RELATED_TO--> [?] Female Genital Mutilation Def: A condition caused by procedures or other interventions for non-medical purposes. This condition is characterised by the partial or total removal of the external female genitalia or other injury to th... --- Walk 6 --- [NB92.6] Injury of uterus --PARENT--> [NB92] Injury of urinary or pelvic organs --RELATED_TO--> [?] Female Genital Mutilation Def: A condition caused by procedures or other interventions for non-medical purposes. This condition is characterised by the partial or total removal of the external female genitalia or other injury to th...	[ "[GA01.Z] Inflammatory disorders of the uterus, except cervix, unspecified\n --PARENT--> [GA01] Inflammatory disorders of the uterus, except cervix\n Def: A spectrum of inflammations involving the Uterus and the supporting tissues. It is usually caused by an ascending infection of organisms from the endocervix. Such inflammation can lead to functional i...\n --PARENT--> [?] Inflammatory disorders of the female genital tract", "[GA01.Z] Inflammatory disorders of the uterus, except cervix, unspecified\n --PARENT--> [GA01] Inflammatory disorders of the uterus, except cervix\n Def: A spectrum of inflammations involving the Uterus and the supporting tissues. It is usually caused by an ascending infection of organisms from the endocervix. Such inflammation can lead to functional i...\n --CHILD--> [GA01.0] Acute inflammatory disease of uterus", "[GA16.Y] Other specified acquired abnormalities of uterus, except cervix\n --PARENT--> [GA16] Acquired abnormalities of uterus, except cervix\n Def: Any condition of the uterus, caused by determinants arising after birth. These conditions are characterised by a malfunction, malformation, or another anomaly of the uterus (excluding the cervix)....\n --CHILD--> [GA16.1] Malposition of uterus\n Def: A condition of the uterus, caused by weakened pelvic ligaments, enlargement of the uterus, scarred pelvic tissue from pregnancy, tumour, menopause, endometriosis, inflammation, or salpingitis. This co...", "[GA16.Y] Other specified acquired abnormalities of uterus, except cervix\n --PARENT--> [GA16] Acquired abnormalities of uterus, except cervix\n Def: Any condition of the uterus, caused by determinants arising after birth. These conditions are characterised by a malfunction, malformation, or another anomaly of the uterus (excluding the cervix)....\n --PARENT--> [?] Noninflammatory disorders of female genital tract\n Def: Any disorder of the female genital tract, characterised by pathological changes, leading to noninflammatory effects....", "[NB92.6] Injury of uterus\n --PARENT--> [NB92] Injury of urinary or pelvic organs\n --RELATED_TO--> [?] Female Genital Mutilation\n Def: A condition caused by procedures or other interventions for non-medical purposes. This condition is characterised by the partial or total removal of the external female genitalia or other injury to th...", "[NB92.6] Injury of uterus\n --PARENT--> [NB92] Injury of urinary or pelvic organs\n --RELATED_TO--> [?] Female Genital Mutilation\n Def: A condition caused by procedures or other interventions for non-medical purposes. This condition is characterised by the partial or total removal of the external female genitalia or other injury to th..." ]	GA1Z&XA99N3	Noninflammatory disorders of uterus, except cervix	[ { "from_icd11": "GA01.Z", "icd10_code": "N719", "icd10_title": "Inflammatory disease of uterus, unspecified" }, { "from_icd11": "GA01.Z", "icd10_code": "N71", "icd10_title": "Inflammatory disease of uterus, except cervix" }, { "from_icd11": "NB92.6", "icd10_code": "S3763XA", "icd10_title": "Laceration of uterus, initial encounter" }, { "from_icd11": "NB92.6", "icd10_code": "S3769XA", "icd10_title": "Other injury of uterus, initial encounter" }, { "from_icd11": "NB92.6", "icd10_code": "S3760XA", "icd10_title": "Unspecified injury of uterus, initial encounter" }, { "from_icd11": "NB92.6", "icd10_code": "S376", "icd10_title": "Injury of uterus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43B0", "icd10_title": "Ophthalmoplegic migraine, not intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43409", "icd10_title": "Hemiplegic migraine, not intractable, without status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43A0", "icd10_title": "Cyclical vomiting, in migraine, not intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43D0", "icd10_title": "Abdominal migraine, not intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43709", "icd10_title": "Chronic migraine without aura, not intractable, without status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43A1", "icd10_title": "Cyclical vomiting, in migraine, intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43509", "icd10_title": "Persistent migraine aura without cerebral infarction, not intractable, without status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43719", "icd10_title": "Chronic migraine without aura, intractable, without status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43501", "icd10_title": "Persistent migraine aura without cerebral infarction, not intractable, with status migrainosus" } ]	N719	Inflammatory disease of uterus, unspecified
A 36 year-old Caucasian male (59 kg, BMI 23.8 kg.m -2 ) presented for urgent coronary artery bypass grafting 4 weeks after admission to the coronary care unit with a non-ST elevation myocardial infarction. He had episodic chest pain for 1 year increasing in frequency over six weeks prior to admission. On admission to hospital he was commenced on medication including acetylsalicylic acid, Clopidogrel, Elantan LA, Bisoprolol, Ramipril, Ezetimibe and Enoxaparin and he remained as an inpatient until his scheduled surgical procedure without requiring heparin, GTN infusion or inotropes. Urgent coronary angiography revealed a critical lesion of the left main stem artery, 70% stenosis of the proximal circumflex and 70% stenosis of the origin of the posterior descending artery and moderate impairment of left ventricular function with inferoposterior hypokinesis. This impairment in left ventricular was confirmed by transthoracic echocardiography. On arrival in the anaesthetic room his initial blood pressure was recorded as 90/50 mmHg and this had no apparent effect on pre-operative organ function considering all blood tests were within normal range and urine output was >1 ml.kg -1 .hr -1 . General anaesthesia was induced and was further maintained with propofol target-controlled infusion (1.5μg.ml -1 ), remifentanil (0.34μg.kg -1 .min -1 ) and isoflurane/oxygen/air mix at FiO 2 of 0.4 and end-tidal isoflurane 0.4-0.6%. Peri-operative transoesophageal echocardiography was used as part of monitoring in this case and epi-aortic ultrasound scanning was not utilised. As the left internal mammary artery was being taken down a dose of 250 mg (4.3 mg/kg) of heparin was given with a subsequent ACT of 559s. During CPB mean arterial pressure was maintained between 50-60 mmHg using boluses of phenylephrine and subsequently an infusion of noradrenaline. The lowest recorded mean arterial pressure was 45 mmHg immediately post institution of CPB. The infusion of noradrenaline ran at doses from 0.05-0.075μg.kg -1 .min -1 throughout the procedure. Other drugs that were used include glyceryl trinitrate at 0.5 mg.hr -1 as well as an infusion of the synthetic anti-fibrinolytic tranexamic acid initially at 1000 mg.hr -1 reduced to 600 mg.hr -1 (0.2 mg.kg -1 .min -1 ) to reduce perioperative blood loss. He received an infusion of milrinone at a dose 2μg.kg -1 .min -1 . Mixed venous saturation ranged from 55-70% in the peri-operative period. Pre-operatively the Hb concentration was 11.5 g.dl -1 . Intra-operatively the range of Hb was 7.5-10.5 g.dl -1 with the nadir on admission to CSICU. He was cooled and reached a nadir of 30°C. He was successfully weaned from CPB after placement of an intra-aortic balloon pump (IABP) and continuation of vasopressor and inotropic support. On admission to the Cardiac Surgical intensive care unit (CSICU) his mean blood pressure was 60 mmHg with a mean PA pressure of 14 mmHg. The balloon pump counter-pulsation remained on a 1:1 ratio with noradrenaline 0.05μg.kg -1 .min -1 , dopamine 5μg.kg -1 .min -1 , adrenaline 0.075μg.kg -1 .min -1 , and milrinone 1.5μg.kg -1 .min -1 to maintain a mean pressure of 75 mmHg, CI> 2.2 and adequate urine output >0.5 ml.kg -1 .hr -1 . The Hb concentration immediately on admission to CSICU post-operatively was 6.4 g.dl -1 , he was transfused with 3 units of packed cells and thereafter it remained between 8-10 g.dl -1 . On arrival in CSICU it was noted that his pupils were fixed and dilated at 5 mm. His intraocular pressure (IOP) was measured and found to be 13 mmHg. The measure of intraocular pressure served to rule out glaucoma and was a surrogate for measurement of intracranial pressure (ICP). During a sedation hold he was able to respond to command appropriately. The intra-aortic balloon pump was weaned quickly post-operatively and removed just over 12 hours after admission to CSICU. On the second day post-operatively whilst being turned he suffered a brief asystolic arrest requiring approximately 1 minute of CPR before return of spontaneous circulation, with similar pre-arrest haemodynamics. There was no warning of this from haemodynamic monitoring and on review of the charts the acute nature of the incident would appear to fit with a failure of pacing capture. However, subsequent urgent transesophageal echocardiography showed non-compressive clot and fluid around the right atrium, with CVP of 10-12 and mean PAP 16 mmHg. He returned to theatre for a re-exploration of his pericardium at which time a small amount of clot was removed and no revision of pacing wires was required. Over the next 36 hours he was weaned from all inotropic and vasopressor medications. He was extubated on day 3 post-operatively, had normal vision at this stage and was able to see staff within CSICU despite still having fixed and dilated pupils. He was re-intubated on day 5 after a failed trial of non-invasive therapy for bibasal collapse and worsening oxygenation. 7 days post-operatively, after being extubated for the second time, the patient complained of poor vision. An urgent ophthalmology review was organised and their findings were as follows: visual acuity showed no perception of light bilaterally, both pupils fixed and dilated, unreactive to light or accommodation, no consensual response, normal intraocular pressure, good red reflex, bilateral disc pallor, indistinct disc margins, retinal pallor at posterior pole, cattle-trucking of retinal arterial circulation. He also had bilateral internuclear ophthalmoplegia with up-gaze and down-gaze weakness. The impression was one of bilateral anterior ischaemic optic neuropathy (AION) and impending bilateral central retinal artery occlusion (CRAO) with brain stem involvement. A CT brain showed marked calcification of the falx, tentorium and choroid plexus with a further abnormality in the corpus callosum representing an unusual area of focal ischaemia (most likely in keeping with recent cardiac surgery). Rescue therapy with acetazolamide and topical timolol to reduce IOP and improve intraocular perfusion pressure, despite normal IOP, was attempted, but there was no improvement in vision. During follow up by neurology and ophthalmology services within the hospital he underwent a MRI of his brain with Magnetic Resonance Angiography of his cerebral vessels. No white matter abnormality was identified within cerebral hemispheres and the brainstem, cerebellum and pituitary fossa were unremarkable. He was also referred to a clinical psychologist and the Royal National Institute for the Blind (RNIB) for support and rehabilitation.	3.990234	0.966309	sec[0]/p[0]	en	0.999996	22104114	https://doi.org/10.1186/1749-8090-6-154	[ "pressure", "mmhg", "operatively", "infusion", "mean", "csicu", "artery", "this", "urgent", "blood" ]	[ { "code": "EH90.Z", "title": "Pressure ulcer of unspecified grade" }, { "code": "MB23.L", "title": "Pressured speech" }, { "code": "MD30.Z", "title": "Chest pain, unspecified" }, { "code": "CB22.Y", "title": "Other specified diseases of mediastinum, not elsewhere classified" }, { "code": "BA2Z", "title": "Hypotension, unspecified" }, { "code": "PL12.5", "title": "Operator error, as mode of injury or harm" }, { "code": "PL11.5", "title": "Procedure undertaken at wrong site or wrong side, as mode of injury or harm" }, { "code": "NE81.0Z", "title": "Haemorrhage or haematoma of other or unspecified site complicating a procedure, not elsewhere classified" }, { "code": "NE81.1", "title": "Disruption of operation wound, not elsewhere classified" }, { "code": "NE81.3", "title": "Postsurgical leak" } ]	=== ICD-11 CODES FOUND === [EH90.Z] Pressure ulcer of unspecified grade Also known as: Pressure ulcer of unspecified grade \| Pressure ulceration \| pressure injury \| pressure ulcer \| decubitus ulcer [MB23.L] Pressured speech Definition: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person talks without any social stimulation and may continue to talk even though no one is listening. Also known as: Pressured speech Excludes: Schizophrenia or other primary psychotic disorders \| Bipolar or related disorders [MD30.Z] Chest pain, unspecified Also known as: Chest pain, unspecified \| Pain in throat or chest \| chest pain NOS \| pain in chest \| chest pressure [CB22.Y] Other specified diseases of mediastinum, not elsewhere classified Also known as: Other specified diseases of mediastinum, not elsewhere classified \| Hernia of mediastinum \| mediastinal hernia \| mediastinal herniation \| Infectious mediastinitis [BA2Z] Hypotension, unspecified Also known as: Hypotension, unspecified \| hypopiesis \| low blood pressure \| arterial hypotension NOS \| decreased blood pressure [PL12.5] Operator error, as mode of injury or harm Definition: Harm arising due to process or procedural issues associated with the use and/or maintenance of a device not related to device failure. Also known as: Operator error, as mode of injury or harm \| operator error due to poor training as mode of injury \| operator error due to poor maintenance as mode of injury \| operator error due to incorrect device installation as mode of injury \| mistake by operator Excludes: Combination or interaction of operator error and device failure, as mode of injury or harm \| Operator error without injury or harm [PL11.5] Procedure undertaken at wrong site or wrong side, as mode of injury or harm Also known as: Procedure undertaken at wrong site or wrong side, as mode of injury or harm \| operation performed on incorrect site \| surgery undergone on incorrect body part \| wrong surgery \| Performance of inappropriate operation Excludes: Patient received diagnostic test or treatment intended for another patient [NE81.0Z] Haemorrhage or haematoma of other or unspecified site complicating a procedure, not elsewhere classified Also known as: Haemorrhage or haematoma of other or unspecified site complicating a procedure, not elsewhere classified \| Haemorrhage or haematoma complicating a procedure, not elsewhere classified \| postoperative haemorrhage \| postoperative bleeding \| Haemorrhage at any site resulting from a procedure [NE81.1] Disruption of operation wound, not elsewhere classified Also known as: Disruption of operation wound, not elsewhere classified \| burst stitches or sutures \| Dehiscence of operation wound \| dehiscence of operation wound nos \| dehiscence of surgical wound Includes: Dehiscence of operation wound \| Rupture of operation wound Excludes: Postsurgical anastomosis leak [NE81.3] Postsurgical leak Also known as: Postsurgical leak \| postoperative leak \| Postsurgical air leak \| postoperative air leak \| Postsurgical bile leak Excludes: Malfunction or complication of external stoma of digestive organs \| Tracheostomy malfunction === GRAPH WALKS === --- Walk 1 --- [EH90.Z] Pressure ulcer of unspecified grade --PARENT--> [EH90] Pressure ulceration Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th... --EXCLUDES--> [?] Erosion or ectropion of cervix uteri Def: A condition of the cervix uteri, caused by an increase in the total estrogen level in the body. This condition is characterised by protrusion and transformation of the endocervical columnar epithelium... --- Walk 2 --- [EH90.Z] Pressure ulcer of unspecified grade --PARENT--> [EH90] Pressure ulceration Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th... --CHILD--> [EH90.0] Pressure ulceration grade 1 Def: Pressure ulceration grade I is a precursor to skin ulceration. The skin remains intact but there is non-blanchable redness of a localised area, usually over a bony prominence. The area may be painful,... --- Walk 3 --- [MB23.L] Pressured speech Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t... --EXCLUDES--> [?] Bipolar or related disorders Def: Bipolar and related disorders are episodic mood disorders defined by the occurrence of Manic, Mixed or Hypomanic episodes or symptoms. These episodes typically alternate over the course of these disor... --CHILD--> [?] Cyclothymic disorder Def: Cyclothymic disorder is characterised by a persistent instability of mood over a period of at least 2 years, involving numerous periods of hypomanic (e.g., euphoria, irritability, or expansiveness, ps... --- Walk 4 --- [MB23.L] Pressured speech Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t... --EXCLUDES--> [?] Schizophrenia or other primary psychotic disorders Def: Schizophrenia and other primary psychotic disorders are characterised by significant impairments in reality testing and alterations in behaviour manifest in positive symptoms such as persistent delusi... --CHILD--> [?] Schizotypal disorder Def: Schizotypal disorder is characterised by an enduring pattern (i.e. characteristic of the person’s functioning over a period of at least several years) of eccentricities in behaviour, appearance and sp... --- Walk 5 --- [MD30.Z] Chest pain, unspecified --PARENT--> [MD30] Pain in throat or chest Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx.... --RELATED_TO--> [?] Precordial pain --- Walk 6 --- [MD30.Z] Chest pain, unspecified --PARENT--> [MD30] Pain in throat or chest Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx.... --CHILD--> [MD30.1] Other chest pain	[ "[EH90.Z] Pressure ulcer of unspecified grade\n --PARENT--> [EH90] Pressure ulceration\n Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th...\n --EXCLUDES--> [?] Erosion or ectropion of cervix uteri\n Def: A condition of the cervix uteri, caused by an increase in the total estrogen level in the body. This condition is characterised by protrusion and transformation of the endocervical columnar epithelium...", "[EH90.Z] Pressure ulcer of unspecified grade\n --PARENT--> [EH90] Pressure ulceration\n Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th...\n --CHILD--> [EH90.0] Pressure ulceration grade 1\n Def: Pressure ulceration grade I is a precursor to skin ulceration. The skin remains intact but there is non-blanchable redness of a localised area, usually over a bony prominence. The area may be painful,...", "[MB23.L] Pressured speech\n Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t...\n --EXCLUDES--> [?] Bipolar or related disorders\n Def: Bipolar and related disorders are episodic mood disorders defined by the occurrence of Manic, Mixed or Hypomanic episodes or symptoms. These episodes typically alternate over the course of these disor...\n --CHILD--> [?] Cyclothymic disorder\n Def: Cyclothymic disorder is characterised by a persistent instability of mood over a period of at least 2 years, involving numerous periods of hypomanic (e.g., euphoria, irritability, or expansiveness, ps...", "[MB23.L] Pressured speech\n Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t...\n --EXCLUDES--> [?] Schizophrenia or other primary psychotic disorders\n Def: Schizophrenia and other primary psychotic disorders are characterised by significant impairments in reality testing and alterations in behaviour manifest in positive symptoms such as persistent delusi...\n --CHILD--> [?] Schizotypal disorder\n Def: Schizotypal disorder is characterised by an enduring pattern (i.e. characteristic of the person’s functioning over a period of at least several years) of eccentricities in behaviour, appearance and sp...", "[MD30.Z] Chest pain, unspecified\n --PARENT--> [MD30] Pain in throat or chest\n Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx....\n --RELATED_TO--> [?] Precordial pain", "[MD30.Z] Chest pain, unspecified\n --PARENT--> [MD30] Pain in throat or chest\n Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx....\n --CHILD--> [MD30.1] Other chest pain" ]	EH90.Z	Pressure ulcer of unspecified grade	[ { "from_icd11": "EH90.Z", "icd10_code": "L89623", "icd10_title": "Pressure ulcer of left heel, stage 3" }, { "from_icd11": "EH90.Z", "icd10_code": "L89621", "icd10_title": "Pressure ulcer of left heel, stage 1" }, { "from_icd11": "EH90.Z", "icd10_code": "L89899", "icd10_title": "Pressure ulcer of other site, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L89620", "icd10_title": "Pressure ulcer of left heel, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89622", "icd10_title": "Pressure ulcer of left heel, stage 2" }, { "from_icd11": "EH90.Z", "icd10_code": "L89892", "icd10_title": "Pressure ulcer of other site, stage 2" }, { "from_icd11": "EH90.Z", "icd10_code": "L89519", "icd10_title": "Pressure ulcer of right ankle, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L89891", "icd10_title": "Pressure ulcer of other site, stage 1" }, { "from_icd11": "EH90.Z", "icd10_code": "L89610", "icd10_title": "Pressure ulcer of right heel, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89893", "icd10_title": "Pressure ulcer of other site, stage 3" }, { "from_icd11": "EH90.Z", "icd10_code": "L89890", "icd10_title": "Pressure ulcer of other site, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89629", "icd10_title": "Pressure ulcer of left heel, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L89619", "icd10_title": "Pressure ulcer of right heel, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L8945", "icd10_title": "Pressure ulcer of contiguous site of back, buttock and hip, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89894", "icd10_title": "Pressure ulcer of other site, stage 4" } ]	L89623	Pressure ulcer of left heel, stage 3
A 60-year-old man noted cold-like symptoms. After a month, he was visited to a local hospital and was suspected with myocarditis because his blood examination showed high inflammatory reactions and his echocardiogram showed severe cardiac dysfunction. He was started on intravenous continuous infusions of dobutamine (5 μg/kg/min) and dopamine (5 μg/kg/min) and transferred to our hospital. Upon admission to the intensive care unit, his consciousness, heart rate, blood pressure, arterial oxygen saturation level (5 L/min of supplemental oxygen via facemask), and body temperature were clear, 105 bpm, 105/66 mmHg, 100%, and 38.2 °C, respectively. His chest radiograph and chest computed tomography showed pulmonary congestion and bilateral pleural effusion without cardiomegaly, and the electrocardiogram showed low voltage of R-wave and intraventricular conduction disturbance in all leads . His laboratory examination showed inflammatory reaction, elevated levels of cardiac enzyme, and serum brain natriuretic peptide but almost normal range of coagulation (Table 1 ). His transthoracic echocardiogram showed a significant decrease in the myocardial contractility of the both ventricles but did not show the enlargement of both ventricles and a thrombus formation in all ventricles. Right cardiac catheterization, under the continuous infusions of dopamine (5 μg/kg/min) and dobutamine (5 μg/kg/min), showed that cardiac output (CO), pulmonary arterial pressure (PAP), pulmonary arterial wedge pressure, and mixed venous oxygenation saturation (S v O 2 ) were 3.60 L/min, 37/18(26) mmHg, 26 mmHg, and 40%, respectively, which indicated cardiogenic shock and post-capillary pulmonary hypertension (Table 2 ). Coronary angiography showed no abnormal findings. Because he was diagnosed with myocarditis and refractory to inotropic agents, he was inserted with intra-aortic balloon pumping (IABP). Despite 6 h of IABP support, his hemodynamic status progressively deteriorated; hence, we decided to start the mechanical ventilation and mechanical circulatory support by a PCPS on him. We administered 1 mg of midazolam, 0.2 mg of fentanyl, and 50 mg of rocuronium, performed endotracheal intubation, and started the airway pressure release ventilation with fraction of inspired oxygen (F I O 2 ) of 0.6, the high continuous airway pressure (CPAP) of 20 cmH 2 O, duration of high CPAP of 9.5 s, low CPAP of 0 cmH 2 O, and duration of low CPAP of 0.5 s in order to avoid the lung collapse. In this setting, his minute ventilation volume was 2.4 L (approximately 400 mL of tidal volume and 6/min of respiratory rate). After the initiation of the mechanical ventilation, his arterial blood gas analysis from the right radial artery showed a pH of 7.499, arterial oxygen tension (PaO 2 ) of 153 mmHg, and arterial carbon dioxide (CO 2 ) tension (PaCO 2 ) of 34.8 mmHg. And we started continuous infusion of propofol (1–2.5 mg/kg/h) and fentanyl (40 μg/h) for sedation. Then, we administered heparin as an intravenous bolus, and his activated clotting time (ACT) was prolonged to 198 s. We placed a venous cannula into the right atrium via the common right femoral vein and an arterial cannula into the left femoral artery and started the PCPS. Fig. 1 The 12-lead electrocardiogram of the patient upon admission to the intensive care unit. Sinus rhythm, low-voltage R-wave, and intraventricular conduction disturbance in all leads were seen Table 1 The blood examination on admission and 1 and 10 days after the admission Admission 1 day after the admission (after PCPS placement) 10 days after the admission (after intensive anticoagulant therapy) White blood cell counts (/μL) 9200 9100 12,400 Hemoglobin (g/dL) 10.2 10.6 10.7 Platelet counts (×10 4 /μL) 38.2 29.3 27.6 Total bilirubin (mg/dL) 0.8 0.7 1.5 Aspartate transaminase (IU/L) 401 431 194 Alanine aminotransferase (IU/L) 403 367 236 Lactate dehydrogenase (IU/L) 1081 926 932 Blood urea nitrogen (mg/dL) 18 17 15 Creatinine (mg/dL) 1.18 1.01 0.94 C-reactive protein (mg/dL) 13.2 10.9 17.1 Creatine kinase (IU/L) 1194 776 871 Creatine kinase-MB (IU/L) 32 32 39 Troponin T (ng/dL) 5.19 – – Brain natriuretic peptide (pg/mL) 3035 2266 – Lactate (mmol/L) 1.20 1.12 0.97 BE (mmol/L) −0.2 −0.1 −1.3 International normalized ratio of prothrombin time 1.14 1.16 1.21 Activated partial thromboplastin time (s) 30.0 38.7 118.9 Activated clotting time (s) 140 198 273 Almost the normal ranges of coagulation test were found on admission. The marked prolonged activated partial thromboplastin time and activated clotting time were found after the intensive anticoagulation therapy for the intraventricular thrombus Table 2 Hemodynamic status of the patient on admission, before and after the initiation of PCPS, after the onset of complete atrioventricular block, and after ventricular pacing Admission Before initiation of PCPS Initiation of PCPS Onset of complete AVB After ventricular pacing Respiratory support Face mask, 5 L/min of oxygen Face mask, 6 L/min of oxygen APRV, F I O 2 = 0.6, P high = 20 cm H 2 O, T high = 9.5 s, P low = 0 cm H 2 O, T low = 0.5 s Same as on the left APRV, F I O 2 = 0.6, P high = 15 cm H 2 O, T high = 5.5 s, P low = 0 cm H 2 O, T low = 0.5 s pH 7.531 7.532 7.449 7.272 7.554 SaO 2 (%) 95.1 97.0 98.5 95.7 98.5 PaO 2 (mmHg) 75.4 97.0 153.0 100.8 158.0 PaCO 2 (mmHg) 25.5 28.1 34.8 62.5 27.3 EtCO 2 (mmHg) – – 21 41 17 BE (mmol/L) −0.2 1.7 −0.1 0.3 1.9 Lac (mmol/L) 1.2 1.3 1.12 1.03 1.28 ABP (mmHg) 110/55 112/42 97/41 70/40 136/37 PAP (mmHg) 37/18 (26) 28/11 19/10 60/20 18/13 PAWP (mmHg) 25 – 12 30 – RAP (mmHg) 18 18 12 18 8 CO (L/min) 3.60 3.80 2.3 <<1.0 1.3 Output of PCPS (L/min) – – 3.0 3.0 3.5 The sum of PCPS blood flow and his own cardiac output (L/min) 3.60 3.80 5.3 3.0 4.8 Total gas flow of oxygenator (L/min) – – 1.0 1.0 4.0 SvO 2 (%) 40 39.7 66 55 69 The cardiogenic shock was improved by the PCPS support. After the onset of complete AV block, significant decrease of CO, pulmonary congestion, and increased PaCO 2 were observed PCPS percutaneous cardiopulmonary support, AVB atrioventricular block, APRV airway pressure release ventilation, F I O 2 fraction of inspired oxygen, P high high continuous positive airway pressure, T high duration of P high , P low low continuous positive airway pressure, T low duration of P low , EtCO 2 end-tidal carbon dioxide tension, PaCO 2 arterial carbon dioxide tension, ABP arterial blood pressure, PAP pulmonary artery pressure, PAWP pulmonary artery wedge pressure, RAP right atrial pressure, CO cardiac output, SvO 2 mixed venous oxygenation saturation	3.982422	0.966309	sec[1]/p[0]	en	0.999997	29492436	https://doi.org/10.1186/s40981-016-0067-0	[ "pressure", "mmhg", "pcps", "arterial", "blood", "oxygen", "pulmonary", "cardiac", "continuous", "time" ]	[ { "code": "EH90.Z", "title": "Pressure ulcer of unspecified grade" }, { "code": "MB23.L", "title": "Pressured speech" }, { "code": "MD30.Z", "title": "Chest pain, unspecified" }, { "code": "CB22.Y", "title": "Other specified diseases of mediastinum, not elsewhere classified" }, { "code": "BA2Z", "title": "Hypotension, unspecified" }, { "code": "6C4D.3", "title": "Dissociative drug intoxication including Ketamine or PCP" }, { "code": "6C4D.Z", "title": "Disorders due to use of dissociative drugs including ketamine and phencyclidine [PCP], unspecified" }, { "code": "6C4D.2Z", "title": "Dissociative drug dependence including ketamine or PCP, unspecified" }, { "code": "6C4D.1Z", "title": "Harmful pattern of use of dissociative drugs, including ketamine or PCP, unspecified" }, { "code": "6C4D.0", "title": "Episode of harmful use of dissociative drugs including ketamine or PCP" } ]	=== ICD-11 CODES FOUND === [EH90.Z] Pressure ulcer of unspecified grade Also known as: Pressure ulcer of unspecified grade \| Pressure ulceration \| pressure injury \| pressure ulcer \| decubitus ulcer [MB23.L] Pressured speech Definition: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person talks without any social stimulation and may continue to talk even though no one is listening. Also known as: Pressured speech Excludes: Schizophrenia or other primary psychotic disorders \| Bipolar or related disorders [MD30.Z] Chest pain, unspecified Also known as: Chest pain, unspecified \| Pain in throat or chest \| chest pain NOS \| pain in chest \| chest pressure [CB22.Y] Other specified diseases of mediastinum, not elsewhere classified Also known as: Other specified diseases of mediastinum, not elsewhere classified \| Hernia of mediastinum \| mediastinal hernia \| mediastinal herniation \| Infectious mediastinitis [BA2Z] Hypotension, unspecified Also known as: Hypotension, unspecified \| hypopiesis \| low blood pressure \| arterial hypotension NOS \| decreased blood pressure [6C4D.3] Dissociative drug intoxication including Ketamine or PCP Definition: Dissociative drug intoxication including Ketamine and PCP is a clinically significant transient condition that develops during or shortly after the consumption of a dissociative drug that is characterised by disturbances in consciousness, cognition, perception, affect, behaviour, or coordination. These disturbances are caused by the known pharmacological effects of a dissociative drug and their intensity is closely related to the amount of the dissociative drug consumed. They are time-limited an Also known as: Dissociative drug intoxication including Ketamine or PCP \| PCP intoxication \| phencyclidine intoxication [6C4D.Z] Disorders due to use of dissociative drugs including ketamine and phencyclidine [PCP], unspecified Also known as: Disorders due to use of dissociative drugs including ketamine and phencyclidine [PCP], unspecified \| Disorders due to use of dissociative drugs including ketamine and phencyclidine [PCP] \| Disorders due to abuse of dissociative drugs including ketamine and phencyclidine [PCP] \| PCP abuse [6C4D.2Z] Dissociative drug dependence including ketamine or PCP, unspecified Also known as: Dissociative drug dependence including ketamine or PCP, unspecified \| Dissociative drug dependence including ketamine or PCP \| Dissociative drug addiction including ketamine or PCP [6C4D.1Z] Harmful pattern of use of dissociative drugs, including ketamine or PCP, unspecified Also known as: Harmful pattern of use of dissociative drugs, including ketamine or PCP, unspecified \| Harmful pattern of use of dissociative drugs, including ketamine or PCP \| dissociative drugs, including ketamine or PCP abuse [6C4D.0] Episode of harmful use of dissociative drugs including ketamine or PCP Definition: An episode of use of a dissociative drug, including Ketamine and PCP, that has caused damage to a person’s physical or mental health or has resulted in behaviour leading to harm to the health of others. Harm to health of the individual occurs due to one or more of the following: (1) behaviour related to intoxication; (2) direct or secondary toxic effects on body organs and systems; or (3) a harmful route of administration. Harm to health of others includes any form of physical harm, including tr Also known as: Episode of harmful use of dissociative drugs including ketamine or PCP Excludes: Dissociative drug dependence including ketamine or PCP \| Harmful pattern of use of dissociative drugs, including ketamine or PCP === GRAPH WALKS === --- Walk 1 --- [EH90.Z] Pressure ulcer of unspecified grade --PARENT--> [EH90] Pressure ulceration Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th... --PARENT--> [?] Skin disorders provoked by external factors Def: A large group of skin disorders due to exposure of the skin to various external physical, chemical or environmental insults including chemical irritants and allergens, poisons, pressure, cold, heat, s... --- Walk 2 --- [EH90.Z] Pressure ulcer of unspecified grade --PARENT--> [EH90] Pressure ulceration Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th... --CHILD--> [EH90.2] Pressure ulceration grade 3 Def: Pressure ulcer with full thickness skin loss. Subcutaneous fat may be visible but bone, tendon or muscle are not exposed. Slough may be present but does not obscure the depth of tissue loss. There may... --- Walk 3 --- [MB23.L] Pressured speech Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t... --EXCLUDES--> [?] Bipolar or related disorders Def: Bipolar and related disorders are episodic mood disorders defined by the occurrence of Manic, Mixed or Hypomanic episodes or symptoms. These episodes typically alternate over the course of these disor... --CHILD--> [?] Bipolar type II disorder Def: Bipolar type II disorder is an episodic mood disorder defined by the occurrence of one or more hypomanic episodes and at least one depressive episode. A hypomanic episode is a persistent mood state la... --- Walk 4 --- [MB23.L] Pressured speech Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t... --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour --RELATED_TO--> [?] Speech dysfluency Def: Speech dysfluency is characterised by the frequent or pervasive disruption of the rhythmic flow of speech that arises subsequent to the developmental period (i.e., adult onset) and is outside the limi... --- Walk 5 --- [MD30.Z] Chest pain, unspecified --PARENT--> [MD30] Pain in throat or chest Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx.... --RELATED_TO--> [?] Pain in throat Def: Pain in throat means having pain sensation in throat. Throat is a tube that carries food to oesophagus and air to windpipe and larynx.... --- Walk 6 --- [MD30.Z] Chest pain, unspecified --PARENT--> [MD30] Pain in throat or chest Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx.... --EXCLUDES--> [?] Mastodynia Def: The symptom of breast pain. This symptom may be classified as cyclic or non-cyclical depending on the clinical patterns....	[ "[EH90.Z] Pressure ulcer of unspecified grade\n --PARENT--> [EH90] Pressure ulceration\n Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th...\n --PARENT--> [?] Skin disorders provoked by external factors\n Def: A large group of skin disorders due to exposure of the skin to various external physical, chemical or environmental insults including chemical irritants and allergens, poisons, pressure, cold, heat, s...", "[EH90.Z] Pressure ulcer of unspecified grade\n --PARENT--> [EH90] Pressure ulceration\n Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th...\n --CHILD--> [EH90.2] Pressure ulceration grade 3\n Def: Pressure ulcer with full thickness skin loss. Subcutaneous fat may be visible but bone, tendon or muscle are not exposed. Slough may be present but does not obscure the depth of tissue loss. There may...", "[MB23.L] Pressured speech\n Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t...\n --EXCLUDES--> [?] Bipolar or related disorders\n Def: Bipolar and related disorders are episodic mood disorders defined by the occurrence of Manic, Mixed or Hypomanic episodes or symptoms. These episodes typically alternate over the course of these disor...\n --CHILD--> [?] Bipolar type II disorder\n Def: Bipolar type II disorder is an episodic mood disorder defined by the occurrence of one or more hypomanic episodes and at least one depressive episode. A hypomanic episode is a persistent mood state la...", "[MB23.L] Pressured speech\n Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t...\n --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour\n --RELATED_TO--> [?] Speech dysfluency\n Def: Speech dysfluency is characterised by the frequent or pervasive disruption of the rhythmic flow of speech that arises subsequent to the developmental period (i.e., adult onset) and is outside the limi...", "[MD30.Z] Chest pain, unspecified\n --PARENT--> [MD30] Pain in throat or chest\n Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx....\n --RELATED_TO--> [?] Pain in throat\n Def: Pain in throat means having pain sensation in throat. Throat is a tube that carries food to oesophagus and air to windpipe and larynx....", "[MD30.Z] Chest pain, unspecified\n --PARENT--> [MD30] Pain in throat or chest\n Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx....\n --EXCLUDES--> [?] Mastodynia\n Def: The symptom of breast pain. This symptom may be classified as cyclic or non-cyclical depending on the clinical patterns...." ]	EH90.Z	Pressure ulcer of unspecified grade	[ { "from_icd11": "EH90.Z", "icd10_code": "L89623", "icd10_title": "Pressure ulcer of left heel, stage 3" }, { "from_icd11": "EH90.Z", "icd10_code": "L89621", "icd10_title": "Pressure ulcer of left heel, stage 1" }, { "from_icd11": "EH90.Z", "icd10_code": "L89899", "icd10_title": "Pressure ulcer of other site, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L89620", "icd10_title": "Pressure ulcer of left heel, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89622", "icd10_title": "Pressure ulcer of left heel, stage 2" }, { "from_icd11": "EH90.Z", "icd10_code": "L89892", "icd10_title": "Pressure ulcer of other site, stage 2" }, { "from_icd11": "EH90.Z", "icd10_code": "L89519", "icd10_title": "Pressure ulcer of right ankle, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L89891", "icd10_title": "Pressure ulcer of other site, stage 1" }, { "from_icd11": "EH90.Z", "icd10_code": "L89610", "icd10_title": "Pressure ulcer of right heel, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89893", "icd10_title": "Pressure ulcer of other site, stage 3" }, { "from_icd11": "EH90.Z", "icd10_code": "L89890", "icd10_title": "Pressure ulcer of other site, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89629", "icd10_title": "Pressure ulcer of left heel, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L89619", "icd10_title": "Pressure ulcer of right heel, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L8945", "icd10_title": "Pressure ulcer of contiguous site of back, buttock and hip, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89894", "icd10_title": "Pressure ulcer of other site, stage 4" } ]	L89623	Pressure ulcer of left heel, stage 3
The patient, female, 58 years old, was admitted to the dermatology department of our hospital on August 1, 2024 due to “generalized rash accompanied by itching for 3 days and frequent urination and urgency for 2 days”. Three days before admission, the patient developed generalized erythema and wheals without obvious inducement, accompanied by itching, fever, chills, abdominal discomfort. The highest recorded body temperature was 39.3 °C. There was a little nausea but no vomiting or chest tightness. She was tested negative for COVID-19 nucleic acid at Nanjing Drum Tower Hospital. Blood routine and CRP tests were normal. She was treated with ReDuNing Injection (a traditional Chinese medicine preparation) and Loratadine Tablets. The body temperature returned to normal but the rash did not subside. Two days later, the patient developed frequent urination, urgency and pain without hematuria. She was treated with “Dexamethasone Sodium Phosphate Injection” and “Levofloxacin”. The rash still did not improve. She had a 20-year history of “hypertension” and usually took “Amlodipine Besylate Tablets” to control blood pressure normally. In 2014 and 2016, the patient underwent coronary CTA examination on two occasions, which revealed no significant obstruction or stenosis in the coronary arteries. She denied a history of drug or food allergy and family genetic disease. She had a history of contact with COVID-19. Physical examination: Multiple erythema and wheals on the face and body, fused into patches, without blisters or erosion. Physical examination of heart and lungs was normal. Blood routine, CRP and serum amyloid A were abnormal ( Table 1 ); immunoglobulin E was 532 IU/ml; urine routine white blood cell count was 24.42/ul; liver and kidney function and myocardial injury markers were normal; mycoplasma pneumoniae antibody and allergens were negative. The electrocardiogram on admission showed (1) sinus rhythm, (2) T wave changes, and (3) V2R/S greater than 1 ; echocardiography showed a left ventricular ejection fraction (LVEF) of 63%; mild tricuspid regurgitation and decreased left ventricular diastolic dysfunction. After admission, she was given Methylprednisolone Sodium Succinate for Injection (40 mg, twice a day), Loratadine (10 mg, once a day), and Ebastine Tablets (10 mg, once a day) for anti-allergy, Omeprazole for Injection (40 mg, twice a day) for protecting gastric mucosa, Levofloxacin and Sodium Chloride Injection (0.5 g, once a day) for anti-infection, and Amlodipine Besylate Tablets (5 mg, once a day) for lowering blood pressure and other symptomatic and supportive treatments. The generalized rash and itching were alleviated. Five days later, the dose of Methylprednisolone Sodium Succinate for Injection was reduced (20 mg, twice a day). Six days later, the patient developed cough and chest tightness. The body temperature was normal. Seven days later, the nucleic acid test for six respiratory pathogens was all negative. The nucleic acid test for novel coronavirus was positive again. The patient was treated with simnotrelvir 0.75 g + ritonavir 0. 1 g (twice a day for 5 days). Nine days later, the patient felt compressive pain in the precordial area. The NRS pain score was 7 points, accompanied by profuse sweating that did not relieve continuously. Electrocardiogram indicated (1) sinus bradycardia; (2) acute extensive anterior wall myocardial infarction; (3) ST-T changes ; Myocardial injury marker troponin I was 0.072 ng/ml. The patient previously underwent two coronary CT angiographies, both of which showed no significant stenosis. This episode of chest pain occurred at rest during nighttime, leading us to suspect acute myocardial infarction associated with coronary artery spasm. Considering the patient's history of allergic reactions and the potential risk of contrast agents used in coronary angiography exacerbating allergic responses, coronary angiography was not performed. Instead, the patient was treated as follows: Aspirin enteric-coated tablets (100 mg, once daily) and clopidogrel bisulfate tablets (75 mg, once daily) were administered for dual antiplatelet therapy. Heparin (0.4 ml, twice daily) was used for anticoagulation. To relieve coronary artery spasm, diltiazem hydrochloride tablets (30 mg four times daily) and nicorandil tablets (5 mg three times daily) were prescribed. Additionally, intravenous methylprednisolone sodium succinate (10 mg once daily) was administered to mitigate potential allergic reactions, and isosorbide dinitrate injection (20 mg once daily) was given to further alleviate coronary spasm and improve myocardial perfusion. Following this medical treatment, the chest pain symptoms were relieved, and the electrocardiogram returnedto normal. However, atrial premature beats were observed . Ten days later, reexamination of echocardiography showed no definite segmental wall motion abnormality in resting state, and LVEF was 60%. Considering the patient's allergic history, the risks associated with coronary angiography, and the symptomatic relief achieved with medication, a conservative drug treatment approach was continued. Methylprednisolone sodium succinate was gradually reduced and changed to prednisone acetate tablets (20 mg, once daily) orally to inhibit inflammatory reaction. Two weeks later, the patient had compressive pain in the chest and back again. The NRS pain score was 4 points, accompanied by radiating pain to the left shoulder and jaw. After isosorbide nitrate and papaverine hydrochloride were pumped in, the symptoms were not significantly relieved. Reexamination of electrocardiogram showed dynamic evolution of T waves in precordial leads , and myocardial injury marker troponin I was 0.093 ng/ml. Considering that the patient's chest pain symptoms did not relieve continuously, coronary angiography was performed on August 14, which indicated mild coronary artery stenosis . Continue to give antiplatelet aggregation, lipid regulation, anti-coronary artery spasm and anti-allergic treatment. Eighteen days later, reexamination of novel coronavirus nucleic acid test was negative. The rash all over the body gradually disappeared, and the patient was discharged after improvement. After discharge, the dosage of prednisone acetate tablets was gradually reduced and eventually discontinued. The patient continued to take nicorandil, diltiazem hydrochloride tablets, aspirin enteric-coated tablets and atorvastatin calcium tablets orally. At the one-month follow-up, no recurrence of urticaria or angina symptoms was observed.	3.814453	0.984863	sec[1]/p[0]	en	0.999996	PMC12006130	https://doi.org/10.3389/fcvm.2025.1542223	[ "tablets", "coronary", "pain", "daily", "injection", "chest", "sodium", "myocardial", "rash", "body" ]	[ { "code": "NE60", "title": "Harmful effects of drugs, medicaments or biological substances, not elsewhere classified" }, { "code": "PL00", "title": "Drugs, medicaments or biological substances associated with injury or harm in therapeutic use" }, { "code": "PL13.1", "title": "Underdosing, as mode of injury or harm" }, { "code": "PL13.0", "title": "Overdose of substance, as mode of injury or harm" }, { "code": "BA8Z", "title": "Diseases of coronary artery, unspecified" }, { "code": "BA4Z", "title": "Acute ischaemic heart disease, unspecified" }, { "code": "BA41.Z", "title": "Acute myocardial infarction, unspecified" }, { "code": "BA5Z", "title": "Chronic ischaemic heart disease, unspecified" }, { "code": "LA8C.2", "title": "Congenital coronary arterial fistula" }, { "code": "MG3Z", "title": "Pain, unspecified" } ]	=== ICD-11 CODES FOUND === [NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified Also known as: Harmful effects of drugs, medicaments or biological substances, not elsewhere classified \| drugs, medicaments or biological substances, toxicity not elsewhere classified \| adverse drug effects \| drug reaction NOS \| drug allergy NOS Excludes: Alcohol intoxication \| pathological drug intoxication \| hypersensitivity reaction to correctly administered drug [PL00] Drugs, medicaments or biological substances associated with injury or harm in therapeutic use Also known as: Drugs, medicaments or biological substances associated with injury or harm in therapeutic use \| Drugs medicaments or biological substances associated with injury or harm in therapeutic use, Systemic antibiotics \| Drugs medicaments or biological substances associated with injury or harm in therapeutic use, Systemic antibiotics, Penicillins \| Drugs medicaments or biological substances associated with injury or harm in therapeutic use, Systemic antibiotics, Cephalosporins or other beta-lactam antibiotics \| Drugs medicaments or biological substances associated with injury or harm in therapeutic use, Systemic antibiotics, Chloramphenicol group Excludes: Circumstances associated with exposure to a drug, medicament or biological substance influencing the episode of care without injury or harm [PL13.1] Underdosing, as mode of injury or harm Also known as: Underdosing, as mode of injury or harm \| underdosing of substance leading to inadequate level or effect of medication or substance as mode of injury \| inadequate or insufficient dosage of substance, as mode of injury \| missed or omitted dose of substance as mode of injury \| dose of substance administered or taken too late or too slowly as mode of injury [PL13.0] Overdose of substance, as mode of injury or harm Definition: Incorrect dose - too high Also known as: Overdose of substance, as mode of injury or harm \| wrong dose of substance as mode of injury \| wrong strength of substance as mode of injury \| dose of substance administered or taken too early or too quickly as a mode of injury \| extra dose of substance administered as mode of injury Includes: overdose of prescribed drug \| medication error leading to excess level or effect of prescribed drug Excludes: Overdose of substance without injury or harm \| Unintentional exposure to or harmful effects of drugs, medicaments or biological substances \| Intentional self-harm by exposure to or harmful effects of drugs, medicaments or biological substances [BA8Z] Diseases of coronary artery, unspecified Also known as: Diseases of coronary artery, unspecified \| coronary artery insufficiency \| coronary artery heart disease \| CAD - [coronary artery disease] \| coronary artery disorder [BA4Z] Acute ischaemic heart disease, unspecified Also known as: Acute ischaemic heart disease, unspecified \| acute coronary syndrome \| ACS - [acute coronary syndrome] \| Silent myocardial ischaemia \| asymptomatic ischemia [BA41.Z] Acute myocardial infarction, unspecified Also known as: Acute myocardial infarction, unspecified \| Acute myocardial infarction \| cardiac attack \| heart attack \| acute cardiac infarction [BA5Z] Chronic ischaemic heart disease, unspecified Also known as: Chronic ischaemic heart disease, unspecified \| Ischaemic heart disease (chronic) NOS \| coronary ischaemia \| coronary damage NOS \| atheroma of heart [LA8C.2] Congenital coronary arterial fistula Definition: A congenital cardiovascular malformation in which a coronary artery communicates, through an anomalous channel, with a cardiac chamber or with any segment of the systemic or pulmonary circulation. Additional information: this communication may be simple and direct or may be tortuous and dilated. In order of frequency the involved coronary artery is the right, the left and, rarely, both coronary arteries. Occasionally multiple fistulas are present. Also known as: Congenital coronary arterial fistula \| coronary fistula \| congenital arteriovenous coronary fistula \| congenital coronary fistula to pulmonary artery \| Congenital coronary arterial fistula to right ventricle Includes: congenital coronary fistula to pulmonary artery Excludes: anomalous origin of coronary artery from pulmonary arterial tree [MG3Z] Pain, unspecified Also known as: Pain, unspecified \| pain observations \| pain NOS \| generalised pain \| generalised pain, NOS === GRAPH WALKS === --- Walk 1 --- [NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified --PARENT--> [?] Harmful effects of substances --PARENT--> [22] Injury, poisoning or certain other consequences of external causes Def: !markdown In the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre... --- Walk 2 --- [NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified --EXCLUDES--> [?] Alcohol intoxication Def: Alcohol intoxication is a clinically significant transient condition that develops during or shortly after the consumption of alcohol that is characterised by disturbances in consciousness, cognition,... --PARENT--> [?] Disorders due to use of alcohol Def: Disorders due to use of alcohol are characterised by the pattern and consequences of alcohol use. Alcohol—more specifically termed ethyl alcohol or ethanol—is an intoxicating compound produced by ferm... --- Walk 3 --- [PL00] Drugs, medicaments or biological substances associated with injury or harm in therapeutic use --EXCLUDES--> [?] Circumstances associated with exposure to a drug, medicament or biological substance influencing the episode of care without injury or harm --EXCLUDES--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance --- Walk 4 --- [PL00] Drugs, medicaments or biological substances associated with injury or harm in therapeutic use --EXCLUDES--> [?] Circumstances associated with exposure to a drug, medicament or biological substance influencing the episode of care without injury or harm --CHILD--> [?] Overdose of substance without injury or harm Def: Overdose of a substance occurs when a patient is given more of a prescribed drug or other substance than is intended. Can be the result of inaccurate measurement of drug, including oral administration... --- Walk 5 --- [PL13.1] Underdosing, as mode of injury or harm --PARENT--> [PL13] Mode of injury or harm associated with exposure to a drug, medicament or biological substance --CHILD--> [PL13.2] Drug-related injury or harm in the context of correct administration or dosage, as mode of injury or harm --- Walk 6 --- [PL13.1] Underdosing, as mode of injury or harm --PARENT--> [PL13] Mode of injury or harm associated with exposure to a drug, medicament or biological substance --CHILD--> [PL13.1] Underdosing, as mode of injury or harm	[ "[NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified\n --PARENT--> [?] Harmful effects of substances\n --PARENT--> [22] Injury, poisoning or certain other consequences of external causes\n Def: !markdown\nIn the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre...", "[NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified\n --EXCLUDES--> [?] Alcohol intoxication\n Def: Alcohol intoxication is a clinically significant transient condition that develops during or shortly after the consumption of alcohol that is characterised by disturbances in consciousness, cognition,...\n --PARENT--> [?] Disorders due to use of alcohol\n Def: Disorders due to use of alcohol are characterised by the pattern and consequences of alcohol use. Alcohol—more specifically termed ethyl alcohol or ethanol—is an intoxicating compound produced by ferm...", "[PL00] Drugs, medicaments or biological substances associated with injury or harm in therapeutic use\n --EXCLUDES--> [?] Circumstances associated with exposure to a drug, medicament or biological substance influencing the episode of care without injury or harm\n --EXCLUDES--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance", "[PL00] Drugs, medicaments or biological substances associated with injury or harm in therapeutic use\n --EXCLUDES--> [?] Circumstances associated with exposure to a drug, medicament or biological substance influencing the episode of care without injury or harm\n --CHILD--> [?] Overdose of substance without injury or harm\n Def: Overdose of a substance occurs when a patient is given more of a prescribed drug or other substance than is intended. Can be the result of inaccurate measurement of drug, including oral administration...", "[PL13.1] Underdosing, as mode of injury or harm\n --PARENT--> [PL13] Mode of injury or harm associated with exposure to a drug, medicament or biological substance\n --CHILD--> [PL13.2] Drug-related injury or harm in the context of correct administration or dosage, as mode of injury or harm", "[PL13.1] Underdosing, as mode of injury or harm\n --PARENT--> [PL13] Mode of injury or harm associated with exposure to a drug, medicament or biological substance\n --CHILD--> [PL13.1] Underdosing, as mode of injury or harm" ]	NE60	Harmful effects of drugs, medicaments or biological substances, not elsewhere classified	[ { "from_icd11": "NE60", "icd10_code": "T50A95A", "icd10_title": "Adverse effect of other bacterial vaccines, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50Z15A", "icd10_title": "Adverse effect of immunoglobulin, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50Z95A", "icd10_title": "Adverse effect of other vaccines and biological substances, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50A95S", "icd10_title": "Adverse effect of other bacterial vaccines, sequela" }, { "from_icd11": "NE60", "icd10_code": "T50B95A", "icd10_title": "Adverse effect of other viral vaccines, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50A25A", "icd10_title": "Adverse effect of mixed bacterial vaccines without a pertussis component, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50A91A", "icd10_title": "Poisoning by other bacterial vaccines, accidental (unintentional), initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T498X5A", "icd10_title": "Adverse effect of other topical agents, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T48905A", "icd10_title": "Adverse effect of unspecified agents primarily acting on the respiratory system, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T48995A", "icd10_title": "Adverse effect of other agents primarily acting on the respiratory system, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50A15A", "icd10_title": "Adverse effect of pertussis vaccine, including combinations with a pertussis component, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50B15A", "icd10_title": "Adverse effect of smallpox vaccines, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T416X3A", "icd10_title": "" }, { "from_icd11": "NE60", "icd10_code": "T419X3A", "icd10_title": "" }, { "from_icd11": "NE60", "icd10_code": "T418X2A", "icd10_title": "" } ]	T50A95A	Adverse effect of other bacterial vaccines, initial encounter
The procedure combines an endoscopic transnasal approach with a transorbital approach via a subciliary incision. The surgeon using the transnasal approach manipulates the maxillary sinus via a prelacrimal approach . The fractured bone fragments are elevated into the maxillary sinus by detaching them from the orbital contents as a local bone–mucosal flap with a posterior pedicle, without detaching them from the maxillary mucosa . Next, the infraorbital nerve is identified . The surgeon proceeds posteriorly along its superior border to identify the inferior orbital fissure . The periosteum is incised to identify the inferior margin of the greater wing of the sphenoid bone . The surgeon then proceeds medially to identify the superior posterior wall of the maxillary sinus . After the fractured bone fragments have been elevated as a bone–mucosal flap and the three landmarks (infraorbital nerve, line of the inferior margin of the greater wing of the sphenoid bone, and superior posterior wall of the maxillary sinus) have been identified, the herniated orbital contents are restored completely with a silicone silastic sheet (Eyeball restraint insert; Koken, Tokyo, Japan) . Next a poly- l -lactic acid/hydroxyapatite (PLLA/HA) sheet (Super Fixorb MX® 0.3 mm sheet; Teijin Medical Technologies, Osaka, Japan), which is a resolvable rigid plate, is molded and implanted transorbitally to cover the blowout site . Next, the silastic sheet is removed after implantation of the PLLA/HA sheet. The sheet is placed so that its deepest part is on the superior posterior wall of the maxillary sinus and its posterior edge is in contact with the line of the inferior margin of the greater wing of the sphenoid bone. The sheet extends to the inferior margin of the greater wing of the sphenoid bone posterior to the posterior wall of the maxillary sinus, making it possible to completely cover the fracture site, even when the slope of the posterior end of the orbital floor is fractured. Finally, the orbit and maxillary sinus are separated completely by covering the fracture site with a local bone–mucosal flap . Preoperative computed tomography (CT) showed that the fracture site extended to the slope of the posterior end of the orbital floor . Postoperative CT showed that the sheet was placed more posterior to the posterior wall of the maxillary sinus and was in contact with the inferior margin of the greater wing of the sphenoid bone . Furthermore, the plate was not exposed to the maxillary sinus, because the orbit was separated from the maxillary sinus by a local bone–mucosal flap . Fig. 2 Stepwise endoscopic images of the procedure: the process for identifying the first landmark. Images A – E are views from the 0 degree telescope with the transnasal approach. Image F is a view from the 0 degree telescope with the transorbital approach. The left nasal cavity was shown ( A ). Anterior ethmoidectomy and maxillectomy were performed ( B ). A prelacrimal approach was performed to manipulate the maxillary sinus ( C , D ). The fractured bone fragments were elevated as a local bone–mucosal flap ( E ). The infraorbital nerve was identified ( F ). BL basal lamella of the middle turbinate, BMF bone–mucosal flap, F orbital floor, IOF inferior orbital fissure, ION infraorbital nerve, IT inferior turbinate, MM medial wall of the maxillary sinus, MS maxillary sinus, MT middle turbinate, NLD nasolacrimal duct, OC orbital contents, S nasal septum Fig. 3 Stepwise endoscopic images of the procedure: identification of the first landmark to identification of the third landmark. Image A is a view from the 0 degree telescope with the transorbital approach. Images B and C are views from the 70 degree telescope with the transnasal approach. Images D – F are views from the 0 degree telescope with the transnasal approach. The zygomatic nerve is lateral to the infraorbital nerve ( A ). The infraorbital nerve was identified ( B ), and proceeding posteriorly along the infraorbital nerve, the cranial side of the pterygopalatine fossa was identified at the inferior orbital fissure ( C ). The periosteum was incised in the layer just above the infraorbital nerve. The inferior margin of the greater wing of the sphenoid bone was identified ( E ). Proceeding medially ( E ), the superior posterior wall of the maxillary sinus was found medial to the inferomedial margin of the greater wing of the sphenoid bone across the inferior orbital fissure ( F ). BMF bone–mucosal flap, F orbital floor, GW greater wing of the sphenoid bone, IOF inferior orbital fissure, ION infraorbital nerve, SP superior posterior wall of the maxillary sinus, ZN zygomatic nerve Fig. 4 Stepwise endoscopic images of the procedure: reconstructing the orbital floor. Images A – F are views from the 0 degree telescope with the transnasal approach. The herniated tissue was placed in the orbit with a silicone silastic sheet ( A ). A rigid plate was implanted. However, there was a space between the inferior margin of the greater wing of the sphenoid bone and the plate and between the superior posterior wall of the maxillary sinus and the plate ( B ). The plate was reshaped to eliminate the spaces between the plate and the inferior margin of the greater wing of the sphenoid bone and the superior posterior wall of the maxillary sinus, respectively ( C ). The orbit and maxillary sinus were separated with a local bone–mucosal flap ( D , E ). The left nasal cavity at the end of the procedure was shown ( F ). BMF bone–mucosal flap, GW greater wing of the sphenoid bone, IOF inferior orbital fissure, ION infraorbital nerve, IT inferior turbinate, MT middle turbinate, RP rigid plate, S nasal septum, SP superior posterior wall of the maxillary sinus, SS silicone silastic sheet Fig. 5 Preoperative computed tomography (CT) images. A1–5 are reformatted coronal CT images in anterior to posterior order. B1–5 are reformatted sagittal CT images in medial to lateral order. Images show an orbital floor fracture that extends to the slope of the posterior end of the orbital floor Fig. 6 Postoperative computed tomography (CT) images. A1–5 are reformatted coronal CT images in anterior to posterior order. B1–5 are reformatted sagittal CT images in medial to lateral order. Images B3 and B4 show the implant inserted posterior to the inferior orbital fissure and in contact with the inferior margin of the greater wing of the sphenoid bone. The orbit and the maxillary sinus were completely separated by a bone–mucosal flap; the implant was not exposed to the maxillary sinus	4.167969	0.52002	sec[1]/p[0]	en	0.999996	35951106	https://doi.org/10.1007/s00405-022-07587-1	[ "bone", "maxillary", "sinus", "orbital", "nerve", "greater", "wing", "sphenoid", "approach", "wall" ]	[ { "code": "FC0Z", "title": "Diseases of the musculoskeletal system or connective tissue, unspecified" }, { "code": "FB84.Z", "title": "Osteomyelitis or osteitis, unspecified" }, { "code": "FB80.Z", "title": "Disorder of bone density or structure, unspecified" }, { "code": "FB86.11", "title": "Hypertrophy of bone" }, { "code": "FB86.1Z", "title": "Bone hyperplasias, unspecified" }, { "code": "DA06.Z", "title": "Diseases of jaws, unspecified" }, { "code": "CA0A.Y&XA1R64", "title": "Maxillary fistula" }, { "code": "DA0E.0Y&XA7VK5", "title": "Maxillary hypoplasia" }, { "code": "CA0J.Y&XA1R64", "title": "Maxillary sinus polyp" }, { "code": "NA0Z&XA7VK5", "title": "Injury of maxilla" } ]	=== ICD-11 CODES FOUND === [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified Also known as: Diseases of the musculoskeletal system or connective tissue, unspecified \| bone disease NOS \| bone disorder NOS \| bone lesion NOS \| musculoskeletal complications NOS [FB84.Z] Osteomyelitis or osteitis, unspecified Also known as: Osteomyelitis or osteitis, unspecified \| Osteomyelitis or osteitis \| bone inflammation \| bone ulcer \| bone inflammatory disease [FB80.Z] Disorder of bone density or structure, unspecified Also known as: Disorder of bone density or structure, unspecified \| Certain specified disorders of bone density or structure [FB86.11] Hypertrophy of bone Also known as: Hypertrophy of bone \| bone thickening \| periosteum thickening \| Infantile cortical hyperostoses \| Post traumatic subperiosteal ossification [FB86.1Z] Bone hyperplasias, unspecified Also known as: Bone hyperplasias, unspecified \| Bone hyperplasias [DA06.Z] Diseases of jaws, unspecified Also known as: Diseases of jaws, unspecified \| Diseases of jaws \| disease of jaw \| diseases of the jaws \| disorder of jaw === GRAPH WALKS === --- Walk 1 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --EXCLUDES--> [?] Endocrine, nutritional or metabolic diseases Def: This chapter includes endocrine diseases, nutritional diseases as well as metabolic diseases.... --- Walk 2 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --RELATED_TO--> [?] Monogenic autoinflammatory syndromes Def: Monogenic hereditary autoinflammatory diseases characterised by apparently unprovoked generalised inflammation in the absence of infection or high titre autoantibodies.... --- Walk 3 --- [FB84.Z] Osteomyelitis or osteitis, unspecified --PARENT--> [FB84] Osteomyelitis or osteitis --CHILD--> [FB84.0] Acute haematogenous osteomyelitis --- Walk 4 --- [FB84.Z] Osteomyelitis or osteitis, unspecified --PARENT--> [FB84] Osteomyelitis or osteitis --EXCLUDES--> [?] Inflammatory conditions of jaws --- Walk 5 --- [FB80.Z] Disorder of bone density or structure, unspecified --PARENT--> [FB80] Certain specified disorders of bone density or structure --EXCLUDES--> [?] Osteopetrosis Def: Osteopetrosis ('marble bone disease') is a descriptive term that refers to a group of rare, heritable disorders of the skeleton characterised by increased bone density on radiographs. Osteopetrotic co... --- Walk 6 --- [FB80.Z] Disorder of bone density or structure, unspecified --PARENT--> [FB80] Certain specified disorders of bone density or structure --CHILD--> [FB80.1] Skeletal fluorosis	[ "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --EXCLUDES--> [?] Endocrine, nutritional or metabolic diseases\n Def: This chapter includes endocrine diseases, nutritional diseases as well as metabolic diseases....", "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --RELATED_TO--> [?] Monogenic autoinflammatory syndromes\n Def: Monogenic hereditary autoinflammatory diseases characterised by apparently unprovoked generalised inflammation in the absence of infection or high titre autoantibodies....", "[FB84.Z] Osteomyelitis or osteitis, unspecified\n --PARENT--> [FB84] Osteomyelitis or osteitis\n --CHILD--> [FB84.0] Acute haematogenous osteomyelitis", "[FB84.Z] Osteomyelitis or osteitis, unspecified\n --PARENT--> [FB84] Osteomyelitis or osteitis\n --EXCLUDES--> [?] Inflammatory conditions of jaws", "[FB80.Z] Disorder of bone density or structure, unspecified\n --PARENT--> [FB80] Certain specified disorders of bone density or structure\n --EXCLUDES--> [?] Osteopetrosis\n Def: Osteopetrosis ('marble bone disease') is a descriptive term that refers to a group of rare, heritable disorders of the skeleton characterised by increased bone density on radiographs. Osteopetrotic co...", "[FB80.Z] Disorder of bone density or structure, unspecified\n --PARENT--> [FB80] Certain specified disorders of bone density or structure\n --CHILD--> [FB80.1] Skeletal fluorosis" ]	FC0Z	Diseases of the musculoskeletal system or connective tissue, unspecified	[ { "from_icd11": "FC0Z", "icd10_code": "XIII", "icd10_title": "" }, { "from_icd11": "FB84.Z", "icd10_code": "M86672", "icd10_title": "Other chronic osteomyelitis, left ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86172", "icd10_title": "Other acute osteomyelitis, left ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86171", "icd10_title": "Other acute osteomyelitis, right ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86671", "icd10_title": "Other chronic osteomyelitis, right ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X7", "icd10_title": "Other osteomyelitis, ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X8", "icd10_title": "Other osteomyelitis, other site" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X6", "icd10_title": "Other osteomyelitis, lower leg" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X9", "icd10_title": "Other osteomyelitis, unspecified sites" }, { "from_icd11": "FB84.Z", "icd10_code": "M8668", "icd10_title": "Other chronic osteomyelitis, other site" }, { "from_icd11": "FB84.Z", "icd10_code": "M86662", "icd10_title": "Other chronic osteomyelitis, left tibia and fibula" }, { "from_icd11": "FB84.Z", "icd10_code": "M86151", "icd10_title": "Other acute osteomyelitis, right femur" }, { "from_icd11": "FB84.Z", "icd10_code": "M86141", "icd10_title": "Other acute osteomyelitis, right hand" }, { "from_icd11": "FB84.Z", "icd10_code": "M86641", "icd10_title": "Other chronic osteomyelitis, right hand" }, { "from_icd11": "FB84.Z", "icd10_code": "M8669", "icd10_title": "Other chronic osteomyelitis, multiple sites" } ]	XIII
A 58-year-old male patient, with a history of a vascular bypass because of occlusion of the infrarenal aorta in September 2010 and rheumatoid arthritis for which he used etanercept weekly, underwent abdominal ultrasound during routine follow-up in September 2012. He mentioned lower abdominal pain since a few weeks, but did not report any night sweats, fever, weight loss, rash or other complaints. He did not have any contact with animals, but lived in the Netherlands in an area where Q fever had been highly epidemic between 2007 and 2010 . Laboratory results on presentation are shown in Table 1 . On abdominal ultrasound, bilateral hydronephrosis with extensive retroperitoneal masses was observed. A subsequent computed tomography scan and positron emission tomography (PET) scan showed extensive retroperitoneal, intra-abdominal, mediastinal, cervical, and axillary lymphadenopathy, a pulmonary mass and a lesion in the right adrenal. In addition, the abdominal aorta and left iliac artery showed increased 18 F-FDG uptake, which was not further analyzed. The PET-scan was made as part of the standard work-up procedure of suspected NHL, in accordance with Dutch guidelines . Biopsy of the retroperitoneal masses and a bone marrow biopsy revealed an Ann Arbor stage IV B cell non-Hodgkin lymphoma (NHL), with initially indefinite histopathological classification of the subtype of NHL. Re-examination of the pathology specimens at an academic hospital confirmed the presence of a B-cell NHL, with a marginal zone lymphoma as the most likely histopathological subtype. Chemo-immunotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) was initiated and etanercept was discontinued. After discontinuation of etanercept, the patient experienced two flares of his rheumatoid arthritis for which he received short courses of prednisone. Follow-up PET scan after three cycles of chemotherapy showed regression of all lymphoma locations, with the exception of pulmonary lesion, adrenal mass, and lower cervical lymph nodes. The increased 18 F-FDG uptake in aorta and left iliac artery was unchanged. Because of unresponsiveness to chemotherapy of the pulmonary lesion, a second malignancy, possibly metastatic lung carcinoma, was suspected and further analysis was planned. Meanwhile, chemotherapy was discontinued, while awaiting analysis of the suspected lung lesion. Shortly after the last PET scan, the patient was diagnosed with pulmonary embolism for which heparin was started. Because of the use of anticoagulants and difficult accessibility of the pulmonary lesion, the diagnosis could not be confirmed pathologically. A follow-up PET scan was performed in February 2013, on which all NHL localizations were persistently in regression. However, the 18 F-FDG uptake in the aorta and left iliac artery had strongly increased and the pulmonary lesion and pathological cervical lymph nodes were unchanged. The adrenal lesion was slightly larger. Patient was referred for second opinion and after due consideration did not want any further diagnostics and treatment of his pulmonary lesion. Analysis of the increased uptake in the aorta and left iliac artery was performed in June 2013. Repeated PET scanning revealed highly increased uptake of 18 F-FDG of the abdominal aorta and left iliac artery, and a lesion suspicious of a small abscess near the left iliac artery . As infection of the vascular bypass was suspected, blood cultures and serology for Coxiella burnetii were performed. Blood cultures were negative, but phase I and II IgG antibodies for C. burnetii were repeatedly positive with a maximum phase I IgG antibody titer of 1:4096 (Indirect Fluorescent-antibody Assay, Focus Diagnostics, Inc., Cypress, CA, USA). Phase I and II IgM antibodies against C. burnetii were negative. Polymerase chain reaction on serum was performed twice, but both samples tested negative. Vascular infection with C. burnetii was diagnosed according to both the Dutch chronic Q fever consensus group criteria and the criteria formulated by Eldin et al., and treatment with doxycycline (200 mg once daily) and hydroxychloroquine (200 mg three times daily) was started in July 2013 . No signs of endocarditis were present on physical examination or PET-CT, but an echocardiogram was not performed. In the absence of an echocardiogram, the presence of concomitant endocarditis could not be excluded with certainty . After start of the treatment, the patient experienced severe gastro-intestinal side effects and refused further therapy in September 2013. In March 2014, he reported repeated melena and rectal bleeding. An aortoduodenal fistula was suspected, but the patient did not want any further diagnostic interventions. His clinical condition deteriorated and he died the same month. Table 1 Laboratory results at the day of presentation Laboratory measurement Result Normal range Units of measurement Hemoglobin 7.2 8.5–11.0 mmol/L Thrombocytes 179 150–400 10 9 /L Leukocytes a 9.2 4.0–10.0 10 9 /L Creatinine a 551 60–110 µmol/L C-reactive protein 14 0–8 mg/L Aspartate aminotransferase a 19 0–34 U/L Alanine aminotransferase a 13 0–44 U/L Gamma-glutamyltransferase a <10 0–54 U/L Alkaline phosphatase a 81 43–115 U/L Lactate dehydrogenase a 246 0–247 U/L a In the days following initial presentation, these laboratory values changed. Maximum values during admission were: leukocyte count 35.6 × 10 9 /L, C-reactive protein 186 mg/L, aspartate aminotransferase 35, alanine aminotransferase 49, alkaline phosphatase 177, gamma-glutamyltransferase 146, lactate dehydrogenase 400 Fig. 1 Composite figure of PET-CT, immunofluorescence (IF) and fluorescence in situ hybridization (FISH). a Highly increased 18 F-FDG uptake in the aortic wall. b Highly increased 18 F-FDG uptake in the left iliac artery. c Microscopic image (original magnification ×100) of immunofluorescence staining (IF) of retroperitoneal lymphoma tissue of a patient with vascular chronic Q fever in which nuclei are stained blue (4′,6-diamidino-2-phenylindole, DAPI), while perinuclear Coxiella burnetii is stained red . d Microscopic image (original magnification ×100) of fluorescence in situ hybridization (FISH) of the same tissue in which nuclei are stained blue (4′,6-diamidino-2-phenylindole, DAPI), while C. burnetii , organized in perinuclear vacuoles, is stained yellow . Yellow signal results of the co-localization of the universal probe EUB ( red ) and specific 16S rRNA C. burnetii probe ( green ). For both c , d Leica DMI6000 B microscope was used	4.015625	0.977539	sec[0]/p[0]	en	0.999996	28840502	https://doi.org/10.1007/s15010-017-1061-9	[ "lesion", "which", "pulmonary", "iliac", "artery", "uptake", "burnetii", "aorta", "abdominal", "scan" ]	[ { "code": "FA5Z", "title": "Arthropathies, unspecified" }, { "code": "FC0Z", "title": "Diseases of the musculoskeletal system or connective tissue, unspecified" }, { "code": "ME60.Z", "title": "Skin lesion of unspecified nature" }, { "code": "MD41", "title": "Clinical findings on diagnostic imaging of lung" }, { "code": "GC2Z&XA6KU8", "title": "Disease of kidney, not elsewhere classified" }, { "code": "BD50.41", "title": "Abdominal aortic aneurysm with rupture" }, { "code": "EK91", "title": "Dermatoses which may presage cutaneous lymphoma" }, { "code": "MH12.1", "title": "Death occurring less than 24 hours from onset of symptoms, not otherwise explained" }, { "code": "8A44.3", "title": "Certain specified leukodystrophies" }, { "code": "CB40.Y", "title": "Other specified diseases of the respiratory system" } ]	=== ICD-11 CODES FOUND === [FA5Z] Arthropathies, unspecified Also known as: Arthropathies, unspecified \| Disorders affecting predominantly peripheral joints \| Disorders affecting predominantly peripheral limb joints \| arthropathy NOS \| arthropathic [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified Also known as: Diseases of the musculoskeletal system or connective tissue, unspecified \| bone disease NOS \| bone disorder NOS \| bone lesion NOS \| musculoskeletal complications NOS [ME60.Z] Skin lesion of unspecified nature Also known as: Skin lesion of unspecified nature \| Skin lesion of uncertain or unspecified nature \| Skin lesion without established diagnosis \| Pigmented skin lesion of unspecified nature \| Ulcer of skin of unspecified nature [MD41] Clinical findings on diagnostic imaging of lung Definition: Clinical findings on diagnostic imaging of lung is findings on diagnostic imaging of the lung which don't appear in normal status of the body. Diagnostic imaging refers to technologies that doctors use to look inside body for clues about a medical condition. X-rays, CT scans, nuclear medicine scans, MRI scans and ultrasound are all types of diagnostic imaging. Also known as: Clinical findings on diagnostic imaging of lung \| abnormal diagnostic imaging of lung \| Hyperinflation of lung \| Lung mass \| Pulmonary lobe mass [BD50.41] Abdominal aortic aneurysm with rupture Also known as: Abdominal aortic aneurysm with rupture \| abdominal aorta aneurysm rupture \| abdominal aorta aneurysm ruptured \| abdominal aortic aneurysm which has ruptured \| ruptured AAA [EK91] Dermatoses which may presage cutaneous lymphoma Definition: Dermatoses which may represent the earliest stages of cutaneous lymphoma but where it is not possible to confirm their neoplastic nature. Also known as: Dermatoses which may presage cutaneous lymphoma [MH12.1] Death occurring less than 24 hours from onset of symptoms, not otherwise explained Also known as: Death occurring less than 24 hours from onset of symptoms, not otherwise explained \| died without sign of disease \| Death known not to be violent or instantaneous for which no cause can be discovered \| death known not to be violent or instantaneous, cause unknown \| Death without sign of disease Includes: Death known not to be violent or instantaneous for which no cause can be discovered \| Death without sign of disease [8A44.3] Certain specified leukodystrophies Also known as: Certain specified leukodystrophies \| CACH syndrome \| Vanishing white matter disease \| Childhood ataxia with central nervous system hypomyelination \| Congenital or early infantile CACH syndrome [CB40.Y] Other specified diseases of the respiratory system Also known as: Other specified diseases of the respiratory system \| Secondary respiratory disorders \| Respiratory disorders in diseases classified elsewhere \| Diseases of bronchus, not elsewhere classified \| Acquired bronchial diverticulum === GRAPH WALKS === --- Walk 1 --- [FA5Z] Arthropathies, unspecified --PARENT--> [?] Arthropathies --CHILD--> [?] Osteoarthritis Def: Osteoarthritis (OA) can be defined as a group of distinct, but overlapping diseases, which may have different etiologies, but similar biological, morphological, and clinical outcomes affecting the art... --- Walk 2 --- [FA5Z] Arthropathies, unspecified --PARENT--> [?] Arthropathies --CHILD--> [?] Osteoarthritis Def: Osteoarthritis (OA) can be defined as a group of distinct, but overlapping diseases, which may have different etiologies, but similar biological, morphological, and clinical outcomes affecting the art... --- Walk 3 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes Def: !markdown In the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre... --- Walk 4 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --EXCLUDES--> [?] Endocrine, nutritional or metabolic diseases Def: This chapter includes endocrine diseases, nutritional diseases as well as metabolic diseases.... --- Walk 5 --- [ME60.Z] Skin lesion of unspecified nature --PARENT--> [ME60] Skin lesion of uncertain or unspecified nature Def: To be used where there is either significant uncertainty or alternatively no information as to the nature of a circumscribed skin lesion. This is of particular importance with regard to whether or not... --CHILD--> [ME60.1] Pigmented skin lesion of uncertain nature Def: This denotes the presence of a pigmented skin lesion but uncertainty as to its nature. No inference as to whether the lesion might be of serious significance (e.g. suspected skin cancer) is made.... --- Walk 6 --- [ME60.Z] Skin lesion of unspecified nature --PARENT--> [ME60] Skin lesion of uncertain or unspecified nature Def: To be used where there is either significant uncertainty or alternatively no information as to the nature of a circumscribed skin lesion. This is of particular importance with regard to whether or not... --PARENT--> [?] Symptoms or signs involving the skin Def: This category allows the capture of imprecise data where a more specific diagnosis cannot be made or to supplement information about a specific diagnosis....	[ "[FA5Z] Arthropathies, unspecified\n --PARENT--> [?] Arthropathies\n --CHILD--> [?] Osteoarthritis\n Def: Osteoarthritis (OA) can be defined as a group of distinct, but overlapping diseases, which may have different etiologies, but similar biological, morphological, and clinical outcomes affecting the art...", "[FA5Z] Arthropathies, unspecified\n --PARENT--> [?] Arthropathies\n --CHILD--> [?] Osteoarthritis\n Def: Osteoarthritis (OA) can be defined as a group of distinct, but overlapping diseases, which may have different etiologies, but similar biological, morphological, and clinical outcomes affecting the art...", "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes\n Def: !markdown\nIn the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre...", "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --EXCLUDES--> [?] Endocrine, nutritional or metabolic diseases\n Def: This chapter includes endocrine diseases, nutritional diseases as well as metabolic diseases....", "[ME60.Z] Skin lesion of unspecified nature\n --PARENT--> [ME60] Skin lesion of uncertain or unspecified nature\n Def: To be used where there is either significant uncertainty or alternatively no information as to the nature of a circumscribed skin lesion. This is of particular importance with regard to whether or not...\n --CHILD--> [ME60.1] Pigmented skin lesion of uncertain nature\n Def: This denotes the presence of a pigmented skin lesion but uncertainty as to its nature. No inference as to whether the lesion might be of serious significance (e.g. suspected skin cancer) is made....", "[ME60.Z] Skin lesion of unspecified nature\n --PARENT--> [ME60] Skin lesion of uncertain or unspecified nature\n Def: To be used where there is either significant uncertainty or alternatively no information as to the nature of a circumscribed skin lesion. This is of particular importance with regard to whether or not...\n --PARENT--> [?] Symptoms or signs involving the skin\n Def: This category allows the capture of imprecise data where a more specific diagnosis cannot be made or to supplement information about a specific diagnosis...." ]	FA5Z	Arthropathies, unspecified	[ { "from_icd11": "FA5Z", "icd10_code": "M00-M25", "icd10_title": "" }, { "from_icd11": "FC0Z", "icd10_code": "XIII", "icd10_title": "" }, { "from_icd11": "ME60.Z", "icd10_code": "L989", "icd10_title": "Disorder of the skin and subcutaneous tissue, unspecified" }, { "from_icd11": "MD41", "icd10_code": "R911", "icd10_title": "Solitary pulmonary nodule" }, { "from_icd11": "MD41", "icd10_code": "R91", "icd10_title": "Abnormal findings on diagnostic imaging of lung" }, { "from_icd11": "BD50.41", "icd10_code": "I713", "icd10_title": "Abdominal aortic aneurysm, ruptured" }, { "from_icd11": "MH12.1", "icd10_code": "R961", "icd10_title": "" } ]	M00-M25
The medical history of the patient reported an appendectomy at 16 years of age and a hysterectomy for uterine fibroid at 60 years of age. The patient was not taking drugs at the time of her ER visit. She was admitted to the Cardiology ward, where a coronarography examination result was negative for stenosis. She was discharged and subsequently had a gastroscopy , which showed mild gastropathy with signs of atrophy, and biopsies on the corpus and fundus seemed to indicate autoimmune atrophic gastritis. An abdominal ultrasound found a suspected pancreatic hypoechoic oval formation. Because the symptoms persisted, she was admitted to the Medicine ward and administered low osmolarity parental nutrition (amino acids, glucose, lipids, sodium, calcium, potassium, magnesium) of about 1,500 ml with 1,960 total kcal. To treat the pain, she began oxycodone hydrochloride 5 mg twice daily and transdermic fentanyl 12 microg/h every three days. The pain-killing drugs were soon increased to a continuous 24-hour intravenous infusion of 80 mg of morphine hydrochloride and 4 mg of dexamethasone administered intravenously twice daily. A chest and abdominal CT scan showed a 36×26 mm mass with a longitudinal diameter of 41 mm, which included the origin of the celiac tripod. The mass seemed to originate exophytically from the gastric wall with an apparent cleavage plane with the head of the pancreas . The patient was transferred to the Gastroenterology ward. An ultrasound endoscopy showed a hypoechoic formation with not well-defined margins measuring 40×30 mm incorporating the origin of the celiac tripod, distancing about 10 mm from the gastric wall. The endoscopic fine-needle aspiration reported a poorly differentiated carcinoma positive for CKAE1/AE3, CK7, CK8, CK 19, focally for CK20 and CDX2, whereas it was negative for CD20 and TTF1. The ultrasound-endoscopy described a lymphadenopathy of 12 mm near the bigger lesion. A PET/CT scan showed a hyperaccumulation of the known expansive formation at the celiac tripod (SUV 11.9) without certain cleavage planes from the stomach. There was also hyperaccumulation at the level of the antrum and duodenal gastric passage (SUV 6). A multidisciplinary team requested a second esophagus-gastro-enteroscopy and a nuclear magnetic cholangio resonance (cNMR). The second gastroscopy - performed at another hospital - showed a regular esophagus and no lesions protruding in the stomach. On the posterior wall of the subcardial area, there was a slightly raised subcentimetric area type 0-IIa covered by bleeding mucosa where the biopsy had been performed . The remaining mucosa was discolored with areas of atrophy. The immunohistochemistry with CK AE1-AE3 confirmed chronic gastritis of moderate degree in the quiescent phase. The report was negative for Helicobacter Pylori. The cNMR excluded hepatobiliary diseases and confirmed the lesion in correspondence to the celiac tripod with an elongated component to a lesser curvature . Given the importance of distinguishing the origin of the primary, a second upper gastrointestinal eco-endoscopy was performed with the pathologist on site while the colonoscopy showed diverticula. The upper eco-endoscopy confirmed a hypoechoic neoformation measuring 30×40 mm, which seemed to originate from the muscular layer of the gastric wall and incorporated the celiac tripod. The lesion appeared to maintain the cleavage plane with the body of the pancreas and the left hepatic lobe. The exam identified many pericentimetric lymphadenopathies in the perilesional area. The results of the pathology report indicated small aggregates of poorly differentiated tumor cells . The cells were positive for broad-spectrum cytokeratins (AE1/AE3), CK7, and p40 . The cells were intensely and diffusely positive for CK5/6 and p63 and negative for CK20, PAS, TTF-1, anti-smooth muscle actin, CD45 (LCA), ERG, and S100. The clinical picture suggested poorly differentiated carcinoma with squamous differentiation. Due to the patient feeling increased epigastric pain, an urgent abdominal CT scan was performed the day after the biopsy. The abdominal CT scan showed an approximately 2 cm wall thickness and an extension of approximately 5 cm at the stomach body along the smaller gastric curvature. This time, there was absence of the cleavage plane of the known celiac tripod lesion. In January 2023, she was admitted to the Oncology ward with a 3 ECOG performance status. The patient was referred to us with a weight of 50 kg, persistent epigastric pain, nausea, and vomiting if she tried to eat. The physical exam was negative. At the last biopsy, a HER 2 evaluation was requested, which was 1+, the PD-L1/CPS score was 45, and the mismatch repair (MLH1, MSH2, MSH6, PMS2) was regular. To complete the staging, she did dermatological, gynecological, and Ear-Nose-Throat (ENT) examinations that excluded primitivity cancers. The research for tumor cells in the urine was also negative. The patient had epigastric pain despite 24-hour continuous intravenous infusion with 80 mg of morphine hydrochloride and 4 mg of dexamethasone intravenous twice daily, so the palliativist added 5 mg of midazolam and pregabalin 75 mg one tablet daily. In one day, the patient asked for four and three intravenous rescue doses of morphine hydrochloride 15 mg and paracetamol 1000 mg respectively, too. Because of severe pain and faint Blumberg positive sign, an abdominal CT was requested to exclude any complications due to the previous biopsy. The last CT scan showed a disease of about 73×44 mm growing quickly on the celiac tripod infiltrating liver, the lesser curve of the stomach, and the body of the pancreas. The palliativist increased the intravenous 24-hour continuous infusion to 100 mg of morphine hydrochloride plus 5 mg of midazolam and pregabalin to 100 mg daily. At the end of January 2023, excluding dihydropyrimidine dehydrogenase deficiency, the patient started a chemotherapy treatment with 85 mg/mg 2 of oxaliplatin on day 1, 200 mg/m 2 of calcium levofolinate on day 1, 400 mg/m 2 of 5-fluorouracil bolus on day 1, 2, 400 mg/m 2 of 5-fluorouracil on day 1 for 48 h (mFOLFOX6). The Supplementary Figure S5 summarizes the diagnostic and therapeutic steps. About two weeks after the chemotherapy, when it was possible to repeat the cycle of chemotherapy, the health condition of the patient progressively worsened. The patient presented frequent episodes of mental confusion and delirium. She was subsequently transferred to hospice care where she died a few days later.	3.980469	0.976563	sec[1]/p[1]	en	0.999997	39634259	https://doi.org/10.3389/fonc.2024.1419923	[ "celiac", "tripod", "pain", "abdominal", "hydrochloride", "daily", "intravenous", "scan", "gastric", "wall" ]	[ { "code": "DA95", "title": "Coeliac disease" }, { "code": "NB90.20", "title": "Minor laceration of coeliac artery" }, { "code": "BD30.10&XA8577", "title": "Coeliac artery embolism" }, { "code": "BD30.11&XA8577", "title": "Coeliac artery thrombosis" }, { "code": "BD52.5", "title": "Coeliac artery compression syndrome" }, { "code": "MG3Z", "title": "Pain, unspecified" }, { "code": "8E43.Z", "title": "Pain disorders, unspecified" }, { "code": "MG31.Z", "title": "Acute pain, unspecified" }, { "code": "MG30.Z", "title": "Chronic pain, unspecified" }, { "code": "FB56.2", "title": "Myalgia" } ]	=== ICD-11 CODES FOUND === [DA95] Coeliac disease Definition: Coeliac disease is a permanent intolerance to gluten proteins, present in wheat, rye, and barley. It is an autoimmune disorder, characterised by a chronic inflammatory state of the small intestinal mucosa and submucosa, which can impair digestion and absorption of nutrients, leading to malnutrition. Also known as: Coeliac disease \| Gluten-sensitive enteropathy \| Nontropical sprue \| coeliac rickets \| Gee disease Includes: Gluten-sensitive enteropathy \| Nontropical sprue \| Idiopathic steatorrhoea [NB90.20] Minor laceration of coeliac artery Also known as: Minor laceration of coeliac artery \| incomplete transection of coeliac artery \| laceration of coeliac artery NOS \| superficial laceration of celiac artery Includes: incomplete transection of coeliac artery \| laceration of coeliac artery NOS \| superficial laceration of celiac artery [BD52.5] Coeliac artery compression syndrome Also known as: Coeliac artery compression syndrome \| celiac artery compression syndrome \| celiac axis compression syndrome \| celiac axis syndrome \| coeliac axis compression syndrome [MG3Z] Pain, unspecified Also known as: Pain, unspecified \| pain observations \| pain NOS \| generalised pain \| generalised pain, NOS [8E43.Z] Pain disorders, unspecified Also known as: Pain disorders, unspecified \| Pain disorders [MG31.Z] Acute pain, unspecified Also known as: Acute pain, unspecified \| Acute pain [MG30.Z] Chronic pain, unspecified Also known as: Chronic pain, unspecified \| Chronic pain [FB56.2] Myalgia Definition: This is a disorder characterised by pain in a muscle or group of muscles. Also known as: Myalgia \| muscle ache \| muscle soreness \| muscular pain \| myalgic Excludes: Chronic primary musculoskeletal pain \| Chronic secondary musculoskeletal pain === GRAPH WALKS === --- Walk 1 --- [DA95] Coeliac disease Def: Coeliac disease is a permanent intolerance to gluten proteins, present in wheat, rye, and barley. It is an autoimmune disorder, characterised by a chronic inflammatory state of the small intestinal mu... --PARENT--> [?] Diseases of small intestine Def: This is a group of conditions characterised as being in or associated with the small intestine.... --EXCLUDES--> [?] Diseases of duodenum Def: This is a group of conditions characterised as being in or associated with the duodenum, the first portion of the small intestine.... --- Walk 2 --- [DA95] Coeliac disease Def: Coeliac disease is a permanent intolerance to gluten proteins, present in wheat, rye, and barley. It is an autoimmune disorder, characterised by a chronic inflammatory state of the small intestinal mu... --PARENT--> [?] Diseases of small intestine Def: This is a group of conditions characterised as being in or associated with the small intestine.... --PARENT--> [13] Diseases of the digestive system --- Walk 3 --- [NB90.20] Minor laceration of coeliac artery --PARENT--> [NB90.2] Injury of coeliac artery --CHILD--> [NB90.20] Minor laceration of coeliac artery --- Walk 4 --- [NB90.20] Minor laceration of coeliac artery --PARENT--> [NB90.2] Injury of coeliac artery --CHILD--> [NB90.20] Minor laceration of coeliac artery --- Walk 5 --- [BD52.5] Coeliac artery compression syndrome --PARENT--> [BD52] Certain specified disorders of arteries or arterioles --EXCLUDES--> [?] Nonorgan specific systemic autoimmune disorders --- Walk 6 --- [BD52.5] Coeliac artery compression syndrome --PARENT--> [BD52] Certain specified disorders of arteries or arterioles --EXCLUDES--> [?] Leukocytoclastic vasculitis Def: Leukocytoclastic vasculitis (hypersensitivity vasculitis; hypersensitivity angiitis) is a histopathological term commonly used to denote a small-vessel vasculitis. It may be localised to the skin or m...	[ "[DA95] Coeliac disease\n Def: Coeliac disease is a permanent intolerance to gluten proteins, present in wheat, rye, and barley. It is an autoimmune disorder, characterised by a chronic inflammatory state of the small intestinal mu...\n --PARENT--> [?] Diseases of small intestine\n Def: This is a group of conditions characterised as being in or associated with the small intestine....\n --EXCLUDES--> [?] Diseases of duodenum\n Def: This is a group of conditions characterised as being in or associated with the duodenum, the first portion of the small intestine....", "[DA95] Coeliac disease\n Def: Coeliac disease is a permanent intolerance to gluten proteins, present in wheat, rye, and barley. It is an autoimmune disorder, characterised by a chronic inflammatory state of the small intestinal mu...\n --PARENT--> [?] Diseases of small intestine\n Def: This is a group of conditions characterised as being in or associated with the small intestine....\n --PARENT--> [13] Diseases of the digestive system", "[NB90.20] Minor laceration of coeliac artery\n --PARENT--> [NB90.2] Injury of coeliac artery\n --CHILD--> [NB90.20] Minor laceration of coeliac artery", "[NB90.20] Minor laceration of coeliac artery\n --PARENT--> [NB90.2] Injury of coeliac artery\n --CHILD--> [NB90.20] Minor laceration of coeliac artery", "[BD52.5] Coeliac artery compression syndrome\n --PARENT--> [BD52] Certain specified disorders of arteries or arterioles\n --EXCLUDES--> [?] Nonorgan specific systemic autoimmune disorders", "[BD52.5] Coeliac artery compression syndrome\n --PARENT--> [BD52] Certain specified disorders of arteries or arterioles\n --EXCLUDES--> [?] Leukocytoclastic vasculitis\n Def: Leukocytoclastic vasculitis (hypersensitivity vasculitis; hypersensitivity angiitis) is a histopathological term commonly used to denote a small-vessel vasculitis. It may be localised to the skin or m..." ]	DA95	Coeliac disease	[ { "from_icd11": "DA95", "icd10_code": "K900", "icd10_title": "Celiac disease" }, { "from_icd11": "BD52.5", "icd10_code": "I774", "icd10_title": "Celiac artery compression syndrome" }, { "from_icd11": "MG3Z", "icd10_code": "R52", "icd10_title": "Pain, unspecified" }, { "from_icd11": "MG3Z", "icd10_code": "R529", "icd10_title": "" }, { "from_icd11": "MG31.Z", "icd10_code": "R520", "icd10_title": "" }, { "from_icd11": "MG30.Z", "icd10_code": "R521", "icd10_title": "" }, { "from_icd11": "MG30.Z", "icd10_code": "R522", "icd10_title": "" }, { "from_icd11": "FB56.2", "icd10_code": "M7918", "icd10_title": "Myalgia, other site" }, { "from_icd11": "FB56.2", "icd10_code": "M7910", "icd10_title": "Myalgia, unspecified site" }, { "from_icd11": "FB56.2", "icd10_code": "M7912", "icd10_title": "Myalgia of auxiliary muscles, head and neck" }, { "from_icd11": "FB56.2", "icd10_code": "M791", "icd10_title": "Myalgia" } ]	K900	Celiac disease
In this case, the patient was diagnosed with systemic multiple metastases after lung adenocarcinoma surgery through imaging combined with pathological puncture biopsy . The patient received “pemetrexed” and “albumin paclitaxel” chemotherapy as the main second-line therapy. After combined radiotherapy, another biopsy was conducted. Approximatively 41 months later, SCLC transformation occurred . Thereafter, the patient was diagnosed and treated by reference to the specifications, guidelines, and consensus for the diagnosis and treatment of extensive-disease SCLC for approximatively 21 months. During the process, the patient developed AP in April 2022, which was timely detected with the support of a multidisciplinary team and controlled by scientific diagnosis and treatment strategies. This approach laid a good foundation for further improving the patient’s quality of life and prolonging survival. AP is a common disease in gastroenterology, with an incidence of approximatively 13–45/100,000, and that shows a continuously increasing trend. Approximatively 75% of AP cases are caused by cholelithiasis, hyperlipidemia, and alcohol abuse, while other rare causes include surgery, trauma, and drugs with approximatively 10% of idiopathic AP cases . Metastasis-induced AP (MIAP) is relatively rare and may be the primary or secondary clinical manifestation of a tumor, and therefore, it is often undetected leading to a poor prognosis. AP can be classified as mild AP (MAP), moderately severe AP (MSAP), and severe AP (SAP) according to the 2012 Atlanta criteria . The incidence rate of pancreatic metastasis in LC patients is low with approximatively 0.12%–7.50%. However, SCLC metastasis to the pancreas in SCLC autopsy reports, is not negligible as it accounts for approximatively 24%–40% . Studies have found that cases of LC-induced MIAP mostly have atypical symptoms, with mild or no symptoms. A small number of patients with pancreatic metastases manifest abdominal pain, AP, and obstructive jaundice. These patients often miss the timing of anti-lung cancer treatment due to the relative lack of a certain degree of clinical vigilance. The early detection of LC-induced MIAP is more conducive to improving LC prognosis. It has also been shown that patients with LC-induced MIAP are older and more vulnerable to anemia, main pancreatic duct dilatation, and abdominal LN enlargement than those with non-tumor-related AP . Xiong et al. reported that 7 out of 8 patients (87.5%) with LC-induced MIAP have a SCLC pathological type. Tanaka et al. retrospectively summarized 25 cases of LC-induced MIAP, including 19 (76.0%) cases of SCLC. Maeno et al. reported that the common pathological type of LC-induced pancreatic metastasis is SCLC, accounting for approximatively 50%, followed by adenocarcinoma (34.6%), squamous cell carcinoma (11.5%), and large cell lung cancer (3.9%). Besides, the proportion of MIAP in SCLC-induced pancreatic metastasis is high, which should be emphasized by clinicians. To date, there are few reports of LC-induced MIAP in both individual and multiple cases. In this case, the patient was diagnosed with the NSCLC transformation into SCLC, namely, SCLC accompanied with MIAP. The diagnosis was mainly confirmed by abdominal CT, MRI and ERCP examinations due to the patient’s abdominal pain, and the abnormal increases in blood amylase, blood lipase, and urinary amylase. Moreover, jaundice was gradually worsening, with a maximum level of total bilirubin of 149.2 μmol/L (normal range: 2.0–22.0 μmol/L). The patient’s clinical experience of MIAP is consistent with that of the results of related previous studies. It is believed that LC-induced MIAP may have multiple pathogeneses: 1) Mechanical compression of the pancreatic ducts by metastatic tumors or enlarged peripancreatic LNs; 2) Ischemia-induced pancreatic injury due to tumor invasion of blood vessels; and 3) Diffuse tumor infiltration to the pancreas and destruction of the pancreatic lobules. Studies have also observed that patients with LC-induced MIAP are mostly at an advanced stage at the time of detection, generally associated with poor conditions and high risks of surgical resection or puncture biopsy of pancreatic lesions or abdominal occupancies. Besides, such patients are often less willing to have a biopsy, and thus, the metastatic lesions of most these patients are clinically diagnosed by imaging findings (excluding AP induced by other reasons) . It was reported that the treatment strategy for LC-induced MIAP mainly favors symptomatic and supporting treatment at the initial stage, and that MAP patients should start chemotherapy and/or radiotherapy as early as possible, to facilitate AP recovery. SAP patients may not be able to tolerate chemotherapy due to their poor physical conditions . In this case, the LC patient developed MAP during treatment, which was improved with pharmacological chemotherapy combined with local radiotherapy and without AP recurrence. The patient has a good survival status with an ECOG score of 2 points at the follow-up to date, resulting in a prolonged median survival, compared to the extensive-disease SCLC reported in the National Comprehensive Cancer Network (NCCN) clinical practice guidelines. Of course, after postoperative, the recurrence of the left lung adenocarcinoma that subsequently transformed into SCLC complicated with LC-induced AP, was controlled. Statistics showed that a total of 5 lines of therapy were conducted after SCLC transformation, including chemotherapeutic agents “etoposide, irinotecan, cisplatin, carboplatin, temozolomide, and paclitaxel,” targeted agents “anlotinib (a new small molecule multi-target tyrosine kinase inhibitor), surufatinib (a selective tyrosine kinase inhibitor targeting vascular endothelial receptor (VEGFR) and fibroblast growth factor receptor (FGFR)), and pamiparib (a DNA repair enzyme (PARP) inhibitor).” Furthermore, a split-course radiotherapy, which significantly improved the patients’ quality of life and prolonged her survival, was also performed . In conclusion, the clinical symptoms of LC-induced MIAP are not specific, and the clinical types are mainly MAP, with poor prognosis, but an early diagnosis and a timely treatment may greatly improve the prognosis. For elderly patients, emaciation, or manifestation of other affected systems, anemia, main pancreatic duct dilatation, pancreatic occupancy and abdominal LN enlargement, are important clues for the diagnosis of LC-induced MIAP that require in clinical treatment.	4.457031	0.575195	sec[2]/p[2]	en	0.999998	PMC10644230	https://doi.org/10.3389/fphar.2023.1259221	[ "induced", "miap", "sclc", "that", "patients", "pancreatic", "approximatively", "cases", "abdominal", "metastasis" ]	[ { "code": "EH6Z", "title": "Drug eruption of unspecified type" }, { "code": "8A06.Y", "title": "Other specified myoclonic disorders" }, { "code": "6A24.Z", "title": "Delusional disorder, unspecified" }, { "code": "ED50.0", "title": "Acquired ichthyosis" }, { "code": "4A85.02", "title": "Drug-induced cytopenia" }, { "code": "8A80.Z", "title": "Migraine, unspecified" }, { "code": "QA76", "title": "Medication or substance that is known to be an allergen without injury or harm" }, { "code": "PL13.6", "title": "Medication or substance that is known to be an allergen, as mode of injury or harm" }, { "code": "9C40.A0", "title": "Papilloedema" }, { "code": "PA6Z", "title": "Unintentional fall from unspecified height" } ]	=== ICD-11 CODES FOUND === [EH6Z] Drug eruption of unspecified type Also known as: Drug eruption of unspecified type \| Drug-induced eruptions \| drug eruption or rash of unspecified type \| rash due to drug NOS \| drug rash [8A06.Y] Other specified myoclonic disorders Also known as: Other specified myoclonic disorders \| Secondary myoclonus \| Myoclonus due to neurodegenerative or storage disorders \| Myoclonus due to neuronal ceroid lipofuscinosis \| Myoclonus due to Sialidosis [6A24.Z] Delusional disorder, unspecified Also known as: Delusional disorder, unspecified \| Delusional disorder \| persistent delusional disorders \| delusional perception \| induced delusional disorder [ED50.0] Acquired ichthyosis Definition: Acquired ichthyosis resembles autosomal dominant ichthyosis vulgaris but develops in adult life in individuals without a previous history of ichthyosis. It may be caused by certain drugs but when associated with underlying malignancy (i.e. paraneoplastic) it is strongly associated with Hodgkin lymphoma and may be the presenting sign of that disease. It may less commonly be associated with other lymphoid neoplasms or solid tumours. Also known as: Acquired ichthyosis \| Drug-induced ichthyosis Excludes: Hereditary ichthyosis [4A85.02] Drug-induced cytopenia Definition: Drug-induced cytopenia is a relatively common immune-mediated cytopenia and the target cells include erythrocytes, leukocytes, platelets and hematopoietic precursor cells in the marrow. The most frequent condition is the drug-induced immune thrombocytopenia and the most frequent implicated drugs are penicillin and structurally related drugs, quinine, quinidine, sulfonamide antibiotics, non-steroidal anti-inflammatory drugs and anticonvulsants. Also known as: Drug-induced cytopenia \| medicament-induced cytopenia [8A80.Z] Migraine, unspecified Also known as: Migraine, unspecified \| Migraine [QA76] Medication or substance that is known to be an allergen without injury or harm Definition: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm. Also known as: Medication or substance that is known to be an allergen without injury or harm Excludes: Medication or substance that is known to be an allergen, as mode of injury or harm [PL13.6] Medication or substance that is known to be an allergen, as mode of injury or harm Also known as: Medication or substance that is known to be an allergen, as mode of injury or harm \| medication error relating to known allergy to drug or substance \| adverse drug event relating to known allergy to drug or substance [9C40.A0] Papilloedema Definition: Optic disc swelling that results from increased intracranial pressure Also known as: Papilloedema \| Optic disc swelling that results from increased intracranial pressure Includes: Optic disc swelling that results from increased intracranial pressure [PA6Z] Unintentional fall from unspecified height Also known as: Unintentional fall from unspecified height \| fall NOS \| accidental fall \| fall causing injury that resulted in death \| fell NOS === GRAPH WALKS === --- Walk 1 --- [EH6Z] Drug eruption of unspecified type --PARENT--> [?] Drug eruptions --CHILD--> [EH62] Lichenoid drug eruption --- Walk 2 --- [EH6Z] Drug eruption of unspecified type --PARENT--> [?] Drug eruptions --RELATED_TO--> [?] Drug-associated immune complex vasculitis Def: Small vessel vasculitis typically presenting predominantly in the skin due to systemic exposure to certain drugs in predisposed individuals.... --- Walk 3 --- [8A06.Y] Other specified myoclonic disorders --PARENT--> [8A06] Myoclonic disorders --CHILD--> [8A06.2] Focal myoclonus Def: Sudden, involuntary twitching or jerking of a muscle or group of muscles which effects a localised area of the body.... --- Walk 4 --- [8A06.Y] Other specified myoclonic disorders --PARENT--> [8A06] Myoclonic disorders --EXCLUDES--> [?] Facial myokymia Def: Facial myokymia is an involuntary twitching, fine rippling or wave-like movement of facial muscles.... --- Walk 5 --- [6A24.Z] Delusional disorder, unspecified --PARENT--> [6A24] Delusional disorder Def: Delusional disorder is characterised by the development of a delusion or set of related delusions, typically persisting for at least 3 months and often much longer, in the absence of a Depressive, Man... --CHILD--> [6A24.2] Delusional disorder, in full remission Def: All definitional requirements for Delusional disorder in terms of symptoms and duration were previously met. Symptoms have ameliorated such that no significant symptoms remain. The remission may have ... --- Walk 6 --- [6A24.Z] Delusional disorder, unspecified --PARENT--> [6A24] Delusional disorder Def: Delusional disorder is characterised by the development of a delusion or set of related delusions, typically persisting for at least 3 months and often much longer, in the absence of a Depressive, Man... --CHILD--> [6A24.0] Delusional disorder, currently symptomatic Def: All definitional requirements for Delusional disorder in terms of symptoms and duration are currently met, or have been met within the past one month....	[ "[EH6Z] Drug eruption of unspecified type\n --PARENT--> [?] Drug eruptions\n --CHILD--> [EH62] Lichenoid drug eruption", "[EH6Z] Drug eruption of unspecified type\n --PARENT--> [?] Drug eruptions\n --RELATED_TO--> [?] Drug-associated immune complex vasculitis\n Def: Small vessel vasculitis typically presenting predominantly in the skin due to systemic exposure to certain drugs in predisposed individuals....", "[8A06.Y] Other specified myoclonic disorders\n --PARENT--> [8A06] Myoclonic disorders\n --CHILD--> [8A06.2] Focal myoclonus\n Def: Sudden, involuntary twitching or jerking of a muscle or group of muscles which effects a localised area of the body....", "[8A06.Y] Other specified myoclonic disorders\n --PARENT--> [8A06] Myoclonic disorders\n --EXCLUDES--> [?] Facial myokymia\n Def: Facial myokymia is an involuntary twitching, fine rippling or wave-like movement of facial muscles....", "[6A24.Z] Delusional disorder, unspecified\n --PARENT--> [6A24] Delusional disorder\n Def: Delusional disorder is characterised by the development of a delusion or set of related delusions, typically persisting for at least 3 months and often much longer, in the absence of a Depressive, Man...\n --CHILD--> [6A24.2] Delusional disorder, in full remission\n Def: All definitional requirements for Delusional disorder in terms of symptoms and duration were previously met. Symptoms have ameliorated such that no significant symptoms remain. The remission may have ...", "[6A24.Z] Delusional disorder, unspecified\n --PARENT--> [6A24] Delusional disorder\n Def: Delusional disorder is characterised by the development of a delusion or set of related delusions, typically persisting for at least 3 months and often much longer, in the absence of a Depressive, Man...\n --CHILD--> [6A24.0] Delusional disorder, currently symptomatic\n Def: All definitional requirements for Delusional disorder in terms of symptoms and duration are currently met, or have been met within the past one month...." ]	EH6Z	Drug eruption of unspecified type	[ { "from_icd11": "EH6Z", "icd10_code": "L270", "icd10_title": "Generalized skin eruption due to drugs and medicaments taken internally" }, { "from_icd11": "EH6Z", "icd10_code": "L27", "icd10_title": "Dermatitis due to substances taken internally" }, { "from_icd11": "6A24.Z", "icd10_code": "F22", "icd10_title": "Delusional disorders" }, { "from_icd11": "6A24.Z", "icd10_code": "F220", "icd10_title": "" }, { "from_icd11": "6A24.Z", "icd10_code": "F228", "icd10_title": "" }, { "from_icd11": "6A24.Z", "icd10_code": "F229", "icd10_title": "" }, { "from_icd11": "6A24.Z", "icd10_code": "F24", "icd10_title": "Shared psychotic disorder" }, { "from_icd11": "ED50.0", "icd10_code": "L850", "icd10_title": "Acquired ichthyosis" }, { "from_icd11": "4A85.02", "icd10_code": "D6959", "icd10_title": "Other secondary thrombocytopenia" }, { "from_icd11": "4A85.02", "icd10_code": "D6951", "icd10_title": "Posttransfusion purpura" }, { "from_icd11": "4A85.02", "icd10_code": "D695", "icd10_title": "Secondary thrombocytopenia" }, { "from_icd11": "4A85.02", "icd10_code": "D69", "icd10_title": "Purpura and other hemorrhagic conditions" }, { "from_icd11": "8A80.Z", "icd10_code": "G43B0", "icd10_title": "Ophthalmoplegic migraine, not intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43409", "icd10_title": "Hemiplegic migraine, not intractable, without status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43A0", "icd10_title": "Cyclical vomiting, in migraine, not intractable" } ]	L270	Generalized skin eruption due to drugs and medicaments taken internally
Table 1. Features of the reported pediatric case of anti-glomerular basement membrane disease. Author Year Age (years/sex) Initial symptom Pulmonary involvement GFR (mL/min) or creatinine (mg/dL) Kidney biopsy Anti-GBM ANCA type Dialysis IS Plasmapheresis Final outcome Our patient 2023 9/M Fever, pallor, lumbar pain, – GFR: 34 Crescents 9/14 + p-ANCA HD MP, CYC, RTX PE ( 11 ) N, 2 yr Anjum et al. 2023 10/M Fever, edema, HU + Crea: 4,5 Crescents/ necrosis + – HD MP, CYC PE N.A. Khan et al. 2022 7/F HU, PU (SUSP) _ N No crescent IF: linear deposits of IgG + – – MP, CYC DFPP N McAllisterr et al. 2022 14/M Diarrhea, vomiting, epistaxis, HU + Crea: 46,8 Not performed + – HD MP, CYC, RTX PE (33) ESRD RT Helander et al. 2021 2/F Sore throat, vomiting, edema, HBP – Crea: 5,27 Crescents/ necrosis + p-ANCA HD MP, CYC, RTX, AZA PE ( 15 ) ESRD RT 18 months Sobotta et al. 2021 17/M Dyspnea, cough, hemoptysis, HU + N Not performed + – – MP, CYC, Eculizumab PE (9) N 6 months Jen et al. 2021 4/M Fever, vomiting cough, HU, PU + N No crescent IF: Linear deposits of IgG + – – MP, RTX, MMF – N 9 months Timmermans et al. 2019 15/F Dyspnea, cough, thoracic pain + Crea: 1,09 Crescents + p-ANCA – MP, CYC, RTX PE (14) N 16 months Mannemuddhu er al. 2019 15/F Edema, oliguria, PU, HBP – Crea: 5,49 Crescents + – HD MP, CYC, RTX, AZA PE (10) ESRD Died Şişmanlar-Eyüboğlu et al. 2018 14/M Fever, malaise, cough, hemoptysis, joint pain + N Not performed + – – MP, CYC PE (11) N 1 yr Agarwal et al. 2017 11/M Vomiting, HU, HBP – Crea: 7,9 Crescents + p-ANCA HD MP, CYC PE ( 21 ) CKD Raj et al. 2017 2/F Fever, sore throat, malaise, edema, HU, PU, HBP + Crea: 7,01 Sclerosis: 9/12 + – HD, PD MP, CYC, MMF PE (5) ESRD, RT Dorval et al. 2017 7/F Fever, abdominal pain, cough, headache, HBP, HU + GFR: 6,3 Crescents (80%) + – – MP, CYC IA (10) CKD 21 months Xie et al. 2015 6/F Edema, oliguria – Crea: 9,45 Crescents 100%/ sclerosis + p-ANCA HD MP, CYC PE (8) ESRD Nagano et al. 2015 8/F HU, PU (SUSP) – N No crescents IF: linear deposits of IgG + – – MP, CYC DFPP ( 3 ) N O’Hagan et al. 2015 7/M Abdominal pain, HU, PU, LU – GFR: 102 Crescents: 95% + – HD ( 4 ) MP, CYC PE (19) CKD 1 yr Gray et al. 2015 9/M HU, fever + Acute renal failure Crescents > 90% + – MP, CYC, RTX PE CKD Bogdanovic et al. 2013 10/F Fever, malaise, leg pain + Normal Crescents/ necrosis + p-ANCA – MP, CYC, MMF – N 10 months Jiao et al. 2012 15/F Fever, cough, hemoptysis, oliguria, edema + Crea: 11 Sclerosis: 11/12 + – HD MP, CYC DFPP (3) ESRD Bayat et al. 2012 14/F Pallor, cough, dyspnea, malaise, HU, PU + DFG: 60 Crescents + – – MP, CYC PE (4) N Dalabih et al. 2012 9/F Respiratory distress, edema, HU, HBP, HPM + Crea: 7,35 Crescents – – HD, PD MP, CYC PE (6) ESRD PD Bjerre et al. 2012 1,5/M Fever, vomiting, edema, HBP, HU – Crea: 1,69 Crescents/necrosis (13/17) + – – MP, CYC, MMF PE (13) N Williamson et al. 2011 10/M Pallor, nausea vomiting, cough, HU – Crea: 11,5 Crescents (100%) + – PD MP, CYC, MMF tacrolimus PE (12) ESRD RT Williamson et al. 2010 17/M Fatigue, edema, HU + Crea: 13,9 Crescents (87%) + – PD, HD MP, CYC, AZA PE (11) ESRD HD 1 yr Williamson et al. 2010 10/M Fever, cough, hemoptysis, abdominal pain, vomiting, headache, edema + Crea: 6,2 Crescents (100%) + – PD MP, CYC, MMF PE (6) ESRD PD 1 yr Williamson et al. 2010 8/F Cough, sore throat + Crea: 7,7 Crescents (83%) + p-ANCA – – – Died Dixit et al. 2010 3/F Fever, pallor, fatigue, edema HU, PU – GFR: 23 Crescents: 6/8 + – – MP, CYC, MMF PE CKD 3 yr Poddar et al. 2010 9/M Fever, vomiting + Crea: 8,8 Not performed + p-ANCA HD MP, CYC PE (21) Died 3 months Naidoo et al. 2009 4/F Epistaxis, lethargy – GFR: 15 Crescents/ sclerosis + p-ANCA – MP, CYC PE (11) CKD 9 months Hecht et al. 2007 9/F Abdominal pain, vomiting, malaise, HU, PU – Crea: 1,4 crescents (60%) + – – MP, CYC PE (13) N 1yr Upshaw et al. 2007 16/F Pallor, lethargy, cough, HBP, HU, PU, LU + Crea: 4 Crescents – – – – – NA Bakkaloglu et al. 2006 5/F Fever, malaise, joint pain, oliguria, HU, PU – Crea: 3,2 Crescents + – PD MP, CYC, MMF PE N 15 months Hijosa et al. 2005 12/M Fever, rash, arthritis + GFR: 16 Crescents + p-ANCA – MP, CYC, MMF PE (10) CKD 18 months Gittins et al. 2004 14/F Pallor, headache, abdominal pain, vomiting, HBP, PU, HU + Crea: 19,25 Crescents/necrotic + – HD MP, CYC PE ESRD: HD Hibbs et al. 2001 4/M ARF, rash, abdominal pain, vomiting, fever, HU – Crea: 8,3 Crescents (100%) + – PD MP, CYC, AZA – ESRD: TR Filho et al. 2001 10/F Pallor, hemoptysis + GFR: 9,3 NP + p-ANCA PD – – Died Paueksakon et al. 1999 12/F Hemoptysis, cough + Normal crescents/necrosis + p-ANCA – MP, CYC PE (7) N Brito et al. 1997 5/F Anorexia, lethargy, HU – GFR: 39 Crescents + – MP, CYC PE (15) N 1 yr Bigler et al. 1997 0,9/F – + GFR: 5 IF: Linear deposits of IgG + – DP MP, CYC PE ESRD TR Boven et al. 1996 2/F Fever, anorexia – Crea:4 ,5 Crescents (90%) /necrosis + – PD MP, CYC PE (18) ESRD McCarthy et al. 1994 10/M Fever, cough, abdominal pain, vomiting, headache, malaise + Crea: 6.2 Crescents (100%) necrosis + – HD MP, CYC PE (6) ESRD TR Gilvarry et al. 1992 6/M Abdominal pain, anorexia, lethargy – Crea: 3,8 Crescents (70%) + – PD MP, CYC PE (14) N Harrity et al. 1991 13/F Cough, fever, weakness, respiratory distress + N Not performed + – – MP, CYC PE Died Levin et al. 1983 10/F Vomiting, sore throat, fever, oliguria – Creat: 4,5 Crescents (80%) + – DP MP, AZA, dipyridamole PE ESRD HD Martini et al. 1981 8/F Pallor, fatigue + GFR: 43 Sclerosis I + – HD MP, CYC PE ESRD Levin et al. 1979 7/F Diarrhea, vomiting, lethargic, anorexic, pale, anuric + Crea: 14,7 Crescents (100%) + – DP MP, CYC, AZA PE (16) ESRD:PD Levin et al. 1977 4/F Abdominal pain, pallor, anorexia, HU, PU – Crea: 5,27 Crescents (100%) + – – MP, AZA, CYC PE Died ANCA = anti-neutrophil cytoplasmic antibodies; AZA = azathioprine; ARF = acute renal failure; MP = methyl-prednisolone; Crea = creatinine; CKD = chronic kidney disease; CYC = cyclophosphamide; DFPP = double- filtration plasmapheresis; ESRD = end-stage renal disease; F = female; GN = glomerulonephritis; GFR = glomerular filtration rate; HBP = high blood pressure; HD = hemodialysis; HU = hematuria; HPM = hepatomegaly; IA = immunoadsorption; IS = immunosuppressant; LU = leucocytosis; M = male; MMF = mycophenolate mofetil; N = normal; N.A = not available; NP = not performed; PD = peritoneal dialysis; PE = plasma exchange; PU = proteinuria; RT = renal transplant; RTX = rituximab; SUSP = school urine screening program; Yr = year(s); (+) = positive/present; (–) = negative/absent.	4.039063	0.654785	sec[7]/p[1]	en	0.999994	39564132	https://doi.org/10.5414/CNCS111439	[ "crescents", "crea", "fever", "esrd", "anca", "pain", "vomiting", "cough", "edema", "pallor" ]	[ { "code": "LA13.6", "title": "Congenital malformations of choroid" }, { "code": "GB40/MF8Y&XT8W", "title": "Chronic nephritic syndrome : diffuse crescentic glomerulonephritis" }, { "code": "MF8Y", "title": "Other specified clinical findings in specimens from the urinary system" }, { "code": "MG26", "title": "Fever of other or unknown origin" }, { "code": "1D81.Z", "title": "Infectious mononucleosis, unspecified" }, { "code": "1B99", "title": "Pasteurellosis" }, { "code": "4A60.0", "title": "Familial Mediterranean fever" }, { "code": "JB40.0", "title": "Puerperal sepsis" }, { "code": "GB61.5", "title": "Chronic kidney disease, stage 5" }, { "code": "4A44.AZ", "title": "Antineutrophil cytoplasmic antibody-associated vasculitis, unspecified" } ]	=== ICD-11 CODES FOUND === [LA13.6] Congenital malformations of choroid Definition: These are single or multiple defects of the morphogenesis of the choroid, the vascular layer of the eye, identifiable at birth or during the intrauterine life. Also known as: Congenital malformations of choroid \| congenital anomaly of choroid \| choroid anomaly \| choroid deformity \| Congenital crescent or conus choroid [MF8Y] Other specified clinical findings in specimens from the urinary system Also known as: Other specified clinical findings in specimens from the urinary system \| Glomerular disease with minor glomerular abnormality \| Glomerular disease with minimal change disease \| Secondary glomerular disease with minor glomerular abnormality \| Glomerular disease with minor glomerular abnormality in diseases classified elsewhere [MG26] Fever of other or unknown origin Definition: An abnormal elevation of body temperature of unknown origin, often as a result of a pathologic process. Also known as: Fever of other or unknown origin \| febrile \| febris \| fever \| feverish Excludes: fever of unknown origin in newborn \| Malignant hyperthermia due to anaesthesia [1D81.Z] Infectious mononucleosis, unspecified Also known as: Infectious mononucleosis, unspecified \| Infectious mononucleosis \| Glandular fever \| Gammaherpesviral mononucleosis \| kissing disease [1B99] Pasteurellosis Definition: A disease caused by an infection with the gram-negative bacteria Pasteurella. This disease is characterised by local cellulitis and may lead to other clinical signs depending on the route of infection. Transmission is commonly by direct contact through the bite, scratch, or lick from an infected animal, inhalation of infected respiratory secretions, or ingestion of contaminated meat. Confirmation is by identification of Pasteurella from the affected individual. Also known as: Pasteurellosis \| pasteurella infection \| shipping fever \| transport fever [4A60.0] Familial Mediterranean fever Definition: FMF is an autoinflammatory disease associated with mutations in pyrin resulting in enhanced IL1 beta production. This results in clinical attacks of inflammation in the form of fever and serositis in the form of peritoneal, pleural or synovial inflammation along with increased acute phase reactants. Also known as: Familial Mediterranean fever \| Periodic disease \| FMF - [familial mediterranean fever] \| periodic fever \| periodic polyserositis [JB40.0] Puerperal sepsis Also known as: Puerperal sepsis \| puerperal fever \| postpartum sepsis \| generalised puerperal infection \| major puerperal infection Excludes: Obstetric pyaemic or septic embolism \| sepsis during labour [GB61.5] Chronic kidney disease, stage 5 Definition: Kidney failure, GFR < 15 ml/min/1.73m² Also known as: Chronic kidney disease, stage 5 \| chronic renal failure, stage 5 \| CKD - [chronic kidney disease] stage 5 \| end stage kidney failure \| end stage renal failure Includes: chronic renal failure, stage 5 [4A44.AZ] Antineutrophil cytoplasmic antibody-associated vasculitis, unspecified Also known as: Antineutrophil cytoplasmic antibody-associated vasculitis, unspecified \| Antineutrophil cytoplasmic antibody-associated vasculitis \| ANCA - [Antineutrophil cytoplasmic antibodies] associated vasculitis \| ANCA [Antineutrophil cytoplasmic antibodies] positive vasculitis === GRAPH WALKS === --- Walk 1 --- [LA13.6] Congenital malformations of choroid Def: These are single or multiple defects of the morphogenesis of the choroid, the vascular layer of the eye, identifiable at birth or during the intrauterine life.... --PARENT--> [LA13] Structural developmental anomalies of the posterior segment of eye Def: Any condition caused by failure of the posterior segment of the eye to correctly develop during the antenatal period. These conditions are characterised by clinical, functional, or morphological chang... --RELATED_TO--> [?] Juvenile retinoschisis Def: X-linked retinoschisis is a genetic ocular disease that is characterised by reduced visual acuity in males due to juvenile macular degeneration.... --- Walk 2 --- [LA13.6] Congenital malformations of choroid Def: These are single or multiple defects of the morphogenesis of the choroid, the vascular layer of the eye, identifiable at birth or during the intrauterine life.... --PARENT--> [LA13] Structural developmental anomalies of the posterior segment of eye Def: Any condition caused by failure of the posterior segment of the eye to correctly develop during the antenatal period. These conditions are characterised by clinical, functional, or morphological chang... --RELATED_TO--> [?] Juvenile retinoschisis Def: X-linked retinoschisis is a genetic ocular disease that is characterised by reduced visual acuity in males due to juvenile macular degeneration.... --- Walk 3 --- [MF8Y] Other specified clinical findings in specimens from the urinary system --PARENT--> [?] Clinical findings in specimens from the urinary system --CHILD--> [MF81] Fibronectin glomerulopathy Def: Fibronectin glomerulopathy is a rare hereditary kidney disease in which fibronectin (FN1) deposits are seen in the mesangium and subendothelial space. The clinical picture is characterised by proteinu... --- Walk 4 --- [MF8Y] Other specified clinical findings in specimens from the urinary system --PARENT--> [?] Clinical findings in specimens from the urinary system --CHILD--> [MF82] Lipoprotein glomerulopathy Def: Characteristic lipoprotein thrombi are found in the glomerulus in this genetically determined disease mainly found in East Asia.... --- Walk 5 --- [MG26] Fever of other or unknown origin Def: An abnormal elevation of body temperature of unknown origin, often as a result of a pathologic process.... --EXCLUDES--> [?] Malignant hyperthermia due to anaesthesia Def: A condition caused by hypermetabolism in response to certain anaesthetic drugs. This condition is characterised by hyperthermia, tachycardia, tachypnoea, increased carbon dioxide production, increased... --PARENT--> [?] Other injury or harm from surgical or medical care, not elsewhere classified --- Walk 6 --- [MG26] Fever of other or unknown origin Def: An abnormal elevation of body temperature of unknown origin, often as a result of a pathologic process.... --EXCLUDES--> [?] Disturbances of temperature regulation of newborn Def: Normal body temperature of newborn is 36.5 degrees Celsius (S.D. = 0.6 degrees Celsius). Temperature above 38.0 and below 36.0 may be regarded as unusual and called hyper- and hypothermia respectively... --CHILD--> [?] Environmental hyperthermia of newborn Def: A paediatric condition characterised by a core body temperature above 37.5 degrees C (99.5 degrees F) in a newborn due to exposure of the newborn to prolonged or extremely high environmental temperatu...	[ "[LA13.6] Congenital malformations of choroid\n Def: These are single or multiple defects of the morphogenesis of the choroid, the vascular layer of the eye, identifiable at birth or during the intrauterine life....\n --PARENT--> [LA13] Structural developmental anomalies of the posterior segment of eye\n Def: Any condition caused by failure of the posterior segment of the eye to correctly develop during the antenatal period. These conditions are characterised by clinical, functional, or morphological chang...\n --RELATED_TO--> [?] Juvenile retinoschisis\n Def: X-linked retinoschisis is a genetic ocular disease that is characterised by reduced visual acuity in males due to juvenile macular degeneration....", "[LA13.6] Congenital malformations of choroid\n Def: These are single or multiple defects of the morphogenesis of the choroid, the vascular layer of the eye, identifiable at birth or during the intrauterine life....\n --PARENT--> [LA13] Structural developmental anomalies of the posterior segment of eye\n Def: Any condition caused by failure of the posterior segment of the eye to correctly develop during the antenatal period. These conditions are characterised by clinical, functional, or morphological chang...\n --RELATED_TO--> [?] Juvenile retinoschisis\n Def: X-linked retinoschisis is a genetic ocular disease that is characterised by reduced visual acuity in males due to juvenile macular degeneration....", "[MF8Y] Other specified clinical findings in specimens from the urinary system\n --PARENT--> [?] Clinical findings in specimens from the urinary system\n --CHILD--> [MF81] Fibronectin glomerulopathy\n Def: Fibronectin glomerulopathy is a rare hereditary kidney disease in which fibronectin (FN1) deposits are seen in the mesangium and subendothelial space. The clinical picture is characterised by proteinu...", "[MF8Y] Other specified clinical findings in specimens from the urinary system\n --PARENT--> [?] Clinical findings in specimens from the urinary system\n --CHILD--> [MF82] Lipoprotein glomerulopathy\n Def: Characteristic lipoprotein thrombi are found in the glomerulus in this genetically determined disease mainly found in East Asia....", "[MG26] Fever of other or unknown origin\n Def: An abnormal elevation of body temperature of unknown origin, often as a result of a pathologic process....\n --EXCLUDES--> [?] Malignant hyperthermia due to anaesthesia\n Def: A condition caused by hypermetabolism in response to certain anaesthetic drugs. This condition is characterised by hyperthermia, tachycardia, tachypnoea, increased carbon dioxide production, increased...\n --PARENT--> [?] Other injury or harm from surgical or medical care, not elsewhere classified", "[MG26] Fever of other or unknown origin\n Def: An abnormal elevation of body temperature of unknown origin, often as a result of a pathologic process....\n --EXCLUDES--> [?] Disturbances of temperature regulation of newborn\n Def: Normal body temperature of newborn is 36.5 degrees Celsius (S.D. = 0.6 degrees Celsius). Temperature above 38.0 and below 36.0 may be regarded as unusual and called hyper- and hypothermia respectively...\n --CHILD--> [?] Environmental hyperthermia of newborn\n Def: A paediatric condition characterised by a core body temperature above 37.5 degrees C (99.5 degrees F) in a newborn due to exposure of the newborn to prolonged or extremely high environmental temperatu..." ]	LA13.6	Congenital malformations of choroid	[ { "from_icd11": "LA13.6", "icd10_code": "Q143", "icd10_title": "Congenital malformation of choroid" }, { "from_icd11": "MG26", "icd10_code": "R5081", "icd10_title": "Fever presenting with conditions classified elsewhere" }, { "from_icd11": "MG26", "icd10_code": "R5084", "icd10_title": "Febrile nonhemolytic transfusion reaction" }, { "from_icd11": "MG26", "icd10_code": "R5082", "icd10_title": "Postprocedural fever" }, { "from_icd11": "MG26", "icd10_code": "R5083", "icd10_title": "Postvaccination fever" }, { "from_icd11": "MG26", "icd10_code": "R509", "icd10_title": "Fever, unspecified" }, { "from_icd11": "MG26", "icd10_code": "R502", "icd10_title": "Drug induced fever" }, { "from_icd11": "MG26", "icd10_code": "R50", "icd10_title": "Fever of other and unknown origin" }, { "from_icd11": "MG26", "icd10_code": "R508", "icd10_title": "Other specified fever" }, { "from_icd11": "1D81.Z", "icd10_code": "B2700", "icd10_title": "Gammaherpesviral mononucleosis without complication" }, { "from_icd11": "1D81.Z", "icd10_code": "B2790", "icd10_title": "Infectious mononucleosis, unspecified without complication" }, { "from_icd11": "1D81.Z", "icd10_code": "B2709", "icd10_title": "Gammaherpesviral mononucleosis with other complications" }, { "from_icd11": "1D81.Z", "icd10_code": "B2780", "icd10_title": "Other infectious mononucleosis without complication" }, { "from_icd11": "1D81.Z", "icd10_code": "B2799", "icd10_title": "Infectious mononucleosis, unspecified with other complication" }, { "from_icd11": "1D81.Z", "icd10_code": "B2701", "icd10_title": "Gammaherpesviral mononucleosis with polyneuropathy" } ]	Q143	Congenital malformation of choroid
An 88‐year‐old man treated with imatinib for CML was admitted to our hospital because of dyspnea and malaise. His current medical history was congestive heart failure, hypertension, type 2 diabetes, and chronic kidney disease G5A3. The patient underwent left nephrectomy for left renal cancer in November 2010. At discharge, decreased renal function was observed with blood urea nitrogen (BUN) 23 mg/dL and creatinine (Cr) 1.5 mg/dL. He was then regularly followed by his family doctor. In February 2014, a routine follow‐up blood test revealed white blood cell (WBC) count of 32.6 × 10 3 /μL, and he was referred to our hematology department in March 2014. At that time, renal function deterioration was recognized, with BUN 46 mg/dL and Cr 4.7 mg/dL, and he was clinically diagnosed with chronic phase Philadelphia‐positive CML, which was later confirmed by blood tests, bone marrow examination, and imaging findings. In April of the same year, imatinib was started at a dose of 100 mg. After 3 weeks, the dose was increased to 200 mg. Blood test during outpatient visits showed gradual deterioration of renal function, and he was scheduled to consult a nephrology department. In May of the same year, he complained of dyspnea and malaise and consulted his family doctor. However, he was unable to go the local hospital because of poor physical condition, and was transported to the emergency department of our hospital and was admitted. On the day of admission (day −8), BUN was 54 mg/dL and Cr was 5.1 mg/dL, indicating impaired renal function. At that time, ejection fraction (EF) was 49.6%, laboratory test values were 23.2 U/L for aspartate aminotransferase (AST), 28.8 U/L for alanine aminotransferase (ALT), and 15.1 U/L for γ‐glutamyl transpeptidase (γ‐GTP), SpO2 was 93.9% (room air). A plain chest radiograph suggested pulmonary edema. Therefore, he was admitted to the Department of Nephrology of our hospital for initiation of dialysis and treatment of respiratory failure. Imatinib was suspected to have caused the rapid decline in renal function, and administration was discontinued on the same day. He had dyspnea and was poorly oxygenated; SpO 2 was 90% with oxygen administration at 10 L/min. Pleural effusion and pulmonary edema observed on chest radiograph were considered to be flooding due to exacerbation of chronic renal failure. A flexible double‐lumen (FDL) catheter was inserted through the right internal jugular vein and emergency dialysis was started. Blood pressure at admission was as high as 182/98 mm Hg, and nitroglycerin was administered at 2 mL/h and human atrial natriuretic peptide (hANP) at 0.75 mL/h. Respiratory distress gradually improved with administration of nitroglycerin and hANP, and water removal by dialysis. On day 2, 2 L/min of oxygen delivered via nasal cannula improved SpO 2 to 98%, and administration of nitroglycerin and hANP was terminated. The patient was taking 5 mg of oral amlodipine at admission, and the dose was increased to 10 mg due to high blood pressure, and returned to 5 mg when blood pressure decreased by water removal. On day 1, SpO 2 was maintained at 98% without oxygen administration, and oxygen therapy was terminated on the same day. Chronic changes in the kidney were observed based on the increase of Cr before admission and CT findings at admission, and maintenance dialysis was considered necessary in the future. Therefore, on day 1, a shunt was built in the left forearm, and maintenance dialysis became possible. After dialysis was initiated, a hematologist re‐administered oral imatinib on day 1. At that time, ejection fraction (EF) was 58.6%, laboratory test values were 26.3 U/L for aspartate aminotransferase (AST), 26.3 U/L for alanine aminotransferase (ALT), and 11.3 U/L for γ‐glutamyl transpeptidase (γ‐GTP), SpO2 was 97.0% (room air). A nephrologist consulted us regarding dosage regiment of imatinib and removal rate of imatinib by dialysis in patients with chronic kidney disease. Our search found no clear guidelines regarding the dose of imatinib for dialysis patients. Since imatinib is metabolized in the liver, and 67% is excreted in feces and 13% in urine, dosage reduction is not recommended even in patients with renal failure. However, several reports indicated that the same dose for patients with normal renal function is used in patients with renal failure, and that adverse effects were severe in dialysis patients due to high plasma imatinib concentration, necessitating dose reduction. 16 Therefore, we suspected that the plasma concentration of imatinib was increased to some extent in this patient. We proposed to measure the plasma concentration of imatinib to monitor how dialysis affects the effects and side effects of imatinib. The dose used to start re‐administration was 200 mg/day, the same as that before treatment suspension. At that time (day 1), WBC was 27.1 × 10 3 /μL, PLT was 423 × 10 3 /μL, BUN was 40.7 mg/dL, and Cr was 5.0 mg/dL. Plasma concentration was determined by a high performance liquid chromatographic method as described previously. 17 Using this method, the plasma concentration of imatinib was 1667 ng/mL at the 8th day of re‐administration. This value was higher than the mean trough concentration of 1002 ng/mL reported to be effective in treating CML by Picard et al 18 After confirming the TDM results, the attending physician decided to continue prescribing the same dose. After resuming imatinib, WBC counts declined markedly to 14.65 × 10 3 /μL on day 20. On day 28 (27 days after resuming administration), steady‐state plasma concentration was confirmed. The trough plasma concentration of imatinib was 2514 ng/mL . This value was lower than the mean trough concentration of 3180 ng/mL reported to be associated with a higher frequency of grade 3/4 adverse events such as neutropenia. 19 Renal function did not exacerbate, and no other adverse events were observed. At that time, WBC was 10.24 × 10 3 /μL, PLT was 155 × 10 3 /μL, BUN was 48.0 mg/dL, and Cr was 6.0 mg/dL. After confirming the TDM results and no adverse events, the attending physician decided to continue prescribing the same dose. On day 35, complete hematologic response (CHR) was achieved with normalization of peripheral blood data and extramedullary lesions. CHR was achieved within 3 months after the start of treatment, corresponding to the criteria of optimal response to TKI treatment. 20 On day 40, the patient's condition was stable with dialysis and imatinib therapy, and he was transferred to another hospital.	3.939453	0.977539	sec[1]/p[0]	en	0.999996	34429976	https://doi.org/10.1002/ccr3.4357	[ "imatinib", "renal", "dialysis", "blood", "that", "concentration", "administration", "function", "plasma", "patients" ]	[ { "code": "GC2Z&XA6KU8", "title": "Disease of kidney, not elsewhere classified" }, { "code": "GB6Z", "title": "Kidney failure, unspecified" }, { "code": "LB30.1", "title": "Renal dysplasia" }, { "code": "NB92.0Y", "title": "Other specified injury of kidney" }, { "code": "LB30.7", "title": "Ectopic or pelvic kidney" }, { "code": "QB94.Z", "title": "Care involving dialysis, unspecified" }, { "code": "QB94.1", "title": "Care involving extracorporeal dialysis" }, { "code": "9B73.0", "title": "Retinal detachment with retinal break" }, { "code": "QB94.2", "title": "Care involving peritoneal dialysis" }, { "code": "QB94.Y", "title": "Care involving other specified dialysis" } ]	=== ICD-11 CODES FOUND === [GB6Z] Kidney failure, unspecified Also known as: Kidney failure, unspecified \| nontraumatic kidney injury \| renal failure NOS \| kidney block \| renal impairment NOS [LB30.1] Renal dysplasia Definition: A condition characterised by abnormal development of one or both kidneys. Also known as: Renal dysplasia \| congenital renal dysplasia \| dysplasia of kidney \| dysplastic kidney \| Primary renal dysplasia Excludes: Autosomal dominant polycystic kidney disease [NB92.0Y] Other specified injury of kidney Also known as: Other specified injury of kidney \| Injury of kidney without open wound into cavity \| Injury of kidney with open wound into cavity \| Haematoma of kidney \| traumatic perirenal haematoma [LB30.7] Ectopic or pelvic kidney Definition: A birth defect characterised by an abnormally positioned kidney; may be asymptomatic or result in urine blockage, infection or kidney stones Also known as: Ectopic or pelvic kidney \| Congenital displaced kidney \| congenital misplaced kidney \| congenital malposition of kidney \| congenital prolapsed kidney Includes: Congenital displaced kidney \| Malrotation of kidney [QB94.Z] Care involving dialysis, unspecified Also known as: Care involving dialysis, unspecified \| Care involving dialysis \| dialysis preparation and treatment \| dialysis NOS [QB94.1] Care involving extracorporeal dialysis Also known as: Care involving extracorporeal dialysis \| encounter for extracorporeal dialysis \| encounter for dialysis NOS \| encounter for renal dialysis NOS \| aftercare involving extracorporeal dialysis [9B73.0] Retinal detachment with retinal break Also known as: Retinal detachment with retinal break \| Rhegmatogenous retinal detachment \| ruptured retina with detachment \| retinal hole with detachment \| Retinal detachment with giant retinal tear Includes: Rhegmatogenous retinal detachment [QB94.2] Care involving peritoneal dialysis Also known as: Care involving peritoneal dialysis \| aftercare involving peritoneal dialysis \| intermittent peritoneal dialysis \| peritoneum dialysis [QB94.Y] Care involving other specified dialysis Also known as: Care involving other specified dialysis === GRAPH WALKS === --- Walk 1 --- [GB6Z] Kidney failure, unspecified --PARENT--> [?] Kidney failure Def: Inability of the kidneys to adequately filter the blood of waste products, with a lower than normal glomerular filtration rate (GFR). Can be abrupt and potentially reversible (acute kidney injury) or ... --RELATED_TO--> [?] Congenital renal failure Def: A severe irreversible decline in the ability of kidneys to remove wastes, concentrate urine, and maintain electrolyte balance; blood pressure; and calcium metabolism which existed at, or often before,... --- Walk 2 --- [GB6Z] Kidney failure, unspecified --PARENT--> [?] Kidney failure Def: Inability of the kidneys to adequately filter the blood of waste products, with a lower than normal glomerular filtration rate (GFR). Can be abrupt and potentially reversible (acute kidney injury) or ... --RELATED_TO--> [?] Renal failure following abortion, ectopic or molar pregnancy --- Walk 3 --- [LB30.1] Renal dysplasia Def: A condition characterised by abnormal development of one or both kidneys.... --EXCLUDES--> [?] Autosomal dominant polycystic kidney disease Def: Multiple cysts in both kidneys increasing in number and size from adolescence, associated with development of hypertension and chronic renal failure. Autosomal dominant familial pattern is usual and d... --PARENT--> [?] Chronic tubulo-interstitial nephritis associated with familial or genetic diseases --- Walk 4 --- [LB30.1] Renal dysplasia Def: A condition characterised by abnormal development of one or both kidneys.... --EXCLUDES--> [?] Autosomal dominant polycystic kidney disease Def: Multiple cysts in both kidneys increasing in number and size from adolescence, associated with development of hypertension and chronic renal failure. Autosomal dominant familial pattern is usual and d... --CHILD--> [?] Autosomal dominant polycystic kidney disease, Type 2 Def: Autosomal dominant polycystic kidney disease due to mutations on Polycystin2 gene on chromosome 4 (PKD1 gene).... --- Walk 5 --- [NB92.0Y] Other specified injury of kidney --PARENT--> [NB92.0] Injury of kidney --CHILD--> [NB92.02] Laceration of kidney, minor --- Walk 6 --- [NB92.0Y] Other specified injury of kidney --PARENT--> [NB92.0] Injury of kidney --CHILD--> [NB92.00] Contusion of kidney, minor	[ "[GB6Z] Kidney failure, unspecified\n --PARENT--> [?] Kidney failure\n Def: Inability of the kidneys to adequately filter the blood of waste products, with a lower than normal glomerular filtration rate (GFR). Can be abrupt and potentially reversible (acute kidney injury) or ...\n --RELATED_TO--> [?] Congenital renal failure\n Def: A severe irreversible decline in the ability of kidneys to remove wastes, concentrate urine, and maintain electrolyte balance; blood pressure; and calcium metabolism which existed at, or often before,...", "[GB6Z] Kidney failure, unspecified\n --PARENT--> [?] Kidney failure\n Def: Inability of the kidneys to adequately filter the blood of waste products, with a lower than normal glomerular filtration rate (GFR). Can be abrupt and potentially reversible (acute kidney injury) or ...\n --RELATED_TO--> [?] Renal failure following abortion, ectopic or molar pregnancy", "[LB30.1] Renal dysplasia\n Def: A condition characterised by abnormal development of one or both kidneys....\n --EXCLUDES--> [?] Autosomal dominant polycystic kidney disease\n Def: Multiple cysts in both kidneys increasing in number and size from adolescence, associated with development of hypertension and chronic renal failure. Autosomal dominant familial pattern is usual and d...\n --PARENT--> [?] Chronic tubulo-interstitial nephritis associated with familial or genetic diseases", "[LB30.1] Renal dysplasia\n Def: A condition characterised by abnormal development of one or both kidneys....\n --EXCLUDES--> [?] Autosomal dominant polycystic kidney disease\n Def: Multiple cysts in both kidneys increasing in number and size from adolescence, associated with development of hypertension and chronic renal failure. Autosomal dominant familial pattern is usual and d...\n --CHILD--> [?] Autosomal dominant polycystic kidney disease, Type 2\n Def: Autosomal dominant polycystic kidney disease due to mutations on Polycystin2 gene on chromosome 4 (PKD1 gene)....", "[NB92.0Y] Other specified injury of kidney\n --PARENT--> [NB92.0] Injury of kidney\n --CHILD--> [NB92.02] Laceration of kidney, minor", "[NB92.0Y] Other specified injury of kidney\n --PARENT--> [NB92.0] Injury of kidney\n --CHILD--> [NB92.00] Contusion of kidney, minor" ]	GC2Z&XA6KU8	Disease of kidney, not elsewhere classified	[ { "from_icd11": "GB6Z", "icd10_code": "N19", "icd10_title": "Unspecified kidney failure" }, { "from_icd11": "GB6Z", "icd10_code": "N17-N19", "icd10_title": "" }, { "from_icd11": "GB6Z", "icd10_code": "N17", "icd10_title": "Acute kidney failure" }, { "from_icd11": "LB30.1", "icd10_code": "Q614", "icd10_title": "Renal dysplasia" }, { "from_icd11": "LB30.7", "icd10_code": "Q632", "icd10_title": "Ectopic kidney" }, { "from_icd11": "LB30.7", "icd10_code": "Q63", "icd10_title": "Other congenital malformations of kidney" }, { "from_icd11": "QB94.Z", "icd10_code": "Z4931", "icd10_title": "Encounter for adequacy testing for hemodialysis" }, { "from_icd11": "QB94.Z", "icd10_code": "Z4932", "icd10_title": "Encounter for adequacy testing for peritoneal dialysis" }, { "from_icd11": "QB94.Z", "icd10_code": "Z49", "icd10_title": "Encounter for care involving renal dialysis" }, { "from_icd11": "QB94.1", "icd10_code": "Z491", "icd10_title": "" }, { "from_icd11": "9B73.0", "icd10_code": "H33051", "icd10_title": "Total retinal detachment, right eye" }, { "from_icd11": "9B73.0", "icd10_code": "H33012", "icd10_title": "Retinal detachment with single break, left eye" }, { "from_icd11": "9B73.0", "icd10_code": "H33052", "icd10_title": "Total retinal detachment, left eye" }, { "from_icd11": "9B73.0", "icd10_code": "H33001", "icd10_title": "Unspecified retinal detachment with retinal break, right eye" }, { "from_icd11": "9B73.0", "icd10_code": "H33009", "icd10_title": "Unspecified retinal detachment with retinal break, unspecified eye" } ]	N19	Unspecified kidney failure
Case 1. A 37-year-old male patient was incidentally found to have a visceral aneurysm on abdominal ultrasound when he had a routine physical examination. A computed tomographic angiography (CTA) confirmed a 40 mm × 38 mm SAA near the origin of the SMA . The aneurysm arose off the superior-lateral margin of the SMA and projected anteriorly and to the right. The SMA was widely patent and angiographic canalization of the celiac trunk did not reveal any branches supplying the spleen. The treatment procedure was performed under local anesthesia. A 6-French catheter sheath was cannulated from the right femoral artery and a pigtail angiographic catheter was put into the abdominal aortic artery via a 0.035 inch guidewire. A digital subtraction angiogram (DSA) showed an aneurysm at the root of an aberrant splenic artery that was arising 3 cm distal to the origin of the SMA and the distal splenic artery (SA) diameter being 6 mm . Percutaneous transabdominal catheterization of the aneurysm was performed to cannulate the distal splenic artery as well as deposit coils. The distal splenic artery was cannulated through the lumen of the aneurysm. The catheter was then pulled back to the mid-splenic artery. Two 6 mm and one 8 mm coils were placed in the splenic artery. Two additional 6 mm coils were placed into the 8 mm coils to effect occlusion of the mid splenic artery. A repeated injection of contrast dye showed occlusion of the mid splenic artery. The left brachial artery was then punctured and cannulated with a 6-French catheter sheath from which a 5 F vertebral catheter was inserted into the abdominal aortic artery via a 0.035 inch guidewire because of the small angle of SMA. On sub-selective catheterization of the SMA we have also used 5 F vertebral catheter and curved Terumo guidewire (stiff type, Terumo Corporation, Japan). The covered stent (8 mm × 40 mm, Wallgraft, Boston Scientific, USA)was cannulated with a 6-French delivery introducer sheath via the 0.035 inch Terumo stiff type guidewire through the left brachial artery . The patient remained asymptomatic, apyrexial and haemodynamically stable after the procedure and was discharged after 48 h of observation. A CT scan of the abdomen 7 days later demonstrated the aberrant splenic artery aneurysm sac was full of thrombi with a tiny endoleak. Follow-up abdominal CT scan in 12 months postoperatively confirmed an occluded aneurysm sac with marked shrinkage and patency of the SMA . There was not any infarction or abscess formation in the spleen. No fever and the inflammatory markers were normal.Case 2. A 36-year-old female patient was admitted to our center just when the first case was discharged. She was diagnosed with mesenteric artery aneurysm at the outpatient department. But the CTA demonstrated a 36 mm × 32 mm splenic artery aneurysm near the origin of the SMA and the proximal caliber of the SAA was 11 mm . The root of the aberrant splenic artery aneurysm was arising at 2 cm distal to the origin of the SMA. The treatment procedure was same as in Case one. Three 8 mm and one 10 mm coils were placed in the splenic artery. The covered stent (10 mm × 40 mm, Wallgraft, Boston Scientific, USA)was cannulated with a 10-French delivery introducer sheath via the 0.035 inch Terumo stiff type guidewire through the left brachial artery. The completion aortogram revealed no evidence of endoleak; disappearance of the aberrant splenic artery aneurysms lumen and patency of the SMA and the branches immediately after the stent deployment . The patient was discharged in 48 h postoperatively and CTA demonstrated the aneurysm sac was full of thrombi with no endoleak on postoperative Day 9. Follow-up abdominal CT scan in 12 months postoperatively confirmed an occluded aneurysm sac with marked shrinkage . There was no infarction or abscess formation in the spleen. No fever and the inflammatory markers were normal.Case 3. A 52-year-old male patient was found to have a visceral aneurysm on abdominal CT when he was diagnosed with gallbladder stone at a routing physical examination. CTA also demonstrated a 35 mm × 34 mm splenic artery aneurysm near the origin of the SMA and the proximal neck of the SAA was 5 mm . The root of the aberrant splenic artery aneurysm was arising 30 mm distal to the origin of the SMA. The treatment procedure was same as in Case one and Case two. Three 7 mm and two 8 mm coils were placed in the splenic artery. The covered stent (10 mm × 50 mm, Wallgraft, Boston Scientific, USA)was cannulated with a 10-French delivery introducer sheath via the 0.035 inch Terumo stiff type guidewire through the left brachial artery. The completion aortogram revealed evidence of a small endoleak; disappearance of the aberrant splenic artery aneurysms lumen and patency of the SMA and the branches immediately after the stent deployment . He was transferred to the Department of Hepatobiliary Surgery to perform laparoscopic cholecystectomy three days postoperatively. No complaint or complication was documented during 10 months follow-up. The CTA demonstrated that the splenic artery aneurysm sac was thrombosed with marked shrinkage; the SMA is patent with no infarction or abscess formation in the spleen . No fever and the inflammatory markers were normal.Case 4. A 73-year-old male patient was found to have a splenic artery aneurysm on abdominal CTA. The CTA demonstrated a 49 mm × 59 mm splenic artery aneurysm near the origin of the SMA and the proximal neck of the SAA was 5 mm . The root of the aberrant splenic artery aneurysm was arising 30 mm distal to the origin of the SMA. The treatment procedure was same as above 3 cases. Two 5 mm and two 8 mm coils were placed in the splenic artery. The covered stent (10 mm × 50 mm, Wallgraft, Boston Scientific, USA)was cannulated with a 10-French delivery introducer sheath via the 0.035 inch Terumo stiff type guidewire through the left brachial artery. The completion aortogram revealed no evidence of endoleak; disappearance of the aberrant splenic artery aneurysms lumen and patency of the SMA and the branches immediately after the stent deployment . The patient was discharged and CTA demonstrated the aneurysm sac was full of thrombi with no endoleak on postoperative Day 5. No complaint or complication was documented during 6 months follow-up. The CTA demonstrated that the splenic artery aneurysm sac was thrombosed with marked shrinkage; the SMA is patent with no infarction or abscess formation in the spleen . No fever and the inflammatory markers were normal.	3.953125	0.976074	sec[1]/p[0]	en	0.999996	25176112	https://doi.org/10.1186/1471-2482-14-62	[ "artery", "splenic", "aneurysm", "origin", "aberrant", "abdominal", "cannulated", "guidewire", "coils", "stent" ]	[ { "code": "BD5Z", "title": "Diseases of arteries or arterioles, unspecified" }, { "code": "BD52", "title": "Certain specified disorders of arteries or arterioles" }, { "code": "BD52.3", "title": "Rupture of artery" }, { "code": "BD52.2", "title": "Stricture of artery" }, { "code": "BD40.Z", "title": "Atherosclerotic chronic arterial occlusive disease, unspecified" }, { "code": "3B8Z", "title": "Diseases of spleen, unspecified" }, { "code": "LA8Z", "title": "Structural developmental anomaly of heart or great vessels, unspecified" }, { "code": "3B81.Y", "title": "Other specified acquired disorders of spleen" }, { "code": "LB22.Y", "title": "Other specified structural developmental anomalies of spleen" }, { "code": "3B81.2", "title": "Atrophy of spleen" } ]	=== ICD-11 CODES FOUND === [BD5Z] Diseases of arteries or arterioles, unspecified Also known as: Diseases of arteries or arterioles, unspecified \| artery disease NOS \| arterial disease NOS \| arteriolar disease NOS \| disorder of artery NOS [BD52] Certain specified disorders of arteries or arterioles Also known as: Certain specified disorders of arteries or arterioles \| Aortic dilatation - joint hypermobility - arterial tortuosity \| Generalised arterial calcification of infancy \| Median arcuate ligament syndrome \| Aortic root abscess Excludes: collagen (vascular) diseases \| Hypersensitivity angiitis \| Acute arterial occlusion [BD52.3] Rupture of artery Also known as: Rupture of artery \| ruptured artery \| artery fistula \| Aortic duodenal fistula \| Aortic colon fistula Excludes: traumatic rupture of artery - see injury of blood vessel by body region [BD52.2] Stricture of artery Also known as: Stricture of artery \| arterial stenosis \| arterial stricture \| artery stricture \| stenosis of artery [BD40.Z] Atherosclerotic chronic arterial occlusive disease, unspecified Also known as: Atherosclerotic chronic arterial occlusive disease, unspecified \| Atherosclerotic chronic arterial occlusive disease \| arteriosclerosis, NOS \| generalised atherosclerosis \| atherosclerosis NOS [3B8Z] Diseases of spleen, unspecified Also known as: Diseases of spleen, unspecified \| splenic disease \| splenopathy [LA8Z] Structural developmental anomaly of heart or great vessels, unspecified Also known as: Structural developmental anomaly of heart or great vessels, unspecified \| Heart malformations \| Cardiac malformations \| congenital anomaly of heart \| congenital heart disease [3B81.Y] Other specified acquired disorders of spleen Also known as: Other specified acquired disorders of spleen \| Peliosis of spleen \| Congestion of spleen \| splenic congestion \| Lymphoid hyperplasia of spleen [LB22.Y] Other specified structural developmental anomalies of spleen Also known as: Other specified structural developmental anomalies of spleen \| Congenital malformation of spleen \| malformations of spleen NOS \| Aberrant spleen \| Congenital lobulation of spleen [3B81.2] Atrophy of spleen Definition: A disease caused by determinants arising after birth, during the antenatal period or by genetically inherited factors. This disease is characterised by partial or complete degradation of the spleen. This disease may present with increased susceptibility to infection. Confirmation is through medical imaging. Also known as: Atrophy of spleen \| hyposplenism \| splenic atrophy \| Hyposplenism due to previous infarction of spleen \| Degenerative diseases of the spleen === GRAPH WALKS === --- Walk 1 --- [BD5Z] Diseases of arteries or arterioles, unspecified --PARENT--> [?] Diseases of arteries or arterioles --EXCLUDES--> [?] Diseases of coronary artery Def: Conditions affecting the blood perfusion of the heart.... --- Walk 2 --- [BD5Z] Diseases of arteries or arterioles, unspecified --PARENT--> [?] Diseases of arteries or arterioles --CHILD--> [BD50] Aortic aneurysm or dissection Def: Aortic aneurysm is a term for any swelling (dilation or aneurysm) of the aorta to greater than 1.5 times normal, usually representing an underlying weakness in the wall of the aorta at that location. ... --- Walk 3 --- [BD52] Certain specified disorders of arteries or arterioles --CHILD--> [BD52.0] Segmental arterial mediolysis Def: Segmental arterial mediolysis is a rare noninflammatory vascular disease of the abdominal splanchnic arteries, characterised by disruption of the arterial medial layer. It will induce multiple aneurys... --PARENT--> [BD52] Certain specified disorders of arteries or arterioles --- Walk 4 --- [BD52] Certain specified disorders of arteries or arterioles --EXCLUDES--> [?] Leukocytoclastic vasculitis Def: Leukocytoclastic vasculitis (hypersensitivity vasculitis; hypersensitivity angiitis) is a histopathological term commonly used to denote a small-vessel vasculitis. It may be localised to the skin or m... --PARENT--> [?] Small vessel vasculitis Def: Vasculitis predominantly affecting small vessels, defined as small intraparenchymal arteries, arterioles, capillaries and venules. Medium sized arteries and veins may be affected.... --- Walk 5 --- [BD52.3] Rupture of artery --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes Def: !markdown In the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre... --CHILD--> [?] Injuries to the head --- Walk 6 --- [BD52.3] Rupture of artery --PARENT--> [BD52] Certain specified disorders of arteries or arterioles --CHILD--> [BD52.2] Stricture of artery	[ "[BD5Z] Diseases of arteries or arterioles, unspecified\n --PARENT--> [?] Diseases of arteries or arterioles\n --EXCLUDES--> [?] Diseases of coronary artery\n Def: Conditions affecting the blood perfusion of the heart....", "[BD5Z] Diseases of arteries or arterioles, unspecified\n --PARENT--> [?] Diseases of arteries or arterioles\n --CHILD--> [BD50] Aortic aneurysm or dissection\n Def: Aortic aneurysm is a term for any swelling (dilation or aneurysm) of the aorta to greater than 1.5 times normal, usually representing an underlying weakness in the wall of the aorta at that location. ...", "[BD52] Certain specified disorders of arteries or arterioles\n --CHILD--> [BD52.0] Segmental arterial mediolysis\n Def: Segmental arterial mediolysis is a rare noninflammatory vascular disease of the abdominal splanchnic arteries, characterised by disruption of the arterial medial layer. It will induce multiple aneurys...\n --PARENT--> [BD52] Certain specified disorders of arteries or arterioles", "[BD52] Certain specified disorders of arteries or arterioles\n --EXCLUDES--> [?] Leukocytoclastic vasculitis\n Def: Leukocytoclastic vasculitis (hypersensitivity vasculitis; hypersensitivity angiitis) is a histopathological term commonly used to denote a small-vessel vasculitis. It may be localised to the skin or m...\n --PARENT--> [?] Small vessel vasculitis\n Def: Vasculitis predominantly affecting small vessels, defined as small intraparenchymal arteries, arterioles, capillaries and venules. Medium sized arteries and veins may be affected....", "[BD52.3] Rupture of artery\n --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes\n Def: !markdown\nIn the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre...\n --CHILD--> [?] Injuries to the head", "[BD52.3] Rupture of artery\n --PARENT--> [BD52] Certain specified disorders of arteries or arterioles\n --CHILD--> [BD52.2] Stricture of artery" ]	BD5Z	Diseases of arteries or arterioles, unspecified	[ { "from_icd11": "BD5Z", "icd10_code": "I7389", "icd10_title": "Other specified peripheral vascular diseases" }, { "from_icd11": "BD5Z", "icd10_code": "I7419", "icd10_title": "Embolism and thrombosis of other parts of aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7411", "icd10_title": "Embolism and thrombosis of thoracic aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7410", "icd10_title": "Embolism and thrombosis of unspecified parts of aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7381", "icd10_title": "Erythromelalgia" }, { "from_icd11": "BD5Z", "icd10_code": "I745", "icd10_title": "Embolism and thrombosis of iliac artery" }, { "from_icd11": "BD5Z", "icd10_code": "I789", "icd10_title": "Disease of capillaries, unspecified" }, { "from_icd11": "BD5Z", "icd10_code": "I748", "icd10_title": "Embolism and thrombosis of other arteries" }, { "from_icd11": "BD5Z", "icd10_code": "I749", "icd10_title": "Embolism and thrombosis of unspecified artery" }, { "from_icd11": "BD5Z", "icd10_code": "I781", "icd10_title": "Nevus, non-neoplastic" }, { "from_icd11": "BD5Z", "icd10_code": "I788", "icd10_title": "Other diseases of capillaries" }, { "from_icd11": "BD5Z", "icd10_code": "I744", "icd10_title": "Embolism and thrombosis of arteries of extremities, unspecified" }, { "from_icd11": "BD5Z", "icd10_code": "I70-I79", "icd10_title": "" }, { "from_icd11": "BD5Z", "icd10_code": "I74", "icd10_title": "Arterial embolism and thrombosis" }, { "from_icd11": "BD5Z", "icd10_code": "I73", "icd10_title": "Other peripheral vascular diseases" } ]	I7389	Other specified peripheral vascular diseases
The clinical course of the three siblings is summarized in Table 1 . All patients presented with similar symptoms at different ages. Patient 1 (oldest sibling) was noted to have a limp and endorsed hip pain at 5 years of age; he was diagnosed with bilateral Legg-Calve-Perthes disease . At 7 years of age, he was referred to orthopedics for further evaluation and treatment at Children’s Hospital of Philadelphia (CHOP). Upon examination at the clinic, he presented with an abnormal wide gait with the lower legs placed in valgus; tenderness on the bilateral greater trochanter; and full range of motion, but with mild tenderness, on both knees and ankles. He underwent hip surgery and experienced a prolonged recovery with worsening of his symptoms. After his recovery, he was lost to follow-up and was seen again by orthopedics at CHOP at 11 years of age. At that time, he was unable to walk independently and was noted to have a wide-based stance with increased weakness. Pelvis radiographs over time demonstrated progressive destructive change of the hips . His progressive weakness prompted a referral to the neuromuscular clinic, and electromyography findings were suggestive of mild myopathy. Radiographs and MRIs of the spine revealed multiple abnormalities, with prominent middle third spine vertebral involvement, consisting of prominent anterior beaking, posterior endplate concavities, and mild platyspondyly . Spondyloepiphyseal dysplasia was suspected due to the combination of proximal femoral epiphyseal abnormalities, odontoid hypoplasia , and platyspondyly, but genetic sequencing and deletion/duplication studies of COL2A1 (collagen type II alpha 1) and SEDL (spondyloepiphyseal dysplasia tarda) were negative. In retrospect, other radiographic features, including anterior vertebral beaking and metacarpal abnormalities , favored MPS IVA. At 5 years of age, Patient 2 (middle sibling) was noted to experience the same pattern of clinical signs and symptoms as Patient 1. A skeletal survey was completed, which showed symmetrically abnormal femoral heads, radial and ulnar epiphyses, and carpal bones. Additionally, the lumbar vertebrae were noted to be slightly flattened and bullet shaped. Following diagnosis of the two older brothers, Patient 3 (youngest sibling) was diagnosed through family screening. The parents observed his wide-based gait at around 2.5 years of age, but the symptom was not brought to medical attention until diagnosis of his older siblings. Table 1 Clinical course for Patients 1, 2, and 3 with MPS IVA Patient 1 Patient 2 Patient 3 Current age 17 years, 8 months 13 years, 1 month 6 years, 2 months Type of genetic analysis Whole-exome sequencing Whole-exome sequencing Targeted GALNS sequencing Results of GALNS gene analysis c. 964G > C, p. A322P (paternally inherited, novel missense, variant of unknown significance) c. 611A > G, p. N204S (maternally inherited, novel missense, variant of unknown significance) Age at onset of symptoms 5 years 5 years 3 years Age at diagnosis 14 years, 7 months 10 years 3 years, 1 month Age at initiation of ERT 14 years, 8 months 10 years, 1 month 3 years, 2 months ERT enzyme replacement therapy, MPS IVA mucopolysaccharidosis type IVA Fig. 1 Anteroposterior radiograph of hips and pelvis in Patient 1 at 6 years of age shows bilateral sclerotic, fragmented, and flattened proximal femoral epiphyses (asterisks), irregular acetabula (arrowheads), and coxa valga deformities. The patient was initially diagnosed with Legg–Calve–Perthes disease; in retrospect, acetabular irregularities and coxa valga would not be typical in this condition Fig. 2 Representative serial radiographs of the proximal right femur of Patient 1 over time show progressive flattening of the epiphysis, concomitant widening of the proximal metaphyses, and increased irregularity of the acetabula resulting in compensatory pseudarthrosis-like formation Fig. 3 Lateral radiographs and MRIs of the thoracic spine in the three siblings. a In Patient 1, lateral thoracic spine radiograph (left) at 10 years of age shows relatively moderate vertebral body height loss (platyspondyly) and anterior vertebral body beaking, most prominent in the mid-to-lower thoracic spine (arrowheads). Sagittal T2-weighted MRI of the thoracic spine (right) shows moderate platyspondyly with prominent anterior beaking of multiple thoracic and upper lumbar vertebral bodies (arrowheads). The patient was initially diagnosed with spondyloepiphyseal dysplasia on the basis of clinical presentation, platyspondyly, and femoral epiphyseal abnormalities on imaging, although anterior beaking in the middle third of the spine is highly suggestive of MPS IVA, and vertebral flattening is not as prominent as would be expected in spondyloepiphyseal dysplasia. b In Patient 2, lateral thoracic spine radiograph at 6 years of age (left) shows anterior beaking of multiple thoracic vertebral bodies (arrowheads). Sagittal T2-weighted MRI of the thoracic spine at 10 years of age (right) shows anterior vertebral body beaking (arrowheads) with mild vertebral height loss without platyspondyly. Presence of anterior vertebral body beaking in this case is strongly suggestive of MPS IVA. c In Patient 3, lateral thoracic spine radiograph at 3 years of age (left) shows anterior beaking of multiple thoracic vertebral bodies without significant vertebral body height loss. Sagittal T2-weighted MRI of the thoracic spine at 5 years of age (right) highlights anterior vertebral body beaking. As with this patient’s siblings, anterior vertebral body beaking supported the diagnosis of MPS IVA Fig. 4 Sagittal T2-weighted TSE MRI of the cervical spine of Patient 1 at 10 years of age shows dysplastic odontoid process of C2, with irregular sclerosis and posterior migration of the tip of the dens (asterisk) Fig. 5 Radiographic features of the hand in the three siblings supportive of MPS IVA. a Patient 1 at 6 years of age showed prominent shortening of the fourth and fifth metacarpals (asterisks) with proximal pointing of the fifth metacarpal (arrowhead) and widening of the first metacarpal. b Patient 2 at 6 years of age showed proximal pointing of the fifth metacarpal (arrowhead) and irregular carpal bones (asterisk). Notably, this patient’s metacarpal abnormalities were much less conspicuous compared with those of his older brother (Patient 1) at the same age. c Patient 3 at 3 years of age showed proximally shortened, widened metacarpals with proximal pointing of the second and fifth metacarpals (arrowheads)	4.15625	0.859375	sec[2]/sec[0]/p[0]	en	0.999999	33256811	https://doi.org/10.1186/s13023-020-01618-y	[ "vertebral", "spine", "beaking", "thoracic", "body", "prominent", "platyspondyly", "arrowheads", "siblings", "abnormalities" ]	[ { "code": "FA72.Z", "title": "Disorders of vertebra, unspecified" }, { "code": "FA72.Y", "title": "Other specified disorders of vertebra" }, { "code": "FA8Z", "title": "Degenerative condition of spine, unspecified" }, { "code": "FB00", "title": "Ankylosis of spinal joint" }, { "code": "FB81.Y", "title": "Other specified osteonecrosis" }, { "code": "FB1Z", "title": "Conditions associated with the spine, unspecified" }, { "code": "FA7Z", "title": "Structural disorders of spine, unspecified" }, { "code": "FA9Z", "title": "Inflammation of spine, unspecified" }, { "code": "LB73.2Z", "title": "Structural developmental anomalies of spine, unspecified" }, { "code": "FA82", "title": "Spinal stenosis" } ]	=== ICD-11 CODES FOUND === [FA72.Z] Disorders of vertebra, unspecified Also known as: Disorders of vertebra, unspecified \| Disorders of vertebra \| vertebra disease \| vertebral disease [FA72.Y] Other specified disorders of vertebra Also known as: Other specified disorders of vertebra \| Destructive spondylopathy \| Destructive spondylopathy with no determinant \| primary spondylopathy \| Destructive spondylopathy with determinant [FA8Z] Degenerative condition of spine, unspecified Also known as: Degenerative condition of spine, unspecified \| spondylosis NOS \| degenerative change of spine or vertebra \| osteoarthritis of spine \| OA - [osteoarthritis of spine] [FB00] Ankylosis of spinal joint Also known as: Ankylosis of spinal joint \| ankylosis of spine nos \| fusion of vertebra NOS \| fusion of spine NOS \| Ankylosis of cervical spinal joint [FB81.Y] Other specified osteonecrosis Also known as: Other specified osteonecrosis \| Other secondary osteonecrosis \| Other secondary osteonecrosis, multiple sites \| Other secondary osteonecrosis, shoulder region \| Other secondary osteonecrosis, acromioclavicular joints [FB1Z] Conditions associated with the spine, unspecified Also known as: Conditions associated with the spine, unspecified \| dorsopathies \| disorder of spine \| spinal disorder [FA7Z] Structural disorders of spine, unspecified Also known as: Structural disorders of spine, unspecified \| spinal disease [FA9Z] Inflammation of spine, unspecified Also known as: Inflammation of spine, unspecified \| spinal inflammation \| discitis, unspecified [LB73.2Z] Structural developmental anomalies of spine, unspecified Also known as: Structural developmental anomalies of spine, unspecified \| Structural developmental anomalies of spine \| Malformations of spine \| maldevelopment of spine [FA82] Spinal stenosis Definition: This is a condition characterised by narrowing of the spinal canal. Also known as: Spinal stenosis \| spinal canal stenosis \| Spinal stenosis with no determinant \| primary spinal stenosis \| Spinal stenosis with determinant === GRAPH WALKS === --- Walk 1 --- [FA72.Z] Disorders of vertebra, unspecified --PARENT--> [FA72] Disorders of vertebra Def: Changes in the structure of the spine causing damage to vertebrae and surrounding tissue secondary to infection, injury, tumours, infections, bone changes that come with age etc. Spinal diseases often... --CHILD--> [FA72.2] Traumatic spondylopathy --- Walk 2 --- [FA72.Z] Disorders of vertebra, unspecified --PARENT--> [FA72] Disorders of vertebra Def: Changes in the structure of the spine causing damage to vertebrae and surrounding tissue secondary to infection, injury, tumours, infections, bone changes that come with age etc. Spinal diseases often... --CHILD--> [FA72.2] Traumatic spondylopathy --- Walk 3 --- [FA72.Y] Other specified disorders of vertebra --PARENT--> [FA72] Disorders of vertebra Def: Changes in the structure of the spine causing damage to vertebrae and surrounding tissue secondary to infection, injury, tumours, infections, bone changes that come with age etc. Spinal diseases often... --CHILD--> [FA72.1] Kissing spine --- Walk 4 --- [FA72.Y] Other specified disorders of vertebra --PARENT--> [FA72] Disorders of vertebra Def: Changes in the structure of the spine causing damage to vertebrae and surrounding tissue secondary to infection, injury, tumours, infections, bone changes that come with age etc. Spinal diseases often... --CHILD--> [FA72.1] Kissing spine --- Walk 5 --- [FA8Z] Degenerative condition of spine, unspecified --PARENT--> [?] Degenerative condition of spine Def: This is a disease characterised by degenerative changes in the intervertebral disc, vertebral end-plates and spinal joints due to aging or structural change.... --CHILD--> [FA82] Spinal stenosis Def: This is a condition characterised by narrowing of the spinal canal.... --- Walk 6 --- [FA8Z] Degenerative condition of spine, unspecified --PARENT--> [?] Degenerative condition of spine Def: This is a disease characterised by degenerative changes in the intervertebral disc, vertebral end-plates and spinal joints due to aging or structural change.... --CHILD--> [FA80] Intervertebral disc degeneration	[ "[FA72.Z] Disorders of vertebra, unspecified\n --PARENT--> [FA72] Disorders of vertebra\n Def: Changes in the structure of the spine causing damage to vertebrae and surrounding tissue secondary to infection, injury, tumours, infections, bone changes that come with age etc. Spinal diseases often...\n --CHILD--> [FA72.2] Traumatic spondylopathy", "[FA72.Z] Disorders of vertebra, unspecified\n --PARENT--> [FA72] Disorders of vertebra\n Def: Changes in the structure of the spine causing damage to vertebrae and surrounding tissue secondary to infection, injury, tumours, infections, bone changes that come with age etc. Spinal diseases often...\n --CHILD--> [FA72.2] Traumatic spondylopathy", "[FA72.Y] Other specified disorders of vertebra\n --PARENT--> [FA72] Disorders of vertebra\n Def: Changes in the structure of the spine causing damage to vertebrae and surrounding tissue secondary to infection, injury, tumours, infections, bone changes that come with age etc. Spinal diseases often...\n --CHILD--> [FA72.1] Kissing spine", "[FA72.Y] Other specified disorders of vertebra\n --PARENT--> [FA72] Disorders of vertebra\n Def: Changes in the structure of the spine causing damage to vertebrae and surrounding tissue secondary to infection, injury, tumours, infections, bone changes that come with age etc. Spinal diseases often...\n --CHILD--> [FA72.1] Kissing spine", "[FA8Z] Degenerative condition of spine, unspecified\n --PARENT--> [?] Degenerative condition of spine\n Def: This is a disease characterised by degenerative changes in the intervertebral disc, vertebral end-plates and spinal joints due to aging or structural change....\n --CHILD--> [FA82] Spinal stenosis\n Def: This is a condition characterised by narrowing of the spinal canal....", "[FA8Z] Degenerative condition of spine, unspecified\n --PARENT--> [?] Degenerative condition of spine\n Def: This is a disease characterised by degenerative changes in the intervertebral disc, vertebral end-plates and spinal joints due to aging or structural change....\n --CHILD--> [FA80] Intervertebral disc degeneration" ]	FA72.Z	Disorders of vertebra, unspecified	[ { "from_icd11": "FA8Z", "icd10_code": "M47892", "icd10_title": "Other spondylosis, cervical region" }, { "from_icd11": "FA8Z", "icd10_code": "M47818", "icd10_title": "Spondylosis without myelopathy or radiculopathy, sacral and sacrococcygeal region" }, { "from_icd11": "FA8Z", "icd10_code": "M47817", "icd10_title": "Spondylosis without myelopathy or radiculopathy, lumbosacral region" }, { "from_icd11": "FA8Z", "icd10_code": "M47896", "icd10_title": "Other spondylosis, lumbar region" }, { "from_icd11": "FA8Z", "icd10_code": "M47815", "icd10_title": "Spondylosis without myelopathy or radiculopathy, thoracolumbar region" }, { "from_icd11": "FA8Z", "icd10_code": "M47898", "icd10_title": "Other spondylosis, sacral and sacrococcygeal region" }, { "from_icd11": "FA8Z", "icd10_code": "M47819", "icd10_title": "Spondylosis without myelopathy or radiculopathy, site unspecified" }, { "from_icd11": "FA8Z", "icd10_code": "M47894", "icd10_title": "Other spondylosis, thoracic region" }, { "from_icd11": "FA8Z", "icd10_code": "M47897", "icd10_title": "Other spondylosis, lumbosacral region" }, { "from_icd11": "FA8Z", "icd10_code": "M47814", "icd10_title": "Spondylosis without myelopathy or radiculopathy, thoracic region" }, { "from_icd11": "FA8Z", "icd10_code": "M47895", "icd10_title": "Other spondylosis, thoracolumbar region" }, { "from_icd11": "FA8Z", "icd10_code": "M47899", "icd10_title": "Other spondylosis, site unspecified" }, { "from_icd11": "FA8Z", "icd10_code": "M47893", "icd10_title": "Other spondylosis, cervicothoracic region" }, { "from_icd11": "FA8Z", "icd10_code": "M47813", "icd10_title": "Spondylosis without myelopathy or radiculopathy, cervicothoracic region" }, { "from_icd11": "FA8Z", "icd10_code": "M479", "icd10_title": "Spondylosis, unspecified" } ]	M47892	Other spondylosis, cervical region
We report a transplantation case of the affected kidney from a viable donor with monolateral FMD, performed by resecting and subsequently reconstructing the pathological arterial segment with a cryopreserved cadaveric iliac graft without any complication. The etiology of FMD is not completely known. It is estimated that the incidence in general population is 2–3% , and it is higher in women. The most common symptom is hypertension, that is more associated with bilateral renal artery FMD , but clinical presentation of FMD varies widely from an asymptomatic condition to a multi-system disease depending on the anatomic distribution, extent of vascular involvement and type of FMD. For this reason, on one hand, despite of thorough preoperative assessments, failure to recognize beforehand grafts with FMD occasionally occurs when it comes to living donor kidney transplantation, especially if donors are clinically asymptomatic . On the other hand, considering the shortage of organs, patients with FMD should not be categorically rejected for donation but instead the decision to resort to them must be taken only after an accurate evaluation of standard criteria, such as age, comorbidities, glomerular filtration rate and creatinine level and after excluding any extra-renal involvement or surgically non correctable moderate or severe renal artery FMD . Our donor had no story of hypertension, and she was completely asymptomatic. As regard to donor safety, a subclinical renovascular disease like FMD has to be accurately diagnosed as well. As part of our transplant protocol, preliminary assessment for donation always includes an abdominal CTA. Due to a higher spatial resolution, CTA reaches better sensitivity than magnetic resonance angiography (97–100% versus 90–97%) [ 21 – 23 ]. In this case report, a diagnosis of monolateral medial FMD subtype was suspected on CTA, which showed “string of beads” appearance, defined as alternating segments of concomitant stenosis and post stenotic dilatation, involving only the right renal artery. Also, the morphological and histochemical findings were later consistent with the diagnosis of medial FMD subtype of the renal artery. We believe that the resection of the affected segment prevents long term vascular complications such as renal artery stenosis (RAS). Then, we decided to use a cryopreserved iliac artery graft from a deceased donor for the right renal artery end-to-end extension. Tondolo and colleagues reported what they described as a non-transplantable cadaveric kidney with FMD, due to the unsafe correction of the intrahilar extension of multiple aneurysms. In contrast, Hyo-Sin et al. reported a case of successful kidney transplantation using a deceased donor graft with severe FMD showing multiple saccular types of aneurysms and focal stenosis in the proximal renal artery, after a safe surgical correction, without resorting to a cryopreserved vascular graft. We believe that, if the renal artery left after correction is not sufficiently long for a safe anastomosis, an unaffected vessel graft could be harvested from the same cadaveric donor. In our opinion, Tondolo et al. reported a severe case of FMD, involving the hilum, which was unsafe to correct. In living donor setting such a situation is not tolerable and must be diagnosed during the preliminary assessment of the donors. In our case the renal artery was mildly involved and the segment affected was safe to be surgically corrected. Literature lacks clear guidelines on the eligibility of potential living renal donors with asymptomatic FMD. Rapid progression of mild FMD one year after transplantation have been reported, suggesting FMD cannot be considered benign in a potential normotensive renal donor . Nevertheless, it has also been reported that carefully selected patients with FMD have been successfully used as renal donors [ 18 – 20 , 24 , 25 ]. In fact, among asymptomatic renal donors with FMD presenting mild irregularity and no significant stenosis of the renal artery, none of them exhibited hypertension, proteinuria, or significant changes in serum creatinine level throughout a mean follow up of 4,5 years after donation . Sun et al. reported a case of renal transplant from living donor using the kidney with FMD that had a higher GFR than the unaffected one, pointing out the donor safety as regard to FMD progression. Nonetheless, the Authors did not describe whether, after nephrectomy, the remaining renal artery was still affected or not, since no surgical resection and subsequent reconstruction were reported. As regard to recipients, this may be judged unsafe, since some studies assessed the natural evolution of FMD, showing worsening stenosis in 33% of cases but no case of complete arterial obstruction. However, methodological problems have been found in those studies, with a risk of progression that may have been overestimated . Kidneys with FMD are generally considered a contraindication for transplantation, because this condition may progress after implantation, leading to the graft loss . This might be the reason why some Authors prefer to resect or repair the affected renal artery [ 1 , 20 , 24 – 26 ]. Even in a non transplant setting, surgical angioplasty is considered the ideal treatment of FMD because removal of the affected artery is only possible by surgical resection . However, interventional angioplasty is preferred over surgical angioplasty because the last one is associated with high morbidity and mortality (6.3% vs 15.4 and 0.9% vs 1.2% respectively) . More recently, Matsushita Y. et al. reported a case on arterial grafting of the donor renal artery affected by FMD ; a limitation of both surgical procedures performed in their and our case was the interruption of the internal iliac artery. The use of internal iliac artery in our patient was possible as the opposite side artery was not involved in a previous surgery. If both internal iliac arteries have been used for transplantation or the opposite side one is involved by severe stenosing atheromatous plaques, claudication is inevitable as is impotence . However, despite Matsushita’s paper looks very similar to ours, we found it less invasive using a cryopreserved arterial graft since it may allow to spare the internal iliac artery in accurately selected cases, by performing an end to side fashion anastomosis between the repaired arterial segment of the graft and the recipient vessels. Both the donor and the recipient need to be on close and long-term follow up.	4.277344	0.890625	sec[2]/p[0]	en	0.999998	33115426	https://doi.org/10.1186/s12882-020-02097-w	[ "renal", "artery", "donor", "graft", "affected", "that", "transplantation", "iliac", "kidney", "arterial" ]	[ { "code": "GC2Z&XA6KU8", "title": "Disease of kidney, not elsewhere classified" }, { "code": "GB6Z", "title": "Kidney failure, unspecified" }, { "code": "LB30.1", "title": "Renal dysplasia" }, { "code": "NB92.0Y", "title": "Other specified injury of kidney" }, { "code": "LB30.7", "title": "Ectopic or pelvic kidney" }, { "code": "BD5Z", "title": "Diseases of arteries or arterioles, unspecified" }, { "code": "BD52", "title": "Certain specified disorders of arteries or arterioles" }, { "code": "BD52.3", "title": "Rupture of artery" }, { "code": "BD52.2", "title": "Stricture of artery" }, { "code": "BD40.Z", "title": "Atherosclerotic chronic arterial occlusive disease, unspecified" } ]	=== ICD-11 CODES FOUND === [GB6Z] Kidney failure, unspecified Also known as: Kidney failure, unspecified \| nontraumatic kidney injury \| renal failure NOS \| kidney block \| renal impairment NOS [LB30.1] Renal dysplasia Definition: A condition characterised by abnormal development of one or both kidneys. Also known as: Renal dysplasia \| congenital renal dysplasia \| dysplasia of kidney \| dysplastic kidney \| Primary renal dysplasia Excludes: Autosomal dominant polycystic kidney disease [NB92.0Y] Other specified injury of kidney Also known as: Other specified injury of kidney \| Injury of kidney without open wound into cavity \| Injury of kidney with open wound into cavity \| Haematoma of kidney \| traumatic perirenal haematoma [LB30.7] Ectopic or pelvic kidney Definition: A birth defect characterised by an abnormally positioned kidney; may be asymptomatic or result in urine blockage, infection or kidney stones Also known as: Ectopic or pelvic kidney \| Congenital displaced kidney \| congenital misplaced kidney \| congenital malposition of kidney \| congenital prolapsed kidney Includes: Congenital displaced kidney \| Malrotation of kidney [BD5Z] Diseases of arteries or arterioles, unspecified Also known as: Diseases of arteries or arterioles, unspecified \| artery disease NOS \| arterial disease NOS \| arteriolar disease NOS \| disorder of artery NOS [BD52] Certain specified disorders of arteries or arterioles Also known as: Certain specified disorders of arteries or arterioles \| Aortic dilatation - joint hypermobility - arterial tortuosity \| Generalised arterial calcification of infancy \| Median arcuate ligament syndrome \| Aortic root abscess Excludes: collagen (vascular) diseases \| Hypersensitivity angiitis \| Acute arterial occlusion [BD52.3] Rupture of artery Also known as: Rupture of artery \| ruptured artery \| artery fistula \| Aortic duodenal fistula \| Aortic colon fistula Excludes: traumatic rupture of artery - see injury of blood vessel by body region [BD52.2] Stricture of artery Also known as: Stricture of artery \| arterial stenosis \| arterial stricture \| artery stricture \| stenosis of artery [BD40.Z] Atherosclerotic chronic arterial occlusive disease, unspecified Also known as: Atherosclerotic chronic arterial occlusive disease, unspecified \| Atherosclerotic chronic arterial occlusive disease \| arteriosclerosis, NOS \| generalised atherosclerosis \| atherosclerosis NOS === GRAPH WALKS === --- Walk 1 --- [GB6Z] Kidney failure, unspecified --PARENT--> [?] Kidney failure Def: Inability of the kidneys to adequately filter the blood of waste products, with a lower than normal glomerular filtration rate (GFR). Can be abrupt and potentially reversible (acute kidney injury) or ... --RELATED_TO--> [?] Renal failure following abortion, ectopic or molar pregnancy --- Walk 2 --- [GB6Z] Kidney failure, unspecified --PARENT--> [?] Kidney failure Def: Inability of the kidneys to adequately filter the blood of waste products, with a lower than normal glomerular filtration rate (GFR). Can be abrupt and potentially reversible (acute kidney injury) or ... --RELATED_TO--> [?] Renal failure following abortion, ectopic or molar pregnancy --- Walk 3 --- [LB30.1] Renal dysplasia Def: A condition characterised by abnormal development of one or both kidneys.... --PARENT--> [LB30] Structural developmental anomalies of kidneys Def: Any condition caused by failure of the kidneys to correctly develop during the antenatal period.... --CHILD--> [LB30.1] Renal dysplasia Def: A condition characterised by abnormal development of one or both kidneys.... --- Walk 4 --- [LB30.1] Renal dysplasia Def: A condition characterised by abnormal development of one or both kidneys.... --EXCLUDES--> [?] Autosomal dominant polycystic kidney disease Def: Multiple cysts in both kidneys increasing in number and size from adolescence, associated with development of hypertension and chronic renal failure. Autosomal dominant familial pattern is usual and d... --CHILD--> [?] Autosomal dominant polycystic kidney disease type 1 without tuberous sclerosis Def: Autosomal dominant polycystic kidney disease due to mutations on Polycystin1 gene on chromosome 16 (PKD1 gene)... --- Walk 5 --- [NB92.0Y] Other specified injury of kidney --PARENT--> [NB92.0] Injury of kidney --CHILD--> [NB92.01] Contusion of kidney, major --- Walk 6 --- [NB92.0Y] Other specified injury of kidney --PARENT--> [NB92.0] Injury of kidney --CHILD--> [NB92.02] Laceration of kidney, minor	[ "[GB6Z] Kidney failure, unspecified\n --PARENT--> [?] Kidney failure\n Def: Inability of the kidneys to adequately filter the blood of waste products, with a lower than normal glomerular filtration rate (GFR). Can be abrupt and potentially reversible (acute kidney injury) or ...\n --RELATED_TO--> [?] Renal failure following abortion, ectopic or molar pregnancy", "[GB6Z] Kidney failure, unspecified\n --PARENT--> [?] Kidney failure\n Def: Inability of the kidneys to adequately filter the blood of waste products, with a lower than normal glomerular filtration rate (GFR). Can be abrupt and potentially reversible (acute kidney injury) or ...\n --RELATED_TO--> [?] Renal failure following abortion, ectopic or molar pregnancy", "[LB30.1] Renal dysplasia\n Def: A condition characterised by abnormal development of one or both kidneys....\n --PARENT--> [LB30] Structural developmental anomalies of kidneys\n Def: Any condition caused by failure of the kidneys to correctly develop during the antenatal period....\n --CHILD--> [LB30.1] Renal dysplasia\n Def: A condition characterised by abnormal development of one or both kidneys....", "[LB30.1] Renal dysplasia\n Def: A condition characterised by abnormal development of one or both kidneys....\n --EXCLUDES--> [?] Autosomal dominant polycystic kidney disease\n Def: Multiple cysts in both kidneys increasing in number and size from adolescence, associated with development of hypertension and chronic renal failure. Autosomal dominant familial pattern is usual and d...\n --CHILD--> [?] Autosomal dominant polycystic kidney disease type 1 without tuberous sclerosis\n Def: Autosomal dominant polycystic kidney disease due to mutations on Polycystin1 gene on chromosome 16 (PKD1 gene)...", "[NB92.0Y] Other specified injury of kidney\n --PARENT--> [NB92.0] Injury of kidney\n --CHILD--> [NB92.01] Contusion of kidney, major", "[NB92.0Y] Other specified injury of kidney\n --PARENT--> [NB92.0] Injury of kidney\n --CHILD--> [NB92.02] Laceration of kidney, minor" ]	GC2Z&XA6KU8	Disease of kidney, not elsewhere classified	[ { "from_icd11": "GB6Z", "icd10_code": "N19", "icd10_title": "Unspecified kidney failure" }, { "from_icd11": "GB6Z", "icd10_code": "N17-N19", "icd10_title": "" }, { "from_icd11": "GB6Z", "icd10_code": "N17", "icd10_title": "Acute kidney failure" }, { "from_icd11": "LB30.1", "icd10_code": "Q614", "icd10_title": "Renal dysplasia" }, { "from_icd11": "LB30.7", "icd10_code": "Q632", "icd10_title": "Ectopic kidney" }, { "from_icd11": "LB30.7", "icd10_code": "Q63", "icd10_title": "Other congenital malformations of kidney" }, { "from_icd11": "BD5Z", "icd10_code": "I7389", "icd10_title": "Other specified peripheral vascular diseases" }, { "from_icd11": "BD5Z", "icd10_code": "I7419", "icd10_title": "Embolism and thrombosis of other parts of aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7411", "icd10_title": "Embolism and thrombosis of thoracic aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7410", "icd10_title": "Embolism and thrombosis of unspecified parts of aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7381", "icd10_title": "Erythromelalgia" }, { "from_icd11": "BD5Z", "icd10_code": "I745", "icd10_title": "Embolism and thrombosis of iliac artery" }, { "from_icd11": "BD5Z", "icd10_code": "I789", "icd10_title": "Disease of capillaries, unspecified" }, { "from_icd11": "BD5Z", "icd10_code": "I748", "icd10_title": "Embolism and thrombosis of other arteries" }, { "from_icd11": "BD5Z", "icd10_code": "I749", "icd10_title": "Embolism and thrombosis of unspecified artery" } ]	N19	Unspecified kidney failure
A 19-year-old woman was referred to our hospital for intermittent abdominal pain and continuous fever which had persisted for a month. She had no obvious medical or familial history. Physical examination revealed no morbid lymph node swelling or skin abnormalities. Blood examination showed normal blood cell counts and no presence of atypical lymphocytes. Biochemical or immunoserum analysis revealed modest inflammation, hypoalbuminemia, slight liver dysfunction, and evidence of past EBV infection (Additional file 1 : Table S1). Although esophagogastroduodenoscopy revealed normal findings, colonoscopy revealed a few small ulcers in the terminal ileum . Enhanced computed tomography showed obvious wall thickness of the small intestine, whereas no significant finding was detected by balloon intestinal endoscopy in the distal ileum. However, various types of ulcers surrounded by completely normal mucosa were found on the jejunum and proximal ileum by capsule endoscopy (CE) . Additionally, multiple aphthous and some linear ulcers were observed on the jejunum, whereas circular ulcers and longitudinal ulcers with a cobblestone appearance were detected on the ileum. The possibility of intestinal tuberculosis and infectious gastroenteritis were serologically and culturally excluded. Although there were no histological findings of noncaseating granuloma on her digestive organs, she was strongly suspected with CD based on her age, clinical symptoms, and the morphology of the characteristic ulcers . Consequently, the patient was treated with an elemental diet, mesalamine, and prednisolone for induction therapy. Although this treatment seemed to be partially effective, her symptoms recurred according to the tapering of prednisolone, and she was subsequently switched to adalimumab (ADA). Her clinical symptoms tended to be gradually modest, and the maintenance ADA therapy was effective for some time. The second CE revealed a definite improvement of the intestinal ulcers ; however, 21 months after the initiation of treatment, her intermittent fever and repeated abdominal pain recurred, and re-induction therapy with prednisolone was restarted . She remained stable for 9 months, before complaining of slight abdominal pain. We set a dose escalation of ADA with the addition of azathioprine; this seemed to be effective, and almost all of the ulcers disappeared, except for a few small erosions on the intestine . She next relapsed approximately 10 months later, and ADA was switched to ustekinumab (USK). However, this treatment was not sufficient, and prednisolone was added to USK for clinical improvement. Unfortunately, disease management was difficult regardless of these treatments, and skin ulcers appeared about 50 months after her initial hospital visit . Immunohistology of her skin biopsy revealed proliferation of EBV-encoded small RNA (EBER)-positive atypical lymphocytes . Retrospective assessments of the previous ileal ulcer biopsy before treatment demonstrated many EBER-positive lymphocytes, indicating that she had been in a continuously active EBV infection state . The blood level of EBV-DNA was also clearly positive (1.5 × 10 4 copy/WBC × 10 6 ). Consequently, she was diagnosed with extranodal NK/T-cell lymphoma (nasal type) related to CAEBV. Advanced-stage malignant lymphoma invading the skin was seen. Then, she was treated with a combination of cyclosporine A and prednisolone for her systemic inflammation related to CAEBV and followed by a systemic chemotherapy for malignant lymphoma. After completing the CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisolone), she gradually showed intermediate liver injury as well as cholestasis, based on which she was diagnosed with secondary hemophagocytic lymphohistiocytosis (HLH) caused by CAEBV. Though etoposide and methylprednisolone combination therapy transiently improved her liver injury and jaundice, CHOP insufficiently controlled the progression of CAEBV. Hence, the combination therapy of etoposide, cytosine arabinoside, l -asparaginase, methylprednisolone, and prednisolone (ESCAP) was preferred over the CHOP therapy. However, her liver injury and jaundice related to HLH rapidly worsened during the recovery period after the first ESCAP therapy. Unfortunately, her disease progression could not be regulated at all and she finally fell into the stage of multiple organ failure; she died at the age of 23 years, 55 months after her first hospital visit. An autopsy was performed with the consent from her parents. Macroscopic findings showed no evidence of ulcer or stenotic changes on her digestive organs, including the small intestine . Microscopic findings showed no evidence of noncaseating granuloma on digestive organs and a remarkable proliferation of lymphocytes in the intra-mucosal/submucosal space of the small intestine . Fig. 1 Various forms of ulcers on digestive organs detected by capsule endoscopy prior to treatment. The surrounding mucosa was free from inflammation. a Multiple aphthous ulcers on the jejunum. b Liner ulcers on the jejunum. c Longitudinal ulcers with a cobblestone appearance on the ileum. d Circular ulcers on ileum Fig. 2 Entire clinical course of the present case Fig. 3 Findings of the second capsule endoscopic examination. a , b Ulcer scars on the jejunum and ileum (18 months after the first treatment). c , d Small recurrent erosions on the jejunum and ileum (42 months after the first treatment) Fig. 4 Histological findings of extranodal NK/T-cell lymphoma (nasal type) associated with continuously activated EBV infection. a Macroscopic finding of skin ulcer on the patient’s thigh, which occurred more than 4 years after the initiation of medical treatment. b Microscopic finding of H&E staining of the skin ulcer. c Microscopic findings revealed high proliferation of EBER-positive atypical lymphocytes in the diseased skin. d Microscopic finding of H&E staining in the intestinal ulcer. e Microscopic immunohistological findings of ileal ulcerative lesions revealed moderate infiltration of many EBER-positive lymphocytes, which demonstrated continuously active EBV infection Fig. 5 Histological findings of the autopsied small intestine. a Macroscopic observation revealed neither ulcerative changes nor stenotic changes in the small intestine. b Microscopic finding of H&E staining of the intestinal tissue. c Microscopic observation revealed mild lymphocyte proliferation with no atrophy of the intestinal villi (large magnification of H&E staining)	4.101563	0.969238	sec[1]/p[0]	en	0.999996	33407170	https://doi.org/10.1186/s12876-020-01589-1	[ "ulcers", "small", "ileum", "skin", "prednisolone", "microscopic", "lymphocytes", "intestine", "intestinal", "jejunum" ]	[ { "code": "FB84.Z", "title": "Osteomyelitis or osteitis, unspecified" }, { "code": "EH90.Z", "title": "Pressure ulcer of unspecified grade" }, { "code": "CB40.Y", "title": "Other specified diseases of the respiratory system" }, { "code": "BD52", "title": "Certain specified disorders of arteries or arterioles" }, { "code": "9A76", "title": "Corneal ulcer" }, { "code": "MF54.2", "title": "Small kidney" }, { "code": "DA9Z", "title": "Diseases of small intestine, unspecified" }, { "code": "GA16.Y", "title": "Other specified acquired abnormalities of uterus, except cervix" }, { "code": "KA20.0Z", "title": "Small for gestational age, unspecified" }, { "code": "1A40.Z", "title": "Infectious gastroenteritis or colitis without specification of infectious agent" } ]	=== ICD-11 CODES FOUND === [FB84.Z] Osteomyelitis or osteitis, unspecified Also known as: Osteomyelitis or osteitis, unspecified \| Osteomyelitis or osteitis \| bone inflammation \| bone ulcer \| bone inflammatory disease [EH90.Z] Pressure ulcer of unspecified grade Also known as: Pressure ulcer of unspecified grade \| Pressure ulceration \| pressure injury \| pressure ulcer \| decubitus ulcer [CB40.Y] Other specified diseases of the respiratory system Also known as: Other specified diseases of the respiratory system \| Secondary respiratory disorders \| Respiratory disorders in diseases classified elsewhere \| Diseases of bronchus, not elsewhere classified \| Acquired bronchial diverticulum [BD52] Certain specified disorders of arteries or arterioles Also known as: Certain specified disorders of arteries or arterioles \| Aortic dilatation - joint hypermobility - arterial tortuosity \| Generalised arterial calcification of infancy \| Median arcuate ligament syndrome \| Aortic root abscess Excludes: collagen (vascular) diseases \| Hypersensitivity angiitis \| Acute arterial occlusion [9A76] Corneal ulcer Definition: Loss of epithelial tissue from the surface of the cornea due to progressive erosion and necrosis of the tissue. It is often caused by bacterial, fungal, or viral infection. Also known as: Corneal ulcer \| cornea ulcer \| ulcerative keratitis \| corneal ulcer NOS \| Central corneal ulcer Includes: Central corneal ulcer \| Ring corneal ulcer \| Corneal ulcer with hypopyon [MF54.2] Small kidney Definition: A condition characterised by a kidney smaller in size and weight than the average (less than 11 centimetres long, 5-7.5 centimetres wide, 2.5 centimetres thick, and weighing less than 120 grams). Also known as: Small kidney \| Small kidney, unilateral \| unilateral small kidney \| Small kidney, bilateral [DA9Z] Diseases of small intestine, unspecified Also known as: Diseases of small intestine, unspecified [GA16.Y] Other specified acquired abnormalities of uterus, except cervix Also known as: Other specified acquired abnormalities of uterus, except cervix \| Polyp of corpus uteri \| intrauterine polyp \| polyp of body of uterus \| polyp of uterus [KA20.0Z] Small for gestational age, unspecified Also known as: Small for gestational age, unspecified \| Small for gestational age \| Small-for-dates \| small fetus or newborn for gestational age \| dysmaturity [1A40.Z] Infectious gastroenteritis or colitis without specification of infectious agent Also known as: Infectious gastroenteritis or colitis without specification of infectious agent \| Gastroenteritis or colitis without specification of infectious agent \| diarrhoea and gastroenteritis of presumed infectious origin \| diarrhoeal enteritis \| GE - [gastroenteritis] === GRAPH WALKS === --- Walk 1 --- [FB84.Z] Osteomyelitis or osteitis, unspecified --PARENT--> [FB84] Osteomyelitis or osteitis --EXCLUDES--> [?] Inflammatory conditions of jaws --- Walk 2 --- [FB84.Z] Osteomyelitis or osteitis, unspecified --PARENT--> [FB84] Osteomyelitis or osteitis --EXCLUDES--> [?] Infection of vertebra Def: A condition of the vertebrae, caused by an infection with a bacterial, viral, fungal, or parasitic source. This condition commonly presents with fever, chills, headache, weight loss, or may be asympto... --- Walk 3 --- [EH90.Z] Pressure ulcer of unspecified grade --PARENT--> [EH90] Pressure ulceration Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th... --PARENT--> [?] Skin disorders provoked by external factors Def: A large group of skin disorders due to exposure of the skin to various external physical, chemical or environmental insults including chemical irritants and allergens, poisons, pressure, cold, heat, s... --- Walk 4 --- [EH90.Z] Pressure ulcer of unspecified grade --PARENT--> [EH90] Pressure ulceration Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th... --EXCLUDES--> [?] Erosion or ectropion of cervix uteri Def: A condition of the cervix uteri, caused by an increase in the total estrogen level in the body. This condition is characterised by protrusion and transformation of the endocervical columnar epithelium... --- Walk 5 --- [CB40.Y] Other specified diseases of the respiratory system --PARENT--> [CB40] Certain diseases of the respiratory system --RELATED_TO--> [?] Airway obstruction in the neonate due to airway abnormality --- Walk 6 --- [CB40.Y] Other specified diseases of the respiratory system --PARENT--> [CB40] Certain diseases of the respiratory system --CHILD--> [CB40.0] Ciliary dyskinesia Def: Defective function of the cilia lining the respiratory tract (lower and upper, sinuses, Eustachian tube, middle ear) resulting in altered mucociliary transport and manifesting as recurrent upper and l...	[ "[FB84.Z] Osteomyelitis or osteitis, unspecified\n --PARENT--> [FB84] Osteomyelitis or osteitis\n --EXCLUDES--> [?] Inflammatory conditions of jaws", "[FB84.Z] Osteomyelitis or osteitis, unspecified\n --PARENT--> [FB84] Osteomyelitis or osteitis\n --EXCLUDES--> [?] Infection of vertebra\n Def: A condition of the vertebrae, caused by an infection with a bacterial, viral, fungal, or parasitic source. This condition commonly presents with fever, chills, headache, weight loss, or may be asympto...", "[EH90.Z] Pressure ulcer of unspecified grade\n --PARENT--> [EH90] Pressure ulceration\n Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th...\n --PARENT--> [?] Skin disorders provoked by external factors\n Def: A large group of skin disorders due to exposure of the skin to various external physical, chemical or environmental insults including chemical irritants and allergens, poisons, pressure, cold, heat, s...", "[EH90.Z] Pressure ulcer of unspecified grade\n --PARENT--> [EH90] Pressure ulceration\n Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th...\n --EXCLUDES--> [?] Erosion or ectropion of cervix uteri\n Def: A condition of the cervix uteri, caused by an increase in the total estrogen level in the body. This condition is characterised by protrusion and transformation of the endocervical columnar epithelium...", "[CB40.Y] Other specified diseases of the respiratory system\n --PARENT--> [CB40] Certain diseases of the respiratory system\n --RELATED_TO--> [?] Airway obstruction in the neonate due to airway abnormality", "[CB40.Y] Other specified diseases of the respiratory system\n --PARENT--> [CB40] Certain diseases of the respiratory system\n --CHILD--> [CB40.0] Ciliary dyskinesia\n Def: Defective function of the cilia lining the respiratory tract (lower and upper, sinuses, Eustachian tube, middle ear) resulting in altered mucociliary transport and manifesting as recurrent upper and l..." ]	FB84.Z	Osteomyelitis or osteitis, unspecified	[ { "from_icd11": "FB84.Z", "icd10_code": "M86672", "icd10_title": "Other chronic osteomyelitis, left ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86172", "icd10_title": "Other acute osteomyelitis, left ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86171", "icd10_title": "Other acute osteomyelitis, right ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86671", "icd10_title": "Other chronic osteomyelitis, right ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X7", "icd10_title": "Other osteomyelitis, ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X8", "icd10_title": "Other osteomyelitis, other site" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X6", "icd10_title": "Other osteomyelitis, lower leg" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X9", "icd10_title": "Other osteomyelitis, unspecified sites" }, { "from_icd11": "FB84.Z", "icd10_code": "M8668", "icd10_title": "Other chronic osteomyelitis, other site" }, { "from_icd11": "FB84.Z", "icd10_code": "M86662", "icd10_title": "Other chronic osteomyelitis, left tibia and fibula" }, { "from_icd11": "FB84.Z", "icd10_code": "M86151", "icd10_title": "Other acute osteomyelitis, right femur" }, { "from_icd11": "FB84.Z", "icd10_code": "M86141", "icd10_title": "Other acute osteomyelitis, right hand" }, { "from_icd11": "FB84.Z", "icd10_code": "M86641", "icd10_title": "Other chronic osteomyelitis, right hand" }, { "from_icd11": "FB84.Z", "icd10_code": "M8669", "icd10_title": "Other chronic osteomyelitis, multiple sites" }, { "from_icd11": "FB84.Z", "icd10_code": "M8639", "icd10_title": "Chronic multifocal osteomyelitis, multiple sites" } ]	M86672	Other chronic osteomyelitis, left ankle and foot
We present the case of a 40-year-old Caucasian woman, a former smoker of 2.5 pack-year with an Eastern Cooperative Oncology Group Performance Status (ECOG PS) of 0. Her past medical history includes Hashimoto’s thyroiditis managed with levothyroxine. She had no surgical history and no family history of lung cancer. In October 2022, the patient presented to her general practitioner with persistent cough, dyspnea, and right hemithorax pain [Numeric Range Scale (NRS) = 6]. The patient was diagnosed with stage IV lung adenocarcinoma with no driver mutations and programmed death-ligand (PD-L1) expression <1%. Baseline computed tomography (CT) scan revealed an extensive solid tissue with irregular margins of 5.5 cm in the right perihilar area, with occlusion of the bronchial branch for the middle lobe. It also showed numerous solid nodular formations ranging from 6 to 36 mm, lymphadenopathy in the ilo-mediastinal area of approximately 2 cm, and morphological alteration of the XII right rib. Radiological staging according to the TNM staging VIII Edition system was cT4cN2cM1b Stage IVA ( 1 ). The patient underwent diagnostic EBUS-TBNA of the lower paratracheal and subcarinal mediastinal lymph nodes. The subject presented at our oncological outpatient department, and considering the absence of driver alterations, the negative value of PD-L1, and the need to start a systemic treatment to counteract oncological disease progression, we chose first-line systemic chemoimmunotherapy, a combination checkpoint blockade with nivolumab 360 mg every 3 weeks plus ipilimumab 1 mg/kg every 6 weeks, along with carboplatin AUC 5 + pemetrexed 500 mg/m 2 administered every 3 weeks for two cycles ( 2 ). Lipegfilgrastim, a G-CSF, was administered as primary prophylaxis of febrile neutropenia. Additionally, palliative radiation therapy was performed for symptomatic bone lesions on the right XII rib, delivering a total dose of 25 Gray in five fractions. However, the subject only received one cycle of the four-drug combination in November 2022, because in December 2022, the patient reported flu-like symptoms and retrosternal chest pain after treatment and was admitted to the oncology department of our center in Sant’Andrea Hospital of “La Sapienza” University of Rome and subsequently transferred to the cardiac intensive care unit for continuous monitoring. The physical examination revealed a visual acuity deficit, diplopia, severe ophthalmoparesis, left eyelid ptosis, mydriasis, rhinolalia, dysphagia, dyspnea, headache, nausea, dry mouth, asthenia, myalgia, neck flexion deficit, and extensor digitorum communis strength deficit. To exclude a secondary brain metastasis, a brain MRI was performed, which had negative results. Electrocardiogram revealed sinus rhythm, normal atrioventricular conduction, ST segment elevation in inferolateral and V3–V6 leads, and right bundle branch block . Echocardiogram showed normal left ventricular ejection fraction, hyperechogenicity of inferolateral wall, mild anterior pericardial detachment, and thickening . Laboratory tests revealed that Troponin I (hs-cTnI) was persistently elevated up to 13,963 U/L, without dynamic changes. The cardiac MRI confirmed the diagnosis of myocarditis demonstrating LVEF within normal values with mild hypokinesia of the lateral wall, edema, subepicardial late gadolinium enhancement at the level of the lateral wall and antero-lateral to the mid-apical segments, and minimal pericardial effusion at the basal level of the left ventricle. Laboratory tests also showed an increase in creatinine kinase (CK) up to 6,388 U/L and positive results for anti-SAE1 and anti-PL-7 antibodies. Open muscle biopsy was performed on the extensor digitorum communis revealing muscle fiber necrosis and regeneration with marked B and T lymphocytic endomysial inflammatory infiltrate (CD4=CD8=CD68) and diffuse expression of MHC-I, consistent with inflammatory myopathy . Concerning neuromuscular transmission assessment, the myasthenia-associated antibodies (anti-AChR and anti-MuSK) and repetitive nerve stimulation were negative, but the ice-pack test was clinically positive. The diagnosis of immune-related myocarditis–myasthenia–myositis association was made, and according to the guidelines ( 9 ), we started a therapy with oral prednisone at 1 mg/kg, pyridostigmine bromide 60 mg (one tablet for 4 days), and IV immunoglobulins at 0.4 g/kg daily for 4 days. Despite these interventions, poor clinical response and high troponin values persisted. Moreover, frequent ventricular ectopic beats, with bigeminy, were detected at ECG monitoring. Following a multidisciplinary discussion, therapy with methylprednisolone sodium succinate 1 g IV/day for 5 days was initiated, resulting in an accelerated decline in cTnI and CK levels and the disappearance of arrhythmias . The patient remained hemodynamically stable with marked improvement of myocarditis and myositis in the early phase and slow improvement of ophthalmoparesis in the following months. After 2 weeks of hospital stay and treatment, our patient showed a significant symptomatic recovery accompanied by a decrease in myocardial and skeletal muscle injury markers. Anti-PD1 and anti-CTLA 4 therapies were permanently discontinued for the manifestations of grade 3–4 adverse events and no other specific anti-tumor therapy was initiated at that time. Owing to persistent asthenia, the case was discussed with a neurologist colleague, leading to the initiation of methotrexate at 7.5 mg weekly. However, after 2 weeks, the treatment was suspended due to hepatic toxicity. In February 2023, restaging imaging following suspension of ipilimumab–nivolumab treatment revealed a partial response of disease. In March 2023, the patient was in remission from severe dose-limiting toxicity associated with previously carried out immunotherapy with regression of diplopia, dysphagia, bilateral ptosis, and asthenia. Blood tests were within normal limits. The dose of prednisone was tapered and pyridostigmine was suspended. Considering the improvement in general conditions, the patient was advised to resume chemotherapy according to the scheme CBDCA AUC5 plus pemetrexed 500 mg/m 2 every 3 weeks for four cycles. Subsequent restaging imaging following completion of treatment showed further reduction of disease. Thus, pemetrexed maintenance therapy was started and it is still ongoing. The patient underwent the last radiographic examination in November 2023, revealing continued partial disease response .	4.003906	0.976563	sec[1]/p[0]	en	0.999998	PMC11502290	https://doi.org/10.3389/fonc.2024.1431971	[ "anti", "every", "according", "pemetrexed", "deficit", "asthenia", "wall", "myocarditis", "muscle", "response" ]	[ { "code": "JA86.Y", "title": "Maternal care for other specified fetal problems" }, { "code": "MB23.1", "title": "Antisocial behaviour" }, { "code": "3B4Z", "title": "Coagulation defects, unspecified" }, { "code": "4A45.Z", "title": "Antiphospholipid syndrome, unspecified" }, { "code": "4A43.Y", "title": "Other specified overlap non-organ specific systemic autoimmune disease" }, { "code": "QA47.Z", "title": "Liveborn infants according to place of birth, unspecified" }, { "code": "EE12.1", "title": "Onychomycosis" }, { "code": "5C70.0", "title": "Dehydration" }, { "code": "9D42.2Z", "title": "Peripheral field deficit, unspecified" }, { "code": "5B7Z", "title": "Unspecified undernutrition" } ]	=== ICD-11 CODES FOUND === [JA86.Y] Maternal care for other specified fetal problems Also known as: Maternal care for other specified fetal problems \| Maternal care for other isoimmunization \| Isoimmunization NOS \| maternal antibodies NOS \| pregnancy management affected by incompatibility of blood groups NOS [MB23.1] Antisocial behaviour Definition: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated. Also known as: Antisocial behaviour \| Child or adolescent antisocial behaviour [3B4Z] Coagulation defects, unspecified Also known as: Coagulation defects, unspecified \| blood clotting disturbance \| blood clotting defect \| blood clotting factor deficiency \| clotting abnormality [4A45.Z] Antiphospholipid syndrome, unspecified Also known as: Antiphospholipid syndrome, unspecified \| Antiphospholipid syndrome \| Hughes syndrome \| Anticardiolipin syndrome [4A43.Y] Other specified overlap non-organ specific systemic autoimmune disease Also known as: Other specified overlap non-organ specific systemic autoimmune disease \| Antisynthetase syndrome \| Reynolds syndrome \| Syndromic multisystem autoimmune disease due to ITCH deficiency \| Eosinophilia myalgia syndrome [QA47.Z] Liveborn infants according to place of birth, unspecified Also known as: Liveborn infants according to place of birth, unspecified \| Liveborn infants according to place of birth [EE12.1] Onychomycosis Definition: Fungal infection of fingernails and/or toenails due most commonly to dermatophytes (tinea unguium) or yeasts, especially Candida species. Also known as: Onychomycosis \| Fungal infection of the nails \| Onychomycosis classified according to clinical pattern \| Superficial white onychomycosis \| Distal and lateral subungual onychomycosis Includes: Fungal infection of the nails Excludes: Candidosis of nail or paronychium [5C70.0] Dehydration Definition: Dehydration occurs when there is an insufficient amount or excessive loss of water in the body. This can be caused by vomiting, diarrhoea, fever, use of diuretics, profuse sweating, or decreased water intake. Also known as: Dehydration \| fluid depletion \| anhydration \| anhydremia \| fluid volume deficit [9D42.2Z] Peripheral field deficit, unspecified Also known as: Peripheral field deficit, unspecified \| Peripheral field deficit [5B7Z] Unspecified undernutrition Also known as: Unspecified undernutrition \| Malnutrition NOS \| nutritional deficiency NOS \| nutritional depletion NOS \| severe malnutrition NOS === GRAPH WALKS === --- Walk 1 --- [JA86.Y] Maternal care for other specified fetal problems --PARENT--> [JA86] Maternal care for other fetal problems Def: A condition characterised by the provision of health interventions to the mother due to any other issue that is either suspected or known to be present in the fetus.... --CHILD--> [JA86.0] Maternal care for red cell antibodies Def: Maternal care for rhesus or other isoimmunization... --- Walk 2 --- [JA86.Y] Maternal care for other specified fetal problems --PARENT--> [JA86] Maternal care for other fetal problems Def: A condition characterised by the provision of health interventions to the mother due to any other issue that is either suspected or known to be present in the fetus.... --EXCLUDES--> [?] Labour or delivery complicated by fetal distress --- Walk 3 --- [MB23.1] Antisocial behaviour Def: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated.... --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour --CHILD--> [MB23.0] Aggressive behaviour Def: Actions intended to threaten or hurt another person or to damage property that may be physical, verbal, or symbolic (e.g., acting against the other person's interests). Aggressive behaviour may be app... --- Walk 4 --- [MB23.1] Antisocial behaviour Def: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated.... --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour --PARENT--> [?] Mental or behavioural symptoms, signs or clinical findings --- Walk 5 --- [3B4Z] Coagulation defects, unspecified --PARENT--> [?] Coagulation defects --CHILD--> [?] Congenital or constitutional haemorrhagic condition Def: A condition caused by determinants arising during the antenatal period or genetically inherited factors, leading to defects in clotting mechanisms or abnormalities causing structural flaws in the bloo... --- Walk 6 --- [3B4Z] Coagulation defects, unspecified --PARENT--> [?] Coagulation defects --CHILD--> [?] Haemorrhagic diseases due to acquired coagulation factor defects Def: Any disease caused by determinants arising after birth. These diseases are characterised by abnormal coagulation of the blood....	[ "[JA86.Y] Maternal care for other specified fetal problems\n --PARENT--> [JA86] Maternal care for other fetal problems\n Def: A condition characterised by the provision of health interventions to the mother due to any other issue that is either suspected or known to be present in the fetus....\n --CHILD--> [JA86.0] Maternal care for red cell antibodies\n Def: Maternal care for rhesus or other isoimmunization...", "[JA86.Y] Maternal care for other specified fetal problems\n --PARENT--> [JA86] Maternal care for other fetal problems\n Def: A condition characterised by the provision of health interventions to the mother due to any other issue that is either suspected or known to be present in the fetus....\n --EXCLUDES--> [?] Labour or delivery complicated by fetal distress", "[MB23.1] Antisocial behaviour\n Def: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated....\n --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour\n --CHILD--> [MB23.0] Aggressive behaviour\n Def: Actions intended to threaten or hurt another person or to damage property that may be physical, verbal, or symbolic (e.g., acting against the other person's interests). Aggressive behaviour may be app...", "[MB23.1] Antisocial behaviour\n Def: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated....\n --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour\n --PARENT--> [?] Mental or behavioural symptoms, signs or clinical findings", "[3B4Z] Coagulation defects, unspecified\n --PARENT--> [?] Coagulation defects\n --CHILD--> [?] Congenital or constitutional haemorrhagic condition\n Def: A condition caused by determinants arising during the antenatal period or genetically inherited factors, leading to defects in clotting mechanisms or abnormalities causing structural flaws in the bloo...", "[3B4Z] Coagulation defects, unspecified\n --PARENT--> [?] Coagulation defects\n --CHILD--> [?] Haemorrhagic diseases due to acquired coagulation factor defects\n Def: Any disease caused by determinants arising after birth. These diseases are characterised by abnormal coagulation of the blood...." ]	JA86.Y	Maternal care for other specified fetal problems	[ { "from_icd11": "JA86.Y", "icd10_code": "O26841 ", "icd10_title": "" }, { "from_icd11": "JA86.Y", "icd10_code": "O26843 ", "icd10_title": "" }, { "from_icd11": "JA86.Y", "icd10_code": "O26849 ", "icd10_title": "" }, { "from_icd11": "JA86.Y", "icd10_code": "O3680X0 ", "icd10_title": "" }, { "from_icd11": "3B4Z", "icd10_code": "D688", "icd10_title": "Other specified coagulation defects" }, { "from_icd11": "3B4Z", "icd10_code": "D689", "icd10_title": "Coagulation defect, unspecified" }, { "from_icd11": "3B4Z", "icd10_code": "D699", "icd10_title": "Hemorrhagic condition, unspecified" }, { "from_icd11": "3B4Z", "icd10_code": "D698", "icd10_title": "Other specified hemorrhagic conditions" }, { "from_icd11": "3B4Z", "icd10_code": "D65-D69", "icd10_title": "" }, { "from_icd11": "3B4Z", "icd10_code": "D69", "icd10_title": "Purpura and other hemorrhagic conditions" }, { "from_icd11": "4A45.Z", "icd10_code": "D6861", "icd10_title": "Antiphospholipid syndrome" }, { "from_icd11": "4A45.Z", "icd10_code": "D6869", "icd10_title": "Other thrombophilia" }, { "from_icd11": "4A45.Z", "icd10_code": "D6862", "icd10_title": "Lupus anticoagulant syndrome" }, { "from_icd11": "4A45.Z", "icd10_code": "D686", "icd10_title": "Other thrombophilia" }, { "from_icd11": "QA47.Z", "icd10_code": "Z38", "icd10_title": "Liveborn infants according to place of birth and type of delivery" } ]	O26841
A 34-year-old Hispanic/Latino male from a marginalized community suffered a fall from his own height. He reported pain in both hips and impaired gait; due to this, he went to a rural health center, where they identified bilateral hip fractures, consisting of a right subtrochanteric femur fracture and a left pertrochanteric femur fracture . Physical examination showed right inferior limb with internal rotation, shortening of the left inferior limb with external rotation, and sharp sarcopenia in both inferior limbs. The patient presented muscle weakness, so the patient had used crutches to support himself in walking in the previous months. For most of his life, the patient lived in the Tarahumara region (a rural zone) and worked at a sawmill. Currently unemployed because of health issues. His highest educational level was high school. He used to smoke cigarettes (a pack every week since he was 16 years old), consume cocaine occasionally, and drink alcohol (every weekend getting drunk) but stopped consuming all these substances four months before the current illness. His prior clinical history included weight loss of 20 kg in a period of 5 years, chronic musculoskeletal pain, fatigue, debility, progressive elevation of serum creatinine and nitrogen compounds, and chronic elevation in alkaline phosphatase. The patient denied the consumption of any medication. At the presentation moment, the patient weighed 45 kg, his height was 160 cm, his temperature was 36.9 ºC, his blood pressure was 116 over 74 mmHg (mean 88 mmHg), respiratory rate 20 bpm, heart rate 99 bpm, oxygen blood saturation was 94%. The laboratory findings were leucocytes in 7.63 k cel/μL, hemoglobin in 6.3 g/dL with MCV in 105.8 fL, MCH in 33 mg/dL, and platelets 169 k/μL. Serum creatinine was 2.31 mg/dL, urea was 83 mg/dL, protein C reactive was 15.6 mg/L, sodium was 138 mEq/L and potassium was 5.9 mEq/L. He was transferred to our Central Hospital in the state of Chihuahua, Mexico, for his examination and management where laboratory exams were done, finding blood calcium increased, resulting in 13 mg/dL (normal 8.8–10.5 mg/dL), urine calcium was also elevated, resulting in 49.9 mmol (normal 2.5–7.5 mmol), and alkaline phosphatase (AP) was also highly elevated, resulting in 2126 IU/L (normal 44–147 IU/L). From the suspicion of PHPT as a result of the hypercalcemia and the pathological fractures, measurements for PTH levels were performed, resulting in 3000 pg/mL (normal range is 10–55 pg/mL). Levels of 25-hydroxyvitamin D were also measured, resulting in 5.7 ng/mL, thus evidencing a clear deficiency. High flow of intravenous (IV) crystalloid fluids (Hartmann 250 ml/hour) and furosemide (40 mg IV every 8 hours) were initiated in order to diminish the hypercalcemia. Analgesic measures with a tramadol IV infusion (200 mg in 100 ml with an infusion speed of 4 ml/hour) and acetaminophen (1 gr IV 3 times daily) were performed. Prophylaxis for venous thromboembolism was also initiated with enoxaparin (40 mg subcutaneously every 24 hours). Neck palpation was negative for adenopathies or masses. Full-body X-ray series were done in the chest, pelvis, femur, knee, tibia, and fibula, showing radiological signs such as moth-eaten pattern, lytic bone lesions, and general cortical bone thinning . A neck ultrasound was performed, finding two solid heterogeneous masses with perinodal and intranodal vascularity inferior to the thyroid gland; the right mass had dimensions of 31 × 18 × 19 mm, and the left was 38 × 15 × 14 mm. Afterward, a neck CT scan with intravenous contrast was performed to dictate surgical treatment, where two heterogeneous nodules in the territory of the superior parathyroid glands were reported . It was not possible to realize the sestamibi parathyroid scan or bone gammagraphy scan because of limited resources in the hospital. A full-body CT scan was done to assess the presence of other tumors, and it demonstrated no disease in other endocrine glands (pituitary gland, pancreas, thyroid gland, and suprarenal glands). The genetic exam for NEM 1/2 was not available, although familiar history was negative for endocrine disorders. The full-body CT scan showed multiple bone skull lesions with rounded and heterogeneous aspects, apparently compatible with lytic lesions . The 3D reconstruction evidenced numerous lytic lesions in multiple bones, numerous pancreatic calcifications, and a right staghorn calculus . An electrocardiogram showed left ventricular hypertrophy (LVH) using Sokolow-Lyon criteria, indicating heart affection secondary to the PHPT. Surgical treatment was the therapeutic decision, with parathyroid removal and an intraoperative histopathological study of both pieces . Prior to surgery, calcitriol prophylaxis was initiated (0.5 mcg orally 3 times daily for 3 days), and calcium carbonate (500 mg orally per day) During surgery, the presence of a tumor in the right inferior parathyroid gland was identified and resected; the dimensions were 3.9 × 2.5 × 2 cm and 9 gr . A left inferior parathyroid gland resection was performed, and the dimensions were 3.8 × 2.3 × 2 cm and 7 gr . The intraoperative histopathologic study reported both pieces as parathyroid adenomas without any features of malignancy, yet parathyroid hyperplasia must be discarded. The definitive study results were the following: The right resected parathyroid gland was reported as a parathyroid adenoma, and the left resected parathyroid gland was reported as a parathyroid adenoma, hence, the existence of a bilateral parathyroid adenoma was concluded. Immediately after the procedure, calcium levels in serum were 9.4 mg/dL, and 2 days later, the calcium levels descended to 8.7 mg/dL, both results being corrected for hypoalbuminemia. In the following days, calcium in serum levels continued descending, so a calcium gluconate infusion had to be started, and the calcitriol dose was also increased, Concomitantly, phosphorus, and magnesium blood levels also went down. The patient was discharged 23 days after this surgery when serum electrolytes were stabilized, and the calcium gluconate infusion was withdrawn. Ambulatory treatment was prescribed with calcitriol 0.5 mcg every 3 hours orally, calcium carbonate 1 gram every 6 hours orally, and anticoagulation prophylaxis with rivaroxaban 10 mg every 24 hours. In the same admission, hip, and femur fracture correction was performed through closed reduction-internal fixation under the biomechanical principle of support with a cephalomedullary nail.	3.953125	0.979004	sec[1]/p[0]	en	0.999997	37653552	https://doi.org/10.1186/s13256-023-04102-w	[ "parathyroid", "calcium", "every", "gland", "both", "serum", "resulting", "hours", "scan", "femur" ]	[ { "code": "5A5Z", "title": "Disorders of the parathyroids or parathyroid hormone system, unspecified" }, { "code": "5A51.0", "title": "Primary hyperparathyroidism" }, { "code": "5A50.Z", "title": "Hypoparathyroidism, unspecified" }, { "code": "5D42", "title": "Postprocedural hypoparathyroidism" }, { "code": "NA21.0&XA1342", "title": "Laceration of parathyroid gland" }, { "code": "5B5K.1Z", "title": "Calcium deficiency, unspecified" }, { "code": "5B91.0", "title": "Hypercalcaemia" }, { "code": "5B5K.1Y", "title": "Other specified calcium deficiency" }, { "code": "5C64.5", "title": "Disorders of calcium metabolism" }, { "code": "FB40.Y", "title": "Other specified tenosynovitis" } ]	=== ICD-11 CODES FOUND === [5A5Z] Disorders of the parathyroids or parathyroid hormone system, unspecified Also known as: Disorders of the parathyroids or parathyroid hormone system, unspecified \| parathyroidal disturbance \| disorder of parathyroid gland \| parathyroid gland disease \| parathyroid disease [5A51.0] Primary hyperparathyroidism Definition: Primary hyperparathyroidism is a condition with enhanced PTH secretion and high circulatory PTH level caused by abnormal parathyroid pathology such as adenoma, hyperplasia and cancer. Primary hyperparathyroidism usually causes hypercalcaemia by enhanced PTH actions. Also known as: Primary hyperparathyroidism \| parathyroid enlargement \| Familial primary hyperparathyroidism \| Hereditary primary hyperparathyroidism \| Familial isolated hyperparathyroidism [5A50.Z] Hypoparathyroidism, unspecified Also known as: Hypoparathyroidism, unspecified \| Hypoparathyroidism \| deficiency of parathyroid hormone \| parathyroid gland insufficiency \| parathyroid insufficiency [5D42] Postprocedural hypoparathyroidism Definition: This refers to a postprocedural decreased function of the parathyroid glands with underproduction of parathyroid hormone. This can lead to low levels of calcium in the blood, often causing cramping and twitching of muscles or tetany (involuntary muscle contraction), and several other symptoms. Also known as: Postprocedural hypoparathyroidism \| absence of parathyroid gland \| postoperative tetany \| Parathyroprival tetany Includes: Parathyroprival tetany [5B5K.1Z] Calcium deficiency, unspecified Also known as: Calcium deficiency, unspecified \| Calcium deficiency \| hypocalcaemia NOS \| disturbance of calcium absorption \| disorder of calcium absorption [5B91.0] Hypercalcaemia Definition: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused by dehydration secondary to urinary losses of calcium, water and other electrolytes, and to an increase in membrane potential caused by the elevation in extracellular fluid ionized calcium concentration. Patients with moderate to severe hypercalcaemia often complain of nausea and vomiting, symptoms Also known as: Hypercalcaemia \| Calcium excess \| elevated serum calcium \| hypercalcaemic crisis \| hypercalcaemic syndrome [5B5K.1Y] Other specified calcium deficiency Also known as: Other specified calcium deficiency \| Dietary hypocalcaemia \| dietary calcium deficiency [5C64.5] Disorders of calcium metabolism Definition: This refers to disorders in the mechanism by which the body maintains adequate calcium levels. Derangements of this mechanism lead to hypercalcaemia or hypocalcaemia, both of which can have important consequences for health. Also known as: Disorders of calcium metabolism \| Calcinosis \| general calcification \| heterotopic calcification \| metastatic calcification Excludes: Hyperparathyroidism \| Chondrocalcinosis [FB40.Y] Other specified tenosynovitis Also known as: Other specified tenosynovitis \| Other tenosynovitis or tendinitis \| synovitis NOS \| Bicipital tendinitis \| biceps tendinitis === GRAPH WALKS === --- Walk 1 --- [5A5Z] Disorders of the parathyroids or parathyroid hormone system, unspecified --PARENT--> [?] Disorders of the parathyroids or parathyroid hormone system Def: Disorders of the parathyroids and parathyroid hormone system generally refer to conditions with inappropriate secretion and/or actions of parathyroid hormone that cause dysregulation of calcium metabo... --EXCLUDES--> [?] Hypocalcaemic vitamin D dependent rickets Def: Hypocalcaemic vitamin D-dependent rickets (VDDR-I) is an early-onset hereditary vitamin D metabolism disorder characterised by severe hypocalcaemia leading to osteomalacia and rachitic bone deformatio... --- Walk 2 --- [5A5Z] Disorders of the parathyroids or parathyroid hormone system, unspecified --PARENT--> [?] Disorders of the parathyroids or parathyroid hormone system Def: Disorders of the parathyroids and parathyroid hormone system generally refer to conditions with inappropriate secretion and/or actions of parathyroid hormone that cause dysregulation of calcium metabo... --EXCLUDES--> [?] Hypocalcaemic vitamin D dependent rickets Def: Hypocalcaemic vitamin D-dependent rickets (VDDR-I) is an early-onset hereditary vitamin D metabolism disorder characterised by severe hypocalcaemia leading to osteomalacia and rachitic bone deformatio... --- Walk 3 --- [5A51.0] Primary hyperparathyroidism Def: Primary hyperparathyroidism is a condition with enhanced PTH secretion and high circulatory PTH level caused by abnormal parathyroid pathology such as adenoma, hyperplasia and cancer. Primary hyperpar... --PARENT--> [5A51] Hyperparathyroidism Def: Hyperparathyroidism refers to overproduction of parathormone and is most frequently due to a tumour in one of the parathyroid glands. It may also occur in response to low calcium levels, as encountere... --EXCLUDES--> [?] Vitamin D deficiency rickets Def: Rickets is a disease of growing bone that is due to unmineralized matrix at the growth plates and occurs in children only before fusion of the epiphyses. There are many causes of rickets, including vi... --- Walk 4 --- [5A51.0] Primary hyperparathyroidism Def: Primary hyperparathyroidism is a condition with enhanced PTH secretion and high circulatory PTH level caused by abnormal parathyroid pathology such as adenoma, hyperplasia and cancer. Primary hyperpar... --PARENT--> [5A51] Hyperparathyroidism Def: Hyperparathyroidism refers to overproduction of parathormone and is most frequently due to a tumour in one of the parathyroid glands. It may also occur in response to low calcium levels, as encountere... --EXCLUDES--> [?] Vitamin D deficiency rickets Def: Rickets is a disease of growing bone that is due to unmineralized matrix at the growth plates and occurs in children only before fusion of the epiphyses. There are many causes of rickets, including vi... --- Walk 5 --- [5A50.Z] Hypoparathyroidism, unspecified --PARENT--> [5A50] Hypoparathyroidism Def: Hypoparathyroidism is a condition with insufficient biological actions of parathyroid hormone due to impaired secretion of parathyroid hormone or refractoriness of target tissues to parathyroid hormon... --EXCLUDES--> [?] Postprocedural hypoparathyroidism Def: This refers to a postprocedural decreased function of the parathyroid glands with underproduction of parathyroid hormone. This can lead to low levels of calcium in the blood, often causing cramping an... --- Walk 6 --- [5A50.Z] Hypoparathyroidism, unspecified --PARENT--> [5A50] Hypoparathyroidism Def: Hypoparathyroidism is a condition with insufficient biological actions of parathyroid hormone due to impaired secretion of parathyroid hormone or refractoriness of target tissues to parathyroid hormon... --CHILD--> [5A50.Y] Other specified hypoparathyroidism	[ "[5A5Z] Disorders of the parathyroids or parathyroid hormone system, unspecified\n --PARENT--> [?] Disorders of the parathyroids or parathyroid hormone system\n Def: Disorders of the parathyroids and parathyroid hormone system generally refer to conditions with inappropriate secretion and/or actions of parathyroid hormone that cause dysregulation of calcium metabo...\n --EXCLUDES--> [?] Hypocalcaemic vitamin D dependent rickets\n Def: Hypocalcaemic vitamin D-dependent rickets (VDDR-I) is an early-onset hereditary vitamin D metabolism disorder characterised by severe hypocalcaemia leading to osteomalacia and rachitic bone deformatio...", "[5A5Z] Disorders of the parathyroids or parathyroid hormone system, unspecified\n --PARENT--> [?] Disorders of the parathyroids or parathyroid hormone system\n Def: Disorders of the parathyroids and parathyroid hormone system generally refer to conditions with inappropriate secretion and/or actions of parathyroid hormone that cause dysregulation of calcium metabo...\n --EXCLUDES--> [?] Hypocalcaemic vitamin D dependent rickets\n Def: Hypocalcaemic vitamin D-dependent rickets (VDDR-I) is an early-onset hereditary vitamin D metabolism disorder characterised by severe hypocalcaemia leading to osteomalacia and rachitic bone deformatio...", "[5A51.0] Primary hyperparathyroidism\n Def: Primary hyperparathyroidism is a condition with enhanced PTH secretion and high circulatory PTH level caused by abnormal parathyroid pathology such as adenoma, hyperplasia and cancer. Primary hyperpar...\n --PARENT--> [5A51] Hyperparathyroidism\n Def: Hyperparathyroidism refers to overproduction of parathormone and is most frequently due to a tumour in one of the parathyroid glands. It may also occur in response to low calcium levels, as encountere...\n --EXCLUDES--> [?] Vitamin D deficiency rickets\n Def: Rickets is a disease of growing bone that is due to unmineralized matrix at the growth plates and occurs in children only before fusion of the epiphyses. There are many causes of rickets, including vi...", "[5A51.0] Primary hyperparathyroidism\n Def: Primary hyperparathyroidism is a condition with enhanced PTH secretion and high circulatory PTH level caused by abnormal parathyroid pathology such as adenoma, hyperplasia and cancer. Primary hyperpar...\n --PARENT--> [5A51] Hyperparathyroidism\n Def: Hyperparathyroidism refers to overproduction of parathormone and is most frequently due to a tumour in one of the parathyroid glands. It may also occur in response to low calcium levels, as encountere...\n --EXCLUDES--> [?] Vitamin D deficiency rickets\n Def: Rickets is a disease of growing bone that is due to unmineralized matrix at the growth plates and occurs in children only before fusion of the epiphyses. There are many causes of rickets, including vi...", "[5A50.Z] Hypoparathyroidism, unspecified\n --PARENT--> [5A50] Hypoparathyroidism\n Def: Hypoparathyroidism is a condition with insufficient biological actions of parathyroid hormone due to impaired secretion of parathyroid hormone or refractoriness of target tissues to parathyroid hormon...\n --EXCLUDES--> [?] Postprocedural hypoparathyroidism\n Def: This refers to a postprocedural decreased function of the parathyroid glands with underproduction of parathyroid hormone. This can lead to low levels of calcium in the blood, often causing cramping an...", "[5A50.Z] Hypoparathyroidism, unspecified\n --PARENT--> [5A50] Hypoparathyroidism\n Def: Hypoparathyroidism is a condition with insufficient biological actions of parathyroid hormone due to impaired secretion of parathyroid hormone or refractoriness of target tissues to parathyroid hormon...\n --CHILD--> [5A50.Y] Other specified hypoparathyroidism" ]	5A5Z	Disorders of the parathyroids or parathyroid hormone system, unspecified	[ { "from_icd11": "5A5Z", "icd10_code": "E213", "icd10_title": "Hyperparathyroidism, unspecified" }, { "from_icd11": "5A5Z", "icd10_code": "E212", "icd10_title": "Other hyperparathyroidism" }, { "from_icd11": "5A5Z", "icd10_code": "E214", "icd10_title": "Other specified disorders of parathyroid gland" }, { "from_icd11": "5A5Z", "icd10_code": "E215", "icd10_title": "Disorder of parathyroid gland, unspecified" }, { "from_icd11": "5A5Z", "icd10_code": "E21", "icd10_title": "Hyperparathyroidism and other disorders of parathyroid gland" }, { "from_icd11": "5A51.0", "icd10_code": "E210", "icd10_title": "Primary hyperparathyroidism" }, { "from_icd11": "5A50.Z", "icd10_code": "E209", "icd10_title": "Hypoparathyroidism, unspecified" }, { "from_icd11": "5A50.Z", "icd10_code": "E208", "icd10_title": "Other hypoparathyroidism" }, { "from_icd11": "5A50.Z", "icd10_code": "E20", "icd10_title": "Hypoparathyroidism" }, { "from_icd11": "5D42", "icd10_code": "E892", "icd10_title": "Postprocedural hypoparathyroidism" }, { "from_icd11": "5B5K.1Z", "icd10_code": "E58", "icd10_title": "Dietary calcium deficiency" }, { "from_icd11": "5C64.5", "icd10_code": "E8352", "icd10_title": "Hypercalcemia" }, { "from_icd11": "5C64.5", "icd10_code": "E8351", "icd10_title": "Hypocalcemia" }, { "from_icd11": "5C64.5", "icd10_code": "E8359", "icd10_title": "Other disorders of calcium metabolism" }, { "from_icd11": "5C64.5", "icd10_code": "E8350", "icd10_title": "Unspecified disorder of calcium metabolism" } ]	E213	Hyperparathyroidism, unspecified
A 60-year-old woman with a history of hypertension, obesity (body mass index, 41 kg/m 2 ) had undergone a routine abdominal CT imaging study for evaluation of hematuria and lower back pain. CT demonstrated a 1.7-cm × 1.1-cm pancreaticoduodenal artery aneurysm with concomitant celiac occlusion . The patient was taken to a hybrid operating room equipped with a robotic angiography system (Artis Pheno VE10B; Siemens Healthineers, Erlangen, Germany) for diagnostic angiography and possible endovascular treatment under general anesthesia. After ultrasound-guided right femoral arterial access, a 5F introducer sheath (Pinnacle, Terumo Medical Corp, Tokyo, Japan) was inserted. The superior mesenteric artery (SMA) was cannulated using a Simmons-1 catheter (Merit Medical, Salt Lake City, UT). To better understand the 3D aneurysm morphology, CBCTA was performed using a 4s digital subtraction angiography protocol after selective contrast injection into the SMA (6 mL/s; 30 mL of contrast diluted with 50% saline, with a 1-second x-ray delay). 3D images were reconstructed in both subtracted and native fill modes and visualized as multiplanar and volume-rendered reconstructions . Next, a 6.5F steerable sheath (Aptus TourGuide; Medtronic, Dublin, Ireland) was exchanged to provide better steerability and stability. After assessing the aneurysm and collateral circulation using CBCTA ( Supplementary Video 2 ), coil embolization of the aneurysm sac, with simultaneous deployment of a braided stent, was performed to assist coil embolization and reconstruct the parent vessel. The C-arm working projection that provided optimal visualization of the proximal and distal parent vessels with minimal overlap was selected from the CBCTA imaging study. 3D segmentation of the aneurysm and ostia of the proximal and distal parent vessels from CBCTA was overlaid on the fluoroscopic images for image guidance . The Navien (Medtronic) intracranial support catheter was advanced into the pancreaticoduodenal artery (PDA) to provide support for stent delivery and the microcatheter for coil embolization. Two parallel wires were used to cannulate the aneurysm sac and distal parent vessel using a double Tuohy-Borst adapter system. Two microcatheters, Headway-21 microcatheter (MicroVention Terumo) and Excelsior SL-10 (Stryker Neurovascular, Fremont, CA), were advanced over the wire. The dual microcatheter technique was adopted to facilitate the controlled delivery of the LVIS stent across the aneurysm neck and to perform coil embolization of the aneurysm sac safely without protruding into the parent vessel lumen. Coil embolization of the aneurysm sac was performed using framing coils (6-mm × 19-cm HydroFrame; MicroVention Terumo), followed by filling coils (7-mm × 20-cm HydroFil; Microvention Terumo) and microcoils . Next, the braided coil-assisted stent (4.5-mm × 32-mm LVIS device, Microvention Terumo) was deployed in the pancreaticoduodenal artery using road mapping and image guidance . After deployment, stent foreshortening was not observed. 2D angiography confirmed aneurysm exclusion and a patent pancreaticoduodenal arcade . Follow-up CBCTA was performed, which demonstrated a well-apposed stent in the PDA and no residual flow in the aneurysm sac . The mean radiation dose from the CBCTA scans was 123.5 mGy and accounted for 22.7% of the total procedural dose . The total procedural time, from vascular access to closure, was ∼180 minutes, with a total fluoroscopy time of 40.6 minutes. Fig 1 Axial (A) , coronal (B) , and volume rendered (C) reconstructions of preoperative computed tomography angiography (CTA) images demonstrating a 1.7- × 1.1-cm aneurysm involving the anterior pancreaticoduodenal artery (PDA; yellow arrow ). Fig 2 A, Selective arteriogram of superior mesenteric artery (SMA; black arrow ) with an aneurysm involving the anterior pancreaticoduodenal arcade ( yellow arrow ). B, Volume rendered reconstruction of cone-beam computed tomography angiography (CBCTA) showing the pancreaticoduodenal arcade, three-dimensional (3D) aneurysm morphology, and inflow and outflow vessels. C and D, 3D planning of aneurysm for embolization with ostia of inflow vessels ( green centerline ) from SMA and outflow vessels ( orange centerline ) electronically marked in two oblique views. Fig 3 A, Three-dimensional (3D) volume rendered reconstruction of cone-beam computed tomography angiography (CBCTA) with ostia and centerlines of inflow and outflow parent vessels of the aneurysm marked electronically ( green circle and green centerline and orange circle and orange centerline , respectively). The optimal C-arm working projection of right anterior oblique ( RAO ) 15°/cranial ( CRAN ) 7° angles was selected based on the CBCTA images and 3D annotated vascular landmarks. B, Two-dimensional (2D) angiographic image of the aneurysm acquired at the optimized working projection, demonstrating inflow from the superior mesenteric artery (SMA) and outflow from the aneurysm sac. C, Snapshot of CBCTA-fluoroscopy image fusion guidance during coil embolization showing overlay of vascular landmarks such as the origin of the SMA ( white arrow ), origin/centerline of inflow vessel ( green ), leading into the aneurysm (marked in yellow outline and indicated by yellow arrow ). Fig 4 A, Deployment of low-profile visualized intraluminal support (LVIS) flow-diverter stent with two-dimensional (2D) road mapping and cone-beam computed tomography angiography (CBCTA) image fusion guidance. Planned proximal and distal landing zones were electronically marked on the CBCTA images ( blue rings and blue centerline ), overlaid on 2D fluoroscopy and roadmap images. B, The proximal ( black arrowhead ) and distal ( white arrowhead ) markers of the flow-diverting stent were positioned using information from CBCTA and deployed. Completion 2D angiography (C) and CBCTA (D) images demonstrating near complete occlusion of aneurysm sac with coil embolization and well-apposed flow-diverting stent in proximal ( black arrowhead ) and distal ( white arrowhead ) parent vessels. Fig 5 Comparison of three-dimensional (3D) volume rendered images of completion cone-beam computed tomography angiography (CBCTA) showing coils and low-profile visualized intraluminal support (LVIS) flow-diverter stent (A) and follow-up CTA (B) images showing patent pancreaticoduodenal arterial arcade with no aneurysmal contrast opacification (B) . Proximal markers of LVIS flow-diverting stent were electronically marked with a black arrowhead .	4.109375	0.925293	sec[0]/p[0]	en	0.999994	35586675	https://doi.org/10.1016/j.jvscit.2022.03.010	[ "aneurysm", "cbcta", "stent", "angiography", "pancreaticoduodenal", "coil", "embolization", "artery", "using", "parent" ]	[ { "code": "BD51.Z", "title": "Aneurysm and dissection of unspecified artery" }, { "code": "BD75.Y", "title": "Venous varicosities of other specified sites" }, { "code": "BA81", "title": "Coronary artery aneurysm" }, { "code": "BB02.1Z", "title": "Aneurysm of pulmonary artery, unspecified" }, { "code": "BD51.4", "title": "Aneurysm or dissection of renal artery" }, { "code": "PK93.2", "title": "Gastroenterology or urology devices associated with injury or harm, prosthetic or other implants, materials or accessory devices" }, { "code": "QB51.1", "title": "Presence of urogenital implants" }, { "code": "QB51.Y", "title": "Presence of other specified devices other than cardiac or vascular implants" }, { "code": "QB51.3", "title": "Presence of otological or audiological implants" }, { "code": "PL12.3", "title": "Obstruction of device, as mode of injury or harm" } ]	=== ICD-11 CODES FOUND === [BD51.Z] Aneurysm and dissection of unspecified artery Also known as: Aneurysm and dissection of unspecified artery \| Arterial aneurysm or dissection, excluding aorta \| cirsoid aneurysm NOS \| false aneurysm NOS \| ruptured aneurysm NOS [BD75.Y] Venous varicosities of other specified sites Also known as: Venous varicosities of other specified sites \| Caput medusae \| Jugular venous aneurysm \| jugular vein aneurysm \| Orbital varices [BA81] Coronary artery aneurysm Definition: Coronary dilatation which exceeds the diameter of normal adjacent segments or the diameter of the patient's largest coronary vessel by 1.5 times. Also known as: Coronary artery aneurysm \| aneurysm of coronary vessels \| aneurysmal lesion of coronary artery \| arteriovenous aneurysm of coronary vessels \| coronary aneurysm Excludes: Congenital coronary arterial aneurysm \| Mucocutaneous lymph node syndrome [BB02.1Z] Aneurysm of pulmonary artery, unspecified Also known as: Aneurysm of pulmonary artery, unspecified \| Aneurysm of pulmonary artery \| pulmonary artery aneurysm \| PA - [pulmonary artery aneurysm] \| pulmonary aneurysm [BD51.4] Aneurysm or dissection of renal artery Also known as: Aneurysm or dissection of renal artery \| aneurysm of renal artery \| renal artery aneurysm \| renal aneurysm [PK93.2] Gastroenterology or urology devices associated with injury or harm, prosthetic or other implants, materials or accessory devices Also known as: Gastroenterology or urology devices associated with injury or harm, prosthetic or other implants, materials or accessory devices \| Surgical operation with implant of artificial internal gastroenterology or urology device associated with abnormal reaction of the patient, or of later complication, without mention of misadventure at the time of the procedure \| Surgical operation with gastroenterological or urological bypass or graft associated with abnormal reaction of the patient, or of later complication, without mention of misadventure at the time of the procedure \| Gastroenterology or urology devices associated with injury or harm, urethral or ureteral stents \| Incrustation or calcification of indwelling ureteral stents Excludes: Circumstances associated with a surgical or other medical device influencing the episode of care without injury or harm [QB51.1] Presence of urogenital implants Also known as: Presence of urogenital implants \| Presence of bladder implant \| bladder replaced by other means \| replacement of bladder by artificial or mechanical device or prosthesis \| Presence of urethral stent [QB51.Y] Presence of other specified devices other than cardiac or vascular implants Also known as: Presence of other specified devices other than cardiac or vascular implants \| Presence of bone or tendon implants other than orthopaedic joint implants \| replacement of tendon by artificial or mechanical device or prosthesis \| presence of tendon implant \| Presence of skull plate [QB51.3] Presence of otological or audiological implants Also known as: Presence of otological or audiological implants \| presence of audiological implant \| presence of hearing device implant \| presence of hearing-aid implant \| presence of otological implant [PL12.3] Obstruction of device, as mode of injury or harm Definition: Obstruction associated with prosthetic devices, grafts or implants Also known as: Obstruction of device, as mode of injury or harm \| occlusion shunt \| blockage of device causing obstruction as mode of injury \| blocked tube causing obstruction as mode of injury \| occlusion of device causing obstruction as mode of injury Excludes: Obstruction of device without injury or harm === GRAPH WALKS === --- Walk 1 --- [BD51.Z] Aneurysm and dissection of unspecified artery --PARENT--> [BD51] Arterial aneurysm or dissection, excluding aorta --EXCLUDES--> [?] Acute myocardial infarction, without specification of ST elevation Def: This is commonly known as a heart attack, results from the interruption of blood supply to a part of the heart, causing heart cells to die. This is most commonly due to occlusion (blockage) of a coron... --- Walk 2 --- [BD51.Z] Aneurysm and dissection of unspecified artery --PARENT--> [BD51] Arterial aneurysm or dissection, excluding aorta --EXCLUDES--> [?] Acute myocardial infarction, without specification of ST elevation Def: This is commonly known as a heart attack, results from the interruption of blood supply to a part of the heart, causing heart cells to die. This is most commonly due to occlusion (blockage) of a coron... --- Walk 3 --- [BD75.Y] Venous varicosities of other specified sites --PARENT--> [BD75] Venous varicosities of sites other than lower extremity --CHILD--> [BD75.2] Vulval varices Def: Congested and dilated vulval veins, occurring particularly in association with pregnancy.... --- Walk 4 --- [BD75.Y] Venous varicosities of other specified sites --PARENT--> [BD75] Venous varicosities of sites other than lower extremity --CHILD--> [BD75.1] Scrotal varices --- Walk 5 --- [BA81] Coronary artery aneurysm Def: Coronary dilatation which exceeds the diameter of normal adjacent segments or the diameter of the patient's largest coronary vessel by 1.5 times.... --EXCLUDES--> [?] Congenital coronary arterial aneurysm Def: A congenital cardiovascular malformation in which there is one or more localized dilation(s) of a coronary vessel. Additional information: coronary artery aneurysms are usually seen in two forms, sa... --PARENT--> [?] Congenital anomaly of coronary artery Def: A congenital cardiovascular malformation of a coronary artery. This includes absence of a coronary, anomalous origin or course, dilation or stenosis, and fistulas. Additional information: congenital ... --- Walk 6 --- [BA81] Coronary artery aneurysm Def: Coronary dilatation which exceeds the diameter of normal adjacent segments or the diameter of the patient's largest coronary vessel by 1.5 times.... --EXCLUDES--> [?] Congenital coronary arterial aneurysm Def: A congenital cardiovascular malformation in which there is one or more localized dilation(s) of a coronary vessel. Additional information: coronary artery aneurysms are usually seen in two forms, sa... --PARENT--> [?] Congenital anomaly of coronary artery Def: A congenital cardiovascular malformation of a coronary artery. This includes absence of a coronary, anomalous origin or course, dilation or stenosis, and fistulas. Additional information: congenital ...	[ "[BD51.Z] Aneurysm and dissection of unspecified artery\n --PARENT--> [BD51] Arterial aneurysm or dissection, excluding aorta\n --EXCLUDES--> [?] Acute myocardial infarction, without specification of ST elevation\n Def: This is commonly known as a heart attack, results from the interruption of blood supply to a part of the heart, causing heart cells to die. This is most commonly due to occlusion (blockage) of a coron...", "[BD51.Z] Aneurysm and dissection of unspecified artery\n --PARENT--> [BD51] Arterial aneurysm or dissection, excluding aorta\n --EXCLUDES--> [?] Acute myocardial infarction, without specification of ST elevation\n Def: This is commonly known as a heart attack, results from the interruption of blood supply to a part of the heart, causing heart cells to die. This is most commonly due to occlusion (blockage) of a coron...", "[BD75.Y] Venous varicosities of other specified sites\n --PARENT--> [BD75] Venous varicosities of sites other than lower extremity\n --CHILD--> [BD75.2] Vulval varices\n Def: Congested and dilated vulval veins, occurring particularly in association with pregnancy....", "[BD75.Y] Venous varicosities of other specified sites\n --PARENT--> [BD75] Venous varicosities of sites other than lower extremity\n --CHILD--> [BD75.1] Scrotal varices", "[BA81] Coronary artery aneurysm\n Def: Coronary dilatation which exceeds the diameter of normal adjacent segments or the diameter of the patient's largest coronary vessel by 1.5 times....\n --EXCLUDES--> [?] Congenital coronary arterial aneurysm\n Def: A congenital cardiovascular malformation in which there is one or more localized dilation(s) of a coronary vessel. \n\nAdditional information: coronary artery aneurysms are usually seen in two forms, sa...\n --PARENT--> [?] Congenital anomaly of coronary artery\n Def: A congenital cardiovascular malformation of a coronary artery. This includes absence of a coronary, anomalous origin or course, dilation or stenosis, and fistulas.\n\nAdditional information: congenital ...", "[BA81] Coronary artery aneurysm\n Def: Coronary dilatation which exceeds the diameter of normal adjacent segments or the diameter of the patient's largest coronary vessel by 1.5 times....\n --EXCLUDES--> [?] Congenital coronary arterial aneurysm\n Def: A congenital cardiovascular malformation in which there is one or more localized dilation(s) of a coronary vessel. \n\nAdditional information: coronary artery aneurysms are usually seen in two forms, sa...\n --PARENT--> [?] Congenital anomaly of coronary artery\n Def: A congenital cardiovascular malformation of a coronary artery. This includes absence of a coronary, anomalous origin or course, dilation or stenosis, and fistulas.\n\nAdditional information: congenital ..." ]	BD51.Z	Aneurysm and dissection of unspecified artery	[ { "from_icd11": "BD51.Z", "icd10_code": "I728", "icd10_title": "Aneurysm of other specified arteries" }, { "from_icd11": "BD51.Z", "icd10_code": "I729", "icd10_title": "Aneurysm of unspecified site" }, { "from_icd11": "BD51.Z", "icd10_code": "I72", "icd10_title": "Other aneurysm" }, { "from_icd11": "BA81", "icd10_code": "I2542", "icd10_title": "Coronary artery dissection" }, { "from_icd11": "BA81", "icd10_code": "I2541", "icd10_title": "Coronary artery aneurysm" }, { "from_icd11": "BA81", "icd10_code": "I254", "icd10_title": "Coronary artery aneurysm and dissection" }, { "from_icd11": "BB02.1Z", "icd10_code": "I281", "icd10_title": "Aneurysm of pulmonary artery" }, { "from_icd11": "BD51.4", "icd10_code": "I722", "icd10_title": "Aneurysm of renal artery" }, { "from_icd11": "PK93.2", "icd10_code": "T83711A", "icd10_title": "Erosion of implanted vaginal mesh to surrounding organ or tissue, initial encounter" }, { "from_icd11": "PK93.2", "icd10_code": "T83718A", "icd10_title": "Erosion of other implanted mesh to organ or tissue, initial encounter" }, { "from_icd11": "PK93.2", "icd10_code": "T83728A", "icd10_title": "Exposure of other implanted mesh into organ or tissue, initial encounter" }, { "from_icd11": "PK93.2", "icd10_code": "T83719A", "icd10_title": "Erosion of other prosthetic materials to surrounding organ or tissue, initial encounter" }, { "from_icd11": "PK93.2", "icd10_code": "T83718S", "icd10_title": "Erosion of other implanted mesh to organ or tissue, sequela" }, { "from_icd11": "PK93.2", "icd10_code": "T83712A", "icd10_title": "Erosion of implanted urethral mesh to surrounding organ or tissue, initial encounter" }, { "from_icd11": "PK93.2", "icd10_code": "T83721A", "icd10_title": "Exposure of implanted vaginal mesh into vagina, initial encounter" } ]	I728	Aneurysm of other specified arteries
A 66-year-old female patient with known arterial hypertension and diaphragmatic hernia was evaluated for palpitations, persistent typical and atypical chest pain which has been present for 3 years. In 2008, the patient underwent stenting of the Cx for a significant lesion. Physical examination was unremarkable except for a body mass index of 27 kg/m 2 . A 12-lead ECG was normal and the results of a bicycle exercise tolerance test were equivocal. Ambulatory ECG recording depicted normal rhythm variations. Echocardiography showed normokinetic biventricular function and normal valvular function with an ejection fraction of 60 %. The myocardial perfusion test showed a fixed perfusion defect in the inferior and apical region without reversibility . Cardiac MRI imaging was normal without evidence of delayed enhancement after gadolinium. She had neither haemoptysis nor a pulmonary disorder. Conventional CAG demonstrated a patent stent in the Cx without other abnormalities. The myocardial fractional flow reserve for the right coronary artery (RCA) was 0.94, proximal left (PL) Cx-II 0.92 and PLCx-I 0.86. A suspicion was raised suggesting a fistula between the proximal part of the RCA and the right bronchial artery . MDCT documented a fistulous connection between the proximal branch of the RCA and the right bronchial artery. Fig. 1 a Myocardial perfusion imaging (MPI) demonstrating the irreversible defect of the inferior segment, b Coronary angiographic frame of RCA demonstrating a CBF between a proximal branch of RCA and bronchial artery before coiling ( arrow ), c Normal findings on the rest 13 N-ammonia polar map (left panel) and a large absolute perfusion defect ( dark blue ) in the inferior wall after pharmacological stress with adenosine ( right panel ) and d Coronary angiographic frame of RCA demonstrating disappearance of the CBF between a proximal branch of RCA and bronchial artery after coiling ( arrow ) Gated adenosine stress/rest 13 N-ammonia PET/CT visually demonstrated an apical left ventricular (LV) defect at rest which increased during adenosine stress reaching the basal inferior and inferolateral regions . The global stress/rest ratio was 0.74 with a high resting flow. The regional stress/rest ratio was LAD 0.81, RCA 0.41 and Cx 0.85. Blood flow through the left anterior descending artery (LAD) and Cx arteries was twofold higher than through the RCA. The RCA was the fistula donor vessel visible on angiography. Normal LV function without stunning was noticed on gated PET. A severe decrease of the segmental perfusion reserve was detected in the basal inferior and basal inferolateral areas. Based on the findings of PET-CT scanning, evidence was delivered for decision-making to perform an intervention. Percutaneous occlusion of the fistulous vessel was achieved with the application of 4 coils. After coiling of the CBF , the procedure was complicated by distal embolisation to the RCA and right ventricular (RV) branch by a thrombus formed at the tip of the micro-perfusion catheter, through which the coils were placed in the fistula, which dislodged on retrieval of the micro-catheter at the end of the procedure. This gave rise to chest pain, ST elevation in the inferior leads, intermittent second-degree AV block and cardiogenic shock. She was treated with 7500 IU of intravenous heparin, and an intracoronary bolus of abciximab accompanied by thrombosuction. The flow in the RCA was restored with a thrombolysis in myocardial infarction (TIMI) flow score of 3. Her haemodynamic condition stabilised further following the administration of atropine 1 mg intravenously and fluid expansion. After full recovery, follow-up trans-thoracic echocardiography (TTE) revealed normal LV systolic function without wall motion disturbances. During follow-up (now over 2 years) she has remained free from chest pain while treated with acetylsalicylic acid, omeprazole and perindopril. A 74-year-old male patient with hypertension, chronic obstructive pulmonary disease and bronchiectasis was evaluated because of angina pectoris due to subacute anterior wall MI. The clinical examination was unremarkable except for a blood pressure of 90/54 mmHg and pulse rate of 92 beats/min. The ECG on admission showed a normal regular sinus rhythm at 94 beats/min, QS in V1-V3 and persistent ST elevation in V1-V6 without reciprocal depression compatible with a semi-recent anterior wall myocardial infarction. The chest X-ray revealed bilateral basal bronchiectasis . Echocardiography revealed akinesia of the anterior segment with moderate LV kinetics and without significant valvular dysfunction. Fig. 2 a Chest X-ray demonstrating bronchiectasis at the right and left lower regions of the lung ( arrows ), b Coronary angiographic frame of RCA in AP position, depicting the CBF between a proximal branch of the RCA and bronchial artery ( arrow ) and c Thoracic computed tomography scan showing the bilateral bronchiectasis Conventional CAG demonstrated one-vessel disease with a subtotal stenosis in the proximal LAD and fistulous multiple tiny vessels exiting from a proximal branch of the small calibre RCA with a possible connection to the right pulmonary artery and right bronchial artery . At cardiac catheterisation mild pulmonary hypertension (capillary wedge pressure 16, RV pressure 53/9 and pulmonary artery pressure 40/16 mmHg), normal cardiac output (6.0 ml/min) and on oximetric series no left-to-right shunt was found. MDCT confirmed bilateral cystic bronchiectasis of the basal pulmonary fields and left middle lobe . The LAD showed calcification with suspected high-grade stenosis, a normal Cx and RCA. Furthermore, a CBF from the proximal part of the RCA communicated with the right bronchial artery. Radionuclide shunt measurement and MPI demonstrated a fixed defect in the anterior and apical region without reversibility with an LV ejection fraction of 48 % without a detectable left-right shunt. Pulmonary ventilation/perfusion scintigraphy revealed bilateral matched wedge-shaped lesions in the basal lung segments excluding pulmonary embolism. Cardiovascular magnetic resonance investigation (CMR) showed bilateral pulmonary lesions and no viable myocardium of the anterior wall with moderate LV dysfunction. PCI was abandoned and medical management was instituted. He was treated with metoprolol, acetylsalicylic acid, perindopril, simvastatin, tiotropium, acetylcysteine, and formoterol/beclometasone. He remains free of symptoms and was scheduled for annual follow-up.	4.191406	0.961426	sec[2]/p[0]	en	0.999998	24464641	https://doi.org/10.1007/s12471-014-0518-z	[ "artery", "without", "pulmonary", "perfusion", "bronchial", "branch", "basal", "chest", "myocardial", "defect" ]	[ { "code": "BD5Z", "title": "Diseases of arteries or arterioles, unspecified" }, { "code": "BD52", "title": "Certain specified disorders of arteries or arterioles" }, { "code": "BD52.3", "title": "Rupture of artery" }, { "code": "BD52.2", "title": "Stricture of artery" }, { "code": "BD40.Z", "title": "Atherosclerotic chronic arterial occlusive disease, unspecified" }, { "code": "MH12.1", "title": "Death occurring less than 24 hours from onset of symptoms, not otherwise explained" }, { "code": "FA36.Y", "title": "Other specified effusion of joint" }, { "code": "3A51.1", "title": "Sickle cell disease without crisis" }, { "code": "QA50", "title": "Embolisation without injury or harm" }, { "code": "LB12.1Z", "title": "Atresia of oesophagus, unspecified" } ]	=== ICD-11 CODES FOUND === [BD5Z] Diseases of arteries or arterioles, unspecified Also known as: Diseases of arteries or arterioles, unspecified \| artery disease NOS \| arterial disease NOS \| arteriolar disease NOS \| disorder of artery NOS [BD52] Certain specified disorders of arteries or arterioles Also known as: Certain specified disorders of arteries or arterioles \| Aortic dilatation - joint hypermobility - arterial tortuosity \| Generalised arterial calcification of infancy \| Median arcuate ligament syndrome \| Aortic root abscess Excludes: collagen (vascular) diseases \| Hypersensitivity angiitis \| Acute arterial occlusion [BD52.3] Rupture of artery Also known as: Rupture of artery \| ruptured artery \| artery fistula \| Aortic duodenal fistula \| Aortic colon fistula Excludes: traumatic rupture of artery - see injury of blood vessel by body region [BD52.2] Stricture of artery Also known as: Stricture of artery \| arterial stenosis \| arterial stricture \| artery stricture \| stenosis of artery [BD40.Z] Atherosclerotic chronic arterial occlusive disease, unspecified Also known as: Atherosclerotic chronic arterial occlusive disease, unspecified \| Atherosclerotic chronic arterial occlusive disease \| arteriosclerosis, NOS \| generalised atherosclerosis \| atherosclerosis NOS [MH12.1] Death occurring less than 24 hours from onset of symptoms, not otherwise explained Also known as: Death occurring less than 24 hours from onset of symptoms, not otherwise explained \| died without sign of disease \| Death known not to be violent or instantaneous for which no cause can be discovered \| death known not to be violent or instantaneous, cause unknown \| Death without sign of disease Includes: Death known not to be violent or instantaneous for which no cause can be discovered \| Death without sign of disease [FA36.Y] Other specified effusion of joint Also known as: Other specified effusion of joint \| Non aspirated effusion of joint \| Effusion of joint without blood \| Effusion of joint, multiple sites \| Effusion of joint, shoulder region [3A51.1] Sickle cell disease without crisis Definition: A disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may present with fatigue, shortness of breath, dizziness, headaches, pallor of skin or mucous membranes, and jaundice. This disorder is confirmed by identification of HbS mutation by genetic testing. Also known as: Sickle cell disease without crisis \| Hb-SS disease without crisis \| HbSS without crisis \| Sickle-cell anaemia without crisis \| SCD - [sickle cell disease] [QA50] Embolisation without injury or harm Definition: An embolisation without documented injury or harm occurs when a solid object within the venous or arterial circulation propagates to a distal location and becomes lodged there. Also known as: Embolisation without injury or harm \| Embolic phenomenon without documented injury or harm \| Air embolism without documented injury or harm \| Injection of air without injury or harm Excludes: Embolisation, as mode of injury or harm [LB12.1Z] Atresia of oesophagus, unspecified Also known as: Atresia of oesophagus, unspecified \| Atresia of oesophagus \| atresia of esophagus \| Atresia of oesophagus without fistula \| congenital atresia of oesophagus === GRAPH WALKS === --- Walk 1 --- [BD5Z] Diseases of arteries or arterioles, unspecified --PARENT--> [?] Diseases of arteries or arterioles --CHILD--> [BD50] Aortic aneurysm or dissection Def: Aortic aneurysm is a term for any swelling (dilation or aneurysm) of the aorta to greater than 1.5 times normal, usually representing an underlying weakness in the wall of the aorta at that location. ... --- Walk 2 --- [BD5Z] Diseases of arteries or arterioles, unspecified --PARENT--> [?] Diseases of arteries or arterioles --CHILD--> [?] Chronic arterial occlusive disease --- Walk 3 --- [BD52] Certain specified disorders of arteries or arterioles --CHILD--> [BD52.2] Stricture of artery --PARENT--> [BD52] Certain specified disorders of arteries or arterioles --- Walk 4 --- [BD52] Certain specified disorders of arteries or arterioles --EXCLUDES--> [?] Acute arterial occlusion --PARENT--> [?] Diseases of arteries or arterioles --- Walk 5 --- [BD52.3] Rupture of artery --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes Def: !markdown In the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre... --EXCLUDES--> [?] Stress fracture, not elsewhere classified --- Walk 6 --- [BD52.3] Rupture of artery --PARENT--> [BD52] Certain specified disorders of arteries or arterioles --EXCLUDES--> [?] Nonorgan specific systemic autoimmune disorders	[ "[BD5Z] Diseases of arteries or arterioles, unspecified\n --PARENT--> [?] Diseases of arteries or arterioles\n --CHILD--> [BD50] Aortic aneurysm or dissection\n Def: Aortic aneurysm is a term for any swelling (dilation or aneurysm) of the aorta to greater than 1.5 times normal, usually representing an underlying weakness in the wall of the aorta at that location. ...", "[BD5Z] Diseases of arteries or arterioles, unspecified\n --PARENT--> [?] Diseases of arteries or arterioles\n --CHILD--> [?] Chronic arterial occlusive disease", "[BD52] Certain specified disorders of arteries or arterioles\n --CHILD--> [BD52.2] Stricture of artery\n --PARENT--> [BD52] Certain specified disorders of arteries or arterioles", "[BD52] Certain specified disorders of arteries or arterioles\n --EXCLUDES--> [?] Acute arterial occlusion\n --PARENT--> [?] Diseases of arteries or arterioles", "[BD52.3] Rupture of artery\n --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes\n Def: !markdown\nIn the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre...\n --EXCLUDES--> [?] Stress fracture, not elsewhere classified", "[BD52.3] Rupture of artery\n --PARENT--> [BD52] Certain specified disorders of arteries or arterioles\n --EXCLUDES--> [?] Nonorgan specific systemic autoimmune disorders" ]	BD5Z	Diseases of arteries or arterioles, unspecified	[ { "from_icd11": "BD5Z", "icd10_code": "I7389", "icd10_title": "Other specified peripheral vascular diseases" }, { "from_icd11": "BD5Z", "icd10_code": "I7419", "icd10_title": "Embolism and thrombosis of other parts of aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7411", "icd10_title": "Embolism and thrombosis of thoracic aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7410", "icd10_title": "Embolism and thrombosis of unspecified parts of aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7381", "icd10_title": "Erythromelalgia" }, { "from_icd11": "BD5Z", "icd10_code": "I745", "icd10_title": "Embolism and thrombosis of iliac artery" }, { "from_icd11": "BD5Z", "icd10_code": "I789", "icd10_title": "Disease of capillaries, unspecified" }, { "from_icd11": "BD5Z", "icd10_code": "I748", "icd10_title": "Embolism and thrombosis of other arteries" }, { "from_icd11": "BD5Z", "icd10_code": "I749", "icd10_title": "Embolism and thrombosis of unspecified artery" }, { "from_icd11": "BD5Z", "icd10_code": "I781", "icd10_title": "Nevus, non-neoplastic" }, { "from_icd11": "BD5Z", "icd10_code": "I788", "icd10_title": "Other diseases of capillaries" }, { "from_icd11": "BD5Z", "icd10_code": "I744", "icd10_title": "Embolism and thrombosis of arteries of extremities, unspecified" }, { "from_icd11": "BD5Z", "icd10_code": "I70-I79", "icd10_title": "" }, { "from_icd11": "BD5Z", "icd10_code": "I74", "icd10_title": "Arterial embolism and thrombosis" }, { "from_icd11": "BD5Z", "icd10_code": "I73", "icd10_title": "Other peripheral vascular diseases" } ]	I7389	Other specified peripheral vascular diseases
Patient 1, male, 42 years old, underwent chest computed tomography (CT) for physical examination in April 2019, which suggested pleural metastasis of the thymoma. Tissue puncture under CT guidance suggested type B2 thymoma. From April 2019 to July 2019, four cycles of docetaxel and nedaplatin chemotherapy were administered, and the efficacy evaluation was stable. Following chemotherapy, apatinib (250 mg/day) was administered. The patient was given irregular oral medication and reexamination, and the condition was stable. On April 12, 2021, he was admitted to our department because of “persistent pain in the left shoulder for one month,” and underwent a CT scan of the chest, abdomen, and pelvis: a malignant mass in the anterior mediastinum with mediastinal lymph nodes, left pleura, left diaphragm, and bilateral lung metastasis, slightly larger lymph node on the left side of the heart, and involvement of the left pericardial wall. Brain magnetic resonance imaging (MRI) and bone scintigraphy revealed no signs of metastasis. On April 15, 2021, pleural puncture was performed. Postoperative pathology revealed type B3 thymoma. Immunohistochemical results: phosphocreatine kinase (CK) broad (+), CK5/6 (+), P63 (+), CK7 (partial +), CK19 (weak +), CD20 (lymphocytes +), WT-1 (-), Calretinin (-), CD5 (sporadic +), CD117 (-), TdT (sporadic +), PD-1 (-), PD-L1 (tumor cells +, 99%; immune cells +, 1%), MSH6 (+), PMS2 (+), MLH1 (+), MSH2 (partial +), CD56 (-), Syn (-), CgA (-), Ki-67 (15%). On April 17, 2021, one cycle of gemcitabine, carboplatin, and sintilimab chemotherapy was administered. On May 2, 2021, he was admitted to our department due to “fever for three days.” The patient developed fever on April 30, 2021, with a maximum temperature of 39.0 °C, accompanied by chest tightness and suffocation, no chest pain, rapid heart rate (110 beats/min), no cough expectoration, no abdominal pain, and diarrhea. Complete relevant tests and inspections, D dimer (D-D):8.58 ug/mL, macrobiochemical and myocardial enzyme spectrum: alanine aminotransferase (ALT) 97 U/L, aspartate aminotransferase (AST) 308 U/L, lactate dehydrogenase (LDH) 805 U/L, α-hydroxybutyrate dehydrogenase (HBDH) 549 U/L, CK 7603 U/L, creatine kinase isoenzyme mass (CK-MB) 53.37 ng/mL, high-sensitivity troponin (hs-CTNI) 1423.7 pg/mL. Interleukin (IL-6) 102.60 pg/mL, C-reactive protein (CRP) 71.1 mg/L, and procalcitonin (PCT) 0.530 ug/L. Routine blood tests revealed that the white blood cells and neutrophils were normal, the percentage of neutrophils was 88.9%, the percentage of neutrophils increased under a microscope, the cytoplasmic granules were coarsely stained, and the brain natriuretic peptide (BNP) level was normal. Lymphocyte subset analysis: CD3+CD4+/CD3+CD8 + 3.67, CD3+CD4 + 61.6%, CD3-CD(16 + 56) + 4.5%, CD3+CD8+ absolute value 218/uL, CD3-CD(16 + 56) + absolute value 59/uL. Electrocardiogram (ECG) showed sinus tachycardia (121 beats/min) and low voltage of the QRS complex in the limb leads. May 3, 2021 Echocardiography: EF67%; pericardial effusion (small amount). The test results after admission showed that liver function transaminase, CK, LDH, CK-MB, and hs-CTNI levels were significantly increased. At present, the BNP level was normal. After questioning the patient’s medical history, the patient had a history of heavy physical labor (carrying goods), and the weather had changed rapidly in recent days. The patient’s immunity was low after chemotherapy, and fever caused by the patient’s infection was not excluded, which further led to immune myocarditis. Consultation of the cardiology, ICU, and rheumatology departments was urgently needed to assist in diagnosis and treatment, and the possibility of immune myocarditis was considered. Methylprednisolone 1000 mg , human immunoglobulin 30 g pulse therapy, coenzyme Q10 nutrition myocardial therapy, metoprolol tablets heart rate lowering therapy, spironolactone tablets, furosemide diuretic therapy, reduced glutathione, magnesium isoglycyrrhizinate, bicyclol tablets liver protection therapy, and piperacillin tazobactam anti-infective therapy. On May 5, 2021, the patient’s general condition was poor, complaining of difficulty in breathing and eating, and the bulbar conjunctiva was edematous. Because the patient had a thymoma, muscle weakness was not excluded, and the oxygen saturation was 98% during nasal cannula inhalation. Blood gas analysis indicated type II respiratory failure, and a noninvasive ventilator was used to assist breathing. The reexamination of liver function transaminases still continued to increase, ALT 336 U/L, AST 649 U/L, immune hepatitis was not excluded, he had been given liver protection and methylprednisolone pulse therapy; the patient’s myocardial enzymes and hs-CTNI continued to increase, LDH 1718 U/L, HBDH 1259 U/L, CK 10802 U/L, CK-MB 272.6 ng/mL, hs-CTNI 12973.9 pg/mL, greatly increased, BNP 108.00 pg/mL, and May 5, 2022 ECG: sinus rhythm (89 beats/min), incomplete right bundle branch block, ST-T changes, QTc interval prolongation. May 5, 2021 Echocardiography: EF60%. Today’s ECG has changed compared with previous days, and cardiac bioelectrophysiology has been affected. It is recommended that the patient be transferred to the ICU or Department of Respiratory Intensive Medicine for continued treatment. The patient’s family refused to transfer the patient for economic reasons. On May 5, 2021, the patient suddenly became unconscious at 23:44, did not respond to calls, could not measure blood pressure, and had a weakened heartbeat and breathing. Continuous chest compressions were immediately administered, and symptomatic treatment such as epinephrine was administered to stimulate the heart and nicotine to stimulate breathing. Emergency consultation with the anesthesiology and intensive medicine departments revealed that the patient’s blood oxygen saturation continued to drop, and tracheal intubation ventilator-assisted ventilation was provided after consultation in the anesthesiology and intensive medicine departments. The patient’s heart rate progressively decreased, and blood pressure remained undetectable. The patient was administered epinephrine and other cardiotonic symptomatic treatments again, but the vital signs did not improve. The patient’s family indicated that they gave up continuing rescue and signed a notice to give up medical treatment. The patient died at 0:54 on May 6, 2021. In Figure 1 , we show the ALT, AST, BNP, hs-CTNI, and ECG of patient 1 during treatment.	3.884766	0.983398	sec[1]/sec[0]/p[0]	en	0.999998	36401466	https://doi.org/10.1097/MD.0000000000031873	[ "april", "blood", "chest", "heart", "ctni", "that", "thymoma", "chemotherapy", "immune", "liver" ]	[ { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" }, { "code": "MA12.1", "title": "Finding of cocaine in blood" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "MA12.2", "title": "Finding of hallucinogen in blood" }, { "code": "CB7Z", "title": "Diseases of the respiratory system, unspecified" }, { "code": "CB27", "title": "Pleural effusion" }, { "code": "CA44", "title": "Pyothorax" }, { "code": "MD30.Z", "title": "Chest pain, unspecified" }, { "code": "NA80.Y&XJ1C6", "title": "Thoracic haematoma" } ]	=== ICD-11 CODES FOUND === [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS [MA12.1] Finding of cocaine in blood Also known as: Finding of cocaine in blood \| cocaine in blood [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [MA12.2] Finding of hallucinogen in blood Also known as: Finding of hallucinogen in blood \| hallucinogen in blood [CB7Z] Diseases of the respiratory system, unspecified Also known as: Diseases of the respiratory system, unspecified \| disorder of respiratory system \| respiratory disease NOS \| respiratory tract disease \| respiratory disorder NOS [CB27] Pleural effusion Definition: Presence of fluid in the pleural cavity resulting from excessive transudation or exudation from the pleural surfaces. Also known as: Pleural effusion \| PE - [pleural effusion] \| Pleurisy with effusion \| pleurisy with effusion NOS \| pleural effusion with transudate Includes: Pleurisy with effusion Excludes: Tuberculosis of the respiratory system \| Chylous effusion \| Pleurisy [CA44] Pyothorax Definition: Suppurative inflammation of the pleural space, typically due to acute bacterial infection. It can occur as a complication of pneumonia, thoracotomy, abscesses (lung, hepatic, or subdiaphragmatic), or penetrating trauma with a secondary infection. Also known as: Pyothorax \| empyema \| pyopneumothorax \| Pyothorax with fistula \| empyema with fistula Includes: empyema \| pyopneumothorax Excludes: due to tuberculosis [MD30.Z] Chest pain, unspecified Also known as: Chest pain, unspecified \| Pain in throat or chest \| chest pain NOS \| pain in chest \| chest pressure === GRAPH WALKS === --- Walk 1 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --EXCLUDES--> [?] Certain conditions originating in the perinatal period Def: This chapter includes conditions that have their origin in the perinatal period even though death or morbidity occurs later.... --- Walk 2 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --CHILD--> [?] Anaemias or other erythrocyte disorders --- Walk 3 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --PARENT--> [MF50] Abnormal micturition --- Walk 4 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --CHILD--> [MF50.4Z] Haematuria, unspecified --- Walk 5 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.1] Finding of cocaine in blood --- Walk 6 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.0] Finding of opiate drug in blood	[ "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --EXCLUDES--> [?] Certain conditions originating in the perinatal period\n Def: This chapter includes conditions that have their origin in the perinatal period even though death or morbidity occurs later....", "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --CHILD--> [?] Anaemias or other erythrocyte disorders", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --PARENT--> [MF50] Abnormal micturition", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --CHILD--> [MF50.4Z] Haematuria, unspecified", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.1] Finding of cocaine in blood", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.0] Finding of opiate drug in blood" ]	3C0Z	Diseases of the blood or blood-forming organs, unspecified	[ { "from_icd11": "3C0Z", "icd10_code": "D75A", "icd10_title": "Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia" }, { "from_icd11": "3C0Z", "icd10_code": "D7581", "icd10_title": "Myelofibrosis" }, { "from_icd11": "3C0Z", "icd10_code": "D7582", "icd10_title": "Heparin induced thrombocytopenia (HIT)" }, { "from_icd11": "3C0Z", "icd10_code": "D7589", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D759", "icd10_title": "Disease of blood and blood-forming organs, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "D763", "icd10_title": "Other histiocytosis syndromes" }, { "from_icd11": "3C0Z", "icd10_code": "Q899", "icd10_title": "Congenital malformation, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "III", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D70-D77", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D75", "icd10_title": "Other and unspecified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D77", "icd10_title": "Other disorders of blood and blood-forming organs in diseases classified elsewhere" }, { "from_icd11": "3C0Z", "icd10_code": "D758", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D76", "icd10_title": "Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue" }, { "from_icd11": "MF50.4Z", "icd10_code": "R310", "icd10_title": "Gross hematuria" }, { "from_icd11": "MF50.4Z", "icd10_code": "R312", "icd10_title": "Other microscopic hematuria" } ]	D75A	Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia
A 75-year-old woman presented to the memory outpatient clinic of the Department of Psychiatry and Psychotherapy of the University Medical Center Göttingen because of concentration difficulties as well as memory and retentiveness disorders that had been gradually developing for 2 years . She could not always remember orders for certain activities. No episodes of transient loss of consciousness have been reported. Similarly, there was no clinical evidence of delirium. She also complained of a gait disorder, but is unsure about when exactly it started. Her recurrent depressive disorder had been documented; she currently suffers mild to no specific depressive symptoms involving reduced drive, impaired concentration, brooding cognitions, apathy, and social withdrawal. The patient is already dependent on her grandson’s help, as he lives with her in the house. She was a saleswoman, and worries a lot about her daughter. Her psychopathological examination confirmed the reported disturbances of memory and retention, but there was no other psychopathology evident. Neurological examination revealed an extinguished patellar reflex on the right side and extinguished Achilles tendon reflexes on both sides. She also revealed mild foot jack paresis on the right. We found no evidence of rigor, tremor, or cogwheel phenomenon. She also complained of urinary incontinence and occasional fecal incontinence. Pre-existing conditions are a chronic pain syndrome with psychological and somatic factors of unclear onset. She has ventrolisthesis of LWK3 versus LWK4 and LWK4 versus LWK5, in addition to facet joint atresia and osteochondrosis intervertebralis. In addition, she has two artificial knee joints, as well as arterial hypertension, obesity, and presbycusis. She suffered a Weber B fracture on the left side in 2017. Neuropsychological testing revealed impaired semantic and phonematic word fluency, reduced cognitive processing speed, faulty switching ability, a pathological clock test, and verbal memory deficits (encoding and delayed recall of verbal, non-associated information, encoding and delayed recall of complex verbal content) . Results from her thorough neuropsychological tests are in Figure 2 . The other parameters tested, such as confrontation recognition, action planning, working and figural memory, verbal discriminability, visuoconstructive skills, and visuospatial perception, corresponded to her educational level. Her profile was thuson the borderline between mild amnestic cognitive impairment and an incipient dementia syndrome. Taking into account her significantly impaired daily living skills (B-ADL = 7.8) (according to her daughter), the dementia criteria are clinically fulfilled. Her magnetic resonance imaging (MRI) findings ( Table 1 ) revealed a Fazekas grade II and medial temporal lobe atrophy score of 2 suggesting mixed dementia. In more detail: the MRI examination she had December of last year showed evidence of a general, primarily internal cerebral volume reduction with an advanced microangiopathic damage pattern. We detected Fazekas grade II microangiopathic anomalies. When she underwent MRI, her cognitive symptoms had been present for 1 year. The computed tomography taken 1 year later (2 months after she presented in our outpatient memory clinic) also showed a dilated ventricular system with widespread brain atrophy. Her (123) I N-omega-fluoropropyl-2beta-carbomethoxy-3beta-{4-iodophenyl}nortropane single photon emission computed tomography examination showed no reduced dopaminergic uptake in the nigrostriatal system. A differential-diagnostic cerebrospinal fluid (CSF) puncture showed mild pleocytosis (6/μL, reference: ≤ 5/μL, Table 1 ) with a predominant pattern of lymphocytes (93%), elevated phosphorylated tau protein 181 (54.8 pg/mL, pathological: > 50 pg/mL, Table 1 ) and total tau protein (418 pg/mL, reference, <415 pg/mL), and anti-CARPVIII autoantibodies in the serum at 1:32 intensity . The CSF analysis revealed no abnormalities in the beta amyloid peptides: amyloid beta 42 (Aß42), amyloid beta 40 (Aß40) and the amyloid beta 42/40 (Aß42/40) ratio were all in the normal range. In a repeated blood test 2 months later, we detected anti-CARPVIII autoantibodies again at a greater intensity of 1:32 . The autoantibodies were determined in the Clinical Immunological Laboratory from Prof. Stöcker. Relying on the dementia syndrome involving signs of CNS inflammation such as pleocytosis and after detecting anti-CARPVIII autoantibodies in serum, we diagnosed an autoimmune dementia as a component of mixed dementia with additional vascular dementia components present. Our repeated detection of anti-CARPVIII autoantibodies in her serum confirmed their presence as a relevant finding. Portions of the dementia syndrome may also be vascular in origin. There was no evidence of dementia with Lewy bodies or dementia due to Parkinson’s disease. In case of an inconspicuous ratio Aß42/40, an Alzheimer pathology as a second etiology of mixed dementia is equally unlikely, but cannot be excluded in the presence of elevated ptau181. Thus, we cannot entirely rule out a typical mixed dementia with both vascular components and Alzheimer pathology, but that is quite unlikely. Furthermore, as her ptau181 was only slightly elevated, Alzheimer’s disease is unlikely as the cause of her cognitive impairment. She suffered from a gait disturbance, urinary incontinence, and dementia-like syndrome, thus fulfilling the Hakim triad, so we considered the differential diagnosis of normal pressure hydrocephalus. However, there was no evidence of normal pressure hydrocephalus on imaging, and the gait disturbance could be attributable to her pre-existing spinal anomalies and foot lifter paresis. Her cognitive impairments could also be explained differently via the mixed dementia, thus ruling out a normal pressure hydrocephalus as a differential diagnosis. Our patient underwent her last follow-up examinations as an inpatient in December last year. She has not undergone any additional follow-up examinations since then. Therapeutically speaking, we limited ourselves to treating her vascular risk factors by prescribing telmisartan 80 mg/d and atorvastatin 20 mg/d. She has not been offered immunotherapy given the slow progression of her symptoms and mixed dementia. However, should her symptoms begin again to deteriorate and were we to detect anti-CARPVIII autoantibodies again, immunotherapy would be an option as an individualized curative approach.	4.160156	0.96875	sec[1]/p[0]	en	0.999998	PMC10196045	https://doi.org/10.3389/fpsyt.2023.1133302	[ "dementia", "memory", "mixed", "autoantibodies", "evidence", "cognitive", "anti", "carpviii", "verbal", "beta" ]	[ { "code": "6D8Z", "title": "Dementia, unknown or unspecified cause" }, { "code": "6D81", "title": "Dementia due to cerebrovascular disease" }, { "code": "6D8Y", "title": "Dementia, other specified cause" }, { "code": "6D84.2", "title": "Dementia due to use of volatile inhalants" }, { "code": "6D85", "title": "Dementia due to diseases classified elsewhere" }, { "code": "MB21.1Z", "title": "Amnesia, unspecified" }, { "code": "MB21.0", "title": "Age-associated cognitive decline" }, { "code": "6D71", "title": "Mild neurocognitive disorder" }, { "code": "PB29", "title": "Unintentional exposure to or harmful effects of multiple drugs, medicaments or biological substances" }, { "code": "PH49", "title": "Exposure to or harmful effects of undetermined intent of multiple drugs, medicaments or biological substances" } ]	=== ICD-11 CODES FOUND === [6D8Z] Dementia, unknown or unspecified cause Also known as: Dementia, unknown or unspecified cause \| Dementia NOS \| Dementia, unspecified \| senile melancholia \| senile depression [6D81] Dementia due to cerebrovascular disease Definition: Dementia due to brain parenchyma injury resulting from cerebrovascular disease (ischemic or haemorrhagic). The onset of the cognitive deficits is temporally related to one or more vascular events. Cognitive decline is typically most prominent in speed of information processing, complex attention, and frontal-executive functioning. There is evidence of the presence of cerebrovascular disease considered to be sufficient to account for the neurocognitive deficits from history, physical examination Also known as: Dementia due to cerebrovascular disease \| arteriosclerotic dementia \| Strategic-infarct dementia \| Post stroke dementia \| vascular cognitive impairment Excludes: Alzheimer disease dementia, mixed type, with cerebrovascular disease [6D8Y] Dementia, other specified cause Also known as: Dementia, other specified cause \| Degenerative dementia \| primary degenerative dementia NOS [6D84.2] Dementia due to use of volatile inhalants Definition: Dementia due to use of volatile inhalants is characterised by the development of persistent cognitive impairments (e.g., memory problems, language impairment, and an inability to perform complex motor tasks) that meet the definitional requirements of Dementia that are judged to be a direct consequence of inhalant use or exposure and that persist beyond the usual duration of action or withdrawal syndrome associated with the substance. The amount and duration of inhalant use or exposure must be su Also known as: Dementia due to use of volatile inhalants \| inhalant dementia \| volatile solvents dementia [6D85] Dementia due to diseases classified elsewhere Also known as: Dementia due to diseases classified elsewhere [MB21.1Z] Amnesia, unspecified Also known as: Amnesia, unspecified \| Amnesia \| disturbance of memory \| lack of memory \| loss of memory [MB21.0] Age-associated cognitive decline Definition: A normative (non-pathological) deterioration of higher cortical functions such as thinking, reasoning, comprehension, calculation, learning, language, and judgment. Also known as: Age-associated cognitive decline \| Age-associated memory impairment \| Age related cognitive decline \| Ageing-related cognitive decline \| Senile degeneration of brain, not elsewhere classified [6D71] Mild neurocognitive disorder Definition: Mild neurocognitive disorder is characterized by mild impairment in one or more cognitive domains relative to that expected given the individual’s age and general premorbid level of cognitive functioning, which represents a decline from the individual’s previous level of functioning. Diagnosis is based on report from the patient, informant, or clinical observation, and is accompanied by objective evidence of impairment by quantified clinical assessment or standardized cognitive testing. Cognitiv Also known as: Mild neurocognitive disorder \| minor neurocognitive disorder \| Postconcussional syndrome \| post-concussion syndrome \| post-concussional syndrome [PB29] Unintentional exposure to or harmful effects of multiple drugs, medicaments or biological substances Also known as: Unintentional exposure to or harmful effects of multiple drugs, medicaments or biological substances \| accidental overdose of multiple drugs, medicaments or biological substances \| accidental poisoning by multiple drugs, medicaments or biological substances \| multiple drugs, medicaments or biological substances taken in error \| combined drug toxicity NOS Includes: accidental overdose of multiple drugs, medicaments or biological substances [PH49] Exposure to or harmful effects of undetermined intent of multiple drugs, medicaments or biological substances Also known as: Exposure to or harmful effects of undetermined intent of multiple drugs, medicaments or biological substances \| mixed substance toxicity NOS \| acute combined drug toxicity === GRAPH WALKS === --- Walk 1 --- [6D8Z] Dementia, unknown or unspecified cause --PARENT--> [?] Dementia Def: Dementia is characterized by the presence of marked impairment in two or more cognitive domains relative to that expected given the individual’s age and general premorbid level of cognitive functionin... --CHILD--> [6D80] Dementia due to Alzheimer disease Def: Dementia due to Alzheimer disease is the most common form of dementia. Onset is insidious with memory impairment typically reported as the initial presenting complaint. The characteristic course is a ... --- Walk 2 --- [6D8Z] Dementia, unknown or unspecified cause --PARENT--> [?] Dementia Def: Dementia is characterized by the presence of marked impairment in two or more cognitive domains relative to that expected given the individual’s age and general premorbid level of cognitive functionin... --EXCLUDES--> [?] Coma Def: Acute state lasting more than one hour and usually less than a month. The comatose patient is unresponsive, lying with their eyes closed and cannot be aroused even by vigorous and noxious stimuli. Mot... --- Walk 3 --- [6D81] Dementia due to cerebrovascular disease Def: Dementia due to brain parenchyma injury resulting from cerebrovascular disease (ischemic or haemorrhagic). The onset of the cognitive deficits is temporally related to one or more vascular events. Cog... --EXCLUDES--> [?] Alzheimer disease dementia, mixed type, with cerebrovascular disease Def: Dementia due to Alzheimer disease and concomitant cerebrovascular disease.... --PARENT--> [?] Dementia due to Alzheimer disease Def: Dementia due to Alzheimer disease is the most common form of dementia. Onset is insidious with memory impairment typically reported as the initial presenting complaint. The characteristic course is a ... --- Walk 4 --- [6D81] Dementia due to cerebrovascular disease Def: Dementia due to brain parenchyma injury resulting from cerebrovascular disease (ischemic or haemorrhagic). The onset of the cognitive deficits is temporally related to one or more vascular events. Cog... --EXCLUDES--> [?] Alzheimer disease dementia, mixed type, with cerebrovascular disease Def: Dementia due to Alzheimer disease and concomitant cerebrovascular disease.... --PARENT--> [?] Dementia due to Alzheimer disease Def: Dementia due to Alzheimer disease is the most common form of dementia. Onset is insidious with memory impairment typically reported as the initial presenting complaint. The characteristic course is a ... --- Walk 5 --- [6D8Y] Dementia, other specified cause --PARENT--> [?] Dementia Def: Dementia is characterized by the presence of marked impairment in two or more cognitive domains relative to that expected given the individual’s age and general premorbid level of cognitive functionin... --CHILD--> [6D81] Dementia due to cerebrovascular disease Def: Dementia due to brain parenchyma injury resulting from cerebrovascular disease (ischemic or haemorrhagic). The onset of the cognitive deficits is temporally related to one or more vascular events. Cog... --- Walk 6 --- [6D8Y] Dementia, other specified cause --PARENT--> [?] Dementia Def: Dementia is characterized by the presence of marked impairment in two or more cognitive domains relative to that expected given the individual’s age and general premorbid level of cognitive functionin... --PARENT--> [?] Neurocognitive disorders Def: Neurocognitive disorders are characterised by primary clinical deficits in cognitive functioning that are acquired rather than developmental. That is, neurocognitive disorders do not include disorders...	[ "[6D8Z] Dementia, unknown or unspecified cause\n --PARENT--> [?] Dementia\n Def: Dementia is characterized by the presence of marked impairment in two or more cognitive domains relative to that expected given the individual’s age and general premorbid level of cognitive functionin...\n --CHILD--> [6D80] Dementia due to Alzheimer disease\n Def: Dementia due to Alzheimer disease is the most common form of dementia. Onset is insidious with memory impairment typically reported as the initial presenting complaint. The characteristic course is a ...", "[6D8Z] Dementia, unknown or unspecified cause\n --PARENT--> [?] Dementia\n Def: Dementia is characterized by the presence of marked impairment in two or more cognitive domains relative to that expected given the individual’s age and general premorbid level of cognitive functionin...\n --EXCLUDES--> [?] Coma\n Def: Acute state lasting more than one hour and usually less than a month. The comatose patient is unresponsive, lying with their eyes closed and cannot be aroused even by vigorous and noxious stimuli. Mot...", "[6D81] Dementia due to cerebrovascular disease\n Def: Dementia due to brain parenchyma injury resulting from cerebrovascular disease (ischemic or haemorrhagic). The onset of the cognitive deficits is temporally related to one or more vascular events. Cog...\n --EXCLUDES--> [?] Alzheimer disease dementia, mixed type, with cerebrovascular disease\n Def: Dementia due to Alzheimer disease and concomitant cerebrovascular disease....\n --PARENT--> [?] Dementia due to Alzheimer disease\n Def: Dementia due to Alzheimer disease is the most common form of dementia. Onset is insidious with memory impairment typically reported as the initial presenting complaint. The characteristic course is a ...", "[6D81] Dementia due to cerebrovascular disease\n Def: Dementia due to brain parenchyma injury resulting from cerebrovascular disease (ischemic or haemorrhagic). The onset of the cognitive deficits is temporally related to one or more vascular events. Cog...\n --EXCLUDES--> [?] Alzheimer disease dementia, mixed type, with cerebrovascular disease\n Def: Dementia due to Alzheimer disease and concomitant cerebrovascular disease....\n --PARENT--> [?] Dementia due to Alzheimer disease\n Def: Dementia due to Alzheimer disease is the most common form of dementia. Onset is insidious with memory impairment typically reported as the initial presenting complaint. The characteristic course is a ...", "[6D8Y] Dementia, other specified cause\n --PARENT--> [?] Dementia\n Def: Dementia is characterized by the presence of marked impairment in two or more cognitive domains relative to that expected given the individual’s age and general premorbid level of cognitive functionin...\n --CHILD--> [6D81] Dementia due to cerebrovascular disease\n Def: Dementia due to brain parenchyma injury resulting from cerebrovascular disease (ischemic or haemorrhagic). The onset of the cognitive deficits is temporally related to one or more vascular events. Cog...", "[6D8Y] Dementia, other specified cause\n --PARENT--> [?] Dementia\n Def: Dementia is characterized by the presence of marked impairment in two or more cognitive domains relative to that expected given the individual’s age and general premorbid level of cognitive functionin...\n --PARENT--> [?] Neurocognitive disorders\n Def: Neurocognitive disorders are characterised by primary clinical deficits in cognitive functioning that are acquired rather than developmental. That is, neurocognitive disorders do not include disorders..." ]	6D8Z	Dementia, unknown or unspecified cause	[ { "from_icd11": "6D8Z", "icd10_code": "F0391", "icd10_title": "Unspecified dementia with behavioral disturbance" }, { "from_icd11": "6D8Z", "icd10_code": "F0150", "icd10_title": "Vascular dementia without behavioral disturbance" }, { "from_icd11": "6D8Z", "icd10_code": "F0151", "icd10_title": "Vascular dementia with behavioral disturbance" }, { "from_icd11": "6D8Z", "icd10_code": "F0281", "icd10_title": "Dementia in other diseases classified elsewhere with behavioral disturbance" }, { "from_icd11": "6D8Z", "icd10_code": "F0390", "icd10_title": "Unspecified dementia without behavioral disturbance" }, { "from_icd11": "6D8Z", "icd10_code": "F00-F09", "icd10_title": "" }, { "from_icd11": "6D8Z", "icd10_code": "F00", "icd10_title": "" }, { "from_icd11": "6D8Z", "icd10_code": "F01", "icd10_title": "Vascular dementia" }, { "from_icd11": "6D8Z", "icd10_code": "F028", "icd10_title": "Dementia in other diseases classified elsewhere" }, { "from_icd11": "6D8Z", "icd10_code": "F03", "icd10_title": "Unspecified dementia" }, { "from_icd11": "6D8Z", "icd10_code": "F02", "icd10_title": "Dementia in other diseases classified elsewhere" }, { "from_icd11": "6D81", "icd10_code": "F010", "icd10_title": "" }, { "from_icd11": "6D81", "icd10_code": "F011", "icd10_title": "" }, { "from_icd11": "6D81", "icd10_code": "F012", "icd10_title": "" }, { "from_icd11": "6D81", "icd10_code": "F013", "icd10_title": "" } ]	F0391	Unspecified dementia with behavioral disturbance
A 71 year-old woman with a history of hypertension, hypothyroidism, and acoustic neuroma was admitted for right retrosigmoid craniotomy and tumor resection. Her operation was uneventful, with no immediate post-surgical complications. She was discharged on dexamethasone 6 mg daily for 3 days, with tapering doses of steroids over the course of 1 week. Three weeks later, she presented to the hospital with persistent fever and chills, and had experienced occasional headaches for five days. On admission, her vitals were significant with a temperature of 38.5 °C, heart rate of 83 beats per minute, respiratory rate of 14 breaths per minute, and blood pressure of 158/83 mmHg. On examination, she was alert and oriented to person, place, and time. Her right posterior auricular incision site looked clean, with mild erythema but no discharge. Sutures were intact. Neurologic exam did not reveal any focal or meningeal signs. Laboratory studies were significant with a white blood cell count of 8.9 k/μL, hemoglobin of 10.9 g/dL, and a platelet count of 313 k/μL. Urinalysis showed 33 white blood cells, positive leukocyte esterase, and negative nitrates. Urine culture grew more than 100,000 colonies of Klebsiella pneumoniae. She was started on cefepime 2 g intravenously every 12 h for presumptive urinary tract infection. Despite antibiotic coverage for 3 days, the patient continued to have fevers up to 38.7 °C. Given her recent surgical intervention, a computed tomography (CT) scan was ordered, which showed a subgaleal collection overlying the craniotomy site and hypodense areas in the right cerebellum that likely represented postoperative changes. A hypodense extra-axial collection was noted along the right cerebellum. Given the concern for postsurgical meningitis, cefepime was switched to meropenem 2 g every 8 h, and vancomycin 1 g every 12 h was added to her antibiotic regimen. The patient showed a rapid clinical deterioration, with the development of nuchal rigidity, altered mental status, and seizures in the next 24 h. She underwent a lumbar puncture, which evidenced an opening pressure of 30 cm H 2 O. Cerebrospinal fluid (CSF) analysis showed 800 white blood cells/mm 3 with 90% neutrophils, low glucose (31 mg/dL), and high protein (115 mg/dL). The smear for acid-fast bacilli in CSF was negative. CSF Cryptococcus antigen was also negative. The FilmArray ® meningitis/encephalitis panel did not identify any organisms. Emergent magnetic resonance imaging (MRI) was ordered, which showed diffuse leptomeningeal enhancement and moderately dilated ventricles. Fluid layering within the bilateral occipital horns was noted, as there were concerns of ventriculitis. Additionally, a 1 cm right cerebellar extra-axial collection was seen . The patient was immediately taken to the operating room for the placement of an external ventricular drain. Intrathecal colistin and vancomycin were initiated afterwards. Follow-up CSF analysis 48 h later showed normalization of white blood cell count (2 cells/mm 3 ); however, low glucose and high protein persisted. Lactic acid from CSF was elevated (35 mg/dL). Bacterial and fungal cultures from the blood and CSF did not show any growth. Intraventricular antibiotics were discontinued after 48 h, and the patient continued with meropenem and vancomycin intravenously for 2 weeks. Despite antibiotic therapy, the patient’s mental status did not improve significantly, and the fevers continued intermittently. Further studies that included serum galactomannan and (1→3)-β- d -glucan were negative. A repeat MRI showed persistent leptomeningeal enhancement at the cervicomedullary junction at the skull. Extra-axial collection adjacent to the retrosigmoid craniotomy was again noted. A CT scan of the chest, abdomen, and pelvis was ordered to rule out other potential sources of infection, but imaging was unremarkable. On further questioning, the patient’s daughter stated that her mother was in India and Nepal three months prior to the initial craniotomy, where she was working as a volunteer in a clinic and was exposed to patients with tuberculosis (TB). The patient had a history of a positive purified protein derivative (PPD), but was never treated for latent TB. Given her epidemiologic risk factors, MRI findings and clinical deterioration, the patient was started on isoniazid, rifampin, pyrazinamide, and levofloxacin for the treatment of presumptive tubercular meningitis. However, after one week of treatment, no clinical improvement was noted, and she was taken to the operating room for exploration. The patient underwent midline limited suboccipital craniotomy and C1 laminectomy. Clear pus was noted in the area of the foramen magnum, foramina of Magendie, and posterior to the upper cervical cord, with evidence of pachymeningitis of the pia of the upper cervical cord and both cerebellar tonsils. The subdural empyema was evacuated and samples were sent for cultures. A biopsy of the arachnoid tissue was obtained. Intra-operative frozen section revealed chronic mixed inflammatory, but no granulomas. Two days later, histopathology reported fungal elements. Immunohistochemistry for Aspergillus was strongly positive . Toxoplasma, acid-fast bacilli, and gram stains were negative. Tissue sample was sent to the University of Washington in Seattle, WA for fungal PCR. Anti-tuberculosis treatment was discontinued and the patient was started on liposomal amphotericin 5 mg/kg daily and voriconazole intravenously 6 mg/kg every 12 h as a loading dose, and then 4 mg/kg every 12 h for maintenance. Five days later, tissue real-time PCR came back positive for Aspergillus fumigatus. Amphotericin was discontinued and the patient was kept on voriconazole. The patient’s mental status improved markedly over the following days. The fever subsided as well. Treatment with intravenous voriconazole was eventually transitioned to oral voriconazole 250 mg every 12 h to complete 3 months of therapy. The voriconazole level at 2 weeks of treatment was 2.6 μg/mL, which was within the therapeutic range for central nervous system (CNS) aspergillosis (reference range 2–5 μg/mL). The patient was later transferred to a rehabilitation facility for physical therapy. At the four month follow-up, the patient was fully alert, and her verbal communication was almost back to her baseline. No focal or meningeal signs were noted on examination. A brain MRI was repeated and showed complete resolution of leptomeningeal enhancement, with no collections.	3.935547	0.976563	sec[1]/p[0]	en	0.999998	29857501	https://doi.org/10.3390/diseases6020046	[ "blood", "every", "craniotomy", "voriconazole", "white", "which", "collection", "count", "cells", "intravenously" ]	[ { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" }, { "code": "MA12.1", "title": "Finding of cocaine in blood" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "MA12.2", "title": "Finding of hallucinogen in blood" }, { "code": "KA05.Y", "title": "Fetus or newborn affected or suspected to be affected by other specified complications of labour or delivery" }, { "code": "JB23.4", "title": "Destructive operation for delivery" }, { "code": "8A84.Y", "title": "Other specified secondary headache" }, { "code": "EF5Y", "title": "Other specified dermatoses attributable to hyperviscosity or microvascular occlusion" }, { "code": "JB41.1", "title": "Deep phlebothrombosis in the puerperium" } ]	=== ICD-11 CODES FOUND === [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS [MA12.1] Finding of cocaine in blood Also known as: Finding of cocaine in blood \| cocaine in blood [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [MA12.2] Finding of hallucinogen in blood Also known as: Finding of hallucinogen in blood \| hallucinogen in blood [KA05.Y] Fetus or newborn affected or suspected to be affected by other specified complications of labour or delivery Also known as: Fetus or newborn affected or suspected to be affected by other specified complications of labour or delivery \| Fetus or newborn affected by abnormality of maternal soft tissues \| Fetus or newborn affected by cervix anomaly in pregnancy or childbirth \| Fetus or newborn affected by stenosis of cervix canal in pregnancy or childbirth \| Fetus or newborn affected by maternal exhaustion complicating delivery [JB23.4] Destructive operation for delivery Definition: A condition caused by the development of a fetus to the culmination of the pregnancy period. This condition is characterised by parturition of a neonate using destructive operation interventions or techniques to assist the delivery. Also known as: Destructive operation for delivery \| Cleidotomy to facilitate delivery \| Craniotomy to facilitate delivery \| Embryotomy to facilitate delivery [8A84.Y] Other specified secondary headache Also known as: Other specified secondary headache \| Headache associated with injury to the head or neck \| Post traumatic headache \| Headache associated with craniotomy \| Acute headache associated with craniotomy [EF5Y] Other specified dermatoses attributable to hyperviscosity or microvascular occlusion Also known as: Other specified dermatoses attributable to hyperviscosity or microvascular occlusion \| Cutaneous microvascular occlusion by platelet plugs \| Cutaneous microvascular disturbances due to myeloproliferative disorder-associated platelet plugs \| Cutaneous microvascular disturbances due to paroxysmal nocturnal haemoglobinuria-associated platelet plugs \| Cutaneous microvascular occlusion by emboli [JB41.1] Deep phlebothrombosis in the puerperium Also known as: Deep phlebothrombosis in the puerperium \| postnatal deep vein thrombosis \| postpartum deep phlebothrombosis \| postpartum deep-vein thrombosis \| DVT - [deep venous thrombosis] postnatal === GRAPH WALKS === --- Walk 1 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --RELATED_TO--> [?] Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system --- Walk 2 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --EXCLUDES--> [?] Diseases of the immune system --- Walk 3 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --CHILD--> [MF50.41] Microscopic haematuria --- Walk 4 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --CHILD--> [MF50.41] Microscopic haematuria --- Walk 5 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --PARENT--> [?] Clinical findings in blood, blood-forming organs, or the immune system --- Walk 6 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.2] Finding of hallucinogen in blood	[ "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --RELATED_TO--> [?] Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system", "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --EXCLUDES--> [?] Diseases of the immune system", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --CHILD--> [MF50.41] Microscopic haematuria", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --CHILD--> [MF50.41] Microscopic haematuria", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --PARENT--> [?] Clinical findings in blood, blood-forming organs, or the immune system", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.2] Finding of hallucinogen in blood" ]	3C0Z	Diseases of the blood or blood-forming organs, unspecified	[ { "from_icd11": "3C0Z", "icd10_code": "D75A", "icd10_title": "Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia" }, { "from_icd11": "3C0Z", "icd10_code": "D7581", "icd10_title": "Myelofibrosis" }, { "from_icd11": "3C0Z", "icd10_code": "D7582", "icd10_title": "Heparin induced thrombocytopenia (HIT)" }, { "from_icd11": "3C0Z", "icd10_code": "D7589", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D759", "icd10_title": "Disease of blood and blood-forming organs, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "D763", "icd10_title": "Other histiocytosis syndromes" }, { "from_icd11": "3C0Z", "icd10_code": "Q899", "icd10_title": "Congenital malformation, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "III", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D70-D77", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D75", "icd10_title": "Other and unspecified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D77", "icd10_title": "Other disorders of blood and blood-forming organs in diseases classified elsewhere" }, { "from_icd11": "3C0Z", "icd10_code": "D758", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D76", "icd10_title": "Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue" }, { "from_icd11": "MF50.4Z", "icd10_code": "R310", "icd10_title": "Gross hematuria" }, { "from_icd11": "MF50.4Z", "icd10_code": "R312", "icd10_title": "Other microscopic hematuria" } ]	D75A	Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia
A 60-year-old man was referred to our hospital with the chief complaints of fever and left lower abdominal pain. Serum carcinoembryonic antigen and carbohydrate antigen 19-9 levels were 483.1 ng/ml and 2034 U/ml, respectively. Contrast-enhanced computed tomography (CT) revealed sigmoid colon cancer with intraabdominal abscess and two synchronous liver metastases. One of the liver tumors (tumor 1) was located in segment 8 near the caval confluence. Tumor 1 was 2.2 cm in size and attached to both the right hepatic vein (RHV) and the middle hepatic vein (MHV) . The other tumor (tumor 2) was located in the left lobe. Tumor 2 was 10 cm in size and invaded the umbilical portion of the portal vein . Sigmoidectomy was performed, and pathological findings revealed moderately differentiated tubular adenocarcinoma. The depth of the tumor was subserosal, and there was no lymph node metastasis. Rat sarcoma viral oncogene homolog was the wild type. Hepatic resection was initially contraindicated because all three major hepatic veins were invaded by the tumors . Tumor 1 near the caval confluence had invaded both the RHV and the MHV, and we could not preserve the left hepatic vein during left hemihepatectomy for tumor 2. Therefore, the patient underwent four cycles of panitumumab/5-fluorouracil, leucovorin, and oxaliplatin (FOLFOX) therapy after sigmoidectomy; following which, both liver metastases decreased in size. Tumor 1 remained attached to the RHV; however, it was located a short distance from the MHV . Serum carcinoembryonic antigen and carbohydrate antigen 19-9 levels decreased to 18.7 ng/ml and 14 U/ml, respectively, and the 15-min indocyanine green retention rate was 9.7%. Based on these findings, we planned radical liver resection. Three-dimensional volumetric software (Synapse Vincent ® ; Fujifilm, Tokyo, Japan) calculated the total liver volume (TLV) at 1344 ml with estimated volumes for limited resection of segment 8 and left hemihepatectomy of 13 ml (0.9% of TLV) and 263 ml (19.6% of TLV), respectively. When the RHV was severed during limited resection of segment 8, the non-congested FLR volume was estimated at 354 ml (26.3% of TLV) because the congested FLR volume was calculated at 714 ml (53.1% of TLV) . We devised a novel TSH procedure because the small non-congested FLR volume was a potential risk for postoperative POLF . We first performed limited resection of segment 8 with resection of the root of the RHV. The left portal vein was neither ligated nor embolized because most of the umbilical portion was invaded by tumor 2 and because we expected not to cause adhesions around the porta hepatis for the second hepatectomy. Macroscopic examination of the resected specimen revealed that the size of tumor 1 was 1.0 × 0.6 cm, and it had invaded the RHV . Histological diagnosis was compatible with metastatic carcinoma of the sigmoid colon, and invasion of the RHV was confirmed . Surgical margins were negative for cancer. The patient was discharged on postoperative day 9 without complications. No local recurrence or new metastatic lesions were detected by contrast-enhanced CT or gadolinium-ethoxybenzyl diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging performed 38 and 42 days after the first hepatectomy, respectively. CT and magnetic resonance imaging also confirmed the development of intrahepatic venous collaterals between the RHV and MHV . Three-dimensional volumetric software estimated the non-congested FLR volume at 1242 ml (78.5% of TLV) . Both serum aspartate aminotransferase level and alanine aminotransferase level were within normal limits. The 15-min indocyanine green retention rate was considered to keep less than 10% as same as before the first hepatectomy because the patient underwent no chemotherapy during a waiting period; therefore, left hemihepatectomy was performed 58 days after the first hepatectomy. Intraoperatively, we saw no adhesions around the porta hepatis, and the left hepatic artery and the left branch of the portal vein were safely exposed and divided. Intraoperative Doppler ultrasonography confirmed the presence of intrahepatic venous collaterals arising from RHV to MHV . The patient’s postoperative course was uneventful, and he was discharged on postoperative day 9 with no signs of POLF. He underwent eight cycles of panitumumab/FOLFOX therapy for 5 months after the second hepatectomy, and his clinical course is summarized in Fig. 4 . Contrast-enhanced CT performed 10 months after the second hepatectomy revealed that the intrahepatic venous collaterals remained patent . Fig. 1 Preoperative images during the first hepatectomy. a Tumor 1 was located in segment 8 near the caval confluence ( white arrowhead ). The borders between the tumor and the right or the middle hepatic veins were unclear. b Tumor 2 was located in the left lobe and had invaded the umbilical portion of the portal vein ( white arrow ). c Tumor 1 decreased in size after chemotherapy but was still attached to the RHV ( white arrowhead ). d Tumor 1 ( white arrowhead ) and tumor 2 ( white arrow ) were visualized by three-dimensional volumetric software. The volumes of the left liver, the non-congested area, and the congested area were estimated at 19.6% ( green-colored area ), 26.3% ( brown-colored area ), and 53.1% ( blue-colored area ) of the total liver volume, respectively Fig. 2 Pathological findings for tumor 1. a The resected specimen in segment 8 with the right hepatic vein (RHV). b The cut surface of the yellow line in a . Tumor 1 ( white arrowhead ) grossly measured 1.0 × 0.6 cm and had invaded the RHV. c The histological diagnosis was compatible with metastatic carcinoma of the sigmoid colon, and invasion into the RHV was confirmed Fig. 3 Development of intrahepatic venous collaterals ( yellow arrowhead ) between RHV and MHV after the first hepatectomy. a Contrast-enhanced computed tomography. b Contrast-enhanced magnetic resonance imaging. c Three-dimensional volumetric software. d Intraoperative Doppler ultrasonography. Intrahepatic venous collaterals derived from the RHV extended into the MHV ( yellow arrows ) Fig. 4 Trends in the tumor markers over the patient’s treatment course. FOLFOX 5-fluorouracil, leucovorin, and oxaliplatin, CEA serum carcinoembryonic antigen, CA19-9 serum carbohydrate antigen 19-9 Fig. 5 Contrast-enhanced CT performed 10 months after the second hepatectomy. Intrahepatic venous collaterals ( yellow arrowhead ) between the RHV and MHV remained patent	4.167969	0.940918	sec[1]/p[0]	en	0.999997	29453737	https://doi.org/10.1186/s40792-018-0424-5	[ "tumor", "hepatectomy", "hepatic", "vein", "enhanced", "liver", "invaded", "antigen", "contrast", "segment" ]	[ { "code": "2F9Z", "title": "Neoplasms of unknown behaviour of unspecified site" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "2E6Z", "title": "Carcinoma in situ of unspecified site" }, { "code": "2F91.1", "title": "Neoplasms of unknown behaviour of trachea, bronchus or lung" }, { "code": "2F92", "title": "Neoplasms of unknown behaviour of skin" }, { "code": "DB9Z", "title": "Diseases of liver, unspecified" }, { "code": "DB97.Z", "title": "Inflammatory liver disease, unspecified" }, { "code": "DB99.7", "title": "Hepatic failure without mention whether acute or chronic" }, { "code": "LB20.0Y", "title": "Other specified structural developmental anomalies of liver" }, { "code": "LB20.0Z", "title": "Structural developmental anomalies of liver, unspecified" } ]	=== ICD-11 CODES FOUND === [2F9Z] Neoplasms of unknown behaviour of unspecified site Also known as: Neoplasms of unknown behaviour of unspecified site \| neoplasia \| neoplasm growth NOS \| tumour of unspecified site \| tumourlet of unspecified site [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [2E6Z] Carcinoma in situ of unspecified site Also known as: Carcinoma in situ of unspecified site \| carcinoma in situ of unspecified site \| carcinoma in situ NOS \| carcinoma in situ \| in situ neoplasm [2F91.1] Neoplasms of unknown behaviour of trachea, bronchus or lung Also known as: Neoplasms of unknown behaviour of trachea, bronchus or lung \| trachea, bronchus or lung tumour NOS \| Intravascular bronchial alveolar tumour of unspecified site \| Bronchial adenoma of unknown behaviour of unspecified site \| Intravascular bronchial alveolar tumour unknown behaviour of unspecified site [2F92] Neoplasms of unknown behaviour of skin Also known as: Neoplasms of unknown behaviour of skin \| skin tumour NOS [DB9Z] Diseases of liver, unspecified Also known as: Diseases of liver, unspecified \| liver disease \| liver condition NOS \| organ liver disease \| hepatopathy [DB97.Z] Inflammatory liver disease, unspecified Also known as: Inflammatory liver disease, unspecified \| Certain specified inflammatory liver diseases \| Nonspecific reactive hepatitis \| inflammatory liver disease \| hepatitis NOS [DB99.7] Hepatic failure without mention whether acute or chronic Also known as: Hepatic failure without mention whether acute or chronic \| liver decompensation \| liver function failure \| hepatic failure NOS \| liver failure NOS [LB20.0Y] Other specified structural developmental anomalies of liver Also known as: Other specified structural developmental anomalies of liver \| Alagille syndrome \| Alagille-Watson syndrome \| Arteriohepatic dysplasia \| Syndromic bile duct paucity [LB20.0Z] Structural developmental anomalies of liver, unspecified Also known as: Structural developmental anomalies of liver, unspecified \| Structural developmental anomalies of liver \| Malformations of liver \| congenital anomaly of liver \| congenital malformation of liver === GRAPH WALKS === --- Walk 1 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2F91] Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs --- Walk 2 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour --- Walk 3 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Enlarged lymph nodes Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes.... --EXCLUDES--> [?] Nonspecific mesenteric lymphadenitis --- Walk 4 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Enlarged lymph nodes Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes.... --EXCLUDES--> [?] Lymphadenitis --- Walk 5 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs --- Walk 6 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E62] Carcinoma in situ of middle ear or respiratory system	[ "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2F91] Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs", "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Enlarged lymph nodes\n Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes....\n --EXCLUDES--> [?] Nonspecific mesenteric lymphadenitis", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Enlarged lymph nodes\n Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes....\n --EXCLUDES--> [?] Lymphadenitis", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E62] Carcinoma in situ of middle ear or respiratory system" ]	2F9Z	Neoplasms of unknown behaviour of unspecified site	[ { "from_icd11": "2F9Z", "icd10_code": "D487", "icd10_title": "Neoplasm of uncertain behavior of other specified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D482", "icd10_title": "Neoplasm of uncertain behavior of peripheral nerves and autonomic nervous system" }, { "from_icd11": "2F9Z", "icd10_code": "D37-D48", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D377", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D48", "icd10_title": "Neoplasm of uncertain behavior of other and unspecified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D489", "icd10_title": "Neoplasm of uncertain behavior, unspecified" }, { "from_icd11": "ME61", "icd10_code": "R2240", "icd10_title": "Localized swelling, mass and lump, unspecified lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2232", "icd10_title": "Localized swelling, mass and lump, left upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2242", "icd10_title": "Localized swelling, mass and lump, left lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2231", "icd10_title": "Localized swelling, mass and lump, right upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2241", "icd10_title": "Localized swelling, mass and lump, right lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2233", "icd10_title": "Localized swelling, mass and lump, upper limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2243", "icd10_title": "Localized swelling, mass and lump, lower limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2230", "icd10_title": "Localized swelling, mass and lump, unspecified upper limb" }, { "from_icd11": "ME61", "icd10_code": "R220", "icd10_title": "Localized swelling, mass and lump, head" } ]	D487	Neoplasm of uncertain behavior of other specified sites
A 23- year-old Caucasian man of Polish origin was seen in The Head and Neck Cancer Clinic of the National Institute of Oncology in Warsaw with a chief compliant of a left-sided neck lump. The patient had noticed the painless mass 6 months earlier. Other symptoms reported were neck pain, which increased in frequency and intensity over time; general weakness; and occasional breathing difficulty. His medical history was insignificant for any surgeries or medical conditions. The patient denied alcohol use disorder, smoking cigarettes, or any recreational drugs. He was employed as a driver. There was no family history of genetic, metabolic, or neoplastic disorders. On presentation, his vital signs were within normal limits with heart rate of 85, respiratory rate of 126/78 mm Hg, and temperature of 36.7°C. On physical examination, a large round lump on the left side of neck above the clavicle was noticed . It was about 10 cm in diameter, soft on palpation, but firmly attached to the underlying tissue. The skin overlying the mass was normal with no redness or edema. Further investigation revealed a similar mass in the patient's left axillary fossa . The axillary lump was similar to that one on the neck in its structure, but its dimensions were bigger. Initial laboratory workup for the cystic masses included a complete blood count, C-reactive protein, erythrocyte sedimentation rate, alkaline phosphatase (ALP), calcium, vitamin D level, and a comprehensive metabolic panel that did not reveal any abnormality. Infectious workup that included blood and urine cultures was negative. Moreover, immunological tests checking mainly the rheumatoid factor level were within normal ranges as well. In addition, results of hematological tests together with a bone marrow biopsy were also unremarkable. As the laboratory results usually do not reveal any abnormality in Gorham–Stout disease, other diagnostic measures should be applied. The patient was also screened for neoplasms and hereditary disorders, but genetic testing and tumor markers were negative. Our patient was subsequently scheduled for an ultrasound-guided biopsy and followed by a CT scan. Histopathological report of both masses showed thin-walled, engorged capillary-like vessels, filled with blood cells and fibrin. A computed tomography scan showed a nodular polycyclic fluid lesion with transverse dimensions on the neck of approximately 101 mm × 65 mm, which was spreading from the level of the hyoid bone to the level of the third rib on the left side. This lesion in the upper part of the neck was displaced and compressed the infrahyoid muscles and the sternocleidomastoid muscle. In the middle part of the neck, it compressed the left internal jugular vein and the left subclavian vein. Moreover, it widened the left subclavian space and filled the left armpit . In addition, multiple lytic focal lesions were seen in the vertebral bodies. These lesions had sclerotic margins and several thickened trabeculae . The axillary and neck lumps noticed during physical examination were in fact distended lymphatic vessels creating cystic masses. With reference to the CT scan findings, an open thoracic vertebrae biopsy procedure was performed. Histopathological findings showed resorption of the bone matrix and angiomatous tissue with abnormal lymphatic channels. No cellular atypia was found. There were no calcifications and the osteoblastic response was minimal. Following this, our patient was consulted by a hematologist and an orthopedist to rule out multiple myeloma, lymphangiomatosis, and other skeletal or hematologic disorders. Since the diagnosis of Gorham–Stout disease is challenging, we applied the diagnostic criteria proposed by Heffez et al. On the basis of the clinical presentation, imaging studies, and histopathological findings together with the criteria used, we made a diagnosis of Gorham–Stout disease. The presented findings are consistent with a recurrent cystic lymphangioma as a main disease manifestation as the patient did not complain of spine symptoms. Due to the large size of a mass with both cervical and thoracic vertebrae involvement, the surgical treatment aiming to remove the mass was not possible. Taking into consideration the patient's young age and the risk of secondary malignancy, he did not agree to radiotherapy. He was given vitamin D and calcium and was started on a bisphosphonate regimen. Due to the side effects of bisphosphonates such as diarrhea, flu-like symptoms, and bone pain, the patient was reluctant to proceed with this therapy. While there is no effective treatment that is recommended, the patient was finally offered an approach based on axillary and neck lump drainage to relieve his symptoms and decrease body deformity. The procedure was performed under local anesthesia with the use of 2% lidocaine solution and with ultrasound imaging. The 20 G spinal needle was used for the procedure. Before needle insertion, the site was cleaned and disinfected with an antiseptic agent. The needle location was confirmed with ultrasound imaging, and the drained fluid was sent for a cytological examination. Then, neomycin was put on the site of needle insertion and a dressing was made, which was removed on a follow-up visit 2 days later. The patient’s symptoms were successfully managed through the years from 2003 till 2023. The procedure of drainage was conducted every 6 months as that was the period of time when the fluid accumulated and caused the patient’s condition to worsen. Right after the procedure, the patient did not suffer from any complications related to the biopsy itself nor present any signs of infection. Several years later, our patient was commenced on treatment with sirolimus as it is proven to decrease the volume of lymphatic vessels in some patients, but unfortunately, no disease stabilization was achieved. The follow-up plan included repeated MRI imaging every 1–2 years, which eventually revealed a decrease in the size of patient's neck and axillary cystic masses. Laboratory tests including full blood count together with ALP, calcium, and vitamin D were ordered once per year, but no significant changes have been detected so far. Nevertheless, the patents’ symptoms were well controlled for over 20 years with no significant side effects related to the treatment provided. Moreover, the patient seemed to be confident with the presented approach as the regular visits enabled him to stay active and have the disease under control.	4.027344	0.978516	sec[1]/p[0]	en	0.999997	PMC10514479	https://doi.org/10.3389/fsurg.2023.1225209	[ "neck", "axillary", "that", "lump", "which", "side", "cystic", "masses", "blood", "bone" ]	[ { "code": "ME86.C", "title": "Symptom or complaint of the neck" }, { "code": "LA6Z", "title": "Structural developmental anomalies of the neck, unspecified" }, { "code": "ME84.0", "title": "Cervical spine pain" }, { "code": "FA71", "title": "Torticollis" }, { "code": "NA23.4Y", "title": "Other specified strain or sprain of cervical spine" }, { "code": "8B91.Y", "title": "Other specified brachial plexus disorders" }, { "code": "LB62", "title": "Supernumerary breasts" }, { "code": "NC1Z&XA17J1", "title": "Injury of axilla, unspecified" }, { "code": "8C12.3", "title": "Lesion of axillary nerve" }, { "code": "LA90.12", "title": "Lymphatic malformations of certain specified sites" } ]	=== ICD-11 CODES FOUND === [ME86.C] Symptom or complaint of the neck Also known as: Symptom or complaint of the neck \| Neck syndrome Excludes: Chronic primary musculoskeletal pain \| Chronic secondary musculoskeletal pain [LA6Z] Structural developmental anomalies of the neck, unspecified Also known as: Structural developmental anomalies of the neck, unspecified \| Malformations of the neck [ME84.0] Cervical spine pain Definition: This is a condition which is usually characterised by pain or discomfort in the neck region and can be caused by numerous spinal problems. It may be a feature of virtually every disorder and disease that occurs above the shoulder blades. Also known as: Cervical spine pain \| cervical pain \| neck ache \| nonspecific pain in the neck region \| cervicalgia Includes: cervicalgia Excludes: cervical disc degeneration \| Chronic primary cervical pain \| Chronic secondary musculoskeletal pain [FA71] Torticollis Also known as: Torticollis \| contracture of neck \| wry neck \| wry neck/torticollis \| Intermittent torticollis Excludes: Cervical dystonia \| Congenital torticollis \| current injury - see injury of spine by body region [NA23.4Y] Other specified strain or sprain of cervical spine Also known as: Other specified strain or sprain of cervical spine \| Strain of cervical spine \| cervical strain \| Strain of cervical anterior longitudinal ligament \| Sprain of cervical spine [8B91.Y] Other specified brachial plexus disorders Also known as: Other specified brachial plexus disorders \| Sporadic acute brachial plexopathy \| Post radiation brachial plexopathy \| Radiation-induced plexopathy \| Thoracic outlet syndrome [LB62] Supernumerary breasts Definition: A condition caused by failure of the breasts to correctly develop during the antenatal period. This condition is characterised by supernumerary breasts, with or without nipples. This condition may be asymptomatic. Also known as: Supernumerary breasts \| Accessory breast \| polymastia \| breast of axilla [8C12.3] Lesion of axillary nerve Also known as: Lesion of axillary nerve [LA90.12] Lymphatic malformations of certain specified sites Also known as: Lymphatic malformations of certain specified sites \| Lymphatic malformation of conjunctiva \| Lymphangioma of conjunctiva \| Lymphatic malformation of orbit \| Lymphangioma of orbit === GRAPH WALKS === --- Walk 1 --- [ME86.C] Symptom or complaint of the neck --EXCLUDES--> [?] Chronic secondary musculoskeletal pain Def: Chronic secondary musculoskeletal pain is chronic pain arising from bone(s), joint(s), muscle(s), vertebral column, tendon(s) or related soft tissue(s). It is a heterogeneous group of chronic pain con... --EXCLUDES--> [?] Chronic primary pain Def: Chronic primary pain is chronic pain in one or more anatomical regions that is characterised by significant emotional distress (anxiety, anger/frustration or depressed mood) or functional disability (... --- Walk 2 --- [ME86.C] Symptom or complaint of the neck --EXCLUDES--> [?] Chronic primary musculoskeletal pain Def: Chronic primary musculoskeletal pain is chronic pain in the muscles, bones, joints or tendons that is characterised by significant emotional distress (anxiety, anger/frustration or depressed mood) or ... --CHILD--> [?] Chronic primary low back pain Def: Chronic primary low back pain is chronic pain in the muscles, bones, joints or tendons of the lumbar region that is characterised by significant emotional distress (anxiety, anger/frustration or depre... --- Walk 3 --- [LA6Z] Structural developmental anomalies of the neck, unspecified --PARENT--> [?] Structural developmental anomalies of the neck Def: Any condition caused by failure of the neck to correctly develop during the antenatal period.... --CHILD--> [LA60] Webbed neck Def: A condition caused by failure of the tissues of the neck to correctly develop during the antenatal period. This condition is characterised by a broad neck due to lateral folds of skin. This condition ... --- Walk 4 --- [LA6Z] Structural developmental anomalies of the neck, unspecified --PARENT--> [?] Structural developmental anomalies of the neck Def: Any condition caused by failure of the neck to correctly develop during the antenatal period.... --RELATED_TO--> [?] Thyroglossal cyst Def: A firm usually tender cyst varying in size from a few millimetres to several centimetres, usually located anywhere along the embryonic thyroglossal tract between the foramen cecum of the tongue and th... --- Walk 5 --- [ME84.0] Cervical spine pain Def: This is a condition which is usually characterised by pain or discomfort in the neck region and can be caused by numerous spinal problems. It may be a feature of virtually every disorder and disease t... --EXCLUDES--> [?] Chronic primary cervical pain Def: Chronic primary cervical pain is chronic pain in the muscles, bones, joints or tendons of the neck region that is characterised by significant emotional distress (anxiety, anger/frustration or depress... --PARENT--> [?] Chronic primary musculoskeletal pain Def: Chronic primary musculoskeletal pain is chronic pain in the muscles, bones, joints or tendons that is characterised by significant emotional distress (anxiety, anger/frustration or depressed mood) or ... --- Walk 6 --- [ME84.0] Cervical spine pain Def: This is a condition which is usually characterised by pain or discomfort in the neck region and can be caused by numerous spinal problems. It may be a feature of virtually every disorder and disease t... --EXCLUDES--> [?] Intervertebral disc degeneration --PARENT--> [?] Degenerative condition of spine Def: This is a disease characterised by degenerative changes in the intervertebral disc, vertebral end-plates and spinal joints due to aging or structural change....	[ "[ME86.C] Symptom or complaint of the neck\n --EXCLUDES--> [?] Chronic secondary musculoskeletal pain\n Def: Chronic secondary musculoskeletal pain is chronic pain arising from bone(s), joint(s), muscle(s), vertebral column, tendon(s) or related soft tissue(s). It is a heterogeneous group of chronic pain con...\n --EXCLUDES--> [?] Chronic primary pain\n Def: Chronic primary pain is chronic pain in one or more anatomical regions that is characterised by significant emotional distress (anxiety, anger/frustration or depressed mood) or functional disability (...", "[ME86.C] Symptom or complaint of the neck\n --EXCLUDES--> [?] Chronic primary musculoskeletal pain\n Def: Chronic primary musculoskeletal pain is chronic pain in the muscles, bones, joints or tendons that is characterised by significant emotional distress (anxiety, anger/frustration or depressed mood) or ...\n --CHILD--> [?] Chronic primary low back pain\n Def: Chronic primary low back pain is chronic pain in the muscles, bones, joints or tendons of the lumbar region that is characterised by significant emotional distress (anxiety, anger/frustration or depre...", "[LA6Z] Structural developmental anomalies of the neck, unspecified\n --PARENT--> [?] Structural developmental anomalies of the neck\n Def: Any condition caused by failure of the neck to correctly develop during the antenatal period....\n --CHILD--> [LA60] Webbed neck\n Def: A condition caused by failure of the tissues of the neck to correctly develop during the antenatal period. This condition is characterised by a broad neck due to lateral folds of skin. This condition ...", "[LA6Z] Structural developmental anomalies of the neck, unspecified\n --PARENT--> [?] Structural developmental anomalies of the neck\n Def: Any condition caused by failure of the neck to correctly develop during the antenatal period....\n --RELATED_TO--> [?] Thyroglossal cyst\n Def: A firm usually tender cyst varying in size from a few millimetres to several centimetres, usually located anywhere along the embryonic thyroglossal tract between the foramen cecum of the tongue and th...", "[ME84.0] Cervical spine pain\n Def: This is a condition which is usually characterised by pain or discomfort in the neck region and can be caused by numerous spinal problems. It may be a feature of virtually every disorder and disease t...\n --EXCLUDES--> [?] Chronic primary cervical pain\n Def: Chronic primary cervical pain is chronic pain in the muscles, bones, joints or tendons of the neck region that is characterised by significant emotional distress (anxiety, anger/frustration or depress...\n --PARENT--> [?] Chronic primary musculoskeletal pain\n Def: Chronic primary musculoskeletal pain is chronic pain in the muscles, bones, joints or tendons that is characterised by significant emotional distress (anxiety, anger/frustration or depressed mood) or ...", "[ME84.0] Cervical spine pain\n Def: This is a condition which is usually characterised by pain or discomfort in the neck region and can be caused by numerous spinal problems. It may be a feature of virtually every disorder and disease t...\n --EXCLUDES--> [?] Intervertebral disc degeneration\n --PARENT--> [?] Degenerative condition of spine\n Def: This is a disease characterised by degenerative changes in the intervertebral disc, vertebral end-plates and spinal joints due to aging or structural change...." ]	ME86.C	Symptom or complaint of the neck	[ { "from_icd11": "LA6Z", "icd10_code": "Q680", "icd10_title": "Congenital deformity of sternocleidomastoid muscle" }, { "from_icd11": "LA6Z", "icd10_code": "Q180", "icd10_title": "Sinus, fistula and cyst of branchial cleft" }, { "from_icd11": "LA6Z", "icd10_code": "Q188", "icd10_title": "Other specified congenital malformations of face and neck" }, { "from_icd11": "LA6Z", "icd10_code": "Q10-Q18", "icd10_title": "" }, { "from_icd11": "LA6Z", "icd10_code": "Q182", "icd10_title": "Other branchial cleft malformations" }, { "from_icd11": "ME84.0", "icd10_code": "M542", "icd10_title": "Cervicalgia" }, { "from_icd11": "ME84.0", "icd10_code": "M530", "icd10_title": "Cervicocranial syndrome" }, { "from_icd11": "ME84.0", "icd10_code": "M531", "icd10_title": "Cervicobrachial syndrome" }, { "from_icd11": "FA71", "icd10_code": "M436", "icd10_title": "Torticollis" }, { "from_icd11": "FA71", "icd10_code": "M43", "icd10_title": "Other deforming dorsopathies" }, { "from_icd11": "LB62", "icd10_code": "Q831", "icd10_title": "Accessory breast" } ]	Q680	Congenital deformity of sternocleidomastoid muscle
Pseudo-glaucoma: A large physiologic cup with no field loss. I have seen children with a diagnosis of NTG who have simply a large disc with a large physiologic cup. Examination of the parents to discover whether they also have a large physiologic cup is helpful. Some of the new diagnostic instruments will fail to find a thinned retinal nerve fiber layer or a reduced area of the neuro-retinal rim. A congenital anomaly of the disc, perhaps with an arcuate field defect. There may be a notch in the rim of the disc with a colobomatous absence of RPE at the bottom edge of the disc, absent nerve fibers in a wedge from that region, and a corresponding defect. The abnormality can be bilateral. Unilateral cases might have amblyopia since childhood, or even secondary strabismus. Anterior ischemic optic neuropathy. The non-arteritic form typically occurs in eyes with small discs, but if the arteritic form occurs in an eye with a large physiologic cup, the loss of nerve fibers can create a localized thinning or notch in the rim, a feature we have come to associate with glaucoma. The acute episode may be asymptomatic if the affected nerve fibers are not near the fovea. Later the patient may be seen with a large cup and a notched rim, just as in glaucoma, and be associated with a dense field defect in either the upper or lower hemifield. An important clue is that the disc is large. If the disease is unilateral, an erythrocyte sedimentation rate and evaluation for giant cell arteritis might be in order so that treatment might be used to protect the other eye or prevent recurrence in the initially involved eye. Branch retinal vessel occlusion. If the acute event went unnoticed by the patient, there may be an arcuate region of lost nerve fibers and a corresponding visual field defect. If the disc had a physiologic cup of moderate or large size, the neuro-retinal rim may be locally thinned in the region with lost nerve fibers. Of course, an arcuate visual field defect would also be expected. Optic nerve “giant” drusen. Discs with multiple drusen are often obvious. However, we have seen cases in which field defects of nerve fiber bundle topography were found in the presence of a normal IOP. A unilateral case with a tentative diagnosis of NTG was sent for neuro-ophthalmic evaluation, which failed to uncover a cause, so was sent to us for management of NTG. The disc was unusual, however, in having no cupping and did not appear glaucomatous. During careful inspection, a fleeting glisten was seen deep in the disc tissue, and ultimately a small deep drusen was found that corresponded to the location of the visual field defect. Orbital or intracranial tumor. I can recall several cases of a unilateral tumor (such as an orbital meningioma) producing a prechiasmal type of visual field defect. Again, the optic nerves were usually larger than average, with correspondingly large cups. Because of nerve fiber loss, there can be localized thinning of the rim, perhaps subtle, and some pallor that may be difficult to appreciate in the thinned region. Unilateral field defects with symmetry (except for subtle thinning that may be equivocal on examination) of the discs might alert one to this. “High-Pressure” glaucoma Inaccurate tonometry. Applanation tonometers may give a false low reading if the cornea is very thin. Indentation tonometers were known long ago to give false low pressure in large, often myopic, eyes. However, as it has become recognized that NTG is fundamentally the same disease as POAG, and the treatment of both is to lower the IOP, an error of diagnosis on this basis is of little importance. Variable intraocular IOP. The IOP may be abnormally high at certain times of the day, or certain days of the week. Again, the treatment is to lower the IOP, and presumably the treatment will lower the IOP at the times when it is high as well as at the times when it is normal. Past elevation of IOP. Possible causes include chronic use of corticosteroids for contact lens comfort, or uveitis that is no longer active. Chronically intermittent angle closure may also have caused considerable elevation of IOP. Pigmentary glaucoma produces elevated pressure during middle-age years, during which time damage to the optic nerve occurs, but the IOP may return to normal before glaucomatous cupping is found during an eye examination for new glasses or early cataract. Episodes of glaucomatocyclitic crisi may have produced high IOP in the past. Finally, it seems that there may be some cases of “burned out” glaucoma in which changes in aqueous humor dynamics has caused a previously high IOP to return to normal. The importance of some of these is that if damage in the past was due to high IOP that is not likely to recur, the condition may now have become stable without the need for treatment. Chronic POAG in which the pressure has been lowered by systemic medication. Beta blockers, for example, while not as effective in lowering IOP when given systemically may, nonetheless, bring the IOP into the normal range. Special forms of NTG Shock-induced neuropathy. Some cases have been identified in which the patient had a severe cardiovascular event in the past, and an excavated optic nerve is recognized on a later routine eye examination. There may also be cases related to chronic atherosclerosis,[ 8 14 ] obstructive arterial disease rather than dysregulation. These cases may tend to be non-progressive, if the underlying vascular etiologic cause has been corrected, just as ordinary glaucoma is expected to become stable after the inciting elevation of IOP is lowered. Of possible interest is that magnetic resonance imaging (MRI) evidence of microinfarcts was found in 22% of cases with NTG, the approximate percentage of cases that progressed despite treatment in the collaborative normal-tension glaucoma study, and in keeping with the findings that damage in glaucoma seemed to be related to IOP in the presence of vasospastic disease, but not to blood abnormalities associated with occlusive vascular disease. Hypothetically, there might be a form of NTG that represents a primary optic neuropathy unaffected by IOP. Such cases seem not to have been identified with certainty, but in principle lowering the IOP would be irrelevant in the treatment. These cases may be represented by the rare, frustrating cases that continue to be progressive even after the IOP has been lowered to levels accurately determined to be in the range of 8 to 10 mm Hg at all times.	4.285156	0.571777	sec[2]/sec[0]/p[2]	en	0.999996	21150042	https://doi.org/10.4103/0301-4738.73695	[ "that", "cases", "nerve", "large", "glaucoma", "field", "disc", "defect", "optic", "which" ]	[ { "code": "8A80.Z", "title": "Migraine, unspecified" }, { "code": "QA76", "title": "Medication or substance that is known to be an allergen without injury or harm" }, { "code": "PL13.6", "title": "Medication or substance that is known to be an allergen, as mode of injury or harm" }, { "code": "9C40.A0", "title": "Papilloedema" }, { "code": "PA6Z", "title": "Unintentional fall from unspecified height" }, { "code": "JB20.Z", "title": "Single spontaneous delivery, unspecified" }, { "code": "QA48.0", "title": "Care or examination immediately after delivery" }, { "code": "8C1Z", "title": "Mononeuropathy of unspecified site" }, { "code": "ND56.4", "title": "Injury of nerve of unspecified body region" }, { "code": "8B80", "title": "Disorders of olfactory nerve" } ]	=== ICD-11 CODES FOUND === [8A80.Z] Migraine, unspecified Also known as: Migraine, unspecified \| Migraine [QA76] Medication or substance that is known to be an allergen without injury or harm Definition: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm. Also known as: Medication or substance that is known to be an allergen without injury or harm Excludes: Medication or substance that is known to be an allergen, as mode of injury or harm [PL13.6] Medication or substance that is known to be an allergen, as mode of injury or harm Also known as: Medication or substance that is known to be an allergen, as mode of injury or harm \| medication error relating to known allergy to drug or substance \| adverse drug event relating to known allergy to drug or substance [9C40.A0] Papilloedema Definition: Optic disc swelling that results from increased intracranial pressure Also known as: Papilloedema \| Optic disc swelling that results from increased intracranial pressure Includes: Optic disc swelling that results from increased intracranial pressure [PA6Z] Unintentional fall from unspecified height Also known as: Unintentional fall from unspecified height \| fall NOS \| accidental fall \| fall causing injury that resulted in death \| fell NOS [JB20.Z] Single spontaneous delivery, unspecified Also known as: Single spontaneous delivery, unspecified \| Single spontaneous delivery \| spontaneous delivery NOS \| normal delivery NOS \| uncomplicated delivery [QA48.0] Care or examination immediately after delivery Also known as: Care or examination immediately after delivery \| care and observation in uncomplicated delivery cases \| postpartum care immediately after delivery \| postpartum examination immediately after delivery \| Postpartum care after hospital delivery Excludes: Complications predominantly related to the puerperium [8C1Z] Mononeuropathy of unspecified site Also known as: Mononeuropathy of unspecified site \| inflammation of nerve NOS \| nerve condition NOS \| neuritis NOS \| nerve disease NOS [ND56.4] Injury of nerve of unspecified body region Also known as: Injury of nerve of unspecified body region \| injuries to nerves, nerve plexuses and roots \| injury to nerves, unspecified site \| nerve damage NOS \| Injury of nerve NOS Excludes: multiple injuries of nerves NOS [8B80] Disorders of olfactory nerve Also known as: Disorders of olfactory nerve \| disorders of olfactory [1st] nerve \| disorders of the first nerve \| first cranial nerve disorder \| disease of first cranial nerve Includes: Disorder of 1st cranial nerve Excludes: Idiopathic anosmia \| Idiopathic parosmia === GRAPH WALKS === --- Walk 1 --- [8A80.Z] Migraine, unspecified --PARENT--> [8A80] Migraine Def: A primary headache disorder, in most cases episodic. Disabling attacks lasting 4-72 hours are characterised by moderate or severe headache, usually accompanied by nausea, vomiting and/or photophobia a... --EXCLUDES--> [?] Headache, not elsewhere classified Def: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above.... --- Walk 2 --- [8A80.Z] Migraine, unspecified --PARENT--> [8A80] Migraine Def: A primary headache disorder, in most cases episodic. Disabling attacks lasting 4-72 hours are characterised by moderate or severe headache, usually accompanied by nausea, vomiting and/or photophobia a... --EXCLUDES--> [?] Headache, not elsewhere classified Def: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above.... --- Walk 3 --- [QA76] Medication or substance that is known to be an allergen without injury or harm Def: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm.... --EXCLUDES--> [?] Medication or substance that is known to be an allergen, as mode of injury or harm --PARENT--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance --- Walk 4 --- [QA76] Medication or substance that is known to be an allergen without injury or harm Def: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm.... --PARENT--> [?] Circumstances associated with exposure to a drug, medicament or biological substance influencing the episode of care without injury or harm --PARENT--> [?] Health care related circumstances influencing the episode of care without injury or harm --- Walk 5 --- [PL13.6] Medication or substance that is known to be an allergen, as mode of injury or harm --PARENT--> [PL13] Mode of injury or harm associated with exposure to a drug, medicament or biological substance --CHILD--> [PL13.2] Drug-related injury or harm in the context of correct administration or dosage, as mode of injury or harm --- Walk 6 --- [PL13.6] Medication or substance that is known to be an allergen, as mode of injury or harm --PARENT--> [PL13] Mode of injury or harm associated with exposure to a drug, medicament or biological substance --CHILD--> [PL13.2] Drug-related injury or harm in the context of correct administration or dosage, as mode of injury or harm	[ "[8A80.Z] Migraine, unspecified\n --PARENT--> [8A80] Migraine\n Def: A primary headache disorder, in most cases episodic. Disabling attacks lasting 4-72 hours are characterised by moderate or severe headache, usually accompanied by nausea, vomiting and/or photophobia a...\n --EXCLUDES--> [?] Headache, not elsewhere classified\n Def: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above....", "[8A80.Z] Migraine, unspecified\n --PARENT--> [8A80] Migraine\n Def: A primary headache disorder, in most cases episodic. Disabling attacks lasting 4-72 hours are characterised by moderate or severe headache, usually accompanied by nausea, vomiting and/or photophobia a...\n --EXCLUDES--> [?] Headache, not elsewhere classified\n Def: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above....", "[QA76] Medication or substance that is known to be an allergen without injury or harm\n Def: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm....\n --EXCLUDES--> [?] Medication or substance that is known to be an allergen, as mode of injury or harm\n --PARENT--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance", "[QA76] Medication or substance that is known to be an allergen without injury or harm\n Def: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm....\n --PARENT--> [?] Circumstances associated with exposure to a drug, medicament or biological substance influencing the episode of care without injury or harm\n --PARENT--> [?] Health care related circumstances influencing the episode of care without injury or harm", "[PL13.6] Medication or substance that is known to be an allergen, as mode of injury or harm\n --PARENT--> [PL13] Mode of injury or harm associated with exposure to a drug, medicament or biological substance\n --CHILD--> [PL13.2] Drug-related injury or harm in the context of correct administration or dosage, as mode of injury or harm", "[PL13.6] Medication or substance that is known to be an allergen, as mode of injury or harm\n --PARENT--> [PL13] Mode of injury or harm associated with exposure to a drug, medicament or biological substance\n --CHILD--> [PL13.2] Drug-related injury or harm in the context of correct administration or dosage, as mode of injury or harm" ]	8A80.Z	Migraine, unspecified	[ { "from_icd11": "8A80.Z", "icd10_code": "G43B0", "icd10_title": "Ophthalmoplegic migraine, not intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43409", "icd10_title": "Hemiplegic migraine, not intractable, without status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43A0", "icd10_title": "Cyclical vomiting, in migraine, not intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43D0", "icd10_title": "Abdominal migraine, not intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43709", "icd10_title": "Chronic migraine without aura, not intractable, without status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43A1", "icd10_title": "Cyclical vomiting, in migraine, intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43509", "icd10_title": "Persistent migraine aura without cerebral infarction, not intractable, without status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43719", "icd10_title": "Chronic migraine without aura, intractable, without status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43501", "icd10_title": "Persistent migraine aura without cerebral infarction, not intractable, with status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43C0", "icd10_title": "Periodic headache syndromes in child or adult, not intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43401", "icd10_title": "Hemiplegic migraine, not intractable, with status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43419", "icd10_title": "Hemiplegic migraine, intractable, without status migrainosus" }, { "from_icd11": "8A80.Z", "icd10_code": "G43B1", "icd10_title": "Ophthalmoplegic migraine, intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43C1", "icd10_title": "Periodic headache syndromes in child or adult, intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43D1", "icd10_title": "Abdominal migraine, intractable" } ]	G43B0	Ophthalmoplegic migraine, not intractable
A 73-year-old Asian woman with an underlying anxiety disorder, functional headache, and hypertension was prescribed escitalopram and lorazepam when she presented with progressively worsening headaches to her primary care doctor. Her symptoms did not improve with the medications, and she was unable to eat well and required bed rest. She was transported to our hospital 4 days later after developing chest and back pain with altered consciousness. She was a housekeeper, had no allergies, and had no alcohol or tobacco smoking history. On arrival, her Glasgow Coma Scale score was 3/15 (E1V1M1); both pupils were approximately 4 mm in diameter and reactive. Her blood pressure was too low to be measured, her carotid artery pulse was palpable, her heart rate was 112 beats/minute, and her respiratory rate was 30 breaths/minute. Her conjunctiva was pale. An auscultation of breath sounds did not reveal upper and lower airway obstructions and was within normal limits. Her abdomen was soft and flat without tenderness. She had no skin abnormalities (such as rash). Both legs had no edema. Echocardiography on arrival was performed as point of care ultrasound and revealed a hypercontractile left ventricle with an eliminated left ventricular cavity and a collapsed inferior vena cava without right ventricular dilation. There was no pericardial effusion or obvious large regurgitant jet observed on color Doppler. In response, we immediately inserted a peripheral venous catheter and began introducing fluid resuscitation; however, she developed PEA. Conventional CPR according to the adult advanced cardiovascular life support guidelines (including adrenaline) was initiated and a return of spontaneous circulation (ROSC) occurred. However, her blood pressure was unstable and PEA returned, prompting repeated CPR with immediate administration of fluids and three adrenaline injections. Venoarterial extracorporeal membrane oxygenation (VA-ECMO) was initiated for refractory PEA. Whole-body contrast-enhanced computed tomography was unremarkable, and the admission laboratory results were also unremarkable, except for anemia (Table 1 ). Her hemoglobin level decreased from 7.1 g/dL to 3.5 g/dL 1 hour later without obvious signs of gastrointestinal hemorrhage. Therefore, 8 units of packed red blood cells were transfused for 1 day, after which her hemodynamic status stabilized. She was in a coma without sedatives; thus, targeted temperature management at 34 °C was initiated on admission to an intensive care unit. Echocardiography in the intensive care unit showed a thickened interventricular septum (which was 12.8 mm), prolonged anterior mitral valve, and contact between the bodies of the anterior and posterior mitral valves, suggesting that the left ventricular obstruction could have potentially occurred through this redundant anterior mitral valve. VA-ECMO was terminated on day 3, and after stabilizing her hemodynamics, transthoracic echocardiography showed a sigmoid septum with normal left ventricular function (ejection fraction, 75%) . On day 26, dobutamine-infused (30 μg/kg per minute) Doppler echocardiography revealed a significant outflow gradient (236 mmHg) accompanied with chest pain and intermittent systolic anterior motion (SAM) of the mitral valve; continuous monitoring during Doppler echocardiography showed a Brockenbrough–Braunwald sign , which is a fall of arterial blood pressure after premature ventricular contraction; these findings confirmed a diagnosis of latent LVOTO due to a sigmoid septum. The significant LVOTO was not dependent on SAM but might have occurred due to the greatly thickened interventricular septum. As a result, carvedilol was initiated with gradual increment up to 10 mg/day on day 35. In addition, verapamil (120 mg/day) was administered on day 29. A follow-up dobutamine-infused Doppler echocardiography on day 40 showed a reduction of the outflow gradient to 14 mmHg, indicating a successful medical therapy. Table 1 Laboratory data on day 1 Patient’s result Reference ranges Complete blood count White blood cell count (/μL) 11,400 2700–10,300 Red blood cell count (× 10,000/μL) 254 357–497 Hemoglobin (g/dL) 7.1 11.4–14.2 Hematocrit (%) 20.7 32.3–43.1 Reticulocyte count (%) 13.4 0.2–2.7 Platelet (× 10,000/μL) 24.8 13.0–35.0 Coagulation test Prothrombin time (%) 97.6 75–120 International normalized ratio of prothrombin time (seconds) 0.99 Activated partial thromboplastin time (seconds) 24.3 24–39 Biochemical test Total protein (g/dL) 6.3 6.0–8.3 Albumin (g/dL) 3.6 3.6–5.1 Blood urea nitrogen (mg/dL) 12 7.0–20.0 Creatinine (mg/dL) 0.8 0.4–0.9 Total bilirubin (mg/dL) 3.1 0.2–1.1 Direct bilirubin (mg/dL) 0.7 < 0.4 Sodium (mEq/L) 135 135–147 Potassium (mEq/L) 3.8 3.6–5.1 Chlorine (mEq/L) 100 98–108 Creatine kinase (U/L) 99 30–150 Creatine kinase-MB (U/L) 11 < 20 Aspartate aminotransferase (U/L) 79 10–37 Alanine aminotransferase (U/L) 45 6–35 Lactate dehydrogenase (U/L) 833 115–250 Alkaline phosphatase (U/L) 240 122–378 Gamma-glutamyl transpeptidase (U/L) 21 1–40 Iron (μg/dL) 143 55–180 Total iron binding capacity (μg/dL) 132 130–320 Unsaturated iron binding capacity (μg/dL) 275 260–420 Glucose (mg/dL) 391 60–110 Hemoglobin A1c (%) 5.1 4.6–6.2 Brain natriuretic peptide (pg/mL) 81.7 0–18.4 Blood gas analysis (10 L/minute O 2 given) pH 7.105 7.35–7.45 PaO 2 (mmHg) 129 ≧ 80 PaCO 2 (mmHg) 37.8 35–45 Hydrogen-carbonate ion (mmol/L) 11.4 22–26 Lactate (mmmol/L) 12.9 0.56–1.39 Anion gap (mmol/L) 27.6 20–26 PaCO 2 partial pressure of carbon dioxide in arterial blood, PaO 2 partial pressure of oxygen in arterial blood Fig. 1 Transthoracic echocardiography on day 10. In the parasternal long axis view ( a ) and four-chamber view ( b ), echocardiography revealed a morphological characteristic of the basal interventricular septum that protrudes into the left ventricular cavity Fig. 2 Provocation with dobutamine infusion (30 μg/kg per minute) during Doppler echocardiography on day 26. Echocardiography reveals a left ventricular outflow gradient of 236 mmHg, which confirms a diagnosis of latent left ventricular outflow tract obstruction. LVOT left ventricular outflow tract Fig. 3 Brockenbrough–Braunwald sign during dobutamine-infused (30 μg/kg per minute) Doppler echocardiography on day 26. Continuous electrocardiogram and invasive radial arterial blood pressure show a Brockenbrough–Braunwald sign, which is a fall of arterial blood pressure ( arrow ) after a premature ventricular contraction ( arrowhead )	3.958984	0.976563	sec[1]/p[0]	en	0.999995	30122151	https://doi.org/10.1186/s13256-018-1767-z	[ "blood", "echocardiography", "ventricular", "pressure", "minute", "doppler", "which", "septum", "outflow", "mmhg" ]	[ { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" }, { "code": "MA12.1", "title": "Finding of cocaine in blood" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "MA12.2", "title": "Finding of hallucinogen in blood" }, { "code": "LA89.Z", "title": "Functionally univentricular heart, unspecified" }, { "code": "BC45", "title": "Cardiomegaly" }, { "code": "BA41.Z", "title": "Acute myocardial infarction, unspecified" }, { "code": "BC46&XA7XU8", "title": "Ventricular thrombosis" }, { "code": "BD1Z&XT5R", "title": "Acute heart failure" } ]	=== ICD-11 CODES FOUND === [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS [MA12.1] Finding of cocaine in blood Also known as: Finding of cocaine in blood \| cocaine in blood [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [MA12.2] Finding of hallucinogen in blood Also known as: Finding of hallucinogen in blood \| hallucinogen in blood [LA89.Z] Functionally univentricular heart, unspecified Also known as: Functionally univentricular heart, unspecified \| Functionally univentricular heart \| Univentricular cardiopathy \| Single ventricle \| univentricular heart [BC45] Cardiomegaly Also known as: Cardiomegaly \| enlargement of heart \| hypertrophic heart \| heart hypertrophy \| Cardiac hypertrophy Includes: Left ventricular hyperplasia [BA41.Z] Acute myocardial infarction, unspecified Also known as: Acute myocardial infarction, unspecified \| Acute myocardial infarction \| cardiac attack \| heart attack \| acute cardiac infarction === GRAPH WALKS === --- Walk 1 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --EXCLUDES--> [?] Certain conditions originating in the perinatal period Def: This chapter includes conditions that have their origin in the perinatal period even though death or morbidity occurs later.... --- Walk 2 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --CHILD--> [?] Diseases of spleen --- Walk 3 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --CHILD--> [MF50.4Z] Haematuria, unspecified --- Walk 4 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --PARENT--> [MF50] Abnormal micturition --- Walk 5 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.1] Finding of cocaine in blood --- Walk 6 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.2] Finding of hallucinogen in blood	[ "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --EXCLUDES--> [?] Certain conditions originating in the perinatal period\n Def: This chapter includes conditions that have their origin in the perinatal period even though death or morbidity occurs later....", "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --CHILD--> [?] Diseases of spleen", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --CHILD--> [MF50.4Z] Haematuria, unspecified", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --PARENT--> [MF50] Abnormal micturition", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.1] Finding of cocaine in blood", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.2] Finding of hallucinogen in blood" ]	3C0Z	Diseases of the blood or blood-forming organs, unspecified	[ { "from_icd11": "3C0Z", "icd10_code": "D75A", "icd10_title": "Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia" }, { "from_icd11": "3C0Z", "icd10_code": "D7581", "icd10_title": "Myelofibrosis" }, { "from_icd11": "3C0Z", "icd10_code": "D7582", "icd10_title": "Heparin induced thrombocytopenia (HIT)" }, { "from_icd11": "3C0Z", "icd10_code": "D7589", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D759", "icd10_title": "Disease of blood and blood-forming organs, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "D763", "icd10_title": "Other histiocytosis syndromes" }, { "from_icd11": "3C0Z", "icd10_code": "Q899", "icd10_title": "Congenital malformation, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "III", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D70-D77", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D75", "icd10_title": "Other and unspecified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D77", "icd10_title": "Other disorders of blood and blood-forming organs in diseases classified elsewhere" }, { "from_icd11": "3C0Z", "icd10_code": "D758", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D76", "icd10_title": "Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue" }, { "from_icd11": "MF50.4Z", "icd10_code": "R310", "icd10_title": "Gross hematuria" }, { "from_icd11": "MF50.4Z", "icd10_code": "R312", "icd10_title": "Other microscopic hematuria" } ]	D75A	Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia
The patient was a 48-year-old man diagnosed with ARVC due to PKP2 mutation and constitutional jaundice. At the age of 35 years, catheter ablation of the right ventricular apical septum was performed for sustained VT with left bundle branch block-type and superior QRS axis. The thyroid function was euthyroid at the time; the thyroid-stimulating hormone (TSH) level was 1.53 (normal range, 0.5–5.5) μ U/mL, the free triiodothyronine (T3) level was 3.6 (normal range, 2.6–4.6) pg/mL, and the free thyroxine (T4) level was 1.5 (normal range, 1.1–1.8) ng/dL. Thereafter, under daily metoprolol intake of 80 mg, VT did not recur. He complained of palpitation six months before admission, and 80 mg/day sotalol was started. His laboratory data are shown in Table 1 . Although palpitation was reduced, he had general fatigue and discontinued taking sotalol one month before consultation. He presented to our emergency department with worsening dyspnea, bilateral lower leg edema, a 5 kg weight gain, and watery diarrhea. As for vital signs, he was alert, his blood pressure was 123/87 mmHg, his heart rate was 150/min and irregular, his blood oxygen saturation was 95% (room air), and his body temperature was 37.7°C. Physical examination revealed jugular venous distention, coarse crackles in both lungs, pitting edema in the legs, and tremors of the upper limbs. Exophthalmos and ocular motility disturbances were not observed. A chest radiograph and electrocardiogram showed cardiac dilation, bilateral pleural effusion, and a rapid atrial fibrillation (AF) rhythm (Figures 1(a) and 2 ). Transthoracic echocardiography clarified dilation of the intravenous cava, right atrium, and right ventricle (RVDd = 57 mm), decreased right ventricular contractile function (TAPSE = 8 mm), compression of the left ventricle by the dilated right ventricle, and tricuspid regurgitation (peak pressure gradient = 15 mmHg) at the right side. LVDd and LVDs were 46 mm and 33 mm, respectively; however, they might have been underestimated owing to compression. Furthermore, a decreased ejection fraction (visual 30%) and absence of vegetation at the valves were observed. The plasma brain natriuretic peptide (BNP) and serum total bilirubin levels were elevated ( Table 1 ). He was diagnosed with both-sided acute heart failure with rapid AF and admitted to the coronary care unit (CCU). The SOFA and APACHE II scores at admission were 2 and 8, respectively. He was given 80 mg/day intravenous furosemide and 0.25 mg intravenous digoxin, and heparinization was started. After admission, sustained VT occurred, so an electrical cardioversion was performed and intravenous amiodarone was administered . Moreover, atrial tachycardia, for which both 50 mg intravenous lidocaine and 20 milliequivalent magnesium sulfate were ineffective, occurred and required electrical cardioversion again, and 1 μ g/kg/min intravenous landiolol was initiated . Thereafter, VT reoccurred; thus, electrical cardioversion was reperformed, and intravenous landiolol was increased to 3 μ g/kg/min. Blood chemistry revealed suppressed TSH level (<0.01 μ U/mL) and elevated free T3 (16.4 pg/mL) and free T4 levels (>7.7 ng/dL), and the patient was referred for hyperthyroidism. Thyrotropin receptor antibody (TRAb) measured using the electrochemiluminescence assay method was 24.7 (normal range, <2.0) IU/L, and goiter with increased blood flow was confirmed by ultrasonography ( Figure 1(b) ). Hence, the patient was diagnosed with Graves' disease. The total bilirubin level increased to 5.6 mg/dL at that time. Thus, he fulfilled the following diagnostic criteria for TS proposed by the Japan Thyroid Association : thyrotoxicosis, tachycardia, and gastrointestinal/hepatic manifestations, and the findings in this case correspond to TS2 (suspected TS). The Burch–Wartofsky Point Scale (BWPS) score, which was proposed in 1993 and had been widely used for the diagnosis of TS, of 45 and >45 is suggestive of TS . Although the total bilirubin level of this patient could not be appropriately evaluated due to constitutional jaundice, BWPS was 60 even when the heading of “gastrointestinal-hepatic dysfunction” among the diagnostic criteria was underscored as “moderate” (if a patient showed jaundice, it was scored as “severe”). Therefore, he was diagnosed with TS, and treatment with 150 mg/day intravenous hydrocortisone, 30 mg/day oral methimazole (MMI), and 200 mg/day potassium iodide (KI) was initiated. After initial treatment of the TS, lethal arrhythmia including VT did not reoccur, and he returned to a normal sinus rhythm several days later . As thyroid function gradually decreased, intravenous hydrocortisone was tapered off and altered to oral prednisolone (PSL), and KI was decreased to 100 mg/day. Regarding his cardiac function, when he was referred for hyperthyroidism, the central venous pressure (CVP) decreased from 25 mmHg at admission to 17 mmHg, and compression of the left ventricle was substantially reduced. However, EF did not improve after the abovementioned therapy. Treatment for TS was started at this time, and the echocardiography performed 6 days after the hyperthyroid treatment showed improvement in contractile function and dilation of the left ventricle. The EF was 60%–65%, and the LVDds and LVDs were 42 mm and 28 mm, respectively. In addition, right ventricular dilation and function improved (RVDd = 52 mm; TAPSE = 11.7 mm). The thyroid function test results 6 days after the initiation of TS therapy were as follows: free T3 = 3.4 pg/mL and free T4 = 3.7 ng/dL . The free T4 level remained high, but the free T3 level returned to normal. Consequently, furosemide and landiolol were also tapered off and discontinued, and amiodarone was adjusted according to the serum concentration and shifted to oral administration. Although an implantable cardioverter-defibrillator placement was recommended for possibly recurrent VT , the patient declined. Finally, symptoms such as dyspnea, leg edema, and watery diarrhea improved, and his body weight reduced approximately 10 kg compared with admission data, so he was discharged on day 28. When he left the hospital, his medications were 2.5 mg PSL on alternate days, 30 mg/day MMI and 100 mg/day KI for hyperthyroidism, 10 mg/day apixaban for anticoagulation, 2.5 mg/day bisoprolol and 150 mg/day amiodarone for the prevention of arrhythmia, and 2.5 mg/day enalapril for heart failure. His blood test results at the time of discharge are shown in Table 1 .	4.105469	0.97168	sec[1]/p[0]	en	0.999997	PMC9246615	https://doi.org/10.1155/2022/6078148	[ "intravenous", "free", "function", "thyroid", "blood", "ventricle", "diagnosed", "time", "range", "mmhg" ]	[ { "code": "QE11.Z", "title": "Hazardous drug use, unspecified" }, { "code": "PK91.16", "title": "Cardiovascular devices associated with injury or harm: peripheral venous catheter" }, { "code": "QE11.3", "title": "Hazardous use of cocaine" }, { "code": "JA61.5", "title": "Cerebral venous thrombosis in pregnancy" }, { "code": "8B22.1", "title": "Cerebral venous thrombosis" }, { "code": "FA34.0", "title": "Loose body in joint" }, { "code": "5C56.4", "title": "Disorders of sialic acid metabolism" }, { "code": "2B6A.Z", "title": "Malignant neoplasms of oropharynx, unspecified" }, { "code": "DB10.00", "title": "Acute appendicitis with generalised peritonitis" }, { "code": "DD91.Z", "title": "Irritable bowel syndrome or functional bowel disorders, unspecified" } ]	=== ICD-11 CODES FOUND === [QE11.Z] Hazardous drug use, unspecified Also known as: Hazardous drug use, unspecified \| Hazardous drug use \| chronic drug use NOS \| chronic IV substance use \| drug use nos [PK91.16] Cardiovascular devices associated with injury or harm: peripheral venous catheter Also known as: Cardiovascular devices associated with injury or harm: peripheral venous catheter \| complication of intravenous line \| IV - [intravenous] line complication Excludes: Circumstances associated with a surgical or other medical device influencing the episode of care without injury or harm [QE11.3] Hazardous use of cocaine Definition: A pattern of cocaine use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health professionals. The increased risk may be from the frequency of cocaine use, from the amount used on a given occasion, from risky behaviours associated with cocaine use or the context of use, from a harmful route of administration, or from a combination of these. The risk may be related to short-term eff Also known as: Hazardous use of cocaine \| cocaine use \| intravenous cocaine use \| Hazardous use of crack cocaine \| crack cocaine use Excludes: Disorders due to use of cocaine [JA61.5] Cerebral venous thrombosis in pregnancy Also known as: Cerebral venous thrombosis in pregnancy \| Cerebrovenous sinus thrombosis in pregnancy \| cerebral venous thrombosis in pregnancy, unspecified trimester \| intracranial venous sinus thrombosis of pregnancy Includes: Cerebrovenous sinus thrombosis in pregnancy [8B22.1] Cerebral venous thrombosis Definition: Thrombosis (blood clot) of the cerebral venous sinuses, which drain blood from brain Also known as: Cerebral venous thrombosis \| nonpyogenic thrombosis of intracranial venous system \| nonpyogenic thrombophlebitis of intracranial venous sinus \| nonpyogenic thrombosis of intracranial venous sinus \| Nonpyogenic thrombosis of cerebral vein Excludes: Cerebral ischaemic stroke \| Cerebral venous thrombosis in the puerperium [FA34.0] Loose body in joint Also known as: Loose body in joint \| arthrolith \| corpora libra in joint \| free bodies in joint \| intra-articular loose body Excludes: Loose body in knee [5C56.4] Disorders of sialic acid metabolism Definition: This refers to any disorders of the N- or O-substituted derivatives of neuraminic acid, a monosaccharide with a nine-carbon backbone. Also known as: Disorders of sialic acid metabolism \| Free sialic acid storage disease \| Salla disease \| Finnish type sialuria \| Sialuria [2B6A.Z] Malignant neoplasms of oropharynx, unspecified Also known as: Malignant neoplasms of oropharynx, unspecified \| Malignant neoplasms of oropharynx \| oropharyngeal cancer \| Malignant neoplasm of anterior surface of epiglottis \| primary malignant neoplasm of Vallecula [DB10.00] Acute appendicitis with generalised peritonitis Definition: This is a condition characterised by acute inflammation of the vermiform appendix that is extending into the free, not contained, inflammation of the peritoneum. There is usually a free perforation and surgical treatment is recommended. Also known as: Acute appendicitis with generalised peritonitis \| acute appendicitis with diffuse peritonitis following rupture or perforation \| Acute appendicitis with free peritonitis \| Acute appendicitis with free perforation to the abdominal cavity \| perforated appendix with generalised peritonitis Includes: Acute appendicitis with free perforation to the abdominal cavity \| acute appendicitis with diffuse peritonitis following rupture or perforation [DD91.Z] Irritable bowel syndrome or functional bowel disorders, unspecified Also known as: Irritable bowel syndrome or functional bowel disorders, unspecified \| Irritable bowel syndrome or certain specified functional bowel disorders \| Functional intestinal disorders NOS === GRAPH WALKS === --- Walk 1 --- [QE11.Z] Hazardous drug use, unspecified --PARENT--> [QE11] Hazardous drug use Def: A pattern of use of psychoactive substance(s) other than nicotine or alcohol that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent... --CHILD--> [QE11.1] Hazardous use of cannabis Def: A pattern of cannabis use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health pro... --- Walk 2 --- [QE11.Z] Hazardous drug use, unspecified --PARENT--> [QE11] Hazardous drug use Def: A pattern of use of psychoactive substance(s) other than nicotine or alcohol that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent... --EXCLUDES--> [?] Disorders due to substance use Def: Disorders due to substance use include disorders that result from a single occasion or repeated use of substances that have psychoactive properties, including certain medications. Disorders related to... --- Walk 3 --- [PK91.16] Cardiovascular devices associated with injury or harm: peripheral venous catheter --EXCLUDES--> [?] Circumstances associated with a surgical or other medical device influencing the episode of care without injury or harm --CHILD--> [?] Dislodgement, misconnection or de-attachment of a surgical or medical device without injury or harm Def: A device that has moved out of place, become disconnected, loosened or unstable, but without documented injury or harm.... --- Walk 4 --- [PK91.16] Cardiovascular devices associated with injury or harm: peripheral venous catheter --EXCLUDES--> [?] Circumstances associated with a surgical or other medical device influencing the episode of care without injury or harm --CHILD--> [?] Functional device failure without injury or harm Def: A device not working or operating correctly, or that has stopped functioning after a period of function, but without documented injury or harm to the patient.... --- Walk 5 --- [QE11.3] Hazardous use of cocaine Def: A pattern of cocaine use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health prof... --EXCLUDES--> [?] Disorders due to use of cocaine Def: Disorders due to use of cocaine are characterised by the pattern and consequences of cocaine use. Cocaine is a compound found in the leaves of the coca plant, Erythroxylum coca, which is indigenous to... --EXCLUDES--> [?] Disorders due to use of stimulants including amphetamines, methamphetamine or methcathinone Def: Disorders due to use of stimulants including amphetamines, methamphetamine or methcathinone are characterised by the pattern and consequences of use of these substances. There is a wide array of natur... --- Walk 6 --- [QE11.3] Hazardous use of cocaine Def: A pattern of cocaine use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health prof... --PARENT--> [QE11] Hazardous drug use Def: A pattern of use of psychoactive substance(s) other than nicotine or alcohol that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent... --EXCLUDES--> [?] Disorders due to substance use Def: Disorders due to substance use include disorders that result from a single occasion or repeated use of substances that have psychoactive properties, including certain medications. Disorders related to...	[ "[QE11.Z] Hazardous drug use, unspecified\n --PARENT--> [QE11] Hazardous drug use\n Def: A pattern of use of psychoactive substance(s) other than nicotine or alcohol that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent...\n --CHILD--> [QE11.1] Hazardous use of cannabis\n Def: A pattern of cannabis use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health pro...", "[QE11.Z] Hazardous drug use, unspecified\n --PARENT--> [QE11] Hazardous drug use\n Def: A pattern of use of psychoactive substance(s) other than nicotine or alcohol that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent...\n --EXCLUDES--> [?] Disorders due to substance use\n Def: Disorders due to substance use include disorders that result from a single occasion or repeated use of substances that have psychoactive properties, including certain medications. Disorders related to...", "[PK91.16] Cardiovascular devices associated with injury or harm: peripheral venous catheter\n --EXCLUDES--> [?] Circumstances associated with a surgical or other medical device influencing the episode of care without injury or harm\n --CHILD--> [?] Dislodgement, misconnection or de-attachment of a surgical or medical device without injury or harm\n Def: A device that has moved out of place, become disconnected, loosened or unstable, but without documented injury or harm....", "[PK91.16] Cardiovascular devices associated with injury or harm: peripheral venous catheter\n --EXCLUDES--> [?] Circumstances associated with a surgical or other medical device influencing the episode of care without injury or harm\n --CHILD--> [?] Functional device failure without injury or harm\n Def: A device not working or operating correctly, or that has stopped functioning after a period of function, but without documented injury or harm to the patient....", "[QE11.3] Hazardous use of cocaine\n Def: A pattern of cocaine use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health prof...\n --EXCLUDES--> [?] Disorders due to use of cocaine\n Def: Disorders due to use of cocaine are characterised by the pattern and consequences of cocaine use. Cocaine is a compound found in the leaves of the coca plant, Erythroxylum coca, which is indigenous to...\n --EXCLUDES--> [?] Disorders due to use of stimulants including amphetamines, methamphetamine or methcathinone\n Def: Disorders due to use of stimulants including amphetamines, methamphetamine or methcathinone are characterised by the pattern and consequences of use of these substances. There is a wide array of natur...", "[QE11.3] Hazardous use of cocaine\n Def: A pattern of cocaine use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health prof...\n --PARENT--> [QE11] Hazardous drug use\n Def: A pattern of use of psychoactive substance(s) other than nicotine or alcohol that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent...\n --EXCLUDES--> [?] Disorders due to substance use\n Def: Disorders due to substance use include disorders that result from a single occasion or repeated use of substances that have psychoactive properties, including certain medications. Disorders related to..." ]	QE11.Z	Hazardous drug use, unspecified	[ { "from_icd11": "QE11.Z", "icd10_code": "Z722", "icd10_title": "" }, { "from_icd11": "PK91.16", "icd10_code": "T8242XA", "icd10_title": "Displacement of vascular dialysis catheter, initial encounter" }, { "from_icd11": "PK91.16", "icd10_code": "T8249XD", "icd10_title": "Other complication of vascular dialysis catheter, subsequent encounter" }, { "from_icd11": "PK91.16", "icd10_code": "T8243XA", "icd10_title": "Leakage of vascular dialysis catheter, initial encounter" }, { "from_icd11": "PK91.16", "icd10_code": "T824", "icd10_title": "Mechanical complication of vascular dialysis catheter" }, { "from_icd11": "JA61.5", "icd10_code": "O2251", "icd10_title": "Cerebral venous thrombosis in pregnancy, first trimester" }, { "from_icd11": "JA61.5", "icd10_code": "O2252", "icd10_title": "Cerebral venous thrombosis in pregnancy, second trimester" }, { "from_icd11": "JA61.5", "icd10_code": "O2253", "icd10_title": "Cerebral venous thrombosis in pregnancy, third trimester" }, { "from_icd11": "JA61.5", "icd10_code": "O225", "icd10_title": "Cerebral venous thrombosis in pregnancy" }, { "from_icd11": "8B22.1", "icd10_code": "G08", "icd10_title": "Intracranial and intraspinal phlebitis and thrombophlebitis" }, { "from_icd11": "8B22.1", "icd10_code": "I676", "icd10_title": "Nonpyogenic thrombosis of intracranial venous system" }, { "from_icd11": "8B22.1", "icd10_code": "I636", "icd10_title": "Cerebral infarction due to cerebral venous thrombosis, nonpyogenic" }, { "from_icd11": "FA34.0", "icd10_code": "M24012", "icd10_title": "Loose body in left shoulder" }, { "from_icd11": "FA34.0", "icd10_code": "M24011", "icd10_title": "Loose body in right shoulder" }, { "from_icd11": "FA34.0", "icd10_code": "M24032", "icd10_title": "Loose body in left wrist" } ]	Z722
Patient 1 is a 75-year old male, who was originally diagnosed with stage IIIB, BRAF-negative melanoma of the upper back and left axillary lymph node (LN) involvement in 2012, treated with wide local excision (Breslow thickness: 2.9 mm) and axillary LN dissection. The patient received adjuvant therapy with a GM-CSF secreting allogeneic melanoma cell vaccine for 3-years, but developed recurrent disease at the right buttock, inguinal nodes and lung in 2015, and was treated with first-line pembrolizumab monotherapy. He received 25 total doses and sustained a radiologic complete response to therapy by RECIST 1.1 v.5.0. After 20 doses of pembrolizumab therapy, he developed acute back pain; a contrast-enhanced MRI of the full spine demonstrated multiple, non-traumatic vertebral compression fractures, rib fractures, and as well as pelvic fractures sustained during therapy, without bone metastases. ICI therapy was continued, however he developed additional compression fractures and more profound vertebral wedging , prompting discontinuation of pembrolizumab after 18-months of therapy. The patient’s biochemical workup was unremarkable. His degree of active bone resorption, as measured by C-telopeptide levels (CTX, Table 2 ) were elevated despite three-weeks of alendronate use prior to appointment. Bone density at the hip (lumbar spine excluded in the setting of fracture) demonstrated osteopenia only. Histomorphometry from transiliac bone biopsy revealed bone resorption (increased eroded surface, osteoclast surface) and bone loss (reduced trabecular and cortical parameters). Given the patient’s continued bone loss on oral bisphosphonate, he received one infusion of intravenous bisphosphonate (zoledronic acid), underwent multiple kyphoplasty procedures, and permanently discontinued pembrolizumab. At present, his melanoma continues to be in complete remission 35-months after commencement of pembrolizumab, and after therapy has been held for 20-months. The patient continues to receive IV bisphosphonate yearly in the form of zoledronic acid. Table 2 Skeletal characteristics and clinical course Patient Treatment and clinical course Dual energy X-ray absorptiometry (DXA) T-scores a Additional imaging Bone resorption and formation markers/biochemical indices Inflammatory markers Biopsy data 1 Intravenous zoledronic acid infusions NED; progressive back pain requiring kyphoplasty T-scores: Lumbar spine, −2.1 Left femoral neck, − 1.2 Left total hip, −0.4 Right femoral neck, − 1.7 Right total hip, − 0.8 MRI: Multilevel compression fractures of T12-L5 vertebral bodies; bilateral posterolateral rib fractures; multiple nondisplaced pelvic fractures superimposed on underlying osteopenia. CTX: 1038 pg/mL (↑) bsALP: 11.6 μg/L Ca: 9.5 mg/dL 25OHD: 48 ng/mL CRP: 1.0 mg/dL (↑) ESR 12 mm/h Transiliac bone biopsy: Reduced cortical and trabecular thickness; no osteoblastic activity; increased eroded surface and osteoclastic resorptive activity. 2 Denosumab q6mo Progressive metastatic disease requiring hospice care; now deceased T-scores: Left femoral neck, − 1.5 Left total hip, 0.1 VFA: Multiple wedge deformities 16.8% in T6, 13.6% in T7, 11.2% in T8, 18% in T9, 4.1% in T10, 6.9% in T12, 13.2% in L1. CT chest 1/17: New compression deformity of the superior endplates of T2-T5 vertebral bodies CTX: 537 pg/mL bsALP: 13.1 μg/L Ca: 8.9. mg/dL 25OHD: 37 ng/mL N/A N/A 3 Conservative management with calcium and vitamin D optimization NED, pain free T-scores: L-spine, − 0.9 Left femoral neck, − 1.8 Left total hip, − 1.2 CT scan: T12 compression fracture with evolving compression deformity at T11; biconcave deformities of lumbar vertebrae CTX: 335 pg/mL bsALP: 8.7 μg/L Ca: 9.4. mg/dL 25OHD: 18 ng/mL (↓) N/A N/A 4 Systemic oral steroids Adalimumab NED; improved inflammatory arthritis but progressive left shoulder pain and lack of mobility; no additional resorptive lesions T-scores: L-spine, − 1.0 Left femoral neck, − 1.2 Left total hip, 0.2 1/3 radius, − 1.9 MRI shoulder: Severe erosive changes of the glenohumeral articulation CTX: 589 pg/mL (↑) bsALP: 18 μg/L (↑) Ca: 9.4. mg/dL 25OHD: 52 ng/mL CRP: 2.7 mg/dL (↑) ESR: 69 mm/h (↑) No evidence of melanoma (S100, HMB-45 and melan-A stains negative). Trabecular bone with bone marrow fibrosis and a scattered, mixed inflammatory cell infiltration. 5 Systemic oral steroids Progressive, metastatic disease; limited response to steroids, currently in hospice N/A Hand X-ray: Loss of ossific densities related to the capitate and also hamate, loss of cortical outline of the capitate and hamate CTX: 877 pg/mL (↑) bsALP: 24 μg/L (↑) Ca: 8.8 mg/dL 25OHD: 51.8 CRP: 13.5 mg/L (↑) ESR: 31 mm/h (↑) N/A 6 NSAIDs, Intraarticular corticosteroids Progressive, metastatic disease; good rheumatologic response to steroids, currently on carboplatin pemetrexed and bevacizumab T-scores: L- spine, 0.8 Left femoral neck, 0. 0 Left total hip, 0.8 MRI clavicle: Acromioclavicular joint arthrosis with disproportionate bone marrow edema affecting the distal, no visible fracture CTX: 681 pg/mL (↑) bsALP: 17 μg/L (↑) Ca: 8.8 mg/dL 25OHD: 22 ng/mL (↓) CRP: 2.0 mg/dL (↑) ESR: 32 mm/h (↑) N/A a T-score criteria by DXA: > − 1, normal bone density; − 1 to − 2.4, osteopenia; < − 2.5, osteoporosis ( L-spine ) Lumbar spine, ( N/A ) Not applicable, ( NED ) No evidence of disease, ( CT ) computed tomography, ( MRI ) magnetic resonance imaging, ( VFA ) Vertebral Fracture Assessment Biochemical parameters, normative levels: C-telopeptides (CTX) < 10–584 pg/mL; bone-specific alkaline phosphatase (bsALP) 7.6–14.9 μg/L; calcium (Ca) 8.4–10.5 mg/dL; 25-hydroxy vitamin D (25OHD); C reactive protein (CRP) < 0.5 mg/dL; erythrocyte sedimentation rate (ESR) 1–20 mm/h Fig. 1 a Patient 1, chest CT scan demonstrating new thoracic and lumbar compression fractures, evolving over one year on immunotherapy, 2016 compared to 2017 (bracket); new sternal deformity in the setting of marked kyphosis with compensatory sternal compression (arrow). b Patient 1, transiliac bone biopsy photographed at 400×; top panel illustrates thin trabeculae and limited connectivity, consistent with osteoporosis (arrow); middle panel demonstrating increased osteoclastic activity at the sites of three Howship’s lacunae, or resorption pits (stars); bottom panel outlines histomorphometry parameters including increased eroded surface as well as decreased trabecular and cortical thickness but increased trabecular separation consistent with low bone mass	4.128906	0.961914	sec[1]/sec[2]/p[0]	en	0.999996	30305172	https://doi.org/10.1186/s40425-018-0417-8	[ "bone", "spine", "compression", "fractures", "total", "bsalp", "scores", "femoral", "neck", "pembrolizumab" ]	[ { "code": "FC0Z", "title": "Diseases of the musculoskeletal system or connective tissue, unspecified" }, { "code": "FB84.Z", "title": "Osteomyelitis or osteitis, unspecified" }, { "code": "FB80.Z", "title": "Disorder of bone density or structure, unspecified" }, { "code": "FB86.11", "title": "Hypertrophy of bone" }, { "code": "FB86.1Z", "title": "Bone hyperplasias, unspecified" }, { "code": "FB1Z", "title": "Conditions associated with the spine, unspecified" }, { "code": "FA7Z", "title": "Structural disorders of spine, unspecified" }, { "code": "FA9Z", "title": "Inflammation of spine, unspecified" }, { "code": "LB73.2Z", "title": "Structural developmental anomalies of spine, unspecified" }, { "code": "FA82", "title": "Spinal stenosis" } ]	=== ICD-11 CODES FOUND === [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified Also known as: Diseases of the musculoskeletal system or connective tissue, unspecified \| bone disease NOS \| bone disorder NOS \| bone lesion NOS \| musculoskeletal complications NOS [FB84.Z] Osteomyelitis or osteitis, unspecified Also known as: Osteomyelitis or osteitis, unspecified \| Osteomyelitis or osteitis \| bone inflammation \| bone ulcer \| bone inflammatory disease [FB80.Z] Disorder of bone density or structure, unspecified Also known as: Disorder of bone density or structure, unspecified \| Certain specified disorders of bone density or structure [FB86.11] Hypertrophy of bone Also known as: Hypertrophy of bone \| bone thickening \| periosteum thickening \| Infantile cortical hyperostoses \| Post traumatic subperiosteal ossification [FB86.1Z] Bone hyperplasias, unspecified Also known as: Bone hyperplasias, unspecified \| Bone hyperplasias [FB1Z] Conditions associated with the spine, unspecified Also known as: Conditions associated with the spine, unspecified \| dorsopathies \| disorder of spine \| spinal disorder [FA7Z] Structural disorders of spine, unspecified Also known as: Structural disorders of spine, unspecified \| spinal disease [FA9Z] Inflammation of spine, unspecified Also known as: Inflammation of spine, unspecified \| spinal inflammation \| discitis, unspecified [LB73.2Z] Structural developmental anomalies of spine, unspecified Also known as: Structural developmental anomalies of spine, unspecified \| Structural developmental anomalies of spine \| Malformations of spine \| maldevelopment of spine [FA82] Spinal stenosis Definition: This is a condition characterised by narrowing of the spinal canal. Also known as: Spinal stenosis \| spinal canal stenosis \| Spinal stenosis with no determinant \| primary spinal stenosis \| Spinal stenosis with determinant === GRAPH WALKS === --- Walk 1 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --CHILD--> [?] Arthropathies --- Walk 2 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --CHILD--> [?] Soft tissue disorders --- Walk 3 --- [FB84.Z] Osteomyelitis or osteitis, unspecified --PARENT--> [FB84] Osteomyelitis or osteitis --CHILD--> [FB84.2] Subacute osteomyelitis --- Walk 4 --- [FB84.Z] Osteomyelitis or osteitis, unspecified --PARENT--> [FB84] Osteomyelitis or osteitis --CHILD--> [FB84.2] Subacute osteomyelitis --- Walk 5 --- [FB80.Z] Disorder of bone density or structure, unspecified --PARENT--> [FB80] Certain specified disorders of bone density or structure --EXCLUDES--> [?] Osteopoikilosis --- Walk 6 --- [FB80.Z] Disorder of bone density or structure, unspecified --PARENT--> [FB80] Certain specified disorders of bone density or structure --EXCLUDES--> [?] Osteopoikilosis	[ "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --CHILD--> [?] Arthropathies", "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --CHILD--> [?] Soft tissue disorders", "[FB84.Z] Osteomyelitis or osteitis, unspecified\n --PARENT--> [FB84] Osteomyelitis or osteitis\n --CHILD--> [FB84.2] Subacute osteomyelitis", "[FB84.Z] Osteomyelitis or osteitis, unspecified\n --PARENT--> [FB84] Osteomyelitis or osteitis\n --CHILD--> [FB84.2] Subacute osteomyelitis", "[FB80.Z] Disorder of bone density or structure, unspecified\n --PARENT--> [FB80] Certain specified disorders of bone density or structure\n --EXCLUDES--> [?] Osteopoikilosis", "[FB80.Z] Disorder of bone density or structure, unspecified\n --PARENT--> [FB80] Certain specified disorders of bone density or structure\n --EXCLUDES--> [?] Osteopoikilosis" ]	FC0Z	Diseases of the musculoskeletal system or connective tissue, unspecified	[ { "from_icd11": "FC0Z", "icd10_code": "XIII", "icd10_title": "" }, { "from_icd11": "FB84.Z", "icd10_code": "M86672", "icd10_title": "Other chronic osteomyelitis, left ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86172", "icd10_title": "Other acute osteomyelitis, left ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86171", "icd10_title": "Other acute osteomyelitis, right ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86671", "icd10_title": "Other chronic osteomyelitis, right ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X7", "icd10_title": "Other osteomyelitis, ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X8", "icd10_title": "Other osteomyelitis, other site" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X6", "icd10_title": "Other osteomyelitis, lower leg" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X9", "icd10_title": "Other osteomyelitis, unspecified sites" }, { "from_icd11": "FB84.Z", "icd10_code": "M8668", "icd10_title": "Other chronic osteomyelitis, other site" }, { "from_icd11": "FB84.Z", "icd10_code": "M86662", "icd10_title": "Other chronic osteomyelitis, left tibia and fibula" }, { "from_icd11": "FB84.Z", "icd10_code": "M86151", "icd10_title": "Other acute osteomyelitis, right femur" }, { "from_icd11": "FB84.Z", "icd10_code": "M86141", "icd10_title": "Other acute osteomyelitis, right hand" }, { "from_icd11": "FB84.Z", "icd10_code": "M86641", "icd10_title": "Other chronic osteomyelitis, right hand" }, { "from_icd11": "FB84.Z", "icd10_code": "M8669", "icd10_title": "Other chronic osteomyelitis, multiple sites" } ]	XIII
A 48-year-old woman, with no personal or family history of thyroid malignancy, was consulted for thyroid nodules in November 2014. Physical examination revealed multiple nodules in both thyroid lobes, without compression effects on adjacent organs. Ultrasonography of the neck documented one hypoechoic nodule 44 × 28 mm with microcalcification in the right lobe and two 14 × 8 mm and 9 × 6 mm isoechoic nodules in the left lobe with no neck lymphadenopathy. Fine needle aspiration cytology of the right lobe nodule was reported as suspicious for MTC and serum Ctn level was 1567 pg/mL (reference value: < 10 pg/mL). The patient was clinically and biochemically euthyroid. Her blood pressure was 110/70 mmHg. She did not complain of paroxysmal hypertension, palpitation, or sweating; also, she did not have personal or family history of renal stone or hypercalcemia. Laboratory tests including serum calcium, parathyroid hormone, and 24-h urine collection for vanillylmandelic acid, norepinephrine, and epinephrine were unremarkable (Table 1 ). The patient was scheduled for total thyroidectomy and bilateral cervical lymph node dissection in November 2014. Histopathological examination confirmed the diagnosis of right lobe MTC without vascular invasion, extracapsular extension, lymph node involvement, or C-cell hyperplasia. Immunohistochemical (IHC) stainings were positive for Ctn, synaptophysin, chromogranin A (CgA), and negative for thyroglobulin (Tg). RET mutation in the patient’s peripheral blood lymphocytes was positive in exon 11, codon 691, and exon 15, codon 904. Two months after thyroidectomy, serum Ctn and CEA levels were 1.3 pg/mL (reference value: < 10 pg/mL) and 4.9 ng/mL (reference value: < 2.5 ng/mL), respectively; with mildly elevated liver enzymes i.e., aspartate aminotransferase: 44 IU/L, alanine aminotransferase: 38 IU/L, and alkaline phosphatase (ALP): 380 IU/L. Since the calcium-loaded calcitonin test was not available at that time and considering the immediate post-operative significant decrement of serum calcitonin level, this test was not performed. On regular follow-up visits, serum Ctn and CEA levels were consistently below the upper limit of reference ranges from 2014 to 2021 , and repeated cervical ultrasonography did not show abnormal findings. According to gradually raised liver enzymes with a cholestatic pattern , the patient was referred to a gastroenterologist for evaluation of liver function in 2016. Abdominopelvic ultrasonography revealed a coarse echo pattern of the liver with normal internal and external bile ducts. Liver fibroScan showeda low fibrosis score (7kPa). Anti-mitochondrial antibody was 1.2 units (reference value: < 1.5 units), and other immunological tests and hepatitis serology panels were normal. Treatment with ursodeoxycholic acid 300 mg daily was initiated and the patient followed clinically and biochemically. Since liver enzymes especially ALP and gamma glutamyl transferase (GGT) gradually elevated in 2020, a liver biopsy was performed. The histopathological examination of the liver biopsy, reported by two experienced pathologists, showed tiny nests of neuroendocrine-like cells, severe portal lymphoplasma cell infiltration, some piecemeal necrosis, mild bile duct proliferation, focal parenchymal destruction of interlobular bile ducts by granulomatous reaction, and fibrous expansion of portal tracts with occasional p-p bridges . IHC staining showed diffuse mild to moderate positivity for synaptophysin. IHC staining for CgA was positive in 80% of cells with moderate intensity . Negative results were reported for Ctn, CEA, and Tg. The proliferation marker of Ki67 was positive in 2–3% of the neuroendocrine-like cells. Histopathologic findings and IHC stainings were compatible with the presence of neuroendocrine-like cells in a background of primary biliary cholangitis (PBC). Results of upper and lower gastrointestinal endoscopies, whole body bone scan with TC 99 and 68 Ga-Dotatate positron emission tomography (PET)-CT scan were unremarkable. Abdominal computed tomography (CT) showed few periportal and portocaval lymph nodes (up to 10 × 14 mm). Endoscopic ultrasound evaluation only suggested a few hilar and peripancreatic lymph nodes. Subsequent FNA of hilar lymph node was negative for NET or any other malignancy, and IHC stainings were negative for cytokeratin, synaptophysin, Ctn, and CgA. Serum CgA and procalcitonin levels were reported 43 ng/mL (reference value: < 100 ng/mL) and 0.1 ng/mL (reference value: < 0.15 ng/mL), respectively. Hence, according to negative biochemical and radiological evidence for primary or secondary hepatic NETs, as well as the presence of obstructive liver pathology, the final diagnosis of anti-mitochondrial autoantibodies (AMA)-negative PBC was made and the patient was managed with glucocorticoid. Table 1 Laboratory tests on the first admission in October 2014 Test Patient value Reference range White blood cell 7700//mm 3 4500–11,000/mm 3 Hemoglobin 14 g/dL 13.5–17.5 g/dL Platelets 178,000 /mm 3 150,000–450,000/mm 3 Erythrocyte sedimentation rate 14 mm/hour 0–20 mm/hour Blood urea nitrogen 14 mg/dL 8–20 mg/dL Creatinine 0.9 mg/dL 0.5–1.5 mg/dL Alanine aminotransferase 27 IU/L 5–40 IU/L Aspartate aminotransferase 34 IU/L 5–40 IU/L Alkaline phosphatase 243 U/L 44–147 U/L Thyroid stimulating hormone 1.65mIU/L 0.5–5 mIU/L Total thyroxine 9.4 mcg/dL 5–12 mcg/dL Calcium 9.4 mg/dL 8.6–10.6 mg/dL Phosphate 3.9 mg/dL 2.5–4.5 mg/dL Parathyroid hormone 18 pg/mL 10–55 pg/mL Calcitonin 1547 pg/mL < 10 pg/mL 24 h urine epinephrine 5 mcg/24 h 0.5–20 mcg/24 h 24 h urine norepinephrine 24 mcg/24 h 15–80 mcg/24 h 24 h urine vanillylmandelic acid 7.3 mcg/24 h < 13.5 mcg/24h Fig. 1 Trend of basal serum Ctn and CEA levels from 2014 to 2021. Ctn: calcitonin, CEA: carcinoembryonic antigen Fig. 2 Trend of serum liver enzymes levels. ALT: Alanine aminotransferase, AST: Aspartate aminotransferase, ALP: alkaline phosphatase Fig. 3 Histopathologic sections of the liver biopsy (×100 and × 400 magnifications): A and B , Liver tissue with cholestatic pattern of injury and bile duct damage (H&E stain); C and D , Fibrous expansion of portal tract with occasional p-p bridges (Trichrome stain); E and F Nests of neuroendocrine-like cells (H&E stain) Fig. 4 Immunohistochemistry (IHC) stained sections of the liver biopsy (×100 and × 400 magnifications): A and B . IHC staining for Chromogranin A ; C and D , IHC staining for synaptophysin	4.0625	0.973633	sec[1]/p[0]	en	0.999995	37649029	https://doi.org/10.1186/s12902-023-01439-7	[ "liver", "serum", "reference", "aminotransferase", "lymph", "cells", "thyroid", "lobe", "blood", "urine" ]	[ { "code": "DB9Z", "title": "Diseases of liver, unspecified" }, { "code": "DB97.Z", "title": "Inflammatory liver disease, unspecified" }, { "code": "DB99.7", "title": "Hepatic failure without mention whether acute or chronic" }, { "code": "LB20.0Y", "title": "Other specified structural developmental anomalies of liver" }, { "code": "LB20.0Z", "title": "Structural developmental anomalies of liver, unspecified" }, { "code": "NE80.3", "title": "Other serum reactions" }, { "code": "5D0Y", "title": "Other specified metabolic disorders" }, { "code": "5B91.0", "title": "Hypercalcaemia" }, { "code": "4A84.Y", "title": "Other specified anaphylaxis" }, { "code": "5C50.F2", "title": "Homocarnosinosis" } ]	=== ICD-11 CODES FOUND === [DB9Z] Diseases of liver, unspecified Also known as: Diseases of liver, unspecified \| liver disease \| liver condition NOS \| organ liver disease \| hepatopathy [DB97.Z] Inflammatory liver disease, unspecified Also known as: Inflammatory liver disease, unspecified \| Certain specified inflammatory liver diseases \| Nonspecific reactive hepatitis \| inflammatory liver disease \| hepatitis NOS [DB99.7] Hepatic failure without mention whether acute or chronic Also known as: Hepatic failure without mention whether acute or chronic \| liver decompensation \| liver function failure \| hepatic failure NOS \| liver failure NOS [LB20.0Y] Other specified structural developmental anomalies of liver Also known as: Other specified structural developmental anomalies of liver \| Alagille syndrome \| Alagille-Watson syndrome \| Arteriohepatic dysplasia \| Syndromic bile duct paucity [LB20.0Z] Structural developmental anomalies of liver, unspecified Also known as: Structural developmental anomalies of liver, unspecified \| Structural developmental anomalies of liver \| Malformations of liver \| congenital anomaly of liver \| congenital malformation of liver [NE80.3] Other serum reactions Also known as: Other serum reactions \| Allergic reaction to serum \| serum allergy \| Complications of vaccination, protein sickness \| Protein sickness Excludes: serum hepatitis [5D0Y] Other specified metabolic disorders Also known as: Other specified metabolic disorders \| Disorders of plasma-protein metabolism, not elsewhere classified \| abnormal protein transport \| dysproteinaemia \| Absence of albumin in blood [5B91.0] Hypercalcaemia Definition: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused by dehydration secondary to urinary losses of calcium, water and other electrolytes, and to an increase in membrane potential caused by the elevation in extracellular fluid ionized calcium concentration. Patients with moderate to severe hypercalcaemia often complain of nausea and vomiting, symptoms Also known as: Hypercalcaemia \| Calcium excess \| elevated serum calcium \| hypercalcaemic crisis \| hypercalcaemic syndrome [4A84.Y] Other specified anaphylaxis Also known as: Other specified anaphylaxis \| Latex-induced anaphylaxis \| Anaphylaxis due to latex \| Latex anaphylaxis \| Anaphylactic shock due to serum [5C50.F2] Homocarnosinosis Definition: Homocarnosinosis is a metabolic defect characterised by progressive spastic diplegia, intellectual deficit and retinitis pigmentosa. This extremely rare disorder has been reported in only one family, namely a woman and three of her children. The latter showed but their mother was symptom free. It is therefore uncertain whether there is a relationship between the biochemical defect and the clinical symptoms. Inheritance in the reported family seems to be autosomal dominant. Also known as: Homocarnosinosis \| Homocarnosinase deficiency \| Serum carnosinase deficiency === GRAPH WALKS === --- Walk 1 --- [DB9Z] Diseases of liver, unspecified --PARENT--> [?] Diseases of liver --RELATED_TO--> [?] Viral hepatitis Def: A group of liver diseases caused by infection with one or more of the five hepatitis viruses, hepatitis A virus, hepatitis B virus, hepatitis C virus, hepatitis D virus and hepatitis E viruses. The in... --- Walk 2 --- [DB9Z] Diseases of liver, unspecified --PARENT--> [?] Diseases of liver --CHILD--> [DB92] Non-alcoholic fatty liver disease Def: NAFLD is characterised by fatty liver related to insulin resistance in the absence of significant alcohol consumption. It embraces a pathological spectrum from simple steatosis to steatohepatitis. 10-... --- Walk 3 --- [DB97.Z] Inflammatory liver disease, unspecified --PARENT--> [DB97] Certain specified inflammatory liver diseases --EXCLUDES--> [?] Acute viral hepatitis Def: A group of liver diseases characterised by liver inflammation and fibrosis, caused by less than 6 months of infection with one or more of hepatitis B virus, hepatitis C virus and hepatitis D virus, wi... --- Walk 4 --- [DB97.Z] Inflammatory liver disease, unspecified --PARENT--> [DB97] Certain specified inflammatory liver diseases --EXCLUDES--> [?] Acute or subacute hepatic failure Def: Acute and subacute liver failure is characterised by onset of coagulopathy and/or hepatic encephalopathy within 8 weeks of onset of symptoms in a patient without previously known liver diseases.... --- Walk 5 --- [DB99.7] Hepatic failure without mention whether acute or chronic --PARENT--> [DB99] Certain specified diseases of liver Def: This is a group of conditions characterised as being in or associated with the liver that are not classified elsewhere.... --RELATED_TO--> [?] Cirrhotic cardiomyopathy Def: Cirrhotic cardiomyopathy is defined as chronic cardiac dysfunction in patients with cirrhosis characterised by blunted contractile responsiveness to stress/exercise and-or altered diastolic relaxation... --- Walk 6 --- [DB99.7] Hepatic failure without mention whether acute or chronic --PARENT--> [DB99] Certain specified diseases of liver Def: This is a group of conditions characterised as being in or associated with the liver that are not classified elsewhere.... --EXCLUDES--> [?] Fibropolycystic liver disease	[ "[DB9Z] Diseases of liver, unspecified\n --PARENT--> [?] Diseases of liver\n --RELATED_TO--> [?] Viral hepatitis\n Def: A group of liver diseases caused by infection with one or more of the five hepatitis viruses, hepatitis A virus, hepatitis B virus, hepatitis C virus, hepatitis D virus and hepatitis E viruses. The in...", "[DB9Z] Diseases of liver, unspecified\n --PARENT--> [?] Diseases of liver\n --CHILD--> [DB92] Non-alcoholic fatty liver disease\n Def: NAFLD is characterised by fatty liver related to insulin resistance in the absence of significant alcohol consumption. It embraces a pathological spectrum from simple steatosis to steatohepatitis. 10-...", "[DB97.Z] Inflammatory liver disease, unspecified\n --PARENT--> [DB97] Certain specified inflammatory liver diseases\n --EXCLUDES--> [?] Acute viral hepatitis\n Def: A group of liver diseases characterised by liver inflammation and fibrosis, caused by less than 6 months of infection with one or more of hepatitis B virus, hepatitis C virus and hepatitis D virus, wi...", "[DB97.Z] Inflammatory liver disease, unspecified\n --PARENT--> [DB97] Certain specified inflammatory liver diseases\n --EXCLUDES--> [?] Acute or subacute hepatic failure\n Def: Acute and subacute liver failure is characterised by onset of coagulopathy and/or hepatic encephalopathy within 8 weeks of onset of symptoms in a patient without previously known liver diseases....", "[DB99.7] Hepatic failure without mention whether acute or chronic\n --PARENT--> [DB99] Certain specified diseases of liver\n Def: This is a group of conditions characterised as being in or associated with the liver that are not classified elsewhere....\n --RELATED_TO--> [?] Cirrhotic cardiomyopathy\n Def: Cirrhotic cardiomyopathy is defined as chronic cardiac dysfunction in patients with cirrhosis characterised by blunted contractile responsiveness to stress/exercise and-or altered diastolic relaxation...", "[DB99.7] Hepatic failure without mention whether acute or chronic\n --PARENT--> [DB99] Certain specified diseases of liver\n Def: This is a group of conditions characterised as being in or associated with the liver that are not classified elsewhere....\n --EXCLUDES--> [?] Fibropolycystic liver disease" ]	DB9Z	Diseases of liver, unspecified	[ { "from_icd11": "DB9Z", "icd10_code": "K7681", "icd10_title": "Hepatopulmonary syndrome" }, { "from_icd11": "DB9Z", "icd10_code": "K7689", "icd10_title": "Other specified diseases of liver" }, { "from_icd11": "DB9Z", "icd10_code": "K769", "icd10_title": "Liver disease, unspecified" }, { "from_icd11": "DB9Z", "icd10_code": "K77", "icd10_title": "Liver disorders in diseases classified elsewhere" }, { "from_icd11": "DB9Z", "icd10_code": "K762", "icd10_title": "Central hemorrhagic necrosis of liver" }, { "from_icd11": "DB9Z", "icd10_code": "K70-K77", "icd10_title": "" }, { "from_icd11": "DB9Z", "icd10_code": "K778", "icd10_title": "" }, { "from_icd11": "DB9Z", "icd10_code": "K72", "icd10_title": "Hepatic failure, not elsewhere classified" }, { "from_icd11": "DB9Z", "icd10_code": "K76", "icd10_title": "Other diseases of liver" }, { "from_icd11": "DB9Z", "icd10_code": "K768", "icd10_title": "Other specified diseases of liver" }, { "from_icd11": "DB97.Z", "icd10_code": "K7581", "icd10_title": "Nonalcoholic steatohepatitis (NASH)" }, { "from_icd11": "DB97.Z", "icd10_code": "K7589", "icd10_title": "Other specified inflammatory liver diseases" }, { "from_icd11": "DB97.Z", "icd10_code": "K759", "icd10_title": "Inflammatory liver disease, unspecified" }, { "from_icd11": "DB97.Z", "icd10_code": "K752", "icd10_title": "Nonspecific reactive hepatitis" }, { "from_icd11": "DB97.Z", "icd10_code": "K75", "icd10_title": "Other inflammatory liver diseases" } ]	K7681	Hepatopulmonary syndrome
Our patient was a 56-year-old postmenopausal African American woman with no past medical history who was previously treated at an outside oncology clinic for breast masses until 2010, when we first saw her. Her family history was negative for breast or ovarian carcinoma. She had a negative smoking history and endorsed drinking one drink per week. Per reports obtained, she first presented in 2006 with left breast lesions located in the upper inner breast that were documented as complicated cystic masses within the 9 o’clock and 9:30 positions on the basis of ultrasound (US). Subsequent US core biopsy in both areas revealed intraductal papilloma (IDP), and the patient was referred for a surgical consultation. No additional documentation of clinical visits was available until 1 year later. That documentation was in the form of a core biopsy pathologic report documenting the patient’s history of IDP at 9:00 and 9:30 positions as well as intraductal papillomatosis of the breast. A core biopsy taken at that time was from the left breast (location not mentioned) as well as the left axilla. The patient’s left breast showed fibrosis of mammary stroma including intralobular stromal sclerosis as well as microcalcifications in the lobular lumens. An axillary core biopsy confirmed IDP of the breast with stromal hyalinization as well as lymph node tissue adjacent to the papilloma. One month later, she underwent lumpectomy, with pathology reporting a 7-mm intracystic papilloma within a lymph node that was completely excised, as well as an epidermal inclusion cyst. The pathologist noted that the tumor was located near the periphery of a lymph node, probably arising in ectopic breast tissue in the capsular region. Approximately 11 months later, she developed another left breast mass. This was excised after a US-guided core biopsy once again revealed a benign IDP. The patient was then lost to follow-up at the outside clinic. She presented to our clinic 2 years later for an evaluation of a new left breast lesion. A bilateral diagnostic mammogram revealed two masses in the left breast, which were not well visualized, owing to heterogeneously dense breast tissue. Diagnostic US revealed a solid superficial mass measuring 0.81 × 0.76 × 0.81 cm corresponding to palpable findings also seen at the 6 o’clock position . Additionally, the patient had a large, complex cystic mass measuring 7 cm at the 1 to 3 o’clock position abutting the pectoralis muscle . A core biopsy of the 6 o’clock lesion was recommended. A US-guided, vacuum-assisted core biopsy of the 6 o’clock mass revealed an intracystic papillary neoplasm. Per the report, the patient denied nipple discharge, dimpling, thickening, redness of the skin, swelling, or tenderness at the time. A few weeks later, she underwent left breast lumpectomy with pathology revealing a complex cystic mass with fibrocystic changes at 1 to 3 o’clock and intraductal papilloma at 6 o’clock. The patient missed her 6-month follow-up mammogram. She returned 8 months later for a bilateral diagnostic mammogram, which showed a new 2.5-cm mass in the deep central aspect of her left breast at the 12 o’clock position. US showed a cystic mass measuring 3 cm and containing an intracystic solid component measuring 1 × 1 × 2 cm. No axillary or supraclavicular adenopathy was noted on the basis of imaging or physical examination. Her surgical team decided on left breast excisional biopsy with preoperative mammogram guidewire localization. Pathology revealed a benign papilloma measuring 1 cm, focally extending into skeletal muscle in the area adjacent to the previous biopsy site, but with negative margins and no signs of atypia. On the patient’s 6-month follow-up surveillance diagnostic mammogram, another new 3-cm density was noted at the 12 o’clock position. This was most consistent with a benign cyst and was aspirated. She was again lost to follow-up for more than 2 years until July 2015, when she presented with a 2-month history of a slowly enlarging left breast mass in the same region as her previous papillomas. A bilateral diagnostic mammogram with US showed a large mass at the 12 o’clock position measuring 7 × 2.5 cm. Her physical examination revealed that there were two areas of concern: first, a mass measuring 7.5 × 6.3 cm in the 1 o’clock position, and second, an area of nodularity measuring 4.6 × 3.1 cm in the 11 o’clock position. One month later, computed tomography (CT) of the chest and magnetic resonance imaging of the breast revealed a predominantly cystic mass with a solid component extending into the chest wall and approaching the pleural space . These tests also revealed a suspicious internal mammary lymph node . A positron emission tomographic (PET)-CT scan showed a hypermetabolic nodule located in the pretracheal space with a corresponding standardized uptake value (SUV) of 6.1 and multiple associated hypermetabolic internal mammary lymph nodes with the highest SUV of 6.0 and nodular hypermetabolic activity along the inferomedial aspect of the cystic mass (SUV, 2.7). Fig. 1 a Ultrasound image of 8-mm solid superficial mass located at six o’clock (white arrow). b Ultrasound image of 7-cm complex cystic mass located at one to three o’clock (white arrow) Fig. 2 Magnetic resonance imaging of the breast with/without contrast enhancement. a Large cystic mass (white arrow) extending through the pectoralis muscle and containing a 1.8-cm area of neoplastic enhancement internally. This lesion was hypermetabolic on the positron emission tomographic scan. b An abnormal left internal mammary lymph node (white arrow) at the level of the sternomanubrial articulation. This was confirmed to be metastatic on the basis of biopsy Fig. 3 Comparison of positron emission tomographic/computed tomographic image from 2015 (prior to neoadjuvant antihormonal therapy and left breast mastectomy) versus follow-up image in 2018 (following left breast mastectomy). a Response was noted in the internal mammary lymph node following combination antihormonal therapy ( arrow ). b 18 F-fluorodeoxyglucose avid mediastinal lymphadenopathy with mild reduction in avidity following combination antihormonal therapy ( arrow ). Of note, no avidity was seen in the internal mammary lymph node, and minimal avidity surrounding the breast papillary mass was observed. c Mass in 2015 prior to antihormonal therapy and mastectomy. Note the size of this mass. d Reconstructed breast following mastectomy	3.923828	0.981934	sec[1]/p[0]	en	0.999999	32070435	https://doi.org/10.1186/s13256-020-2354-7	[ "breast", "clock", "biopsy", "cystic", "lymph", "measuring", "core", "node", "position", "that" ]	[ { "code": "GB23", "title": "Certain specified disorders of breast" }, { "code": "GB21.Z", "title": "Inflammatory disorders of breast, unspecified" }, { "code": "GB21.Y", "title": "Other specified inflammatory disorders of breast" }, { "code": "QF01.0", "title": "Acquired absence of breast" }, { "code": "GB23.3", "title": "Atrophy of breast" }, { "code": "BC81.20", "title": "Cavotricuspid isthmus dependent macroreentry tachycardia" }, { "code": "JA85.Y", "title": "Maternal care for other specified fetal abnormality or damage" }, { "code": "KD39.3", "title": "Fetus or newborn affected by complications of fetal surgery" }, { "code": "PK81.5", "title": "Biopsy procedure, not elsewhere classified, associated with injury or harm in therapeutic use" }, { "code": "PK81.4", "title": "Bone marrow aspiration or biopsy associated with injury or harm in therapeutic use" } ]	=== ICD-11 CODES FOUND === [GB23] Certain specified disorders of breast Definition: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere. Also known as: Certain specified disorders of breast \| disease of breast \| mastopathy [GB21.Z] Inflammatory disorders of breast, unspecified Also known as: Inflammatory disorders of breast, unspecified \| Inflammatory disorders of breast \| breast inflammation \| inflammatory breast disease \| mastitis NOS [GB21.Y] Other specified inflammatory disorders of breast Also known as: Other specified inflammatory disorders of breast \| Breast antibioma \| Infective mastitis \| acute infective mastitis \| nonpuerperal infective mastitis [QF01.0] Acquired absence of breast Also known as: Acquired absence of breast \| absence of breast \| mastectomy status \| Acquired absence of breast, partial \| Acquired absence of breast, total [GB23.3] Atrophy of breast Definition: A condition of the breast, caused by apoptosis of the cells commonly due to prolonged estrogen reduction, diminished cellular proliferation, decreased cellular volume, decreased function, ischaemia, malnutrition, disease, or mutation. This condition is characterised by a partial or complete decrease in size and function of the breast tissue. Also known as: Atrophy of breast \| Hypoplasia of breast \| hypoplastic breast \| mammary hypoplasia [BC81.20] Cavotricuspid isthmus dependent macroreentry tachycardia Definition: A macro re-entrant atrial tachycardia that rotates around the tricuspid annulus. Also known as: Cavotricuspid isthmus dependent macroreentry tachycardia \| Cavotricuspid isthmus dependent reentry atrial tachycardia \| Cavotricuspid isthmus dependent macroreentry tachycardia, clockwise \| Atypical atrial flutter clockwise \| Cavotricuspid isthmus dependent macroreentry tachycardia, counterclockwise Includes: Idiopathic neonatal atrial flutter [JA85.Y] Maternal care for other specified fetal abnormality or damage Also known as: Maternal care for other specified fetal abnormality or damage \| Maternal care for damage to fetus from alcohol \| suspected damage to fetus from maternal alcohol addiction affecting management of mother \| pregnancy management affected by fetal damage from maternal alcohol addiction \| maternal care for known or suspected damage to fetus from alcohol [KD39.3] Fetus or newborn affected by complications of fetal surgery Definition: A condition in the fetus due to an unfavourable evolution of a condition (complication) associated with a surgical health intervention applied to the fetus. Also known as: Fetus or newborn affected by complications of fetal surgery \| Adverse outcome following fetal skin biopsy [PK81.5] Biopsy procedure, not elsewhere classified, associated with injury or harm in therapeutic use Also known as: Biopsy procedure, not elsewhere classified, associated with injury or harm in therapeutic use \| complication during or following biopsy procedure, other than bone marrow Excludes: Bone marrow aspiration or biopsy associated with injury or harm in therapeutic use \| Circumstances associated with a surgical or other medical procedure influencing the episode of care, without injury or harm [PK81.4] Bone marrow aspiration or biopsy associated with injury or harm in therapeutic use Also known as: Bone marrow aspiration or biopsy associated with injury or harm in therapeutic use \| complication during or following bone marrow aspiration or biopsy Excludes: Circumstances associated with a surgical or other medical procedure influencing the episode of care, without injury or harm === GRAPH WALKS === --- Walk 1 --- [GB23] Certain specified disorders of breast Def: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere.... --PARENT--> [?] Disorders of breast Def: Any disorder characterised by pathological changes to the breast or breast tissue.... --EXCLUDES--> [?] Certain specified disorders of breast or lactation associated with childbirth --- Walk 2 --- [GB23] Certain specified disorders of breast Def: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere.... --CHILD--> [GB23.1] Fissure or fistula of nipple Def: A condition characterised by the formation of a deep furrow or crack-like lesion on the nipple and an abnormal passage between the nipple and adjacent tissues or surfaces.... --PARENT--> [GB23] Certain specified disorders of breast Def: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere.... --- Walk 3 --- [GB21.Z] Inflammatory disorders of breast, unspecified --PARENT--> [GB21] Inflammatory disorders of breast Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function.... --RELATED_TO--> [?] Neonatal infectious mastitis Def: A disease of the breasts in neonates, may be caused by a maternal infection with a bacterial source. This disease is characterised by swelling, erythema, warmth, tenderness, induration of the breast, ... --- Walk 4 --- [GB21.Z] Inflammatory disorders of breast, unspecified --PARENT--> [GB21] Inflammatory disorders of breast Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function.... --CHILD--> [GB21.Y] Other specified inflammatory disorders of breast --- Walk 5 --- [GB21.Y] Other specified inflammatory disorders of breast --PARENT--> [GB21] Inflammatory disorders of breast Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function.... --RELATED_TO--> [?] Neonatal infectious mastitis Def: A disease of the breasts in neonates, may be caused by a maternal infection with a bacterial source. This disease is characterised by swelling, erythema, warmth, tenderness, induration of the breast, ... --- Walk 6 --- [GB21.Y] Other specified inflammatory disorders of breast --PARENT--> [GB21] Inflammatory disorders of breast Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function.... --RELATED_TO--> [?] Neonatal infectious mastitis Def: A disease of the breasts in neonates, may be caused by a maternal infection with a bacterial source. This disease is characterised by swelling, erythema, warmth, tenderness, induration of the breast, ...	[ "[GB23] Certain specified disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere....\n --PARENT--> [?] Disorders of breast\n Def: Any disorder characterised by pathological changes to the breast or breast tissue....\n --EXCLUDES--> [?] Certain specified disorders of breast or lactation associated with childbirth", "[GB23] Certain specified disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere....\n --CHILD--> [GB23.1] Fissure or fistula of nipple\n Def: A condition characterised by the formation of a deep furrow or crack-like lesion on the nipple and an abnormal passage between the nipple and adjacent tissues or surfaces....\n --PARENT--> [GB23] Certain specified disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere....", "[GB21.Z] Inflammatory disorders of breast, unspecified\n --PARENT--> [GB21] Inflammatory disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function....\n --RELATED_TO--> [?] Neonatal infectious mastitis\n Def: A disease of the breasts in neonates, may be caused by a maternal infection with a bacterial source. This disease is characterised by swelling, erythema, warmth, tenderness, induration of the breast, ...", "[GB21.Z] Inflammatory disorders of breast, unspecified\n --PARENT--> [GB21] Inflammatory disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function....\n --CHILD--> [GB21.Y] Other specified inflammatory disorders of breast", "[GB21.Y] Other specified inflammatory disorders of breast\n --PARENT--> [GB21] Inflammatory disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function....\n --RELATED_TO--> [?] Neonatal infectious mastitis\n Def: A disease of the breasts in neonates, may be caused by a maternal infection with a bacterial source. This disease is characterised by swelling, erythema, warmth, tenderness, induration of the breast, ...", "[GB21.Y] Other specified inflammatory disorders of breast\n --PARENT--> [GB21] Inflammatory disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function....\n --RELATED_TO--> [?] Neonatal infectious mastitis\n Def: A disease of the breasts in neonates, may be caused by a maternal infection with a bacterial source. This disease is characterised by swelling, erythema, warmth, tenderness, induration of the breast, ..." ]	GB23	Certain specified disorders of breast	[ { "from_icd11": "GB23", "icd10_code": "N6459", "icd10_title": "Other signs and symptoms in breast" }, { "from_icd11": "GB23", "icd10_code": "N6489", "icd10_title": "Other specified disorders of breast" }, { "from_icd11": "GB23", "icd10_code": "N6481", "icd10_title": "Ptosis of breast" }, { "from_icd11": "GB23", "icd10_code": "N6482", "icd10_title": "Hypoplasia of breast" }, { "from_icd11": "GB23", "icd10_code": "N6452", "icd10_title": "Nipple discharge" }, { "from_icd11": "GB23", "icd10_code": "N6451", "icd10_title": "Induration of breast" }, { "from_icd11": "GB23", "icd10_code": "N6453", "icd10_title": "Retraction of nipple" }, { "from_icd11": "GB23", "icd10_code": "N64", "icd10_title": "Other disorders of breast" }, { "from_icd11": "GB23", "icd10_code": "N648", "icd10_title": "Other specified disorders of breast" }, { "from_icd11": "GB23", "icd10_code": "N645", "icd10_title": "Other signs and symptoms in breast" }, { "from_icd11": "GB21.Z", "icd10_code": "N610", "icd10_title": "Mastitis without abscess" }, { "from_icd11": "GB21.Z", "icd10_code": "N611", "icd10_title": "Abscess of the breast and nipple" }, { "from_icd11": "GB21.Z", "icd10_code": "N61", "icd10_title": "Inflammatory disorders of breast" }, { "from_icd11": "QF01.0", "icd10_code": "Z9012", "icd10_title": "Acquired absence of left breast and nipple" }, { "from_icd11": "QF01.0", "icd10_code": "Z9011", "icd10_title": "Acquired absence of right breast and nipple" } ]	N6459	Other signs and symptoms in breast
The patient was a 32-year-old Caucasian female, the third-born to healthy non-consanguineous parents with negative family history of neurologic disorders. The pregnancy, birth and early psychomotor development were uneventful. At the age of 7 years chronic urinary incontinence commenced and remained the unexplained clinical abnormality for 20 years. Prompt nephrologic, gynecologic, and urologic investigations (also including voiding cystourethrogram, cystoscopy, renal scintigraphy, ultrasonography, biochemical analyses of blood and urine) were normal, and there were no accompanying neurologic signs in the overall clinical status. The persisting urinary disturbance brought the patient to neurologic attention only at 32 years of age when the assessment disclosed a 5-year history of slowly progressive cerebellar ataxia followed by pyramidal signs, and then focal upper extremity dystonia. The neuropsychological evaluation at the age of 32 years documented a mild intellectual disability for the first time (full-scale IQ of 57; Wechsler Adult Intelligence Scale-IV). It was thought to be a decline compared to the patient’s historical level of functioning: the patient had finished the compulsory education (age 7–15 years) with regular psychological assessments, was employed as a factory worker, and was able to live independently. She stopped working at the age of 32 years during the neurologic follow-ups. Magnetic resonance imaging (MRI) of the brain revealed diffuse supratentorial hypomyelination that bilaterally spread along the posterior limb of the internal capsule affecting the pyramidal tracts, the middle and inferior cerebellar peduncles . T2-weighted hypointensities were observed in the globi pallidi, the optic radiations and the dentate nuclei . There was a marked atrophy of the cerebellum and corpus callosum, as well as of the pontine and midbrain tegmentum . The adenohypophysis was small . Multivoxel MR Spectroscopy of the brain detected a markedly decreased choline/creatine ratio in the abnormal white matter . Diffusion tensor imaging of the brain showed a slightly reduced fractional anisotropy consistent with hypomyelination . MRI of the brain pointed to Pol III-related leukodystrophy . MRI of the spinal cord was normal . Electromyoneurography was unremarkable. Urodynamic tests (uroflow study, postvoid residual volume, filling-voiding cystometry, abdominal leak-point pressure, external sphincter electromyography-EMG) revealed decreased bladder capacity with preserved sensation, uninhibited detrusor muscle contractions, and detrusor- external sphincter synergy, while sphincter EMG recorded normal amplitudes and duration of muscle potentials. The urodynamic study proved the bladder overactivity due to neurologic, suprapontine lesion . The repeated urologic, nephrologic, and metabolic investigations remained normal. At 26 years of age the patient had no sexual development (Tanner stage 1, primary amenorrhea). Hypogonadotropic hypogonadism, low baseline prolactin level and growth hormone (GH) deficiency were confirmed (Table 1 ). Her height was below the 3 rd percentile and she was normosmic. The karyotype was normal. The patient received sexual hormone replacement for 3 years. At 32 years of age hypogonadotropic hypogonadism was proven by the absence of luteinizing hormone pulsatility (Table 1 ). Hypoprolactinemia was confirmed by two stimulatory tests (insulin tolerance test-ITT and thyrotropin-releasing hormone-TRH test), while growth hormone level was normal (Table 1 ). Other hormonal values were normal (Table 1 ). Her final adult height was 155 cm (10 th percentile) and Tanner stage was 4, with amenorrhea. Given the accompanied hypoestrogenemia, gynecologic and urogynecologic examinations excluded atrophic vaginitis, vulvar structural anomalies, and decreased strength of the pelvic floor and bladder muscles, as possible estrogen-related causes of the urinary incontinence . Dental examination confirmed hypodontia with the lack of second and third molars. A genetic evaluation was necessitated: sequencing of the key exons and exon-intron boundaries of POLR3A using previously reported methods , revealed two already known disease-causing mutations, c.272C > T (p.P91L) in exon 3 and c.3014G > A in exon 23 which segregated in the parents and proved the diagnosis of 4H leukodystrophy. Figure 1 Magnetic resonance imaging (MRI) study in the patient with 4H leukodystrophy. (a) T1W (T1-weighted) axial scan of the brain indicating hypomyelination; (b) T2W (T2-weighted) scan of the brain and T2W-hypointensity of the globi pallidi; (c) affection of the middle cerebellar peduncles:T2W scan; (d) T2W-hypointensity of the nucleus dentatus; (e) atrophy of the cerebellum, brainstem, corpus callosum and the adenohypophisis: sagittal T1W scan; (f) the small adenohypophysis: coronal T1W scan + contrast; (g) Magnetic resonance spectroscopy: decreased choline/creatine ratio in the lesions; (h) Diffusion tensor imaging of the brain supporting hypomyelination; (i, j) the unaffected spinal cord: T2W scans. Table 1 Endocrine evaluation in the patient with 4H leukodystrophy Analysis At age of 26 yrs At age of 32 yrs Normal range FSH (IU/L) 1.1 1.4 2.5-15 Peak FSH after LHRH test (IU/L) 1.5 1.5 >5 LH (IU/L) a <1.0 1.1 4-20 Peak LH after LHRH test (IU/L) <1.0 1.3 >5 Estradiol (pmol/L) 46.3 13.1 105-217 Prolactin (mean, daily curve, ng/mL) b 1.5 2.2 4.8-23.4 Peak prolactin after ITT (ng/mL) c 4.7 4.5 >> > b Peak prolactin after TRH test (ng/mL) d N/A 6 >> > b Thyroxine (nmol/L) 134.7 N/A 60-170 Free thyroxine (pmol/L) N/A 14.1 7-18 Triiodothyronine (nmol/L) N/A 1.4 0.9-2.4 Free triiodothyronine (pmol/L) N/A 3.8 2.62-5.7 TSH (mIU/L) 1.0 1.7 0.15-5.0 Cortisol (nmol/L) 332 428 131-642 Peak cortisol after ITT (nmol/L) c 922 827 >550 IGF-1 (nmol/L) N/A 26.5 15.1-40.2 Growth hormone (daily curve, μg/L) e 11.5…0.1…0.1 1.6…0.2…0.4 <0.1 Peak GH after ITT (μg/L) c 2.4 6.1 >3 Abbreviations: FSH , follicle-stimulating hormone; LHRH , luteinizing hormone-releasing hormone; LH , luteinizing hormone; ITT , insulin tolerance test; TRH , thyrotropin-releasing hormone; IGF-1 , insulin-like growth factor 1; GH , growth hormone. a LH measured every 15 minutes during 8 hours; b Mean of three diurnal values measured at 08 h-11 h-13 h; c After insulin tolerance test; d After TRH test; e Three diurnal values measured at 08 h-11 h-13 h; >> > b : Several-fold increase compared to the mean daily prolactin.	4.195313	0.968262	sec[1]/p[0]	en	0.999998	25868523	https://doi.org/10.1186/s12883-015-0283-7	[ "hormone", "brain", "peak", "neurologic", "prolactin", "growth", "scan", "nmol", "imaging", "hypomyelination" ]	[ { "code": "5B3Z", "title": "Endocrine diseases, unspecified" }, { "code": "6C4H.1Z", "title": "Harmful pattern of use of non-psychoactive substances, unspecified" }, { "code": "5A61.3", "title": "Growth hormone deficiency" }, { "code": "5A61.0", "title": "Hypopituitarism" }, { "code": "5A81.1", "title": "Testicular hypofunction" }, { "code": "8E7Y", "title": "Other specified diseases of the nervous system" }, { "code": "LA05.Z", "title": "Cerebral structural developmental anomalies, unspecified" }, { "code": "1D00.Z", "title": "Infectious encephalitis, unspecified" }, { "code": "LA00.0Z", "title": "Anencephaly, unspecified" }, { "code": "NA07.3Y", "title": "Other specified diffuse brain injury" } ]	=== ICD-11 CODES FOUND === [5B3Z] Endocrine diseases, unspecified Also known as: Endocrine diseases, unspecified \| endocrine disorder NOS \| disorder of endocrine gland \| disease of endocrine gland \| disorder of endocrine system [6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified Also known as: Harmful pattern of use of non-psychoactive substances, unspecified \| Harmful pattern of use of non-psychoactive substances \| harmful use of nonprescribed drugs, non-dependence producing \| Abuse of antacids \| Abuse of herbal or folk remedies [5A61.3] Growth hormone deficiency Definition: Deficiency of growth hormone in children, adolescents and adults. Includes deficiency of growth hormone releasing hormone (GHRH) and excess of central somatostatin, leading to growth hormone deficiency. Includes idiopathic, inborn and acquired forms of growth hormone deficiency. Also known as: Growth hormone deficiency Excludes: Hypopituitarism [5A61.0] Hypopituitarism Definition: A disorder manifesting a deficiency or decrease of one or more pituitary hormones, which is caused by a variety of diseases such as tumour, trauma/surgery, irradiation, inflammation and haemorrhage/infarction. Also known as: Hypopituitarism \| subpituitarism \| hypophyseal dystrophy \| hypohypophysism \| anterior pituitary insufficiency (in part) Includes: pituitary cachexia \| pituitary short stature [5A81.1] Testicular hypofunction Definition: In pre-puberty, a disorder characterised by atrophied testes and sterility, abnormal height and absence of secondary sex characteristics. In post-puberty, a disorder characterised by depressed sexual function, loss of sex drive and sterility, muscle weakness and osteoporosis (due to loss of the androgen anabolic effect). Also known as: Testicular hypofunction \| hypofunction testicle \| gonadal insufficiency of testis \| Testicular hypogonadism NOS \| undeveloped testis [8E7Y] Other specified diseases of the nervous system Also known as: Other specified diseases of the nervous system \| Circumscribed brain atrophy \| circumscribed cerebral atrophy \| atrophic lobar sclerosis \| atrophic lobar brain sclerosis [LA05.Z] Cerebral structural developmental anomalies, unspecified Also known as: Cerebral structural developmental anomalies, unspecified \| Cerebral structural developmental anomalies \| Malformations of brain \| brain abnormality NOS \| brain deformity NOS [1D00.Z] Infectious encephalitis, unspecified Also known as: Infectious encephalitis, unspecified \| Infectious encephalitis, not elsewhere classified \| encephalitis NOS \| acute encephalitis NOS \| acute brain inflammation [LA00.0Z] Anencephaly, unspecified Also known as: Anencephaly, unspecified \| Anencephaly \| anencephalic monster \| anencephalus \| brain absence [NA07.3Y] Other specified diffuse brain injury Also known as: Other specified diffuse brain injury \| Brain contusion \| Cerebral contusion NOS \| Diffuse cortex contusion \| diffuse cortical contusion === GRAPH WALKS === --- Walk 1 --- [5B3Z] Endocrine diseases, unspecified --PARENT--> [?] Endocrine diseases --CHILD--> [?] Diabetes mellitus Def: A metabolic disorder with heterogenous aetiologies which is characterised by chronic hyperglycaemia and disturbances of carbohydrate, fat and protein metabolism resulting from defects in insulin secre... --- Walk 2 --- [5B3Z] Endocrine diseases, unspecified --PARENT--> [?] Endocrine diseases --CHILD--> [?] Diabetes mellitus Def: A metabolic disorder with heterogenous aetiologies which is characterised by chronic hyperglycaemia and disturbances of carbohydrate, fat and protein metabolism resulting from defects in insulin secre... --- Walk 3 --- [6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified --PARENT--> [6C4H.1] Harmful pattern of use of non-psychoactive substances Def: A pattern of use of non-psychoactive substances that has caused clinically significant harm to a person’s physical or mental health. The pattern of use is evident over a period of at least 12 months i... --CHILD--> [6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified --- Walk 4 --- [6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified --PARENT--> [6C4H.1] Harmful pattern of use of non-psychoactive substances Def: A pattern of use of non-psychoactive substances that has caused clinically significant harm to a person’s physical or mental health. The pattern of use is evident over a period of at least 12 months i... --CHILD--> [6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified --- Walk 5 --- [5A61.3] Growth hormone deficiency Def: Deficiency of growth hormone in children, adolescents and adults. Includes deficiency of growth hormone releasing hormone (GHRH) and excess of central somatostatin, leading to growth hormone deficienc... --EXCLUDES--> [?] Hypopituitarism Def: A disorder manifesting a deficiency or decrease of one or more pituitary hormones, which is caused by a variety of diseases such as tumour, trauma/surgery, irradiation, inflammation and haemorrhage/in... --CHILD--> [?] Argonz-del Castillo Syndrome --- Walk 6 --- [5A61.3] Growth hormone deficiency Def: Deficiency of growth hormone in children, adolescents and adults. Includes deficiency of growth hormone releasing hormone (GHRH) and excess of central somatostatin, leading to growth hormone deficienc... --EXCLUDES--> [?] Hypopituitarism Def: A disorder manifesting a deficiency or decrease of one or more pituitary hormones, which is caused by a variety of diseases such as tumour, trauma/surgery, irradiation, inflammation and haemorrhage/in... --PARENT--> [?] Hypofunction or certain other specified disorders of pituitary gland Def: Clinical status with disordered function of the pituitary gland without excessive pituitary hormone production, which is caused by a variety of diseases...	[ "[5B3Z] Endocrine diseases, unspecified\n --PARENT--> [?] Endocrine diseases\n --CHILD--> [?] Diabetes mellitus\n Def: A metabolic disorder with heterogenous aetiologies which is characterised by chronic hyperglycaemia and disturbances of carbohydrate, fat and protein metabolism resulting from defects in insulin secre...", "[5B3Z] Endocrine diseases, unspecified\n --PARENT--> [?] Endocrine diseases\n --CHILD--> [?] Diabetes mellitus\n Def: A metabolic disorder with heterogenous aetiologies which is characterised by chronic hyperglycaemia and disturbances of carbohydrate, fat and protein metabolism resulting from defects in insulin secre...", "[6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified\n --PARENT--> [6C4H.1] Harmful pattern of use of non-psychoactive substances\n Def: A pattern of use of non-psychoactive substances that has caused clinically significant harm to a person’s physical or mental health. The pattern of use is evident over a period of at least 12 months i...\n --CHILD--> [6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified", "[6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified\n --PARENT--> [6C4H.1] Harmful pattern of use of non-psychoactive substances\n Def: A pattern of use of non-psychoactive substances that has caused clinically significant harm to a person’s physical or mental health. The pattern of use is evident over a period of at least 12 months i...\n --CHILD--> [6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified", "[5A61.3] Growth hormone deficiency\n Def: Deficiency of growth hormone in children, adolescents and adults. Includes deficiency of growth hormone releasing hormone (GHRH) and excess of central somatostatin, leading to growth hormone deficienc...\n --EXCLUDES--> [?] Hypopituitarism\n Def: A disorder manifesting a deficiency or decrease of one or more pituitary hormones, which is caused by a variety of diseases such as tumour, trauma/surgery, irradiation, inflammation and haemorrhage/in...\n --CHILD--> [?] Argonz-del Castillo Syndrome", "[5A61.3] Growth hormone deficiency\n Def: Deficiency of growth hormone in children, adolescents and adults. Includes deficiency of growth hormone releasing hormone (GHRH) and excess of central somatostatin, leading to growth hormone deficienc...\n --EXCLUDES--> [?] Hypopituitarism\n Def: A disorder manifesting a deficiency or decrease of one or more pituitary hormones, which is caused by a variety of diseases such as tumour, trauma/surgery, irradiation, inflammation and haemorrhage/in...\n --PARENT--> [?] Hypofunction or certain other specified disorders of pituitary gland\n Def: Clinical status with disordered function of the pituitary gland without excessive pituitary hormone production, which is caused by a variety of diseases..." ]	5B3Z	Endocrine diseases, unspecified	[ { "from_icd11": "5B3Z", "icd10_code": "E342", "icd10_title": "Ectopic hormone secretion, not elsewhere classified" }, { "from_icd11": "5B3Z", "icd10_code": "E348", "icd10_title": "Other specified endocrine disorders" }, { "from_icd11": "5B3Z", "icd10_code": "E349", "icd10_title": "Endocrine disorder, unspecified" }, { "from_icd11": "5B3Z", "icd10_code": "E20-E35", "icd10_title": "" }, { "from_icd11": "5B3Z", "icd10_code": "E34", "icd10_title": "Other endocrine disorders" }, { "from_icd11": "5B3Z", "icd10_code": "E35", "icd10_title": "Disorders of endocrine glands in diseases classified elsewhere" }, { "from_icd11": "5B3Z", "icd10_code": "E351", "icd10_title": "" }, { "from_icd11": "6C4H.1Z", "icd10_code": "F558", "icd10_title": "Abuse of other non-psychoactive substances" }, { "from_icd11": "6C4H.1Z", "icd10_code": "F552", "icd10_title": "Abuse of laxatives" }, { "from_icd11": "6C4H.1Z", "icd10_code": "F553", "icd10_title": "Abuse of steroids or hormones" }, { "from_icd11": "6C4H.1Z", "icd10_code": "F551", "icd10_title": "Abuse of herbal or folk remedies" }, { "from_icd11": "6C4H.1Z", "icd10_code": "F55", "icd10_title": "Abuse of non-psychoactive substances" }, { "from_icd11": "5A61.0", "icd10_code": "E230", "icd10_title": "Hypopituitarism" }, { "from_icd11": "5A61.0", "icd10_code": "Q044", "icd10_title": "Septo-optic dysplasia of brain" }, { "from_icd11": "5A61.0", "icd10_code": "E231", "icd10_title": "Drug-induced hypopituitarism" } ]	E342	Ectopic hormone secretion, not elsewhere classified
A 67-year-old Japanese woman (body mass index [BMI], 16.9), who was initially diagnosed with left distal femur GCTB and graded as Campanacci grade II, was treated with phenol and ethanol adjuvant curettage and the filling of the cavity with CPC (Biopex®-R; Pentax Co., Tokyo, Japan) following the intraoperative pathological diagnosis of GCTB . The final diagnosis by permanent section was also confirmed as GCTB . The postoperative course was uneventful; however, 5 years postoperatively, she gradually started complaining of knee pain . The pain subsequently became severe, and magnetic resonance imaging 5 years postoperatively revealed synovitis without apparent local recurrence . Needle biopsy confirmed chronic synovitis , and plain radiogram 6 years postoperatively showed K-L grade 4 (lateral tibiofemoral and patellofemoral) and grade 2 (medial tibiofemoral) osteoarthritis . Moreover, the patient complained of a restricted knee range of motion (− 20 to 130 degrees); however, she did not show lateral instability. Knee Society Knee Score (KSKS) and Knee Society Function Score (KSFS) were 27 and 29, respectively . Preoperative radiogram showed K-L grade 1 osteoarthritis that progressed during the last 6 years, without major risk factors, including high BMI (> 30) and injury, other than the female sex and older age (> 65 years old) . Therefore, we diagnosed the case as secondary osteoarthritis. Standard TKA was initially planned; however, cutting the femoral surface using an intramedullary alignment nail was seemingly difficult due to considerable amounts of CPC. Furthermore, we were concerned about the risk of CPC cracks during femoral surface cutting using a standard bone saw. Therefore, we planned to use a CT-free navigation system (Navigation: Stryker Navigation Cart System: Stryker Orthopaedics, Mahwah, NJ, USA, Software: Stryker Knee Navigation Ver2.0: Stryker Orthopaedics, Mahwah, NJ, USA), which enables accurate bone cutting without an intramedullary alignment rod. Preoperative planning was performed using a 3D-templating software (ZedView, ZedKnee; LEXI Co., Ltd., Tokyo, Japan) to determine the size and position of the implant . Intraoperatively, the knee joint was approached via a midline skin incision, and the suprapatellar pouch was filled with the proliferative synovial tissue. CPC and degenerative joint surface were exposed following synovial tissue resection, which was histologically diagnosed as chronic synovitis ; two truckers were set on the femur and tibia. According to a previous report, we set the mechanical axes of both the femur and tibia as an anatomical index for the image-free navigation system . First, in the femur, we registered at the center of the femoral head, lateral and medial epicondyles of the femur, surface of the medial and lateral condyle, Whiteside line, and knee center. Next, in the tibia, we registered at the anterior-posterior axis, lateral and medial malleolus of the ankle, medial and lateral tibial articular surface, and knee center. After registration, cutting blocks were set at the distal end of the femur and proximal end of the tibia perpendicular to their bone axes. The amount of femoral osteotomy was defined as the thickness of the implant from the femur medial joint surface, and the amount of tibial osteotomy was determined using the fibula head height. A total of 9 mm of the distal medial condyle was cut using abductor rotation, as determined by the surgical epicondylar axis (SEA). The anterior and posterior surfaces were cut via a cutting block using a Stryker Precision® blade and a Stryker Precision System 6 saw (Stryker Instruments) according to the anterior surface, which enabled the cutting of CPC without cracks. Subsequently, the tibial cutting guide was mounted using the support of navigation . After the tibial surface was cut, the implant trial was placed, and the flexion and extension gap were assessed. Finally, the component (Triathlon CR [Stryker]) was implanted with cement; 4-, 3-, and 9-mm inserts were used for the femur, tibia, and CS, respectively . The patella was resurfacing. A postoperative radiogram showed the precise alignment of the component and mechanical axis, which was cited at the center of the knee joint . The postoperative course was uneventful without any adverse event. Four years later, radiogram showed good alignment and no loosening of the component . The knee range of motion was 0 to 95 degrees. KSKS and KSFS ameliorated to 64 and 68, respectively. Fig. 1 Preoperative radiogram images. Preoperative plain radiograms of the left knee joint showed an osteolytic lesion with scalloping ( a ). A postoperative plain radiogram showed that the cavity was filled with calcium phosphate cement ( b ). A plain radiogram after 5 years showed a K-L grade 3 joint space narrowing ( c ). A plain radiogram after 6 years showed the progressive joint space narrowing; the narrowing by the time of this radiogram was graded as K-L grade 4 (lateral tibiofemoral) and grade 2 (medial tibiofemoral) osteoarthritis ( d ). A CT imaging showed K-L grade 4 patellofemoral osteoarthritis Fig. 2 Three-dimensional templating imaging ( a : coronal, b : sagittal, c : axial, d : 3D view) for computed tomography-free navigation assisted-surgery. The distal anterior part of calcium phosphate cement needed to be resected ( b, c ). The size and alignment of the component were three-dimensionally visualized ( d ) Fig. 3 Intraoperative photographs. Degenerative changes in the femoral articular surface and synovitis were observed. The black arrow showed calcium phosphate cement ( a ). After femoral and tibial cutting, no crack was observed. Two trackers were set on the femur and tibia ( b ). The femoral and tibial components were placed. The patella was also resurfaced ( c ) Fig. 4 Postoperative radiogram images. Postoperative radiograms showed that the implant was placed with accurate alignment (anterior-posterior view [ a ]: aLDFA: 83.4° and MPTA: 89.8°; and lateral view [ b ]: femoral component flexion: 5.6° and tibial tilt: 3.8°). Whole leg radiograms in a standing position taken 1 year postoperatively showed that the mechanical axis passed the center of the tibial component ( c ). aLDFA: anatomical lateral distal femur angle, MPTA: medial proximal tibia angle Fig. 5 Plain radiogram images. Plain radiograms taken 4 years postoperatively showed no implant loosening. Anterior-posterior view ( a ). Lateral view ( b )	4.113281	0.958496	sec[1]/p[0]	en	0.999997	35193563	https://doi.org/10.1186/s12891-022-05131-0	[ "knee", "radiogram", "femur", "surface", "grade", "cutting", "femoral", "stryker", "tibial", "plain" ]	[ { "code": "FA2Z", "title": "Inflammatory arthropathies, unspecified" }, { "code": "NC90.Y", "title": "Other specified superficial injury of knee or lower leg" }, { "code": "FA34.4", "title": "Ankylosis of joint" }, { "code": "FA33.4Z", "title": "Chronic instability of knee, unspecified" }, { "code": "NC90.0", "title": "Abrasion of knee" }, { "code": "LB9A.8", "title": "Femoral agenesis or hypoplasia" }, { "code": "FA31.8", "title": "Acquired unequal limb length" }, { "code": "FA31.Y", "title": "Other specified acquired deformities of limbs" }, { "code": "8C11.2", "title": "Lesion of femoral nerve" }, { "code": "FB86.11", "title": "Hypertrophy of bone" } ]	=== ICD-11 CODES FOUND === [FA2Z] Inflammatory arthropathies, unspecified Also known as: Inflammatory arthropathies, unspecified \| polyarthritis NOS \| inflammatory joint disease NOS \| nonpyogenic arthritis NOS \| arthritic nodosa [NC90.Y] Other specified superficial injury of knee or lower leg Also known as: Other specified superficial injury of knee or lower leg \| Nonthermal blister of other or unspecified parts of lower leg \| Nonvenomous insect bite of other or unspecified parts of lower leg \| Superficial foreign body in other or unspecified parts of lower leg \| Splinter in other or unspecified parts of lower leg [FA34.4] Ankylosis of joint Definition: The term ankylosis denotes restricted movement in the joint, and it can be bony or fibrous. Most cases are caused by trauma, infection, radiotherapy, or severe arthritic condition. Also known as: Ankylosis of joint \| ankylosis \| ankylosis of joint, site unspecified \| frozen joint \| fusion of joint Excludes: stiffness of joint without ankylosis \| Ankylosis of spinal joint [FA33.4Z] Chronic instability of knee, unspecified Also known as: Chronic instability of knee, unspecified \| Chronic instability of knee \| instability of knee \| old disruption of ligament of knee [NC90.0] Abrasion of knee Also known as: Abrasion of knee [LB9A.8] Femoral agenesis or hypoplasia Definition: Femoral agenesis/hypoplasia is a rare malformation of variable severity ranging from mild hypoplasia to complete absence of the femur. Also known as: Femoral agenesis or hypoplasia \| absence of femur \| absent femur \| agenesis of femur \| congenital absence of femur [FA31.8] Acquired unequal limb length Also known as: Acquired unequal limb length \| unequal length of limbs \| unequal limb length \| Acquired unequal limb length, multiple sites \| Acquired unequal limb length, shoulder region [FA31.Y] Other specified acquired deformities of limbs Also known as: Other specified acquired deformities of limbs \| Acquired deformity of forearm \| Deflection of radius \| Bowing of the radius \| Bowing of forearm [8C11.2] Lesion of femoral nerve Also known as: Lesion of femoral nerve \| Femoral neuropathy \| Lesion of saphenous nerve Excludes: Injury of femoral nerve at hip or thigh level [FB86.11] Hypertrophy of bone Also known as: Hypertrophy of bone \| bone thickening \| periosteum thickening \| Infantile cortical hyperostoses \| Post traumatic subperiosteal ossification === GRAPH WALKS === --- Walk 1 --- [FA2Z] Inflammatory arthropathies, unspecified --PARENT--> [?] Inflammatory arthropathies --RELATED_TO--> [?] Peripheral spondyloarthritis Def: Experts from the Assessment of SpondyloArthritis international Society (ASAS) developed classification criteria for axSpA and peripheral SpA. These criteria were developed for patients with peripheral... --- Walk 2 --- [FA2Z] Inflammatory arthropathies, unspecified --PARENT--> [?] Inflammatory arthropathies --RELATED_TO--> [?] Peripheral spondyloarthritis Def: Experts from the Assessment of SpondyloArthritis international Society (ASAS) developed classification criteria for axSpA and peripheral SpA. These criteria were developed for patients with peripheral... --- Walk 3 --- [NC90.Y] Other specified superficial injury of knee or lower leg --PARENT--> [NC90] Superficial injury of knee or lower leg --CHILD--> [NC90.0] Abrasion of knee --- Walk 4 --- [NC90.Y] Other specified superficial injury of knee or lower leg --PARENT--> [NC90] Superficial injury of knee or lower leg --PARENT--> [?] Injuries to the knee or lower leg --- Walk 5 --- [FA34.4] Ankylosis of joint Def: The term ankylosis denotes restricted movement in the joint, and it can be bony or fibrous. Most cases are caused by trauma, infection, radiotherapy, or severe arthritic condition.... --EXCLUDES--> [?] Ankylosis of spinal joint --CHILD--> [?] Ankylosis of cervical spinal joint --- Walk 6 --- [FA34.4] Ankylosis of joint Def: The term ankylosis denotes restricted movement in the joint, and it can be bony or fibrous. Most cases are caused by trauma, infection, radiotherapy, or severe arthritic condition.... --EXCLUDES--> [?] Ankylosis of spinal joint --PARENT--> [?] Spondylopathies	[ "[FA2Z] Inflammatory arthropathies, unspecified\n --PARENT--> [?] Inflammatory arthropathies\n --RELATED_TO--> [?] Peripheral spondyloarthritis\n Def: Experts from the Assessment of SpondyloArthritis international Society (ASAS) developed classification criteria for axSpA and peripheral SpA. These criteria were developed for patients with peripheral...", "[FA2Z] Inflammatory arthropathies, unspecified\n --PARENT--> [?] Inflammatory arthropathies\n --RELATED_TO--> [?] Peripheral spondyloarthritis\n Def: Experts from the Assessment of SpondyloArthritis international Society (ASAS) developed classification criteria for axSpA and peripheral SpA. These criteria were developed for patients with peripheral...", "[NC90.Y] Other specified superficial injury of knee or lower leg\n --PARENT--> [NC90] Superficial injury of knee or lower leg\n --CHILD--> [NC90.0] Abrasion of knee", "[NC90.Y] Other specified superficial injury of knee or lower leg\n --PARENT--> [NC90] Superficial injury of knee or lower leg\n --PARENT--> [?] Injuries to the knee or lower leg", "[FA34.4] Ankylosis of joint\n Def: The term ankylosis denotes restricted movement in the joint, and it can be bony or fibrous. Most cases are caused by trauma, infection, radiotherapy, or severe arthritic condition....\n --EXCLUDES--> [?] Ankylosis of spinal joint\n --CHILD--> [?] Ankylosis of cervical spinal joint", "[FA34.4] Ankylosis of joint\n Def: The term ankylosis denotes restricted movement in the joint, and it can be bony or fibrous. Most cases are caused by trauma, infection, radiotherapy, or severe arthritic condition....\n --EXCLUDES--> [?] Ankylosis of spinal joint\n --PARENT--> [?] Spondylopathies" ]	FA2Z	Inflammatory arthropathies, unspecified	[ { "from_icd11": "FA2Z", "icd10_code": "M1389", "icd10_title": "Other specified arthritis, multiple sites" }, { "from_icd11": "FA2Z", "icd10_code": "M1380", "icd10_title": "Other specified arthritis, unspecified site" }, { "from_icd11": "FA2Z", "icd10_code": "M13862", "icd10_title": "Other specified arthritis, left knee" }, { "from_icd11": "FA2Z", "icd10_code": "M13872", "icd10_title": "Other specified arthritis, left ankle and foot" }, { "from_icd11": "FA2Z", "icd10_code": "M13871", "icd10_title": "Other specified arthritis, right ankle and foot" }, { "from_icd11": "FA2Z", "icd10_code": "M13861", "icd10_title": "Other specified arthritis, right knee" }, { "from_icd11": "FA2Z", "icd10_code": "M13879", "icd10_title": "Other specified arthritis, unspecified ankle and foot" }, { "from_icd11": "FA2Z", "icd10_code": "M13842", "icd10_title": "Other specified arthritis, left hand" }, { "from_icd11": "FA2Z", "icd10_code": "M13841", "icd10_title": "Other specified arthritis, right hand" }, { "from_icd11": "FA2Z", "icd10_code": "M13811", "icd10_title": "Other specified arthritis, right shoulder" }, { "from_icd11": "FA2Z", "icd10_code": "M13162", "icd10_title": "Monoarthritis, not elsewhere classified, left knee" }, { "from_icd11": "FA2Z", "icd10_code": "M13869", "icd10_title": "Other specified arthritis, unspecified knee" }, { "from_icd11": "FA2Z", "icd10_code": "M1388", "icd10_title": "Other specified arthritis, other site" }, { "from_icd11": "FA2Z", "icd10_code": "M13171", "icd10_title": "Monoarthritis, not elsewhere classified, right ankle and foot" }, { "from_icd11": "FA2Z", "icd10_code": "M13152", "icd10_title": "Monoarthritis, not elsewhere classified, left hip" } ]	M1389	Other specified arthritis, multiple sites
A 74-year old woman was admitted to the geriatric department with weakness, recurrent falls, confusion, episodes of irresponsiveness, anorexia and weight loss (10 kg over 6 months). Her medical history consisted of herpes encephalitis 8 years ago, postlesional epilepsy, osteoporosis and a bariatric Scopinaro procedure at the age of 52 . In the previous 3 months, she had already been hospitalised on two occasions to the geriatric department. During her first admission for recurrent falls, a multifactorial origin was assigned to the falling including (i) malabsorption with deficiencies of vitamin A and E, (ii) a urinary tract infection, and (iii) recurrent episodes of irresponsiveness that were thought to be epilepsy. However, serum levels of anti-epileptic drugs and an electroencephalogram did not confirm the epilepsy diagnosis. During this hospitalisation, there was a suspicion of early-stage dementia. Since a few months before the first hospitalisation, there were episodes of confusion, visual hallucinations and day-night reversal. However, the patient still lived alone without any professional support. She required assistance from her daughter for daily-life activities including shopping, laundry and housekeeping. During the admission, a mini-mental state examination (MMSE) revealed a MMSE-score of 28/30. While the Montreal Cognitive Assessment Examination (MOCA) revealed attention and working memory deficits with a MOCA-score of 22/30. Consequently, a transfer to the neuropsychiatric department was recommended for further diagnostic workout. Against medical advice, her daughter took her away from the hospital for a holiday abroad without continuing oral vitamin and mineral supplementation. A few weeks later, the patient was readmitted to the geriatrics department with increasing episodes of confusion and irresponsiveness, complications of being very weak and nearly without any oral intake. Laboratory blood examination revealed persisting deficiencies of vitamin A and E. While a cerebral MRI showed postherpetic sequelae in the right hippocampus and temporal lobe, limited cortico-subcortical and periventricular leuco-atrophy with multiple vasculo-ischemic supratentorial white matter lesions. Moreover, lumbar puncture findings were normal including normal levels of biomarkers for Alzheimer’s disease. Consequently, the presence of delirium was suspected. Considering the diagnosis, the patient was transferred to the neuropsychiatric unit after initiation of oral nutritional support in combination with vitamin and mineral supplementation. Here, a recurrent MMSE revealed a score of 13/30. After 2 weeks, the patient was transferred again to the geriatrics department due to persisting anorexia with difficult oral intake and a continuous decline of her cognitive and functional status. From walking with a walking aid, she had become totally dependent for her daily life activities. Moreover, she remained confused with fluctuating consciousness, which was suggestive for persistent delirium. No other concomitant symptoms were observed or reported, neither diarrhoea nor steatorrhea. However, clinical examination revealed significant hypotension (73/46 mmHg) without fever or tachycardia. In addition, the patient had a cachectic appearance and marked pitting oedema of the lower body. Her BMI amounted 20 kg/m 2 (weight: 48.5 kg, length: 1.55 m), which is an overestimation due to the oedematous state. Her daily medication schedule consisted of vitamin A , vitamin E (dl-alpha-tocopheryl acetate 200 units = 200 mg), iron sulphate 525 mg (105 mg elementary iron), a multivitamin B complex: B1, B2, B6 and B12 (thiamine mononitrate 250 mg, riboflavin 10 mg, pyridoxine hydrochloride 250 mg, cyanocobalamin 0.02 mg; twice daily), folic acid 0.4 mg, a calcium and vitamin D supplement , valproic acid (450 mg; twice daily) and lamotrigine (100 mg; twice daily). Despite the oral nutritional support and supplementation, recurrent laboratory blood examination revealed persistent nutritional deficits including severe hypalbuminaemia (16 g/l; normal range: 35–52), vitamin A deficiency (115 μg/l; normal range: 300–650), vitamin E deficiency (1.5 mg/l; normal range: 5–20) and zinc deficiency (36 μg/dl; normal range: 80–120). While other nutritional markers were within normal range including calcium, 25-OH vitamin D, prothrombin time, folic acid and iron panel (intravenous iron and vitamin K had been administered during the first admission). Moreover, vitamin B1 and B12 levels were high. Unfortunately, copper was not measured on admission. There was no proteinuria and the creatinine level was within the low normal range. Again, plasma antiepileptic drug concentrations were normal. Venous ammonia was normal. Regarding glycaemic control, a glycated haemoglobin of 4.0% (4.0–6.0%), a morning glucose of 94 mg/dl and at the same time, a slightly elevated C-peptide was observed. However, recurrent hypoglycaemia up to 43 mg/dl was observed after her meals and was accompanied by confusion and blurred consciousness. After a multidisciplinary consultation between the geriatrics, endocrinology and abdominal surgery department, treatment with parenteral nutrition was initiated in combination with oral micronutrient supplementation. In the course of the next 3 weeks, there was a clear improvement in her cognitive status and daily functioning. After 4 weeks, her MMSE score increased again up to 28/30. In addition, weight gain was observed (5 kg) with an increase in albumin level up to 30.8 g/l. Consequently, she was transferred to a rehabilitation centre while continuing parenteral nutrition for four more weeks. On day seven of the rehabilitation, her MOCA score was 25/30. During the 4 weeks, parenteral nutrition was gradually combined with an oral diet, consisting of small frequent meals rich in proteins, complex carbohydrates, fibres and a limited amount of fat. During this period, her daily functioning improved significantly to a point where she regained her independence in daily life activities except for the use of a walking aid. After her stay in the rehabilitation centre, revision surgery was planned to reduce malabsorption by lengthening the common limb. Unfortunately, she developed catheter sepsis in the rehabilitation centre during the fourth week. Consequently, she was transferred to a high dependency unit were she died from subsequent Clostridium difficile pseudomembranous colitis.	3.972656	0.977051	sec[1]/p[0]	en	0.999997	34736423	https://doi.org/10.1186/s12877-021-02578-z	[ "vitamin", "daily", "oral", "department", "recurrent", "range", "including", "score", "confusion", "episodes" ]	[ { "code": "5B7Z", "title": "Unspecified undernutrition" }, { "code": "5B90.Z", "title": "Unspecified vitamin excesses" }, { "code": "5B55.Z", "title": "Vitamin A deficiency, unspecified" }, { "code": "6C4H.1Z", "title": "Harmful pattern of use of non-psychoactive substances, unspecified" }, { "code": "5B90.Y", "title": "Other specified vitamin excess" }, { "code": "QF21", "title": "Difficulty or need for assistance with general life tasks or life management" }, { "code": "8A83", "title": "Other primary headache disorder" }, { "code": "QB42", "title": "Dependence on renal dialysis" }, { "code": "MD11.8Z", "title": "Mouth breathing, unspecified" }, { "code": "DA01.00", "title": "Oral leukoplakia" } ]	=== ICD-11 CODES FOUND === [5B7Z] Unspecified undernutrition Also known as: Unspecified undernutrition \| Malnutrition NOS \| nutritional deficiency NOS \| nutritional depletion NOS \| severe malnutrition NOS [5B90.Z] Unspecified vitamin excesses Also known as: Unspecified vitamin excesses \| Vitamin excesses [5B55.Z] Vitamin A deficiency, unspecified Also known as: Vitamin A deficiency, unspecified \| Vitamin A deficiency \| Hypovitaminosis A [6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified Also known as: Harmful pattern of use of non-psychoactive substances, unspecified \| Harmful pattern of use of non-psychoactive substances \| harmful use of nonprescribed drugs, non-dependence producing \| Abuse of antacids \| Abuse of herbal or folk remedies [5B90.Y] Other specified vitamin excess Also known as: Other specified vitamin excess [QF21] Difficulty or need for assistance with general life tasks or life management Also known as: Difficulty or need for assistance with general life tasks or life management \| difficulty with carrying out tasks and daily routine \| life management problem \| difficulty with life management tasks \| Difficulty with dealing with change such as relocation Includes: difficulty with carrying out tasks and daily routine [8A83] Other primary headache disorder Definition: A group of clinically heterogeneous headache disorders, believed to be primary. Although largely unrelated, they fall into four categories: headaches associated with physical exertion; headaches attributed to direct physical but innocuous stimuli; epicranial headaches; and other miscellaneous primary headache disorders. Also known as: Other primary headache disorder \| Primary cough headache \| Primary exercise headache \| Primary headache associated with sexual activity \| Preorgasmic headache [QB42] Dependence on renal dialysis Also known as: Dependence on renal dialysis \| renal dialysis status \| presence of arteriovenous shunt for dialysis \| dependence on haemodialysis \| Dependence on renal dialysis, acute haemodialysis Includes: renal dialysis status Excludes: dialysis preparation, treatment or session [MD11.8Z] Mouth breathing, unspecified Also known as: Mouth breathing, unspecified \| Mouth breathing \| breathing orally \| mouth respiration [DA01.00] Oral leukoplakia Definition: Leukoplakia is a condition where areas of keratosis appear as adherent white patches on the mucous membranes of the oral cavity. Leukoplakia may affect other gastrointestinal tract mucosal sites, or mucosal surfaces of the urinary tract and genitals. Also known as: Oral leukoplakia \| Leukoplakia of gingiva \| leukoplakia of oral epithelium \| leucoplakia of oral mucosa \| leukokeratosis of oral mucosa Includes: Leukoplakia of gingiva Excludes: Hairy leukoplakia === GRAPH WALKS === --- Walk 1 --- [5B7Z] Unspecified undernutrition --PARENT--> [?] Undernutrition Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c... --CHILD--> [5B51] Wasting in infants, children or adolescents --- Walk 2 --- [5B7Z] Unspecified undernutrition --PARENT--> [?] Undernutrition Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c... --CHILD--> [5B51] Wasting in infants, children or adolescents --- Walk 3 --- [5B90.Z] Unspecified vitamin excesses --PARENT--> [5B90] Vitamin excesses --CHILD--> [5B90.2] Hypervitaminosis D Def: Hypervitaminosis D is secondary to excessive intake of vitamin D. It can occur with long-term high intake or with a substantial, acute ingestion. Excess amounts result in abnormally high concentration... --- Walk 4 --- [5B90.Z] Unspecified vitamin excesses --PARENT--> [5B90] Vitamin excesses --CHILD--> [5B90.2] Hypervitaminosis D Def: Hypervitaminosis D is secondary to excessive intake of vitamin D. It can occur with long-term high intake or with a substantial, acute ingestion. Excess amounts result in abnormally high concentration... --- Walk 5 --- [5B55.Z] Vitamin A deficiency, unspecified --PARENT--> [5B55] Vitamin A deficiency Def: Vitamin A deficiency (VAD) is defined as a state in which tissue concentrations of vitamin A are low enough to have adverse health consequences even if there is no evidence of clinical xerophthalmia. ... --RELATED_TO--> [?] Acquired vitamin A deficiency anaemia --- Walk 6 --- [5B55.Z] Vitamin A deficiency, unspecified --PARENT--> [5B55] Vitamin A deficiency Def: Vitamin A deficiency (VAD) is defined as a state in which tissue concentrations of vitamin A are low enough to have adverse health consequences even if there is no evidence of clinical xerophthalmia. ... --RELATED_TO--> [?] Acquired vitamin A deficiency anaemia	[ "[5B7Z] Unspecified undernutrition\n --PARENT--> [?] Undernutrition\n Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c...\n --CHILD--> [5B51] Wasting in infants, children or adolescents", "[5B7Z] Unspecified undernutrition\n --PARENT--> [?] Undernutrition\n Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c...\n --CHILD--> [5B51] Wasting in infants, children or adolescents", "[5B90.Z] Unspecified vitamin excesses\n --PARENT--> [5B90] Vitamin excesses\n --CHILD--> [5B90.2] Hypervitaminosis D\n Def: Hypervitaminosis D is secondary to excessive intake of vitamin D. It can occur with long-term high intake or with a substantial, acute ingestion. Excess amounts result in abnormally high concentration...", "[5B90.Z] Unspecified vitamin excesses\n --PARENT--> [5B90] Vitamin excesses\n --CHILD--> [5B90.2] Hypervitaminosis D\n Def: Hypervitaminosis D is secondary to excessive intake of vitamin D. It can occur with long-term high intake or with a substantial, acute ingestion. Excess amounts result in abnormally high concentration...", "[5B55.Z] Vitamin A deficiency, unspecified\n --PARENT--> [5B55] Vitamin A deficiency\n Def: Vitamin A deficiency (VAD) is defined as a state in which tissue concentrations of vitamin A are low enough to have adverse health consequences even if there is no evidence of clinical xerophthalmia. ...\n --RELATED_TO--> [?] Acquired vitamin A deficiency anaemia", "[5B55.Z] Vitamin A deficiency, unspecified\n --PARENT--> [5B55] Vitamin A deficiency\n Def: Vitamin A deficiency (VAD) is defined as a state in which tissue concentrations of vitamin A are low enough to have adverse health consequences even if there is no evidence of clinical xerophthalmia. ...\n --RELATED_TO--> [?] Acquired vitamin A deficiency anaemia" ]	5B7Z	Unspecified undernutrition	[ { "from_icd11": "5B7Z", "icd10_code": "E43", "icd10_title": "Unspecified severe protein-calorie malnutrition" }, { "from_icd11": "5B7Z", "icd10_code": "E538", "icd10_title": "Deficiency of other specified B group vitamins" }, { "from_icd11": "5B7Z", "icd10_code": "E569", "icd10_title": "Vitamin deficiency, unspecified" }, { "from_icd11": "5B7Z", "icd10_code": "E638", "icd10_title": "Other specified nutritional deficiencies" }, { "from_icd11": "5B7Z", "icd10_code": "E639", "icd10_title": "Nutritional deficiency, unspecified" }, { "from_icd11": "5B7Z", "icd10_code": "E41", "icd10_title": "Nutritional marasmus" }, { "from_icd11": "5B7Z", "icd10_code": "E539", "icd10_title": "Vitamin B deficiency, unspecified" }, { "from_icd11": "5B7Z", "icd10_code": "E568", "icd10_title": "Deficiency of other vitamins" }, { "from_icd11": "5B7Z", "icd10_code": "E649", "icd10_title": "Sequelae of unspecified nutritional deficiency" }, { "from_icd11": "5B7Z", "icd10_code": "E618", "icd10_title": "Deficiency of other specified nutrient elements" }, { "from_icd11": "5B7Z", "icd10_code": "E40-E46", "icd10_title": "" }, { "from_icd11": "5B7Z", "icd10_code": "E50-E64", "icd10_title": "" }, { "from_icd11": "5B7Z", "icd10_code": "E53", "icd10_title": "Deficiency of other B group vitamins" }, { "from_icd11": "5B7Z", "icd10_code": "E56", "icd10_title": "Other vitamin deficiencies" }, { "from_icd11": "5B7Z", "icd10_code": "E61", "icd10_title": "Deficiency of other nutrient elements" } ]	E43	Unspecified severe protein-calorie malnutrition
A previously healthy 21 year old Sri Lankan female university student admitted with shortness of breath for 1 week duration. Shortness of breath was mainly on exertion, however at the time of admission it was present even at rest. She had low grade fever for the last 1 week associated with malaise and profuse sweating. Her weight and appetite have been steady throughout. She was not on any long term medication and did not take medication for minor ailments in the recent past to suggest a drug induced hypersensitivity reaction. She does not have a history of conjunctivitis, rhinitis, sinusitis or allergy to any drug or food. She took worm treatment 6 month prior. History was negative for malignancy, thromboembolic disorders and connective tissue diseases. She denied family history or other risk factors for cardiovascular disease. She is a non alcoholic, non smoker and no history of illicit drug use. She was mildly febrile and dyspnoeic at rest. There was no associated pallor or icterus. Generalized oedema or ankle oedema was absent. Physical examination was negative for malignancy, thromboembolic disorders and connective tissue diseases. She was tachycardic with regular, low volume pulse at rate of 120 beats per minute. Jugular venous pressure was elevated 5 cm above the angle of Louis with negative Kussmaul sign. Her blood pressure was 100/70 mmHg on admission. The cardiac apex was at its normal position and heart sounds were slightly muffled with gallop rhythm. There were bilateral basal fine end inspiratory crackles. Firm, non tender mild hepatomegaly with mild splenomegaly were present. Otherwise her clinical examination was normal. Her full blood count revealed absolute rise in eosinophil count of 21.6 × 10 3 per microliter (63.5%) with 34 leukocytes per microliter with normal platelet count, haemoglobin and red cell indices. Blood picture showed high total white cell count with severe eosinophilia with no abnormal cells. Her erythrocyte sedimentation rate was 60 mm in 1st h in the presence of normal C-reactive protein. Sinus tachycardia with wide spread ST depression was evident on electrocardiogram. Chest X-ray was normal other than the evidence of heart failure. 2D-echocardiogram showed global left ventricular hypokinesia with 40% ejection fraction and thin layer of pericardial effusion. There was no associated intra cardiac thrombus. Troponin I was elevated up to 35.4 ng/dL (< 1.0 ng/dL) and Brain natriuretic peptide was 1280.5 pg/mL. Her renal function, thyroid function, serum electrolyte, calcium, magnesium levels, lipid profile were all normal with normal liver function but elevated liver enzymes (both were at 3 times upper limit of normal). Both serum IgM and IgG were negative for Filaria, Toxoplasma and toxocara infection and stool examination was negative for parasites. Serology for Epstein-Barr virus, Cytomegalo virus and Mycoplasma were negative. Retroviral infection and tuberculosis were excluded. Her blood culture and autoimmune screen were negative. Mild hepatosplenomegaly without lymphadenopathy was detected in otherwise normal ultrasound scan. Bone marrow biopsy showed hypereosinophilia with no evidence of bone marrow infiltration by lymphoproliferative malignancy. FIP1L1–PDGFRA fusion transcript and BCR–ABL transcript were not detected. The patient declined to undergo a confirmatory endomyocardial biopsy. Coronary angiogram and other non invasive cardiac imaging were not performed due to the practical problems of getting them done as they were not freely available. Clinically detected heart failure was confirmed by 2D ECHO and biochemical markers. The presence of global left ventricular dysfunction was better explained with myocarditis than myocardial infarction as there should be multi territory ischaemia to explain it which was less likely in our patient given that she was young and did not had any cardiovascular risk factors. This would have been excluded in certainty with coronary angiogram if it was freely available to us. Peripheral blood eosinophilia pointed towards a probable cause of cardiac damage in an otherwise healthy young female without cardiovascular risk factors. Drug hypersensitivity as a cause of eosinophilia was excluded from history. Bone marrow examination confirmed the diagnosis of hypereosinophilia. Myeloproliferative hypereosinophilic syndrome can give rise to chronic eosinophilic leukaemia. The possibility of this was excluded by negative FIP1L1–PDGFRA and BCR–ABL gene transcription. Secondary causes for hypereosinophilia were excluded and the diagnosis of idiopathic hypereosinophilic syndrome and eosinophilic myocarditis was made depending on the available investigations. Normal thyroid function test, negative ANA, DsDNA, negative antibody tires of common viral infection were used to exclude other differential diagnosis. This young female was given supportive care and treatment for heart failure and monitored in the high dependency unit to observe for possible deterioration. Her blood pressure dropped to 80/60 mmHg on day four of the admission requiring inotropic support. She was treated with intravenous Noradrenaline 0.3 μg/kg/min initially through L/Internal jugular venous catheter and then it was titrated up to 0.5 μg/kg/min to maintain mean arterial pressure of 70 mmHg. As soon as the diagnosis of hypereosinophilic syndrome with eosinophilic myocarditis was made, she was started on methylprednisolone 1 g/day for 3 days and continued with prednisolone 1 mg/kg to a total dose of 50 mg. Her symptoms started responding after the 3rd dose of methylprednisolone. Clinical improvement was observed in terms of symptoms and other parameters like blood pressure and pulse rate. Noradrenaline was then tailed off gradually and stopped after 5 days of starting steroids. Eosinophil count started to drop on day 5 of steroid treatment and it was then 16.31 × 10 3 per microliter. Troponin level gradually normalized over the period of the next 2 weeks. She was discharged after 3 weeks of hospital stay and by this time Eosinophil count was 1.11 × 10 3 per microliter (7.5%) and 2D echocardiography showed ejection fraction of 50% with thin layer of pericardial effusion. Pericardial effusion completely resolved and left ventricular function became normal (EF 60%) in the follow up 2D-ECHO and eosinophil count was at just upper limit of normal (0.52 × 10 3 per microliter) after 2 week of discharge from the hospital.	3.955078	0.981445	sec[1]/p[0]	en	0.999996	29523179	https://doi.org/10.1186/s13104-018-3273-1	[ "blood", "count", "pressure", "microliter", "function", "excluded", "drug", "cardiac", "heart", "eosinophil" ]	[ { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" }, { "code": "MA12.1", "title": "Finding of cocaine in blood" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "MA12.2", "title": "Finding of hallucinogen in blood" }, { "code": "3B63.1Z", "title": "Acquired thrombocytosis, unspecified" }, { "code": "3B64.Z", "title": "Thrombocytopenia, unspecified" }, { "code": "4B0Z", "title": "Immune system disorders involving white cell lineages, unspecified" }, { "code": "4B03.Z", "title": "Eosinophilia, unspecified" }, { "code": "4B00.1Z", "title": "Neutrophilia, unspecified" } ]	=== ICD-11 CODES FOUND === [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS [MA12.1] Finding of cocaine in blood Also known as: Finding of cocaine in blood \| cocaine in blood [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [MA12.2] Finding of hallucinogen in blood Also known as: Finding of hallucinogen in blood \| hallucinogen in blood [3B63.1Z] Acquired thrombocytosis, unspecified Also known as: Acquired thrombocytosis, unspecified \| Acquired thrombocytosis \| Idiopathic haemorrhagic thrombocythaemia \| Essential thrombocythaemia \| primary haemorrhagic thrombocythaemia [3B64.Z] Thrombocytopenia, unspecified Also known as: Thrombocytopenia, unspecified \| Thrombocytopenia \| low platelet count \| low platelets \| decreased platelets [4B0Z] Immune system disorders involving white cell lineages, unspecified Also known as: Immune system disorders involving white cell lineages, unspecified [4B03.Z] Eosinophilia, unspecified Also known as: Eosinophilia, unspecified \| Eosinophilia \| Disorders with increased eosinophil counts \| Idiopathic hypereosinophilic syndrome [4B00.1Z] Neutrophilia, unspecified Also known as: Neutrophilia, unspecified \| Neutrophilia \| Disorders with increased neutrophil counts === GRAPH WALKS === --- Walk 1 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --EXCLUDES--> [?] Certain conditions originating in the perinatal period Def: This chapter includes conditions that have their origin in the perinatal period even though death or morbidity occurs later.... --- Walk 2 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --PARENT--> [?] ICD-11 for Mortality and Morbidity Statistics --- Walk 3 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --CHILD--> [MF50.40] Macroscopic haematuria --- Walk 4 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --EXCLUDES--> [?] Recurrent or persistent glomerular haematuria Def: The presence in the urine of glomerular origin red blood cells (RBCs). In microscopic haematuria the urine appears normal to the naked eye, but examination with a microscope shows a high number of RBC... --- Walk 5 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.1] Finding of cocaine in blood --- Walk 6 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.2] Finding of hallucinogen in blood	[ "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --EXCLUDES--> [?] Certain conditions originating in the perinatal period\n Def: This chapter includes conditions that have their origin in the perinatal period even though death or morbidity occurs later....", "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --PARENT--> [?] ICD-11 for Mortality and Morbidity Statistics", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --CHILD--> [MF50.40] Macroscopic haematuria", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --EXCLUDES--> [?] Recurrent or persistent glomerular haematuria\n Def: The presence in the urine of glomerular origin red blood cells (RBCs). In microscopic haematuria the urine appears normal to the naked eye, but examination with a microscope shows a high number of RBC...", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.1] Finding of cocaine in blood", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.2] Finding of hallucinogen in blood" ]	3C0Z	Diseases of the blood or blood-forming organs, unspecified	[ { "from_icd11": "3C0Z", "icd10_code": "D75A", "icd10_title": "Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia" }, { "from_icd11": "3C0Z", "icd10_code": "D7581", "icd10_title": "Myelofibrosis" }, { "from_icd11": "3C0Z", "icd10_code": "D7582", "icd10_title": "Heparin induced thrombocytopenia (HIT)" }, { "from_icd11": "3C0Z", "icd10_code": "D7589", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D759", "icd10_title": "Disease of blood and blood-forming organs, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "D763", "icd10_title": "Other histiocytosis syndromes" }, { "from_icd11": "3C0Z", "icd10_code": "Q899", "icd10_title": "Congenital malformation, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "III", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D70-D77", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D75", "icd10_title": "Other and unspecified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D77", "icd10_title": "Other disorders of blood and blood-forming organs in diseases classified elsewhere" }, { "from_icd11": "3C0Z", "icd10_code": "D758", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D76", "icd10_title": "Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue" }, { "from_icd11": "MF50.4Z", "icd10_code": "R310", "icd10_title": "Gross hematuria" }, { "from_icd11": "MF50.4Z", "icd10_code": "R312", "icd10_title": "Other microscopic hematuria" } ]	D75A	Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia
An 81-year-old Japanese man, with a weight of 58.7 kg and a height of 166 cm, was scheduled to undergo TURBT for bladder cancer. He had a history of atrial fibrillation and chronic kidney disease. He had a history of smoking 20 cigarettes per day from the age of 20 years until the age of 60 years and consumed one glass of whiskey daily. His performance status was good, and he showed no deficiencies in activities of daily living (ADL). The patient’s family and employment history were unknown. The preoperative chest X-ray was normal, but an electrocardiogram showed atrial fibrillation with a heart rate (HR) of 82 beats/minute. Blood tests taken before surgery revealed relatively high hematocrit and elevated creatinine levels (Table 1 ). His regular oral medications included 5 mg apixaban twice per day and 200 μg tamsulosin hydrochloride once per day, but apixaban was discontinued 2 days before surgery. He was admitted as a walk-in patient the day before surgery. A non per os (NPO) status was determined according to the American Society of Anesthesiologists (ASA) guidelines , and the patient stopped drinking clear liquids 2 hours before the surgery. The patient took 20 mg/kg of 5-ALA orally 2 hours before entering the operating room. At 1 hour after taking 5-ALA, his blood pressure (BP) was 118/78 mmHg. He did not feel unwell, and he walked into the operating room. An intravenous catheter was inserted into the forearm vein, and standard monitoring (electrocardiogram, noninvasive blood pressure, and pulse oximetry) was performed. The patient’s BP and HR before the induction of anesthesia were 95/67 mmHg and 110 beats/minute, respectively. After preoxygenation, general anesthesia was induced with 5 mg of remimazolam, 50 μg of fentanyl, 0.2 μg/kg/minute of remifentanil, and 40 mg of rocuronium. Anesthesia was maintained using 1 mg/kg/hour remimazolam and 0.05 μg/kg/minute remifentanil. After the induction of anesthesia, the patient’s BP decreased to 47/33 mmHg. The electrocardiogram showed atrial fibrillation, and the patient’s HR increased to approximately 135 beats/minute. A supraglottic device (SGD) was inserted for airway management. There were no findings suggestive of anaphylaxis, such as flushing or increased airway pressure. Hypotension persisted despite fluid resuscitation, head-down positioning, and repeated boluses of phenylephrine 0.1 mg (1 mg total), so a bolus of 10 μg noradrenaline (NA) was administered, followed by continuous administration of NA at 0.05 μg/kg/minute. The patient stopped taking anticoagulants before surgery, so there was a possibility that a thrombus had formed in the atrium. Considering the risk of thromboembolism, we did not perform electrical cardioversion. We administered landiolol at 3.5 μg/kg/minute, but rate control was not possible. Hypotension persisted even after NA was administered at 0.05 μg/kg/minute, so we decided to cancel the surgery and woke the patient by administering flumazenil. After awakening from anesthesia, the patient’s systolic blood pressure (sBP) increased to approximately 100 mmHg, so he was extubated. From the induction of general anesthesia to awakening, the sBP was below 80 mmHg for approximately 35 minutes. The patient was conscious and had no neurological abnormalities. The chest X-ray showed no significant changes from before the surgery . After gradually discontinuing NA and observing the patient for 40 minutes, his sBP remained at at least 80 mmHg. The hospital did not have an intensive care unit (ICU), and the use of NA was not permitted in the general ward; therefore, the patient returned to the general ward after it was confirmed that his BP could be maintained without administering NA. After returning to the ward, the patient’s sBP remained at approximately 100 mmHg, but approximately 5 hours after returning to the ward (approximately 9.5 hours after the oral administration of 5-ALA), cardiac arrest and loss of consciousness occurred for approximately 12 seconds . The patient recovered consciousness without the need for resuscitation measures. The patient was conscious and had no neurological abnormalities. The electrocardiogram performed immediately after the patient regained consciousness showed no significant changes from before the surgery, and arterial blood gas analysis (Table 2 ) revealed no obvious pH changes or electrolyte abnormalities. Blood tests conducted at the same time showed an increase in creatinine and a decrease in hematocrit (Table 1 ). The patient’s blood glucose level was normal at 118 mg/dL. He was taken to a hospital with an ICU and admitted to the ICU for further management and care. Echocardiography showed moderate aortic regurgitation, an ejection fraction of 59% and no asynergy. After admission to the ICU, no new medications were administered, and the patient did not experience cardiac arrest. He was discharged from the ICU the next day and was discharged from the hospital 11 days after the cardiac arrest event. The cardiologist determined that a pacemaker and new medical management were not necessary, and the patient was regularly monitored as an outpatient, but no other cardiac arrest events occurred. He underwent TURBT under general anesthesia without oral 5-ALA after 1 and 5 months but had no problems during or after surgery. The exact cause of cardiac arrest is unknown, but given these circumstances, the influence of 5-ALA cannot be ruled out. Table 1 Laboratory blood tests before and after surgery Before surgery After surgery White blood cell count (/μL) 8200 9800 Hemoglobin level (g/dL) 16.8 12.2 Hematocrit level (%) 47.6 34.6 Platelet count (× 10 4 /μL) 16.5 13.9 PT-INR 1.14 1.12 APTT (sec) 37.5 30.4 AST level (IU/L) 18 16 ALT level (IU/L) 18 13 Blood urea nitrogen level (mg/dL) 25.5 24.4 Creatinine level (mg/dL) 1.38 1.55 PT-INR, prothrombin time-international normalized ratio; APTT, activated partial thromboplastin time; AST, aspartate aminotransferase; ALT, alanine aminotransferase Fig. 1 Postoperative chest X-ray Fig. 2 The waveform on the electrocardiogram was monitored before and after cardiac arrest. The duration of cardiac arrest is indicated by the range of the arrow. Since the waveform was 30 seconds per line, the cardiac arrest time was approximately 12 seconds Table 2 Arterial blood gas after cardiac arrest pH 7.401 PaCO 2 (mmHg) 35.2 PaO 2 (mmHg) 86.7 HCO 3 − (mmol/L) 21.4 Na + (mmol/L) 136 K + (mmol/L) 4.2 Cl − (mmol/L) 111	3.919922	0.974121	sec[1]/p[0]	en	0.999995	38811941	https://doi.org/10.1186/s13256-024-04589-x	[ "blood", "mmhg", "cardiac", "arrest", "minute", "approximately", "anesthesia", "electrocardiogram", "general", "hours" ]	[ { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" }, { "code": "MA12.1", "title": "Finding of cocaine in blood" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "MA12.2", "title": "Finding of hallucinogen in blood" }, { "code": "BE2Y", "title": "Other specified diseases of the circulatory system" }, { "code": "BC4Z", "title": "Diseases of the myocardium or cardiac chambers, unspecified" }, { "code": "BD1Z", "title": "Heart failure, unspecified" }, { "code": "LA8Z", "title": "Structural developmental anomaly of heart or great vessels, unspecified" }, { "code": "BA41.Z", "title": "Acute myocardial infarction, unspecified" } ]	=== ICD-11 CODES FOUND === [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS [MA12.1] Finding of cocaine in blood Also known as: Finding of cocaine in blood \| cocaine in blood [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [MA12.2] Finding of hallucinogen in blood Also known as: Finding of hallucinogen in blood \| hallucinogen in blood [BE2Y] Other specified diseases of the circulatory system Also known as: Other specified diseases of the circulatory system \| Certain specified forms of heart disease \| Cardiac disorder, unspecified \| disorder of heart NOS \| organic disease or disorder of heart [BC4Z] Diseases of the myocardium or cardiac chambers, unspecified Also known as: Diseases of the myocardium or cardiac chambers, unspecified \| Heart disease NOS \| cardiac disease NOS [BD1Z] Heart failure, unspecified Also known as: Heart failure, unspecified \| myocardial failure \| cardiac decompensation \| cardiac failure \| cardiac failure NOS [LA8Z] Structural developmental anomaly of heart or great vessels, unspecified Also known as: Structural developmental anomaly of heart or great vessels, unspecified \| Heart malformations \| Cardiac malformations \| congenital anomaly of heart \| congenital heart disease [BA41.Z] Acute myocardial infarction, unspecified Also known as: Acute myocardial infarction, unspecified \| Acute myocardial infarction \| cardiac attack \| heart attack \| acute cardiac infarction === GRAPH WALKS === --- Walk 1 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --CHILD--> [?] Anaemias or other erythrocyte disorders --- Walk 2 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --EXCLUDES--> [?] Certain conditions originating in the perinatal period Def: This chapter includes conditions that have their origin in the perinatal period even though death or morbidity occurs later.... --- Walk 3 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --CHILD--> [MF50.40] Macroscopic haematuria --- Walk 4 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --CHILD--> [MF50.41] Microscopic haematuria --- Walk 5 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.0] Finding of opiate drug in blood --- Walk 6 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.2] Finding of hallucinogen in blood	[ "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --CHILD--> [?] Anaemias or other erythrocyte disorders", "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --EXCLUDES--> [?] Certain conditions originating in the perinatal period\n Def: This chapter includes conditions that have their origin in the perinatal period even though death or morbidity occurs later....", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --CHILD--> [MF50.40] Macroscopic haematuria", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --CHILD--> [MF50.41] Microscopic haematuria", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.0] Finding of opiate drug in blood", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.2] Finding of hallucinogen in blood" ]	3C0Z	Diseases of the blood or blood-forming organs, unspecified	[ { "from_icd11": "3C0Z", "icd10_code": "D75A", "icd10_title": "Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia" }, { "from_icd11": "3C0Z", "icd10_code": "D7581", "icd10_title": "Myelofibrosis" }, { "from_icd11": "3C0Z", "icd10_code": "D7582", "icd10_title": "Heparin induced thrombocytopenia (HIT)" }, { "from_icd11": "3C0Z", "icd10_code": "D7589", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D759", "icd10_title": "Disease of blood and blood-forming organs, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "D763", "icd10_title": "Other histiocytosis syndromes" }, { "from_icd11": "3C0Z", "icd10_code": "Q899", "icd10_title": "Congenital malformation, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "III", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D70-D77", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D75", "icd10_title": "Other and unspecified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D77", "icd10_title": "Other disorders of blood and blood-forming organs in diseases classified elsewhere" }, { "from_icd11": "3C0Z", "icd10_code": "D758", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D76", "icd10_title": "Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue" }, { "from_icd11": "MF50.4Z", "icd10_code": "R310", "icd10_title": "Gross hematuria" }, { "from_icd11": "MF50.4Z", "icd10_code": "R312", "icd10_title": "Other microscopic hematuria" } ]	D75A	Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia
A 60-year-old female patient was admitted to our hospital on October 28th, 2016 with the symptoms of abdominal pain, distension, dark urine, cough, expectoration, chills and fever. The highest temperature was 39 °C before her admission. She had been taking iguratimod (25 mg twice per day) because of Sjoren’s syndrome (SS) for about 15 days prior to her admission. The patient didn’t have hepatobiliary disease and history of excessive alcohol intake, recent travel, blood transfusion. According to the physical examination, her vital signs were normal. Despite sever jaundice, she was conscious. There was no bleeding points or spider angioma or liver palm on her skin. Her abdomen was flat and soft, with no tenderness or rebounding tenderness. Additionally, her liver and spleen were untouched, without shifting dullness. Besides, no edema was seen in her entire body. Her blood test results (Table 1 ) were as follows: complete blood count: WBC 3.54 × 10 ^9 /L, NE 61.00%, Hb 119 g/L, PLT 130 × 10 ^9 /L, PT 22.9 s, APTT 60.2 s, PTA 78%. Abnormal liver tests: TBIL 263.62 umol/L, DBIL 211.34 umol/L, IBIL 52.28 umol/L, ALT 747 U/L, AST 986 U/L, gamma-GPT 256 U/L, ALP 184 U/L, TBA 205.85 umol/L, LDH 346 U/L. The serum IgG was 13.68 g/L, and the level of IgG4 was 298 μg/ml. The patient was negative for IgM anti-HA, anti-HCV, anti-HEV, HBsAg, anti-EBV-VCA IgM. The serologic markers of hepatitis B virus HBsAb, HBcAb, HBeAb were positive, but the quantification of hepatitis B virus was normal. The results of ANAs were as follows: ANA , anti-SS-A (60) antibody (positive), anti-SS-A (52) antibody (positive), anti- La/SS-B antibody (positive). The results of autoimmune hepatitis markers (AMA), ANCA, ACL, anti-O antibody, rheumatoid factor, thyroid function and T-SPOT test were also normal. And phlegm etiology detection, blood culture, G and GM tests were normal. The serum C3 and C4 were normal. The CRP and PCT were moderately elevated which the highest level of CRP and PCT was 10.87 mg/L and 1.780 ng/ml, respectively. The result of ECG was normal. Abdominal ultrasound scan showed cholecystitis and ascites. The chest computed tomography (CT) found that bilateral pleural effusion and pneumonia at the lower right on November 31th, 2016. After 1 week, the review of chest CT revealed that bilateral pleural effusion was absorbed a little compared with the last result, and pneumonia was still existed. Combined with the history of related medication, symptoms, signs and the relative auxiliary examination, the patient was diagnosed as likely to be suffering from DILI, Sjogren’s syndrome, pneumonia, and hypoproteinemia. However, considering the patient with Sjogren’s syndrome and the higher level of ANA, antoimmune liver injury couldn’t be ruled out. According to the International Autoimmune Hepatitis Group’s (IAIHG) AIH diagnostic scoring system, the patient was rated 6 points and the AIH could be ruled out basically. To confirm the diagnosis, liver biopsy was proposed, but the patient refused due to economic reasons.Taking into account of the long-term use of hormones in patient, the routine blood and inflammation index couldn’t reflect the severity of infection, and the patient was given the treatment such as controlling infection, liver-protecting, albumin replenishing and other supporting treatments. However, the symptoms such as jaundice, abdominal distension increased and fever still appeared intermittently, while cough and expectoration have been improved obviously. And review of the relevant indicators prompted that serum bilirubin level increased, transaminase and albumin level decreased and blood coagulation declined further. The above indicators suggested that abnormal liver function has reached to a higher level. The patient was given the treatment like infusion plasma to improve the function of blood coagulation. At the same time, the anti-infection therapeutic regimen was strengthened. On the 7th day of admission, we conducted a peritoneal puncture and checked the relevant indicators, and finally, the results suggest that the diagnosis of spontaneous peritonitis was ruled out. In order to clarify the cause and guide the next treatments, a multidisciplinary discussion was organized. Then the treatment was adjusted as follows: methylprednisolone was taking to control Sjogren’s syndrome, and we could use hormone via statics drop when the fever occurred again. About one month after given up taking iguratimod, the patient’s symptoms improved significantly, and the relevant indicators such as abnormal liver tests, blood coagulation function returned to normal basically . According to the updated RUCAM diagnostic scoring system, the patient was rated 9 points and was a suspected DILI. After discharged, the patient was re-adjudicated a one-month follow-up by the same reviewers and the diagnosis was confirmed. Table 1 Mainly described some of the laboratory tests during the hospitalization of this patient WBC(10 ^ 9/L) 3.54 Albumin(g/L) 28.0 ANCA negative Neutrophils(%) 61.00 TBIL(umol/L) 263.62 ACL negative Lymphocytes(%) 29.70 DBIL(umol/L) 211.34 AMA-M negative Eosinophils(%) 0.60 IBIL(umol/L) 52.28 HBsAg negative Basophils(%) 0.80 AST(U/L) 747 HBsAb positive RBC(10 ^ 12/L) 3.93 ALT(U/L) 986 HBeAb positive Hemoglobin(g/L) 119 ALP(U/L) 184 HbcAb positive Platelets(*10 ^ 9/L) 130 r-GTP(U/L) 256 HBV-DNA(IU/ml) < 20 PT(S) 22.9 LDH(U/L) 346 anti-HA negative APTT(S) 60.2 TBA(umol/L) 205.85 anti-HCV negative PTA(%) 40.0 IgG4(ug/ml) 265 anti-HEV negative CRP(mg/L) 6.23 IgG(g/L) 13.68 anti-HSV negative PCT(ng/ml) 0.56 IgA(g/L) 2.41 anti-CMV IgM negative G test negative IgM(g/L) 2.62 anti-CMV IgG positive GM test negative complementC3(g/L) 0.26 anti-EBV VCA IgM negative Blood culture negative complement C4(g/L) 0.07 T-SPOT test negative ANA positive,1:3200 RF normal anti-SS-A(60) positive Anti-O negtive anti-SS-A(52) positive anti-La/SS-B positive Fig. 1 included five pictures, respectively ( a , b , c , d and e ), which mainly showed the trends of some laboratory test results of this patient. For example, a mainly showed the changes of TIBL and DIBL during the course of the disease; b represented the changes of ALT, AST in the course of disease; c mainly showed the changes of r-GT, ALP and TBA during the course of the disease; d represented the changes of ALB in the course of disease; And E mainly showed the changes of PT, APTT and PTA during the course of the disease	3.945313	0.979004	sec[1]/p[0]	en	0.999996	30143001	https://doi.org/10.1186/s12876-018-0858-z	[ "anti", "blood", "liver", "umol", "that", "mainly", "changes", "course", "taking", "hepatitis" ]	[ { "code": "JA86.Y", "title": "Maternal care for other specified fetal problems" }, { "code": "MB23.1", "title": "Antisocial behaviour" }, { "code": "3B4Z", "title": "Coagulation defects, unspecified" }, { "code": "4A45.Z", "title": "Antiphospholipid syndrome, unspecified" }, { "code": "4A43.Y", "title": "Other specified overlap non-organ specific systemic autoimmune disease" }, { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" }, { "code": "MA12.1", "title": "Finding of cocaine in blood" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "MA12.2", "title": "Finding of hallucinogen in blood" } ]	=== ICD-11 CODES FOUND === [JA86.Y] Maternal care for other specified fetal problems Also known as: Maternal care for other specified fetal problems \| Maternal care for other isoimmunization \| Isoimmunization NOS \| maternal antibodies NOS \| pregnancy management affected by incompatibility of blood groups NOS [MB23.1] Antisocial behaviour Definition: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated. Also known as: Antisocial behaviour \| Child or adolescent antisocial behaviour [3B4Z] Coagulation defects, unspecified Also known as: Coagulation defects, unspecified \| blood clotting disturbance \| blood clotting defect \| blood clotting factor deficiency \| clotting abnormality [4A45.Z] Antiphospholipid syndrome, unspecified Also known as: Antiphospholipid syndrome, unspecified \| Antiphospholipid syndrome \| Hughes syndrome \| Anticardiolipin syndrome [4A43.Y] Other specified overlap non-organ specific systemic autoimmune disease Also known as: Other specified overlap non-organ specific systemic autoimmune disease \| Antisynthetase syndrome \| Reynolds syndrome \| Syndromic multisystem autoimmune disease due to ITCH deficiency \| Eosinophilia myalgia syndrome [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS [MA12.1] Finding of cocaine in blood Also known as: Finding of cocaine in blood \| cocaine in blood [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [MA12.2] Finding of hallucinogen in blood Also known as: Finding of hallucinogen in blood \| hallucinogen in blood === GRAPH WALKS === --- Walk 1 --- [JA86.Y] Maternal care for other specified fetal problems --PARENT--> [JA86] Maternal care for other fetal problems Def: A condition characterised by the provision of health interventions to the mother due to any other issue that is either suspected or known to be present in the fetus.... --CHILD--> [JA86.2] Maternal care for signs of fetal hypoxia --- Walk 2 --- [JA86.Y] Maternal care for other specified fetal problems --PARENT--> [JA86] Maternal care for other fetal problems Def: A condition characterised by the provision of health interventions to the mother due to any other issue that is either suspected or known to be present in the fetus.... --CHILD--> [JA86.0] Maternal care for red cell antibodies Def: Maternal care for rhesus or other isoimmunization... --- Walk 3 --- [MB23.1] Antisocial behaviour Def: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated.... --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour --RELATED_TO--> [?] Speech dysfluency Def: Speech dysfluency is characterised by the frequent or pervasive disruption of the rhythmic flow of speech that arises subsequent to the developmental period (i.e., adult onset) and is outside the limi... --- Walk 4 --- [MB23.1] Antisocial behaviour Def: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated.... --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour --PARENT--> [?] Mental or behavioural symptoms, signs or clinical findings --- Walk 5 --- [3B4Z] Coagulation defects, unspecified --PARENT--> [?] Coagulation defects --CHILD--> [?] Congenital or constitutional haemorrhagic condition Def: A condition caused by determinants arising during the antenatal period or genetically inherited factors, leading to defects in clotting mechanisms or abnormalities causing structural flaws in the bloo... --- Walk 6 --- [3B4Z] Coagulation defects, unspecified --PARENT--> [?] Coagulation defects --CHILD--> [?] Haemorrhagic diseases due to acquired coagulation factor defects Def: Any disease caused by determinants arising after birth. These diseases are characterised by abnormal coagulation of the blood....	[ "[JA86.Y] Maternal care for other specified fetal problems\n --PARENT--> [JA86] Maternal care for other fetal problems\n Def: A condition characterised by the provision of health interventions to the mother due to any other issue that is either suspected or known to be present in the fetus....\n --CHILD--> [JA86.2] Maternal care for signs of fetal hypoxia", "[JA86.Y] Maternal care for other specified fetal problems\n --PARENT--> [JA86] Maternal care for other fetal problems\n Def: A condition characterised by the provision of health interventions to the mother due to any other issue that is either suspected or known to be present in the fetus....\n --CHILD--> [JA86.0] Maternal care for red cell antibodies\n Def: Maternal care for rhesus or other isoimmunization...", "[MB23.1] Antisocial behaviour\n Def: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated....\n --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour\n --RELATED_TO--> [?] Speech dysfluency\n Def: Speech dysfluency is characterised by the frequent or pervasive disruption of the rhythmic flow of speech that arises subsequent to the developmental period (i.e., adult onset) and is outside the limi...", "[MB23.1] Antisocial behaviour\n Def: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated....\n --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour\n --PARENT--> [?] Mental or behavioural symptoms, signs or clinical findings", "[3B4Z] Coagulation defects, unspecified\n --PARENT--> [?] Coagulation defects\n --CHILD--> [?] Congenital or constitutional haemorrhagic condition\n Def: A condition caused by determinants arising during the antenatal period or genetically inherited factors, leading to defects in clotting mechanisms or abnormalities causing structural flaws in the bloo...", "[3B4Z] Coagulation defects, unspecified\n --PARENT--> [?] Coagulation defects\n --CHILD--> [?] Haemorrhagic diseases due to acquired coagulation factor defects\n Def: Any disease caused by determinants arising after birth. These diseases are characterised by abnormal coagulation of the blood...." ]	JA86.Y	Maternal care for other specified fetal problems	[ { "from_icd11": "JA86.Y", "icd10_code": "O26841 ", "icd10_title": "" }, { "from_icd11": "JA86.Y", "icd10_code": "O26843 ", "icd10_title": "" }, { "from_icd11": "JA86.Y", "icd10_code": "O26849 ", "icd10_title": "" }, { "from_icd11": "JA86.Y", "icd10_code": "O3680X0 ", "icd10_title": "" }, { "from_icd11": "3B4Z", "icd10_code": "D688", "icd10_title": "Other specified coagulation defects" }, { "from_icd11": "3B4Z", "icd10_code": "D689", "icd10_title": "Coagulation defect, unspecified" }, { "from_icd11": "3B4Z", "icd10_code": "D699", "icd10_title": "Hemorrhagic condition, unspecified" }, { "from_icd11": "3B4Z", "icd10_code": "D698", "icd10_title": "Other specified hemorrhagic conditions" }, { "from_icd11": "3B4Z", "icd10_code": "D65-D69", "icd10_title": "" }, { "from_icd11": "3B4Z", "icd10_code": "D69", "icd10_title": "Purpura and other hemorrhagic conditions" }, { "from_icd11": "4A45.Z", "icd10_code": "D6861", "icd10_title": "Antiphospholipid syndrome" }, { "from_icd11": "4A45.Z", "icd10_code": "D6869", "icd10_title": "Other thrombophilia" }, { "from_icd11": "4A45.Z", "icd10_code": "D6862", "icd10_title": "Lupus anticoagulant syndrome" }, { "from_icd11": "4A45.Z", "icd10_code": "D686", "icd10_title": "Other thrombophilia" }, { "from_icd11": "3C0Z", "icd10_code": "D75A", "icd10_title": "Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia" } ]	O26841
A 3-day-old 60 kg Swiss Warmblood colt was presented to the university veterinary hospital approximately two hours after the onset of clinical symptoms observed by the owner in the morning. The foal was recumbent, tachycardic (160 beats/minute), tachypneic (60 breaths/minute), with abdominal distention, pale-pink mucous membranes and a capillary refill time of 2.5 s. Uroperitoneum due to a ruptured bladder was diagnosed with abdominal ultrasonography and peritoneal fluid aspirate analysis (yellow, cloudy aspirate; creatinine 1evel: 1,264 µmol/l). Surgical bladder repair after medical stabilization was planned. Complete blood count analyzed at initial presentation revealed leukocytosis with left shift, lymphopenia and monocytosis. Complete serum biochemistry revealed hyponatremia, hyperkalemia, hypochloremia, normocalcemia, (Na+: 107 mmol/l, K+: 4.9 mmol/l; Cl- 71 mmol/l, Ca2+: 3.24 mmol/l), hyperglycemia (11.71 mmol/l), azotemia (BUN: 10.01 mmol/l, creatinine: 327 µmol/l) and increased activities of liver enzymes. Packed cell volume and total solids were measured at 46% and 71.2 g/l; respectively. Fluid therapy with 3 litres of 0.9% NaCl given as bolus followed by 5% glucose solution in saline solution at 5 ml/kg/hr and an intramuscular injection of insulin 0.1 IU/kg (Novo Rapid, Novo Nordisk, Switzerland) were initiated. Abdominal distension increased due to inability to drain the uroabdomen as the omentum repeatedly occluded the abdominal drain. Plasma electrolytes, glucose and lactate were controlled after four hours (Na+: 113 mmol/l, K+: 5.9 mmol/l; Cl- 77 mmol/l, glucose: 9.4 mmol/l, lactate: 2.1 mmol/l) and it was decided to perform the surgery despite the electrolyte imbalances. The foal was premedicated with butorphanol 0.05 mg/kg IV (Morphasol, Dr. E. Graeub, Switzerland) and diazepam 0.1 mg/kg IV (Valium, Roche, Switzerland) and general anaesthesia was induced with ketamine 1 mg/kg IV (Narketan, Vetoquinol, Switzerland) and propofol 1.5 mg/kg IV (Propofol, Fresenius Kabi, Germany). The trachea was intubated with a 12 mm cuffed silicone endotracheal tube which was connected to a circle system anaesthesia machine (Fabius, Dräger Medical, Germany). Anaesthesia monitoring (multiparameter analyzer, Datex Ohmeda S5, GE Healthcare, USA) consisted of a 3-lead base apex ECG lead II derivation, hemoglobin saturation by pulse oximetry, end-tidal carbon dioxide (ETCO 2 ) and inspired fraction of oxygen (FiO 2 ) with gas analyzer and invasive and non-invasive blood pressure. Blood pressure was monitored with an inflatable cuff placed at the base of the front limb and with an invasive arterial 22-gauge catheter placed in the facial artery. Fluid therapy administered during anaesthesia was based of saline solution 0.9% (10 ml/kg/hr) supplemented with glucose 5% (glucose: 0.5 g/kg/hr). Anaesthesia was maintained with isoflurane (Attane Isoflurane, Provet, Switzerland) in oxygen with a fresh gas flow set a 2 L/min. The foal was breathing spontaneously and the first recorded pulse oximetry measurement indicated an oxygen saturation of 78% (FiO 2 0.87). Thus, five minutes after connection to the anaesthesia machine, controlled mandatory ventilation with high peak inspiratory pressure (PIP: 40 cmH 2 O) and positive end-expiratory pressure (PEEP: 5 cmH 2 O) was initiated. An arterial blood gas analysis was performed revealing following values: pH: 7.27; PaO 2 : 3.2 kPa, 61.9 mmHg; PaCO 2 : 6 kPa, 45.2 mmHg; HCO3-: 19.7 mmol/l; TCO2: 21.1 mmol/l; BE: −5.8; Lactate: 2.6 mmol/l; Na+: 113 mmol/l; K+: 4.9 mmol/l; Cl-: 81: mmol/l. One puff of nebulized salbutamol (100 mcg; Ventolin, GlaxoSmithKline AG, Switzerland) was delivered via endotracheal tube. Because of mild hypotension (invasive mean arterial pressure of 60 mmHg) dobutamine (Dobutrex, Teva Pharma, Switzerland) at 0.6 mcg/kg/min has been initiated. A normal sinus rhythm of 90 beats/minute was recorded before surgical incision. A midline abdominal incision was performed and approximately 10–12 liters of intraabdominal fluids were released by free flow over approximately thirty seconds. Cardiac dysrhythmia with long periods of absent atrioventricular conduction developed. P-P and P-R intervals did appear to be slightly irregular but each QRS complex was always preceded by a P-wave. QRS complexes did not appear wide and bizarre. Progressive decrease of the ETCO 2 (from 6 kPa; 45 mmHg to 2.9 kPa; 22 mmHg) values were observed. Periods of advanced atrioventricular blocks were characterized by the absence of pulse oximeter and invasive blood pressure waves. After development of AV blocks, dobutamine administration was stopped and atropine 0.02 mg/kg IV (Atropinsulfat, Amino, Switzerland) was given. Because no improvements in the rhythm was observed, four additional doses of atropine were injected (0.01–0.02 mg/kg IV), but none was successful. Thereafter, a prolonged period of ventricular asystole occurred (approximately 30 s), intravenous epinephrine 0.01 mg/kg was administered, the isoflurane vaporizer was turned off, the foal placed in lateral recumbency and chest compressions (approximately 120/minute) were initiated. A sinus tachycardia at 130 beats/minute was observed. Normocapnia was restored within 30 s of cardiopulmonary resuscitation (CPR). Electrolytes concentrations (Na+: 116 mmol/l, K+: 6.3 mmol/l; Cl- 89 mmol/l) and arterial blood gas analysis (PaO2: 29.6 kPa, 222 mmHg; PaCO2: 7.8 kPa, 58.7 mmHg) were measured shortly after CPR. No more cardiac dysrhythmias were observed, surgery was completed within 100 min and total anaesthesia time was 160 min. Efforts to keep normothermia during anaesthesia included: use of a medical forced-air warming device, abdominal flushing with fluids at body temperature and administration of warm IV fluid therapy during anaesthesia. The lowest temperature was measured at the end of anaesthesia (37.4°C). The foal exhibited signs of excitement (paddling, shaking) during recovery which resolved after 10 min of manual restraint. The day after surgery, blood work was repeated, showing the following values (Na +128 mmol/l, K + 3.5 mmol/l, Cl- 109 mmol/l, iCa ++ 1.54 mmol/l, glucose 9.0 mmol/l, lactate 1.9 mmol/l). The postoperative period was uneventful and the foal was discharged three days later. Eighteen months later the owner reported that the foal had been healthy since discharge. Written signed owner consent had been obtained for publication of the present case.	4.039063	0.961426	sec[0]/p[0]	en	0.999997	29951488	https://doi.org/10.3389/fvets.2018.00096	[ "mmol", "anaesthesia", "switzerland", "foal", "blood", "mmhg", "abdominal", "glucose", "pressure", "approximately" ]	[ { "code": "GB42.1", "title": "Albuminuria, Grade A3" }, { "code": "GB42.0", "title": "Albuminuria, Grade A2" }, { "code": "MA18.0Y", "title": "Other specified elevated blood glucose level" }, { "code": "MB40.3", "title": "Anaesthesia of skin" }, { "code": "9A78.6", "title": "Anaesthesia of cornea" }, { "code": "MG30.5Z", "title": "Chronic neuropathic pain, unspecified" }, { "code": "JA67.Z", "title": "Complications of anaesthesia during pregnancy, unspecified" }, { "code": "JB43.Z", "title": "Complications of anaesthesia during the puerperium, unspecified" }, { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" } ]	=== ICD-11 CODES FOUND === [GB42.1] Albuminuria, Grade A3 Definition: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted to the urine creatinine concentration to correct for variations in overall urine concentration. When persistent and of moderate or greater severity usually indicates overt glomerular disease such as caused by diabetic glomerulosclerosis, glomerulonephritis or amyloid. Also known as: Albuminuria, Grade A3 \| albuminuria >30 mg/mmol creatinine \| macroalbuminuria \| overt albuminuria \| overt nephropathy [GB42.0] Albuminuria, Grade A2 Definition: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted to the urine creatinine concentration to correct for variations in overall urine concentration. When persistent and of moderate or greater severity usually indicates overt glomerular disease such as caused by diabetic glomerulosclerosis, glomerulonephritis or amyloid. Also known as: Albuminuria, Grade A2 \| microalbuminuria \| incipient nephropathy \| mild to moderate albuminuria \| albuminuria 3-30 mg/mmol creatinine [MA18.0Y] Other specified elevated blood glucose level Also known as: Other specified elevated blood glucose level \| Blood glucose between 8.0 - 11.9 mmol/L pre-meal or fasting \| Blood glucose between 12.0 - 13.9 mmol/L pre-meal or fasting \| Blood glucose greater than or equal to 14.0 mmol/L pre-meal or fasting \| Blood glucose between 12.0 - 13.9 mmol/L post-meal or not otherwise specified [MB40.3] Anaesthesia of skin Definition: Partial or complete loss of sensation affecting the skin, most commonly affecting a circumscribed area and resulting from sensory nerve damage as from injury or leprosy. Also known as: Anaesthesia of skin \| Hypoaesthesia of skin \| Loss of cutaneous sensation \| Numbness of skin \| Loss of sensation Includes: Numbness of skin [9A78.6] Anaesthesia of cornea Definition: This is the condition of having sensation (including the feeling of pain) blocked or temporarily taken away, of the transparent front part of the eye that covers the iris, pupil, and anterior chamber. Also known as: Anaesthesia of cornea \| corneal anaesthesia [MG30.5Z] Chronic neuropathic pain, unspecified Also known as: Chronic neuropathic pain, unspecified \| Chronic neuropathic pain \| anaesthesia dolorosa \| neuralgia \| chronic neurogenic pain (deprecated) [JA67.Z] Complications of anaesthesia during pregnancy, unspecified Also known as: Complications of anaesthesia during pregnancy, unspecified \| Complications of anaesthesia during pregnancy [JB43.Z] Complications of anaesthesia during the puerperium, unspecified Also known as: Complications of anaesthesia during the puerperium, unspecified \| Complications of anaesthesia during the puerperium [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS === GRAPH WALKS === --- Walk 1 --- [GB42.1] Albuminuria, Grade A3 Def: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted... --PARENT--> [GB42] Persistent proteinuria or albuminuria Def: Persistent albuminuria >3mg/mmol creatinine or >30mg/day is regarded as abnormal, indicative and often the first manifestation of chronic kidney disease (CKD). In surveillance for CKD in “at risk” pat... --PARENT--> [?] Glomerular diseases Def: Any disease characterised by pathological changes to the glomerulus.... --- Walk 2 --- [GB42.1] Albuminuria, Grade A3 Def: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted... --PARENT--> [GB42] Persistent proteinuria or albuminuria Def: Persistent albuminuria >3mg/mmol creatinine or >30mg/day is regarded as abnormal, indicative and often the first manifestation of chronic kidney disease (CKD). In surveillance for CKD in “at risk” pat... --EXCLUDES--> [?] Proteinuria Def: Excessive serum proteins in the urine, such as in renal disease when albumin is the main protein, but also may be due to other proteins such as immunoglobulin light chains in plasma cell dyscrasia suc... --- Walk 3 --- [GB42.0] Albuminuria, Grade A2 Def: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted... --PARENT--> [GB42] Persistent proteinuria or albuminuria Def: Persistent albuminuria >3mg/mmol creatinine or >30mg/day is regarded as abnormal, indicative and often the first manifestation of chronic kidney disease (CKD). In surveillance for CKD in “at risk” pat... --CHILD--> [GB42.Y] Other specified persistent proteinuria or albuminuria --- Walk 4 --- [GB42.0] Albuminuria, Grade A2 Def: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted... --PARENT--> [GB42] Persistent proteinuria or albuminuria Def: Persistent albuminuria >3mg/mmol creatinine or >30mg/day is regarded as abnormal, indicative and often the first manifestation of chronic kidney disease (CKD). In surveillance for CKD in “at risk” pat... --CHILD--> [GB42.1] Albuminuria, Grade A3 Def: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted... --- Walk 5 --- [MA18.0Y] Other specified elevated blood glucose level --PARENT--> [MA18.0] Elevated blood glucose level --CHILD--> [MA18.0Y] Other specified elevated blood glucose level --- Walk 6 --- [MA18.0Y] Other specified elevated blood glucose level --PARENT--> [MA18.0] Elevated blood glucose level --RELATED_TO--> [?] Neonatal hyperglycaemia	[ "[GB42.1] Albuminuria, Grade A3\n Def: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted...\n --PARENT--> [GB42] Persistent proteinuria or albuminuria\n Def: Persistent albuminuria >3mg/mmol creatinine or >30mg/day is regarded as abnormal, indicative and often the first manifestation of chronic kidney disease (CKD). In surveillance for CKD in “at risk” pat...\n --PARENT--> [?] Glomerular diseases\n Def: Any disease characterised by pathological changes to the glomerulus....", "[GB42.1] Albuminuria, Grade A3\n Def: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted...\n --PARENT--> [GB42] Persistent proteinuria or albuminuria\n Def: Persistent albuminuria >3mg/mmol creatinine or >30mg/day is regarded as abnormal, indicative and often the first manifestation of chronic kidney disease (CKD). In surveillance for CKD in “at risk” pat...\n --EXCLUDES--> [?] Proteinuria\n Def: Excessive serum proteins in the urine, such as in renal disease when albumin is the main protein, but also may be due to other proteins such as immunoglobulin light chains in plasma cell dyscrasia suc...", "[GB42.0] Albuminuria, Grade A2\n Def: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted...\n --PARENT--> [GB42] Persistent proteinuria or albuminuria\n Def: Persistent albuminuria >3mg/mmol creatinine or >30mg/day is regarded as abnormal, indicative and often the first manifestation of chronic kidney disease (CKD). In surveillance for CKD in “at risk” pat...\n --CHILD--> [GB42.Y] Other specified persistent proteinuria or albuminuria", "[GB42.0] Albuminuria, Grade A2\n Def: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted...\n --PARENT--> [GB42] Persistent proteinuria or albuminuria\n Def: Persistent albuminuria >3mg/mmol creatinine or >30mg/day is regarded as abnormal, indicative and often the first manifestation of chronic kidney disease (CKD). In surveillance for CKD in “at risk” pat...\n --CHILD--> [GB42.1] Albuminuria, Grade A3\n Def: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted...", "[MA18.0Y] Other specified elevated blood glucose level\n --PARENT--> [MA18.0] Elevated blood glucose level\n --CHILD--> [MA18.0Y] Other specified elevated blood glucose level", "[MA18.0Y] Other specified elevated blood glucose level\n --PARENT--> [MA18.0] Elevated blood glucose level\n --RELATED_TO--> [?] Neonatal hyperglycaemia" ]	GB42.1	Albuminuria, Grade A3	[ { "from_icd11": "MB40.3", "icd10_code": "R200", "icd10_title": "Anesthesia of skin" }, { "from_icd11": "MB40.3", "icd10_code": "R201", "icd10_title": "Hypoesthesia of skin" }, { "from_icd11": "JA67.Z", "icd10_code": "O298X1", "icd10_title": "Other complications of anesthesia during pregnancy, first trimester" }, { "from_icd11": "JA67.Z", "icd10_code": "O29", "icd10_title": "Complications of anesthesia during pregnancy" }, { "from_icd11": "JA67.Z", "icd10_code": "O295", "icd10_title": "Other complications of spinal and epidural anesthesia during pregnancy" }, { "from_icd11": "JA67.Z", "icd10_code": "O298", "icd10_title": "Other complications of anesthesia during pregnancy" }, { "from_icd11": "JA67.Z", "icd10_code": "O299", "icd10_title": "Unspecified complication of anesthesia during pregnancy" }, { "from_icd11": "JB43.Z", "icd10_code": "O898", "icd10_title": "Other complications of anesthesia during the puerperium" }, { "from_icd11": "JB43.Z", "icd10_code": "O89", "icd10_title": "Complications of anesthesia during the puerperium" }, { "from_icd11": "JB43.Z", "icd10_code": "O893", "icd10_title": "Toxic reaction to local anesthesia during the puerperium" }, { "from_icd11": "JB43.Z", "icd10_code": "O899", "icd10_title": "Complication of anesthesia during the puerperium, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "D75A", "icd10_title": "Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia" }, { "from_icd11": "3C0Z", "icd10_code": "D7581", "icd10_title": "Myelofibrosis" }, { "from_icd11": "3C0Z", "icd10_code": "D7582", "icd10_title": "Heparin induced thrombocytopenia (HIT)" }, { "from_icd11": "3C0Z", "icd10_code": "D7589", "icd10_title": "Other specified diseases of blood and blood-forming organs" } ]	R200	Anesthesia of skin
A written consent for this case report has been obtained by the patient. The patient is a 52-year-old male Caucasian, residing in the south of Germany. There was no specific family history of malignancy. As far as risk factors are concerned the patient has a smoking history adding up to 45 pack years and still smokes about 10 to 15 cigarettes per day. There was no alcohol consumption reported. He was referred by his dentist to our Department in February 2013 with a suspected OSCC of the floor of the mouth. The conducted head and neck computed tomography (CT) strengthened the clinical suspicion of an OSCC of the right floor of the mouth; a tumor that was also infiltrating the right mandible was demonstrated, along with enlarged lymph nodes at both sides of the neck [Figure 1 ]. As a part of the staging and planning procedures a laryngoscopy and a pharyngoscopy were performed, as well as biopsies and marking of the boundaries of the tumor with a safety distance. Upon histopathological confirmation of the suspected OSCC diagnosis, the case was discussed during a meeting with our Multidisciplinary Team (MDT), when a primary surgical treatment was recommended. In March 2013 a tumor resection combined with a right mandibulectomy, a neck dissection at level I-V at the right side and level I-III at the left side and a simultaneous reconstruction with a vascular fibular transplant were performed. The histopathological examination of the resected specimen showed a moderately differentiated squamous cell carcinoma with perineural infiltration and one nodal metastasis (pT3 pN1 (1/46) L0 V2 Pn1 G3, local R0) [Figure 2 a]. The histology of a macroscopically visible nodule in the neck dissection specimen showed a hemangiosis carcinomatosa (V2) [Figure 2 b]. During his stay a heparin-induced thrombocytopenia (HIT) was diagnosed. After a consultation with our Department of Transfusion Medicine the thromboprophylaxis regime was switched from Enoxaparin 20 mg s.c. 1-0-0 to Danaparoid 750 IE s.c. 1-0-1. The postoperative MDT recommended radiation therapy. A locoregional radiation was performed from April 2013 to June 2013 (intensity modulated radiation therapy (IMRT) with a daily fraction of 2,1/1,8 GyHD 65,1/55,8 Gy). After radiation the patient joined our six-weekly tumor-follow-up at our department. Seven months later the decision for the removal of the osteosynthesis plate (Stryker GmbH & Co.KG, Duisburg, Germany, 2.7 mm) of the lower jaw was made and a panoramic radiograph as well as a CT were performed [Figure 3 ]. It showed no signs for a relapse of the OSCC but pseudarthrosis at two sides of the reconstructed mandible. The pseudarthrosis was treated with a bone graft from the iliac crest. During the surgical procedure several soft tissue biopsies from the right floor of the mouth were taken. No relapse was found within the obtained material. Five months later the decision for the removal of the miniplates (Stryker GmbH & Co.KG, Duisburg, Germany, 2.0 mm), used for the fixation of the iliac crest bone graft, was made. In March 2014 the patient showed up for surgery [Figure 4 ]. During the clinical examination the patient reported that he has detected a growing mass at his left upper arm about four weeks ago. Clinically the tumor was miming an abscess with redness, heat, swelling and moderate pain. The upper arm was x-rayed and a swelling of the soft-tissue in projection to the lower margin of the deltoid muscle was found [Figure 5 ]. Sonographically a differentiation between abscess and soft-tissue tumor could not be done [Figure 6 ]. Therefore MR-Imaging was performed. The MRI described an inhomogeneous tumor at the proximal upper arm with circular extent around the humerus up to the axilla [Figure 7 ]. Local arrosions of the corticalis suggested malignancy; however, a differentiation between a soft-tissue tumor and a metastatic tumor spread was not possible. Therefore a biopsy was taken and the histological examination revealed a widely necrotic moderately differentiated keratinizing squamous cell carcinoma (G2) [Figure 8 ]. The case was discussed in an Interdisciplinary MDT. Due to the tumor localization - wrapping the humerus and in close proximity to the brachial plexus and the vascular bundle - the decision to manage it with radiation was made. The patient decided to start radiation therapy at his local hospital. When he showed up for therapy a picture was taken. Noticeable was the rapid progress of the metastasis after the biopsy was taken with a clear infiltration of the skin. Experimental and clinical studies have shown rapid local growth after traumatic interventions, possibly explaining the progress [Figure 9 ] . Figure 1 The computed tomography illustrates a tumor on the right floor of the mouth (arrows). Radiographically there is the suspicion of an erosion of the cortical bone of the lower jaw. Figure 2 Microscopic findings of the primary tumor: moderately differentiated squamous cell carcinoma with perineural infiltration (a). The microscopic findings of a macroscopically visible nodule in the neck dissection specimen (Level Ib, right side) showed a hemangiosis carcinomatosa (V2) (b) . Figure 3 The panoramic radiograph, taken right before the osteosynthesis plates removal, shows the reconstructed lower jaw with a double-barreled fibula. Figure 4 Showing the patient 12 months after resection of the OSCC of the floor of the mouth (a & b). Figure 5 X-ray of the upper arm shows a swelling of the soft-tissue in projection to the lower margin of the deltoid muscle (arrow). Figure 6 Sonography of the upper arm shows the tumor (a & b; mark). A discrimination between abscess and soft-tissue tumor cannot be done. Figure 7 MR-Imaging of the upper arm in axial (a) and coronal (b) orientation. Both images (fat saturated T1 weighted Images after contrast administration) show a contrast enhanced soft tissue mass in the proximal upper arm with a circular extent around the proximal humeral bone. Extensive soft tissue involvement suggested malignancy but a definitive diagnosis was not possible. On both images a large necrotic central part without contrast enhancement is visible. Figure 8 Microscopic findings of the punch biopsy of the left arm: infiltration by a moderately differentiated squamous cell carcinoma. Figure 9 Upper arm metastasis right before radiation therapy. Remarkable is the rapid progress of the metastasis after the biopsy was taken.	3.855469	0.981934	sec[1]/p[0]	en	0.999996	25884373	https://doi.org/10.1186/s12903-015-0007-9	[ "tumor", "soft", "tissue", "radiation", "oscc", "floor", "mouth", "neck", "moderately", "differentiated" ]	[ { "code": "2F9Z", "title": "Neoplasms of unknown behaviour of unspecified site" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "2E6Z", "title": "Carcinoma in situ of unspecified site" }, { "code": "2F91.1", "title": "Neoplasms of unknown behaviour of trachea, bronchus or lung" }, { "code": "2F92", "title": "Neoplasms of unknown behaviour of skin" }, { "code": "FB6Z", "title": "Soft tissue disorders, unspecified" }, { "code": "FB56.6", "title": "Other specified soft tissue disorders" }, { "code": "GB61.Z", "title": "Chronic kidney disease, stage unspecified" }, { "code": "FB56.1", "title": "Residual foreign body in soft tissue" }, { "code": "FB52", "title": "Soft tissue disorders in diseases classified elsewhere" } ]	=== ICD-11 CODES FOUND === [2F9Z] Neoplasms of unknown behaviour of unspecified site Also known as: Neoplasms of unknown behaviour of unspecified site \| neoplasia \| neoplasm growth NOS \| tumour of unspecified site \| tumourlet of unspecified site [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [2E6Z] Carcinoma in situ of unspecified site Also known as: Carcinoma in situ of unspecified site \| carcinoma in situ of unspecified site \| carcinoma in situ NOS \| carcinoma in situ \| in situ neoplasm [2F91.1] Neoplasms of unknown behaviour of trachea, bronchus or lung Also known as: Neoplasms of unknown behaviour of trachea, bronchus or lung \| trachea, bronchus or lung tumour NOS \| Intravascular bronchial alveolar tumour of unspecified site \| Bronchial adenoma of unknown behaviour of unspecified site \| Intravascular bronchial alveolar tumour unknown behaviour of unspecified site [2F92] Neoplasms of unknown behaviour of skin Also known as: Neoplasms of unknown behaviour of skin \| skin tumour NOS [FB6Z] Soft tissue disorders, unspecified Also known as: Soft tissue disorders, unspecified \| disease of soft tissue NOS \| unspecified soft tissue disorder, site unspecified \| disorder of soft tissue \| disorder of soft tissue NOS [FB56.6] Other specified soft tissue disorders Also known as: Other specified soft tissue disorders \| Fat necrosis \| fatty necrosis \| Profichet's disease \| Sloughing of fascia [GB61.Z] Chronic kidney disease, stage unspecified Also known as: Chronic kidney disease, stage unspecified \| Chronic kidney disease \| chronic renal failure \| chronic kidney failure \| chronic renal disease [FB56.1] Residual foreign body in soft tissue Also known as: Residual foreign body in soft tissue \| residual foreign body in soft tissue, site unspecified \| foreign body in soft tissue \| Residual foreign body in soft tissue, multiple sites \| Residual foreign body in soft tissue, shoulder region Excludes: Foreign body granuloma of skin \| Foreign body granuloma of soft tissue, not elsewhere classified [FB52] Soft tissue disorders in diseases classified elsewhere Definition: This is a group of disorders affecting tissues that connect, support, or surround other structures and organs of the body, not being bone and occur in diseases classified elsewhere. Also known as: Soft tissue disorders in diseases classified elsewhere === GRAPH WALKS === --- Walk 1 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2F92] Neoplasms of unknown behaviour of skin --- Walk 2 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2F91] Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs --- Walk 3 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Enlarged lymph nodes Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes.... --EXCLUDES--> [?] Chronic lymphadenitis --- Walk 4 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Localised adiposity Def: A condition characterised by accumulation of adipose tissue in specific regions of the body.... --CHILD--> [?] Fatty apron --- Walk 5 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs --- Walk 6 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs	[ "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2F92] Neoplasms of unknown behaviour of skin", "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2F91] Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Enlarged lymph nodes\n Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes....\n --EXCLUDES--> [?] Chronic lymphadenitis", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Localised adiposity\n Def: A condition characterised by accumulation of adipose tissue in specific regions of the body....\n --CHILD--> [?] Fatty apron", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs" ]	2F9Z	Neoplasms of unknown behaviour of unspecified site	[ { "from_icd11": "2F9Z", "icd10_code": "D487", "icd10_title": "Neoplasm of uncertain behavior of other specified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D482", "icd10_title": "Neoplasm of uncertain behavior of peripheral nerves and autonomic nervous system" }, { "from_icd11": "2F9Z", "icd10_code": "D37-D48", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D377", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D48", "icd10_title": "Neoplasm of uncertain behavior of other and unspecified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D489", "icd10_title": "Neoplasm of uncertain behavior, unspecified" }, { "from_icd11": "ME61", "icd10_code": "R2240", "icd10_title": "Localized swelling, mass and lump, unspecified lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2232", "icd10_title": "Localized swelling, mass and lump, left upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2242", "icd10_title": "Localized swelling, mass and lump, left lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2231", "icd10_title": "Localized swelling, mass and lump, right upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2241", "icd10_title": "Localized swelling, mass and lump, right lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2233", "icd10_title": "Localized swelling, mass and lump, upper limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2243", "icd10_title": "Localized swelling, mass and lump, lower limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2230", "icd10_title": "Localized swelling, mass and lump, unspecified upper limb" }, { "from_icd11": "ME61", "icd10_code": "R220", "icd10_title": "Localized swelling, mass and lump, head" } ]	D487	Neoplasm of uncertain behavior of other specified sites
Case 1 . A 79 years-old man presented with obstructive lower urinary tract symptoms at another hospital. His prostate-specific antigen (PSA) level was 15.54 ng/mL. Pathological diagnosis from transrectal needle biopsy was adenocarcinoma with a Gleason Score of 5 + 5. Staging computed tomography (CT) scan showed regional lymph node (LN) metastases. He received combined androgen blockade (CAB) therapy initially, but after a decrease in PSA, his levels eventually increased. He was diagnosed with castration-resistant prostate cancer (CRPC) and began enzalutamide but despite continuous treatment for 6 months, chemotherapy was required. However, although chemotherapy initially lowered his PSA, eventually it increased a lot subsequently. He had difficulty urinating smoothly because of disease progression-related obstruction requiring clean intermittent catheterization. Subsequently, he received abiraterone but it was ineffective. Three years after the original diagnosis, he was referred to our hospital for further treatment of CRPC. He had bloody urine and difficulty with self-catheterization for 5 months after starting. Cystoscopy showed several nodular polyps in the penile urethra . Magnetic resonance imaging (MRI) demonstrated that the tumor had grown to 8 cm in diameter and invaded the rectum . MRI also showed metastases of the prostate cancer extended with skip lesions along the corpus spongiosum in the entire anterior urethra . Moreover, he developed a catheter obstruction caused by hematuria, so a suprapubic cystostomy tube was placed and transurethral resection of the prostate (TURP) was performed to achieve tumor bleeding coagulation. Simultaneously, he underwent endoscopic resection of the urethra polyps. Histology showed metastasis of prostatic adenocarcinoma with aggressive variant ; particularly, as illustrated in Fig. 1 e and f with immunolabeling for hematoxylin and eosin staining, the pathological findings of Case 1 exhibited a high-grade tumor defined by characteristic nuclear features, including lack of prominent nucleoli and high nuclear to cytoplasmic ratio. He was treated using cisplatin and etoposide. After two cycles, he achieved a progressive disease and he was treated with the best supportive care. Fig. 1 Cystoscopic, imaging, and pathological examination results and genomic sequencing in Case 1. a , b Cystoscopic findings in the urethra. The cystoscope shows several nodular polyps in the proximal penile urethra and distal bulbar urethra. c Prostate magnetic resonance imaging (MRI). The prostate was almost entirely replaced by the tumor, which has invaded the rectum. d MR image of the urethra. Metastases of the prostate cancer extended with skip lesions along the corpus spongiosum in the entire anterior urethra. e – h Representative microscopic images of hematoxylin and eosin (HE) staining and prostate-specific antigen and androgen receptor immunohistochemical staining of transurethral resections of urethra tumor specimens. These images were obtained using the following equipment: microscope, BX53; objective lens, UPLXAPO; camera, DP27; adapter, U-TV1XC. NanoZoomer-XR C12000 was used as acquisition software and the measured resolution was 500 dpi. i Examined genes (horizontal axis) and the copy number in Case 1 (vertical axis) Targeted next-generation sequencing using an in-house assay of the resected specimen from the urethra polyps was performed (Additional file 1 ). A TP53 somatic point mutation (p.H193Y) was detected as a pathogenic variant. Gene amplification was detected in androgen receptor ( AR ) and KRAS (estimated copy number (CN): 35.3, 5.8, respectively). Loss of genetic heterozygosity (LOH) without mutation was observed in BRCA2 . CN variation box indicated a high LOH frequency, which is common in homologous recombination-deficient tumors. Case 2 . A 69 years old man was diagnosed with Gleason score 4 + 5 prostate cancer at another hospital. His serum PSA level was 81.5 ng/mL, and his clinical stage from CT and whole-body bone scans was T3aN1M1 (multiple lung and bone metastases). CAB therapy was started, and his PSA decreased to 0.36 ng/mL. However, after 1 year on androgen deprivation therapy (ADT), resistance to castration developed (PSA: 54.22 ng/mL), so docetaxel was started. For ten cycles of chemotherapy, his PSA decreased to a nadir of 17.2 ng/mL but subsequently increased to 84.0 ng/mL. MRI and CT of the abdomen and pelvis showed non-muscle-invasive masses in the neck and urinary bladder posterior wall, although chest CT of the lung metastases showed partial responses (PRs). Cystoscopy showed bladder-neck obstruction by a tumor invading from the prostate gland and revealed another group of nodular masses at the left posterior bladder wall . He was referred to our hospital for TURP and transurethral resection of the bladder tumor because of hematuria and urinary obstructive symptoms. Both pathological diagnoses of the bladder neck and posterior wall showed neuroendocrine differentiation prostate cancer (NEPC) . Specifically, high mitotic rate cells were detected using immunolabeling for H&E staining, as illustrated in Fig. 2 e, and the signals of synaptophysin were found in over 50% tumor cells . From the abovementioned points, we identified Case 2 as treatment-induced NEPC (tNEPC). He underwent two cycles of etoposide and carboplatin. However, the disease progressed, and the anticancer treatment was eventually discontinued. Fig. 2 Cystoscopic, imaging, and pathological examination results and genomic sequencing in Case 2. a Bladder magnetic resonance imaging. b Pelvic computed tomography. Non-muscle-invasive masses in the neck and posterior wall of the urinary bladder are shown. c , d Cystoscopic findings. The tumor invaded from the prostate gland and has obstructed the bladder neck ( c ) and nodular masses are shown at the left posterior bladder wall ( d ). e – h Representative microscopic images of hematoxylin and eosin (HE) staining and prostate-specific antigen, androgen receptor, and synaptophysin immunohistochemical staining and transurethral resection of the bladder tumor specimens. These images were obtained using the following equipment: microscope, BX53; objective lens, UPLXAPO; camera, DP27; adapter, U-TV1XC. NanoZoomer-XR C12000 was used as acquisition software and the measured resolution was 500 dpi. i Examined genes (horizontal axis) and the copy number in Case 2 (vertical axis)	4.058594	0.976563	sec[0]/sec[0]/p[0]	en	0.999996	35598018	https://doi.org/10.1186/s12920-022-01267-z	[ "prostate", "urethra", "tumor", "bladder", "staining", "pathological", "metastases", "androgen", "cancer", "imaging" ]	[ { "code": "GA90", "title": "Hyperplasia of prostate" }, { "code": "GA91.Z", "title": "Inflammatory or other diseases of prostate, unspecified" }, { "code": "GA91.Y", "title": "Other specified inflammatory or other diseases of prostate" }, { "code": "GA91.0", "title": "Chronic prostatitis" }, { "code": "MF40.1", "title": "Problems of the prostate" }, { "code": "GC0Y", "title": "Other diseases of urinary system" }, { "code": "GC02.Z", "title": "Urethritis and urethral syndrome, unspecified" }, { "code": "GC02.Y", "title": "Other specified urethritis and urethral syndrome" }, { "code": "GC03", "title": "Urethral stricture" }, { "code": "LB31.C", "title": "Congenital absence of bladder or urethra" } ]	=== ICD-11 CODES FOUND === [GA90] Hyperplasia of prostate Definition: A condition of the prostate, caused by an increased rate of cellular division of the glandular and stromal cells. This condition is characterised by enlargement of the prostatic tissue, dysuria, urinary urgency, nocturia, weak urine stream, straining while urinating, incomplete bladder emptying during urination, or increased frequency of urinary tract infection. Also known as: Hyperplasia of prostate \| Adenofibromatous hypertrophy of prostate \| benign prostatic hyperplasia \| prostate hyperplasia \| prostatic area hypertrophy Includes: Adenofibromatous hypertrophy of prostate Excludes: Benign neoplasms of prostate [GA91.Z] Inflammatory or other diseases of prostate, unspecified Also known as: Inflammatory or other diseases of prostate, unspecified \| Inflammatory or other diseases of prostate \| inflammation of prostate NOS \| prostatitis NOS \| disease of prostate NOS [GA91.Y] Other specified inflammatory or other diseases of prostate Also known as: Other specified inflammatory or other diseases of prostate \| Acute bacterial prostatitis \| acute prostatitis \| Prostatitis category I (NIH classification) \| Prostatitis category I [GA91.0] Chronic prostatitis Definition: A condition caused by obstruction of the prostate glands. This condition is characterised by inflammation of the prostate gland, dysuria, pollakiuria, urinary urgency, genital pain, lower back pain, abdominal pain, and repeated bladder infections that last for at least three months. Also known as: Chronic prostatitis \| Fibrous prostatitis \| Hypertrophic prostatitis \| Subacute prostatitis \| Chronic bacterial prostatitis [MF40.1] Problems of the prostate Definition: A group of disorders associated with the prostate occurring in diseases more specifically classified elsewhere. Also known as: Problems of the prostate [GC0Y] Other diseases of urinary system Also known as: Other diseases of urinary system \| Acquired urethral deformity, not elsewhere classified \| acquired urethral deformity NOS \| Urethral bleeding \| bleeding from urethra [GC02.Z] Urethritis and urethral syndrome, unspecified Also known as: Urethritis and urethral syndrome, unspecified \| Urethritis and urethral syndrome \| urethral syndrome NOS \| urethra syndrome [GC02.Y] Other specified urethritis and urethral syndrome Also known as: Other specified urethritis and urethral syndrome \| Other urethritis \| inflammation of urethra \| paraurethritis \| periurethritis [GC03] Urethral stricture Definition: Stenosis of the urethra accompanied by fibrosis and scarring of the spongiosal body Also known as: Urethral stricture \| ankylurethria \| urethral stenosis \| urethral contracture \| urethral obstruction [LB31.C] Congenital absence of bladder or urethra Definition: Any condition caused by failure of both the bladder and the urethra to develop during the antenatal period. This condition may result in fetal death, or sepsis and sever complications in cases of live births. Also known as: Congenital absence of bladder or urethra \| Congenital absence of bladder \| acystia \| agenesis of bladder \| Congenital absence of urethra === GRAPH WALKS === --- Walk 1 --- [GA90] Hyperplasia of prostate Def: A condition of the prostate, caused by an increased rate of cellular division of the glandular and stromal cells. This condition is characterised by enlargement of the prostatic tissue, dysuria, urina... --PARENT--> [?] Diseases of prostate --CHILD--> [GA90] Hyperplasia of prostate Def: A condition of the prostate, caused by an increased rate of cellular division of the glandular and stromal cells. This condition is characterised by enlargement of the prostatic tissue, dysuria, urina... --- Walk 2 --- [GA90] Hyperplasia of prostate Def: A condition of the prostate, caused by an increased rate of cellular division of the glandular and stromal cells. This condition is characterised by enlargement of the prostatic tissue, dysuria, urina... --EXCLUDES--> [?] Benign neoplasm of male genital organs Def: A non-metastasizing neoplasm that arises from the male reproductive system. Representative examples include benign prostate phyllodes tumour, benign Sertoli cell tumour, seminal vesicle cystadenoma, a... --PARENT--> [?] Benign neoplasms except of mesenchymal origin --- Walk 3 --- [GA91.Z] Inflammatory or other diseases of prostate, unspecified --PARENT--> [GA91] Inflammatory or other diseases of prostate Def: Any disease caused by obstruction of the prostate gland. These diseases are characterised by a build-up of secretions and inflammation of the prostate.... --CHILD--> [GA91.1] Abscess of prostate Def: A condition caused by infection with the gram-negative bacteria Neisseria gonorrhoeae and Escherichia coli, or the gram-positive bacteria Staphylococcus aureus or Mycobacterium tuberculosis. This cond... --- Walk 4 --- [GA91.Z] Inflammatory or other diseases of prostate, unspecified --PARENT--> [GA91] Inflammatory or other diseases of prostate Def: Any disease caused by obstruction of the prostate gland. These diseases are characterised by a build-up of secretions and inflammation of the prostate.... --RELATED_TO--> [?] Gonococcal prostatitis --- Walk 5 --- [GA91.Y] Other specified inflammatory or other diseases of prostate --PARENT--> [GA91] Inflammatory or other diseases of prostate Def: Any disease caused by obstruction of the prostate gland. These diseases are characterised by a build-up of secretions and inflammation of the prostate.... --RELATED_TO--> [?] Gonococcal prostatitis --- Walk 6 --- [GA91.Y] Other specified inflammatory or other diseases of prostate --PARENT--> [GA91] Inflammatory or other diseases of prostate Def: Any disease caused by obstruction of the prostate gland. These diseases are characterised by a build-up of secretions and inflammation of the prostate.... --CHILD--> [GA91.0] Chronic prostatitis Def: A condition caused by obstruction of the prostate glands. This condition is characterised by inflammation of the prostate gland, dysuria, pollakiuria, urinary urgency, genital pain, lower back pain, a...	[ "[GA90] Hyperplasia of prostate\n Def: A condition of the prostate, caused by an increased rate of cellular division of the glandular and stromal cells. This condition is characterised by enlargement of the prostatic tissue, dysuria, urina...\n --PARENT--> [?] Diseases of prostate\n --CHILD--> [GA90] Hyperplasia of prostate\n Def: A condition of the prostate, caused by an increased rate of cellular division of the glandular and stromal cells. This condition is characterised by enlargement of the prostatic tissue, dysuria, urina...", "[GA90] Hyperplasia of prostate\n Def: A condition of the prostate, caused by an increased rate of cellular division of the glandular and stromal cells. This condition is characterised by enlargement of the prostatic tissue, dysuria, urina...\n --EXCLUDES--> [?] Benign neoplasm of male genital organs\n Def: A non-metastasizing neoplasm that arises from the male reproductive system. Representative examples include benign prostate phyllodes tumour, benign Sertoli cell tumour, seminal vesicle cystadenoma, a...\n --PARENT--> [?] Benign neoplasms except of mesenchymal origin", "[GA91.Z] Inflammatory or other diseases of prostate, unspecified\n --PARENT--> [GA91] Inflammatory or other diseases of prostate\n Def: Any disease caused by obstruction of the prostate gland. These diseases are characterised by a build-up of secretions and inflammation of the prostate....\n --CHILD--> [GA91.1] Abscess of prostate\n Def: A condition caused by infection with the gram-negative bacteria Neisseria gonorrhoeae and Escherichia coli, or the gram-positive bacteria Staphylococcus aureus or Mycobacterium tuberculosis. This cond...", "[GA91.Z] Inflammatory or other diseases of prostate, unspecified\n --PARENT--> [GA91] Inflammatory or other diseases of prostate\n Def: Any disease caused by obstruction of the prostate gland. These diseases are characterised by a build-up of secretions and inflammation of the prostate....\n --RELATED_TO--> [?] Gonococcal prostatitis", "[GA91.Y] Other specified inflammatory or other diseases of prostate\n --PARENT--> [GA91] Inflammatory or other diseases of prostate\n Def: Any disease caused by obstruction of the prostate gland. These diseases are characterised by a build-up of secretions and inflammation of the prostate....\n --RELATED_TO--> [?] Gonococcal prostatitis", "[GA91.Y] Other specified inflammatory or other diseases of prostate\n --PARENT--> [GA91] Inflammatory or other diseases of prostate\n Def: Any disease caused by obstruction of the prostate gland. These diseases are characterised by a build-up of secretions and inflammation of the prostate....\n --CHILD--> [GA91.0] Chronic prostatitis\n Def: A condition caused by obstruction of the prostate glands. This condition is characterised by inflammation of the prostate gland, dysuria, pollakiuria, urinary urgency, genital pain, lower back pain, a..." ]	GA90	Hyperplasia of prostate	[ { "from_icd11": "GA90", "icd10_code": "N402", "icd10_title": "Nodular prostate without lower urinary tract symptoms" }, { "from_icd11": "GA90", "icd10_code": "N403", "icd10_title": "Nodular prostate with lower urinary tract symptoms" }, { "from_icd11": "GA90", "icd10_code": "N400", "icd10_title": "Benign prostatic hyperplasia without lower urinary tract symptoms" }, { "from_icd11": "GA90", "icd10_code": "N401", "icd10_title": "Benign prostatic hyperplasia with lower urinary tract symptoms" }, { "from_icd11": "GA90", "icd10_code": "N40", "icd10_title": "Benign prostatic hyperplasia" }, { "from_icd11": "GA91.Z", "icd10_code": "N414", "icd10_title": "Granulomatous prostatitis" }, { "from_icd11": "GA91.Z", "icd10_code": "N4289", "icd10_title": "Other specified disorders of prostate" }, { "from_icd11": "GA91.Z", "icd10_code": "N4283", "icd10_title": "Cyst of prostate" }, { "from_icd11": "GA91.Z", "icd10_code": "N410", "icd10_title": "Acute prostatitis" }, { "from_icd11": "GA91.Z", "icd10_code": "N419", "icd10_title": "Inflammatory disease of prostate, unspecified" }, { "from_icd11": "GA91.Z", "icd10_code": "N429", "icd10_title": "Disorder of prostate, unspecified" }, { "from_icd11": "GA91.Z", "icd10_code": "N418", "icd10_title": "Other inflammatory diseases of prostate" }, { "from_icd11": "GA91.Z", "icd10_code": "N41", "icd10_title": "Inflammatory diseases of prostate" }, { "from_icd11": "GA91.Z", "icd10_code": "N42", "icd10_title": "Other and unspecified disorders of prostate" }, { "from_icd11": "GA91.Z", "icd10_code": "N428", "icd10_title": "Other specified disorders of prostate" } ]	N402	Nodular prostate without lower urinary tract symptoms
At the first examination, she was very talkative and presented severely disorganized thoughts and behavior, as well as inappropriately expressed emotions. She was readmitted to the hospital for treatment of psychotic episodes. After discharge, she was continuously treated as an outpatient; however, symptoms including avolition, asociality, moderately disorganized thinking, and emotional vulnerability to psychological stress in daily life persisted. She married at 28 years of age in 1984, and gave birth to a boy the following year. She was maintained in a generally stable mental state with the support of a community health nurse and her family. Long-acting injectable (LAI) fluphenazine enanthate was introduced in 1993 to overcome poor adherence when she was 37 years of age. At approximately the same time, she was treated for diabetes. In 1994, at 38 years of age, an overnight stay with her sister-in-law resulted in rehospitalization for a worsening of disorganized thought, anxiety, and impatience. After being admitted for a few weeks, she regularly visited our hospital as an outpatient. In 2004, she transiently presented with dizziness from standing up too quickly at around 48 years of age. In 2011, she began complaining of urinary incontinence, nocturnal polyuria, and dysarthria at approximately 55 years of age; however, it was unlikely that such symptoms were side effects of her antipsychotics or diabetic autonomic neuropathy. When she was 56 years of age, she gradually developed pain in the right knee and was diagnosed with osteoarthritis of the right knee. In the following year, she underwent artificial joint replacement surgery for her right knee in the Department of Orthopaedic Surgery, Tohoku Rosai Hospital. Bilateral hand muscle weakness, predominantly in the right hand, and dysarthria were observed while she was hospitalized in 2013. Although she underwent brain magnetic resonance imaging (MRI), findings revealed no remarkable abnormalities . She used a pick-up walker at home after the knee surgery; however, at the end of the same year, she fell at home and was diagnosed with a right femoral neck fracture. She was treated operatively for the fracture in the same orthopedic surgery department as before. After 4 months as an inpatient, she was discharged to her home, and resumed outpatient treatment at the MPC in 2014. She presented with depressed mood, decreased appetite, and body weight loss after discharge, and was prescribed sertraline for symptoms of depression. In 2014, at 58 years of age, she underwent video fluoroscopic examination of her swallowing, demonstrating increased dysphagia, bradykinesia, and upper-limb muscle rigidity, predominantly in the right limb. Drug-induced parkinsonism was suspected based on the results of the examination, which indicated no organic abnormality. LAI fluphenazine enanthate was discontinued, and she was switched from oral haloperidol to aripiprazole. She was readmitted to our hospital at 58 years of age upon appearance of emotional instability, hallucinations, and negativism following the drug switch. Moreover, she also presented with worsening of urinary incontinence and retention, which required urethral catheterization. The volume of post-catheterization urinary output was greater than 600 mL. Dosage adjustment of the antipsychotics, including tests with aripiprazole, zotepine, blonanserin, and perospirone, failed to improve the parkinsonism, mutism, and negativism (e.g., food and drug rejection); consequently, modified electroconvulsive therapy (mECT) was administered for 20 treatments. mECT produced slight improvements in the symptoms, and it became possible for her to ingest a small amount of food and speak a little . However, she returned to her symptomatic state after a short period of time. She then underwent careful examination for persistent parkinsonism and autonomic dysfunction (including urinary retention, urinary incontinence, and constipation) in the Department of Neurology, Kohnan Hospital . Brain MRI revealed a T2-hyperintense lateral rim and hypointense putamen that had progressed over the year . Cardiac 123 I-meta-iodobenzylguanidine (MIBG) scintigraphy demonstrated normal myocardial MIBG uptake . In 123 I-FP-CIT (DaTscan) single-photon emission computed tomography (SPECT) imaging, decreased uptake of 123 I-FP-CIT was observed in the bilateral striatum, but predominantly in the left dorsolateral striatum . MSA-P was thus suspected from the clinical features (including parkinsonism, urinary dysfunction, and constipation), neuroimaging, and nuclear medicine findings. L-dopa was prescribed and the dose was increased to 700 mg/day; however, her response was poor, and she died of aspiration pneumonia in 2016 at 60 years of age. Fig. 1 Brain magnetic resonance images of the patient at 57 years of age . T2-weighted images (T2WI) demonstrated no remarkable abnormalities, including fresh cerebral infarction, bleeding, space-occupying lesions, and prominent atrophic changes in brain regions including the bilateral putamen. Moreover, there was no high-intensity signal of the lateral rim of the putamen Fig. 2 Brain magnetic resonance images of the patient at 58 years of age ( a ) and 59 years of age ( b ). a Putaminal atrophic change and low- and high-intensity signals of the lateral rim of the putamen were observed predominantly in the left putamen in the T2-weighted image (T2WI). However, T2WI did not show the hot-cross bun sign, which may reflect pontine atrophy. b As in the previous year, magnetic resonance imaging findings revealed putaminal atrophic change and both low- and high-intensity signals of the lateral rim of the bilateral putamen, again predominantly in the left putamen. There were slight atrophic changes in the cerebellum and brain stem Fig. 3 123 I-FP-CIT (DaTscan) SPECT image results in 2015. DaTscan data demonstrate reduced bilateral striatal ioflupane ( 123 I) uptake predominantly in the dorsolateral striatum. There were asymmetrical changes, with a greater decrease in 123 I uptake in the left dorsolateral striatum Fig. 4 Cardiac 123 I-meta-iodobenzylguanidine (MIBG) scintigraphy results in 2015. The values of the heart to mediastinum (H/M) ratio in the early and delayed phases were 3.26 and 4.02 (normal range is > 2.20), respectively. The washout rate was − 12.9% (normal range is < 22%). 123 I-MIBG scintigraphy showed normal cardiac MIBG uptake	3.759766	0.980957	sec[1]/p[1]	en	0.999996	29783976	https://doi.org/10.1186/s12888-018-1714-y	[ "putamen", "however", "including", "urinary", "predominantly", "brain", "mibg", "uptake", "knee", "magnetic" ]	[ { "code": "7A24", "title": "Hypersomnia due to a medication or substance" }, { "code": "9D90", "title": "Vision impairment including blindness" }, { "code": "NE61", "title": "Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified" }, { "code": "3A50.02", "title": "Haemoglobin H disease (– α/– – included)" }, { "code": "LA8B.Z", "title": "Congenital anomaly of great arteries including arterial duct, unspecified" }, { "code": "GB6Z", "title": "Kidney failure, unspecified" }, { "code": "GC04.Z", "title": "Fistula of the genitourinary tract, unspecified" }, { "code": "MF51", "title": "Anuria or oliguria" }, { "code": "GB70.Z", "title": "Calculus of upper urinary tract, unspecified" }, { "code": "GC2Z", "title": "Diseases of the urinary system, unspecified" } ]	=== ICD-11 CODES FOUND === [7A24] Hypersomnia due to a medication or substance Definition: Hypersomnia due to a medication or substance is characterised by excessive nocturnal sleep, daytime sleepiness, or excessive napping that is attributable to the sedating effects of medications, alcohol, or other psychoactive substances, including withdrawal syndromes (e.g., from stimulants) and is sufficiently severe to constitute an independent focus of clinical attention. Note: A definitive diagnosis requires use of polysomnography and multiple sleep latency test (MSLT) to rule out other hype Also known as: Hypersomnia due to a medication or substance \| Hypersomnia due to substances including medications \| Hypersomnolence due to substances including medications Includes: Hypersomnia due to substances including medications [9D90] Vision impairment including blindness Definition: !markdown The table below gives a classification of severity of vision impairment based on visual acuity. For epidemiological studies, it is recommended to collect the following information on visual acuity for each eye, for both eyes open and for distance and near. a) Uncorrected visual acuity b) Presenting visual acuity c) Best corrected visual acuity Blindness is also categorized according to the degree of constriction of the central visual field in the better eye to less than 10 Also known as: Vision impairment including blindness [NE61] Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified Also known as: Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified \| Harmful effects of or exposure to noxious substances chiefly nonmedicinal as to source, alcohols \| alcohol poisoning \| alcohol toxicity \| Harmful effects of or exposure to noxious substances chiefly nonmedicinal as to source, Ethanol Excludes: corrosions \| Bacterial foodborne intoxications [3A50.02] Haemoglobin H disease (– α/– – included) Definition: Haemoglobin H (HbH) disease is a moderate to severe form of alpha-thalassemia characterised by pronounced microcytic hypochromic haemolytic anaemia. Also known as: Haemoglobin H disease (– α/– – included) \| alpha - /- - or mutational forms of alpha-thalassaemia \| Alpha thalassaemia intermedia [LA8B.Z] Congenital anomaly of great arteries including arterial duct, unspecified Also known as: Congenital anomaly of great arteries including arterial duct, unspecified \| Congenital anomaly of great arteries including arterial duct [GB6Z] Kidney failure, unspecified Also known as: Kidney failure, unspecified \| nontraumatic kidney injury \| renal failure NOS \| kidney block \| renal impairment NOS [GC04.Z] Fistula of the genitourinary tract, unspecified Also known as: Fistula of the genitourinary tract, unspecified \| Fistula of the genitourinary tract \| persistent urinary fistula \| persistent urinary tract fistula \| recurrent urinary fistula [MF51] Anuria or oliguria Definition: Anuria means nonpassage of urine, in practice is defined as passage of less than 50 millilitres of urine in a day. Oliguria is the low output of urine. It is clinically classified as an output below 300-500ml/day. Also known as: Anuria or oliguria \| Anuria \| suppression of urinary secretion \| ischuria \| Oliguria Excludes: Maternal care for other conditions predominantly related to pregnancy [GB70.Z] Calculus of upper urinary tract, unspecified Also known as: Calculus of upper urinary tract, unspecified \| Calculus of upper urinary tract \| calculus of urinary tract NOS \| urinary calculi \| urinary calculus, unspecified [GC2Z] Diseases of the urinary system, unspecified Also known as: Diseases of the urinary system, unspecified \| urinary tract disease NOS \| Abnormal renal function \| kidney dysfunction NOS \| kidney hypofunction === GRAPH WALKS === --- Walk 1 --- [7A24] Hypersomnia due to a medication or substance Def: Hypersomnia due to a medication or substance is characterised by excessive nocturnal sleep, daytime sleepiness, or excessive napping that is attributable to the sedating effects of medications, alcoho... --PARENT--> [?] Hypersomnolence disorders Def: Hypersomnolence disorders are characterised by a complaint of daytime sleepiness that is not due to another sleep-wake disorder (e.g. disturbed nocturnal sleep, misaligned circadian rhythm, or breathi... --CHILD--> [7A20] Narcolepsy Def: Narcolepsy is a disorder characterised by daily periods of irrepressible need to sleep or daytime lapses into sleep occurring for at least several months, accompanied by abnormal manifestations of REM... --- Walk 2 --- [7A24] Hypersomnia due to a medication or substance Def: Hypersomnia due to a medication or substance is characterised by excessive nocturnal sleep, daytime sleepiness, or excessive napping that is attributable to the sedating effects of medications, alcoho... --PARENT--> [?] Hypersomnolence disorders Def: Hypersomnolence disorders are characterised by a complaint of daytime sleepiness that is not due to another sleep-wake disorder (e.g. disturbed nocturnal sleep, misaligned circadian rhythm, or breathi... --CHILD--> [7A22] Kleine-Levin syndrome Def: Kleine-Levin syndrome is characterised by recurrent episodes of severe sleepiness in association with cognitive, psychiatric, and behavioural disturbances. A typical episode lasts a median of 10 days ... --- Walk 3 --- [9D90] Vision impairment including blindness Def: !markdown The table below gives a classification of severity of vision impairment based on visual acuity. For epidemiological studies, it is recommended to collect the following information on visual... --RELATED_TO--> [?] No vision impairment --PARENT--> [?] Vision impairment including blindness Def: !markdown The table below gives a classification of severity of vision impairment based on visual acuity. For epidemiological studies, it is recommended to collect the following information on visual... --- Walk 4 --- [9D90] Vision impairment including blindness Def: !markdown The table below gives a classification of severity of vision impairment based on visual acuity. For epidemiological studies, it is recommended to collect the following information on visual... --RELATED_TO--> [?] No vision impairment --PARENT--> [?] Examination of eyes or vision --- Walk 5 --- [NE61] Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified --EXCLUDES--> [?] Bacterial foodborne intoxications Def: Any condition caused by an intoxication due to a bacterial toxin. Intoxication is by ingestion of contaminated food.... --CHILD--> [?] Botulism Def: A disease caused by an infection with the gram-positive bacteria Clostridium botulinum. This disease commonly presents with abdominal pain, vomiting, acute paralysis, blurred vision, diplopia, and may... --- Walk 6 --- [NE61] Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified --EXCLUDES--> [?] Bacterial foodborne intoxications Def: Any condition caused by an intoxication due to a bacterial toxin. Intoxication is by ingestion of contaminated food.... --CHILD--> [?] Foodborne Clostridium perfringens intoxication	[ "[7A24] Hypersomnia due to a medication or substance\n Def: Hypersomnia due to a medication or substance is characterised by excessive nocturnal sleep, daytime sleepiness, or excessive napping that is attributable to the sedating effects of medications, alcoho...\n --PARENT--> [?] Hypersomnolence disorders\n Def: Hypersomnolence disorders are characterised by a complaint of daytime sleepiness that is not due to another sleep-wake disorder (e.g. disturbed nocturnal sleep, misaligned circadian rhythm, or breathi...\n --CHILD--> [7A20] Narcolepsy\n Def: Narcolepsy is a disorder characterised by daily periods of irrepressible need to sleep or daytime lapses into sleep occurring for at least several months, accompanied by abnormal manifestations of REM...", "[7A24] Hypersomnia due to a medication or substance\n Def: Hypersomnia due to a medication or substance is characterised by excessive nocturnal sleep, daytime sleepiness, or excessive napping that is attributable to the sedating effects of medications, alcoho...\n --PARENT--> [?] Hypersomnolence disorders\n Def: Hypersomnolence disorders are characterised by a complaint of daytime sleepiness that is not due to another sleep-wake disorder (e.g. disturbed nocturnal sleep, misaligned circadian rhythm, or breathi...\n --CHILD--> [7A22] Kleine-Levin syndrome\n Def: Kleine-Levin syndrome is characterised by recurrent episodes of severe sleepiness in association with cognitive, psychiatric, and behavioural disturbances. A typical episode lasts a median of 10 days ...", "[9D90] Vision impairment including blindness\n Def: !markdown\nThe table below gives a classification of severity of vision impairment based on visual acuity. \nFor epidemiological studies, it is recommended to collect the following information on visual...\n --RELATED_TO--> [?] No vision impairment\n --PARENT--> [?] Vision impairment including blindness\n Def: !markdown\nThe table below gives a classification of severity of vision impairment based on visual acuity. \nFor epidemiological studies, it is recommended to collect the following information on visual...", "[9D90] Vision impairment including blindness\n Def: !markdown\nThe table below gives a classification of severity of vision impairment based on visual acuity. \nFor epidemiological studies, it is recommended to collect the following information on visual...\n --RELATED_TO--> [?] No vision impairment\n --PARENT--> [?] Examination of eyes or vision", "[NE61] Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified\n --EXCLUDES--> [?] Bacterial foodborne intoxications\n Def: Any condition caused by an intoxication due to a bacterial toxin. Intoxication is by ingestion of contaminated food....\n --CHILD--> [?] Botulism\n Def: A disease caused by an infection with the gram-positive bacteria Clostridium botulinum. This disease commonly presents with abdominal pain, vomiting, acute paralysis, blurred vision, diplopia, and may...", "[NE61] Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified\n --EXCLUDES--> [?] Bacterial foodborne intoxications\n Def: Any condition caused by an intoxication due to a bacterial toxin. Intoxication is by ingestion of contaminated food....\n --CHILD--> [?] Foodborne Clostridium perfringens intoxication" ]	7A24	Hypersomnia due to a medication or substance	[ { "from_icd11": "7A24", "icd10_code": "G4719", "icd10_title": "Other hypersomnia" }, { "from_icd11": "7A24", "icd10_code": "G4710", "icd10_title": "Hypersomnia, unspecified" }, { "from_icd11": "7A24", "icd10_code": "G4712", "icd10_title": "Idiopathic hypersomnia without long sleep time" }, { "from_icd11": "7A24", "icd10_code": "G4714", "icd10_title": "Hypersomnia due to medical condition" }, { "from_icd11": "7A24", "icd10_code": "G4713", "icd10_title": "Recurrent hypersomnia" }, { "from_icd11": "7A24", "icd10_code": "G471", "icd10_title": "Hypersomnia" }, { "from_icd11": "9D90", "icd10_code": "H5461", "icd10_title": "Unqualified visual loss, right eye, normal vision left eye" }, { "from_icd11": "9D90", "icd10_code": "H5440", "icd10_title": "Blindness, one eye, unspecified eye" }, { "from_icd11": "9D90", "icd10_code": "H5442", "icd10_title": "Blindness, left eye, normal vision right eye" }, { "from_icd11": "9D90", "icd10_code": "H5441", "icd10_title": "Blindness, right eye, normal vision left eye" }, { "from_icd11": "9D90", "icd10_code": "H5462", "icd10_title": "Unqualified visual loss, left eye, normal vision right eye" }, { "from_icd11": "9D90", "icd10_code": "H5442A3", "icd10_title": "Blindness left eye category 3, normal vision right eye" }, { "from_icd11": "9D90", "icd10_code": "H54413A", "icd10_title": "Blindness right eye category 3, normal vision left eye" }, { "from_icd11": "9D90", "icd10_code": "H5442A5", "icd10_title": "Blindness left eye category 5, normal vision right eye" }, { "from_icd11": "9D90", "icd10_code": "H5460", "icd10_title": "Unqualified visual loss, one eye, unspecified" } ]	G4719	Other hypersomnia
A 64-year-old Japanese woman was referred to us for the treatment of acute renal failure and elevated of free light chain κ . Her past medical history was unremarkable except for treatment of hypertension. One week before her referral, she was admitted to the local hospital for malaise and anorexia. Laboratory examination revealed anemia, (hemoglobin 9.2 g/dL) increased serum creatinine (5.6 mg/dL; normal, 0.6–1.2 mg/dL) and hypogammaglobulinemia ). She had 2+ urine protein by dipstick and urine protein was 1800 mg/day with 72.8% of albumin. Hematuria was negative. Hepatitis A, B, and C viruses and HIV were negative. The tentative diagnosis was acute renal failure due to glomerulonephritis of unknown cause. She was treated with 1 mg/kg of prednisone, but her symptoms had worsened. On admission to our hospital, mild ascites, pleural effusion, and enlarged both kidneys (13 × 7 cm) were noted by whole-body computed tomography (CT). Whole body diffusion weighted magnetic resonance imaging detected a focal plasmacytoma in the left jaw. Laboratory findings (Table 1 ) showed marked elevation of serum creatinine (6.31 mg/dL), free light chain κ (763.0 mg/L), and N-terminal pro-brain natriuretic peptide levels. Liver function test were normal, including aspartate aminotransferase (AST) alanine aminotransferase (ALT), and alkaline phosphatase were normal. Urinalysis showed proteinuria of 1800 mg/day with 73% of albumin. Serum protein electrophoresis (SPEP) showed a a small notch in the γ-lesion. Serum and urine immunofixation (IFx) revealed a faint monoclonal IgD κ band and a clear κ -light chain band, respectively. Serological studies for anti-nuclear antibody was negative and complement levels (C3 and C4) were within normal limits. Bone marrow biopsy showed 19% of plasma cell infiltration by CD138 immunostaining. Multicolor flow cytometry showed that 5% of monoclonal plasma cells those were restricted to κ light chain and were positive for CD56 and negative for CD19. Cytogenetic abnormalities including del17p, t (11;14), t (4;14), t (14;16), and del13p, and 1q gain were negative by fluorescence in situ hybridization (FISH) using the CD138 purified bone marrow cells. A kidney biopsy revealed the nodular glomerulosclerosis and casts by light microscopy . Congo red staining was negative. An IF study demonstrated the positive staining of Igκ and negative staining of Igλ light chain along the glomerular and tubular basement membranes. Immunohistochemistry showed linear staining of glomerular basement membrane positive for Igκ and negative staining for Igλ. Ribbon-like deposits by periodic acid Schiff (PAS) staining. Tubular casts positive for Igκ and negative for PAS and Igλ were also seen . Electron microscopy showed the powdery electron-dense deposits along the tubular and glomerular basement membrane consistent with the diagnosis of LCDD involving the kidney. To determine the glomerular deposits, laser microdissection (LMD) of the of the glomerulus of paraffin-embedded kidney biopsy material followed by liquid chromatography and mass spectrometry (MS) was performed. By LMD/MS, we identified Igκ light chain with more than 95% probability but not IgD heavy chain and Igλ light chain . Taken together, these observations support the diagnosis of κ-LCDD of the kidney. Although the patient did not show any evidence of portal hypertension or liver cirrhosis, but had unexplained ascites. There was a possibility that ascites could be caused by liver involvement of LCDD. Therefore, we performed a liver biopsy was performed using the CT-guided transjugular catheter technique. Liver biopsy specimens revealed the atrophy of hepatocytes and expansion of sinusoid . IF revealed the positive staining for anti-κ and negative staining for anti-λ along with the perisinusoidal membrane and vessels consisting with the involvement of κ-LCDD in the liver. Other tissue involvement included skin and salivary gland were negative. The patient was diagnosed symptomatic IgD MM and LCDD involving kidney and liver. Table 1 Laboratory data on admission and on discharge the hospital Laboratory data On admission On discharge Reference ranges Hemoglobin(g/dL) 9.5 9.2 11.0–15.3 White blood cell (/μL) 11,700 5200 3500–9800 Platelet (× 10 3 /μL) 305 214 130–370 Urine protein (dipstick) 2+ 2+ Negative Urine Sugar (dipstick) Negative Negative Negative Serum creatinine (mg/dL) 6.31 3.19 0.6–1.2 eGFR (mL/min/1.73m 2 ) 5.79 12.22 > 60 Urea Nitrogen (mg/dL) 80 22.0 8.0–22.0 Uric acid (mg/dL) 10 5.0 2.6–6.0 AST (U/L) 13 8 13–33 ALT (U/L) 21 7 8–42 LDH (U/L) 330 300 124–222 Total protein 6.0 5.1 6.7–8.3 Albumin 3.8 3.8 3.4–5.8 Serum IgG (mg/dL) 282 612 680–1620 Serum IgA (mg/dL) 46 34 84–438 Serum IgM (mg/dL) 5 15 57–288 Free light chain κ (mg/L) 763 20.3 2.42–18.92 Free light chain λ (mg/L) 5.8 13.3 4.44–26.18 Free light chain κ/λ 131.55 1.526 0.248–1.804 β2-microglobulin (mg/L) 9.21 5.48 0.8–2.0 NT-proBNP (pg/mL) 3643 1068 0–125 Troponin I (pg/mL) 323.0 7.1 0–40 Abbreviations: eGFR; estimated glomerular filtration rate, AST; aspartate aminotransferase, ALT; alanine aminotransferase, LDH; lactate dehydrogenase, NT-proBNP; N-terminal prohormone of brain natriuretic peptide Fig. 1 Histopathologic pictures of kidney biopsy. Light microscopy showed a nodular glomerular sclerosis by Periodic acid Schiff stain showed the positive staining of glomerular nodules and thickening of glomerular and tubular basement membranes (× 100). b , c Immunofluorescence showed positive staining for Igκ and negative staining for Igλ (× 100). d Electron microscopy showed powdery electron dense deposit along the glomerular and tubular basement membranes (arrow head) Fig. 2 Results of laser microdissection followed by mass spectrometry (LMD/MS). LMD/MS Scaffold (Proteome Software Inc., Portland, OR, USA) identified 25 spectra numbers for Ig-kappa chain C region with more than 95% probability. Ig heavy chain, Ig-delta (IgD), and Igλ chain could not be detected, indicating the diagnosis of pure Igκ light chain deposition disease Fig. 3 Hematoxylin-eosin staining (H-E) of liver biopsy showed the atrophy of hepatocytes and dilatation of sinusoidal space (× 100) ( a ). Masson-trichrome staining (M-T) showed the blue deposits along the perisinusoidal space (× 200) ( b ). Immunofluorescence showed positive for anti-Igκ ( c ) and negative for anti-Igλ ( d ) along the sinusoidal space and hepatic vein (× 100)	4.179688	0.958008	sec[1]/p[0]	en	0.999996	33485303	https://doi.org/10.1186/s12882-021-02246-9	[ "chain", "light", "staining", "glomerular", "serum", "liver", "biopsy", "kidney", "along", "free" ]	[ { "code": "2A84.Z", "title": "Heavy chain diseases, unspecified" }, { "code": "QE13", "title": "Tobacco use" }, { "code": "4A01.04", "title": "Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells" }, { "code": "2A83.52", "title": "Light chain deposition disease" }, { "code": "5C53.23", "title": "Mitochondrial protein translation defects" }, { "code": "KA21.2Z", "title": "Low birth weight of newborn, unspecified" }, { "code": "MB48.3", "title": "Light-headedness" }, { "code": "9D45", "title": "Impairment of light sensitivity" }, { "code": "GA20.51", "title": "Light menstrual bleeding" }, { "code": "KD38", "title": "Meconium staining" } ]	=== ICD-11 CODES FOUND === [2A84.Z] Heavy chain diseases, unspecified Also known as: Heavy chain diseases, unspecified \| Heavy chain diseases or malignant immunoproliferative diseases \| malignant immunoproliferative diseases without mention of remission [QE13] Tobacco use Also known as: Tobacco use \| current tobacco use \| tobacco use NOS \| cigarette smoking \| active smoking Excludes: Disorders due to use of nicotine \| Use of Nicotine in non-tobacco forms [4A01.04] Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells Also known as: Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells \| Recurrent infections associated with immunoglobulin isotypes deficiency \| Recurrent infections associated with immunoglobulin isotypes \| Deficiency of immunoglobulin A with immunoglobulin G subclasses deficiency \| Deficiency of IgA with IgG subclasses deficiency [2A83.52] Light chain deposition disease Definition: A disease of the kidney, caused by the deposition of pieces of truncated or abnormal light chain segments of white blood cells. This disease is characterised by fibrillar or granular tissue deposits and renal dysfunction, which may lead to organ failure. Confirmation is by identification of light chain deposition tissue biopsy under an electron microscope. Also known as: Light chain deposition disease \| systemic light chain disease Excludes: Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells [5C53.23] Mitochondrial protein translation defects Definition: This refers to defects in the enzyme that belongs to the family of hydrolases, specifically those acting on acid anhydrides to catalyse transmembrane movement of substances. Also known as: Mitochondrial protein translation defects \| Combined oxidative phosphorylation deficiency \| Combined mitochondrial respiratory chain complex deficiency \| Respiratory chain multiple deficiencies \| COXPD - [Combined oxidative phosphorylation deficiency] [KA21.2Z] Low birth weight of newborn, unspecified Also known as: Low birth weight of newborn, unspecified \| Low birth weight of newborn \| birthweight low for gestational age \| fetal malnutrition, light-for-dates \| intrauterine or fetal malnutrition, light-for-dates [MB48.3] Light-headedness Also known as: Light-headedness \| light headed [9D45] Impairment of light sensitivity Also known as: Impairment of light sensitivity \| Vision sensitivity deficiencies \| Day blindness \| Impairment of dark adaptation \| Moderate Impairment of Dark adaptation [GA20.51] Light menstrual bleeding Definition: Menstruation with light (< 5 ml) volume of monthly blood loss Also known as: Light menstrual bleeding \| light menstrual period \| scanty menses \| scanty menstruation \| scanty period [KD38] Meconium staining Definition: Green or yellowish appearing amniotic fluid, indicating presence of meconium. The newborn’s skin, nail beds or the umbilical cord may be stained. Also known as: Meconium staining \| meconium amniotic fluid \| meconium staining of amniotic fluid Excludes: Neonatal aspiration of meconium \| Meconium passage during delivery === GRAPH WALKS === --- Walk 1 --- [2A84.Z] Heavy chain diseases, unspecified --PARENT--> [2A84] Heavy chain diseases or malignant immunoproliferative diseases Def: A group of rare disorders of immunoglobulin synthesis associated with B-cell proliferative disorders that produce monoclonal heavy chains and typically no light chains.... --CHILD--> [2A84.2] Mu heavy chain disease --- Walk 2 --- [2A84.Z] Heavy chain diseases, unspecified --PARENT--> [2A84] Heavy chain diseases or malignant immunoproliferative diseases Def: A group of rare disorders of immunoglobulin synthesis associated with B-cell proliferative disorders that produce monoclonal heavy chains and typically no light chains.... --CHILD--> [2A84.1] Gamma heavy chain disease Def: A clonal disorder characterised by the secretion of a truncated gamma chain. In most cases, it is associated with morphologic changes also seen in lymphoplasmacytic lymphomas, but the clinical course ... --- Walk 3 --- [QE13] Tobacco use --EXCLUDES--> [?] Hazardous nicotine use Def: A pattern of nicotine use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health pro... --EXCLUDES--> [?] Tobacco use --- Walk 4 --- [QE13] Tobacco use --EXCLUDES--> [?] Hazardous nicotine use Def: A pattern of nicotine use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health pro... --PARENT--> [?] Hazardous substance use Def: Hazardous substance use is a pattern of psychoactive substance use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warr... --- Walk 5 --- [4A01.04] Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects... --PARENT--> [4A01] Primary immunodeficiencies due to disorders of adaptive immunity --- Walk 6 --- [4A01.04] Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects... --CHILD--> [4A01.00] Hereditary agammaglobulinaemia with profoundly reduced or absent B cells Def: This refers to a hereditary type of primary immune deficiency disease characterised by a reduction in all types of gamma globulins, and rare X-linked genetic disorder that affects the body's ability t...	[ "[2A84.Z] Heavy chain diseases, unspecified\n --PARENT--> [2A84] Heavy chain diseases or malignant immunoproliferative diseases\n Def: A group of rare disorders of immunoglobulin synthesis associated with B-cell proliferative disorders that produce monoclonal heavy chains and typically no light chains....\n --CHILD--> [2A84.2] Mu heavy chain disease", "[2A84.Z] Heavy chain diseases, unspecified\n --PARENT--> [2A84] Heavy chain diseases or malignant immunoproliferative diseases\n Def: A group of rare disorders of immunoglobulin synthesis associated with B-cell proliferative disorders that produce monoclonal heavy chains and typically no light chains....\n --CHILD--> [2A84.1] Gamma heavy chain disease\n Def: A clonal disorder characterised by the secretion of a truncated gamma chain. In most cases, it is associated with morphologic changes also seen in lymphoplasmacytic lymphomas, but the clinical course ...", "[QE13] Tobacco use\n --EXCLUDES--> [?] Hazardous nicotine use\n Def: A pattern of nicotine use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health pro...\n --EXCLUDES--> [?] Tobacco use", "[QE13] Tobacco use\n --EXCLUDES--> [?] Hazardous nicotine use\n Def: A pattern of nicotine use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health pro...\n --PARENT--> [?] Hazardous substance use\n Def: Hazardous substance use is a pattern of psychoactive substance use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warr...", "[4A01.04] Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells\n --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects\n Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects...\n --PARENT--> [4A01] Primary immunodeficiencies due to disorders of adaptive immunity", "[4A01.04] Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells\n --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects\n Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects...\n --CHILD--> [4A01.00] Hereditary agammaglobulinaemia with profoundly reduced or absent B cells\n Def: This refers to a hereditary type of primary immune deficiency disease characterised by a reduction in all types of gamma globulins, and rare X-linked genetic disorder that affects the body's ability t..." ]	2A84.Z	Heavy chain diseases, unspecified	[ { "from_icd11": "2A84.Z", "icd10_code": "C888", "icd10_title": "Other malignant immunoproliferative diseases" }, { "from_icd11": "2A84.Z", "icd10_code": "C88", "icd10_title": "Malignant immunoproliferative diseases and certain other B-cell lymphomas" }, { "from_icd11": "2A84.Z", "icd10_code": "C882", "icd10_title": "Heavy chain disease" }, { "from_icd11": "2A84.Z", "icd10_code": "C889", "icd10_title": "Malignant immunoproliferative disease, unspecified" }, { "from_icd11": "2A84.Z", "icd10_code": "C887", "icd10_title": "" }, { "from_icd11": "QE13", "icd10_code": "Z720", "icd10_title": "Tobacco use" }, { "from_icd11": "4A01.04", "icd10_code": "D803", "icd10_title": "Selective deficiency of immunoglobulin G [IgG] subclasses" }, { "from_icd11": "4A01.04", "icd10_code": "D802", "icd10_title": "Selective deficiency of immunoglobulin A [IgA]" }, { "from_icd11": "4A01.04", "icd10_code": "D804", "icd10_title": "Selective deficiency of immunoglobulin M [IgM]" }, { "from_icd11": "2A83.52", "icd10_code": "D477", "icd10_title": "" }, { "from_icd11": "KA21.2Z", "icd10_code": "P071", "icd10_title": "Other low birth weight newborn" }, { "from_icd11": "9D45", "icd10_code": "H538", "icd10_title": "Other visual disturbances" }, { "from_icd11": "9D45", "icd10_code": "H536", "icd10_title": "Night blindness" } ]	C888	Other malignant immunoproliferative diseases
Table 1 Scenario template used to program mannequin by technical staff for simulation exercise Pre-Scenario You are an emergency room physician in a remote community. A 15-year-old male is brought into the trauma room by his mother. The teenager was playing hockey on a local pond when he fell and hit the back of his head. The mother informs you that he had a period of unconsciousness but then became responsive again. Since arriving in the trauma bay, his level of consciousness has been waxing and waning. There is a tertiary care trauma center 5 hours away by ambulance and 90 minutes by helicopter medevac. The ambulance is immediately available. The medevac helicopter will take 60 minutes to arrive. Begin Scenario – Learner enters the trauma room Objective 1: Trauma Assessment and Projected Course Scenario Details Vital Signs/Physical Findings Appropriate Learner Actions Mother: “My son fell on the ice! He was unconscious! You need to help!” Mom is distressed and visibly worried. Vital Signs: BP145/70 / HR 40 / T (Rectal) 35.5°C / RR 22 / SpO2 92% RA. Weight = 52kg Order: Cardiac and O2 Monitor; Labs (CBC, Electrolytes, BUN, Glucose, INR, Blood type and Screen); 2 Large bore IVs; Preoxygenate; C-spine collar; Broselow tape Physical Findings: Patient opens eyes in response to voice (GCS Eyes 3); Patient appears confused and disoriented (GCS Verbal 4); Patient withdraws from painful stimuli (GCS Motor 4); Brisk symmetrical reflexes Learner Verbalizes: A – Airway is protected with C-spine control; B – Breathing is distressed and tachypnic; C – Circulation is adequate; D –Recognize GCS levels and state that GCS is 11; E – Boggy hematoma on occiput Mother (yelling): “He seems to be getting worse. Doctor, you need to help.” Vital Signs: BP145/65 / HR 40 / T (Rectal) 35.5°C / RR 22 / SpO2 92% RA Order: EKG ; Warm blankets Physical Findings: Patient will only open eyes to painful stimuli; (GCS Eyes 2); Patient is making incomprehensible sounds (GCS Verbal 2) Patient has abnormal flexion to painful stimuli (GCS Motor 3) Lerner Verbalizes/Actions: Recognize decreased GCS and state GCS is 7; Maintain inline C-spine immobilization; Prepare for rapid sequence intubation Objective 2: Resuscitation Scenario Details Vital Signs/Physical Findings Appropriate Learner Actions Intubation with appropriate agents Vital Signs: BP145/65 / HR 40 / T (Rectal) 35.5°C / / RR 12 / SpO2 98% Bag Rapid sequence intubation with appropriate agents If no intubation Vital Signs: BP145/60 / HR 40 / T (Rectal) 35.5°C / RR 8 / SpO2 82% RA Initiate rapid sequence intubation with appropriate agents Warm blankets ordered Vital Signs: BP145/65 / HR 40 / T (Rectal) 36.5°C / RR 10 / SpO2 98% Bag If no warm blankets used Vital Signs: BP145/65 / HR 40 / T (Rectal) 35.0°C / RR 10 / SpO2 98% Bag Order warm blankets Objective 3: Neurological Status and Management Scenario Details Vital Signs/Physical Findings Appropriate Learner Actions Reassess vital signs Vital Signs: BP145/65 / HR 40 / T (Rectal) 36.5°C / RR 12 / SpO2 98% Bag Verbalize vital signs Reassess neurological status Physical Findings: Patient will only open eyes to painful stimuli (GCS Eyes 2); Patient is non-verbal (GCS Verbal 1); Patient extends to painful stimuli in a decerebrate posture (GCS Motor 2). The patient has a dilated left pupil with sluggish reaction to light Recognize decreased GCS of 5 and combine clinical findings of bradycardia, tachypnea, and hypertension with wide pulse pressure as Cushing’s triad of vital signs with neurological signs of increased cranial pressure indicative of intracerebral hematoma Order: Medevac helicopter; Portable chest X-ray (CXR) to assess ET tube placement Order: Extra RSI equipment; Extra IV equipment; Portable vitals monitor; Bougie Check results of ordered tests Labs: Normal Consult neurosurgery via telephone Type and Screen:TA+ EKG: Sinus bradycardia ER Nurse: “Neurosurgery advises that patient is critical and needs to be medically transported to tertiary care within 3 hours.” Vital Signs: BP145/70 / HR 40 / T (Rectal) 36.5°C / RR 12 / SpO2 98% Bag (Patient is intubated) Order: Status of medevac helicopter. Call in extra staff to accompany patient to tertiary care Objective 4: Management and Preparation for Transport Scenario Details Vital Signs/Physical Findings Appropriate Learner Actions ER Nurse: “Neurosurgery suggests Mannitol 0.5g/Kg IV, 30° elevation of bed, and hyperventilate 30-35 mmHG CO2 as measured by ABG." (if institution has capability) See debriefing connection to objective 4 Vital Signs: BP 145/70 / HR 50 / T (Rectal) 36.5°C / RR 12 / SpO2 98% Bag Initiate medical management of ICP as indicated by neurosurgery ER Nurse: “Medevac helicopter has arrived and is awaiting us in the hospital parking lot.” Vital Signs: BP 130/70 / HR 50 / T (Rectal) 36.5°C / RR 25 / SpO2 98% Bag Move to medevac helicopter environment Scenario is moved to helicopter safety simulator in Foxtrap, NL with CAE Human Patient Simulator as patient. Endotracheal tube has already been inserted while in the trauma bay and portable vitals monitor is connected to the patient. Aircraft sounds are easily audible. Objective 5: Transportation Management and Difficult Intubation Scenario Details Vital Signs/Physical Findings Appropriate Learner Actions Re-check vitals Vital Signs: BP 140/70 / HR 60/ T (Rectal) 36.5°C / RR 25 / SpO2 95% Bag Learner Verbalizes: Patient is stable Physical Findings: Patient shows tachycardia with pain Order: Estimated time of arrival to tertiary care hospital from pilot (Answer: 30 minutes); Sedative and analgesia Medevac Paramedic: “O2 Sats are dropping!” ET tube is removed from patient by technical staff to simulate ET tube slipping out See debriefing connection to objective 5 Vital Signs: BP 140/70 / HR 70/ T36.5°C / RR 10 / SpO2 80% RA Order: Immediate landing of medevac helicopter; Bag-valve mask respirations by paramedic Prepare for re-intubation with bougie Helicopter lands on stable ground Vital Signs: BP 140/70 / HR 70/ T36.5°C / RR 10 / SpO2 70% RA Begin endotracheal tube reinsertion with bougie Endotracheal tube reinserted with bougie Vital Signs: BP 140/70 / HR 60/ T36.5°C / RR 10 / SpO2 97% Bag Instruct pilot to proceed to tertiary care hospital Rapid sequence intubation not completed N/A End Scenario Medevac helicopter arrives at tertiary care trauma center Patient is consulted to neurosurgery and tertiary care trauma team End Scenario	3.714844	0.709961	sec[1]/p[7]	en	0.999996	27081585	https://doi.org/10.7759/cureus.524	[ "vital", "scenario", "rectal", "helicopter", "medevac", "physical", "trauma", "learner", "appropriate", "order" ]	[ { "code": "MG22", "title": "Fatigue" }, { "code": "MD42", "title": "Results of function studies of the respiratory system" }, { "code": "6A71.3", "title": "Recurrent depressive disorder, current episode severe, without psychotic symptoms" }, { "code": "KD3Y", "title": "Other specified disorders originating in the perinatal period" }, { "code": "6A70.3", "title": "Single episode depressive disorder, severe, without psychotic symptoms" }, { "code": "DB30.4", "title": "Stenosis of the rectum" }, { "code": "DB31.0&XA4KU2", "title": "Rectal fistula" }, { "code": "NB91.91", "title": "Laceration of rectum" }, { "code": "2F90.1", "title": "Neoplasms of unknown behaviour of rectum" }, { "code": "DB32.2Z&XA4KU2", "title": "Rectal dilatation" } ]	=== ICD-11 CODES FOUND === [MG22] Fatigue Definition: A feeling of exhaustion, lethargy, or decreased energy, usually experienced as a weakening or depletion of one's physical or mental resource and characterised by a decreased capacity for work and reduced efficiency in responding to stimuli. Fatigue is normal following a period of exertion, mental or physical, but sometimes may occur in the absence of such exertion as a symptom of health conditions. Also known as: Fatigue \| decreased energy \| worn out \| Lethargy \| lethargic Includes: General physical deterioration \| Lethargy Excludes: Combat fatigue \| Exhaustion due to exposure \| heat exhaustion [MD42] Results of function studies of the respiratory system Also known as: Results of function studies of the respiratory system \| Abnormal results of pulmonary function studies \| nonspecific abnormal results of function study of pulmonary system \| abnormal lung function testing \| abnormal lung function studies [6A71.3] Recurrent depressive disorder, current episode severe, without psychotic symptoms Definition: Recurrent depressive disorder, current episode severe, without psychotic symptoms is diagnosed when the definitional requirements for Recurrent depressive disorder are met and the current episode is severe and there are no delusions or hallucinations during the episode. A depressive episode is characterised by a period of depressed mood or diminished interest in activities occurring most of the day, nearly every day during a period lasting at least two weeks accompanied by other symptoms such as Also known as: Recurrent depressive disorder, current episode severe, without psychotic symptoms \| major depressive disorder, recurrent, severe \| recurrent depressive disorder, current episode severe, without psychotic features \| bipolar affective disorder, depressed, unspecified degree \| bipolar type 1 disorder, most recent episode depressed Includes: Endogenous depression without psychotic symptoms \| Major depression, recurrent without psychotic symptoms \| Manic-depressive psychosis, depressed type without psychotic symptoms [KD3Y] Other specified disorders originating in the perinatal period Also known as: Other specified disorders originating in the perinatal period \| Perinatal morbidity other \| Newborn weakness \| newborn weak \| newborn lack of vitality [6A70.3] Single episode depressive disorder, severe, without psychotic symptoms Definition: Single episode depressive disorder, severe, without psychotic symptoms is diagnosed when the definitional requirements for Single episode depressive disorder are met and the current episode is severe and there are no delusions or hallucinations during the episode. A depressive episode is characterised by a period of depressed mood or diminished interest in activities occurring most of the day, nearly every day during a period lasting at least two weeks accompanied by other symptoms such as diffi Also known as: Single episode depressive disorder, severe, without psychotic symptoms \| single episode depressive disorder, severe, without psychotic features \| major depression NOS \| major depression \| Major depression single episode without psychotic symptoms Includes: Major depression single episode without psychotic symptoms \| Vital depression single episode without psychotic symptoms [DB30.4] Stenosis of the rectum Definition: Rectal stenosis is defined as narrowing of the rectum. Also known as: Stenosis of the rectum \| obstructed rectum \| rectal stenosis \| stricture of rectum \| rectal obstruction [NB91.91] Laceration of rectum Also known as: Laceration of rectum [2F90.1] Neoplasms of unknown behaviour of rectum Also known as: Neoplasms of unknown behaviour of rectum \| rectum tumour NOS === GRAPH WALKS === --- Walk 1 --- [MG22] Fatigue Def: A feeling of exhaustion, lethargy, or decreased energy, usually experienced as a weakening or depletion of one's physical or mental resource and characterised by a decreased capacity for work and redu... --EXCLUDES--> [?] Exhaustion due to exposure --PARENT--> [?] Effects of other deprivation --- Walk 2 --- [MG22] Fatigue Def: A feeling of exhaustion, lethargy, or decreased energy, usually experienced as a weakening or depletion of one's physical or mental resource and characterised by a decreased capacity for work and redu... --EXCLUDES--> [?] Exhaustion due to exposure --PARENT--> [?] Effects of other deprivation --- Walk 3 --- [MD42] Results of function studies of the respiratory system --PARENT--> [?] Clinical findings in the respiratory system --PARENT--> [?] Symptoms, signs or clinical findings of the respiratory system --- Walk 4 --- [MD42] Results of function studies of the respiratory system --PARENT--> [?] Clinical findings in the respiratory system --PARENT--> [?] Symptoms, signs or clinical findings of the respiratory system --- Walk 5 --- [6A71.3] Recurrent depressive disorder, current episode severe, without psychotic symptoms Def: Recurrent depressive disorder, current episode severe, without psychotic symptoms is diagnosed when the definitional requirements for Recurrent depressive disorder are met and the current episode is s... --PARENT--> [6A71] Recurrent depressive disorder Def: Recurrent depressive disorder is characterised by a history of at least two depressive episodes separated by at least several months without significant mood disturbance. A depressive episode is chara... --EXCLUDES--> [?] Single episode depressive disorder Def: Single episode depressive disorder is characterised by the presence or history of one depressive episode when there is no history of prior depressive episodes. A depressive episode is characterised by... --- Walk 6 --- [6A71.3] Recurrent depressive disorder, current episode severe, without psychotic symptoms Def: Recurrent depressive disorder, current episode severe, without psychotic symptoms is diagnosed when the definitional requirements for Recurrent depressive disorder are met and the current episode is s... --PARENT--> [6A71] Recurrent depressive disorder Def: Recurrent depressive disorder is characterised by a history of at least two depressive episodes separated by at least several months without significant mood disturbance. A depressive episode is chara... --EXCLUDES--> [?] Bipolar or related disorders Def: Bipolar and related disorders are episodic mood disorders defined by the occurrence of Manic, Mixed or Hypomanic episodes or symptoms. These episodes typically alternate over the course of these disor...	[ "[MG22] Fatigue\n Def: A feeling of exhaustion, lethargy, or decreased energy, usually experienced as a weakening or depletion of one's physical or mental resource and characterised by a decreased capacity for work and redu...\n --EXCLUDES--> [?] Exhaustion due to exposure\n --PARENT--> [?] Effects of other deprivation", "[MG22] Fatigue\n Def: A feeling of exhaustion, lethargy, or decreased energy, usually experienced as a weakening or depletion of one's physical or mental resource and characterised by a decreased capacity for work and redu...\n --EXCLUDES--> [?] Exhaustion due to exposure\n --PARENT--> [?] Effects of other deprivation", "[MD42] Results of function studies of the respiratory system\n --PARENT--> [?] Clinical findings in the respiratory system\n --PARENT--> [?] Symptoms, signs or clinical findings of the respiratory system", "[MD42] Results of function studies of the respiratory system\n --PARENT--> [?] Clinical findings in the respiratory system\n --PARENT--> [?] Symptoms, signs or clinical findings of the respiratory system", "[6A71.3] Recurrent depressive disorder, current episode severe, without psychotic symptoms\n Def: Recurrent depressive disorder, current episode severe, without psychotic symptoms is diagnosed when the definitional requirements for Recurrent depressive disorder are met and the current episode is s...\n --PARENT--> [6A71] Recurrent depressive disorder\n Def: Recurrent depressive disorder is characterised by a history of at least two depressive episodes separated by at least several months without significant mood disturbance. A depressive episode is chara...\n --EXCLUDES--> [?] Single episode depressive disorder\n Def: Single episode depressive disorder is characterised by the presence or history of one depressive episode when there is no history of prior depressive episodes. A depressive episode is characterised by...", "[6A71.3] Recurrent depressive disorder, current episode severe, without psychotic symptoms\n Def: Recurrent depressive disorder, current episode severe, without psychotic symptoms is diagnosed when the definitional requirements for Recurrent depressive disorder are met and the current episode is s...\n --PARENT--> [6A71] Recurrent depressive disorder\n Def: Recurrent depressive disorder is characterised by a history of at least two depressive episodes separated by at least several months without significant mood disturbance. A depressive episode is chara...\n --EXCLUDES--> [?] Bipolar or related disorders\n Def: Bipolar and related disorders are episodic mood disorders defined by the occurrence of Manic, Mixed or Hypomanic episodes or symptoms. These episodes typically alternate over the course of these disor..." ]	MG22	Fatigue	[ { "from_icd11": "MG22", "icd10_code": "R5382", "icd10_title": "Chronic fatigue, unspecified" }, { "from_icd11": "MG22", "icd10_code": "R530", "icd10_title": "Neoplastic (malignant) related fatigue" }, { "from_icd11": "MG22", "icd10_code": "R532", "icd10_title": "Functional quadriplegia" }, { "from_icd11": "MG22", "icd10_code": "R531", "icd10_title": "Weakness" }, { "from_icd11": "MG22", "icd10_code": "R5383", "icd10_title": "Other fatigue" }, { "from_icd11": "MG22", "icd10_code": "R53", "icd10_title": "Malaise and fatigue" }, { "from_icd11": "MD42", "icd10_code": "R942", "icd10_title": "Abnormal results of pulmonary function studies" }, { "from_icd11": "6A71.3", "icd10_code": "F332", "icd10_title": "Major depressive disorder, recurrent severe without psychotic features" }, { "from_icd11": "KD3Y", "icd10_code": "P9689", "icd10_title": "Other specified conditions originating in the perinatal period" }, { "from_icd11": "6A70.3", "icd10_code": "F329", "icd10_title": "Major depressive disorder, single episode, unspecified" }, { "from_icd11": "6A70.3", "icd10_code": "F322", "icd10_title": "Major depressive disorder, single episode, severe without psychotic features" }, { "from_icd11": "DB30.4", "icd10_code": "K624", "icd10_title": "Stenosis of anus and rectum" }, { "from_icd11": "2F90.1", "icd10_code": "D375", "icd10_title": "Neoplasm of uncertain behavior of rectum" } ]	R5382	Chronic fatigue, unspecified
The patient was a 44-year-old Chinese woman with G1P0 + 1 and body mass index of 19. She sought treatment in our emergency surgery department due to sudden onset of intermittent abdominal colic with vomiting for 1 day after eating indigestible food. The patient also had the symptoms of intestinal obstruction such as nausea, anal pendant expansion, and no gas nor defecation but did not have discomforts such as fever, bloody stool and vaginal bleeding. The patient underwent laparoscopic cyst excision due to ovarian endometriotic cysts 7 years ago and laparoscopic separating surgery due to infertility 4 years ago. The patient had regular menstrual periods and secondary infertility for 20 years. She received one in vitro fertilization and the embryo transfer 4 years ago; however, embryo implantation failed. Physical examination discovered a 2 cm ovarian cyst 1 year ago but the patient did not receive treatment and follow up. The patient did not have a history of long-term exposure to estrogen, oral short-acting contraceptives, or other drugs. She had no known family history of cancer. Abdominal examination suggested lower abdomen tenderness without rebound pain and no palpable abdominal mass. Gynecological examination revealed smooth cervical appearance, uniformly enlarged uterus with a size close to the size of a 2-month pregnancy, left adnexa had patchy thickness, and left parametrial tissues had a palpable hard and irregular nodule of approximately 3 cm that involved left vaginal fornix. Gynecological color ultrasound indicated that the endometrium was 0.6 cm. The thickness of the anterior and posterior walls of myometrium was not even, myometrial echo was coarse and uneven especially the posterior wall, and there was an echo from a mass with unclear boundaries at the size of 3.93.3 cm. The posterior cervical wall had a detected hypoechoic nodule at the size of 1.71.5 cm, there were abundant blood flow signals, and the boundaries were clear. The renogram showed the curve of left renal incomplete obstruction. The transrectal contrast-enhanced ultrasound examination detected a solid hypoechoic mass of approximately 1.8*3.5 cm in posterior cervical wall; the mass had irregular morphology and unclear boundary, the mass invaded the anterior wall of rectum through the rectouterine pouch. The color Doppler flow imaging suggested that there were more abundant blood flow signals in the hypoechoic mass. Gastroscopy and colonoscopy did not show obvious abnormality. The patient had normal liver and kidney functions, CA-125 was 183.4U/ml, and CA-199 was 66.7U/ml. Therefore, adenomyosis combined with adenomyoma and deep endometriosis combined with ureteral obstruction and intestinal obstruction were considered. The patient underwent robot-assisted laparoscopic subtotal hysterectomy, bilateral adnexa resection, deep endometriosis lesion resection and bilateral ureteral stent placement in the Department of Genecology of our hospital. The surgery showed that uterus enlarged to the size of approximately 2 month pregnancy, the appearance of bilateral oviducts did not have obvious abnormality, the surface of left ovary had brown endometriotic lesions, the right ovary was swelling with a diameter of 3 cm and had adhesion with the posterior wall of the uterus and rectum, and the proper ligament of the left ovary had dense adhesion with left ureter, left posterior wall of uterus, rectum . There was a 3 cm hard lesion with unclear boundaries in the adhesion area that involved left vaginal fornix, lower segment of left ureter, and rectum. There was a chocolate cyst-like lesion of 1 cm that contained chocolate-like old bleeding fluid . The lower segment of left ureter at length of 10 cm had thickened and was hard and the segment of rectum at approximately 4 cm behind cervix had thickened and hard muscular wall. There was no lesion involvement in the upper abdomen. Dissection of endometrium after hysterectomy did not show obvious lesions, the myometrium was diffuse and thickened, and the posterior wall of uterus had an adenomyoma-like lesion of approximately 4 cm. Postoperative pathological examinations suggested eutopic endometrium had multifocal atypical hyperplasia of endometrial glands and formations of highly/moderately differentiated endometrioid adenocarcinoma. The maximum diameter of intramyometrial adenomyosis was 5 cm and most gland components exhibited atypical hyperplasia and highly/moderately differentiated endometrioid adenocarcinoma. Other myometrium wall tissues were scattered in the ectopic endometrium tissues combined with atypical hyperplasia and formation of endometrioid adenocarcinoma . Left parametrial lesions suggested poorly differentiated endometrioid adenocarcinoma combined with partial clear cell carcinoma involvement . Uterine specimens showed tumor thrombus in vessels and perineurium invasion. There was no tumor involvement in cervix. Left ovary had endometriotic cysts with no tumor involvement, and right ovary had tumor involvement. Immunohistochemistry results of left parametrial lesions suggested ER(+)PR(+); CK7(+); CD10(-); CEA(+); Ki67 (50%). Immunohistochemistry results of adenomyosis suggested ER(+); PR(+); CK7(+); CD10(-); CEA(focal+); Ki67(20%). The patient was considered to have carcinomatous changes of both uterine adenomyoma and deep endometriotic lesions. On 8th day after the first surgery, the patient underwent transabdominal tumor cell reduction surgery including greater omentum resection, appendectomy, pelvic lymph node dissection and para-abdominal aortic lymph node dissection, partial rectectomy, intestinal anastomosis and resection of the lower segment of left ureter, left ureteral cystoplasty and left ureteral stent implantation. Postoperative pathological examination suggested that ureter had tumor infiltration, bilateral pelvic lymph nodes and para-abdominal aorta lymph nodes all had tumor metastases, tumor infiltration was observed on the serosal surface of the intestinal wall of of partial rectum, the muscular layer of intestinal wall had tumor infiltration, and the appendix and greater omentum did not have tumor infiltration. After a satisfactory postoperative recovery, the patient was treated with Taxol and Carboplatin chemotherapy regimen. After chemotherapy completion, the patient has been undergoing regular follow-up examinations; no recurrence has been noted at 18 months.	3.869141	0.984375	sec[2]/p[0]	en	0.999997	39090585	https://doi.org/10.1186/s12905-024-03170-4	[ "wall", "tumor", "suggested", "that", "rectum", "abdominal", "intestinal", "uterus", "approximately", "lesion" ]	[ { "code": "LB0Y", "title": "Other specified structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord" }, { "code": "PA82", "title": "Unintentional striking against stationary object" }, { "code": "NB50.Y&XA3KX0&XJ1C6", "title": "Haematoma of abdominal wall" }, { "code": "DC51.1", "title": "Peritoneal adhesions" }, { "code": "LB73.1Z", "title": "Structural developmental anomalies of chest wall, unspecified" }, { "code": "2F9Z", "title": "Neoplasms of unknown behaviour of unspecified site" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "2E6Z", "title": "Carcinoma in situ of unspecified site" }, { "code": "2F91.1", "title": "Neoplasms of unknown behaviour of trachea, bronchus or lung" }, { "code": "2F92", "title": "Neoplasms of unknown behaviour of skin" } ]	=== ICD-11 CODES FOUND === [LB0Y] Other specified structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord Also known as: Other specified structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord \| Congenital deformity of abdominal wall \| abdominal wall defect NOS [PA82] Unintentional striking against stationary object Also known as: Unintentional striking against stationary object \| striking against stationary object \| striking against or struck by other objects \| Walked into wall [DC51.1] Peritoneal adhesions Definition: Disorders of peritoneum sticking by scar tissue or fibrosis Also known as: Peritoneal adhesions \| abdominal adhesion \| adhesive peritoneal band \| peritoneal adhesion \| peritoneal band Excludes: Adhesions of large intestine with obstruction \| Postprocedural pelvic peritoneal adhesions \| Intestinal adhesions or bands of small intestine with obstruction [LB73.1Z] Structural developmental anomalies of chest wall, unspecified Also known as: Structural developmental anomalies of chest wall, unspecified \| Structural developmental anomalies of chest wall \| Malformations of chest wall [2F9Z] Neoplasms of unknown behaviour of unspecified site Also known as: Neoplasms of unknown behaviour of unspecified site \| neoplasia \| neoplasm growth NOS \| tumour of unspecified site \| tumourlet of unspecified site [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [2E6Z] Carcinoma in situ of unspecified site Also known as: Carcinoma in situ of unspecified site \| carcinoma in situ of unspecified site \| carcinoma in situ NOS \| carcinoma in situ \| in situ neoplasm [2F91.1] Neoplasms of unknown behaviour of trachea, bronchus or lung Also known as: Neoplasms of unknown behaviour of trachea, bronchus or lung \| trachea, bronchus or lung tumour NOS \| Intravascular bronchial alveolar tumour of unspecified site \| Bronchial adenoma of unknown behaviour of unspecified site \| Intravascular bronchial alveolar tumour unknown behaviour of unspecified site [2F92] Neoplasms of unknown behaviour of skin Also known as: Neoplasms of unknown behaviour of skin \| skin tumour NOS === GRAPH WALKS === --- Walk 1 --- [LB0Y] Other specified structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord --PARENT--> [?] Structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord Def: Any condition caused by failure of the diaphragm, abdominal wall or umbilical cord to correctly develop during the antenatal period.... --EXCLUDES--> [?] Prune belly syndrome Def: A syndrome is characterised by cryptorchidism, urinary tract defects, and poor development of the abdominal muscles causing the skin on the abdomen to wrinkle.... --- Walk 2 --- [LB0Y] Other specified structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord --PARENT--> [?] Structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord Def: Any condition caused by failure of the diaphragm, abdominal wall or umbilical cord to correctly develop during the antenatal period.... --CHILD--> [LB01] Omphalocele Def: Omphalocele is an embryopathy classified in the group of abdominal celosomias and is characterised by a large hernia of the abdominal wall, centred on the umbilical cord, in which the protruding visce... --- Walk 3 --- [PA82] Unintentional striking against stationary object --PARENT--> [?] Unintentional exposure to object, not elsewhere classified --EXCLUDES--> [?] Assault by exposure to object not elsewhere classified --- Walk 4 --- [PA82] Unintentional striking against stationary object --PARENT--> [?] Unintentional exposure to object, not elsewhere classified --CHILD--> [PA81] Unintentionally struck by moving object --- Walk 5 --- [DC51.1] Peritoneal adhesions Def: Disorders of peritoneum sticking by scar tissue or fibrosis... --EXCLUDES--> [?] Adhesions of large intestine with obstruction Def: Large bowel obstruction resulting from intraabdominal adhesion due to laparotomy, trauma, and intraabdominal inflammation such as endometriosis.... --CHILD--> [?] Postoperative obstruction of the large intestine --- Walk 6 --- [DC51.1] Peritoneal adhesions Def: Disorders of peritoneum sticking by scar tissue or fibrosis... --EXCLUDES--> [?] Intestinal adhesions or bands of small intestine with obstruction Def: Small bowel obstruction resulting from intraabdominal adhesion due to laparotomy, trauma, and intraabdominal inflammation such as endometriosis.... --PARENT--> [?] Obstruction of small intestine Def: Hindrance of the passage of luminal contents in the small intestine. Obstruction of the small intestine can be partial or complete, and caused by intrinsic or extrinsic factors. Simple obstruction is ...	[ "[LB0Y] Other specified structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord\n --PARENT--> [?] Structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord\n Def: Any condition caused by failure of the diaphragm, abdominal wall or umbilical cord to correctly develop during the antenatal period....\n --EXCLUDES--> [?] Prune belly syndrome\n Def: A syndrome is characterised by cryptorchidism, urinary tract defects, and poor development of the abdominal muscles causing the skin on the abdomen to wrinkle....", "[LB0Y] Other specified structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord\n --PARENT--> [?] Structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord\n Def: Any condition caused by failure of the diaphragm, abdominal wall or umbilical cord to correctly develop during the antenatal period....\n --CHILD--> [LB01] Omphalocele\n Def: Omphalocele is an embryopathy classified in the group of abdominal celosomias and is characterised by a large hernia of the abdominal wall, centred on the umbilical cord, in which the protruding visce...", "[PA82] Unintentional striking against stationary object\n --PARENT--> [?] Unintentional exposure to object, not elsewhere classified\n --EXCLUDES--> [?] Assault by exposure to object not elsewhere classified", "[PA82] Unintentional striking against stationary object\n --PARENT--> [?] Unintentional exposure to object, not elsewhere classified\n --CHILD--> [PA81] Unintentionally struck by moving object", "[DC51.1] Peritoneal adhesions\n Def: Disorders of peritoneum sticking by scar tissue or fibrosis...\n --EXCLUDES--> [?] Adhesions of large intestine with obstruction\n Def: Large bowel obstruction resulting from intraabdominal adhesion due to laparotomy, trauma, and intraabdominal inflammation such as endometriosis....\n --CHILD--> [?] Postoperative obstruction of the large intestine", "[DC51.1] Peritoneal adhesions\n Def: Disorders of peritoneum sticking by scar tissue or fibrosis...\n --EXCLUDES--> [?] Intestinal adhesions or bands of small intestine with obstruction\n Def: Small bowel obstruction resulting from intraabdominal adhesion due to laparotomy, trauma, and intraabdominal inflammation such as endometriosis....\n --PARENT--> [?] Obstruction of small intestine\n Def: Hindrance of the passage of luminal contents in the small intestine. Obstruction of the small intestine can be partial or complete, and caused by intrinsic or extrinsic factors. Simple obstruction is ..." ]	LB0Y	Other specified structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord	[ { "from_icd11": "PA82", "icd10_code": "W2209XA", "icd10_title": "Striking against other stationary object, initial encounter" }, { "from_icd11": "PA82", "icd10_code": "W2201XA", "icd10_title": "Walked into wall, initial encounter" }, { "from_icd11": "PA82", "icd10_code": "W2211XA", "icd10_title": "Striking against or struck by driver side automobile airbag, initial encounter" }, { "from_icd11": "PA82", "icd10_code": "W228XXS", "icd10_title": "Striking against or struck by other objects, sequela" }, { "from_icd11": "PA82", "icd10_code": "W2203XD", "icd10_title": "Walked into furniture, subsequent encounter" }, { "from_icd11": "PA82", "icd10_code": "W2203XA", "icd10_title": "Walked into furniture, initial encounter" }, { "from_icd11": "PA82", "icd10_code": "W228XXD", "icd10_title": "Striking against or struck by other objects, subsequent encounter" }, { "from_icd11": "PA82", "icd10_code": "W2212XA", "icd10_title": "Striking against or struck by front passenger side automobile airbag, initial encounter" }, { "from_icd11": "PA82", "icd10_code": "W2209XS", "icd10_title": "Striking against other stationary object, sequela" }, { "from_icd11": "PA82", "icd10_code": "W228XXA", "icd10_title": "Striking against or struck by other objects, initial encounter" }, { "from_icd11": "PA82", "icd10_code": "W22", "icd10_title": "Striking against or struck by other objects" }, { "from_icd11": "DC51.1", "icd10_code": "K660", "icd10_title": "Peritoneal adhesions (postprocedural) (postinfection)" }, { "from_icd11": "LB73.1Z", "icd10_code": "Q766", "icd10_title": "Other congenital malformations of ribs" }, { "from_icd11": "2F9Z", "icd10_code": "D487", "icd10_title": "Neoplasm of uncertain behavior of other specified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D482", "icd10_title": "Neoplasm of uncertain behavior of peripheral nerves and autonomic nervous system" } ]	W2209XA	Striking against other stationary object, initial encounter
A 28-year-old previously healthy Caucasian female (ethnic group: Slovak, West Slavic region) presented to the Emergency department of the Department of Infectology and Geographical Medicine, University Hospital Bratislava, with a history of headache, disorientation and tonic-clonic seizures. Two days before admission, she developed a low-grade fever, rhinitis and mild tiredness. A SARS-CoV-2 antigen self-test (nasal swab, lateral flow assay) was positive. One day before admission, she developed a mild headache. In the evening, according to her husband, she was mildly confused. During the night, she had at least five tonic-clonic seizures and was transferred to the emergency department via ambulance. At the emergency department, the seizures were terminated using 10 mg of intravenous diazepam. Subsequently, she was obnubilated and did not respond to commands. Neurologic examination revealed nuchal rigidity (opposition to flexion up to 5 cm) with no focal signs. Here body temperature was elevated to 39 °C, but otherwise, the physical examination was unremarkable. The biochemical examination of plasma including C-reactive protein and procalcitonin was unremarkable. A complete blood count revealed mild monocytosis (750 cells/mL; reference range: 100–700 cells/mL). Native computed tomography (CT) revealed no abnormalities. We performed a lumbar puncture, and an examination of the cerebrospinal fluid (CSF) revealed pleocytosis with 37 mononuclear cells/μL, 19 polymorphonuclear cells/μL and 56 erythrocytes/μL. The total protein concentration was elevated at 815 mg/L (reference range: 200–500 mg/l). The CSF albumin level (584 mg/L; reference range: 120/300 mg/:) and immunoglobulin G level (128 mg/L; reference range: 12–40 mg/L) were also elevated. The CSF glucose and lactate concentrations were unremarkable. Polymerase chain reaction (PCR) was negative for herpes simplex virus 1 and 2, varicella zoster virus, tick borne encephalitis, enterovirus, Listeria monocytogenes , Neisseria meningitidis , Streptococcus pneumoniae , Haemophilus influenzae and Escherichia coli nucleic acid in the CSF. The patient was admitted to the intensive care unit (ICU) and parenteral treatment with acyclovir 500 mg every 8 h and ceftriaxone 2 g every 12 h was started. In addition, antiseizure therapy with intravenous levetiracetam 2 g per day was started. During the first 2 days after admission, the patient remained soporous, she did not respond to commands and her Glasgow Coma Scale (GCS) score was 7 points (eye response: 2 points; motoric response: 4 points; verbal response: 1 point). The nonconvulsive epileptic state was suggested to be the cause of the quantitative impairment of consciousness. Therefore, the dose of levetiracetam was increased to 4 g per day. On day 3 after admission, the patient’s state of consciousness improved: she was awake, responded to commands and fully cooperated during diagnostic and therapeutic procedures. However, she was apathetic and did not provide verbal responses. Magnetic resonance imaging (MRI) with gadolinium contrast was conducted and revealed no abnormalities. SARS-CoV-2 PCR from a nasopharyngeal swab was positive (cycle threshold: 16 cycles), which confirmed the COVID-19 diagnosis. On day 4 after admission, the patient developed frequent focal tonic-clonic seizures limited to facial muscles and upper extremities (FAS) and focal seizures with impaired awareness (FIAS). The seizures were provoked by drinking and eating or touching the lips or perioral area. She also had up to five focal to bilateral tonic-clonic seizures (FBTCS) per day. She developed a severe headache; nausea and retching; and psychiatric symptoms including agitation, insomnia and repetitive and stereotypical behaviour. CT ruled out new focal neurologic involvement or significant brain oedema. By day 8 after admission, the frequency of FAS and FIAS had gradually increased up to 50 per day. At this time, MRI with gadolinium contrast was repeated; it again revealed no abnormalities. Lacosamide 400 mg per day was added and the levetiracetam dose was increased to 6 g per day with little to no effects on the seizure frequency or duration. Because the seizures were resistant to treatment, AIE was proposed and intravenous immunoglobulin (IVIG) at a dose of 20 g per day was introduced. Serum was drawn to examine antineuronal antibodies prior to commencing IVIG treatment. Electroencephalography (EEG) was conducted on day 10 after admission. It revealed generalised rhythmic delta frequency activity with superimposed fast activity known as extreme delta brush . Examination of antineuronal antibodies associated with AIE revealed anti-SOX1 autoantibodies in the serum and cerebrospinal fluid. A course of parenteral methylprednisolone at a dose of 1 g per day was started. On the day 11 after admission – corresponding to the second day of the methylprednisolone course and the fourth day of the IVIG course – the frequency of seizures decreased to five per day. The patient’s nausea and retching resolved and her headache became milder. On day 13 after admission, the patient completed the 3-day course of methylprednisolone and the 5-day course of IVIG. Her clinical condition further improved in the following 5 days of hospitalisation. Her seizures and aphasia and psychiatric symptoms resolved completely. The patient was discharged on day 20 after admission. At the time of hospital discharge, she experienced only mild fatigue and a self-reported mild impairment of concentration. After discharge, the patient underwent oncologic screening. The gynaecologic examination, including gynaecologic and breast ultrasound and mammography, was negative. As the diagnosis of AIE was conclusive, the antineuronal antibody test was not repeated. The abdominal ultrasound and occult faecal bleeding test were unremarkable. The patient underwent whole-body [¹ ⁸F]fluorodeoxyglucose positron emission tomography; the findings were unremarkable and study found no sign of a tumour. Her out-patient follow-up at 4 months indicated complete remission of clinical symptoms, including a normal EEG . Fig. 1 Encephalogram at the time of presentation. It shows generalised rhythmic delta with superimposed fast activity: extreme delta brush pattern (marked by black arrow). Fig. 1 Fig. 2 An electroencephalogram at the patient’s 4-month follow-up. It shows physiologic patterns. Fig. 2	3.978516	0.979004	sec[1]/p[0]	en	0.999998	40330578	https://doi.org/10.1016/j.idcr.2025.e02220	[ "seizures", "unremarkable", "course", "department", "headache", "tonic", "clonic", "including", "cells", "reference" ]	[ { "code": "8A68.Z", "title": "Type of seizure, unspecified" }, { "code": "8A6Z", "title": "Epilepsy or seizures, unspecified" }, { "code": "8A63.Y", "title": "Seizure due to other acute cause" }, { "code": "8A67", "title": "Acute repetitive seizures" }, { "code": "8A68.Y", "title": "Other specified type of seizure" }, { "code": "LA8Y", "title": "Other specified structural developmental anomaly of heart or great vessels" }, { "code": "8B10.Y", "title": "Other specified transient ischaemic attack" }, { "code": "6A80", "title": "Symptomatic and course presentations for mood episodes in mood disorders" }, { "code": "LA8C.1", "title": "Anomalous aortic origin or course of coronary artery" }, { "code": "8B22.Y", "title": "Other specified cerebrovascular disease" } ]	=== ICD-11 CODES FOUND === [8A68.Z] Type of seizure, unspecified Also known as: Type of seizure, unspecified \| Types of seizures \| uncontrolled seizures \| Seizure NOS \| fits NOS [8A6Z] Epilepsy or seizures, unspecified Also known as: Epilepsy or seizures, unspecified \| Cerebral seizures \| Seizure disorder \| seizure disorder, so described \| epilepsy NOS [8A63.Y] Seizure due to other acute cause Also known as: Seizure due to other acute cause \| Seizures due to immune disorders \| Seizures due to medications \| Toxic syndrome with generalised seizures, drug related \| Acute seizures due to central nervous system infections or infestations [8A67] Acute repetitive seizures Definition: Acute repetitive seizures are multiple seizures, with a distinct time of onset, with recovery between each seizure, occurring within 24 hours in adults, or 12 hours in children. Also known as: Acute repetitive seizures \| complex partial status epilepticus \| Cluster seizures \| Serial seizures \| Recurrent seizures [8A68.Y] Other specified type of seizure Also known as: Other specified type of seizure \| Absence episode \| Absence seizure episode \| Pseudotetanus \| Clonic seizure disorder [LA8Y] Other specified structural developmental anomaly of heart or great vessels Also known as: Other specified structural developmental anomaly of heart or great vessels \| Congenital anomaly of position or spatial relationships of thoraco-abdominal organs \| Usual atrial arrangement \| atrial situs solitus \| Abnormal atrial arrangement [8B10.Y] Other specified transient ischaemic attack Also known as: Other specified transient ischaemic attack \| Vertebrobasilar artery syndrome \| vertebrobasilar arterial insufficiency \| vertebrobasilar insufficiency \| vertebro-basilar artery syndrome, course of resolution unspecified [6A80] Symptomatic and course presentations for mood episodes in mood disorders Definition: These categories may be applied to describe the presentation and characteristics of mood episodes in the context of single episode depressive disorder, recurrent depressive disorder, bipolar type I disorder, or bipolar type II disorder. These categories indicate the presence of specific, important features of the clinical presentation or of the course, onset, and pattern of mood episodes. These categories are not mutually exclusive, and as many may be added as apply. Also known as: Symptomatic and course presentations for mood episodes in mood disorders [LA8C.1] Anomalous aortic origin or course of coronary artery Definition: A congenital cardiovascular malformation in which the origin and/or course of a coronary artery is abnormal. This is where coronary "anomalies" in the presence of discordant ventriculo-arterial connections should be coded. Also known as: Anomalous aortic origin or course of coronary artery \| Anomalous aortic origin of coronary artery with ventriculo-arterial concordance \| Anomalous aortic origin of coronary artery \| AAOCA - [Anomalous aortic origin of coronary artery] \| Right coronary artery from left aortic sinus with ventriculo-arterial concordance [8B22.Y] Other specified cerebrovascular disease Also known as: Other specified cerebrovascular disease \| Posterior reversible encephalopathy \| Multiple or bilateral precerebral artery syndromes \| multiple or bilateral precerebral artery syndromes, course of resolution unspecified \| precerebral artery insufficiency NOS === GRAPH WALKS === --- Walk 1 --- [8A68.Z] Type of seizure, unspecified --PARENT--> [8A68] Types of seizures --EXCLUDES--> [?] Dissociative neurological symptom disorder, with non-epileptic seizures Def: Dissociative neurological symptom disorder, with non-epileptic seizures is characterised by a symptomatic presentation of seizures or convulsions that are not consistent with a recognised disease of t... --- Walk 2 --- [8A68.Z] Type of seizure, unspecified --PARENT--> [8A68] Types of seizures --CHILD--> [8A68.1] Absence seizures, atypical Def: Absence seizures with changes in tone more pronounced than in typical absences or with non-abrupt onset and/or cessation, often associated with slow, irregular, generalised spike-wave activity.... --- Walk 3 --- [8A6Z] Epilepsy or seizures, unspecified --PARENT--> [?] Epilepsy or seizures Def: At least 2 unprovoked (or reflex) seizures occurring more than 24 hours apart.... --CHILD--> [8A61] Genetic or presumed genetic syndromes primarily expressed as epilepsy Def: The epilepsy is, as best as understood, the direct result of one or more known or presumed genetic defects in which seizures are the core symptom of the disorder.... --- Walk 4 --- [8A6Z] Epilepsy or seizures, unspecified --PARENT--> [?] Epilepsy or seizures Def: At least 2 unprovoked (or reflex) seizures occurring more than 24 hours apart.... --EXCLUDES--> [?] Syncope or collapse Def: Syncope is also called fainting, temporary loss of consciousness. Syncope and collapse is temporary loss of consciousness with a fall down.... --- Walk 5 --- [8A63.Y] Seizure due to other acute cause --PARENT--> [8A63] Seizure due to acute causes Def: A clinical seizure occurring at the time of a systemic insult or in close temporal association with a documented brain insult.... --CHILD--> [8A63.0] Febrile seizures Def: Seizures associated with a rise of the body temperature in the absence of intracranial infection, metabolic disturbance, or history of afebrile seizures. They most commonly occur in children between t... --- Walk 6 --- [8A63.Y] Seizure due to other acute cause --PARENT--> [8A63] Seizure due to acute causes Def: A clinical seizure occurring at the time of a systemic insult or in close temporal association with a documented brain insult.... --EXCLUDES--> [?] Migraine aura-triggered seizures Def: A seizure triggered by an attack of migraine with aura....	[ "[8A68.Z] Type of seizure, unspecified\n --PARENT--> [8A68] Types of seizures\n --EXCLUDES--> [?] Dissociative neurological symptom disorder, with non-epileptic seizures\n Def: Dissociative neurological symptom disorder, with non-epileptic seizures is characterised by a symptomatic presentation of seizures or convulsions that are not consistent with a recognised disease of t...", "[8A68.Z] Type of seizure, unspecified\n --PARENT--> [8A68] Types of seizures\n --CHILD--> [8A68.1] Absence seizures, atypical\n Def: Absence seizures with changes in tone more pronounced than in typical absences or with non-abrupt onset and/or cessation, often associated with slow, irregular, generalised spike-wave activity....", "[8A6Z] Epilepsy or seizures, unspecified\n --PARENT--> [?] Epilepsy or seizures\n Def: At least 2 unprovoked (or reflex) seizures occurring more than 24 hours apart....\n --CHILD--> [8A61] Genetic or presumed genetic syndromes primarily expressed as epilepsy\n Def: The epilepsy is, as best as understood, the direct result of one or more known or presumed genetic defects in which seizures are the core symptom of the disorder....", "[8A6Z] Epilepsy or seizures, unspecified\n --PARENT--> [?] Epilepsy or seizures\n Def: At least 2 unprovoked (or reflex) seizures occurring more than 24 hours apart....\n --EXCLUDES--> [?] Syncope or collapse\n Def: Syncope is also called fainting, temporary loss of consciousness. Syncope and collapse is temporary loss of consciousness with a fall down....", "[8A63.Y] Seizure due to other acute cause\n --PARENT--> [8A63] Seizure due to acute causes\n Def: A clinical seizure occurring at the time of a systemic insult or in close temporal association with a documented brain insult....\n --CHILD--> [8A63.0] Febrile seizures\n Def: Seizures associated with a rise of the body temperature in the absence of intracranial infection, metabolic disturbance, or history of afebrile seizures. They most commonly occur in children between t...", "[8A63.Y] Seizure due to other acute cause\n --PARENT--> [8A63] Seizure due to acute causes\n Def: A clinical seizure occurring at the time of a systemic insult or in close temporal association with a documented brain insult....\n --EXCLUDES--> [?] Migraine aura-triggered seizures\n Def: A seizure triggered by an attack of migraine with aura...." ]	8A68.Z	Type of seizure, unspecified	[ { "from_icd11": "8A68.Z", "icd10_code": "R561", "icd10_title": "Post traumatic seizures" }, { "from_icd11": "8A68.Z", "icd10_code": "R569", "icd10_title": "Unspecified convulsions" }, { "from_icd11": "8A68.Z", "icd10_code": "R56", "icd10_title": "Convulsions, not elsewhere classified" }, { "from_icd11": "8A68.Z", "icd10_code": "R568", "icd10_title": "" }, { "from_icd11": "8A6Z", "icd10_code": "G40A09", "icd10_title": "Absence epileptic syndrome, not intractable, without status epilepticus" }, { "from_icd11": "8A6Z", "icd10_code": "G40B09", "icd10_title": "Juvenile myoclonic epilepsy, not intractable, without status epilepticus" }, { "from_icd11": "8A6Z", "icd10_code": "G40B19", "icd10_title": "Juvenile myoclonic epilepsy, intractable, without status epilepticus" }, { "from_icd11": "8A6Z", "icd10_code": "G40A19", "icd10_title": "Absence epileptic syndrome, intractable, without status epilepticus" }, { "from_icd11": "8A6Z", "icd10_code": "G40A11", "icd10_title": "Absence epileptic syndrome, intractable, with status epilepticus" }, { "from_icd11": "8A6Z", "icd10_code": "G40A01", "icd10_title": "Absence epileptic syndrome, not intractable, with status epilepticus" }, { "from_icd11": "8A6Z", "icd10_code": "G40409", "icd10_title": "Other generalized epilepsy and epileptic syndromes, not intractable, without status epilepticus" }, { "from_icd11": "8A6Z", "icd10_code": "G40802", "icd10_title": "Other epilepsy, not intractable, without status epilepticus" }, { "from_icd11": "8A6Z", "icd10_code": "G40801", "icd10_title": "Other epilepsy, not intractable, with status epilepticus" }, { "from_icd11": "8A6Z", "icd10_code": "G40901", "icd10_title": "Epilepsy, unspecified, not intractable, with status epilepticus" }, { "from_icd11": "8A6Z", "icd10_code": "G4089", "icd10_title": "Other seizures" } ]	R561	Post traumatic seizures
A 62-year-old man with a history significant for chronic hepatitis B initially presented with hematuria and urine cytology positive for malignant cells. A CT urogram revealed a large ill-defined mass of the left posterolateral aspect of the urinary bladder with extramural extension, likely involvement of the seminal vesicles and the prostate, and bilateral enlargement of the external iliac chain lymph nodes. Partial transurethral resection of the tumor was performed, and pathology confirmed an invasive high-grade urothelial carcinoma with squamous differentiation with muscularis propria and lymphovascular invasion. A CT scan of the chest revealed several pulmonary nodules concerning for metastatic disease. An MRI of the liver did not reveal presence of metastatic disease or concern for hepatocellular carcinoma (HCC). Concurrent testing of serum alpha-fetoprotein (AFP) intended for HCC screening (for chronic, but inactive hepatitis B virus infection) showed a very high value of 934.7 ng/mL (normal < 5 ng/mL). He next received neoadjuvant gemcitabine/cisplatin followed by radical cystoprostatectomy and pelvic lymphadenectomy. Pathologic examination revealed a high-grade, poorly differentiated urothelial carcinoma with squamous differentiation with involvement of the left ureter, lymphovascular invasion, extension into perivesical fat, and involvement of 8 of 9 resected lymph nodes. Immunohistochemistry (IHC) of the primary tumor revealed strong staining for AFP , confirming tumor-derived AFP production. Serum AFP levels showed a steep decline following surgery, further validating this as a tumor marker, which was subsequently followed throughout the patient’s treatment course . Molecular testing of the primary tumor using a targeted next-generation sequencing assay (SNaPshot V1) revealed a single nucleotide variant in TP53 (Arg282Trp). FISH was consistent with amplification of the HER2 gene, but there were no targetable alterations. On surveillance CT of the abdomen and pelvis three months following surgery, the patient experienced a significant disease relapse, initially deferred initiation of chemotherapy, however, ultimately began treatment with pemetrexed for a total of three cycles . He continued to demonstrate rising serum AFP levels that correlated with progressive disease, now with palpable metastatic lesions in the head and neck area and continued visceral progression. Finally, he received paclitaxel monotherapy for two cycles, but did not tolerate this therapy well and continued to experience rapidly progressing disease. Histologic examination of his primary tumor revealed strong staining for PD-L1 . Due to his chronic hepatitis B infection, however, he was not eligible for clinical trials of immunotherapies. We therefore initiated therapy with the anti-PD-1 checkpoint inhibitor pembrolizumab (2 mg/kg every 3 weeks). Within 6 weeks, his AFP levels dropped from a peak level of ~ 3800 ng/mL to 42 ng/mL. This coincided with a dramatic clinical response, with reduction or resolution of all palpable metastatic lesions. After 4 doses of pembrolizumab, imaging revealed significant shrinkage of all metastatic lesions in the abdomen, including peritoneal masses and mesenteric lymphadenopathy . Notably, no lung nodules were appreciated on these imaging studies. The patient went on to receive a total of 16 cycles of pembrolizumab and sustained complete remission also reflected by normalized AFP levels, while only experiencing minimal adverse effects. However, after 12 cycles of pembrolizumab therapy, the patient was noted to have a left lower lobe lung nodule measuring ~ 7 mm in largest dimension, which increased to 10 mm on subsequent imaging. Due to concern for a metastatic escape lesion, and in the absence of other evidence of disease, the patient underwent wedge resection of this lung nodule. Of note, this lesion occurred in the absence of AFP elevation. Histopathologic assessment of the resection specimen demonstrated a benign intraparenchymal lymph node with no malignant cells seen . Since the resection, the patient received no additional systemic therapy and continues to have no evidence of disease for 18 months . Fig. 1 a H&E staining of the primary urothelial tumor (40X magnification). b Immunohistochemistry (IHC) reveals strong staining of AFP in the primary resection specimen (40X magnification). c Staining for PD-L1 shows very strong expression in more than 50% of cancer cells (40X magnification) Fig. 2 Levels of AFP over the entire clinical course of this patient correlated strongly with tumor burden. The patient had initially (indicated by ) presented with hematuria and concerning cytology, but was lost to follow up. Upon re-presentation with hematuria more than one year later, he underwent full work-up and was found to have urothelial transitional cell carcinoma. Despite receiving neo-adjuvant chemotherapy (cisplatin/gemcitabine), his AFP level strongly increased and he underwent surgery without further delay leading to a sharp decline in AFP levels. Within 3 months following surgery, his AFP level rose again, and after initially declining chemotherapy, he was started on pemetrexed, receiving three cycles total (indicated by short black arrows), however, AFP levels continued to rise. He then received paclitaxel for two cycles (indicated by arrow heads) without response. Ultimately, pembrolizumab was started (indicated by a green arrows) to which he had a sharp decline in AFP levels, significant response on imaging and dramatic clinical improvement. AFP levels normalized after the third infusion. After 12 cycles of pembrolizumab, he was noted to have an isolated lung nodule and underwent wedge resection (indicated by blue *). He remains off pembrolizumab with continued complete remission and normalized AFP levels Fig. 3 Representative coronal reconstruction from CT scans obtained throughout the clinical course. () indicate retroperitoneal lymphadenopathy and white arrows indicate peritoneal metastases. a CT scan from initial staging (prior to neo-adjuvant chemotherapy). b Progressive disease while on pemetrexed. c Further progression on paclitaxel. d Significant reduction in tumor burden following third dose of pembrolizumab Fig. 4 H&E staining of the resected lung lesion, which revealed an intrapulmonary lymph node/lymphoid aggregates (40X magnification, left; 200X magnification, right)	4.105469	0.970215	sec[1]/p[0]	en	0.999996	30208947	https://doi.org/10.1186/s40425-018-0394-y	[ "tumor", "pembrolizumab", "cycles", "resection", "metastatic", "staining", "this", "lung", "magnification", "initially" ]	[ { "code": "2F9Z", "title": "Neoplasms of unknown behaviour of unspecified site" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "2E6Z", "title": "Carcinoma in situ of unspecified site" }, { "code": "2F91.1", "title": "Neoplasms of unknown behaviour of trachea, bronchus or lung" }, { "code": "2F92", "title": "Neoplasms of unknown behaviour of skin" }, { "code": "6A80.5", "title": "Rapid cycling" }, { "code": "PA03", "title": "Unintentional land transport traffic event injuring a motor cyclist" }, { "code": "5C50.AZ", "title": "Disorders of urea cycle metabolism, unspecified" }, { "code": "PA23", "title": "Unintentional land transport event unknown whether traffic or nontraffic injuring a motor cyclist" }, { "code": "PA02", "title": "Unintentional land transport traffic event injuring a pedal cyclist" } ]	=== ICD-11 CODES FOUND === [2F9Z] Neoplasms of unknown behaviour of unspecified site Also known as: Neoplasms of unknown behaviour of unspecified site \| neoplasia \| neoplasm growth NOS \| tumour of unspecified site \| tumourlet of unspecified site [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [2E6Z] Carcinoma in situ of unspecified site Also known as: Carcinoma in situ of unspecified site \| carcinoma in situ of unspecified site \| carcinoma in situ NOS \| carcinoma in situ \| in situ neoplasm [2F91.1] Neoplasms of unknown behaviour of trachea, bronchus or lung Also known as: Neoplasms of unknown behaviour of trachea, bronchus or lung \| trachea, bronchus or lung tumour NOS \| Intravascular bronchial alveolar tumour of unspecified site \| Bronchial adenoma of unknown behaviour of unspecified site \| Intravascular bronchial alveolar tumour unknown behaviour of unspecified site [2F92] Neoplasms of unknown behaviour of skin Also known as: Neoplasms of unknown behaviour of skin \| skin tumour NOS [6A80.5] Rapid cycling Definition: In the context of bipolar type I or bipolar type II disorder, there has been a high frequency of mood episodes (at least four) over the past 12 months. There may be a switch from one polarity of mood to the other, or the mood episodes may be demarcated by a period of remission. In individuals with a high frequency of mood episodes, some may have a shorter duration than those usually observed in bipolar type I or bipolar type II disorder. In particular, depressive periods may only last several da Also known as: Rapid cycling [PA03] Unintentional land transport traffic event injuring a motor cyclist Also known as: Unintentional land transport traffic event injuring a motor cyclist \| motorcycle rider injured in transport accident \| unintentional land transport accident motorbike \| motorbike accident \| motorbike traffic accident Excludes: Unintentional land transport traffic event injuring an occupant of a low powered passenger vehicle [5C50.AZ] Disorders of urea cycle metabolism, unspecified Also known as: Disorders of urea cycle metabolism, unspecified \| Disorders of urea cycle metabolism \| disorder of urea cycle \| disorders of metabolism of ornithine, citrulline, argininosuccinic acid, arginine and ammonia \| ammonia metabolic disorder [PA23] Unintentional land transport event unknown whether traffic or nontraffic injuring a motor cyclist Also known as: Unintentional land transport event unknown whether traffic or nontraffic injuring a motor cyclist \| unintentional off-road crash injuring a motor cyclist, unknown whether on road \| motor bike crash NOS \| motor cycle crash NOS \| Motorcycle rider injured in collision with railway train or railway vehicle [PA02] Unintentional land transport traffic event injuring a pedal cyclist Also known as: Unintentional land transport traffic event injuring a pedal cyclist \| Pedal cyclist injured in collision with pedestrian or animal \| Pedal cyclist injured in collision with pedestrian or animal, person injured while boarding or alighting \| Pedal cyclist injured in collision with pedestrian or animal, driver injured in traffic accident \| Pedal cyclist injured in collision with pedestrian or animal, passenger injured in traffic accident === GRAPH WALKS === --- Walk 1 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2F92] Neoplasms of unknown behaviour of skin --- Walk 2 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2F90] Neoplasms of unknown behaviour of oral cavity or digestive organs --- Walk 3 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Localised adiposity Def: A condition characterised by accumulation of adipose tissue in specific regions of the body.... --CHILD--> [?] Fat pad Def: A condition characterised by a mass of closely packed adipose cells surrounded by fibrous tissue septa. When localised in the heel this condition may present with dull pain when pressure is applied.... --- Walk 4 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Enlarged lymph nodes Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes.... --CHILD--> [?] Localised lymph node enlargement --- Walk 5 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E60] Carcinoma in situ of oral cavity, oesophagus or stomach --- Walk 6 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs	[ "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2F92] Neoplasms of unknown behaviour of skin", "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2F90] Neoplasms of unknown behaviour of oral cavity or digestive organs", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Localised adiposity\n Def: A condition characterised by accumulation of adipose tissue in specific regions of the body....\n --CHILD--> [?] Fat pad\n Def: A condition characterised by a mass of closely packed adipose cells surrounded by fibrous tissue septa. When localised in the heel this condition may present with dull pain when pressure is applied....", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Enlarged lymph nodes\n Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes....\n --CHILD--> [?] Localised lymph node enlargement", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E60] Carcinoma in situ of oral cavity, oesophagus or stomach", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs" ]	2F9Z	Neoplasms of unknown behaviour of unspecified site	[ { "from_icd11": "2F9Z", "icd10_code": "D487", "icd10_title": "Neoplasm of uncertain behavior of other specified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D482", "icd10_title": "Neoplasm of uncertain behavior of peripheral nerves and autonomic nervous system" }, { "from_icd11": "2F9Z", "icd10_code": "D37-D48", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D377", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D48", "icd10_title": "Neoplasm of uncertain behavior of other and unspecified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D489", "icd10_title": "Neoplasm of uncertain behavior, unspecified" }, { "from_icd11": "ME61", "icd10_code": "R2240", "icd10_title": "Localized swelling, mass and lump, unspecified lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2232", "icd10_title": "Localized swelling, mass and lump, left upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2242", "icd10_title": "Localized swelling, mass and lump, left lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2231", "icd10_title": "Localized swelling, mass and lump, right upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2241", "icd10_title": "Localized swelling, mass and lump, right lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2233", "icd10_title": "Localized swelling, mass and lump, upper limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2243", "icd10_title": "Localized swelling, mass and lump, lower limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2230", "icd10_title": "Localized swelling, mass and lump, unspecified upper limb" }, { "from_icd11": "ME61", "icd10_code": "R220", "icd10_title": "Localized swelling, mass and lump, head" } ]	D487	Neoplasm of uncertain behavior of other specified sites
A 51-year-old morbidly obese African-American woman with a 6-month history of progressive voice change, dysphagia, and dyspnea on exertion presented to the clinic. Her past medical history was significant for hypertension, obstructive sleep apnea, gastrointestinal reflux disease, and generalized anxiety disorder. Her social history included a status of a former smoker and being married without any biological children. She reports no history of drinking or illicit drug use and has been employed at Tyson Foods as a Manager. No known history of exposure to environmental chemicals. The family history was significant for hypertensive status of both parents. At the time of office visit, her medication included escitalopram, 10 mg tablet orally daily; hydrocholorothiazide 25 mg tablet orally daily, omeprazole 40 mg capsule every morning before breakfast orally and ranitidine 300 mg orally daily. She has been reported to be on these medications for last 5 years. Blood pressure, pulse and temperature were 114/80, 92 and 36.3 °C (97.4 °F), respectively. The overall physical examination was unremarkable except for scattered, intermittent wheezing and prominent vasculature on the chest indicative of Superior Vena Cava (SVC) syndrome. The remaining details of the physical examination are provided in Additional file 1 : Table S1. The CBC showed WBC: 12,700/µl; RBC: 5.24 million/µl; Hb:13.3 g/dl; Hct: 41; MCV: 78, MCHC: 25 and platelets: 381,000/µl. The CMP showed glucose: 108 mg/dl; urea nitrogen: 15 mg/dl; creatinine: 0.69 mg/dl; sodium: 138 mmol/l; potassium: 4.2 mmol/l; chloride: 103 mmol/l; carbon dioxide: 26 mol/l and anion gap: 9. The urine analysis was unremarkable. On CT examination, a large, lobulated, heterogenous mass centered in the subcarinal region (10.5 × 6.3 × 6.0 cm) infiltrating the subcarinal and right infrahilar soft tissues as well as the azygoesophageal recess was evident . The mass was causing bronchus distortion and right lower lobe collapse. Abnormal air-fluid level in the esophageal lumen causing stasis of contents was evident due to obstruction from the mass. In addition, multifocal mediastinal lymphadenopathy was seen in the right paratracheal, pretracheal and precarinal lymph nodes. This mass demonstrated fluorodeoxyglucose (FDG) activity in the range of 6.5 to 8.5 . The immunoglobulin quantification showed increased serum IgM (663 mg/dl) with normal levels for IgG and IgA. There was also an increase in kappa light chain concentration to 28.7 mg/L with an increased kappa: lambda ratio of 4.35 by serum protein electrophoresis (SPEP) analysis . The patient underwent subcarinal lymph node biopsy via endobronchoscopy procedure. On the day of brief hospitalization for this procedure patient continued her routine medications without any changes. The flow cytometry analysis was normal . Microscopic examination of the biopsy specimen revealed a lymph node replaced by sheets of lesional cells with oval to round eccentric nuclei and gray-pink cytoplasm with readily evident Dutcher bodies . The immunohistochemical stains performed on the biopsy specimen showed that the lesional cells demonstrated diffuse positive staining for CD138, CD79a, CD56, and MUM1 while negative staining for CD20, Pax5, CD5, CD3 with a high Ki67 proliferation index of 60% . The immunohistochemical stains for OCT2, CD10, CD30, BCL-2, EBER, BCL 1, CD25, CD45, and CD117 were negative in lesional cells (not shown). These lesional cells demonstrated a kappa-restricted IgM immunophenotype . These findings prompted the diagnosis of a clonal lymphoid lineage with plasmacytic differentiation with a differential diagnosis for B-cell lymphoma (i.e. lymphoplasmacytic differentiation vs marginal zone lymphoma) with an extreme level of plasmacytic differentiation and primary de novo IgM plasma cell neoplasm. Cytogenetic studies, karyotyping, FISH studies for PCN, and NGS for lymphoma panel were then performed to further characterized the mass and establish the final diagnosis. The FISH studies revealed an abnormal tetraploid clone with gain of 1q21 and borderline loss of chromosome 17 ( TP53 ), while negative for t(4;14), t(11;14), and t(14;16). NGS analysis detected no disease-associated variants or variants of uncertain significance including MYD88 and CXCR4. The final diagnosis was rendered as IgM solitary extramedullary plasmacytoma. The subsequent bone marrow biopsy was normal without any involvement of bone marrow by plasma cell neoplasm. Radiation therapy to the mediastinum (50 Gy) with reassessment after 3 months of treatment demonstrated a reduction in mass. Post-radiation SPEP showed a decrease in IgM levels to 82 mg/dl and free light chain kappa to 10.7 mg/dl with a normal kappa: lambda ratio of 1.51. Three years after the radiation, patient is in complete remission with no interval recurrence with 3 years of follow-up. Fig. 1 Imaging of the mediastinum mass. A Transverse computed tomography image demonstrating the mass (arrow). B , C Transverse and coronal plane positron emission tomography scan images highlighting the mediastinal mass obstructing the esophagus (arrows) Fig. 2 Serum protein electrophoresis (SPEP) and flow cytometry analysis of mass. A , B The SPEP shows an increase in gamma globulins correlating with an increase in kappa-predominant immunoglobulin M. C Flow cytometry analysis of the mediastinal mass, however, failed to detect any immunophenotypic aberrancy Fig. 3 Microscopic description and immunophenotype of mediastinum mass. A , B The Hematoxylin and Eosin examination of mass showed sheets of lesional cells with oval to round eccentric nuclei and gray-pink cytoplasm (red arrows) with readily evident Ducher bodies (black arrows). The immunohistochemistry performed on the biopsy specimen demonstrated positive staining for CD138 ( C ), CD 79a ( D ), CD56 ( E ) and MUM1 ( F ) confirming the plasmacytic immunophenotype Fig. 4 Immunohistochemistry to assess the immunophenotype of mediastinal mass. A , B , D , E The lesional cells are negative for CD20, Pax5, CD3, and CD5 with a proliferative index, assessed with Ki67, of 60% ( C ) Fig. 5 Immunohistochemistry to assess the immunophenotype of mediastinal mass. A , B The lesional cells demonstrate kappa light chain overexpression alongside increased immunoglobulin M, immunoglobulins with normal immunoglobulin G and immunoglobulin A expression ( C , D , and E )	4.09375	0.968262	sec[1]/p[0]	en	0.999998	37749639	https://doi.org/10.1186/s13256-023-04082-x	[ "kappa", "lesional", "cells", "mediastinal", "immunoglobulin", "biopsy", "immunophenotype", "orally", "evident", "spep" ]	[ { "code": "4A01.0Y", "title": "Other specified immunodeficiencies with predominantly antibody defects" }, { "code": "FA5Z", "title": "Arthropathies, unspecified" }, { "code": "FC0Z", "title": "Diseases of the musculoskeletal system or connective tissue, unspecified" }, { "code": "ME60.Z", "title": "Skin lesion of unspecified nature" }, { "code": "MD41", "title": "Clinical findings on diagnostic imaging of lung" }, { "code": "GC2Z&XA6KU8", "title": "Disease of kidney, not elsewhere classified" }, { "code": "MF9Y", "title": "Other specified clinical findings on examination of urine, without diagnosis" }, { "code": "5C56.20", "title": "Mucolipidosis" }, { "code": "3A51.1", "title": "Sickle cell disease without crisis" }, { "code": "9A96.3", "title": "Primary anterior uveitis" } ]	=== ICD-11 CODES FOUND === [4A01.0Y] Other specified immunodeficiencies with predominantly antibody defects Also known as: Other specified immunodeficiencies with predominantly antibody defects \| Common variable immunodeficiency \| B-cell activating factor receptor deficiency \| BAFF - [ B-cell activating factor] receptor deficiency \| Cluster of differentiation 19 deficiency [FA5Z] Arthropathies, unspecified Also known as: Arthropathies, unspecified \| Disorders affecting predominantly peripheral joints \| Disorders affecting predominantly peripheral limb joints \| arthropathy NOS \| arthropathic [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified Also known as: Diseases of the musculoskeletal system or connective tissue, unspecified \| bone disease NOS \| bone disorder NOS \| bone lesion NOS \| musculoskeletal complications NOS [ME60.Z] Skin lesion of unspecified nature Also known as: Skin lesion of unspecified nature \| Skin lesion of uncertain or unspecified nature \| Skin lesion without established diagnosis \| Pigmented skin lesion of unspecified nature \| Ulcer of skin of unspecified nature [MD41] Clinical findings on diagnostic imaging of lung Definition: Clinical findings on diagnostic imaging of lung is findings on diagnostic imaging of the lung which don't appear in normal status of the body. Diagnostic imaging refers to technologies that doctors use to look inside body for clues about a medical condition. X-rays, CT scans, nuclear medicine scans, MRI scans and ultrasound are all types of diagnostic imaging. Also known as: Clinical findings on diagnostic imaging of lung \| abnormal diagnostic imaging of lung \| Hyperinflation of lung \| Lung mass \| Pulmonary lobe mass [MF9Y] Other specified clinical findings on examination of urine, without diagnosis Also known as: Other specified clinical findings on examination of urine, without diagnosis \| Methaemoglobinuria \| Other and unspecified abnormal findings in urine \| Calciuria \| Cells and casts in urine [5C56.20] Mucolipidosis Also known as: Mucolipidosis \| Mucolipidosis type 3 \| Pseudo-Hurler polydystrophy \| Pseudo-Hurler disease \| Mucolipidosis type 2 Excludes: Sialidosis (mucolipidosis type 1) [3A51.1] Sickle cell disease without crisis Definition: A disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may present with fatigue, shortness of breath, dizziness, headaches, pallor of skin or mucous membranes, and jaundice. This disorder is confirmed by identification of HbS mutation by genetic testing. Also known as: Sickle cell disease without crisis \| Hb-SS disease without crisis \| HbSS without crisis \| Sickle-cell anaemia without crisis \| SCD - [sickle cell disease] [9A96.3] Primary anterior uveitis Definition: This refers to primary inflammation of the uvea. The uvea consists of the middle, pigmented, vascular structures of the eye and includes the iris, ciliary body, and choroid. Also known as: Primary anterior uveitis \| anterior chamber cell === GRAPH WALKS === --- Walk 1 --- [4A01.0Y] Other specified immunodeficiencies with predominantly antibody defects --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects... --CHILD--> [4A01.02] Specific antibody deficiency with normal immunoglobulin concentrations or normal number of B cells --- Walk 2 --- [4A01.0Y] Other specified immunodeficiencies with predominantly antibody defects --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects... --PARENT--> [4A01] Primary immunodeficiencies due to disorders of adaptive immunity --- Walk 3 --- [FA5Z] Arthropathies, unspecified --PARENT--> [?] Arthropathies --CHILD--> [?] Osteoarthritis Def: Osteoarthritis (OA) can be defined as a group of distinct, but overlapping diseases, which may have different etiologies, but similar biological, morphological, and clinical outcomes affecting the art... --- Walk 4 --- [FA5Z] Arthropathies, unspecified --PARENT--> [?] Arthropathies --CHILD--> [?] Osteoarthritis Def: Osteoarthritis (OA) can be defined as a group of distinct, but overlapping diseases, which may have different etiologies, but similar biological, morphological, and clinical outcomes affecting the art... --- Walk 5 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes Def: !markdown In the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre... --- Walk 6 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes Def: !markdown In the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre...	[ "[4A01.0Y] Other specified immunodeficiencies with predominantly antibody defects\n --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects\n Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects...\n --CHILD--> [4A01.02] Specific antibody deficiency with normal immunoglobulin concentrations or normal number of B cells", "[4A01.0Y] Other specified immunodeficiencies with predominantly antibody defects\n --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects\n Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects...\n --PARENT--> [4A01] Primary immunodeficiencies due to disorders of adaptive immunity", "[FA5Z] Arthropathies, unspecified\n --PARENT--> [?] Arthropathies\n --CHILD--> [?] Osteoarthritis\n Def: Osteoarthritis (OA) can be defined as a group of distinct, but overlapping diseases, which may have different etiologies, but similar biological, morphological, and clinical outcomes affecting the art...", "[FA5Z] Arthropathies, unspecified\n --PARENT--> [?] Arthropathies\n --CHILD--> [?] Osteoarthritis\n Def: Osteoarthritis (OA) can be defined as a group of distinct, but overlapping diseases, which may have different etiologies, but similar biological, morphological, and clinical outcomes affecting the art...", "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes\n Def: !markdown\nIn the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre...", "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes\n Def: !markdown\nIn the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre..." ]	4A01.0Y	Other specified immunodeficiencies with predominantly antibody defects	[ { "from_icd11": "FA5Z", "icd10_code": "M00-M25", "icd10_title": "" }, { "from_icd11": "FC0Z", "icd10_code": "XIII", "icd10_title": "" }, { "from_icd11": "ME60.Z", "icd10_code": "L989", "icd10_title": "Disorder of the skin and subcutaneous tissue, unspecified" }, { "from_icd11": "MD41", "icd10_code": "R911", "icd10_title": "Solitary pulmonary nodule" }, { "from_icd11": "MD41", "icd10_code": "R91", "icd10_title": "Abnormal findings on diagnostic imaging of lung" }, { "from_icd11": "3A51.1", "icd10_code": "D571", "icd10_title": "Sickle-cell disease without crisis" } ]	M00-M25
A 6-year-old boy presented to an emergency room with subacute abdominal fullness and acute abdominal pain. Computed tomography (CT) showed multiple solid tumors in his pelvis, with a thickened omentum and ascites . Because a malignant tumor with peritoneal dissemination was suspected, he was remitted to our hospital. He underwent biopsy and was diagnosed with retroperitoneal rhabdomyosarcoma-embryonal type (positive for myogenin, myogenic differentiation 1, and desmin; and negative for α-smooth muscle actin and leukocyte common antigen). The patient was diagnosed with stage IV disease due to dissemination. However, considering that he was less than 10 years old and the histology demonstrated good prognosis, he was classified as an intermediate risk by IRS-V preoperative system. He was treated using the COG D9803 VAC protocol . The schedule of chemotherapy, surgery, and radiotherapy is shown in Fig. 2 . G4 hematotoxicity (leukopenia and thrombocytopenia) was observed (CTCAE version 5) after VAC, before the administration of radiotherapy, but had recovered quickly without delaying the chemo-schedule. He responded extremely well to the VAC, and after the first course of VAC, his ascites almost disappeared. By the 13th week, just before the surgery, CT showed a 2-cm residual tumor in his pelvis. He underwent tumor resection during the 14th week. However, innumerable nodules that were suspected to be peritoneal disseminated tumors were observed in the abdominopelvic region and resecting all of them was not feasible. The nodules were conspicuous in the pelvis; thus, the surgeon randomly picked three nodules for pathological diagnosis. Within the 2-cm tumor, observed via CT prior to the surgery, there was 5% residual tumor, but the tumor was resected without any marginal tumor cells. The three nodules that were suspected to be peritoneal dissemination and were resected for pathological analysis had no residual tumor cells. We concluded that the patient had achieved complete resection. However, there is a high chance of recurrence in the pelvis and abdomen. Therefore, we planned and delivered 30-Gy/20-fr whole abdominopelvic radiotherapy and a 6 Gy/4-fr sequential boost to the pelvis with 10-MV photons. For radiotherapy treatment planning, Eclipse version 13.6 (Varian Medical Systems, Palo Alto, CA) was used. The patient was placed in the supine position with vacuum fixation cushions and a thermoplastic body shell to immobilize his body and suppress breathing motions. He could not follow our instruction and hold his breath, thus CT was performed without holding the breath. The clinical target volume (CTV) for whole abdominopelvic radiotherapy consisted of the peritoneal cavity, excluding the kidney and liver and CTV for sequential pelvic boost consisted of the pelvic cavity caudal to the kidney. The planning target volume (PTV) was obtained by adding 5 mm to the CTV in all directions. The planning CT was obtained without holding breath, but we evaluated the dose distribution in both the inspiration and exhalation phases and calculated the CT dose in both phases to ensure a sufficient dose distribution for both phases. We adopted the volumetric modulated arc therapy (VMAT) technique for the initial and boost IMRT treatments plan. The dose-volume histogram (DVH) was as shown in Fig. 3 . The dose covering 95% of liver, right kidney, and left kidney were 16Gy, 12Gy, and 12Gy. The percent of the right and left kidney volume receiving at lease 20Gy were 31 and 27%, respectively. Clinac ix (Varian Medical Systems, Palo Alto, CA) was used to carry out radiotherapy. We used kilovoltage X-ray imaging system first to apply the bone-match, then achieved cone beam CT image to check if the diaphragm was included in the PTV. During and after the radiotherapy, G1 dermatitis, G2 enteritis and cystitis, and G4 hematotoxicity (leukopenia and thrombocytopenia) were observed (CTCAE version 5). Leukopenia and thrombocytopenia were observed both before and after the radiotherapy, but was extremely severe, particularly after radiotherapy; as a result, the patient required multiple platelet transfusions . Just after irradiation, the patient’s total bilirubin level transiently increased but eventually decreased to a normal level without any treatment. Transfusion dependence lasted for 1 month after the entire treatment was completed. However, eventually, the patient no longer required transfusion and was discharged. Considering the late adverse event, the patient experienced G1 cystitis and hepatic damage (CTCAE version 5), but did not experience kidney toxicity or radiation pneumonitis. He was in good health with no recurrence for 3 years. In terms of growth, his height increased from 121.7 to 132.4 cm, and his weight increased from 23.5 to 36.1 kg during 3 years of follow-up. The average height and weight for Japanese boys of his age are 119.6 ± 5.1 cm and 23.1 ± 4.1 kg before the treatment and 134.5 ± 5.8 cm and 31.9 ± 7 kg after 3 years; thus, his height and weight are within the 1SD for Japanese boys of the same age. The fusion of images of the pelvis to the femur before treatment and 2 years after treatment is shown in Fig. 4 . The volume of his left iliac bone, which was within the irradiating field, increased from 66.1 to 81.1 cm 3 (23% increase), whereas that of the femur, which was outside of the irradiating field, increased from 154.7 to 202.5 cm 3 (31% increase). Fig. 1 Computed tomography image obtained upon diagnosis showing multiple solid tumors in the pelvis, with a thickened omentum and ascites Fig. 2 Therapeutic schedule and hematotoxicity. Platelet transfusion was performed 56 times within 180 days (every 2–4 days) after the completion of radiotherapy, and the patient withdrew from transfusion dependence 1 month after the last chemotherapy treatment Fig. 3 Intensity-modulated radiation therapy plan and dose-volume histogram (DVH): whole abdominopelvic irradiation (WAPI) of 30 Gy/20 fr and sequential pelvic boost (PB) of 6 Gy/4 fr (right). Images are color washed to > 90% dose with a global dose maximum < 109% of the dose for WAPI and < 108% of the dose for PB Fig. 4 The fusion of images of the pelvis to the femur before treatment and 2 years after treatment. The volume of the left iliac bone increased from 66.1 to 81.1 cm 3 (23% increase) and that of the femur increased from 154.7 to 202.5 cm 3 (31% increase)	4.007813	0.965332	sec[1]/p[0]	en	0.999995	31307511	https://doi.org/10.1186/s13014-019-1333-x	[ "radiotherapy", "pelvis", "tumor", "that", "kidney", "without", "peritoneal", "however", "version", "nodules" ]	[ { "code": "QB96", "title": "Contact with health services for radiotherapy session" }, { "code": "EL63", "title": "Radionecrosis of skin due to therapeutic ionizing irradiation" }, { "code": "EL61", "title": "Chronic radiodermatitis following radiotherapy" }, { "code": "PK81.C", "title": "Radiation therapy associated with injury or harm in therapeutic use" }, { "code": "2C3Y", "title": "Other specified malignant neoplasms of skin" }, { "code": "LD2F.1Y", "title": "Other specified syndromes with multiple structural anomalies, not of environmental origin" }, { "code": "FC00.3", "title": "Acquired deformity of pelvis" }, { "code": "MD82", "title": "Intra-abdominal or pelvic swelling, mass or lump" }, { "code": "BD75.3", "title": "Pelvic varices" }, { "code": "NB9Z", "title": "Injuries to the abdomen, lower back, lumbar spine or pelvis, unspecified" } ]	=== ICD-11 CODES FOUND === [QB96] Contact with health services for radiotherapy session Also known as: Contact with health services for radiotherapy session \| admission for radiotherapy [EL63] Radionecrosis of skin due to therapeutic ionizing irradiation Definition: Necrosis and ulceration of skin attributable to radiotherapy Also known as: Radionecrosis of skin due to therapeutic ionizing irradiation \| Ulceration of skin due to radiotherapy \| Radionecrosis of skin due to radiotherapy [EL61] Chronic radiodermatitis following radiotherapy Definition: The late cutaneous sequelae of the therapeutic use of ionising radiation. It may take five to ten years to develop and is characterised by cutaneous atrophy, fibrosis, dyspigmentation, alopecia and telangiectasia with associated damage to underlying subcutaneous fat. Also known as: Chronic radiodermatitis following radiotherapy \| Late radiation reaction \| Telangiectasia following radiotherapy \| Poikiloderma following radiotherapy \| Radiation keratosis following radiotherapy [PK81.C] Radiation therapy associated with injury or harm in therapeutic use Also known as: Radiation therapy associated with injury or harm in therapeutic use \| complication during or following radiotherapy \| radiotherapy associated with abnormal reaction of the patient, or of later complication, without mention of misadventure at the time of the procedure \| therapeutic ionizing radiation associated with injury or harm \| radiotherapy associated with injury or harm in therapeutic use Excludes: Circumstances associated with a surgical or other medical procedure influencing the episode of care, without injury or harm [2C3Y] Other specified malignant neoplasms of skin Also known as: Other specified malignant neoplasms of skin \| Malignant neoplasm of eyelid NOS \| Malignant pilonidal cyst \| Radiotherapy-induced skin malignancy \| Cutaneous carcinoma [LD2F.1Y] Other specified syndromes with multiple structural anomalies, not of environmental origin Also known as: Other specified syndromes with multiple structural anomalies, not of environmental origin \| 46,XX disorder of sex development - anorectal anomalies \| 46,XX DSD - anorectal anomalies \| Aarskog-Scott syndrome \| Aarskog syndrome [FC00.3] Acquired deformity of pelvis Also known as: Acquired deformity of pelvis \| deformity of pelvis \| pelvic deformity \| ischium deformity \| ilium deformity Excludes: Maternal care for disproportion \| Obstructed labour due to maternal pelvic abnormality \| Obstructed labour due to deformed pelvis [MD82] Intra-abdominal or pelvic swelling, mass or lump Definition: This refers to the presence of abdominal or pelvic wall swelling, mass or tumour in the abdominal and pelvic regions. These mass or tumours can be recognised by visual examination and/or palpation. Also known as: Intra-abdominal or pelvic swelling, mass or lump \| Abdominal mass without further specification \| mass in abdomen \| intra-abdominal lump \| intra-abdominal mass Excludes: Abdominal distension \| Ascites [BD75.3] Pelvic varices Definition: The presence of dilated and incompetent ovarian and pelvic veins in women. These may cause no symptoms but may be associated with chronic pelvic pain (pelvic congestion syndrome) or with externally apparent vulvovaginal varicosities. Also known as: Pelvic varices \| varix of pelvis \| pelvic varicose vein \| Bladder varices \| Varix of bladder [NB9Z] Injuries to the abdomen, lower back, lumbar spine or pelvis, unspecified Also known as: Injuries to the abdomen, lower back, lumbar spine or pelvis, unspecified \| Injury of abdomen \| abdominal injury NOS \| injury of abdomen NOS \| Injuries to external genital organ === GRAPH WALKS === --- Walk 1 --- [QB96] Contact with health services for radiotherapy session --PARENT--> [?] Contact with health services for nonsurgical interventions not involving devices --CHILD--> [QB90] Contact with health services for ear piercing --- Walk 2 --- [QB96] Contact with health services for radiotherapy session --PARENT--> [?] Contact with health services for nonsurgical interventions not involving devices --CHILD--> [QB90] Contact with health services for ear piercing --- Walk 3 --- [EL63] Radionecrosis of skin due to therapeutic ionizing irradiation Def: Necrosis and ulceration of skin attributable to radiotherapy... --PARENT--> [?] Adverse cutaneous effects of therapeutic ionizing irradiation --CHILD--> [EL60] Acute radiodermatitis following radiotherapy Def: The reaction of the skin, and in particular the epidermis, to acute exposure to ionising radiation directed at the skin for therapeutic purposes. It manifests as inflammation, erosion and crusting.... --- Walk 4 --- [EL63] Radionecrosis of skin due to therapeutic ionizing irradiation Def: Necrosis and ulceration of skin attributable to radiotherapy... --PARENT--> [?] Adverse cutaneous effects of therapeutic ionizing irradiation --RELATED_TO--> [?] Radiotherapy-induced xerostomia Def: A reduction of salivary flow rate due to medication used by radiotherapy treatments... --- Walk 5 --- [EL61] Chronic radiodermatitis following radiotherapy Def: The late cutaneous sequelae of the therapeutic use of ionising radiation. It may take five to ten years to develop and is characterised by cutaneous atrophy, fibrosis, dyspigmentation, alopecia and te... --PARENT--> [?] Adverse cutaneous effects of therapeutic ionizing irradiation --CHILD--> [EL60] Acute radiodermatitis following radiotherapy Def: The reaction of the skin, and in particular the epidermis, to acute exposure to ionising radiation directed at the skin for therapeutic purposes. It manifests as inflammation, erosion and crusting.... --- Walk 6 --- [EL61] Chronic radiodermatitis following radiotherapy Def: The late cutaneous sequelae of the therapeutic use of ionising radiation. It may take five to ten years to develop and is characterised by cutaneous atrophy, fibrosis, dyspigmentation, alopecia and te... --PARENT--> [?] Adverse cutaneous effects of therapeutic ionizing irradiation --RELATED_TO--> [?] Scarring alopecia following radiotherapy Def: Permanent hair loss due to the late effects of therapeutic ionizing radiation...	[ "[QB96] Contact with health services for radiotherapy session\n --PARENT--> [?] Contact with health services for nonsurgical interventions not involving devices\n --CHILD--> [QB90] Contact with health services for ear piercing", "[QB96] Contact with health services for radiotherapy session\n --PARENT--> [?] Contact with health services for nonsurgical interventions not involving devices\n --CHILD--> [QB90] Contact with health services for ear piercing", "[EL63] Radionecrosis of skin due to therapeutic ionizing irradiation\n Def: Necrosis and ulceration of skin attributable to radiotherapy...\n --PARENT--> [?] Adverse cutaneous effects of therapeutic ionizing irradiation\n --CHILD--> [EL60] Acute radiodermatitis following radiotherapy\n Def: The reaction of the skin, and in particular the epidermis, to acute exposure to ionising radiation directed at the skin for therapeutic purposes. It manifests as inflammation, erosion and crusting....", "[EL63] Radionecrosis of skin due to therapeutic ionizing irradiation\n Def: Necrosis and ulceration of skin attributable to radiotherapy...\n --PARENT--> [?] Adverse cutaneous effects of therapeutic ionizing irradiation\n --RELATED_TO--> [?] Radiotherapy-induced xerostomia\n Def: A reduction of salivary flow rate due to medication used by radiotherapy treatments...", "[EL61] Chronic radiodermatitis following radiotherapy\n Def: The late cutaneous sequelae of the therapeutic use of ionising radiation. It may take five to ten years to develop and is characterised by cutaneous atrophy, fibrosis, dyspigmentation, alopecia and te...\n --PARENT--> [?] Adverse cutaneous effects of therapeutic ionizing irradiation\n --CHILD--> [EL60] Acute radiodermatitis following radiotherapy\n Def: The reaction of the skin, and in particular the epidermis, to acute exposure to ionising radiation directed at the skin for therapeutic purposes. It manifests as inflammation, erosion and crusting....", "[EL61] Chronic radiodermatitis following radiotherapy\n Def: The late cutaneous sequelae of the therapeutic use of ionising radiation. It may take five to ten years to develop and is characterised by cutaneous atrophy, fibrosis, dyspigmentation, alopecia and te...\n --PARENT--> [?] Adverse cutaneous effects of therapeutic ionizing irradiation\n --RELATED_TO--> [?] Scarring alopecia following radiotherapy\n Def: Permanent hair loss due to the late effects of therapeutic ionizing radiation..." ]	QB96	Contact with health services for radiotherapy session	[ { "from_icd11": "QB96", "icd10_code": "Z510", "icd10_title": "Encounter for antineoplastic radiation therapy" }, { "from_icd11": "QB96", "icd10_code": "Z51", "icd10_title": "Encounter for other aftercare and medical care" }, { "from_icd11": "EL63", "icd10_code": "L598", "icd10_title": "Other specified disorders of the skin and subcutaneous tissue related to radiation" }, { "from_icd11": "EL61", "icd10_code": "L581", "icd10_title": "Chronic radiodermatitis" }, { "from_icd11": "PK81.C", "icd10_code": "Y842", "icd10_title": "Radiological procedure and radiotherapy as the cause of abnormal reaction of the patient, or of later complication, without mention of misadventure at the time of the procedure" }, { "from_icd11": "FC00.3", "icd10_code": "M955", "icd10_title": "Acquired deformity of pelvis" }, { "from_icd11": "MD82", "icd10_code": "R1900", "icd10_title": "Intra-abdominal and pelvic swelling, mass and lump, unspecified site" }, { "from_icd11": "MD82", "icd10_code": "R1909", "icd10_title": "Other intra-abdominal and pelvic swelling, mass and lump" }, { "from_icd11": "MD82", "icd10_code": "R1902", "icd10_title": "Left upper quadrant abdominal swelling, mass and lump" }, { "from_icd11": "MD82", "icd10_code": "R1904", "icd10_title": "Left lower quadrant abdominal swelling, mass and lump" }, { "from_icd11": "MD82", "icd10_code": "R1903", "icd10_title": "Right lower quadrant abdominal swelling, mass and lump" }, { "from_icd11": "MD82", "icd10_code": "R1901", "icd10_title": "Right upper quadrant abdominal swelling, mass and lump" }, { "from_icd11": "MD82", "icd10_code": "R1907", "icd10_title": "Generalized intra-abdominal and pelvic swelling, mass and lump" }, { "from_icd11": "MD82", "icd10_code": "R1906", "icd10_title": "Epigastric swelling, mass or lump" }, { "from_icd11": "MD82", "icd10_code": "R190", "icd10_title": "Intra-abdominal and pelvic swelling, mass and lump" } ]	Z510	Encounter for antineoplastic radiation therapy
The present case report should also be considered a warning in terms of the possible risk of MRONJ occurrence, both for oncologists who prescribe Osimertinib and for oral and maxillo-facial surgeons . A bilateral maxillary II stage necrosis was reported during therapy with Osimertinib. The Denosumab drug holiday in cancer patients is still under debate, and there is no general consensus about their management prior to any surgical procedure . Denosumab’s mean half-life is 25.4 days because RANK-L inhibitors do not bind bone, unlike bisphosphonates. Non-significant plasmatic levels of Denosumab are found after 4 months , so this time could indeed represent a sufficient drug-free period to prevent tooth extraction-related necrosis (in surgically triggered MRONJ). Nevertheless, there are some studies that do not suggest a Denosumab drug holiday in cancer patients because of the increased risk of disease progression . On the other hand, there is a case series that suggests Denosumab suspension 12 months prior to any surgical procedures . Thus, the authors considered the 2022 AAOMS position paper as guidelines, which suggests a Denosumab drug holiday of 3–4 months, due to the insignificant levels of osteoclast inhibition after that period . In particular, the onset of this maxillary MRONJ was 15 months after Denosumab suspension, so we believe that Denosumab can be excluded as the etiopathogenetic cause, which is mostly related to the exclusive Osimertinib regimen or eventually a drug combination of Denosumab (albeit suspended following the AAOMS’s suggestions) and Osimertinib. The etiopathogenetic cause of MRONJ in the current case is still unknown because it could either be surgically triggered or removable prosthesis-triggered. Nevertheless, the patient underwent tooth extractions and removable-denture application both in the upper and lower arches but developed MRONJ only in the upper, so attributing it to a single cause is challenging. Such a consideration arises as, besides teeth extractions (considered the most common risk factor), removable denture-related trauma could certainly be related to MRONJ occurrence, as already reported in the literature . After this, to the best of our knowledge, MRONJ during Osimertinib therapy has only been described in one similar case report by Wang et al. in 2022 , who reported a maxillary MRONJ after tooth extraction in a 69-year-old female patient affected by NSCLC, who had been taking Osimertinib for the last 4 years. Then, we reported our bilateral stage 2 MRONJ during the same therapy. Overall, there are three cases of MRONJ during anti-EGFR therapy, sustained by the timing and the anti-vascular and bone alteration properties of these drugs, which we exposed previously. The rare occurrence, however, does not exclude the possibility of MRONJ developing in these patients. Consequently, the lack of data in the literature about it leads there being no evidence of any consensus about these patients’ management or the need for an Osimertinib holiday period. Usually, in the management of patients at risk of MRONJ, a drug holiday is always suggested prior to surgical procedures, but it must be conducted according to the oncologist. However, in this case, the patient was not allowed to suspend Osimertinib by the oncologist, because the risk of cancer progression during their suspension was higher than the benefits, and there are no data available in the current literature about the management of MRONJ during Osimertinib therapy. Additionally, as for therapy, our report provides evidence that a conservative surgery, preceded by the conventional medical therapy (three cycles therapy with Ceftriaxone (1 g daily, IM) and metronidazole (250 mg, twice a day per os for 7 days), with a 10-day drug-free period between each cycle, together with a local antimicrobial rinse of 0.2% Chlorhexidine), was resolutive. It is important to underline this, as a consensus about the therapeutical management of MRONJ, which is generally classified as conservative (non-operative) and surgical, is still lacking. Also, the same antibiotic use (type, dosage, and duration) in MRONJ patients lacks evidence; generally, a broad-spectrum antibiotic, mainly a combination of amoxicillin/clavulanic acid (effective against Gram-negative and β-lactamase-resistant Gram-positive bacteria) and metronidazole (effective against Gram-positive cocci and anaerobic), is suggested for infection control along with antiseptic rinses . On the basis of our experience, a medical therapy as performed in the current case (the combination of Ceftriaxone and metronidazole) provides excellent control of infection in the pre-operative stage, leading to a good mucosal inflammation reduction (mandatory for closure of the surgical site) along with perilesional bone remodeling with necrosis delimitation (helpful to limit perilesional bone extension during surgery) . As said before, our case is consistent with Wang et al.’s report and supports their hypothesis. Considering that NSCLC represents 81% of all lung cancers and lung cancer is the second most diagnosed cancer worldwide , Osimertinib-related MRONJ cases are expected to increase in incidence. This alert must be spread and shared with dentists and oncologists in order to discuss together the management of these patients to possibly determine an Osimertinib drug holiday before surgical treatments of jaws, considering that there are no data about TKI suspensions before oral surgery to prevent MRONJ. Moreover, we suggest adding Osimertinib to the MRONJ-related drug list in order to follow the surgical procedures reported by the AAOMS that are useful to reduce the risk of MRONJ, such as primary wound closure, preventive antibiotic administration, and adjunctive therapies such as low-level laser therapy and chlorhexidine mouthwash . There are several limitations of this study: the combination with Denosumab could be considered a bias, and the unknown etiopathogenetic cause (surgical- or denture-triggered) and lack of studies that directly relate MRONJ to Osimertinib therapy make it impossible to draw firm conclusions. Further studies are needed to confirm the hypothesis that Osimertinib monotherapy can cause MRONJ. Finally, a routine oral examination and monitoring should be advised and suggested in these patients, especially when oral surgery procedures should be planned.	4.339844	0.820801	sec[2]/sec[5]/p[0]	en	0.999995	38391832	https://doi.org/10.3390/healthcare12040457	[ "mronj", "osimertinib", "denosumab", "that", "drug", "patients", "this", "risk", "holiday", "about" ]	[ { "code": "FB81.2", "title": "Drug-induced osteonecrosis" }, { "code": "8A80.Z", "title": "Migraine, unspecified" }, { "code": "QA76", "title": "Medication or substance that is known to be an allergen without injury or harm" }, { "code": "PL13.6", "title": "Medication or substance that is known to be an allergen, as mode of injury or harm" }, { "code": "9C40.A0", "title": "Papilloedema" }, { "code": "PA6Z", "title": "Unintentional fall from unspecified height" }, { "code": "QE11.Z", "title": "Hazardous drug use, unspecified" }, { "code": "6C4G.2Z", "title": "Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified" }, { "code": "NE60", "title": "Harmful effects of drugs, medicaments or biological substances, not elsewhere classified" }, { "code": "4A85.0Z", "title": "Drug hypersensitivity of unspecified type" } ]	=== ICD-11 CODES FOUND === [FB81.2] Drug-induced osteonecrosis Definition: Alteration of the normal structure of orofacial tissues resulting from medicinal substances acting locally or systemically. Also known as: Drug-induced osteonecrosis \| osteonecrosis due to drugs, site unspecified \| Osteonecrosis due to chemical burn of oral mucosa \| medicament-induced osteonecrosis \| Bisphosphonates induced osteonecrosis Includes: Osteonecrosis due to chemical burn of oral mucosa [8A80.Z] Migraine, unspecified Also known as: Migraine, unspecified \| Migraine [QA76] Medication or substance that is known to be an allergen without injury or harm Definition: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm. Also known as: Medication or substance that is known to be an allergen without injury or harm Excludes: Medication or substance that is known to be an allergen, as mode of injury or harm [PL13.6] Medication or substance that is known to be an allergen, as mode of injury or harm Also known as: Medication or substance that is known to be an allergen, as mode of injury or harm \| medication error relating to known allergy to drug or substance \| adverse drug event relating to known allergy to drug or substance [9C40.A0] Papilloedema Definition: Optic disc swelling that results from increased intracranial pressure Also known as: Papilloedema \| Optic disc swelling that results from increased intracranial pressure Includes: Optic disc swelling that results from increased intracranial pressure [PA6Z] Unintentional fall from unspecified height Also known as: Unintentional fall from unspecified height \| fall NOS \| accidental fall \| fall causing injury that resulted in death \| fell NOS [QE11.Z] Hazardous drug use, unspecified Also known as: Hazardous drug use, unspecified \| Hazardous drug use \| chronic drug use NOS \| chronic IV substance use \| drug use nos [6C4G.2Z] Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified Also known as: Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified \| Unknown or unspecified psychoactive substance dependence \| Drug dependence NOS [NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified Also known as: Harmful effects of drugs, medicaments or biological substances, not elsewhere classified \| drugs, medicaments or biological substances, toxicity not elsewhere classified \| adverse drug effects \| drug reaction NOS \| drug allergy NOS Excludes: Alcohol intoxication \| pathological drug intoxication \| hypersensitivity reaction to correctly administered drug [4A85.0Z] Drug hypersensitivity of unspecified type Also known as: Drug hypersensitivity of unspecified type \| Drug or pharmacological agents hypersensitivity \| medicinal hypersensitivity \| drug sensitivity NOS === GRAPH WALKS === --- Walk 1 --- [FB81.2] Drug-induced osteonecrosis Def: Alteration of the normal structure of orofacial tissues resulting from medicinal substances acting locally or systemically.... --PARENT--> [FB81] Osteonecrosis Def: Osteonecrosis is the medical term for death of bone tissue that occurs when the supply of blood to the bone is cut off for some reason. Doctors sometimes refer to the condition as avascular necrosis, ... --CHILD--> [FB81.0] Idiopathic aseptic osteonecrosis --- Walk 2 --- [FB81.2] Drug-induced osteonecrosis Def: Alteration of the normal structure of orofacial tissues resulting from medicinal substances acting locally or systemically.... --PARENT--> [FB81] Osteonecrosis Def: Osteonecrosis is the medical term for death of bone tissue that occurs when the supply of blood to the bone is cut off for some reason. Doctors sometimes refer to the condition as avascular necrosis, ... --CHILD--> [FB81.0] Idiopathic aseptic osteonecrosis --- Walk 3 --- [8A80.Z] Migraine, unspecified --PARENT--> [8A80] Migraine Def: A primary headache disorder, in most cases episodic. Disabling attacks lasting 4-72 hours are characterised by moderate or severe headache, usually accompanied by nausea, vomiting and/or photophobia a... --EXCLUDES--> [?] Headache, not elsewhere classified Def: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above.... --- Walk 4 --- [8A80.Z] Migraine, unspecified --PARENT--> [8A80] Migraine Def: A primary headache disorder, in most cases episodic. Disabling attacks lasting 4-72 hours are characterised by moderate or severe headache, usually accompanied by nausea, vomiting and/or photophobia a... --CHILD--> [8A80.0] Migraine without aura Def: Recurrent headache disorder manifesting in attacks lasting 4-72 hours. The duration of attacks may be shorter in children. Typical characteristics of the headache are unilateral location, pulsating qu... --- Walk 5 --- [QA76] Medication or substance that is known to be an allergen without injury or harm Def: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm.... --EXCLUDES--> [?] Medication or substance that is known to be an allergen, as mode of injury or harm --PARENT--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance --- Walk 6 --- [QA76] Medication or substance that is known to be an allergen without injury or harm Def: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm.... --EXCLUDES--> [?] Medication or substance that is known to be an allergen, as mode of injury or harm --PARENT--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance	[ "[FB81.2] Drug-induced osteonecrosis\n Def: Alteration of the normal structure of orofacial tissues resulting from medicinal substances acting locally or systemically....\n --PARENT--> [FB81] Osteonecrosis\n Def: Osteonecrosis is the medical term for death of bone tissue that occurs when the supply of blood to the bone is cut off for some reason. Doctors sometimes refer to the condition as avascular necrosis, ...\n --CHILD--> [FB81.0] Idiopathic aseptic osteonecrosis", "[FB81.2] Drug-induced osteonecrosis\n Def: Alteration of the normal structure of orofacial tissues resulting from medicinal substances acting locally or systemically....\n --PARENT--> [FB81] Osteonecrosis\n Def: Osteonecrosis is the medical term for death of bone tissue that occurs when the supply of blood to the bone is cut off for some reason. Doctors sometimes refer to the condition as avascular necrosis, ...\n --CHILD--> [FB81.0] Idiopathic aseptic osteonecrosis", "[8A80.Z] Migraine, unspecified\n --PARENT--> [8A80] Migraine\n Def: A primary headache disorder, in most cases episodic. Disabling attacks lasting 4-72 hours are characterised by moderate or severe headache, usually accompanied by nausea, vomiting and/or photophobia a...\n --EXCLUDES--> [?] Headache, not elsewhere classified\n Def: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above....", "[8A80.Z] Migraine, unspecified\n --PARENT--> [8A80] Migraine\n Def: A primary headache disorder, in most cases episodic. Disabling attacks lasting 4-72 hours are characterised by moderate or severe headache, usually accompanied by nausea, vomiting and/or photophobia a...\n --CHILD--> [8A80.0] Migraine without aura\n Def: Recurrent headache disorder manifesting in attacks lasting 4-72 hours. The duration of attacks may be shorter in children. Typical characteristics of the headache are unilateral location, pulsating qu...", "[QA76] Medication or substance that is known to be an allergen without injury or harm\n Def: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm....\n --EXCLUDES--> [?] Medication or substance that is known to be an allergen, as mode of injury or harm\n --PARENT--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance", "[QA76] Medication or substance that is known to be an allergen without injury or harm\n Def: Medication that has previously been identified as an allergen to the patient is administered, but does not result in injury or harm....\n --EXCLUDES--> [?] Medication or substance that is known to be an allergen, as mode of injury or harm\n --PARENT--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance" ]	FB81.2	Drug-induced osteonecrosis	[ { "from_icd11": "FB81.2", "icd10_code": "M87151", "icd10_title": "Osteonecrosis due to drugs, right femur" }, { "from_icd11": "FB81.2", "icd10_code": "M87152", "icd10_title": "Osteonecrosis due to drugs, left femur" }, { "from_icd11": "FB81.2", "icd10_code": "M87180", "icd10_title": "Osteonecrosis due to drugs, jaw" }, { "from_icd11": "FB81.2", "icd10_code": "M87121", "icd10_title": "Osteonecrosis due to drugs, right humerus" }, { "from_icd11": "FB81.2", "icd10_code": "M87122", "icd10_title": "Osteonecrosis due to drugs, left humerus" }, { "from_icd11": "FB81.2", "icd10_code": "M87159", "icd10_title": "Osteonecrosis due to drugs, unspecified femur" }, { "from_icd11": "FB81.2", "icd10_code": "M87188", "icd10_title": "Osteonecrosis due to drugs, other site" }, { "from_icd11": "FB81.2", "icd10_code": "M87176", "icd10_title": "Osteonecrosis due to drugs, unspecified foot" }, { "from_icd11": "FB81.2", "icd10_code": "M8710", "icd10_title": "Osteonecrosis due to drugs, unspecified bone" }, { "from_icd11": "FB81.2", "icd10_code": "M87174", "icd10_title": "Osteonecrosis due to drugs, right foot" }, { "from_icd11": "FB81.2", "icd10_code": "M87175", "icd10_title": "Osteonecrosis due to drugs, left foot" }, { "from_icd11": "FB81.2", "icd10_code": "M87162", "icd10_title": "Osteonecrosis due to drugs, left tibia" }, { "from_icd11": "FB81.2", "icd10_code": "M871", "icd10_title": "Osteonecrosis due to drugs" }, { "from_icd11": "8A80.Z", "icd10_code": "G43B0", "icd10_title": "Ophthalmoplegic migraine, not intractable" }, { "from_icd11": "8A80.Z", "icd10_code": "G43409", "icd10_title": "Hemiplegic migraine, not intractable, without status migrainosus" } ]	M87151	Osteonecrosis due to drugs, right femur
An 83-year-old man with DM and chronic kidney disease noticed color changes in his toes for 4 years and had small ulcers and purplish-reddish plaques on his right toe for 2 years. The patient received conservative treatment for the skin lesions at a local dermatology clinic, resulting in repeated exacerbations and remissions. Skin biopsy was performed to exclude malignant skin diseases. During that time, arterial bleeding from the punctured site was confirmed. Consequently, he was referred to our department with a possible AVM. During physical examination at his first visit to our department, dark reddish-brownish macules and ulcers were observed on the right second and third toes . Although the pulsation of the dorsalis pedis artery (DPA) of the affected limb was palpable and the ankle–brachial index was 1.10, the skin perfusion pressure was only 20 and 30 mmHg on the dorsal and plantar surface, respectively, indicating severe ischemia of toe and forefoot. Ultrasonography revealed an AVM around the right second metatarsophalangeal joint , which attributed to arterial steal phenomenon. Computed tomography (CT) angiography revealed an AVM around the right second metatarsophalangeal joint . The right DPA was occluded in the middle, raising suspicion of CLTI . Routine hematological and biochemical tests indicated elevated blood urea nitrogen and creatinine (28 and 1.7 mg/dl, respectively), and HbA1c was 7.1%. Serological testing for HIV yielded negative results. Skin biopsy revealed dermal capillary proliferation, extravascular leakage of red blood cells, and hemosiderin deposition in the interstitium. Immunohistochemical analysis revealed negative staining for HHV-8 and positive staining for CD34 in the vascular endothelial cells . Based on these results, a diagnosis of PKS with CLTI. The following points were discussed for treatment: (1) ligation of AVM was difficult due to bleeding from AVM; (2) embolization could control the AVM itself, however, ischemia in the toes, where blood flow is reduced by diabetic atherosclerosis, was thought to be exacerbated by embolization. Due to the difficulty of eliminating AVM without resorting to minor amputation, conservative treatment was recommended. The skin lesion was managed without ulcer recurrence for 2 years since it healed with conservative treatment, such as antibiotic ointments and Fiblast Spray, known as recombinant human basic fibroblast growth factor (KAKEN PHARMACEUTICAL CO., LTD. Tokyo, Japan). Unfortunately, the lesion finally deteriorated due to infection, after 2 years of follow-up , leading to increased foot pain, and C-reactive protein was detected (3.0 mg/dl). The patient was treated with intravenous ampicillin/sulbactam (1.5 g q12hr) for 14 days based on the wound culture results. Next, to manage this deterioration of the skin lesions, minor amputation was necessary, but arterial bleeding was identified during biopsy, so it was considered that amputation was high risk of bleeding. Therefore, endovascular embolization was primarily indicated because of controlling AVF flow for a preoperative adjuvant before minor amputation and reduction of the risk of bleeding during surgical resection, with risk of progression of foot ischemia. As a result, the patient was admitted and AVM was controlled through transarterial embolization. To perform embolization, a 4-French sheath was anterogradely inserted into the right common femoral artery. The nidus structure of the AVM was identified and embolized using a mixture of N -butyl cyanoacrylate (NBCA) and lipiodol at a 1:5 dilution, introduced through the DPA and plantar artery . The patient was discharged once the ulcer improved after the antibiotic treatment and embolization . This procedure resulted in decreased contrast enhancement of the nidus. CT angiography after embolization showed decreased AVM flow compared with before . Unfortunately, he was readmitted owing to deterioration of the skin lesions on the toes 14 days after embolization . It could be deteriorated owing to progression of toe ischemia and infection caused by the embolizing to blood flow not only AVM but outside the AVM. To address this worsening situation, forefoot amputation was performed at the Lisfranc joint, accompanied by surgical resection of the AVM . The wound healed successfully, and gait function was regained with the use of assistance equipment . The entire treatment process required 4 months. Postoperatively, the patient was followed by CT angiography . No recurrence was observed for 1 year. Fig. 1 Preoperative findings. Color changes in the toes ( A ). Arteriovenous malformation (AVM) around the right third metatarsophalangeal joint detected by ultrasound sonography ( B ). Computed tomography angiography showed arterial and venous perfusion in the right lower extremity at the arterial phase and atherosclerotic lesions of the left infrapopliteal arteries ( C ). AVM around the right second metatarsophalangeal joint and atherosclerotic lesions in the right dorsalis pedis artery, indicated by arrowhead ( D ). Skin biopsy revealed dermal capillary proliferation by arrowhead, extravascular leakage of red blood cells by arrows, and hemosiderin deposition in the interstitium (H&E × 100). Human herpes virus-8 staining was negative (×100). CD34 staining was only positive in the vascular endothelial cells (×100) ( E ) Fig. 2 Right toe appearance and embolization findings. The second and third toes were infected ( A ). Digital subtraction angiography showed arteriovenous malformation (AVM) ( B ). Embolization using a mixture of N -butyl cyanoacrylate (NBCA) and lipiodol from the right plantar artery ( C ) and dorsalis pedis artery ( D ). Significant decrease in AVM perfusion after embolization ( E ). Infection of the toes was improved by antibiotic use and embolization ( F ). Computed tomography angiography after embolization showed decreased AVM flow compared to Fig. 1 D. The embolic agent is indicated by the yellow segments ( G ) Fig. 3 Ulceration and surgical resection of arteriovenous malformation (AVM). Ulcers occurred on the treated right toes after embolization ( A ). Disarticulation at the Lisfranc joint, including surgical resection of AVM ( B , C ). Wound healing and ambulation were achieved with assistance equipment ( D – F ). Postoperative computed tomography angiography showed that AVM was completely resected ( G )	4.058594	0.976563	sec[1]/p[0]	en	0.999996	38842785	https://doi.org/10.1186/s40792-024-01933-7	[ "embolization", "skin", "toes", "angiography", "artery", "joint", "lesions", "arterial", "bleeding", "blood" ]	[ { "code": "DD30.Z", "title": "Acute vascular disorders of intestine, unspecified" }, { "code": "JB42.2", "title": "Obstetric blood-clot embolism" }, { "code": "NF0A.0", "title": "Air embolism, traumatic, not elsewhere classified" }, { "code": "BA41.Z", "title": "Acute myocardial infarction, unspecified" }, { "code": "BB00.Z", "title": "Pulmonary thromboembolism, unspecified" }, { "code": "ME67", "title": "Skin disorder of uncertain or unspecified nature" }, { "code": "ME66.Y", "title": "Other specified skin changes" }, { "code": "EM0Y", "title": "Other specified diseases of the skin" }, { "code": "ME60.Z", "title": "Skin lesion of unspecified nature" }, { "code": "ME66.1", "title": "Changes in skin texture" } ]	=== ICD-11 CODES FOUND === [DD30.Z] Acute vascular disorders of intestine, unspecified Also known as: Acute vascular disorders of intestine, unspecified \| Acute vascular disorders of intestine \| acute intestinal ischemia NOS \| acute intestinal ischemic syndrome \| acute ischaemic bowel disease [JB42.2] Obstetric blood-clot embolism Definition: A condition characterised by the lodging of a blood clot (a specific type of embolus known as a thrombus) in the bloodstream, which can cause a blockage associated with the physiological and other changes that occur during the period of time from conception to delivery (pregnancy), during labour and delivery (childbirth) or during the approximately six weeks after delivery during which the uterus returns to the original size (puerperium). Also known as: Obstetric blood-clot embolism \| obstetrical blood-clot embolism \| blood clot embolism in pregnancy, childbirth or puerperium \| puerperal embolism \| Puerperal embolism NOS [NF0A.0] Air embolism, traumatic, not elsewhere classified Also known as: Air embolism, traumatic, not elsewhere classified \| pulmonary air embolism \| air embolism \| arterial air embolism \| arterial air embolus Excludes: air embolism complicating abortion or ectopic or molar pregnancy \| air embolism complicating pregnancy, childbirth and the puerperium [BA41.Z] Acute myocardial infarction, unspecified Also known as: Acute myocardial infarction, unspecified \| Acute myocardial infarction \| cardiac attack \| heart attack \| acute cardiac infarction [BB00.Z] Pulmonary thromboembolism, unspecified Also known as: Pulmonary thromboembolism, unspecified \| Pulmonary thromboembolism \| pulmonary thromboembolus \| pulmonary embolism NOS \| pulmonary embolus [ME67] Skin disorder of uncertain or unspecified nature Definition: A category to enable the presence of a skin disorder to be recorded without making assumptions as to the precise nature of the disorder in question. Also known as: Skin disorder of uncertain or unspecified nature \| Skin disorder without established diagnosis \| change of skin NOS \| dermatological disease NOS \| dermatological disorder NOS [ME66.Y] Other specified skin changes Also known as: Other specified skin changes \| Cutis marmorata \| Fear of skin disease \| Retention hyperkeratosis \| Dermatitis neglecta [EM0Y] Other specified diseases of the skin Also known as: Other specified diseases of the skin \| Adverse cutaneous effects of healthcare related interventions \| Cutaneous complications of surgical, laser or other interventional procedures \| Postprocedural cutaneous complications of surgical, laser or other interventions \| Cutaneous complications of surgical procedures [ME60.Z] Skin lesion of unspecified nature Also known as: Skin lesion of unspecified nature \| Skin lesion of uncertain or unspecified nature \| Skin lesion without established diagnosis \| Pigmented skin lesion of unspecified nature \| Ulcer of skin of unspecified nature [ME66.1] Changes in skin texture Definition: Alterations in skin texture of unspecified cause. Also known as: Changes in skin texture \| Skin textural disturbance \| Thickening of skin \| induration of skin \| Skin sclerosis === GRAPH WALKS === --- Walk 1 --- [DD30.Z] Acute vascular disorders of intestine, unspecified --PARENT--> [DD30] Acute vascular disorders of intestine Def: Intestinal ischaemia has an associated vascular block, usually due to atheroma, thrombus, or embolus but occasionally the result of an arteritis, vasculitis, or other condition.... --CHILD--> [DD30.0] Acute mesenteric arterial infarction Def: Acute mesenteric arterial infarction is an ischemic disorder of sudden interruption of mesenteric arterial flow because of occlusion of mesenteric artery. This may be further subdivided into acute mes... --- Walk 2 --- [DD30.Z] Acute vascular disorders of intestine, unspecified --PARENT--> [DD30] Acute vascular disorders of intestine Def: Intestinal ischaemia has an associated vascular block, usually due to atheroma, thrombus, or embolus but occasionally the result of an arteritis, vasculitis, or other condition.... --CHILD--> [DD30.2] Acute mesenteric venous occlusion Def: Acute mesenteric venous occlusion is an ischemic disorder of sudden interruption of mesenteric venous flow because of venous thrombosis.... --- Walk 3 --- [JB42.2] Obstetric blood-clot embolism Def: A condition characterised by the lodging of a blood clot (a specific type of embolus known as a thrombus) in the bloodstream, which can cause a blockage associated with the physiological and other cha... --PARENT--> [JB42] Obstetric embolism Def: A condition characterised by the lodging of a blood clot, a fat globule or a gas bubble (embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes tha... --CHILD--> [JB42.1] Amniotic fluid embolism Def: Amniotic fluid embolism is a rare obstetric emergency in which it is postulated that amniotic fluid, fetal cells, hair, or other debris enter the maternal circulation, causing cardiorespiratory collap... --- Walk 4 --- [JB42.2] Obstetric blood-clot embolism Def: A condition characterised by the lodging of a blood clot (a specific type of embolus known as a thrombus) in the bloodstream, which can cause a blockage associated with the physiological and other cha... --PARENT--> [JB42] Obstetric embolism Def: A condition characterised by the lodging of a blood clot, a fat globule or a gas bubble (embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes tha... --CHILD--> [JB42.2] Obstetric blood-clot embolism Def: A condition characterised by the lodging of a blood clot (a specific type of embolus known as a thrombus) in the bloodstream, which can cause a blockage associated with the physiological and other cha... --- Walk 5 --- [NF0A.0] Air embolism, traumatic, not elsewhere classified --EXCLUDES--> [?] Obstetric air embolism Def: A condition characterised by the lodging of a gas bubble (air embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes that occur during the period o... --PARENT--> [?] Obstetric embolism Def: A condition characterised by the lodging of a blood clot, a fat globule or a gas bubble (embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes tha... --- Walk 6 --- [NF0A.0] Air embolism, traumatic, not elsewhere classified --EXCLUDES--> [?] Obstetric air embolism Def: A condition characterised by the lodging of a gas bubble (air embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes that occur during the period o... --PARENT--> [?] Obstetric embolism Def: A condition characterised by the lodging of a blood clot, a fat globule or a gas bubble (embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes tha...	[ "[DD30.Z] Acute vascular disorders of intestine, unspecified\n --PARENT--> [DD30] Acute vascular disorders of intestine\n Def: Intestinal ischaemia has an associated vascular block, usually due to atheroma, thrombus, or embolus but occasionally the result of an arteritis, vasculitis, or other condition....\n --CHILD--> [DD30.0] Acute mesenteric arterial infarction\n Def: Acute mesenteric arterial infarction is an ischemic disorder of sudden interruption of mesenteric arterial flow because of occlusion of mesenteric artery. This may be further subdivided into acute mes...", "[DD30.Z] Acute vascular disorders of intestine, unspecified\n --PARENT--> [DD30] Acute vascular disorders of intestine\n Def: Intestinal ischaemia has an associated vascular block, usually due to atheroma, thrombus, or embolus but occasionally the result of an arteritis, vasculitis, or other condition....\n --CHILD--> [DD30.2] Acute mesenteric venous occlusion\n Def: Acute mesenteric venous occlusion is an ischemic disorder of sudden interruption of mesenteric venous flow because of venous thrombosis....", "[JB42.2] Obstetric blood-clot embolism\n Def: A condition characterised by the lodging of a blood clot (a specific type of embolus known as a thrombus) in the bloodstream, which can cause a blockage associated with the physiological and other cha...\n --PARENT--> [JB42] Obstetric embolism\n Def: A condition characterised by the lodging of a blood clot, a fat globule or a gas bubble (embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes tha...\n --CHILD--> [JB42.1] Amniotic fluid embolism\n Def: Amniotic fluid embolism is a rare obstetric emergency in which it is postulated that amniotic fluid, fetal cells, hair, or other debris enter the maternal circulation, causing cardiorespiratory collap...", "[JB42.2] Obstetric blood-clot embolism\n Def: A condition characterised by the lodging of a blood clot (a specific type of embolus known as a thrombus) in the bloodstream, which can cause a blockage associated with the physiological and other cha...\n --PARENT--> [JB42] Obstetric embolism\n Def: A condition characterised by the lodging of a blood clot, a fat globule or a gas bubble (embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes tha...\n --CHILD--> [JB42.2] Obstetric blood-clot embolism\n Def: A condition characterised by the lodging of a blood clot (a specific type of embolus known as a thrombus) in the bloodstream, which can cause a blockage associated with the physiological and other cha...", "[NF0A.0] Air embolism, traumatic, not elsewhere classified\n --EXCLUDES--> [?] Obstetric air embolism\n Def: A condition characterised by the lodging of a gas bubble (air embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes that occur during the period o...\n --PARENT--> [?] Obstetric embolism\n Def: A condition characterised by the lodging of a blood clot, a fat globule or a gas bubble (embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes tha...", "[NF0A.0] Air embolism, traumatic, not elsewhere classified\n --EXCLUDES--> [?] Obstetric air embolism\n Def: A condition characterised by the lodging of a gas bubble (air embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes that occur during the period o...\n --PARENT--> [?] Obstetric embolism\n Def: A condition characterised by the lodging of a blood clot, a fat globule or a gas bubble (embolus) in the bloodstream, which can cause a blockage associated with the physiological and other changes tha..." ]	DD30.Z	Acute vascular disorders of intestine, unspecified	[ { "from_icd11": "DD30.Z", "icd10_code": "K55019", "icd10_title": "Acute (reversible) ischemia of small intestine, extent unspecified" }, { "from_icd11": "DD30.Z", "icd10_code": "K55029", "icd10_title": "Acute infarction of small intestine, extent unspecified" }, { "from_icd11": "DD30.Z", "icd10_code": "K55069", "icd10_title": "Acute infarction of intestine, part and extent unspecified" }, { "from_icd11": "DD30.Z", "icd10_code": "K55021", "icd10_title": "Focal (segmental) acute infarction of small intestine" }, { "from_icd11": "DD30.Z", "icd10_code": "K55039", "icd10_title": "Acute (reversible) ischemia of large intestine, extent unspecified" }, { "from_icd11": "DD30.Z", "icd10_code": "K55059", "icd10_title": "Acute (reversible) ischemia of intestine, part and extent unspecified" }, { "from_icd11": "DD30.Z", "icd10_code": "K55042", "icd10_title": "Diffuse acute infarction of large intestine" }, { "from_icd11": "DD30.Z", "icd10_code": "K55041", "icd10_title": "Focal (segmental) acute infarction of large intestine" }, { "from_icd11": "DD30.Z", "icd10_code": "K55011", "icd10_title": "Focal (segmental) acute (reversible) ischemia of small intestine" }, { "from_icd11": "DD30.Z", "icd10_code": "K55012", "icd10_title": "Diffuse acute (reversible) ischemia of small intestine" }, { "from_icd11": "DD30.Z", "icd10_code": "K55049", "icd10_title": "Acute infarction of large intestine, extent unspecified" }, { "from_icd11": "DD30.Z", "icd10_code": "K55061", "icd10_title": "Focal (segmental) acute infarction of intestine, part unspecified" }, { "from_icd11": "DD30.Z", "icd10_code": "K55022", "icd10_title": "Diffuse acute infarction of small intestine" }, { "from_icd11": "DD30.Z", "icd10_code": "K55031", "icd10_title": "Focal (segmental) acute (reversible) ischemia of large intestine" }, { "from_icd11": "DD30.Z", "icd10_code": "K55032", "icd10_title": "Diffuse acute (reversible) ischemia of large intestine" } ]	K55019	Acute (reversible) ischemia of small intestine, extent unspecified
A 73-year-old Japanese man with a 2-month history of dysphasia and heartburn first presented to his local doctor and was later admitted to our hospital. He had difficulties in swallowing and eating; did not have melena, epigastralgia, or hematemesis; and had a history of hypertension and no known allergies. At the time of admission, he was taking at lansoprazole 15 mg/day and olmesartan medoxomil 10 mg/day. He did not drink alcohol but used to smoke 30 cigarettes per day for 45 years. His environmental and employment histories were unremarkable. His family history was remarkable for colon cancer in his father and lung cancer in his brother. On admission, his height was 161 cm, body weight was 56.5 kg, blood pressure was 126/62 mm Hg, pulse was 70 beats per minute, temperature was 36.9 °C, and oxygen saturation was 98% while he was breathing ambient air. His conjunctiva was not icteric but slightly anemic. On chest examination, his heart rhythm was regular with no murmur, and his lungs were clear to auscultation. His abdomen was soft, not distended, and not tender. A soft and movable mass was palpable around the epigastrium. The legs and feet showed no edema. Laboratory tests showed a creatinine level of 0.89 mg/dl, blood urea nitrogen level of 12.6 mg/dl, total bilirubin level of 0.3 mg/dl, aspartate transaminase level of 17 IU/L, and alanine transaminase level of 19 IU/L. The patient’s white blood cell count was 8930 per cubic milliliter, hemoglobin was 9.2 g/dl, and platelet count was 438,000 per cubic milliliter. An esophagogastric fiber (EGF) showed type 3 gastric carcinoma in the antrum. The tumor caused pyloric stenosis and invasion to the duodenum, so the patient was admitted to the hospital . Staging laparoscopy was performed to assess the extent of tumor spread, and laparoscopic bypass was performed. Staging laparoscopy revealed peritoneal dissemination, and peritoneal lavage cytology revealed tumor cells in the abdominal cavity. We diagnosed L, type 3, circ, cT4a(SE), cNx, pP1, pCY1, M0, stage IV (the Japanese classification of gastric carcinoma). The patient was initially treated with docetaxel 40 mg/m 2 on day 1, cisplatin (CDDP) 60 mg/m 2 on day 1, and TS-1 120 mg/day on days 1–14, followed by a 2-week recovery period (DCS regimen). Dexamethasone 9.9 mg and palonosetron 0.75 mg were administered on day 1, and dexamethasone 6.6 mg was administered on days 2 and 3 as premedication. The patient had grade 3 diarrhea (according to Common Terminology Criteria for Adverse Events criteria) after one course . Then TS-1 was reduced (100 mg). After two courses of the DCS regimen, EGF and computed tomography (CT) showed that the tumor had shrunk , and then staging laparoscopy was performed to evaluate a response. Peritoneal dissemination disappeared, and peritoneal lavage cytology revealed no tumor cells in the abdominal cavity. Then salvage operation, laparoscopic distal gastrectomy with D1+ dissection, was performed. Pathological findings were ypT2(MP), ypN2(3/15), ypP0, ypCY0, M0, ystage II . TS-1100 mg/day on days 1–14, every 3 weeks was started as adjuvant chemotherapy. After 15 months, CT revealed multiple peritoneal nodules . They were highly suspected as a recurrence. Paclitaxel 80 mg/m 2 on days 1, 8, and 15 was started as a second regimen. Dexamethasone 6.6 mg, famotidine 20 mg, and granisetron 3 mg were administered on days 1, 8, and 15 as premedication. This regimen achieved partial response , but its efficacy did not last. After 3 months, CT revealed progressive disease . The original gastric carcinoma was HER2-positive . The patient’s Eastern Cooperative Oncology Group performance status was 2; his body weight was 50.7 kg; and he complained of appetite loss. We concluded that the patient could not tolerate doublet therapy. Therefore, TS-1100 mg on days 1–14 with Herceptin 6 mg/kg (Roche/Genentech, South San Francisco, CA, USA) on day 1 every 3 weeks was introduced. This regimen was substantially effective and achieved CR after 9 months based on CT findings . The patient had no adverse effects while receiving this regimen . Since then, the patient has been treated with only Herceptin 6 mg/kg every 3 weeks without any side effects, and no radiological findings of recurrence had yet occurred for 6 years, 7 months after surgery . Fig. 1 Computed tomography (CT) and esophagogastric fiber (EGF) findings at diagnosis and after two courses of docetaxel, cisplatin, and TS-1 chemotherapy. a , b Abdominal CT showed thickened wall of the antrum and a bulky lymph node ( arrows ). c EGF showed type 3 tumor in the antrum. d , e Thickened antrum wall was significantly improved, and the bulky lymph node was significantly shrunk ( arrows ). f Type 3 tumor was remarkably shrunk. Side bar = 1 cm Fig. 2 a Changes in tumor markers (CEA, CA19-9 and CA125), CBC, and CRP. b Changes in liver function, serum albumin, renal fanction and electrolytes. CEA Carcinoembryonic antigen (U/ml), CA19-9 Cancer antigen 19-9 (U/ml), CA125 Cancer antigen 125 (U/ml), WBC White blood cell count (× 100 per cubic milliliter), Neut Neutrophil (× 100 per cubic milliliter), Hg Hemoglobin (g/dl), PLT Platelet count (× 10,000 per cubic milliliter), CRP C-reactive protein (mg/L), T-Bil Total bilirubin (mg/dl), AST Aspartate transaminase (IU/L), ALT Alanine transaminase (IU/L), ALP Alkaline phosphatase (IU/L), Alb Albumin (g/dl), BUN Blood urea nitrogen (mg/dl), CRE Creatinine (mg/dl), Na Sodium (mEq/L), Cl Chloride (mEq/L), P Potassium (mEq/L) Fig. 3 Pathological findings. a Microscopic view of the cancer (H&E stain, × 12.5). b Microscopic view showed fibrosis in subserosa in which the cancer cells seemed to have disappeared (H&E stain, × 12.5). c H&E stain showed massive number of cancer cells were present in subserosa (× 40). d Cancer cells were present in muscularis propria (H&E stain, × 100) Fig. 4 Trastuzumab is effective against HER2-positive gastric cancer. a Abdominal computed tomography (CT) showed a peritoneal nodule ( arrow ). b Paclitaxel was effective against peritoneal dissemination ( arrow ). c CT showed a new peritoneal nodule appeared (arrow) . d , e Peritoneal dissemination disappeared ( arrows ). f CT showed peritoneal dissemination was disappeared for 6 years and 3 months after surgery (arrow) Fig. 5 Immunostaining of the cancer. Immunostaining with HER2 showed the cancer cells were HER2-positive (× 200)	3.849609	0.982422	sec[1]/p[0]	en	0.999995	30791934	https://doi.org/10.1186/s13256-019-2001-3	[ "cancer", "peritoneal", "tumor", "cells", "regimen", "blood", "cubic", "milliliter", "dissemination", "transaminase" ]	[ { "code": "2D4Z", "title": "Unspecified malignant neoplasms of unspecified sites" }, { "code": "2C0Z", "title": "Malignant neoplasms of intestine, unspecified" }, { "code": "2B5Z", "title": "Malignant mesenchymal neoplasm of unspecified type" }, { "code": "2E2Z", "title": "Malignant neoplasm metastasis, unspecified" }, { "code": "2D42", "title": "Malignant neoplasms of ill-defined sites" }, { "code": "DC5Z", "title": "Diseases of peritoneum, unspecified" }, { "code": "DC50.Z", "title": "Peritonitis, unspecified" }, { "code": "NB91.Y&XA0KZ0", "title": "Peritoneal laceration" }, { "code": "2F94", "title": "Neoplasms of unknown behaviour of peritoneum" }, { "code": "DC51.Y", "title": "Other specified disorders of peritoneum or retroperitoneum" } ]	=== ICD-11 CODES FOUND === [2D4Z] Unspecified malignant neoplasms of unspecified sites Also known as: Unspecified malignant neoplasms of unspecified sites \| malignancy of unspecified site \| malignancy unspecified primary site \| malignant growth of unspecified site \| malignant mass of unspecified site [2C0Z] Malignant neoplasms of intestine, unspecified Also known as: Malignant neoplasms of intestine, unspecified \| cancer of intestine \| malignant neoplasm of intestine NOS \| malignant tumour of intestine NOS \| intestinal cancer NOS [2B5Z] Malignant mesenchymal neoplasm of unspecified type Also known as: Malignant mesenchymal neoplasm of unspecified type \| calvarium cancer \| ethmoid bone cancer \| facial bone cancer \| frontal bone cancer [2E2Z] Malignant neoplasm metastasis, unspecified Also known as: Malignant neoplasm metastasis, unspecified \| secondary malignant neoplasm \| metastasis \| metastases \| disseminated metastases [2D42] Malignant neoplasms of ill-defined sites Definition: Malignant neoplasms of ill defined sites is used for cases where the documentation refers to a site that includes multiple organ systems and tissue types that should be coded separately. Also known as: Malignant neoplasms of ill-defined sites \| Malignant neoplasm of ill-defined site of head, face or neck \| Malignant neoplasm of nose NOS \| Primary malignant neoplasm of cheek \| malignant neoplasm of cheek NOS [DC5Z] Diseases of peritoneum, unspecified Also known as: Diseases of peritoneum, unspecified \| peritoneal disease [DC50.Z] Peritonitis, unspecified Also known as: Peritonitis, unspecified \| Peritonitis \| peritoneum inflammation \| peritonitis of undetermined cause \| peritonitis of unspecified cause [2F94] Neoplasms of unknown behaviour of peritoneum Also known as: Neoplasms of unknown behaviour of peritoneum \| peritoneum tumour NOS [DC51.Y] Other specified disorders of peritoneum or retroperitoneum Also known as: Other specified disorders of peritoneum or retroperitoneum \| Abdominal granuloma \| Peritoneal granuloma \| Epiploic appendagitis \| Male frozen pelvis === GRAPH WALKS === --- Walk 1 --- [2D4Z] Unspecified malignant neoplasms of unspecified sites --PARENT--> [?] Malignant neoplasms of ill-defined or unspecified primary sites --EXCLUDES--> [?] Malignant mesenchymal neoplasms Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn... --- Walk 2 --- [2D4Z] Unspecified malignant neoplasms of unspecified sites --PARENT--> [?] Malignant neoplasms of ill-defined or unspecified primary sites --EXCLUDES--> [?] Malignant mesenchymal neoplasms Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn... --- Walk 3 --- [2C0Z] Malignant neoplasms of intestine, unspecified --PARENT--> [?] Malignant neoplasms of intestine --EXCLUDES--> [?] Malignant mesenchymal neoplasms Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn... --- Walk 4 --- [2C0Z] Malignant neoplasms of intestine, unspecified --PARENT--> [?] Malignant neoplasms of intestine --CHILD--> [?] Malignant neoplasms of large intestine --- Walk 5 --- [2B5Z] Malignant mesenchymal neoplasm of unspecified type --PARENT--> [?] Malignant mesenchymal neoplasms Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn... --PARENT--> [?] Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues --- Walk 6 --- [2B5Z] Malignant mesenchymal neoplasm of unspecified type --PARENT--> [?] Malignant mesenchymal neoplasms Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn... --CHILD--> [2B51] Osteosarcoma, primary site Def: A usually aggressive malignant bone-forming mesenchymal tumour, predominantly affecting adolescents and young adults. It usually involves bones and less frequently extraosseous sites. It often involve...	[ "[2D4Z] Unspecified malignant neoplasms of unspecified sites\n --PARENT--> [?] Malignant neoplasms of ill-defined or unspecified primary sites\n --EXCLUDES--> [?] Malignant mesenchymal neoplasms\n Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn...", "[2D4Z] Unspecified malignant neoplasms of unspecified sites\n --PARENT--> [?] Malignant neoplasms of ill-defined or unspecified primary sites\n --EXCLUDES--> [?] Malignant mesenchymal neoplasms\n Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn...", "[2C0Z] Malignant neoplasms of intestine, unspecified\n --PARENT--> [?] Malignant neoplasms of intestine\n --EXCLUDES--> [?] Malignant mesenchymal neoplasms\n Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn...", "[2C0Z] Malignant neoplasms of intestine, unspecified\n --PARENT--> [?] Malignant neoplasms of intestine\n --CHILD--> [?] Malignant neoplasms of large intestine", "[2B5Z] Malignant mesenchymal neoplasm of unspecified type\n --PARENT--> [?] Malignant mesenchymal neoplasms\n Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn...\n --PARENT--> [?] Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues", "[2B5Z] Malignant mesenchymal neoplasm of unspecified type\n --PARENT--> [?] Malignant mesenchymal neoplasms\n Def: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The progn...\n --CHILD--> [2B51] Osteosarcoma, primary site\n Def: A usually aggressive malignant bone-forming mesenchymal tumour, predominantly affecting adolescents and young adults. It usually involves bones and less frequently extraosseous sites. It often involve..." ]	2D4Z	Unspecified malignant neoplasms of unspecified sites	[ { "from_icd11": "2D4Z", "icd10_code": "C802", "icd10_title": "Malignant neoplasm associated with transplanted organ" }, { "from_icd11": "2D4Z", "icd10_code": "C7650", "icd10_title": "Malignant neoplasm of unspecified lower limb" }, { "from_icd11": "2D4Z", "icd10_code": "C7642", "icd10_title": "Malignant neoplasm of left upper limb" }, { "from_icd11": "2D4Z", "icd10_code": "C7640", "icd10_title": "Malignant neoplasm of unspecified upper limb" }, { "from_icd11": "2D4Z", "icd10_code": "C7652", "icd10_title": "Malignant neoplasm of left lower limb" }, { "from_icd11": "2D4Z", "icd10_code": "C7651", "icd10_title": "Malignant neoplasm of right lower limb" }, { "from_icd11": "2D4Z", "icd10_code": "C7641", "icd10_title": "Malignant neoplasm of right upper limb" }, { "from_icd11": "2D4Z", "icd10_code": "C801", "icd10_title": "Malignant (primary) neoplasm, unspecified" }, { "from_icd11": "2D4Z", "icd10_code": "C768", "icd10_title": "Malignant neoplasm of other specified ill-defined sites" }, { "from_icd11": "2D4Z", "icd10_code": "C761", "icd10_title": "Malignant neoplasm of thorax" }, { "from_icd11": "2D4Z", "icd10_code": "C762", "icd10_title": "Malignant neoplasm of abdomen" }, { "from_icd11": "2D4Z", "icd10_code": "C763", "icd10_title": "Malignant neoplasm of pelvis" }, { "from_icd11": "2D4Z", "icd10_code": "C800", "icd10_title": "Disseminated malignant neoplasm, unspecified" }, { "from_icd11": "2D4Z", "icd10_code": "C76-C80", "icd10_title": "" }, { "from_icd11": "2D4Z", "icd10_code": "C76", "icd10_title": "Malignant neoplasm of other and ill-defined sites" } ]	C802	Malignant neoplasm associated with transplanted organ
This is the case of an endoscopic endonasal resection of a brainstem cav mal. It is a 25-year-old woman who had two episodes of hemorrhage, one with initial diplopia and headache, then developing ventriculomegaly and hydrocephalus treated with an ETV. She then presented with a clear second hemorrhage and worsening double vision. Here you can see her progression over time of this midbrain malformation. It did not quite present to the floor of the third ventricle; it seemed to present in the midline to the mesencephalon. Careful examination of imaging presented concern for multiple approaches; here we see a FIESTA sagittal where again you can see the central location of the cavernous malformation. High-def fiber tracking was performed to try to understand the relationship of critical tracts. They showed lateral displacement of the motor fibers as well as third nerve tract, which made a ventral medial approach theoretically an ideal approach for this lesion. Endonasal and transcranial options were both considered; however, an interhemispheric transcallosal option was abandoned due to the location in the posterior third ventricle and after review of the sagittal FIESTA imaging revealed a thin rim of viable tissue between the cavernous malformation and the floor of the third ventricle. We therefore elected for an endoscopic endonasal resection. Of course, this requires multiple stages to it, but did require a pituitary hemitransposition on the right side of the pituitary gland in order to access this region of the mesencephalon. Here we can see the region of access just below the mamillary bodies, above the basilar apex. Nasal stage consisted on the resection of the middle turbinate on the right side. A right-sided nasoseptal flap with a left-sided reverse flap, so-called Caisedo flap. Next a wide sphenoidotomy was performed as well as a posterior ethmoidectomy. Here we can see the wide sphenoidotomy. A posterior septectomy is part of the reverse flap, and it helps provide wide view and access. The bone overlying the sella and the tuberculum and planum were carefully thinned and removed. This is done in the usual fashion where rather than biting with a rongeour, the bone is carefully thinned and then pealed away. Here the right parasellar carotid artery is exposed by again carefully blue-lining the bone and then carefully pealing the bone from the cavernous sinus. The upper and midclivus is necessary in order to have access to the basilar apex and have proximal control in the event of a vascular injury. Here we can see the midclivus being opened. Next, for a superior clival access, the cavernous sinus is opened on the right side and packed off with Gelfoam packing, and then the remainder of the medial cavernous sinus is widely opened. This is taken up to the diaphragm, which is also split adjacent to the pituitary stalk. This allows for complete pituitary transposition and mobilization of the gland towards the left side. Here we can see access to the very tall posterior clinoid. Once this has been dissected free, and the inferior hypophyseal artery is sacrificed, the posterior clinoid can be completely resected and the cap of it can be peeled from behind the carotid artery. This then allowed us to open the upper clival dura in addition. We can now see arachnoid being open, the third nerve. We can then dissect back along the third nerve to the basilar apex and then the mamillary bodies come into view. Here, examination of the prior endoscopic third ventriculostomy showed lack of patency. This was dissected and slightly reopened to allow relaxation of the mamillary bodies away from the basilar apex. We can then dissect and evaluate the thalamoperforators, the basilar apex, and also see some vague discoloration in the midbrain where the cavernous malformation essentially presents to the surface. Indocyanine green angiography with the endoscope was performed to evaluate the vasculature, and again here we see the region of the mesencephalon where the cavernous malformation presents to the surface in the anterior midline. We check again for perforators; we confirm our localization. Here you see a dorsal view of the brainstem anatomy related to this cavernous malformation. Midline dissection with microendoscopic forceps is performed and gain us access into the hematoma cavity. We begin to see the lesion and is removed piecemeal with careful visualization, dissection around the edge and grasping of the lesion, and delivery into the surgical field. Each pass is taken carefully with visualization of the thalamoperforators and the mamillary bodies. Image guidance helps confirm depth within the brainstem as we carefully evaluate for complete resection. Persistent bleeding reveals that there is likely residual left, so further exploration is performed for continued piecemeal removal. Dynamic endoscopy is critical for this portion as two suctions and forceps are used to carefully clear the field, evaluate the hematoma cavity, and visualize more malformation. Abnormal fluorescence is evaluated throughout. The window here between the mamillary bodies and the basilar apex is key. Image guidance helps confirm our depth. Obviously in this location in the brainstem image guidance is important for confirmation of location. This midline approach has allowed us to evaluate the cavernous malformation directly, deliver large portions like this without any disruption or transgression of the laterally displaced corticospinal tracts, oculomotor nuclei, and nerves. Careful inspection of the cavity at the end confirms complete resection. Meticulous hemostasis. After meticulous hemostasis repeat ICG angiography confirms patency of the basilar as well as the PCAs and the thalamoperforators. Reconstruction is performed in a multilayered fashion with a collagen matrix inlay graft. This is then followed by a vascularized nasoseptal flap. The large size of the flap compared to the relatively large opening allowed us to only use these two layers for reconstruction. Given the clival exposure we then placed a lumbar drain in this patient. She was discharged five days later. She had a slightly worsened hemiparesis and difficulty with eyelid opening, but that slowly improved over time and is now back to her preoperative baseline. Postoperative imaging shows a complete removal of the cavernous malformation with no new infarct.	4.054688	0.960938	sec[0]/p[0]	en	0.999997	PMC9541779	https://doi.org/10.3171/2019.10.FocusVid.19399	[ "this", "cavernous", "malformation", "access", "basilar", "carefully", "apex", "flap", "resection", "mamillary" ]	[ { "code": "4A01.03", "title": "Transient hypogammaglobulinaemia of infancy" }, { "code": "9C81.4", "title": "Cavernous sinus syndromes" }, { "code": "GB06.Y", "title": "Other specified disorders of penis" }, { "code": "LA90.10", "title": "Macrocystic lymphatic malformation" }, { "code": "8B22.43", "title": "Carotid cavernous fistula" }, { "code": "NA07.Y", "title": "Other specified intracranial injury" }, { "code": "LD9Z", "title": "Developmental anomalies, unspecified" }, { "code": "LD0Z", "title": "Structural developmental anomalies primarily affecting one body system, unspecified" }, { "code": "LA90.1Z", "title": "Lymphatic malformations, unspecified" }, { "code": "LB73.2Z", "title": "Structural developmental anomalies of spine, unspecified" } ]	=== ICD-11 CODES FOUND === [4A01.03] Transient hypogammaglobulinaemia of infancy Also known as: Transient hypogammaglobulinaemia of infancy \| immunoglobulin maturational delay \| THI - [transient hypogammaglobulinaemia of infancy] [9C81.4] Cavernous sinus syndromes Also known as: Cavernous sinus syndromes [GB06.Y] Other specified disorders of penis Also known as: Other specified disorders of penis \| Other inflammatory disorders of penis \| cavernositis \| Abscess of corpus cavernosum and penis \| abscess of corpus cavernosum [LA90.10] Macrocystic lymphatic malformation Definition: A condition caused by failure of the lymphatic system to correctly develop during the antenatal period. This condition is characterised by large, soft, smooth clear masses under normal or bluish skin. This condition may be associated with cellulitis, bleeding within the malformation, pain, or leakage of lymphatic fluid internally. Also known as: Macrocystic lymphatic malformation \| Cavernous lymphangioma \| Cavernous lymphatic malformation \| Macrocystic lymphangioma \| Circumscribed lymphatic malformation [8B22.43] Carotid cavernous fistula Definition: A carotid-cavernous fistula results from an abnormal communication between the arterial and venous systems within the cavernous sinus in the skull. Also known as: Carotid cavernous fistula [NA07.Y] Other specified intracranial injury Also known as: Other specified intracranial injury \| Traumatic intracranial haemorrhage, not elsewhere classified \| Traumatic cranium cavity haemorrhage \| traumatic intracranium haemorrhage \| Intracranial haematoma [LD9Z] Developmental anomalies, unspecified Also known as: Developmental anomalies, unspecified \| congenital malformations, deformations and chromosomal abnormalities \| congenital malformation NOS \| developmental abnormality NOS \| fetal abnormality NOS [LD0Z] Structural developmental anomalies primarily affecting one body system, unspecified Also known as: Structural developmental anomalies primarily affecting one body system, unspecified \| Structural malformations [LA90.1Z] Lymphatic malformations, unspecified Also known as: Lymphatic malformations, unspecified \| Lymphatic malformations \| Superficial haemosiderotic lymphovascular malformation \| Hobnail haemangioma \| Targetoid haemosiderotic haemangioma [LB73.2Z] Structural developmental anomalies of spine, unspecified Also known as: Structural developmental anomalies of spine, unspecified \| Structural developmental anomalies of spine \| Malformations of spine \| maldevelopment of spine === GRAPH WALKS === --- Walk 1 --- [4A01.03] Transient hypogammaglobulinaemia of infancy --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects... --CHILD--> [4A01.01] Immunodeficiencies with severe reduction in at least two serum immunoglobulin isotypes with normal or low numbers of B cells Def: This refers to a nonfamilial type of primary immune deficiency disease characterised by a reduction in at least two serum immunoglobulin isotypes. Circulating B cells may be normal or low.... --- Walk 2 --- [4A01.03] Transient hypogammaglobulinaemia of infancy --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects... --CHILD--> [4A01.00] Hereditary agammaglobulinaemia with profoundly reduced or absent B cells Def: This refers to a hereditary type of primary immune deficiency disease characterised by a reduction in all types of gamma globulins, and rare X-linked genetic disorder that affects the body's ability t... --- Walk 3 --- [9C81.4] Cavernous sinus syndromes --PARENT--> [9C81] Ocular motor nerve palsies --CHILD--> [9C81.0] Third nerve palsy --- Walk 4 --- [9C81.4] Cavernous sinus syndromes --PARENT--> [9C81] Ocular motor nerve palsies --CHILD--> [9C81.0] Third nerve palsy --- Walk 5 --- [GB06.Y] Other specified disorders of penis --PARENT--> [GB06] Certain specified disorders of penis --RELATED_TO--> [?] Male erectile dysfunction Def: Male erectile dysfunction is characterised by inability or marked reduction in the ability in men to attain or sustain a penile erection of sufficient duration or rigidity to allow for sexual activity... --- Walk 6 --- [GB06.Y] Other specified disorders of penis --PARENT--> [GB06] Certain specified disorders of penis --CHILD--> [GB06.1] Priapism Def: A condition of the penis, caused by acute leukaemia, sickle cell anaemia, infection, a penile or central nervous system lesion, or the use of certain pharmacological agents. This condition is characte...	[ "[4A01.03] Transient hypogammaglobulinaemia of infancy\n --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects\n Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects...\n --CHILD--> [4A01.01] Immunodeficiencies with severe reduction in at least two serum immunoglobulin isotypes with normal or low numbers of B cells\n Def: This refers to a nonfamilial type of primary immune deficiency disease characterised by a reduction in at least two serum immunoglobulin isotypes. Circulating B cells may be normal or low....", "[4A01.03] Transient hypogammaglobulinaemia of infancy\n --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects\n Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects...\n --CHILD--> [4A01.00] Hereditary agammaglobulinaemia with profoundly reduced or absent B cells\n Def: This refers to a hereditary type of primary immune deficiency disease characterised by a reduction in all types of gamma globulins, and rare X-linked genetic disorder that affects the body's ability t...", "[9C81.4] Cavernous sinus syndromes\n --PARENT--> [9C81] Ocular motor nerve palsies\n --CHILD--> [9C81.0] Third nerve palsy", "[9C81.4] Cavernous sinus syndromes\n --PARENT--> [9C81] Ocular motor nerve palsies\n --CHILD--> [9C81.0] Third nerve palsy", "[GB06.Y] Other specified disorders of penis\n --PARENT--> [GB06] Certain specified disorders of penis\n --RELATED_TO--> [?] Male erectile dysfunction\n Def: Male erectile dysfunction is characterised by inability or marked reduction in the ability in men to attain or sustain a penile erection of sufficient duration or rigidity to allow for sexual activity...", "[GB06.Y] Other specified disorders of penis\n --PARENT--> [GB06] Certain specified disorders of penis\n --CHILD--> [GB06.1] Priapism\n Def: A condition of the penis, caused by acute leukaemia, sickle cell anaemia, infection, a penile or central nervous system lesion, or the use of certain pharmacological agents. This condition is characte..." ]	4A01.03	Transient hypogammaglobulinaemia of infancy	[ { "from_icd11": "4A01.03", "icd10_code": "D807", "icd10_title": "Transient hypogammaglobulinemia of infancy" }, { "from_icd11": "LD9Z", "icd10_code": "Q898", "icd10_title": "Other specified congenital malformations" }, { "from_icd11": "LD9Z", "icd10_code": "Q899", "icd10_title": "Congenital malformation, unspecified" }, { "from_icd11": "LD9Z", "icd10_code": "Q89", "icd10_title": "Other congenital malformations, not elsewhere classified" }, { "from_icd11": "LD9Z", "icd10_code": "XVII", "icd10_title": "" }, { "from_icd11": "LD9Z", "icd10_code": "Q10-Q18", "icd10_title": "" }, { "from_icd11": "LD9Z", "icd10_code": "Q38-Q45", "icd10_title": "" }, { "from_icd11": "LD9Z", "icd10_code": "Q80-Q89", "icd10_title": "" }, { "from_icd11": "LD0Z", "icd10_code": "Q6412", "icd10_title": "Cloacal exstrophy of urinary bladder" }, { "from_icd11": "LD0Z", "icd10_code": "Q6419", "icd10_title": "Other exstrophy of urinary bladder" }, { "from_icd11": "LD0Z", "icd10_code": "Q6410", "icd10_title": "Exstrophy of urinary bladder, unspecified" }, { "from_icd11": "LD0Z", "icd10_code": "Q791", "icd10_title": "Other congenital malformations of diaphragm" }, { "from_icd11": "LD0Z", "icd10_code": "Q688", "icd10_title": "Other specified congenital musculoskeletal deformities" }, { "from_icd11": "LD0Z", "icd10_code": "Q799", "icd10_title": "Congenital malformation of musculoskeletal system, unspecified" }, { "from_icd11": "LD0Z", "icd10_code": "Q892", "icd10_title": "Congenital malformations of other endocrine glands" } ]	D807	Transient hypogammaglobulinemia of infancy
A 43-year-old female patient was admitted to our hospital in August 2024 after exhibiting consistently high blood glucose levels for 17 years and experiencing uncontrolled blood glucose for the past two months. Seventeen years ago, the patient was found to have increased blood glucose levels during a physical examination, but the specific value was not known. She was diagnosed with type 2 diabetes at a local hospital and was treated with oral hypoglycemic drugs (specific details unknown). After reviewing the medical records, we did not find any information pertaining to the patient from 17 years ago. However, we located the patient's medical records from six years ago, which showed the following: C-peptide (fasting) at 0.81 nmol/L, C-peptide (two-hour postprandial) at 0.94 nmol/L, insulin (fasting) at 257.5 pmol/L, and insulin (two-hour postprandial) at 300.79 pmol/L. The results for the antibodies against glutamic acid decarboxylase, anti-islet cell antibodies, and insulin autoantibodies were all negative. Ten years ago, Aspart Insulin 30 was prescribed for subcutaneous injection before meals due to elevated blood sugar levels. The dosage was set at 20 units for breakfast and 16 units for dinner. She also took voglibose 0.2 mg orally three times a day, metformin hydrochloride tablets 0.5 g orally twice a day, and sitagliptin 100 mg orally once a day to manage her blood sugar levels. Overall, her blood glucose control was deemed satisfactory. The patient had difficulty controlling her blood glucose levels two months ago for unknown reasons. Her self-monitoring of blood glucose (SMBG) reveals that her fasting blood glucose levels fluctuate between 180 and 216 mg/dL, while her postprandial blood glucose readings two hours after meals fluctuate between 234 and 288 mg/dL. She experienced palpitations, hunger, and hand tremors two to three times before meals, which improved after eating. However, she did not monitor her blood glucose levels during this time. To better manage her blood glucose, she sought consultation at our hospital, and the outpatient department diagnosed her with type 2 diabetes, admitting her for further treatment. The patient did not have a past record of hyperthyroidism and reported not using thiol-containing medications like thiamazole, alpha-lipoic acid, and glutathione. Upon physical examination, the following results were observed: temperature of 36.9°C, pulse of 96 beats per minute, respiration rate of 17 breaths per minute, blood pressure of 129/89 mmHg, medium stature, and a body mass index (BMI) of 21.33 kg/m². Decreased temperature sensation, pinprick sensation, and pressure sensation have been noted in both feet. After being admitted, the patient underwent the following additional tests: glycated hemoglobin A1c level was 7.0%, fasting insulin level was greater than 2,152.5 pmol/L, and two-hour postprandial insulin level was also greater than 2,152.5 pmol/L. After dilution, the insulin levels were retested and found to be 9,375.0 pmol/L for fasting and 14,770.0 pmol/L for two-hour postprandial. C-peptide levels were 0.75 nmol/L for fasting and 0.91 nmol/L for two-hour postprandial. Insulin antibodies were measured, and the insulin autoantibody level was greater than 400.0 RU/mL. The antibodies against glutamic acid decarboxylase and anti-islet cell antibodies were all negative. Urine routine test results showed no presence of glucose or ketone bodies. Triglycerides are at 1.07 mmol/L, total cholesterol is at 3.49 mmol/L, and low-density lipoprotein cholesterol is at 2.31 mmol/L. The electrophysiological examination indicates a decreased conduction velocity in the superficial peroneal nerve and sural nerve. A color Doppler ultrasound was performed on the liver, gallbladder, pancreas, spleen, and both kidneys, with no abnormalities noted in the pancreas. An abdominal CT scan also showed no abnormalities in the pancreas. Following admission, the patient was given 20 units of Insulin Aspart 30 Injection subcutaneously before breakfast and 16 units before dinner, along with 0.2 mg of Voglibose orally three times a day, 0.5g of Metformin Hydrochloride Tablets orally twice a day, and 100 mg of Sitagliptin Tablet orally once a day for hypoglycemic treatment. Despite adjustments to the dosage, the patient's blood glucose levels remained unstable, with varying fasting readings between 6.2 and 9.9 mmol/L and two-hour postprandial readings between 3.5 and 16.6 mmol/L. Due to concerns about IAS, Insulin Aspart 30 Injection was discontinued and replaced with Insulin Degludec/Insulin Aspart Injection, leading to a significant improvement in blood glucose levels within two days. The insulin dosage was gradually decreased to prevent hypoglycemia, with the patient being discharged on 15 units before breakfast and 10 units before dinner of Insulin Degludec/Insulin Aspart Injection. The oral medication regimen was also simplified to 0.2 mg of Voglibose three times a day and one tablet of Sitagliptin Metformin (50 mg-0.5 g) twice a day. Following the switch in insulin, the patient no longer experienced symptoms such as palpitations, hand tremors, or hunger. Following discharge, the patient gradually decreased their dose of Insulin Degludec/Insulin Aspart Injection to 12 units before breakfast and eight units before dinner. Their fasting blood glucose remained stable at around 4-5 mmol/L, while two-hour postprandial blood glucose levels ranged between 5 and 8 mmol/L, showing a continued decrease in blood glucose levels. A retest of insulin levels conducted three weeks after switching insulin types showed elevated levels: fasting insulin > 2,152.5 pmol/L, two-hour insulin > 2,152.5 pmol/L. The insulin levels were re-monitored after eight weeks. The fasting insulin level was 8,244.0 pmol/L, and the two-hour postprandial insulin level was 12,650.0 pmol/L. The slight decrease in the patient's insulin levels may be attributed to the brief discontinuation of Insulin Aspart 30. In conclusion, after a decade of Insulin Aspart 30 Injection use, the patient experienced significant increases in fasting and postprandial blood glucose levels. Monitoring of insulin and C-peptide levels revealed elevated insulin autoantibody levels. Switching insulin types led to reduced dosage and stable blood glucose levels within the desired range, clearly indicating a diagnosis of EIAS in this patient.	3.953125	0.979004	sec[1]/p[0]	en	0.999994	39575003	https://doi.org/10.7759/cureus.72067	[ "insulin", "blood", "glucose", "fasting", "postprandial", "pmol", "hour", "aspart", "injection", "mmol" ]	[ { "code": "5A44", "title": "Insulin-resistance syndromes" }, { "code": "5A4Y", "title": "Other specified disorders of glucose regulation or pancreatic internal secretion" }, { "code": "QB51.5", "title": "Presence of endocrine implants" }, { "code": "EF02.0", "title": "Fat hypertrophy" }, { "code": "PK9C.2", "title": "Other or unspecified medical devices associated with injury or harm, prosthetic or other implants, materials or accessory devices" }, { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" }, { "code": "MA12.1", "title": "Finding of cocaine in blood" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "MA12.2", "title": "Finding of hallucinogen in blood" } ]	=== ICD-11 CODES FOUND === [5A44] Insulin-resistance syndromes Also known as: Insulin-resistance syndromes \| Insulin-resistance syndrome type A \| Insulin-resistance syndrome type B \| Rabson-Mendenhall syndrome \| Laminopathy type Decaudain-Vigouroux [5A4Y] Other specified disorders of glucose regulation or pancreatic internal secretion Also known as: Other specified disorders of glucose regulation or pancreatic internal secretion \| Other hypoglycaemia \| Hyperinsulinaemia \| hyperinsulinism \| functional hyperinsulinaemia [QB51.5] Presence of endocrine implants Also known as: Presence of endocrine implants \| presence of insulin pump Includes: presence of insulin pump [EF02.0] Fat hypertrophy Definition: Focal hypertrophy of subcutaneous adipose tissue. It is a common sequela of long-term insulin injection into the skin. Also known as: Fat hypertrophy \| Insulin-induced localised fat hypertrophy \| Insulin-induced lipohypertrophy [PK9C.2] Other or unspecified medical devices associated with injury or harm, prosthetic or other implants, materials or accessory devices Also known as: Other or unspecified medical devices associated with injury or harm, prosthetic or other implants, materials or accessory devices \| Surgical operation with implant of other or unspecified artificial internal device associated with abnormal reaction of the patient, or of later complication, without mention of misadventure at the time of the procedure \| Mechanical complication of other specified internal prosthetic devices, implants and grafts \| Mechanical complication of insulin pump Excludes: Circumstances associated with a surgical or other medical device influencing the episode of care without injury or harm [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS [MA12.1] Finding of cocaine in blood Also known as: Finding of cocaine in blood \| cocaine in blood [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [MA12.2] Finding of hallucinogen in blood Also known as: Finding of hallucinogen in blood \| hallucinogen in blood === GRAPH WALKS === --- Walk 1 --- [5A44] Insulin-resistance syndromes --PARENT--> [?] Other disorders of glucose regulation or pancreatic internal secretion --RELATED_TO--> [?] Somatostatinoma Def: A rare, usually malignant neuroendocrine tumour arizing from delta cells. This neoplasm produces large amounts of somatostatin, which may result in a syndrome characterised by diarrhea, steatorrhea, w... --- Walk 2 --- [5A44] Insulin-resistance syndromes --PARENT--> [?] Other disorders of glucose regulation or pancreatic internal secretion --EXCLUDES--> [?] Malignant neoplasm of pancreas Def: A primary malignant tumour of the pancreas. Most are adenocarcinomas.... --- Walk 3 --- [5A4Y] Other specified disorders of glucose regulation or pancreatic internal secretion --PARENT--> [?] Other disorders of glucose regulation or pancreatic internal secretion --RELATED_TO--> [?] PPoma Def: A benign or malignant neuroendocrine tumour that arises from the pancreas and secretes pancreatic polypeptide.... --- Walk 4 --- [5A4Y] Other specified disorders of glucose regulation or pancreatic internal secretion --PARENT--> [?] Other disorders of glucose regulation or pancreatic internal secretion --EXCLUDES--> [?] Malignant neoplasm of pancreas Def: A primary malignant tumour of the pancreas. Most are adenocarcinomas.... --- Walk 5 --- [QB51.5] Presence of endocrine implants --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants --EXCLUDES--> [?] Fitting, adjustment or management of devices --- Walk 6 --- [QB51.5] Presence of endocrine implants --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants --PARENT--> [?] Presence of device, implants or grafts	[ "[5A44] Insulin-resistance syndromes\n --PARENT--> [?] Other disorders of glucose regulation or pancreatic internal secretion\n --RELATED_TO--> [?] Somatostatinoma\n Def: A rare, usually malignant neuroendocrine tumour arizing from delta cells. This neoplasm produces large amounts of somatostatin, which may result in a syndrome characterised by diarrhea, steatorrhea, w...", "[5A44] Insulin-resistance syndromes\n --PARENT--> [?] Other disorders of glucose regulation or pancreatic internal secretion\n --EXCLUDES--> [?] Malignant neoplasm of pancreas\n Def: A primary malignant tumour of the pancreas. Most are adenocarcinomas....", "[5A4Y] Other specified disorders of glucose regulation or pancreatic internal secretion\n --PARENT--> [?] Other disorders of glucose regulation or pancreatic internal secretion\n --RELATED_TO--> [?] PPoma\n Def: A benign or malignant neuroendocrine tumour that arises from the pancreas and secretes pancreatic polypeptide....", "[5A4Y] Other specified disorders of glucose regulation or pancreatic internal secretion\n --PARENT--> [?] Other disorders of glucose regulation or pancreatic internal secretion\n --EXCLUDES--> [?] Malignant neoplasm of pancreas\n Def: A primary malignant tumour of the pancreas. Most are adenocarcinomas....", "[QB51.5] Presence of endocrine implants\n --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants\n --EXCLUDES--> [?] Fitting, adjustment or management of devices", "[QB51.5] Presence of endocrine implants\n --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants\n --PARENT--> [?] Presence of device, implants or grafts" ]	5A44	Insulin-resistance syndromes	[ { "from_icd11": "5A44", "icd10_code": "E10-E14", "icd10_title": "" }, { "from_icd11": "QB51.5", "icd10_code": "Z9641", "icd10_title": "Presence of insulin pump (external) (internal)" }, { "from_icd11": "QB51.5", "icd10_code": "Z964", "icd10_title": "Presence of endocrine implants" }, { "from_icd11": "EF02.0", "icd10_code": "L988", "icd10_title": "Other specified disorders of the skin and subcutaneous tissue" }, { "from_icd11": "EF02.0", "icd10_code": "E881", "icd10_title": "Lipodystrophy, not elsewhere classified" }, { "from_icd11": "PK9C.2", "icd10_code": "T85694A", "icd10_title": "Other mechanical complication of insulin pump, initial encounter" }, { "from_icd11": "PK9C.2", "icd10_code": "T85614A", "icd10_title": "Breakdown (mechanical) of insulin pump, initial encounter" }, { "from_icd11": "PK9C.2", "icd10_code": "T85624A", "icd10_title": "Displacement of insulin pump, initial encounter" }, { "from_icd11": "PK9C.2", "icd10_code": "T85618A", "icd10_title": "Breakdown (mechanical) of other specified internal prosthetic devices, implants and grafts, initial encounter" }, { "from_icd11": "PK9C.2", "icd10_code": "T85628A", "icd10_title": "Displacement of other specified internal prosthetic devices, implants and grafts, initial encounter" }, { "from_icd11": "PK9C.2", "icd10_code": "T85621A", "icd10_title": "Displacement of intraperitoneal dialysis catheter, initial encounter" }, { "from_icd11": "PK9C.2", "icd10_code": "T85611A", "icd10_title": "Breakdown (mechanical) of intraperitoneal dialysis catheter, initial encounter" }, { "from_icd11": "PK9C.2", "icd10_code": "T85698A", "icd10_title": "Other mechanical complication of other specified internal prosthetic devices, implants and grafts, initial encounter" }, { "from_icd11": "PK9C.2", "icd10_code": "T85638A", "icd10_title": "Leakage of other specified internal prosthetic devices, implants and grafts, initial encounter" }, { "from_icd11": "PK9C.2", "icd10_code": "T85610A", "icd10_title": "Breakdown (mechanical) of cranial or spinal infusion catheter, initial encounter" } ]	E10-E14
We describe the case of a 79-years-old female patient born in the Azores Islands, living in mainland Portugal since her twenties, who was diagnosed with recurrent symptomatic hydronephrosis due to left nephrolithiasis in 1969, at the age of 25 years. She had no relevant past or family history. After 15 years of recurrent symptomatic hydronephrosis, the patient underwent unilateral nephrectomy. At the age of 50 years, she began to be followed at our institution. The diagnosis of multinodular goiter and primary hyperparathyroidism was established, with serum calcium (sCa) of 11.5 mg/dL (normal range: 8.4–10.2), parathyroid hormone (PTH) of 264 pg/mL (normal range: 10–60), and normal 25- and 1,25-hydroxyvitamin D. She was also diagnosed with autoimmune pernicious anemia with positive anti-intrinsic factor antibodies and anti-parietal cell antibodies, and primary autoimmune hypothyroidism with high titers of anti-thyroperoxidase antibodies (>10,000 IU/mL; normal range: <35). She was started on levothyroxine 50 μ/day, oral vitamin B12, and iron supplements. Fine-needle aspiration of the multinodular goiter revealed lymphocytic thyroiditis. Due to hypercalcemia and multiple thyroid nodules, she underwent subtotal thyroidectomy, leaving a remnant of the left lobe as well as right and left inferior parathyroidectomy. Histology confirmed the presence of Hashimoto's thyroiditis and parathyroid adenoma with lymphocytic infiltration. At 58 years of age, she underwent esophagogastroduodenoscopy due to dyspepsia, which showed multiple small (>20) red, sessile polyps in the gastric body and fundus. Biopsy of the lesions revealed a well-differentiated G1 NET (Ki-67 of 1%) in the body of the stomach, with associated lesions of chronic atrophic gastritis and intestinal metaplasia. Computed tomography (CT) revealed bilateral metastatic lung lesions, which showed high uptake in the [111In]-octreotide scintigraphy. CT-guided lung biopsy of these lesions confirmed a neuroendocrine origin. Increased serum levels of chromogranin A were documented (820 nmol/L; normal range: <45), and gastrin levels were also high . The patient was started on octreotide (20 mg/month). At this point, MEN1 was suspected, and biochemical screening of pituitary function revealed a high serum prolactin level of 56 ng/mL (normal range < 20) and inappropriately low FSH (<0.1 UI/L) and LH (<0.3UI/L) levels in menopausal women. Further investigation revealed that hyperprolactinemia was due to macroprolactinemia (recovery of monomeric prolactin after precipitation with polyethylene glycol: 9%; normal >40%). The remaining pituitary function and pituitary magnetic resonance imaging (MRI) scan were normal. A presumptive diagnosis of autoimmune hypophysitis has been proposed. After informed consent was obtained from the patient, genetic MEN1 screening performed with single-strand conformational polymorphism (SSCP) was negative. At 61 years of age, hypercalcemia recurred, and a lesion in the topography of the left upper parathyroid was found by technetium-99m sestamibi scintigraphy. Three years later, due to an increase in sCa (12.2 mg/dL) and PTH (191 pg/mL), the patient underwent removal of the left upper parathyroid, along with an enlarged left thyroid lobe. Histological examination showed an 18-mm parathyroid adenoma with lymphocytic infiltration and low-grade mucosa-associated lymphoid tissue (MALT) B lymphoma of the thyroid gland. Serum anti-calcium sensor receptor (CaSR) antibodies were positive. A few months later, the patient underwent urgent cholecystectomy for acute cholecystitis, probably because of the prolonged use of somatostatin analogs. She remained under surveillance without any new manifestations until she was 68 years old when, due to abnormal uterine bleeding, she underwent a pelvic ultrasound that revealed two large uterine leiomyomas. At 69-years-old the patient was diagnosed with a left breast invasive carcinoma and underwent successful surgery followed by tamoxifen therapy, which was later discontinued. At this time, due to the high clinical probability of MEN1, after further informed consent from the patient her leukocyte DNA was analyzed by next-generation sequencing (TruSight Cancer Sequencing panel, Illumina), and a novel heterozygous germline mutation in exon 9 of the MEN1 gene was found, which is expected to lead to a duplication of the tryptophan residue (p.Trp441dup) of MENIN. This variant was absent from controls in the databases ALFA Allele Frequency, 1000 Genomes Project, or ExAC. In silico analysis performed using the Mutation Taster software tool classified this variant as disease-causing. The altered amino acid is localized to a highly conserved site during evolution. Based on the guidelines of the American College of Medical Genetics and Genomics (ACMG), this variant is classified as likely pathogenic. NGS analysis also detected a variant in the Cell Division Cycle 73 ( CDC73 ) gene (c.-4_-11insG). This variant is described in the database ALFA Allele Frequency and gnomAD exomes, with an allele frequency of 0.21% (European population) and 1.02% (non-Finnish European), respectively. The in silico analysis performed using the Mutation Taster software tool classified this variant as disease-causing. Sequence alignment of CDC73 encoding the proximal 5’UTR revealed that this region showed limited phylogenetic conservation. This variant has eight submissions in ClinVar: benign (5) and uncertain significance (3). Based on the ACMG guidelines, this variant is classified as benign. Due to the multiple autoimmune conditions of this patient (pernicious anemia, lymphocytic thyroiditis, macroprolactinemia, possible lymphocytic hypophysitis, anti-CaSr antibodies), the autoimmune regulator ( AIRE ) gene was sequenced, but no pathogenic mutations were found. Breast cancer ( BRCA ) genes 1/2 testing was negative. Recently, a 68 Gallium-DOTANOC position emission tomography/CT showed two foci of increased uptake in the pancreatic body and tail. The patient refused abdominal MRI due to claustrophobia, and an abdominal CT scan did not detect any pancreatic lesions. Since then, she has been clinically stable, without evidence of recurrent primary hyperparathyroidism or progression of her metastatic gastric neuroendocrine tumor. The patient was kept under octreotide 20 mg/month because of intolerance to higher doses.	4.082031	0.976074	sec[1]/p[0]	en	0.999997	34889280	https://doi.org/10.1097/MD.0000000000028145	[ "this", "variant", "range", "parathyroid", "autoimmune", "anti", "antibodies", "lymphocytic", "lesions", "serum" ]	[ { "code": "4A01.03", "title": "Transient hypogammaglobulinaemia of infancy" }, { "code": "4A00.0Y", "title": "Other specified functional neutrophil defects" }, { "code": "8A40.Y", "title": "Other specified multiple sclerosis" }, { "code": "8E4A.0", "title": "Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord" }, { "code": "8E01.2", "title": "Variant Creutzfeldt-Jakob Disease" }, { "code": "5C56.00", "title": "Gangliosidosis" }, { "code": "QA00.6Y", "title": "Other specified examination of eyes or vision" }, { "code": "4B00.0Z", "title": "Neutropaenia, unspecified" }, { "code": "3B63.1Z", "title": "Acquired thrombocytosis, unspecified" }, { "code": "MA14.1C", "title": "Raised antibody titre" } ]	=== ICD-11 CODES FOUND === [4A01.03] Transient hypogammaglobulinaemia of infancy Also known as: Transient hypogammaglobulinaemia of infancy \| immunoglobulin maturational delay \| THI - [transient hypogammaglobulinaemia of infancy] [4A00.0Y] Other specified functional neutrophil defects Also known as: Other specified functional neutrophil defects \| Chronic granulomatous disease \| Chronic septic granulomatosis \| CGD - [chronic granulomatous disease] \| chronic granulomatous disorder [8A40.Y] Other specified multiple sclerosis Also known as: Other specified multiple sclerosis \| Certain specified rare variants of multiple sclerosis \| Multiple sclerosis, Marburg variant \| Myelinoclastic diffuse sclerosis \| Schilder disease [8E4A.0] Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord Definition: Paraneoplastic and autoimmune disorders of the central nervous system, brain and spinal cord nervous system result from a targeted immune attack on neurons or glial cells in the central (e.g. encephalopathy, ataxia, myelopathy, myelitis) nervous system. In the paraneoplastic context, this attack is a consequence of a potentially effective tumour immune response initiated by onco-neural antigens derived from a systemic cancer. In the non-paraneoplastic context termed ‘autoimmune’ the etiology rem Also known as: Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord \| Paraneoplastic encephalitis \| Paraneoplastic encephalitis, neural autoantibody positive \| Paraneoplastic encephalitis, neural autoantibody negative \| Autoimmune encephalitis [8E01.2] Variant Creutzfeldt-Jakob Disease Definition: A disease of the brain, that is suspected to be caused by a prion associated with Bovine Spongiform Encephalopathy. This disease is characterised by a long incubation period, psychiatric symptoms followed by neurological deficits, and is fatal. Transmission may be by ingestion of food (with a bovine origin) contaminated with infected brain or spinal cord from an infected cow, or blood transfusion. Confirmation is by pathological examination of the brain. Also known as: Variant Creutzfeldt-Jakob Disease \| vCJD - [Variant Creutzfeldt-Jakob Disease] [5C56.00] Gangliosidosis Also known as: Gangliosidosis \| GM1 gangliosidosis \| Landing disease \| GM1 gangliosidosis type 1 \| Generalised gangliosidosis [QA00.6Y] Other specified examination of eyes or vision Also known as: Other specified examination of eyes or vision \| No Impairment of Contrast vision \| Normal colour vision \| No Impairment of Dark adaptation \| No diplopia [4B00.0Z] Neutropaenia, unspecified Also known as: Neutropaenia, unspecified \| Neutropenia \| Disorders with decreased neutrophil counts \| neutropaenic disorder \| neutrophil count below reference range [3B63.1Z] Acquired thrombocytosis, unspecified Also known as: Acquired thrombocytosis, unspecified \| Acquired thrombocytosis \| Idiopathic haemorrhagic thrombocythaemia \| Essential thrombocythaemia \| primary haemorrhagic thrombocythaemia [MA14.1C] Raised antibody titre Also known as: Raised antibody titre \| antibody titre above reference range \| high antibody titre \| increased antibody titre Excludes: isoimmunization, in pregnancy affecting fetus or newborn === GRAPH WALKS === --- Walk 1 --- [4A01.03] Transient hypogammaglobulinaemia of infancy --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects... --CHILD--> [4A01.01] Immunodeficiencies with severe reduction in at least two serum immunoglobulin isotypes with normal or low numbers of B cells Def: This refers to a nonfamilial type of primary immune deficiency disease characterised by a reduction in at least two serum immunoglobulin isotypes. Circulating B cells may be normal or low.... --- Walk 2 --- [4A01.03] Transient hypogammaglobulinaemia of infancy --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects... --CHILD--> [4A01.01] Immunodeficiencies with severe reduction in at least two serum immunoglobulin isotypes with normal or low numbers of B cells Def: This refers to a nonfamilial type of primary immune deficiency disease characterised by a reduction in at least two serum immunoglobulin isotypes. Circulating B cells may be normal or low.... --- Walk 3 --- [4A00.0Y] Other specified functional neutrophil defects --PARENT--> [4A00.0] Functional neutrophil defects --RELATED_TO--> [?] Haemolytic anaemia due to glucose-6-phosphate dehydrogenase deficiency Def: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary erythrocyte enzyme deficiency that can manifest with severe neonatal jaundice which can lead to serious neurological c... --- Walk 4 --- [4A00.0Y] Other specified functional neutrophil defects --PARENT--> [4A00.0] Functional neutrophil defects --RELATED_TO--> [?] Haemolytic anaemia due to glucose-6-phosphate dehydrogenase deficiency Def: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary erythrocyte enzyme deficiency that can manifest with severe neonatal jaundice which can lead to serious neurological c... --- Walk 5 --- [8A40.Y] Other specified multiple sclerosis --PARENT--> [8A40] Multiple sclerosis Def: Multiple Sclerosis (MS) is a chronic, inflammatory demyelinating disease of the central nervous system. Three categories of multiple sclerosis have been outlined: Relapsing/remitting, secondary progre... --CHILD--> [8A40.0] Relapsing-remitting multiple sclerosis Def: Clearly defined disease relapses with full recovery or with sequelae and residual deficit upon recovery. The periods between disease relapses are characterised by a lack of disease progression.... --- Walk 6 --- [8A40.Y] Other specified multiple sclerosis --PARENT--> [8A40] Multiple sclerosis Def: Multiple Sclerosis (MS) is a chronic, inflammatory demyelinating disease of the central nervous system. Three categories of multiple sclerosis have been outlined: Relapsing/remitting, secondary progre... --CHILD--> [8A40.0] Relapsing-remitting multiple sclerosis Def: Clearly defined disease relapses with full recovery or with sequelae and residual deficit upon recovery. The periods between disease relapses are characterised by a lack of disease progression....	[ "[4A01.03] Transient hypogammaglobulinaemia of infancy\n --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects\n Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects...\n --CHILD--> [4A01.01] Immunodeficiencies with severe reduction in at least two serum immunoglobulin isotypes with normal or low numbers of B cells\n Def: This refers to a nonfamilial type of primary immune deficiency disease characterised by a reduction in at least two serum immunoglobulin isotypes. Circulating B cells may be normal or low....", "[4A01.03] Transient hypogammaglobulinaemia of infancy\n --PARENT--> [4A01.0] Immunodeficiencies with predominantly antibody defects\n Def: A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects...\n --CHILD--> [4A01.01] Immunodeficiencies with severe reduction in at least two serum immunoglobulin isotypes with normal or low numbers of B cells\n Def: This refers to a nonfamilial type of primary immune deficiency disease characterised by a reduction in at least two serum immunoglobulin isotypes. Circulating B cells may be normal or low....", "[4A00.0Y] Other specified functional neutrophil defects\n --PARENT--> [4A00.0] Functional neutrophil defects\n --RELATED_TO--> [?] Haemolytic anaemia due to glucose-6-phosphate dehydrogenase deficiency\n Def: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary erythrocyte enzyme deficiency that can manifest with severe neonatal jaundice which can lead to serious neurological c...", "[4A00.0Y] Other specified functional neutrophil defects\n --PARENT--> [4A00.0] Functional neutrophil defects\n --RELATED_TO--> [?] Haemolytic anaemia due to glucose-6-phosphate dehydrogenase deficiency\n Def: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary erythrocyte enzyme deficiency that can manifest with severe neonatal jaundice which can lead to serious neurological c...", "[8A40.Y] Other specified multiple sclerosis\n --PARENT--> [8A40] Multiple sclerosis\n Def: Multiple Sclerosis (MS) is a chronic, inflammatory demyelinating disease of the central nervous system. Three categories of multiple sclerosis have been outlined: Relapsing/remitting, secondary progre...\n --CHILD--> [8A40.0] Relapsing-remitting multiple sclerosis\n Def: Clearly defined disease relapses with full recovery or with sequelae and residual deficit upon recovery. The periods between disease relapses are characterised by a lack of disease progression....", "[8A40.Y] Other specified multiple sclerosis\n --PARENT--> [8A40] Multiple sclerosis\n Def: Multiple Sclerosis (MS) is a chronic, inflammatory demyelinating disease of the central nervous system. Three categories of multiple sclerosis have been outlined: Relapsing/remitting, secondary progre...\n --CHILD--> [8A40.0] Relapsing-remitting multiple sclerosis\n Def: Clearly defined disease relapses with full recovery or with sequelae and residual deficit upon recovery. The periods between disease relapses are characterised by a lack of disease progression...." ]	4A01.03	Transient hypogammaglobulinaemia of infancy	[ { "from_icd11": "4A01.03", "icd10_code": "D807", "icd10_title": "Transient hypogammaglobulinemia of infancy" }, { "from_icd11": "8E4A.0", "icd10_code": "G3183", "icd10_title": "Dementia with Lewy bodies" }, { "from_icd11": "8E4A.0", "icd10_code": "G2581", "icd10_title": "Restless legs syndrome" }, { "from_icd11": "8E4A.0", "icd10_code": "G3184", "icd10_title": "Mild cognitive impairment, so stated" }, { "from_icd11": "8E4A.0", "icd10_code": "G9349", "icd10_title": "Other encephalopathy" }, { "from_icd11": "8E4A.0", "icd10_code": "G0481", "icd10_title": "Other encephalitis and encephalomyelitis" }, { "from_icd11": "8E4A.0", "icd10_code": "G9589", "icd10_title": "Other specified diseases of spinal cord" }, { "from_icd11": "8E4A.0", "icd10_code": "G2589", "icd10_title": "Other specified extrapyramidal and movement disorders" }, { "from_icd11": "8E4A.0", "icd10_code": "G3189", "icd10_title": "Other specified degenerative diseases of nervous system" }, { "from_icd11": "8E4A.0", "icd10_code": "G2582", "icd10_title": "Stiff-man syndrome" }, { "from_icd11": "8E4A.0", "icd10_code": "G0489", "icd10_title": "Other myelitis" }, { "from_icd11": "8E4A.0", "icd10_code": "G9581", "icd10_title": "Conus medullaris syndrome" }, { "from_icd11": "8E4A.0", "icd10_code": "G3185", "icd10_title": "Corticobasal degeneration" }, { "from_icd11": "8E4A.0", "icd10_code": "G3181", "icd10_title": "Alpers disease" }, { "from_icd11": "8E4A.0", "icd10_code": "G3182", "icd10_title": "Leigh's disease" } ]	D807	Transient hypogammaglobulinemia of infancy
A 35-year-old male with end-stage renal disease of unknown cause underwent cadaveric renal transplantation in our department 6 years ago. Due to a lack of kidney biopsy, we did not know the exact etiology causing him to develop an end-stage renal disease. The postoperative immunosuppression regimen consisted of tacrolimus, mycophenolate mofetil and prednisolone. The patient was followed at another clinic at approximately 8-month intervals. There was no obvious abnormality except for medium microscopic haematuria for his latest follow-up. Unfortunately, the doctor in that clinic did not recommend further examination, so we had no idea about the source or morphology of the red blood cells in urine. The patient did not have a history of hypertension, diabetes or hyperparathyroidism. He had a desk job, so he might have been too sedentary. He did not have a bad lifestyle, such as drinking alcohol or smoking, except for drinking Coca-Cola (1 can per day) for 3 to 4 years. On the 6th year after transplantation, the patient suddenly fevered with a temperature of 38.8 °C and shiver, accompanied by oliguria with 400–500 mL urine/d and little gross haematuria. He was admitted to emergency department. Physical examination indicated a heart rate (HR) of 92 bpm, blood pressure (BP) of 131/88 mmHg, Body Mass Index (BMI) of 27.5 kg/m 2 , and mild tenderness in the graft area. Blood chemical test showed a white blood cell count (WBC) of 7.37 × 10 9 /L, lymphocytes 11%, neutrophils 79%, serum creatinine 4.04 mg/dL, blood glucose 109.8 mg/dL, serum uric acid 6.4 mg/dL, blood calcium 8.90 mg/dL, serum albumin 2.73 g/dL, and corrected calcium 9.90 mg/dL. The pH value of the urine was 6.2. Computerized tomography (CT) showed that there was a stone with a size of 18 mm in the ureteropelvic junction, which caused mild hydronephrosis . An emergent operation was arranged in combination with efficient antimicrobial therapy. A ureteroscope (URS) was performed in the lithotomy position under general anesthesia. We could not insert the guide wire into the new orifice with a 70° lens ureteroscope, although we switched to a semirigid ureteroscope. Therefore, we had to perform percutaneous nephrolithotripsy (PCNL) to remove the stones. The anterior calyx in the upper pole was chosen for puncture under ultrasonography guidance. An nephroscope was inserted through the sheath to inspect the pelvicalyceal system, and a 9.5–10 Fr flexible URS was used to inspect the ureter. A brown stone of 18 mm was located in the ureteropelvic junction. The stone was fragmented and extracted. After the stone was confirmed free by intraoperative ultrasonography, a 4.8 Fr double-pigtail stent was introduced. A 14 Fr nephrostomy tube was placed. After operation, the patient’s condition improved quickly, with normal temperature and gradually increased urine , and serum creatinine decreased to 1.65 mg/dL. The nephrostomy tube was removed 3 days later. The patient was discharged 10 days postoperation with normal urine and serum creatinine, and a stone-free condition was confirmed by ultrasonography. A double-pigtail stent was removed 4 weeks later. After discharge, the patient was followed up every 3 months and then every 6 months 1 year later. There were no abnormalities during follow-up. Unexpectedly, the patient was admitted to the hospital again 16 months after the operation for similar symptoms, including fever (39 °C), shivers, and anuria, but without pain. Blood chemical tests indicated severe infection and ARF (WBC 8.14 × 10 9 /L, lymphocytes 11.2%, neutrophils 84.2%, and serum creatine 6.68 mg/dL), blood glucose was 113.4 mg/dL, serum uric acid was 6.6 mg/dL, blood calcium was 8.82 mg/dL, serum albumin was 2.55 g/dL, and corrected calcium was 10.0 mg/dL. The pH value of the urine was 5.7. CT demonstrated a 12 mm calculus in the proximal ureter with severe extension of the ureter and hydronephrosis . A 14 Fr percutaneous nephrostomy tube was first placed emergently to promptly decompress and relieve the symptoms; in this case, an interpolar middle calyx was accessed. The patient gradually recovered, with normal temperature and approximately 1800 mL urine/day through the nephrostomy tube, and serum creatinine decreased to 3.58 mg/dL. After the improvement of allograft function, PCNL was performed. The nephroscope and flexible URS were advanced into the pelvicalyceal system or ureter through the existing access tract. Holmium laser lithotripsy was performed to fragment the yellow-brown solid calculus located in the ureter, and fragments were completely removed. After confirming that all stones were taken out, a 16 Fr nephrostomy tube was placed, without introducing a double-pigtail stent. After the operation, allograft function was further improved, with urine 1500 mL/day and serum creatine of 1.33 mg/dL. The percutaneous nephrostomy tube was removed 4 days later. Analysis of stone composition indicated a uric acid calculus, so potassium sodium hydrogen citrate (2.5 g, 3 times a day) was administered. On the 19th day postoperation, serum creatine decreased to 1.07 mg/dL. Ultrasonography indicated hyperechogenicity in the kidney. Therefore, CT was performed again and indicated that there was a small residual stone with a size of 3 mm in the calyx . The patient had normal urine and renal function without pain or haematuria, and he was discharged with strict follow-up in the clinic. As the BMI of the patient was slightly high, we recommended that he lose some weight, do some exercise, and drink more water instead of Coca-Cola. Fortunately, the calculus passed through the urine tract spontaneously about 1 month later. Now the patient is healthy with normal allograft function and free of renal transplant lithiasis. Fig. 1 Preoperative CT scan showing a ureteral calculus in the ureteropelvic junction. a Coronal plane, b transverse plane Fig. 2 Preoperative CT scan showing a proximal ureteral calculus with severe hydronephrosis and extension of the ureter. a Longitudinal plane left view, b longitudinal plane right view, c transverse plane Fig. 3 Postoperative CT scan showing a residual calculus in the lateral calyx. a Transverse plane. The red arrowhead shows the percutaneous nephrostomy tube, b Longitudinal plane. The red arrowhead shows the percutaneous nephrostomy tube, c Transverse plane. The red arrow shows the residual calculus	3.890625	0.980469	sec[2]/sec[0]/p[0]	en	0.999997	32576135	https://doi.org/10.1186/s12882-020-01896-5	[ "serum", "urine", "blood", "nephrostomy", "tube", "calculus", "plane", "stone", "ureter", "renal" ]	[ { "code": "NE80.3", "title": "Other serum reactions" }, { "code": "5D0Y", "title": "Other specified metabolic disorders" }, { "code": "5B91.0", "title": "Hypercalcaemia" }, { "code": "4A84.Y", "title": "Other specified anaphylaxis" }, { "code": "5C50.F2", "title": "Homocarnosinosis" }, { "code": "MF9Y", "title": "Other specified clinical findings on examination of urine, without diagnosis" }, { "code": "MF50.6Z", "title": "Difficulties with micturition, unspecified" }, { "code": "MF50.0", "title": "Frequent micturition" }, { "code": "MF50.3", "title": "Retention of urine" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" } ]	=== ICD-11 CODES FOUND === [NE80.3] Other serum reactions Also known as: Other serum reactions \| Allergic reaction to serum \| serum allergy \| Complications of vaccination, protein sickness \| Protein sickness Excludes: serum hepatitis [5D0Y] Other specified metabolic disorders Also known as: Other specified metabolic disorders \| Disorders of plasma-protein metabolism, not elsewhere classified \| abnormal protein transport \| dysproteinaemia \| Absence of albumin in blood [5B91.0] Hypercalcaemia Definition: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused by dehydration secondary to urinary losses of calcium, water and other electrolytes, and to an increase in membrane potential caused by the elevation in extracellular fluid ionized calcium concentration. Patients with moderate to severe hypercalcaemia often complain of nausea and vomiting, symptoms Also known as: Hypercalcaemia \| Calcium excess \| elevated serum calcium \| hypercalcaemic crisis \| hypercalcaemic syndrome [4A84.Y] Other specified anaphylaxis Also known as: Other specified anaphylaxis \| Latex-induced anaphylaxis \| Anaphylaxis due to latex \| Latex anaphylaxis \| Anaphylactic shock due to serum [5C50.F2] Homocarnosinosis Definition: Homocarnosinosis is a metabolic defect characterised by progressive spastic diplegia, intellectual deficit and retinitis pigmentosa. This extremely rare disorder has been reported in only one family, namely a woman and three of her children. The latter showed but their mother was symptom free. It is therefore uncertain whether there is a relationship between the biochemical defect and the clinical symptoms. Inheritance in the reported family seems to be autosomal dominant. Also known as: Homocarnosinosis \| Homocarnosinase deficiency \| Serum carnosinase deficiency [MF9Y] Other specified clinical findings on examination of urine, without diagnosis Also known as: Other specified clinical findings on examination of urine, without diagnosis \| Methaemoglobinuria \| Other and unspecified abnormal findings in urine \| Calciuria \| Cells and casts in urine [MF50.6Z] Difficulties with micturition, unspecified Also known as: Difficulties with micturition, unspecified \| Other difficulties with micturition \| difficulty passing urine NOS \| difficulty urinating NOS \| other difficulties with urination [MF50.0] Frequent micturition Definition: Needing to urinate more often than normal. Also known as: Frequent micturition \| Urinary frequency \| Frequency of micturition NOS \| Frequent urination \| Micturition frequency Excludes: Pollakiuria [MF50.3] Retention of urine Definition: Incomplete emptying of the bladder Also known as: Retention of urine \| bladder retention of urine \| not passing urine \| unable to empty bladder \| unable to pass urine [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS === GRAPH WALKS === --- Walk 1 --- [NE80.3] Other serum reactions --EXCLUDES--> [?] Acute hepatitis B without Hepatitis D virus co-infection Def: Acute liver injury related with hepatitis B virus (HBV). Acute hepatitis B is suspected based on positive HBsAg and high-titer IgM anti-HBc. However, other causes of acute viral hepatitis may not be f... --PARENT--> [?] Acute hepatitis B Def: Acute liver injury and inflammation caused by recent and short-term (less than 6 months) infection with hepatitis B virus (HBV). Transmission is by sexual, blood and body fluid contamination (parenter... --- Walk 2 --- [NE80.3] Other serum reactions --RELATED_TO--> [?] Serum sickness vasculitis Def: An acute vasculitic illness typified by fever, arthralgia and urticarial vasculitis resulting from an immune complex-mediated (type III) immune reaction to foreign protein. Historically this was large... --PARENT--> [?] Vasculitis associated with probable aetiology Def: Vasculitis that is associated with a probable specific etiology. The name (diagnosis) should have a prefix term specifying the association (e.g. hydralazine-associated microscopic polyangiitis, hepati... --- Walk 3 --- [5D0Y] Other specified metabolic disorders --PARENT--> [?] Other metabolic disorders --EXCLUDES--> [?] Langerhans cell histiocytosis Def: A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognised: eosinophilic granuloma, Letterer-Siwe di... --- Walk 4 --- [5D0Y] Other specified metabolic disorders --PARENT--> [?] Other metabolic disorders --CHILD--> [5D0Y] Other specified metabolic disorders --- Walk 5 --- [5B91.0] Hypercalcaemia Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ... --PARENT--> [5B91] Mineral excesses --RELATED_TO--> [?] Hyperkalaemia --- Walk 6 --- [5B91.0] Hypercalcaemia Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ... --RELATED_TO--> [?] Myopathy due to hypercalcaemia --PARENT--> [?] Hypercalcaemia Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ...	[ "[NE80.3] Other serum reactions\n --EXCLUDES--> [?] Acute hepatitis B without Hepatitis D virus co-infection\n Def: Acute liver injury related with hepatitis B virus (HBV). Acute hepatitis B is suspected based on positive HBsAg and high-titer IgM anti-HBc. However, other causes of acute viral hepatitis may not be f...\n --PARENT--> [?] Acute hepatitis B\n Def: Acute liver injury and inflammation caused by recent and short-term (less than 6 months) infection with hepatitis B virus (HBV). Transmission is by sexual, blood and body fluid contamination (parenter...", "[NE80.3] Other serum reactions\n --RELATED_TO--> [?] Serum sickness vasculitis\n Def: An acute vasculitic illness typified by fever, arthralgia and urticarial vasculitis resulting from an immune complex-mediated (type III) immune reaction to foreign protein. Historically this was large...\n --PARENT--> [?] Vasculitis associated with probable aetiology\n Def: Vasculitis that is associated with a probable specific etiology. The name (diagnosis) should have a prefix term specifying the association (e.g. hydralazine-associated microscopic polyangiitis, hepati...", "[5D0Y] Other specified metabolic disorders\n --PARENT--> [?] Other metabolic disorders\n --EXCLUDES--> [?] Langerhans cell histiocytosis\n Def: A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognised: eosinophilic granuloma, Letterer-Siwe di...", "[5D0Y] Other specified metabolic disorders\n --PARENT--> [?] Other metabolic disorders\n --CHILD--> [5D0Y] Other specified metabolic disorders", "[5B91.0] Hypercalcaemia\n Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ...\n --PARENT--> [5B91] Mineral excesses\n --RELATED_TO--> [?] Hyperkalaemia", "[5B91.0] Hypercalcaemia\n Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ...\n --RELATED_TO--> [?] Myopathy due to hypercalcaemia\n --PARENT--> [?] Hypercalcaemia\n Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ..." ]	NE80.3	Other serum reactions	[ { "from_icd11": "NE80.3", "icd10_code": "T880XXA", "icd10_title": "Infection following immunization, initial encounter" }, { "from_icd11": "NE80.3", "icd10_code": "T8061XA", "icd10_title": "Other serum reaction due to administration of blood and blood products, initial encounter" }, { "from_icd11": "NE80.3", "icd10_code": "T8069XA", "icd10_title": "Other serum reaction due to other serum, initial encounter" }, { "from_icd11": "NE80.3", "icd10_code": "T8062XA", "icd10_title": "Other serum reaction due to vaccination, initial encounter" }, { "from_icd11": "NE80.3", "icd10_code": "T806", "icd10_title": "Other serum reactions" }, { "from_icd11": "NE80.3", "icd10_code": "T880", "icd10_title": "Infection following immunization" }, { "from_icd11": "5C50.F2", "icd10_code": "E7281", "icd10_title": "Disorders of gamma aminobutyric acid metabolism" }, { "from_icd11": "5C50.F2", "icd10_code": "E728", "icd10_title": "Other specified disorders of amino-acid metabolism" }, { "from_icd11": "MF50.6Z", "icd10_code": "R3915", "icd10_title": "Urgency of urination" }, { "from_icd11": "MF50.6Z", "icd10_code": "R3911", "icd10_title": "Hesitancy of micturition" }, { "from_icd11": "MF50.6Z", "icd10_code": "R3914", "icd10_title": "Feeling of incomplete bladder emptying" }, { "from_icd11": "MF50.6Z", "icd10_code": "R3912", "icd10_title": "Poor urinary stream" }, { "from_icd11": "MF50.6Z", "icd10_code": "R39198", "icd10_title": "Other difficulties with micturition" }, { "from_icd11": "MF50.6Z", "icd10_code": "R3916", "icd10_title": "Straining to void" }, { "from_icd11": "MF50.6Z", "icd10_code": "R3919", "icd10_title": "Other difficulties with micturition" } ]	T880XXA	Infection following immunization, initial encounter
The patient was a 30-year-old Asian woman who visited her previous doctor with a chief complaint of an enlarged right breast mass while breastfeeding. She had two previous deliveries. Her children were 4 years old and 1 year old. Her grandmother had developed breast cancer when she was 60 years old, but the histological type was unknown. There was no history of alcohol, tobacco, or drug use. She was diagnosed with angiosarcoma by core needle biopsy and referred to our department for further examination and treatment. She had a surgical history of appendectomy for appendicitis when she was 25 years old. She had never had previous radiation exposure. On physical examination, a 40-mm-sized mass was palpable mainly in medial upper area of the right breast. There were no skin abnormalities, ulcers, or enlarged lymph nodes. No axillary lymph nodes were palpable. Magnetic resonance imaging (MRI) showed bilateral multiple breast masses with a maximum diameter of 46 mm. It demonstrated rapid enhancement post-contrast administration with progressive and prolonged enhancement in the delay phase . There was no evidence of metastasis. After needle biopsy of bilateral breast, a diagnosis of bilateral primary angiosarcoma of the breast was made. Bilateral total mastectomy was performed. This was her first hospitalization. Vital signs were stable, and laboratory results were unremarkable (Table 1 ). Histologically, a heterogeneous brownish nodule with a maximum diameter of 40 mm was found in the upper area of the right breast . The area showed increased vascularity with branching, anastomosis, slit-like changes, and hemorrhage . Immunohistochemistry studies showed the neoplastic cells were positive for CD31 and CD34 but negative for CKAE1/AE3 and D2-40 . Surgical margin was negative. A diagnosis of intermediate to high risk angiosarcoma of the breast was made. Adjuvant therapy consisted of irradiation to the right chest wall (60 Gy/30 sessions) because the right tumor was larger in size and closer to the margin and weekly paclitaxel (75 mg/m 2 ) administered intravenously for 12 cycles. Three months after surgery, multiple liver metastases were detected. She was still receiving chemotherapy with paclitaxel, but it was determined to be ineffective and was switched to chemotherapy with doxorubicin (60 mg/m 2 ). Whole exome sequencing was performed, and MLL2 and TGFBR2 were found to be abnormal, but there was no druggable gene abnormality. Tumor mutation burden (TMB) was 1 mutation/Mb. Systemic chemotherapy with doxorubicin was administered only once. Systemic therapy was discontinued due to increased liver metastases, and the patient was switched to local therapy. The patient underwent three times of hepatic arterial infusion chemotherapy for liver metastases. The first infusion of adriamycin 20 mg, fluorouracil 250 mg, bevacizumab 100 mg, carboplatin 30 mg, and HepaSphere as embolic material was used. It was determined that a change in the injected drug was desirable due to the increase in tumor size. The second and third infusions were epirubicin 20 mg, fluorouracil 250 mg, cisplatin 20 mg, docetaxel 20 mg, bevacizumab 200 mg, and HepaSphere as embolization material. After the third infusion , multiple enlarged liver metastases, small intestinal metastases, and peritoneal dissemination were observed, and it was determined that local treatment with intravenous chemotherapy was not feasible. Eight months after surgery, the patient was switched to systemic chemotherapy with eribulin (1.0 mg/m 2 ). She was started on eribulin, administered intravenously. Computed tomography (CT) scan showed persistent intratumoral bleeding in the liver, requiring frequent blood transfusions, and the patient was given eribulin only twice. Nine months after surgery, the liver metastasis ruptured and the patient was rushed to the emergency department. However, the liver metastases continued to rupture repeatedly, and she underwent a total of five vascular embolization procedures. Laboratory results from the time of admission until death are presented in Table 1 . The patient died 10 months after surgery. Autopsy was not performed. Fig. 1 MRI findings. High T2 signal intensity mass occupied the entire right breast. It demonstrated rapid enhancement post-contrast administration with progressive and prolonged enhancement in the delay phase Table 1 Summary of the laboratory results WBC Hb Plt BUN Cre ALP AST ALT γGTP LDH Alb First hospitalization 4.62 12,4 282 10 0.54 62 13 9 8 158 3.9 Start of paclitaxel 3.19 11.3 212 11 0.53 92 17 13 26 185 4.4 Start of doxorubicin 4.46 10.8 278 8 0.49 112 36 30 60 195 4.3 1 month after third HAIC 3.11 7.9 116 12 0.49 237 129 196 284 305 4.1 Start of eribulin 11.13 9.7 181 16 0.45 392 67 176 453 891 3.3 2 weeks before first TAE 8.66 9.1 127 18 0.47 338 49 128 442 763 3.3 Before first TAE 7.06 2.7 65 21 0.47 117 25 50 217 275 2.6 Before second TAE 7.14 5.2 84 16 0.43 270 46 36 346 605 2.0 Before third TAE 10.23 6.4 94 18 0.45 566 115 84 585 1094 2.7 Before fourth TAE 8.31 4.5 117 26 0.60 345 76 100 354 788 1.8 Before fifth TAE 9.21 6.1 100 20 0.59 406 104 161 371 1147 2.2 WBC white blood cell count (× 10 3 /μL), Hb hemoglobin (g/dL), Plt platelet (× 10 3 /μL), BUN blood urea nitrogen (mg/dL), Cre creatinine (mg/dL), ALP alkaline phosphatase (IU/L), AST aspartate aminotransferase (IU/L), ALT alanine aminotransferase (IU/L), γGTP γ-glutamyl transpeptidase (IU/L), LDH lactate dehydrogenase (IU/L), Alb albumin (g/dL) Fig. 2 Surgical specimen. The tumor entirely replaces in the right breast. There are small regions in the left breast. It was blackish and hemorrhagic and measured 40 mm in the greatest dimension Fig. 3 Pathologic findings. The area showed branching vascular structures with slit-like changes and hemorrhage (hematoxylin–eosin–safran ×5 and ×40) Fig. 4 Immunohistochemistry studies. The neoplastic cells were positive for CD31 and CD34 but negative for CKAE1/AE3 and D2-40 Fig. 5 a CT imaging of liver metastasis before the first hepatic arterial infusion chemotherapy (HAIC). b CT imaging of liver metastasis before the second HAIC. Metastatic lesions were once under control after the second HAIC. c CT imaging of liver metastasis before the third HAIC. d CT imaging taken at 1 month after the third HAIC. One month after the third HAIC, the liver metastases were rapidly enlarged	3.978516	0.980469	sec[1]/p[0]	en	0.999996	PMC9942292	https://doi.org/10.1186/s13256-023-03791-7	[ "breast", "liver", "metastases", "chemotherapy", "haic", "imaging", "metastasis", "enlarged", "area", "enhancement" ]	[ { "code": "GB23", "title": "Certain specified disorders of breast" }, { "code": "GB21.Z", "title": "Inflammatory disorders of breast, unspecified" }, { "code": "GB21.Y", "title": "Other specified inflammatory disorders of breast" }, { "code": "QF01.0", "title": "Acquired absence of breast" }, { "code": "GB23.3", "title": "Atrophy of breast" }, { "code": "DB9Z", "title": "Diseases of liver, unspecified" }, { "code": "DB97.Z", "title": "Inflammatory liver disease, unspecified" }, { "code": "DB99.7", "title": "Hepatic failure without mention whether acute or chronic" }, { "code": "LB20.0Y", "title": "Other specified structural developmental anomalies of liver" }, { "code": "LB20.0Z", "title": "Structural developmental anomalies of liver, unspecified" } ]	=== ICD-11 CODES FOUND === [GB23] Certain specified disorders of breast Definition: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere. Also known as: Certain specified disorders of breast \| disease of breast \| mastopathy [GB21.Z] Inflammatory disorders of breast, unspecified Also known as: Inflammatory disorders of breast, unspecified \| Inflammatory disorders of breast \| breast inflammation \| inflammatory breast disease \| mastitis NOS [GB21.Y] Other specified inflammatory disorders of breast Also known as: Other specified inflammatory disorders of breast \| Breast antibioma \| Infective mastitis \| acute infective mastitis \| nonpuerperal infective mastitis [QF01.0] Acquired absence of breast Also known as: Acquired absence of breast \| absence of breast \| mastectomy status \| Acquired absence of breast, partial \| Acquired absence of breast, total [GB23.3] Atrophy of breast Definition: A condition of the breast, caused by apoptosis of the cells commonly due to prolonged estrogen reduction, diminished cellular proliferation, decreased cellular volume, decreased function, ischaemia, malnutrition, disease, or mutation. This condition is characterised by a partial or complete decrease in size and function of the breast tissue. Also known as: Atrophy of breast \| Hypoplasia of breast \| hypoplastic breast \| mammary hypoplasia [DB9Z] Diseases of liver, unspecified Also known as: Diseases of liver, unspecified \| liver disease \| liver condition NOS \| organ liver disease \| hepatopathy [DB97.Z] Inflammatory liver disease, unspecified Also known as: Inflammatory liver disease, unspecified \| Certain specified inflammatory liver diseases \| Nonspecific reactive hepatitis \| inflammatory liver disease \| hepatitis NOS [DB99.7] Hepatic failure without mention whether acute or chronic Also known as: Hepatic failure without mention whether acute or chronic \| liver decompensation \| liver function failure \| hepatic failure NOS \| liver failure NOS [LB20.0Y] Other specified structural developmental anomalies of liver Also known as: Other specified structural developmental anomalies of liver \| Alagille syndrome \| Alagille-Watson syndrome \| Arteriohepatic dysplasia \| Syndromic bile duct paucity [LB20.0Z] Structural developmental anomalies of liver, unspecified Also known as: Structural developmental anomalies of liver, unspecified \| Structural developmental anomalies of liver \| Malformations of liver \| congenital anomaly of liver \| congenital malformation of liver === GRAPH WALKS === --- Walk 1 --- [GB23] Certain specified disorders of breast Def: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere.... --RELATED_TO--> [?] Other signs or symptoms in breast Def: Any sign or symptom of the breast, not classified elsewhere.... --CHILD--> [?] Haemorrhage breast --- Walk 2 --- [GB23] Certain specified disorders of breast Def: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere.... --RELATED_TO--> [?] Other signs or symptoms in breast Def: Any sign or symptom of the breast, not classified elsewhere.... --CHILD--> [?] Nipple discharge --- Walk 3 --- [GB21.Z] Inflammatory disorders of breast, unspecified --PARENT--> [GB21] Inflammatory disorders of breast Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function.... --RELATED_TO--> [?] Neonatal infectious mastitis Def: A disease of the breasts in neonates, may be caused by a maternal infection with a bacterial source. This disease is characterised by swelling, erythema, warmth, tenderness, induration of the breast, ... --- Walk 4 --- [GB21.Z] Inflammatory disorders of breast, unspecified --PARENT--> [GB21] Inflammatory disorders of breast Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function.... --RELATED_TO--> [?] Nonpurulent mastitis associated with childbirth --- Walk 5 --- [GB21.Y] Other specified inflammatory disorders of breast --PARENT--> [GB21] Inflammatory disorders of breast Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function.... --RELATED_TO--> [?] Nonpurulent mastitis associated with childbirth --- Walk 6 --- [GB21.Y] Other specified inflammatory disorders of breast --PARENT--> [GB21] Inflammatory disorders of breast Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function.... --RELATED_TO--> [?] Nonpurulent mastitis associated with childbirth	[ "[GB23] Certain specified disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere....\n --RELATED_TO--> [?] Other signs or symptoms in breast\n Def: Any sign or symptom of the breast, not classified elsewhere....\n --CHILD--> [?] Haemorrhage breast", "[GB23] Certain specified disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by pathological changes, not classified elsewhere....\n --RELATED_TO--> [?] Other signs or symptoms in breast\n Def: Any sign or symptom of the breast, not classified elsewhere....\n --CHILD--> [?] Nipple discharge", "[GB21.Z] Inflammatory disorders of breast, unspecified\n --PARENT--> [GB21] Inflammatory disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function....\n --RELATED_TO--> [?] Neonatal infectious mastitis\n Def: A disease of the breasts in neonates, may be caused by a maternal infection with a bacterial source. This disease is characterised by swelling, erythema, warmth, tenderness, induration of the breast, ...", "[GB21.Z] Inflammatory disorders of breast, unspecified\n --PARENT--> [GB21] Inflammatory disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function....\n --RELATED_TO--> [?] Nonpurulent mastitis associated with childbirth", "[GB21.Y] Other specified inflammatory disorders of breast\n --PARENT--> [GB21] Inflammatory disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function....\n --RELATED_TO--> [?] Nonpurulent mastitis associated with childbirth", "[GB21.Y] Other specified inflammatory disorders of breast\n --PARENT--> [GB21] Inflammatory disorders of breast\n Def: Any disorder of the breast or breast tissue, characterised by inflammatory effects, pain, heat, redness, swelling, and loss of function....\n --RELATED_TO--> [?] Nonpurulent mastitis associated with childbirth" ]	GB23	Certain specified disorders of breast	[ { "from_icd11": "GB23", "icd10_code": "N6459", "icd10_title": "Other signs and symptoms in breast" }, { "from_icd11": "GB23", "icd10_code": "N6489", "icd10_title": "Other specified disorders of breast" }, { "from_icd11": "GB23", "icd10_code": "N6481", "icd10_title": "Ptosis of breast" }, { "from_icd11": "GB23", "icd10_code": "N6482", "icd10_title": "Hypoplasia of breast" }, { "from_icd11": "GB23", "icd10_code": "N6452", "icd10_title": "Nipple discharge" }, { "from_icd11": "GB23", "icd10_code": "N6451", "icd10_title": "Induration of breast" }, { "from_icd11": "GB23", "icd10_code": "N6453", "icd10_title": "Retraction of nipple" }, { "from_icd11": "GB23", "icd10_code": "N64", "icd10_title": "Other disorders of breast" }, { "from_icd11": "GB23", "icd10_code": "N648", "icd10_title": "Other specified disorders of breast" }, { "from_icd11": "GB23", "icd10_code": "N645", "icd10_title": "Other signs and symptoms in breast" }, { "from_icd11": "GB21.Z", "icd10_code": "N610", "icd10_title": "Mastitis without abscess" }, { "from_icd11": "GB21.Z", "icd10_code": "N611", "icd10_title": "Abscess of the breast and nipple" }, { "from_icd11": "GB21.Z", "icd10_code": "N61", "icd10_title": "Inflammatory disorders of breast" }, { "from_icd11": "QF01.0", "icd10_code": "Z9012", "icd10_title": "Acquired absence of left breast and nipple" }, { "from_icd11": "QF01.0", "icd10_code": "Z9011", "icd10_title": "Acquired absence of right breast and nipple" } ]	N6459	Other signs and symptoms in breast
A 39-year-old female patient was referred in April 2007 to the Surgical Department of the University Hospital of Heidelberg with a 3-year history of relapsing episodes of symptomatic portal biliopathy. The patient had a history of a hypercoagulable state owing to an abnormally elevated factor VIII level (factor VIII activity concentration 360% of normal, normal: 60–150), which was evident in October 2003 after an extensive diagnostic thrombophilic evaluation for idiopathic extrahepatic portal vein thrombosis complicated with PH. Extrahepatic PH was manifested with ascites, splenomegaly, and bleeding episodes from grade II oesophageal varices. The latter was treated by endoscopic sclerotherapy . At the time of initial presentation spiral computed tomography (CT) scan of the abdomen depicted a superior mesenteric and portal vein thrombosis in addition to cavernomatous transformation of the portal vein. Her family history was negative for thromboembolic events while she denied the use of oral contraceptive at the time of presentation. After diagnosis she was started on phenprocoumon for anticoagulation, which however discontinued because of episodes of variceal bleeding. A beta-blocker and nitrate were also administered for the treatment of portal hypertension. Six months following the initial presentation the patient started to complain of recurrent episodes of right upper quadrant pain, typically of biliary origin. Laboratory values showed a cholestatic pattern while abdominal ultrasound demonstrated a normal echogenicity of the liver as well as dilatation and irregularity of the common bile duct (CBD), which appeared surrounded by multiple tortuous collaterals in the region of the porta hepatis. The gallbladder appeared contracted and had multiple overlying collaterals, and contained stones and debris. Endoscopic retrograde cholangiopancreatography (ERCP) established the diagnosis of portal biliopathy by revealing irregular outline of the CBD in addition to several flat impressions, presumably caused by venous collaterals . These findings were consistent with grade I portal biliopathy . After papillotomy, complete bile duct stone clearance was accomplished, followed by the insertion of an Amsterdam type biliary endoprosthesis. Laboratory values of cholestatic pattern improved in the immediate post-ERCP period. She was treated with ursodesoxicholic acid and was scheduled for a repeat ERCP session after a three-month interval. However, two months later a relapsing episode of obstructive jaundice and cholangitis ensued necessitating a repeat ERCP session and stent change. During the last 3-years, multiple ERCP sessions and stent changes were required, because of relapsing attacks of cholangitis, without, however, a definitive improvement. The patient was therefore referred for evaluation to our department and after a thorough consultation with the patient, the gastroenterologist and haematologist a decision was made for an open cholecystectomy and a portosystemic shunt. Preoperative magnetic resonance imaging (MRI) of the abdomen showed persistence of superior mesenteric and portal vein thrombosis, and cavernous transformation of the portal vein . Laparotomy was performed through an L-incision. After entering the peritoneal cavity large, dilated, tortous collaterals were found at the porta hepatis and around the CBD, crossing over the hepatoduodenal ligament and the gallbladder. Multiple inflammatory adhesions between the omentum and the contracted gallbladder were also present. Cholecystectomy was performed in a fundus-first approach. After careful dissection, and ligation of several tortous collaterals, the vessels and cystic duct in Calot's triangle were identified and ligated. Following cholecystectomy, it was decided to proceed with a portosystemic shunt. The distal portal vein (PV) was carefully isolated by minimal dissection avoiding the cavernoma in the liver hilum. The anterior portion of the adjacent inferior vena cava (IVC) was also dissected at the site chosen for the portocaval anastomosis. It was at this stage decided to perform a PV and IVC pressure measurement. The PV pressure was measured 30 mm Hg, while IVC pressure 9 mm Hg. However, the creation of a tension free side-to-side portocaval shunt proved technically not possible because of approximation difficulty, owing to the long vertical distance between the distal PV and IVC. In a search for potential H-shunt options, and considering the fact that patient's thrombophilia would made the use of a synthetic graft prone to thrombosis, a large 6 mm in diameter right ovarian vein draining into the IVC just caudal to the right renal vein appeared to be of adequate size and quality to be utilized as an autologous conduit, to perform a portosystemic shunt . Indeed, approximately 8 cm of its length was mobilized and the right ovarian vein was divided and ligated distally. After systemic anticoagulation with heparin, the proximal end of the ovarian vein was anastomosed end-to-side to the distal portal vein with a continuous 6-0 PDS suture (Figures 4(a) and 4(b) ). After clamp removal, Doppler confirmed excellent flow, while the pressure gradient between PV and IVC was once again measured confirming a reduction of at least 57% (the PV pressure was measured 20 mm Hg, while IVC pressure 8 mm Hg). The right ovarian vein had a smooth curve under the duodenum when the retractors were relaxed. This technique allowed bypassing the thrombosed portion of the portal vein by avoiding dissection of the cavernoma in the liver hilum and related risk of massive hemorrhage. The patient had an uncomplicated postoperative course and an abdominal CT scan performed prior to discharge home confirmed patency of the shunt, despite difficulty visualizing the shunt by US examination. Low-molecular weight heparin was administered subcutaneously for three weeks, as she was scheduled to undergo a new ERCP session and stent removal by the end of this period. Following ERCP and stent removal, phenprocoumon was administered for anticoagulation. The patient did not developed encephalopathy while she has had no further episodes of bleeding, jaundice, abdominal pain, or recurrent fever, and no therapeutic intervention was required during a 19-month follow-up period ( Table 1 ), although morphologic biliary abnormalities seen at US tended to persist.	3.996094	0.978027	sec[1]/p[0]	en	0.999997	19584934	https://doi.org/10.1155/2009/152195	[ "vein", "portal", "ercp", "shunt", "while", "pressure", "episodes", "collaterals", "thrombosis", "however" ]	[ { "code": "BD7Z", "title": "Diseases of veins, unspecified" }, { "code": "MC88", "title": "Prominent veins" }, { "code": "BD7Y", "title": "Other specified diseases of veins" }, { "code": "BD75.Y", "title": "Venous varicosities of other specified sites" }, { "code": "BD73.2Z", "title": "Systemic vein obstruction, unspecified" }, { "code": "DB98.3", "title": "Portal vein thrombosis" }, { "code": "DB98.7Z", "title": "Portal hypertension, unspecified" }, { "code": "NB90.4Y&XA1E17", "title": "Portal venous laceration" }, { "code": "LA90.21", "title": "Congenital portosystemic shunt" }, { "code": "DB98.7Y", "title": "Other specified portal hypertension" } ]	=== ICD-11 CODES FOUND === [BD7Z] Diseases of veins, unspecified Also known as: Diseases of veins, unspecified [MC88] Prominent veins Also known as: Prominent veins [BD7Y] Other specified diseases of veins Also known as: Other specified diseases of veins [BD75.Y] Venous varicosities of other specified sites Also known as: Venous varicosities of other specified sites \| Caput medusae \| Jugular venous aneurysm \| jugular vein aneurysm \| Orbital varices [BD73.2Z] Systemic vein obstruction, unspecified Also known as: Systemic vein obstruction, unspecified \| Systemic vein obstruction [DB98.3] Portal vein thrombosis Definition: Portal vein thrombosis is a condition where the portal vein and/or its branches are obstructed, mainly by a blood clot or malignant tumour invasion. Also known as: Portal vein thrombosis \| Phlebitis of portal vein \| deep vein thrombosis of portal vein \| portal thrombosis \| PVT - [portal vein thrombosis] Includes: Phlebitis of portal vein [DB98.7Z] Portal hypertension, unspecified Also known as: Portal hypertension, unspecified \| Portal hypertension \| PHT - [portal hypertension] \| Portal HTN [LA90.21] Congenital portosystemic shunt Also known as: Congenital portosystemic shunt \| anomalous pulmonary venous drainage to hepatic veins \| anomaly of portal vein connection \| portal vein deformity \| portal vein anomaly [DB98.7Y] Other specified portal hypertension Also known as: Other specified portal hypertension \| Banti syndrome \| Banti spleen \| fibrocongestive splenomegaly \| congestive splenomegaly === GRAPH WALKS === --- Walk 1 --- [BD7Z] Diseases of veins, unspecified --PARENT--> [?] Diseases of veins --CHILD--> [BD71] Deep vein thrombosis Def: The process whereby thrombus (blood clot) forms in the large veins of the peripheral venous system. In addition to obstructing venous return it possesses a hazard whereby thrombus may detach and embol... --- Walk 2 --- [BD7Z] Diseases of veins, unspecified --PARENT--> [?] Diseases of veins --CHILD--> [BD70] Superficial thrombophlebitis --- Walk 3 --- [MC88] Prominent veins --PARENT--> [?] Symptoms or signs involving the circulatory system --CHILD--> [MC82] Cardiac arrest Def: A sudden, sometimes temporary, cessation of heart function resulting in hemodynamic collapse.... --- Walk 4 --- [MC88] Prominent veins --PARENT--> [?] Symptoms or signs involving the circulatory system --RELATED_TO--> [?] Fear of cardiovascular disease --- Walk 5 --- [BD7Y] Other specified diseases of veins --PARENT--> [?] Diseases of veins --CHILD--> [BD71] Deep vein thrombosis Def: The process whereby thrombus (blood clot) forms in the large veins of the peripheral venous system. In addition to obstructing venous return it possesses a hazard whereby thrombus may detach and embol... --- Walk 6 --- [BD7Y] Other specified diseases of veins --PARENT--> [?] Diseases of veins --CHILD--> [BD71] Deep vein thrombosis Def: The process whereby thrombus (blood clot) forms in the large veins of the peripheral venous system. In addition to obstructing venous return it possesses a hazard whereby thrombus may detach and embol...	[ "[BD7Z] Diseases of veins, unspecified\n --PARENT--> [?] Diseases of veins\n --CHILD--> [BD71] Deep vein thrombosis\n Def: The process whereby thrombus (blood clot) forms in the large veins of the peripheral venous system. In addition to obstructing venous return it possesses a hazard whereby thrombus may detach and embol...", "[BD7Z] Diseases of veins, unspecified\n --PARENT--> [?] Diseases of veins\n --CHILD--> [BD70] Superficial thrombophlebitis", "[MC88] Prominent veins\n --PARENT--> [?] Symptoms or signs involving the circulatory system\n --CHILD--> [MC82] Cardiac arrest\n Def: A sudden, sometimes temporary, cessation of heart function resulting in hemodynamic collapse....", "[MC88] Prominent veins\n --PARENT--> [?] Symptoms or signs involving the circulatory system\n --RELATED_TO--> [?] Fear of cardiovascular disease", "[BD7Y] Other specified diseases of veins\n --PARENT--> [?] Diseases of veins\n --CHILD--> [BD71] Deep vein thrombosis\n Def: The process whereby thrombus (blood clot) forms in the large veins of the peripheral venous system. In addition to obstructing venous return it possesses a hazard whereby thrombus may detach and embol...", "[BD7Y] Other specified diseases of veins\n --PARENT--> [?] Diseases of veins\n --CHILD--> [BD71] Deep vein thrombosis\n Def: The process whereby thrombus (blood clot) forms in the large veins of the peripheral venous system. In addition to obstructing venous return it possesses a hazard whereby thrombus may detach and embol..." ]	BD7Z	Diseases of veins, unspecified	[ { "from_icd11": "BD7Z", "icd10_code": "I82412", "icd10_title": "Acute embolism and thrombosis of left femoral vein" }, { "from_icd11": "BD7Z", "icd10_code": "I82621", "icd10_title": "Acute embolism and thrombosis of deep veins of right upper extremity" }, { "from_icd11": "BD7Z", "icd10_code": "I82432", "icd10_title": "Acute embolism and thrombosis of left popliteal vein" }, { "from_icd11": "BD7Z", "icd10_code": "I82C11", "icd10_title": "Acute embolism and thrombosis of right internal jugular vein" }, { "from_icd11": "BD7Z", "icd10_code": "I82441", "icd10_title": "Acute embolism and thrombosis of right tibial vein" }, { "from_icd11": "BD7Z", "icd10_code": "I82422", "icd10_title": "Acute embolism and thrombosis of left iliac vein" }, { "from_icd11": "BD7Z", "icd10_code": "I82622", "icd10_title": "Acute embolism and thrombosis of deep veins of left upper extremity" }, { "from_icd11": "BD7Z", "icd10_code": "I824Z1", "icd10_title": "Acute embolism and thrombosis of unspecified deep veins of right distal lower extremity" }, { "from_icd11": "BD7Z", "icd10_code": "I82401", "icd10_title": "Acute embolism and thrombosis of unspecified deep veins of right lower extremity" }, { "from_icd11": "BD7Z", "icd10_code": "I824Z2", "icd10_title": "Acute embolism and thrombosis of unspecified deep veins of left distal lower extremity" }, { "from_icd11": "BD7Z", "icd10_code": "I82612", "icd10_title": "Acute embolism and thrombosis of superficial veins of left upper extremity" }, { "from_icd11": "BD7Z", "icd10_code": "I82721", "icd10_title": "Chronic embolism and thrombosis of deep veins of right upper extremity" }, { "from_icd11": "BD7Z", "icd10_code": "I82C12", "icd10_title": "Acute embolism and thrombosis of left internal jugular vein" }, { "from_icd11": "BD7Z", "icd10_code": "I82B12", "icd10_title": "Acute embolism and thrombosis of left subclavian vein" }, { "from_icd11": "BD7Z", "icd10_code": "I82421", "icd10_title": "Acute embolism and thrombosis of right iliac vein" } ]	I82412	Acute embolism and thrombosis of left femoral vein
To our knowledge, this is the first case reported in the literature of an adolescent suffering from headache and ophthalmoplegia with a 7-year brain MRI follow-up, raising the question on the relationship between RPON and schwannoma. The evolutive changes in the presentation of symptoms and the different findings over time in brain MRI images observed in this patient repurpose some open issues on the classification and pathophysiological mechanisms of RPON. The patient had his first clinical manifestations of recurrent headaches at the age of 4 years agreeing to diagnostic criteria of migraine without aura. Since he was 7 years old, the patient started to have migraine episodes with the same clinical features but lasting more time and with partial responsiveness to administration of paracetamol with association of right ocular pain, ipsilateral ptosis, and diplopia. After two episodes with these features and with MRI that showed normal findings in the image scans without any appreciable enhancement or thickening of the nerve, he was diagnosed with “ophthalmoplegic migraine” according to ICHD-II . Clinical and instrumental data at next follow-ups were in agreement with the reclassification of RPON proposed in the ICHD-3 . Brain MRI follow-ups of 2013 and 2016 both during an acute episode of headache with ophthalmoplegia confirmed normal findings in the image scans in absence of any brain MRI data supporting a secondary cause. At this regard, according to our review, patients diagnosed with OM/RPON have MRI exams that show thickening and enhancing of the oculomotor nerve [ 9 , 16 – 24 ] or normal findings in image scans [ 18 , 20 , 22 , 23 , 25 – 30 ]. Nevertheless, the last brain MRI examination of our patient showed the presence of 5-6 mm nodular enhanced mass located within the fork of the right basilar artery near to perimesencephalic and interpeduncular cisterns, enhanced after contrast, which could be described as III cranial nerve schwannoma . This case highlights the important role of longitudinal follow-up of brain MRI in RPON and raises the question on the relationship between RPON and schwannoma. The exact pathophysiology of RPON is unknown, but the most accepted postulated mechanisms include microvascular ischemia, demyelization, and inflammation involving the oculomotor nerve . Despite the reclassification as a neuropathy, a balanced debate on arguments pro and contra a migrainous background of RPON have been published. Friedman DI proposed to consider OM as a syndrome that may be “primary” as a variant of migraine with aura for cases with normal imaging and spontaneous resolution and “secondary” as a cranial neuropathy with focal nerve enhancement on neuroimaging . The case we described is far from this classification hypothesis, considering that three different brain MRIs during an acute attack of OM showed normal findings inducing to consider the clinical manifestation of the patient as a “primary” migraine, while the last brain MRI showed a focal enhancement of contrast, described as a schwannoma. The review of literature on MRI findings in child and adolescent patients affected by OM/RPON (according to ICHD classifications over time) showed only three cases with isolated schwannoma of the oculomotor nerve associated with migraine and oculomotor nerve palsy after a long time history of III nerve paresis and/or migraine ( Table 1 ). In 2005, Murakami et al. published the first histologically proved case of schwannoma in an 11-year-old girl with a previous history of OM. The initial diagnosis of OM was corroborated by brain MRI. Five years later, the OM attacks became more frequent and not controlled by prophylaxis or acute episodes' drugs, and brain MRI with three-dimensional reconstruction shows a 5 mm nodular lesion highly suggestive of a neoplastic process involving the oculomotor nerve in its course from the brainstem through the prepontine cistern continuously. Histopathological finding after surgical treatment confirmed the diagnosis of schwannoma . Riahi et al. in 2014 published the case of a 12-year-old girl who presented three episodes of left eye painful ophthalmoplegia during a period of 3 years. Two previous brain MRIs produced normal image scans, while the brain MRI made after the third episode showed a mass of tissue in the cavernous sinus suggesting a third nerve schwannoma . Jibia et al. in 2015 published the case of a 13-year-old girl with a previous medical history of migraine since she was 6 years old. In coincidence of hospitalization because of an attack of disabling ophthalmic migraine, the brain MRI revealed a right nodular schwannoma located within the cisternal segment of the oculomotor nerve (patient was treated with analgesic and corticosteroid therapy with complete regression of symptoms three weeks later and a normal MRI follow-up) . The most part of other cases included in our review, with OM/RPON diagnosis have short-term MRI follow-ups performed less than two years after the first evaluation that can show persistent thickening and enhancement [ 16 , 17 , 20 – 24 ] or their negativization . Therefore, it is important to consider the long-term evolution of RPON and its relationship with oculomotor nerve schwannoma. Two different speculative explanations of the eventual pathophysiological relationship between RPON and schwannoma have been proposed. The first one is related to the intermittent release of a chemical substance from the tumor, which results in the stimulation of the trigeminal nerve receptors leading to a migrainous headache. According to this hypothesis, the oculomotor nerve schwannoma mimics RPON, so the painful ophthalmoplegia may be considered as the initial manifestation of a tumor and may be too small to be appreciated in MRI scans . A review of pediatric isolated oculomotor nerve schwannoma showed OM as one among the clinical signs of presentation of this tumor . The alternative hypothesis is based on the idea that the underlying pathophysiology of RPON is an inflammatory cranial neuralgia and not migraine, so that repeated episodes of inflammation leading to demyelination and remyelination may be associated with a potential transformation into schwannoma from Schwann cells proliferation. According to this interpretation, isolated oculomotor schwannoma may be considered as a long-term complication of RPON.	4.339844	0.925293	sec[3]/p[0]	en	0.999998	31662812	https://doi.org/10.1155/2019/5392945	[ "rpon", "schwannoma", "nerve", "brain", "migraine", "oculomotor", "this", "that", "episodes", "scans" ]	[ { "code": "2F3Y", "title": "Benign neoplasms except of mesenchymal origin, of other specified site" }, { "code": "2F24", "title": "Benign cutaneous neoplasms of neural or nerve sheath origin" }, { "code": "2A02.3", "title": "Benign neoplasm of cranial nerves" }, { "code": "2F30.Y", "title": "Other specified benign neoplasm of breast" }, { "code": "LD2D.11", "title": "Neurofibromatosis type 2" }, { "code": "8C1Z", "title": "Mononeuropathy of unspecified site" }, { "code": "ND56.4", "title": "Injury of nerve of unspecified body region" }, { "code": "8B80", "title": "Disorders of olfactory nerve" }, { "code": "8C0Z", "title": "Polyneuropathy, unspecified" }, { "code": "9C40.Z", "title": "Disorder of the optic nerve, unspecified" } ]	=== ICD-11 CODES FOUND === [2F3Y] Benign neoplasms except of mesenchymal origin, of other specified site Also known as: Benign neoplasms except of mesenchymal origin, of other specified site \| Benign neoplasm of lymph vessels \| Benign neoplasm of lymph nodes \| Benign neoplasm of peripheral nerves or autonomic nervous system \| benign neoplasm of the nerve NOS [2F24] Benign cutaneous neoplasms of neural or nerve sheath origin Also known as: Benign cutaneous neoplasms of neural or nerve sheath origin \| Palisaded encapsulated neuroma \| Cutaneous neurofibroma \| Cutaneous schwannoma \| Cutaneous neurolemmoma [2A02.3] Benign neoplasm of cranial nerves Definition: This is a tumour of cranial nerves having none of the characteristics of a malignant neoplasm. Also known as: Benign neoplasm of cranial nerves \| neurinoma of unspecified site \| Vestibular schwannoma \| acoustic schwannoma \| Benign neoplasm of abducens nerve Includes: Vestibular schwannoma [2F30.Y] Other specified benign neoplasm of breast Also known as: Other specified benign neoplasm of breast \| Benign cystosarcoma phyllodes \| Breast apocrine adenoma \| Serocystic disease of Brodie \| Pleomorphic adenoma of breast [LD2D.11] Neurofibromatosis type 2 Definition: Neurofibromatosis type 2 (NF2) is a tumour-prone disorder characterised by the development of multiple schwannomas and meningiomas. Also known as: Neurofibromatosis type 2 \| Familial acoustic neuroma \| Familial vestibular schwannoma \| Neurofibromatosis, central type \| Bilateral acoustic neurofibromatosis [8C1Z] Mononeuropathy of unspecified site Also known as: Mononeuropathy of unspecified site \| inflammation of nerve NOS \| nerve condition NOS \| neuritis NOS \| nerve disease NOS [ND56.4] Injury of nerve of unspecified body region Also known as: Injury of nerve of unspecified body region \| injuries to nerves, nerve plexuses and roots \| injury to nerves, unspecified site \| nerve damage NOS \| Injury of nerve NOS Excludes: multiple injuries of nerves NOS [8B80] Disorders of olfactory nerve Also known as: Disorders of olfactory nerve \| disorders of olfactory [1st] nerve \| disorders of the first nerve \| first cranial nerve disorder \| disease of first cranial nerve Includes: Disorder of 1st cranial nerve Excludes: Idiopathic anosmia \| Idiopathic parosmia [8C0Z] Polyneuropathy, unspecified Also known as: Polyneuropathy, unspecified \| multiple neuropathy \| peripheral neuropathy NOS \| peripheral polyneuropathy \| multiple peripheral neuritis [9C40.Z] Disorder of the optic nerve, unspecified Also known as: Disorder of the optic nerve, unspecified \| Disorder of the optic nerve \| disease of optic cranial nerve \| disease of optic nerve \| disease of second cranial nerve === GRAPH WALKS === --- Walk 1 --- [2F3Y] Benign neoplasms except of mesenchymal origin, of other specified site --PARENT--> [?] Benign neoplasms except of mesenchymal origin --CHILD--> [2E91] Benign neoplasm of major salivary glands --- Walk 2 --- [2F3Y] Benign neoplasms except of mesenchymal origin, of other specified site --PARENT--> [?] Benign neoplasms except of mesenchymal origin --CHILD--> [2E91] Benign neoplasm of major salivary glands --- Walk 3 --- [2F24] Benign cutaneous neoplasms of neural or nerve sheath origin --PARENT--> [?] Benign cutaneous neoplasms Def: Abnormal growth of the cells that comprise the tissues of the skin, without any evidence of malignancy.... --CHILD--> [2F22] Benign neoplasms of epidermal appendages Def: A range of benign neoplasms arising from the hair follicle, its associated glands or from sweat glands.... --- Walk 4 --- [2F24] Benign cutaneous neoplasms of neural or nerve sheath origin --PARENT--> [?] Benign cutaneous neoplasms Def: Abnormal growth of the cells that comprise the tissues of the skin, without any evidence of malignancy.... --PARENT--> [?] Benign neoplasms except of mesenchymal origin --- Walk 5 --- [2A02.3] Benign neoplasm of cranial nerves Def: This is a tumour of cranial nerves having none of the characteristics of a malignant neoplasm.... --PARENT--> [2A02] Primary neoplasm of spinal cord, cranial nerves, paraspinal nerves or remaining parts of central nervous system --CHILD--> [2A02.2] Primary neoplasm of spinal cord of unknown or unspecified type --- Walk 6 --- [2A02.3] Benign neoplasm of cranial nerves Def: This is a tumour of cranial nerves having none of the characteristics of a malignant neoplasm.... --PARENT--> [2A02] Primary neoplasm of spinal cord, cranial nerves, paraspinal nerves or remaining parts of central nervous system --PARENT--> [?] Neoplasms of central nervous system or related structures Def: A benign or malignant neoplasm that affects the brain, meninges, or spinal cord. Representative examples of primary neoplasms include astrocytoma, oligodendroglioma, ependymoma, and meningioma....	[ "[2F3Y] Benign neoplasms except of mesenchymal origin, of other specified site\n --PARENT--> [?] Benign neoplasms except of mesenchymal origin\n --CHILD--> [2E91] Benign neoplasm of major salivary glands", "[2F3Y] Benign neoplasms except of mesenchymal origin, of other specified site\n --PARENT--> [?] Benign neoplasms except of mesenchymal origin\n --CHILD--> [2E91] Benign neoplasm of major salivary glands", "[2F24] Benign cutaneous neoplasms of neural or nerve sheath origin\n --PARENT--> [?] Benign cutaneous neoplasms\n Def: Abnormal growth of the cells that comprise the tissues of the skin, without any evidence of malignancy....\n --CHILD--> [2F22] Benign neoplasms of epidermal appendages\n Def: A range of benign neoplasms arising from the hair follicle, its associated glands or from sweat glands....", "[2F24] Benign cutaneous neoplasms of neural or nerve sheath origin\n --PARENT--> [?] Benign cutaneous neoplasms\n Def: Abnormal growth of the cells that comprise the tissues of the skin, without any evidence of malignancy....\n --PARENT--> [?] Benign neoplasms except of mesenchymal origin", "[2A02.3] Benign neoplasm of cranial nerves\n Def: This is a tumour of cranial nerves having none of the characteristics of a malignant neoplasm....\n --PARENT--> [2A02] Primary neoplasm of spinal cord, cranial nerves, paraspinal nerves or remaining parts of central nervous system\n --CHILD--> [2A02.2] Primary neoplasm of spinal cord of unknown or unspecified type", "[2A02.3] Benign neoplasm of cranial nerves\n Def: This is a tumour of cranial nerves having none of the characteristics of a malignant neoplasm....\n --PARENT--> [2A02] Primary neoplasm of spinal cord, cranial nerves, paraspinal nerves or remaining parts of central nervous system\n --PARENT--> [?] Neoplasms of central nervous system or related structures\n Def: A benign or malignant neoplasm that affects the brain, meninges, or spinal cord. Representative examples of primary neoplasms include astrocytoma, oligodendroglioma, ependymoma, and meningioma...." ]	2F3Y	Benign neoplasms except of mesenchymal origin, of other specified site	[ { "from_icd11": "2A02.3", "icd10_code": "D333", "icd10_title": "Benign neoplasm of cranial nerves" }, { "from_icd11": "8C1Z", "icd10_code": "G59", "icd10_title": "Mononeuropathy in diseases classified elsewhere" }, { "from_icd11": "8C1Z", "icd10_code": "G598", "icd10_title": "" }, { "from_icd11": "ND56.4", "icd10_code": "T144", "icd10_title": "" }, { "from_icd11": "8B80", "icd10_code": "G520", "icd10_title": "Disorders of olfactory nerve" }, { "from_icd11": "8C0Z", "icd10_code": "G629", "icd10_title": "Polyneuropathy, unspecified" }, { "from_icd11": "8C0Z", "icd10_code": "G53", "icd10_title": "Cranial nerve disorders in diseases classified elsewhere" }, { "from_icd11": "8C0Z", "icd10_code": "G538", "icd10_title": "" }, { "from_icd11": "9C40.Z", "icd10_code": "H47012", "icd10_title": "Ischemic optic neuropathy, left eye" }, { "from_icd11": "9C40.Z", "icd10_code": "H47099", "icd10_title": "Other disorders of optic nerve, not elsewhere classified, unspecified eye" }, { "from_icd11": "9C40.Z", "icd10_code": "H47091", "icd10_title": "Other disorders of optic nerve, not elsewhere classified, right eye" }, { "from_icd11": "9C40.Z", "icd10_code": "H47093", "icd10_title": "Other disorders of optic nerve, not elsewhere classified, bilateral" }, { "from_icd11": "9C40.Z", "icd10_code": "H47019", "icd10_title": "Ischemic optic neuropathy, unspecified eye" }, { "from_icd11": "9C40.Z", "icd10_code": "H47013", "icd10_title": "Ischemic optic neuropathy, bilateral" }, { "from_icd11": "9C40.Z", "icd10_code": "H47011", "icd10_title": "Ischemic optic neuropathy, right eye" } ]	D333	Benign neoplasm of cranial nerves
We first visited the patient BG in July 2004, when she was 90 years old. She was a right-handed woman, with 5 years of education, and she was formerly a seamstress. She had one daughter and had been a widow for 10 years. BG lived alone in a flat, with a good level of autonomy in her daily living. She had no previous history of severe neurological or psychopathological disorders, alcoholism, or medical conditions, which could potentially cause cognitive impairment or psychiatric symptoms. She had never smoked and now suffered from mild hypertension. Her family history was positive for ischemic heart attack and stroke and negative for dementia. BG had no visual or auditory sensory impairments. During the visit, albeit reluctantly, she referred to a history of frequent complex hallucinations that started approximately 10 years previously, precisely, on June 2, 1994, when she was nearly 80 years old. She was able to be accurate in dating the onset of hallucinations because the second day of June is the Italian Republic anniversary, and exactly three months before, on March 2 of the same year, her husband had died. Hallucinations had been particularly evident during the last two years and had increased in frequency over the ten months before referral. BG, mainly, when relaxed on her bed before sleeping or during the night, saw people, heard their voices, or felt herself touched by them. These people were mostly unfamiliar adults. At other times, her husband or a pretty round-headed child appeared inside a see-through case. The phenomena lasted only few seconds or few minutes and made BG alternately surprised and curious or annoyed and irritated. The “wandering souls,” in BG's words, inhabited her house and appeared mostly in her bedroom, inside the pillow or the mattress, and in the bathroom. People usually spoke aloud or whispered to her and to each other, attended to her, hit her body as she lay down on the bed, or made some grooves in the bed. The patient and her daughter did not report cognitive impairments or other neurological symptoms aside from the hallucinations. A neurological exam was fully negative. No cognitive impairment emerged on a mini mental state examination (MMSE = 29/30) , and no signs of depression appeared on the Geriatric Depression Scale (GDS = 5/30) . Blood tests were normal, and the Hachinski scale score was 3 points. A CT-scan revealed mild diffuse white matter vascular damage with leukoaraiosis and small, disseminate ischemic microlesions as well as very mild cortical atrophy. At this point, we were not able to make a definite diagnosis; there were different hypotheses regarding the origin of the complex hallucinations, and they all seemed acceptable (e.g., functional psychiatric illness, CBS, preclinical vascular dementia with psychosis, preclinical DLB, etc.). To improve the case study, we planned to analyze the phenomenology of hallucinations and to deeply examine the cognitive profile. Thus, in the aftermath, we interviewed BG and performed a selective neuropsychological assessment. Because some slight cognitive deficits without a diffuse cognitive decline had been highlighted in a neuropsychological evaluation, we made a diagnosis of MCI of unknown etiology. No therapy for hallucinations was administered because BG did not seem to be very distressed by them. The clinical picture remained stable over three years as shown by subsequent follow-up examinations, until October 2007, when we suddenly and quickly registered a deterioration of cognitive functions (see Table 1 ). Her MMSE score was 25 points in October, and after only two months, in December, it was 18 points. Her autonomy in daily living had become severely impaired (activities of daily living scale, ADL = 3/6; instrumental activities of daily living scale, IADL = 1/8) . In January 2008, her MMSE score was 15, and a further neuropsychological evaluation showed severe temporal and spatial disorientation, attention impairments, a dysexecutive prefrontal syndrome, and reduced insight. Anterograde and retrograde amnesia with temporal transpositions of recalled episodes and autobiographical dysmnesia were also present. Conversational speech showed signs of dysarthria and dysphasia, with slow articulation, word-finding problems, and some phonological paraphasias. Moreover, BG's speech was often confused, and we noticed auditory comprehension difficulties. Relatives referred dressing apraxia. Moreover, constructional apraxia with closing-in phenomena was revealed by a psychometric test. A complex delusional misidentification syndrome emerged, with simple misidentifications of her relatives; Capgras syndrome with reduplication involving her daughter; misidentification of her own self in the mirror; delusions of inanimate objects, mostly concerning furniture; and, finally, signs of reduplicative paramnesia involving her home. Other types of delusions also emerged; for example, BG hid money and jewelry because she was frightened that the double of her daughter might steal them. Finally, psychomotor slowness and remarkable cognitive and attentive fluctuations were apparent. Behavioral assessment revealed purposeless motor activity, akathisia, and hostility. Relatives did not report any symptoms suggestive of RBD. Complex hallucinations, after being present for over 13 years, had now completely ceased. Occasionally, BG remembered people who had been the subjects of her hallucinations and addressed them as “those who disturbed me,” but she said that she had no longer “seen” them or “listened” to their voices. A neurological exam revealed a rigid-akinetic parkinsonism, with marked bradykinesia and bradyphrenia, mild rigidity, and increased muscular tone. A follow-up CT-scan confirmed mild diffuse cerebral vascular damage to the white matter (periventricular damage and small, dispersed ischemic microlesions), with no new ischemic lesions, and revealed more pronounced cortical and subcortical atrophy . Blood tests were normal. At this point, when BG was 93 years old, dementia was evident. We made a diagnosis of probable DLB, according to known criteria and suggested therapy with rivastigmine. In May 2008, BG's MMSE score was 10 and there was a further reduction of autonomy in daily living (ADL = 2/6 and IADL = 1/8). Further evaluations were no longer possible because of severe dementia.	3.943359	0.979492	sec[1]/sec[0]/p[0]	en	0.999996	24868122	https://doi.org/10.1155/2014/694296	[ "hallucinations", "cognitive", "daily", "living", "because", "them", "daughter", "neurological", "ischemic", "dementia" ]	[ { "code": "MB27.2Z", "title": "Hallucinations, unspecified" }, { "code": "MB27.2Y", "title": "Other specified hallucinations" }, { "code": "6E61.0", "title": "Secondary psychotic syndrome, with hallucinations" }, { "code": "MB27.27", "title": "Visual hallucinations" }, { "code": "MB27.20", "title": "Auditory hallucinations" }, { "code": "MB21.Z", "title": "Symptoms, signs or clinical findings involving cognition, unspecified" }, { "code": "6D81", "title": "Dementia due to cerebrovascular disease" }, { "code": "8D43.1", "title": "Cognitive impairment due to toxicity" }, { "code": "6D71", "title": "Mild neurocognitive disorder" }, { "code": "6B60.9", "title": "Dissociative neurological symptom disorder, with cognitive symptoms" } ]	=== ICD-11 CODES FOUND === [MB27.2Z] Hallucinations, unspecified Also known as: Hallucinations, unspecified \| Hallucinations [MB27.2Y] Other specified hallucinations Also known as: Other specified hallucinations \| Hallucinosis \| Cacosmia [6E61.0] Secondary psychotic syndrome, with hallucinations Definition: A syndrome characterised by the presence of prominent hallucinations that is judged to be a direct pathophysiological consequence of a health condition not classified under mental and behavioural disorders, based on evidence from the history, physical examination, or laboratory findings. Delusions are not a prominent aspect of the clinical presentation. The symptoms are not accounted for by delirium or by another mental and behavioural disorder, and are not a psychologically mediated response to Also known as: Secondary psychotic syndrome, with hallucinations \| psychotic disorder with hallucinations in conditions classified elsewhere \| transient organic psychotic condition, hallucinatory type \| Organic hallucinatory state \| organic hallucination Excludes: Delirium \| Mood disorders [MB27.27] Visual hallucinations Definition: Hallucinations involving sight in the absence of an actual visual stimulus that are not restricted to the period of awakening or the onset of sleep. Visual hallucinations may involve formed images, such as of people, or of unformed images, such as flashes of light. Visual hallucinations must be distinguished from illusions, which are visual misperceptions of real external stimuli. Also known as: Visual hallucinations \| seeing things [MB27.20] Auditory hallucinations Definition: Hallucinations involving the perception of sound, most frequently of voices but sometimes of clicks or other noises, that are not restricted to the period of awakening or the onset of sleep. Also known as: Auditory hallucinations [MB21.Z] Symptoms, signs or clinical findings involving cognition, unspecified Also known as: Symptoms, signs or clinical findings involving cognition, unspecified \| Symptoms, signs or clinical findings involving cognition \| cognitive impairment NOS [6D81] Dementia due to cerebrovascular disease Definition: Dementia due to brain parenchyma injury resulting from cerebrovascular disease (ischemic or haemorrhagic). The onset of the cognitive deficits is temporally related to one or more vascular events. Cognitive decline is typically most prominent in speed of information processing, complex attention, and frontal-executive functioning. There is evidence of the presence of cerebrovascular disease considered to be sufficient to account for the neurocognitive deficits from history, physical examination Also known as: Dementia due to cerebrovascular disease \| arteriosclerotic dementia \| Strategic-infarct dementia \| Post stroke dementia \| vascular cognitive impairment Excludes: Alzheimer disease dementia, mixed type, with cerebrovascular disease [8D43.1] Cognitive impairment due to toxicity Definition: These are conditions of impaired cognition due to the toxicity of substances. Also known as: Cognitive impairment due to toxicity \| Cognitive impairment due to lead toxicity [6D71] Mild neurocognitive disorder Definition: Mild neurocognitive disorder is characterized by mild impairment in one or more cognitive domains relative to that expected given the individual’s age and general premorbid level of cognitive functioning, which represents a decline from the individual’s previous level of functioning. Diagnosis is based on report from the patient, informant, or clinical observation, and is accompanied by objective evidence of impairment by quantified clinical assessment or standardized cognitive testing. Cognitiv Also known as: Mild neurocognitive disorder \| minor neurocognitive disorder \| Postconcussional syndrome \| post-concussion syndrome \| post-concussional syndrome [6B60.9] Dissociative neurological symptom disorder, with cognitive symptoms Definition: Dissociative neurological symptom disorder, with cognitive symptoms is characterised by impaired cognitive performance in memory, language or other cognitive domains that is internally inconsistent and not consistent with a recognised disease of the nervous system, a neurodevelopmental or neurocognitive disorder, other mental, behavioural or neurodevelopmental disorder, or another medical condition and does not occur exclusively during another dissociative disorder. Also known as: Dissociative neurological symptom disorder, with cognitive symptoms \| Functional neurological symptom disorder, with cognitive symptoms Excludes: Dissociative amnesia === GRAPH WALKS === --- Walk 1 --- [MB27.2Z] Hallucinations, unspecified --PARENT--> [MB27.2] Hallucinations Def: Sensory perceptions of any modality occurring in the absence of the appropriate (external) stimulus. The person may or may not have insight into the unreal nature of the perception.... --CHILD--> [MB27.21] Gustatory hallucinations Def: Hallucinations of taste in the absence of an actual external stimulus.... --- Walk 2 --- [MB27.2Z] Hallucinations, unspecified --PARENT--> [MB27.2] Hallucinations Def: Sensory perceptions of any modality occurring in the absence of the appropriate (external) stimulus. The person may or may not have insight into the unreal nature of the perception.... --CHILD--> [MB27.21] Gustatory hallucinations Def: Hallucinations of taste in the absence of an actual external stimulus.... --- Walk 3 --- [MB27.2Y] Other specified hallucinations --PARENT--> [MB27.2] Hallucinations Def: Sensory perceptions of any modality occurring in the absence of the appropriate (external) stimulus. The person may or may not have insight into the unreal nature of the perception.... --CHILD--> [MB27.21] Gustatory hallucinations Def: Hallucinations of taste in the absence of an actual external stimulus.... --- Walk 4 --- [MB27.2Y] Other specified hallucinations --PARENT--> [MB27.2] Hallucinations Def: Sensory perceptions of any modality occurring in the absence of the appropriate (external) stimulus. The person may or may not have insight into the unreal nature of the perception.... --CHILD--> [MB27.22] Hypnopompic hallucinations Def: Hallucinations that occur during the period of awakening, most commonly of the visual, tactile or auditory modality.... --- Walk 5 --- [6E61.0] Secondary psychotic syndrome, with hallucinations Def: A syndrome characterised by the presence of prominent hallucinations that is judged to be a direct pathophysiological consequence of a health condition not classified under mental and behavioural diso... --EXCLUDES--> [?] Mood disorders Def: Mood Disorders refers to a superordinate grouping of Bipolar and Depressive Disorders. Mood disorders are defined according to particular types of mood episodes and their pattern over time. The primar... --CHILD--> [?] Depressive disorders Def: Depressive disorders are characterised by depressive mood (e.g., sad, irritable, empty) or loss of pleasure accompanied by other cognitive, behavioural, or neurovegetative symptoms that significantly ... --- Walk 6 --- [6E61.0] Secondary psychotic syndrome, with hallucinations Def: A syndrome characterised by the presence of prominent hallucinations that is judged to be a direct pathophysiological consequence of a health condition not classified under mental and behavioural diso... --EXCLUDES--> [?] Mood disorders Def: Mood Disorders refers to a superordinate grouping of Bipolar and Depressive Disorders. Mood disorders are defined according to particular types of mood episodes and their pattern over time. The primar... --CHILD--> [?] Symptomatic and course presentations for mood episodes in mood disorders Def: These categories may be applied to describe the presentation and characteristics of mood episodes in the context of single episode depressive disorder, recurrent depressive disorder, bipolar type I di...	[ "[MB27.2Z] Hallucinations, unspecified\n --PARENT--> [MB27.2] Hallucinations\n Def: Sensory perceptions of any modality occurring in the absence of the appropriate (external) stimulus. The person may or may not have insight into the unreal nature of the perception....\n --CHILD--> [MB27.21] Gustatory hallucinations\n Def: Hallucinations of taste in the absence of an actual external stimulus....", "[MB27.2Z] Hallucinations, unspecified\n --PARENT--> [MB27.2] Hallucinations\n Def: Sensory perceptions of any modality occurring in the absence of the appropriate (external) stimulus. The person may or may not have insight into the unreal nature of the perception....\n --CHILD--> [MB27.21] Gustatory hallucinations\n Def: Hallucinations of taste in the absence of an actual external stimulus....", "[MB27.2Y] Other specified hallucinations\n --PARENT--> [MB27.2] Hallucinations\n Def: Sensory perceptions of any modality occurring in the absence of the appropriate (external) stimulus. The person may or may not have insight into the unreal nature of the perception....\n --CHILD--> [MB27.21] Gustatory hallucinations\n Def: Hallucinations of taste in the absence of an actual external stimulus....", "[MB27.2Y] Other specified hallucinations\n --PARENT--> [MB27.2] Hallucinations\n Def: Sensory perceptions of any modality occurring in the absence of the appropriate (external) stimulus. The person may or may not have insight into the unreal nature of the perception....\n --CHILD--> [MB27.22] Hypnopompic hallucinations\n Def: Hallucinations that occur during the period of awakening, most commonly of the visual, tactile or auditory modality....", "[6E61.0] Secondary psychotic syndrome, with hallucinations\n Def: A syndrome characterised by the presence of prominent hallucinations that is judged to be a direct pathophysiological consequence of a health condition not classified under mental and behavioural diso...\n --EXCLUDES--> [?] Mood disorders\n Def: Mood Disorders refers to a superordinate grouping of Bipolar and Depressive Disorders. Mood disorders are defined according to particular types of mood episodes and their pattern over time. The primar...\n --CHILD--> [?] Depressive disorders\n Def: Depressive disorders are characterised by depressive mood (e.g., sad, irritable, empty) or loss of pleasure accompanied by other cognitive, behavioural, or neurovegetative symptoms that significantly ...", "[6E61.0] Secondary psychotic syndrome, with hallucinations\n Def: A syndrome characterised by the presence of prominent hallucinations that is judged to be a direct pathophysiological consequence of a health condition not classified under mental and behavioural diso...\n --EXCLUDES--> [?] Mood disorders\n Def: Mood Disorders refers to a superordinate grouping of Bipolar and Depressive Disorders. Mood disorders are defined according to particular types of mood episodes and their pattern over time. The primar...\n --CHILD--> [?] Symptomatic and course presentations for mood episodes in mood disorders\n Def: These categories may be applied to describe the presentation and characteristics of mood episodes in the context of single episode depressive disorder, recurrent depressive disorder, bipolar type I di..." ]	MB27.2Z	Hallucinations, unspecified	[ { "from_icd11": "MB27.2Z", "icd10_code": "R443", "icd10_title": "Hallucinations, unspecified" }, { "from_icd11": "MB27.2Z", "icd10_code": "R442", "icd10_title": "Other hallucinations" }, { "from_icd11": "6E61.0", "icd10_code": "F060", "icd10_title": "Psychotic disorder with hallucinations due to known physiological condition" }, { "from_icd11": "MB27.27", "icd10_code": "R441", "icd10_title": "Visual hallucinations" }, { "from_icd11": "MB27.20", "icd10_code": "R440", "icd10_title": "Auditory hallucinations" }, { "from_icd11": "MB21.Z", "icd10_code": "R419", "icd10_title": "Unspecified symptoms and signs involving cognitive functions and awareness" }, { "from_icd11": "MB21.Z", "icd10_code": "R414", "icd10_title": "Neurologic neglect syndrome" }, { "from_icd11": "MB21.Z", "icd10_code": "R41", "icd10_title": "Other symptoms and signs involving cognitive functions and awareness" }, { "from_icd11": "6D81", "icd10_code": "F0150", "icd10_title": "Vascular dementia without behavioral disturbance" }, { "from_icd11": "6D81", "icd10_code": "F0151", "icd10_title": "Vascular dementia with behavioral disturbance" }, { "from_icd11": "6D81", "icd10_code": "F00-F09", "icd10_title": "" }, { "from_icd11": "6D81", "icd10_code": "F01", "icd10_title": "Vascular dementia" }, { "from_icd11": "6D81", "icd10_code": "F010", "icd10_title": "" }, { "from_icd11": "6D81", "icd10_code": "F011", "icd10_title": "" }, { "from_icd11": "6D81", "icd10_code": "F012", "icd10_title": "" } ]	R443	Hallucinations, unspecified
In 2003, a 36-year-old Caucasian woman was referred to the hospital with right upper abdominal pain. An initial ultrasound of the liver demonstrated a large unclear lesion of the left lobe of the liver. Serum level of y-GT was slightly elevated at 84 U/mL. The AFP value was negative. Medical history of the patient revealed a slight mitral valve prolapse, latent hypothyroidism, a condition after reconstructive burn surgery in 1969 and previous nicotine consumption. There was no evidence for any underlying liver disease, including viral hepatitis, non-alcoholic fatty liver, autoimmune pathologies, or genetic metabolic disorders. No intakes of alcohol, hepatotoxic drugs, or hormone substitution and no contact with hepatotoxins were documented. Magnet resonance imaging (MRI) findings were consistent with a liver adenoma. However, a highly differentiated HCC could not be ruled out. According to the decision of our interdisciplinary tumor board and after excluding extra-hepatic tumor manifestations (colonoscopy, gastroscopy, CT scan, and whole body FDG-PET/CT scan), an explorative laparotomy with the intent of a left hepatectomy was performed. Intra-operatively, an approx. 9.5 cm large tumor of the left liver lobe was resected. Histopathologic specimen confirmed a moderately differentiated HCC in a NC liver . However, intra-operatively performed ultrasound detected irresectable multifocal metastases in the right liver. In December 2003, as part of an individual therapy concept, the patient received an orthotopic LDLT of the right hepatic lobe from her mother. LDLT was approved by the ethics committee of the Medical Chamber of North Rhine-Westphalia, Germany. The liver explant contained up to further 7 lesions of a highly differentiated HCC. The definitive tumor stage of the explanted liver was pT3 pN0 (0/4) M0 with no microvascular involvement. Immunosuppression was initially started with tacrolimus and mycophenolate mofetil. In the following time period, the patient showed an excellent graft function. CT and MRI scans were carried out at intervals of about 3 to 6 months as follow-up. Seven years later, in February 2010, CT scan revealed lung metastases . Further tumor manifestations were ruled out with FDG-PET/CT scan and MRI of the liver. Immunosuppression was switched to sirolimus. According to the decision of our interdisciplinary tumor board, resection of the pulmonary lesions with curative intention was performed. In February 2010, May 2010, October 2013, and November 2013, further partial lung resections of new lung metastases were performed. Histologically, metastases of the previously known HCC were confirmed . In January 2014, an atypical liver resection was performed due to intra-hepatic HCC recurrence. Pulmonary segment resections followed again in July 2014, November 2014, and August 2015. Because of a new intra-hepatic HCC recurrence in January 2015, RFA of 2 liver metastases was performed. After yet another tumor recurrence in the lung with confirmation of HCC cells in the cytology by bronchoscopy in December 2015, our tumor board decided to start palliative first line systemic therapy with sorafenib. Sorafenib therapy with 800 mg per day was accompanied by grade 3 hand-foot syndrome and pronounced head pruritus. Even after dose reduction to initially 600 mg after 1 month and later 400 mg per day, sorafenib had to be discontinued in February 2016 due to intolerable grade 3 to 4 side effects, such as mucositis, hand-foot skin reaction, diarrhea, liver dysfunction with transaminase elevation, and hyperthyroidism, requiring hospitalization of the patient. Several weeks after the discontinuation of sorafenib and switching the immunosuppression to tacrolimus again, the patient recovered from the side effects. However, grade 1 hyperthyroidism and hand-foot skin reaction persisted. An initial second line therapy with regorafenib was contraindicated, since the patient did not tolerate sorafenib. In February 2017, a further progression of the HCC showing a mediastinal lymph node was documented by CT scan and confirmed by FDG-PET/CT scan . In April 2017, due to a lack of further systemic therapeutic options, it was decided to perform right lateral resection of the mediastinal lymph node metastases. The histology again confirmed HCC spreading. Further solitary pulmonary recurrences of HCC could be treated locally by CT-guided RFA in October 2017 and November 2017. In April 2018, MRI scan of the liver revealed newly appeared high-grade HCC-suspected lesions subcapsular in liver segment 8 (10 × 7 mm) and at the segment border 6/7 (7 × 6 mm). After tumor board discussion, a switch to an off label second line systemic therapy with cabozantinib was recommended. Thus, from June 2018 to April 2019, our patient received second line therapy with cabozantinib. An initial start with 40 mg was accompanied by mild (grade 1–2) oropharyngeal mucositis and mild hypertension, both of which were easy to manage. Therefore, the dose could be increased to 60 mg per day with acceptable tolerability. Occasionally, due to mild mucositis and abdominal distension, cabozantinib had to be temporarily reduced to 40 or 20 mg. After 11 months of therapy with cabozantinib, the CT scan revealed complete remission of the pulmonary lesions and low partial remission of the liver metastases as a best response to cabozantinib. After discussion of the case in our interdisciplinary tumor conference, a new round of RFA of the remaining liver metastasis could be completed in April 2019, following discontinuation of cabozantinib. Unfortunately, 2 months later, new progression of the pulmonary metastasis was observed and the therapy with cabozantinib had to be restarted in August 2019. Again, partial remission of all tumor manifestations in the lung was achieved and the therapy with cabozantinib was well tolerated with only mild arterial hypertension, grade 1 to 2 mucositis and gastrointestinal symptoms, all of which could be easily managed. In the further course of therapy, the initial dose of 40 mg/day could be increased to 60 mg/day, while the dose was adjusted to 20 to 60 mg to control the gastrointestinal side effects and mucositis. Liver transplant function and blood count cells remained stable during therapy and no drug interaction with immunosuppressive drugs was observed at any time .	3.958984	0.978516	sec[1]/p[0]	en	0.999996	34559100	https://doi.org/10.1097/MD.0000000000027082	[ "liver", "tumor", "scan", "cabozantinib", "metastases", "grade", "lung", "pulmonary", "sorafenib", "mucositis" ]	[ { "code": "DB9Z", "title": "Diseases of liver, unspecified" }, { "code": "DB97.Z", "title": "Inflammatory liver disease, unspecified" }, { "code": "DB99.7", "title": "Hepatic failure without mention whether acute or chronic" }, { "code": "LB20.0Y", "title": "Other specified structural developmental anomalies of liver" }, { "code": "LB20.0Z", "title": "Structural developmental anomalies of liver, unspecified" }, { "code": "2F9Z", "title": "Neoplasms of unknown behaviour of unspecified site" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "2E6Z", "title": "Carcinoma in situ of unspecified site" }, { "code": "2F91.1", "title": "Neoplasms of unknown behaviour of trachea, bronchus or lung" }, { "code": "2F92", "title": "Neoplasms of unknown behaviour of skin" } ]	=== ICD-11 CODES FOUND === [DB9Z] Diseases of liver, unspecified Also known as: Diseases of liver, unspecified \| liver disease \| liver condition NOS \| organ liver disease \| hepatopathy [DB97.Z] Inflammatory liver disease, unspecified Also known as: Inflammatory liver disease, unspecified \| Certain specified inflammatory liver diseases \| Nonspecific reactive hepatitis \| inflammatory liver disease \| hepatitis NOS [DB99.7] Hepatic failure without mention whether acute or chronic Also known as: Hepatic failure without mention whether acute or chronic \| liver decompensation \| liver function failure \| hepatic failure NOS \| liver failure NOS [LB20.0Y] Other specified structural developmental anomalies of liver Also known as: Other specified structural developmental anomalies of liver \| Alagille syndrome \| Alagille-Watson syndrome \| Arteriohepatic dysplasia \| Syndromic bile duct paucity [LB20.0Z] Structural developmental anomalies of liver, unspecified Also known as: Structural developmental anomalies of liver, unspecified \| Structural developmental anomalies of liver \| Malformations of liver \| congenital anomaly of liver \| congenital malformation of liver [2F9Z] Neoplasms of unknown behaviour of unspecified site Also known as: Neoplasms of unknown behaviour of unspecified site \| neoplasia \| neoplasm growth NOS \| tumour of unspecified site \| tumourlet of unspecified site [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [2E6Z] Carcinoma in situ of unspecified site Also known as: Carcinoma in situ of unspecified site \| carcinoma in situ of unspecified site \| carcinoma in situ NOS \| carcinoma in situ \| in situ neoplasm [2F91.1] Neoplasms of unknown behaviour of trachea, bronchus or lung Also known as: Neoplasms of unknown behaviour of trachea, bronchus or lung \| trachea, bronchus or lung tumour NOS \| Intravascular bronchial alveolar tumour of unspecified site \| Bronchial adenoma of unknown behaviour of unspecified site \| Intravascular bronchial alveolar tumour unknown behaviour of unspecified site [2F92] Neoplasms of unknown behaviour of skin Also known as: Neoplasms of unknown behaviour of skin \| skin tumour NOS === GRAPH WALKS === --- Walk 1 --- [DB9Z] Diseases of liver, unspecified --PARENT--> [?] Diseases of liver --CHILD--> [DB90] Infectious liver disease --- Walk 2 --- [DB9Z] Diseases of liver, unspecified --PARENT--> [?] Diseases of liver --RELATED_TO--> [?] Structural developmental anomalies of liver --- Walk 3 --- [DB97.Z] Inflammatory liver disease, unspecified --PARENT--> [DB97] Certain specified inflammatory liver diseases --EXCLUDES--> [?] Acute viral hepatitis Def: A group of liver diseases characterised by liver inflammation and fibrosis, caused by less than 6 months of infection with one or more of hepatitis B virus, hepatitis C virus and hepatitis D virus, wi... --- Walk 4 --- [DB97.Z] Inflammatory liver disease, unspecified --PARENT--> [DB97] Certain specified inflammatory liver diseases --CHILD--> [DB97.2] Chronic hepatitis, not elsewhere classified --- Walk 5 --- [DB99.7] Hepatic failure without mention whether acute or chronic --PARENT--> [DB99] Certain specified diseases of liver Def: This is a group of conditions characterised as being in or associated with the liver that are not classified elsewhere.... --CHILD--> [DB99.1] Hepatic cyst Def: This is a closed sac, having a distinct membrane and division compared to the nearby tissue. It may contain air, fluids, or semi-solid material of the liver.... --- Walk 6 --- [DB99.7] Hepatic failure without mention whether acute or chronic --PARENT--> [DB99] Certain specified diseases of liver Def: This is a group of conditions characterised as being in or associated with the liver that are not classified elsewhere.... --RELATED_TO--> [?] Cirrhotic cardiomyopathy Def: Cirrhotic cardiomyopathy is defined as chronic cardiac dysfunction in patients with cirrhosis characterised by blunted contractile responsiveness to stress/exercise and-or altered diastolic relaxation...	[ "[DB9Z] Diseases of liver, unspecified\n --PARENT--> [?] Diseases of liver\n --CHILD--> [DB90] Infectious liver disease", "[DB9Z] Diseases of liver, unspecified\n --PARENT--> [?] Diseases of liver\n --RELATED_TO--> [?] Structural developmental anomalies of liver", "[DB97.Z] Inflammatory liver disease, unspecified\n --PARENT--> [DB97] Certain specified inflammatory liver diseases\n --EXCLUDES--> [?] Acute viral hepatitis\n Def: A group of liver diseases characterised by liver inflammation and fibrosis, caused by less than 6 months of infection with one or more of hepatitis B virus, hepatitis C virus and hepatitis D virus, wi...", "[DB97.Z] Inflammatory liver disease, unspecified\n --PARENT--> [DB97] Certain specified inflammatory liver diseases\n --CHILD--> [DB97.2] Chronic hepatitis, not elsewhere classified", "[DB99.7] Hepatic failure without mention whether acute or chronic\n --PARENT--> [DB99] Certain specified diseases of liver\n Def: This is a group of conditions characterised as being in or associated with the liver that are not classified elsewhere....\n --CHILD--> [DB99.1] Hepatic cyst\n Def: This is a closed sac, having a distinct membrane and division compared to the nearby tissue. It may contain air, fluids, or semi-solid material of the liver....", "[DB99.7] Hepatic failure without mention whether acute or chronic\n --PARENT--> [DB99] Certain specified diseases of liver\n Def: This is a group of conditions characterised as being in or associated with the liver that are not classified elsewhere....\n --RELATED_TO--> [?] Cirrhotic cardiomyopathy\n Def: Cirrhotic cardiomyopathy is defined as chronic cardiac dysfunction in patients with cirrhosis characterised by blunted contractile responsiveness to stress/exercise and-or altered diastolic relaxation..." ]	DB9Z	Diseases of liver, unspecified	[ { "from_icd11": "DB9Z", "icd10_code": "K7681", "icd10_title": "Hepatopulmonary syndrome" }, { "from_icd11": "DB9Z", "icd10_code": "K7689", "icd10_title": "Other specified diseases of liver" }, { "from_icd11": "DB9Z", "icd10_code": "K769", "icd10_title": "Liver disease, unspecified" }, { "from_icd11": "DB9Z", "icd10_code": "K77", "icd10_title": "Liver disorders in diseases classified elsewhere" }, { "from_icd11": "DB9Z", "icd10_code": "K762", "icd10_title": "Central hemorrhagic necrosis of liver" }, { "from_icd11": "DB9Z", "icd10_code": "K70-K77", "icd10_title": "" }, { "from_icd11": "DB9Z", "icd10_code": "K778", "icd10_title": "" }, { "from_icd11": "DB9Z", "icd10_code": "K72", "icd10_title": "Hepatic failure, not elsewhere classified" }, { "from_icd11": "DB9Z", "icd10_code": "K76", "icd10_title": "Other diseases of liver" }, { "from_icd11": "DB9Z", "icd10_code": "K768", "icd10_title": "Other specified diseases of liver" }, { "from_icd11": "DB97.Z", "icd10_code": "K7581", "icd10_title": "Nonalcoholic steatohepatitis (NASH)" }, { "from_icd11": "DB97.Z", "icd10_code": "K7589", "icd10_title": "Other specified inflammatory liver diseases" }, { "from_icd11": "DB97.Z", "icd10_code": "K759", "icd10_title": "Inflammatory liver disease, unspecified" }, { "from_icd11": "DB97.Z", "icd10_code": "K752", "icd10_title": "Nonspecific reactive hepatitis" }, { "from_icd11": "DB97.Z", "icd10_code": "K75", "icd10_title": "Other inflammatory liver diseases" } ]	K7681	Hepatopulmonary syndrome
He was brought to our hospital because he was diagnosed with anemia (hemoglobin [Hb] 8.0 g/dL) during a medical checkup. During EGD, whitish, edematous multiple polyps in the antrum and corpus of the stomach without hemorrhages were noted, which made us suspect FGPs according to endoscopic findings, and a biopsy was subsequently performed . Pathological findings revealed dilated and proliferated fundic glands with foveolar hyperplasia. A colon endoscopy did not reveal any abnormalities. However, the patient complained of general fatigue and a large amount of tarry stools was observed. The patient was admitted to our hospital 8 days after his initial EGD. The patient’s family history was unremarkable and he did not have a history of allergies. His alcohol consumption was around 350 mL/day of beer for 20 years. A blood test showed iron deficiency anemia. Serum anti- H. pylori antibody level and antigen in stool were within the normal range. The anti-gastric parietal cell antibody test was also negative (Table 1 ). Computed tomography did not identify any cause for gastrointestinal bleeding. The patient underwent a capsule endoscopy, which did not reveal any abnormalities in the small intestine, although a large amount of black residue was observed in the stomach. EGD revealed multiple polyps and mild oozing was observed from the polyps and gastric mucosa . We tried to perform endoscopic hemostasis by using hemostatic forceps in the soft coagulation mode, but the observed hemorrhaging increased. Because the gastric mucosa was vulnerable to further hemorrhage and the oozing still persisted, we resected three bleeding polyps. The patient progressed satisfactorily and he was temporarily discharged. Two weeks after discharge, the patient was rehospitalized with tarry stools. EGD revealed coagula in the stomach, and multiple polyps in the corpus were observed to be hemorrhagic . Endoscopic mucosal resection and polypectomy were performed at 21 sites. Furthermore, we stopped the administration of a PPI. Microscopically, characteristics of 20 polyps in the body of the stomach were almost same. Significant cystic dilatation of glands was observed. Polyps showed characteristics of hyperplasia of the foveolar epithelium, extended fundic glands and edema of the stroma, suggesting lesions that differed from typical FGPs . Together with hyperplasia of the foveolar epithelium and extended mucous glands, the proliferation of parietal and chief cells were also observed, but not parietal cell protrusion or inflammatory cell infiltration. Apoptotic bodies were detected in the boundary region between fundic and neck mucous glands . Crypt epithelial cells (MUC5AC) were mainly observed superficially and cervical mucous cells (MUC6) were observed beneath the crypt epithelium. Fundic glands were positive for H+/K+-ATPase, and showed a proliferation of predominantly parietal cells. Only a very slight accumulation of chromogranin A staining was found, and enterochromaffin-like (ECL) cells were not detected. Few positive cells were found by Ki67 staining and polyps lacked dysplasia . Aquaporin-4 (AQP4) and KCNQ1-positive parietal cells and dilated mucous glands were found from the basal side to the apical side of the mucosa. The extension of the distribution of AQP4 and KCNQ1-positive cells toward the apical side of the fundic glands was observed . Overexpression of gastrin receptors was not detected. A reddish polyp in the antrum showed infiltration of inflammatory cells and it was similarpathologically to inflammatory polyp. Gastrin overexpression was not observed in the antral polyp immunohistochemically . Fig. 1 Esophagogastroduodenoscopic findings. a Multiple white edematous polyps ( arrows ) were observed in the corpus and antrum, which were considered to be fundic gland polyps (FGPs) as determined endoscopically before admission. b Esophagogastroduodenoscopy (EGD) revealed mild oozing from polyps of the gastric corpus ( arrows ) after admission. c EGD findings at the time of readmission: a reddish, hemorrhagic polyp was observed in the antrum, together with coagula. d Coagula were observed in the stomach, and multiple polyps in the corpus were hemorrhagic Table 1 Laboratory data on admission Hematology WBC 6300 /μL RBC 413 × 10 4 /μL Hb 7.6 g/dL Ht 27.3 % Plt 25.6 × 10 4 /μL Coagulation PT 76.0 % APTT 37.6 s Blood chemistry TP 7.2 g/dL Alb 4.6 g/dL T-Bil 0.35 g/dL AST 14 IU/L ALT 13 IU/L ALP 174 IU/L LDH 179 IU/L γ-GTP 31 IU/L ChE 324 U/L BUN 10 mg/dL Cre 0.81 mg/dL Na 143 mmol/L K 3.8 mmol/L Cl 105 mmol/L Glu 103 mg/dL Fe 19 μg/dL TIBC 551 μg/dL Ferritin 4 ng/dL HbA1c 5.6 % Gastrin 89 pg/mL Tumor marker CEA 2.0 ng/mL CA19-9 11 U/mL Serological test CRP 0.0 mg/dL HBsAg (–) U/mL HCVAb (–) Hp-IgG <3 U/mL Anti-parietal cell antibody (–) Hp antigen in stool (–) Fig. 2 1 Pathological findings of resected polyps that looked like fundic gland polyp (hematoxylin & eosin [HE] staining, loupe image). Polyps showed characteristics of hyperplasia of the foveolar epithelium, extended fundic glands and edema of the stroma. 2 Characteristics of pathological findings of resected polyps that looked like fundic gland polyp (hematoxylin & eosin [HE] staining). Mixed with hyperplasia of foveolar epithelium and extended mucous glands ( A ), the proliferation of parietal and chief cells was also observed ( B ), but not parietal cell protrusion or inflammatory cell infiltration ( C ). Apoptotic bodies were detected in the boundary region between fundic and neck mucous glands ( D ). 3 Immunohistochemistry for MUC5AC, MUC6, H + /K + -ATPase, pepsinogen I, Ki-67. Crypt epithelial cells (MUC5AC) were mainly observed superficially, and cervical mucous cells (MUC6) were observed beneath the crypt epithelium. Fundic glands were positive for H+/K+-ATPase, and showed proliferation of predominantly parietal cells. Few Ki67 positive cells were found. 4 Immunohistochemistry for aquaporin-4, KCNQ1, gastrin and gastrin receptor. Aquaporin-4 (AQP4) and KCNQ1-positive parietal cells and dilated mucous glands were found from the basal side to the apical side of the mucosa. The extension of the distribution of AQP4 and KCNQ1-positive cells toward the apical side of the fundic glands were observed. Overexpression of gastrin receptors was not detected. Gastrin overexpression was not observed in the antral polyp	4.125	0.967773	sec[1]/p[1]	en	0.999996	28660530	https://doi.org/10.1007/s12328-017-0756-x	[ "cells", "polyps", "glands", "fundic", "parietal", "mucous", "gastrin", "polyp", "cell", "epithelium" ]	[ { "code": "MF9Y", "title": "Other specified clinical findings on examination of urine, without diagnosis" }, { "code": "5C56.20", "title": "Mucolipidosis" }, { "code": "3A51.1", "title": "Sickle cell disease without crisis" }, { "code": "9A96.3", "title": "Primary anterior uveitis" }, { "code": "3A61.Z", "title": "Acquired pure red cell aplasia, unspecified" }, { "code": "CA0J.Y", "title": "Other specified nasal polyp" }, { "code": "DB71.Z", "title": "Anal polyp, unspecified" }, { "code": "DA09.0", "title": "Pulpitis" }, { "code": "DB71.1", "title": "Lymphoid polyp" }, { "code": "GA16.Y", "title": "Other specified acquired abnormalities of uterus, except cervix" } ]	=== ICD-11 CODES FOUND === [MF9Y] Other specified clinical findings on examination of urine, without diagnosis Also known as: Other specified clinical findings on examination of urine, without diagnosis \| Methaemoglobinuria \| Other and unspecified abnormal findings in urine \| Calciuria \| Cells and casts in urine [5C56.20] Mucolipidosis Also known as: Mucolipidosis \| Mucolipidosis type 3 \| Pseudo-Hurler polydystrophy \| Pseudo-Hurler disease \| Mucolipidosis type 2 Excludes: Sialidosis (mucolipidosis type 1) [3A51.1] Sickle cell disease without crisis Definition: A disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may present with fatigue, shortness of breath, dizziness, headaches, pallor of skin or mucous membranes, and jaundice. This disorder is confirmed by identification of HbS mutation by genetic testing. Also known as: Sickle cell disease without crisis \| Hb-SS disease without crisis \| HbSS without crisis \| Sickle-cell anaemia without crisis \| SCD - [sickle cell disease] [9A96.3] Primary anterior uveitis Definition: This refers to primary inflammation of the uvea. The uvea consists of the middle, pigmented, vascular structures of the eye and includes the iris, ciliary body, and choroid. Also known as: Primary anterior uveitis \| anterior chamber cell [3A61.Z] Acquired pure red cell aplasia, unspecified Also known as: Acquired pure red cell aplasia, unspecified \| Acquired pure red cell aplasia \| acquired red cell aplasia \| red cell aplasia NOS \| pure red cell aplastic anaemia [CA0J.Y] Other specified nasal polyp Also known as: Other specified nasal polyp \| Polyp of nasal cavity \| Polyp of the nasopharynx \| nasopharyngeal polyp \| Polyp of adenoid tissue [DB71.Z] Anal polyp, unspecified Also known as: Anal polyp, unspecified \| Anal polyp [DA09.0] Pulpitis Definition: Inflammation of pulpal tissue resulting from irritating factors of diverse nature such as bacterial, hyperaemic, chemical or thermal that act directly or indirectly on the dental pulp. Also known as: Pulpitis \| Pulpitis NOS \| Suppurative pulpitis \| Acute pulpitis \| Chronic pulpitis Includes: Suppurative pulpitis [DB71.1] Lymphoid polyp Definition: Lymphoid polyp is a benign, focal or diffuse small polypoid lesion composed of well-differentiated lymphoid tissue. Also known as: Lymphoid polyp [GA16.Y] Other specified acquired abnormalities of uterus, except cervix Also known as: Other specified acquired abnormalities of uterus, except cervix \| Polyp of corpus uteri \| intrauterine polyp \| polyp of body of uterus \| polyp of uterus === GRAPH WALKS === --- Walk 1 --- [MF9Y] Other specified clinical findings on examination of urine, without diagnosis --PARENT--> [?] Clinical findings on examination of urine, without diagnosis --EXCLUDES--> [?] Inborn errors of amino acid or other organic acid metabolism --- Walk 2 --- [MF9Y] Other specified clinical findings on examination of urine, without diagnosis --PARENT--> [?] Clinical findings on examination of urine, without diagnosis --EXCLUDES--> [?] Inborn errors of amino acid or other organic acid metabolism --- Walk 3 --- [5C56.20] Mucolipidosis --RELATED_TO--> [?] Wolman disease Def: Wolman disease represents the most severe manifestation of lysosomal acid lipase deficiency and can present in the fetus (hepatomegaly, ascites, calcified adrenal glands), or more typically in the fir... --PARENT--> [?] Lysosomal acid lipase deficiency Def: Lysosomal Acid Lipase (LAL) Deficiency happens when the body does not produce enough active lysosomal acid lipase (LAL or LIPA) enzyme. This enzyme plays an important role in breaking down fatty mater... --- Walk 4 --- [5C56.20] Mucolipidosis --EXCLUDES--> [?] Sialidosis --CHILD--> [?] Sialidosis type 1 Def: Type 1 sialidosis, also called normomorphic or 'cherry-red-spot, myoclonus' syndrome is a form of sialidosis, a lysosomal storage disease belonging to the group of oligosaccharidoses or glycoproteinos... --- Walk 5 --- [3A51.1] Sickle cell disease without crisis Def: A disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may pr... --PARENT--> [3A51] Sickle cell disorders or other haemoglobinopathies Def: Any disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may ... --CHILD--> [3A51.1] Sickle cell disease without crisis Def: A disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may pr... --- Walk 6 --- [3A51.1] Sickle cell disease without crisis Def: A disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may pr... --PARENT--> [3A51] Sickle cell disorders or other haemoglobinopathies Def: Any disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may ... --CHILD--> [3A51.0] Sickle cell trait Def: A disease caused by genetic inheritance of one abnormal allele of the haemoglobin gene. This disease does not display the severe symptoms of sickle cell disease that occurs in homozygous individuals. ...	[ "[MF9Y] Other specified clinical findings on examination of urine, without diagnosis\n --PARENT--> [?] Clinical findings on examination of urine, without diagnosis\n --EXCLUDES--> [?] Inborn errors of amino acid or other organic acid metabolism", "[MF9Y] Other specified clinical findings on examination of urine, without diagnosis\n --PARENT--> [?] Clinical findings on examination of urine, without diagnosis\n --EXCLUDES--> [?] Inborn errors of amino acid or other organic acid metabolism", "[5C56.20] Mucolipidosis\n --RELATED_TO--> [?] Wolman disease\n Def: Wolman disease represents the most severe manifestation of lysosomal acid lipase deficiency and can present in the fetus (hepatomegaly, ascites, calcified adrenal glands), or more typically in the fir...\n --PARENT--> [?] Lysosomal acid lipase deficiency\n Def: Lysosomal Acid Lipase (LAL) Deficiency happens when the body does not produce enough active lysosomal acid lipase (LAL or LIPA) enzyme. This enzyme plays an important role in breaking down fatty mater...", "[5C56.20] Mucolipidosis\n --EXCLUDES--> [?] Sialidosis\n --CHILD--> [?] Sialidosis type 1\n Def: Type 1 sialidosis, also called normomorphic or 'cherry-red-spot, myoclonus' syndrome is a form of sialidosis, a lysosomal storage disease belonging to the group of oligosaccharidoses or glycoproteinos...", "[3A51.1] Sickle cell disease without crisis\n Def: A disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may pr...\n --PARENT--> [3A51] Sickle cell disorders or other haemoglobinopathies\n Def: Any disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may ...\n --CHILD--> [3A51.1] Sickle cell disease without crisis\n Def: A disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may pr...", "[3A51.1] Sickle cell disease without crisis\n Def: A disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may pr...\n --PARENT--> [3A51] Sickle cell disorders or other haemoglobinopathies\n Def: Any disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may ...\n --CHILD--> [3A51.0] Sickle cell trait\n Def: A disease caused by genetic inheritance of one abnormal allele of the haemoglobin gene. This disease does not display the severe symptoms of sickle cell disease that occurs in homozygous individuals. ..." ]	MF9Y	Other specified clinical findings on examination of urine, without diagnosis	[ { "from_icd11": "3A51.1", "icd10_code": "D571", "icd10_title": "Sickle-cell disease without crisis" }, { "from_icd11": "3A61.Z", "icd10_code": "D609", "icd10_title": "Acquired pure red cell aplasia, unspecified" }, { "from_icd11": "3A61.Z", "icd10_code": "D608", "icd10_title": "Other acquired pure red cell aplasias" }, { "from_icd11": "3A61.Z", "icd10_code": "D60", "icd10_title": "Acquired pure red cell aplasia [erythroblastopenia]" }, { "from_icd11": "DB71.Z", "icd10_code": "K620", "icd10_title": "Anal polyp" }, { "from_icd11": "DA09.0", "icd10_code": "K0402", "icd10_title": "Irreversible pulpitis" }, { "from_icd11": "DA09.0", "icd10_code": "K0401", "icd10_title": "Reversible pulpitis" }, { "from_icd11": "DA09.0", "icd10_code": "K040", "icd10_title": "Pulpitis" } ]	D571	Sickle-cell disease without crisis
An 81-year-old woman was transferred to our hospital by ambulance with massive hemoptysis. She reported a gradually increasing frequency of blood sputum for one month prior to this episode. The patient had a history of aortic valve replacement combined with graft replacement of the ascending aorta and aortic arch and graft replacement of the thoraco-abdominal aorta for dissecting aortic aneurysms 5 and 4 years prior, respectively. She also underwent thoracic endovascular aortic repair (TEVAR) for impending rupture of a descending aortic aneurysm 2 years prior. The patient was taking aspirin (100 mg/day). On admission, she was conscious and alert and her vital signs were stable. Her blood oxygen saturation level was 93% on room air. A complete blood count analysis revealed a hemoglobin level of 10.0 g/dL, white blood cell count of 8,500/µL, and platelet count of 144,000/µL. Serum biochemical tests showed no obvious abnormalities except for a slightly elevated C-reactive protein level (0.8 mg/dL). No abnormalities were detected on a clotting test. Computed tomography (CT) showed a large atelectasis of the left lower lung lobe adjacent to the descending aorta treated with TEVAR. Unenhanced CT showed a heterogeneous high attenuation area within the atelectasis suggestive of a concomitant hematoma. Contrast-enhanced CT revealed a pseudoaneurysm, and abnormal proliferative vessels with continuity with hypertrophied left pulmonary ligament artery (PLA) were detected within the atelectasis . Furthermore, the PLA continued to the right subscapular artery via a complex collateral pathway including the right intercostal artery (ICA) . Since disruption of the pseudoaneurysm and abnormal vessels of the left PLA were considered the cause of massive hemoptysis, we decided to perform transcatheter arterial embolization (TAE) with the patient’s consent. First, the right femoral artery was punctured and an aortography was performed; however, it did not show any type 1, 2, 3, or 4 endoleaks. Next, an access was obtained via the right brachial artery using a 4-Fr sheath. Diagnostic digital subtraction angiography (DSA) of the right subclavian and subscapular arteries revealed a hypertrophied left PLA adjacent to the stent graft of the descending aorta that originated from the right subscapular artery via a complex collateral blood pathway including the right ICA and thoracodorsal artery . We subsequently attempted to select the left PLA with the triple coaxial (triaxial) system, which consists of a 155-cm-long 1.9-Fr microcatheter (Carnelian MARVEL, Tokai Medical Products, Kasugai, Japan), a 110-cm-long 2.7-Fr high-flow microcatheter (Sniper 2 High-Flow; Terumo, Tokyo, Japan), and a 65-cm-long 4-Fr angiographic catheter (Impress; Merit Medical Japan, Tokyo, Japan) and finally reached the proximal side of the left PLA. During the procedure, we confirmed no involvement of the anterior spinal artery by DSA of the intercostal artery. Selective DSA of the left PLA showed proliferation of small tortuous vessels and a pseudoaneurysm at the peripheral side . In addition, small systemic-pulmonary artery shunts were observed at the peripheral side of this lesion . Based on the angiographic findings, we decided to use an N -butyl cyanoacrylate (NBCA) (B. Braun, Melsungen, Germany)-Lipiodol (Lipiodol Ultra-Fluid; Guerbet, Roissy, France) mixture for embolization, which should reach the embolic material on the peripheral side of the PLA while avoiding large amounts of embolic material migration to the pulmonary artery branches via the systemic-pulmonary artery shunt. We carefully injected 0.8 mL of an NBCA:Lipiodol (1:6) mixture in a stepwise fashion under fluoroscopic guidance until it reached the pseudoaneurysm and abnormal proliferative vessels . DSA of the right subscapular artery after TAE showed disappearance of the pseudoaneurysm and abnormal vessels of the left PLA . We subsequently performed DSA of the bilateral subclavian arteries and celiac artery and confirmed that none supplied the target lesions. Pulmonary angiography of the left pulmonary trunk performed via the right femoral vein showed no pseudoaneurysm or contrast filling defect due to the systemic-pulmonary artery shunt. After TAE, the hemoptysis was controlled without complications, and the patient was discharged 5 days after TAE. Chest radiography performed 2 weeks after the TAE revealed improved consolidation in the left lower field. Six months after TAE, no recurrence of the hemoptysis was noted. Fig. 1 Computed tomography (CT) findings. A Contrast-enhanced CT showing the large atelectasis (asterisk) adjacent to a thoracic aortic aneurysm treated with a stent graft. The arrows and arrowheads indicate the hypertrophied left pulmonary ligament artery (PLA) and abnormal proliferative vessels in the atelectasis, respectively. B Slab-maximum intensity projection image in the oblique coronal view. Abnormal proliferation of small vessels is observed at the peripheral side of the left PLA. The dashed arrow indicates a pseudoaneurysm. C Volume-rendering image. The continuity from the right subscapular artery to the left PLA via the tortuous complex collateral pathway is clearly visible (arrowheads) Fig. 2 Angiographic findings. A Selective digital subtraction angiography (DSA) of the right subclavian artery. Consistent with contrast-enhanced computed tomography image, the continuity from the right subscapular artery to the left pulmonary ligament artery (PLA) (arrow) is shown. B and C Selective DSA of the left PLA. Note that the abnormal proliferation of small vessels is visible at the peripheral side of the left PLA. The arrowheads in b indicate the reversal blood flow of pulmonary artery, suggesting the presence of a systemic-pulmonary artery shunt. The dashed arrow in c indicates the pseudoaneurysm Fig. 3 Transcatheter arterial embolization (TAE). A Pre-TAE image. The arrowhead indicates the tip of the microcatheter placed at the proximal side of the left pulmonary ligament artery (PLA). B Angiographic image acquired during TAE. The depositions of the Lipiodol at the sites corresponding to the pseudoaneurysm (dashed arrow) and abnormal vessels (arrows) are visible. C Digital subtraction angiography of the right subscapular artery after TAE. The pseudoaneurysm and abnormal vessels of the left PLA disappeared	4.066406	0.969727	sec[1]/p[0]	en	0.999995	35290529	https://doi.org/10.1186/s42155-022-00293-3	[ "artery", "pulmonary", "pseudoaneurysm", "vessels", "subscapular", "side", "blood", "aortic", "atelectasis", "hemoptysis" ]	[ { "code": "BD5Z", "title": "Diseases of arteries or arterioles, unspecified" }, { "code": "BD52", "title": "Certain specified disorders of arteries or arterioles" }, { "code": "BD52.3", "title": "Rupture of artery" }, { "code": "BD52.2", "title": "Stricture of artery" }, { "code": "BD40.Z", "title": "Atherosclerotic chronic arterial occlusive disease, unspecified" }, { "code": "CB40.Y", "title": "Other specified diseases of the respiratory system" }, { "code": "LA75.1", "title": "Agenesis of lung" }, { "code": "CA40.Z", "title": "Pneumonia, organism unspecified" }, { "code": "CB41", "title": "Respiratory failure" }, { "code": "NB32.3Y", "title": "Other injury of lung" } ]	=== ICD-11 CODES FOUND === [BD5Z] Diseases of arteries or arterioles, unspecified Also known as: Diseases of arteries or arterioles, unspecified \| artery disease NOS \| arterial disease NOS \| arteriolar disease NOS \| disorder of artery NOS [BD52] Certain specified disorders of arteries or arterioles Also known as: Certain specified disorders of arteries or arterioles \| Aortic dilatation - joint hypermobility - arterial tortuosity \| Generalised arterial calcification of infancy \| Median arcuate ligament syndrome \| Aortic root abscess Excludes: collagen (vascular) diseases \| Hypersensitivity angiitis \| Acute arterial occlusion [BD52.3] Rupture of artery Also known as: Rupture of artery \| ruptured artery \| artery fistula \| Aortic duodenal fistula \| Aortic colon fistula Excludes: traumatic rupture of artery - see injury of blood vessel by body region [BD52.2] Stricture of artery Also known as: Stricture of artery \| arterial stenosis \| arterial stricture \| artery stricture \| stenosis of artery [BD40.Z] Atherosclerotic chronic arterial occlusive disease, unspecified Also known as: Atherosclerotic chronic arterial occlusive disease, unspecified \| Atherosclerotic chronic arterial occlusive disease \| arteriosclerosis, NOS \| generalised atherosclerosis \| atherosclerosis NOS [CB40.Y] Other specified diseases of the respiratory system Also known as: Other specified diseases of the respiratory system \| Secondary respiratory disorders \| Respiratory disorders in diseases classified elsewhere \| Diseases of bronchus, not elsewhere classified \| Acquired bronchial diverticulum [LA75.1] Agenesis of lung Definition: This refers to the absence or rudimentary residua of an undeveloped lung. Also known as: Agenesis of lung \| Pulmonary agenesis \| absence of lung \| aplasia of lung \| apulmonism [CA40.Z] Pneumonia, organism unspecified Also known as: Pneumonia, organism unspecified \| Pneumonia \| infectious pneumonia \| PN - [pneumonia] \| lobar pneumonia NOS [CB41] Respiratory failure Definition: Respiratory failure is a life-threatening impairment of oxygenation or carbon dioxide (CO2) elimination. Respiratory failure may occur because of impaired gas exchange, decreased ventilation, or both. The level of oxygen in the blood becomes dangerously low or the level of carbon dioxide becomes dangerously high. Also known as: Respiratory failure \| lung failure NOS \| pulmonary failure Excludes: Acute respiratory distress syndrome \| Respiratory arrest \| Respiratory distress of newborn [NB32.3Y] Other injury of lung Also known as: Other injury of lung \| Haematoma of lung \| Traumatic hydropneumothorax \| Acute traumatic lung congestion \| Rupture of lung === GRAPH WALKS === --- Walk 1 --- [BD5Z] Diseases of arteries or arterioles, unspecified --PARENT--> [?] Diseases of arteries or arterioles --CHILD--> [?] Chronic arterial occlusive disease --- Walk 2 --- [BD5Z] Diseases of arteries or arterioles, unspecified --PARENT--> [?] Diseases of arteries or arterioles --CHILD--> [BD30] Acute arterial occlusion --- Walk 3 --- [BD52] Certain specified disorders of arteries or arterioles --CHILD--> [BD52.0] Segmental arterial mediolysis Def: Segmental arterial mediolysis is a rare noninflammatory vascular disease of the abdominal splanchnic arteries, characterised by disruption of the arterial medial layer. It will induce multiple aneurys... --PARENT--> [BD52] Certain specified disorders of arteries or arterioles --- Walk 4 --- [BD52] Certain specified disorders of arteries or arterioles --CHILD--> [BD52.0] Segmental arterial mediolysis Def: Segmental arterial mediolysis is a rare noninflammatory vascular disease of the abdominal splanchnic arteries, characterised by disruption of the arterial medial layer. It will induce multiple aneurys... --PARENT--> [BD52] Certain specified disorders of arteries or arterioles --- Walk 5 --- [BD52.3] Rupture of artery --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes Def: !markdown In the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre... --PARENT--> [?] ICD Category --- Walk 6 --- [BD52.3] Rupture of artery --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes Def: !markdown In the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre... --CHILD--> [?] Injuries to the neck	[ "[BD5Z] Diseases of arteries or arterioles, unspecified\n --PARENT--> [?] Diseases of arteries or arterioles\n --CHILD--> [?] Chronic arterial occlusive disease", "[BD5Z] Diseases of arteries or arterioles, unspecified\n --PARENT--> [?] Diseases of arteries or arterioles\n --CHILD--> [BD30] Acute arterial occlusion", "[BD52] Certain specified disorders of arteries or arterioles\n --CHILD--> [BD52.0] Segmental arterial mediolysis\n Def: Segmental arterial mediolysis is a rare noninflammatory vascular disease of the abdominal splanchnic arteries, characterised by disruption of the arterial medial layer. It will induce multiple aneurys...\n --PARENT--> [BD52] Certain specified disorders of arteries or arterioles", "[BD52] Certain specified disorders of arteries or arterioles\n --CHILD--> [BD52.0] Segmental arterial mediolysis\n Def: Segmental arterial mediolysis is a rare noninflammatory vascular disease of the abdominal splanchnic arteries, characterised by disruption of the arterial medial layer. It will induce multiple aneurys...\n --PARENT--> [BD52] Certain specified disorders of arteries or arterioles", "[BD52.3] Rupture of artery\n --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes\n Def: !markdown\nIn the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre...\n --PARENT--> [?] ICD Category", "[BD52.3] Rupture of artery\n --EXCLUDES--> [?] Injury, poisoning or certain other consequences of external causes\n Def: !markdown\nIn the ICD, injury means physical or physiological bodily harm resulting from interaction of the body with energy (mechanical, thermal, electrical, chemical or radiant, or due to extreme pre...\n --CHILD--> [?] Injuries to the neck" ]	BD5Z	Diseases of arteries or arterioles, unspecified	[ { "from_icd11": "BD5Z", "icd10_code": "I7389", "icd10_title": "Other specified peripheral vascular diseases" }, { "from_icd11": "BD5Z", "icd10_code": "I7419", "icd10_title": "Embolism and thrombosis of other parts of aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7411", "icd10_title": "Embolism and thrombosis of thoracic aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7410", "icd10_title": "Embolism and thrombosis of unspecified parts of aorta" }, { "from_icd11": "BD5Z", "icd10_code": "I7381", "icd10_title": "Erythromelalgia" }, { "from_icd11": "BD5Z", "icd10_code": "I745", "icd10_title": "Embolism and thrombosis of iliac artery" }, { "from_icd11": "BD5Z", "icd10_code": "I789", "icd10_title": "Disease of capillaries, unspecified" }, { "from_icd11": "BD5Z", "icd10_code": "I748", "icd10_title": "Embolism and thrombosis of other arteries" }, { "from_icd11": "BD5Z", "icd10_code": "I749", "icd10_title": "Embolism and thrombosis of unspecified artery" }, { "from_icd11": "BD5Z", "icd10_code": "I781", "icd10_title": "Nevus, non-neoplastic" }, { "from_icd11": "BD5Z", "icd10_code": "I788", "icd10_title": "Other diseases of capillaries" }, { "from_icd11": "BD5Z", "icd10_code": "I744", "icd10_title": "Embolism and thrombosis of arteries of extremities, unspecified" }, { "from_icd11": "BD5Z", "icd10_code": "I70-I79", "icd10_title": "" }, { "from_icd11": "BD5Z", "icd10_code": "I74", "icd10_title": "Arterial embolism and thrombosis" }, { "from_icd11": "BD5Z", "icd10_code": "I73", "icd10_title": "Other peripheral vascular diseases" } ]	I7389	Other specified peripheral vascular diseases
A 71-year-old woman consulted her physician complaining of hoarseness. She was referred to our hospital with suspected thyroid cancer with recurrent laryngeal nerve invasion and cervical lymph node metastasis based on the results of ultrasonography. A diffuse goiter and cervical lymphadenopathy were noted on examination. Blood testing demonstrated a white blood cell count level of 5440 /μL, thyroid stimulating hormone level of 0.44 µIU/ml, free triiodothyronine level of 3.01 pg/ml, free thyroxine level of 1.05 ng/ml, thyroglobulin level of 285 ng/ml, and anti-thyroglobulin antibody level of less than 10 IU/ml. Ultrasonography revealed a hypoechoic mass in the left lobe of the thyroid and lymphadenopathy suspicious for metastasis in the right paratracheal and left cervical regions . Computed tomography (CT) demonstrated the presence of a thyroid tumor invading the trachea and esophagus with no evidence of distant metastasis . Fluorodeoxyglucose positron emission tomography revealed significant uptake by the thyroid tumor and a left cervical lymph node without uptake in distant organs. Fine needle aspiration of the left cervical lymph node indicated the lymph node metastasis of ATC. Biopsy of the left cervical lymph node was performed to screen for RET mutations using the Oncomine Dx Target Test (Thermo-Fisher, USA). The sampled lymph node tissue was negative for RET mutations; however, a BRAF mutation was identified. As the tumor had widely invaded the trachea and esophagus, unresectable ATC was diagnosed and treatment with lenvatinib was initiated at a dose of 24 mg/day. Since there was the fatal risk of fistula formation, we had fully informed the patient about serious adverse events before starting lenvatinib. Furthermore, we decided to perform CT earlier to investigate whether fistula formation occurred. On day 2 of lenvatinib administration, the patient developed grade 3 hypertension and treatment with a calcium channel blocker and an angiotensin receptor blocker was initiated accordingly. On day 7 of lenvatinib treatment, the patient developed grade 2 palmar–plantar erythrodysesthesia that was managed with managed conservatively. On day 13 of lenvatinib treatment, the primary tumor and lymph node metastases demonstrated a partial response to therapy . As the tumor was now considered resectable, the decision was made to perform conversion surgery. On day 14 of lenvatinib administration, the patient was discharged and the lenvatinib dose was reduced to 14 mg/day to reduce the risk of further adverse events (AEs) such as fistula formation. Total thyroidectomy and left lateral neck node dissection were performed 7 days after the withdrawal of lenvatinib. The operative duration was 163 min with minimal blood loss. As the tumor encased the left recurrent laryngeal nerve, we performed combined resection of the tumor and left recurrent laryngeal nerve. As the tumor was found to be invading the first tracheal cartilage, tracheal shaving was performed to preserve the trachea. On the other hand, there was no obvious invasion into the esophagus and dissection was easy. The patient was discharged on postoperative day 5 with no complications. Histopathological examination demonstrated that the tumor contained the component of papillary thyroid carcinoma (PTC), squamoid ATC cells, and granulation tissue . Those squamoid ATC cells with prominent nuclear atypia were weakly positive for cytokeratin AE1/AE3, thyroid transcription factor 1 (TTF-1), and thyroglobulin. Furthermore, PAX8 staining was negative and p53 staining was positive. These results were considered representative of ATC. In areas of granulation tissue, atypical cells with spindle-shaped or polygonal morphology, pyknotic nuclei, and scant cytoplasm were observed . Immunohistochemically, these cells were positive for cytokeratin AE1/AE3 , TTF-1, and p53 and negative for thyroglobulin and PAX8. Given the above findings, the areas of granulation tissue observed within tumor samples were also considered ATC that were affected by lenvatinib treatment. The tracheal invasion area also had similar findings that contained squamoid ATC cells and granulation tissues. In total, the proportion of ATC component was approximately 50% among the whole tumor. Additionally, we diagnosed that 70% of ATC component had been changed to granulation tissue . Postoperative radiotherapy was planned; however, lenvatinib was restarted at 30 days postoperatively due to tumor recurrence in the subcutaneous tissues and lymph nodes of the right neck. The summary of treatment and clinical course is shown in Fig. 5 . Fig. 1 Ultrasonography before and after lenvatinib treatment. a – c Ultrasonography revealed a hypoechoic mass in the left lobe of the thyroid and lymph node metastases in the right paratracheal and left cervical regions. d – f A hypoechoic mass in the left lobe of the thyroid and lymph node metastases in the right paratracheal and left cervical regions shrank after lenvatinib treatment Fig. 2 Computed tomography before and after lenvatinib treatment. a – c Computed tomography demonstrated the presence of a thyroid tumor measuring 4.3 × 3.3 cm and left cervical lymph node metastasis measuring 1.9 × 1.6 cm. The thyroid tumor widely invaded the trachea and esophagus. There was no evidence of distant metastasis. d – f . The thyroid tumor and left cervical lymph node metastasis shrank to 3.3 × 2.1 cm and 0.8 × 0.8 cm, respectively, after lenvatinib treatment Fig. 3 Histopathological examination of surgical specimens. Low-power view of the anaplastic thyroid carcinoma (ATC) component ( a ). Inflammatory granulation tissue is observed on the left side, whereas highly atypical squamoid ATC cells are still remaining on the right. The granulation tissue contains many small spindle cells with degenerative pyknotic nuclei ( b ) which are distinctly positive for cytokeratin AE1/AE3 ( c ). High-power view of the viable ATC cells ( d ). Papillary thyroid carcinoma is observed on the periphery of the ATC ( e ). Fig. 4 Distribution of tumor. In total, approximately 50% of resected tumor comprised anaplastic thyroid carcinoma (ATC), and 70% of them had been changed to granulation tissue. ★: ATC, *: granulation with degenerative ATC, ▲: papillary thyroid carcinoma, ■: parathyroid gland Fig. 5 Treatment and clinical course	3.962891	0.976074	sec[1]/p[0]	en	0.999997	36920674	https://doi.org/10.1186/s40792-023-01619-6	[ "tumor", "thyroid", "lenvatinib", "lymph", "node", "cervical", "granulation", "tissue", "cells", "metastasis" ]	[ { "code": "2F9Z", "title": "Neoplasms of unknown behaviour of unspecified site" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "2E6Z", "title": "Carcinoma in situ of unspecified site" }, { "code": "2F91.1", "title": "Neoplasms of unknown behaviour of trachea, bronchus or lung" }, { "code": "2F92", "title": "Neoplasms of unknown behaviour of skin" }, { "code": "5A03.Z", "title": "Thyroiditis, unspecified" }, { "code": "5A0Z", "title": "Disorders of the thyroid gland or thyroid hormones system, unspecified" }, { "code": "5A03.Y", "title": "Other specified thyroiditis" }, { "code": "5A00.2Z", "title": "Acquired hypothyroidism, unspecified" }, { "code": "5A03.0", "title": "Acute thyroiditis" } ]	=== ICD-11 CODES FOUND === [2F9Z] Neoplasms of unknown behaviour of unspecified site Also known as: Neoplasms of unknown behaviour of unspecified site \| neoplasia \| neoplasm growth NOS \| tumour of unspecified site \| tumourlet of unspecified site [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [2E6Z] Carcinoma in situ of unspecified site Also known as: Carcinoma in situ of unspecified site \| carcinoma in situ of unspecified site \| carcinoma in situ NOS \| carcinoma in situ \| in situ neoplasm [2F91.1] Neoplasms of unknown behaviour of trachea, bronchus or lung Also known as: Neoplasms of unknown behaviour of trachea, bronchus or lung \| trachea, bronchus or lung tumour NOS \| Intravascular bronchial alveolar tumour of unspecified site \| Bronchial adenoma of unknown behaviour of unspecified site \| Intravascular bronchial alveolar tumour unknown behaviour of unspecified site [2F92] Neoplasms of unknown behaviour of skin Also known as: Neoplasms of unknown behaviour of skin \| skin tumour NOS [5A03.Z] Thyroiditis, unspecified Also known as: Thyroiditis, unspecified \| Thyroiditis \| inflammation of thyroid \| thyroiditis NOS [5A0Z] Disorders of the thyroid gland or thyroid hormones system, unspecified Also known as: Disorders of the thyroid gland or thyroid hormones system, unspecified [5A03.Y] Other specified thyroiditis Also known as: Other specified thyroiditis \| Riedel thyroiditis \| Chronic invasive fibrous thyroiditis \| Ligneous thyroiditis \| Riedel struma [5A00.2Z] Acquired hypothyroidism, unspecified Also known as: Acquired hypothyroidism, unspecified \| Acquired hypothyroidism \| hypothyrea \| thyroid insufficiency \| hypothyroidea [5A03.0] Acute thyroiditis Definition: Acute thyroiditis is a rare form of thyroiditis directly caused by an infection, frequently bacterial. Also known as: Acute thyroiditis \| infectious thyroiditis \| Acute thyroiditis due to bacterial infection \| Acute thyroiditis due to fungal infection \| Abscess of thyroid === GRAPH WALKS === --- Walk 1 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour --- Walk 2 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2F91] Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs --- Walk 3 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Breast lump or mass female --PARENT--> [?] Symptoms, signs or clinical findings involving the female genital system --- Walk 4 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Enlarged lymph nodes Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes.... --CHILD--> [?] Localised lymph node enlargement --- Walk 5 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E62] Carcinoma in situ of middle ear or respiratory system --- Walk 6 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E62] Carcinoma in situ of middle ear or respiratory system	[ "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour", "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2F91] Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Breast lump or mass female\n --PARENT--> [?] Symptoms, signs or clinical findings involving the female genital system", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Enlarged lymph nodes\n Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes....\n --CHILD--> [?] Localised lymph node enlargement", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E62] Carcinoma in situ of middle ear or respiratory system", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E62] Carcinoma in situ of middle ear or respiratory system" ]	2F9Z	Neoplasms of unknown behaviour of unspecified site	[ { "from_icd11": "2F9Z", "icd10_code": "D487", "icd10_title": "Neoplasm of uncertain behavior of other specified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D482", "icd10_title": "Neoplasm of uncertain behavior of peripheral nerves and autonomic nervous system" }, { "from_icd11": "2F9Z", "icd10_code": "D37-D48", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D377", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D48", "icd10_title": "Neoplasm of uncertain behavior of other and unspecified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D489", "icd10_title": "Neoplasm of uncertain behavior, unspecified" }, { "from_icd11": "ME61", "icd10_code": "R2240", "icd10_title": "Localized swelling, mass and lump, unspecified lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2232", "icd10_title": "Localized swelling, mass and lump, left upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2242", "icd10_title": "Localized swelling, mass and lump, left lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2231", "icd10_title": "Localized swelling, mass and lump, right upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2241", "icd10_title": "Localized swelling, mass and lump, right lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2233", "icd10_title": "Localized swelling, mass and lump, upper limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2243", "icd10_title": "Localized swelling, mass and lump, lower limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2230", "icd10_title": "Localized swelling, mass and lump, unspecified upper limb" }, { "from_icd11": "ME61", "icd10_code": "R220", "icd10_title": "Localized swelling, mass and lump, head" } ]	D487	Neoplasm of uncertain behavior of other specified sites
We start with vignettes of two families affected by TB – one family where the health and social care systems are struggling, and another family where the health and social infrastructure are able to operate and respond more effectively (Table 2 ). The vignettes illustrate numerous distinctive features of paediatric TB, including the even more complex threat of multidrug-resistant (MDR)-TB. TB can occur almost anywhere - in both developed and less developed countries. The scenario of the first family exemplifies failures that ultimately deny children their rights to life and health: failures in screening contacts of TB patients; failures to provide preventive therapy; diagnostic delays; poor access to health; lack of appreciation that paediatric TB is not the same as adult TB; treatment failures; and systems failures. Although this particular scenario involves MDR-TB, most of the failures equally apply to children suffering from drug-susceptible TB. In contrast, the second family benefits from an integrated approach between health, education, and social care, highlighting the interdependence and indivisibility of children’s rights. Table 2 Illustrative case vignettes of families affected by TB, highlighting the inequality in existing health provision. Key issues raised by the vignettes are summarized on the right Tuberculosis – a family disease across the world Struggling health and social care system Effective health and social care system Key lessons illustrated Six-year-old Jamil lives with his mother and 2-year-old sister, Zahra, in a rural village in a low income country. Jamil has been coughing for weeks. His mother has taken him to the traditional healer, but he worsens. She takes him to the nearest clinic where the healthcare worker gives antibiotics with no improvement. Eventually he is referred to the nearest hospital where an x-ray is taken and he is diagnosed with pneumonia and further antibiotics are prescribed. Repeated sputum examinations show no signs of TB but given the lack of response he is started on the standard four-drug TB regimen. He continues to deteriorate and so the health care workers explore Jamil’s case further. They realize that Jamil’s father had similar symptoms following his release from prison many months ago and was eventually diagnosed with multidrug-resistant TB and is an inpatient at the national sanatorium. Jamil is therefore referred three hours away to the national children’s hospital, where he starts MDR-TB treatment with daily injections and tablets. His mother, already struggling with an absent father does not have the means to visit him. Jamil will remain an inpatient for 8 months, separated from his family. His schooling stops and he gradually loses his hearing due to the medication he is receiving. Shortly after Jamil is admitted to the national children’s hospital, his younger sister, Zahra, becomes lethargic and spikes fevers. One day, his mother is unable to wake her up and she is taken by cart to the nearest hospital. The doctors suspect TB meningitis and start her on treatment, but she dies two days later. Six-year-old Jamil lives with his mother and 2-year-old sister, Zahra, in social housing in a large city of a high income country. Public health officials visit the family as part of a contact tracing program because their father was recently diagnosed with multidrug-resistant TB following his release from prison several months ago. Jamil’s youngest sister Zahra is 2 years old. Although she appears healthy and her investigations are normal, given her young age and close contact with a known MDR-TB case, her team of doctors start her on medication to prevent her from developing TB disease. Jamil has no symptoms yet, but the public health team request a chest radiograph and other tests, the results of which suggest he has early TB disease. Given his father is known to have MDR-TB, he is admitted to the regional children’s hospital where he undergoes paediatric-specific investigations for TB and he is treated with injections and tablets. He receives education from the hospital teaching team whilst an inpatient. His case is discussed at the multidisciplinary meeting to help his mother access benefits enabling her to visit him in hospital. Once his treatment regime has been stabilized, an arrangement is made between the hospital and the school so that he returns to live at home and attends the hospital daily after school. Following questions raised by Jamil and his family, the public health authorities liaise with the school to reassure and educate that there is no risk of transmission to others as he is on treatment and not coughing. In view of the potential side effects of the drugs that Jamil is taking, his hearing is tested regularly. After 2 months of treatment, there are early signs that his hearing is affected so his medication regimen is changed before he experiences hearing loss that might compromise his ability to communicate. As his disease was identified early, he is treated for a shorter duration of therapy then is required for adults with extensive disease. • TB disproportionately affects marginalized populations across the world – e.g. those living in poverty, difficult access to healthcare, migrants and refugees. • Effective public health mechanisms and infection control measures are necessary to identify linked cases and prevent further transmission. • Contact tracing can lead to identification of contacts eligible for therapy to prevent TB disease developing. • Contact tracing can lead to early detection and treatment of paediatric TB cases. • Understanding the differences between adult and paediatric TB is key to diagnosis and treatment initiation. • Continued education is possible and requires coordination of health, education, and social sectors. • Even when separation of children and their families during treatment is necessary, the impact can be minimized and the duration limited to the absolute minimum. • Children can and should be involved in and understand their own care and be communicated with in an age-appropriate manner. • Education of communities and increased awareness around TB will reduce stigma, diagnostic delays and improve access and uptake of TB services. • Appropriate provision of care to children affected by TB can prevent disability and death.	4.046875	0.618652	sec[0]/sec[0]/p[0]	en	0.999997	27931215	https://doi.org/10.1186/s12914-016-0105-z	[ "jamil", "children", "family", "that", "social", "failures", "mother", "paediatric", "education", "vignettes" ]	[ { "code": "5B51&XS25", "title": "Marasmus in children" }, { "code": "5B7Z", "title": "Unspecified undernutrition" }, { "code": "5B51", "title": "Wasting in infants, children or adolescents" }, { "code": "5B53", "title": "Stunting in infants, children or adolescents" }, { "code": "DD93.Y", "title": "Other functional digestive disorders of infants, neonates or toddlers" }, { "code": "QE70.Z", "title": "Problems related to primary support group, including family circumstances, unspecified" }, { "code": "8C74.1Z", "title": "Periodic paralysis, unspecified" }, { "code": "2B90.Y", "title": "Other specified malignant neoplasms of colon" }, { "code": "EE61", "title": "Superficial fibromatoses" }, { "code": "9B70", "title": "Inherited retinal dystrophies" } ]	=== ICD-11 CODES FOUND === [5B7Z] Unspecified undernutrition Also known as: Unspecified undernutrition \| Malnutrition NOS \| nutritional deficiency NOS \| nutritional depletion NOS \| severe malnutrition NOS [5B51] Wasting in infants, children or adolescents Also known as: Wasting in infants, children or adolescents \| Moderate wasting in infants, children or adolescents \| moderate thinness in school-aged children and adolescents \| Moderate acute undernutrition \| Severe wasting in infants, children or adolescents [5B53] Stunting in infants, children or adolescents Also known as: Stunting in infants, children or adolescents \| Moderate stunting in infants, children or adolescents \| Moderate chronic undernutrition \| Severe stunting in infants, children or adolescents \| Severe chronic undernutrition [DD93.Y] Other functional digestive disorders of infants, neonates or toddlers Also known as: Other functional digestive disorders of infants, neonates or toddlers \| Functional vomiting or aerophagia in childhood \| Adolescent rumination syndrome \| rumination syndrome in children \| Cyclic vomiting syndrome in children [QE70.Z] Problems related to primary support group, including family circumstances, unspecified Also known as: Problems related to primary support group, including family circumstances, unspecified \| Problems related to primary support group, including family circumstances \| family problem \| problem related to primary support group \| Problem related to gambling in the family [8C74.1Z] Periodic paralysis, unspecified Also known as: Periodic paralysis, unspecified \| Periodic paralysis \| Westphal disease \| periodic myotonia \| myoplegic dystrophy [2B90.Y] Other specified malignant neoplasms of colon Also known as: Other specified malignant neoplasms of colon \| Neuroendocrine neoplasm of colon \| Colon endocrine neoplasm \| Neuroendocrine carcinoma of colon \| NEC - [neuroendocrine carcinoma] of colon [EE61] Superficial fibromatoses Also known as: Superficial fibromatoses \| Pachydermodactyly \| Camptodactyly or streblodactyly \| Familial camptodactyly \| Sporadic camptodactyly [9B70] Inherited retinal dystrophies Also known as: Inherited retinal dystrophies \| hereditary retinal dystrophies \| Amaurosis - hypertrichosis \| Autosomal dominant late-onset retinal degeneration \| Bothnia retinal dystrophy Includes: Leber congenital amaurosis \| Stargardt disease \| Vitreoretinal dystrophy === GRAPH WALKS === --- Walk 1 --- [5B7Z] Unspecified undernutrition --PARENT--> [?] Undernutrition Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c... --EXCLUDES--> [?] Intestinal malabsorption Def: Intestinal malabsorption (syndrome) occurs due to pathological interference with the normal physiological sequence of digestion (intraluminal process), absorption (mucosal process), and transport (pos... --- Walk 2 --- [5B7Z] Unspecified undernutrition --PARENT--> [?] Undernutrition Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c... --RELATED_TO--> [?] Malnutrition in pregnancy Def: A condition caused by ingestion of a diet in which the nutrients are lacking or are in excess.... --- Walk 3 --- [5B51] Wasting in infants, children or adolescents --PARENT--> [?] Undernutrition Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c... --CHILD--> [5B52] Acute malnutrition in infants, children or adolescents --- Walk 4 --- [5B51] Wasting in infants, children or adolescents --PARENT--> [?] Undernutrition Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c... --CHILD--> [5B52] Acute malnutrition in infants, children or adolescents --- Walk 5 --- [5B53] Stunting in infants, children or adolescents --PARENT--> [?] Undernutrition Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c... --CHILD--> [5B50] Underweight in infants, children or adolescents --- Walk 6 --- [5B53] Stunting in infants, children or adolescents --PARENT--> [?] Undernutrition Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c... --EXCLUDES--> [?] Effects of hunger	[ "[5B7Z] Unspecified undernutrition\n --PARENT--> [?] Undernutrition\n Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c...\n --EXCLUDES--> [?] Intestinal malabsorption\n Def: Intestinal malabsorption (syndrome) occurs due to pathological interference with the normal physiological sequence of digestion (intraluminal process), absorption (mucosal process), and transport (pos...", "[5B7Z] Unspecified undernutrition\n --PARENT--> [?] Undernutrition\n Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c...\n --RELATED_TO--> [?] Malnutrition in pregnancy\n Def: A condition caused by ingestion of a diet in which the nutrients are lacking or are in excess....", "[5B51] Wasting in infants, children or adolescents\n --PARENT--> [?] Undernutrition\n Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c...\n --CHILD--> [5B52] Acute malnutrition in infants, children or adolescents", "[5B51] Wasting in infants, children or adolescents\n --PARENT--> [?] Undernutrition\n Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c...\n --CHILD--> [5B52] Acute malnutrition in infants, children or adolescents", "[5B53] Stunting in infants, children or adolescents\n --PARENT--> [?] Undernutrition\n Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c...\n --CHILD--> [5B50] Underweight in infants, children or adolescents", "[5B53] Stunting in infants, children or adolescents\n --PARENT--> [?] Undernutrition\n Def: Undernutrition is a condition in which the body’s requirements are unmet due to underconsumption or to impaired absorption and use of nutrients. It can be produced by lack of access to food, or as a c...\n --EXCLUDES--> [?] Effects of hunger" ]	5B51&XS25	Marasmus in children	[ { "from_icd11": "5B7Z", "icd10_code": "E43", "icd10_title": "Unspecified severe protein-calorie malnutrition" }, { "from_icd11": "5B7Z", "icd10_code": "E538", "icd10_title": "Deficiency of other specified B group vitamins" }, { "from_icd11": "5B7Z", "icd10_code": "E569", "icd10_title": "Vitamin deficiency, unspecified" }, { "from_icd11": "5B7Z", "icd10_code": "E638", "icd10_title": "Other specified nutritional deficiencies" }, { "from_icd11": "5B7Z", "icd10_code": "E639", "icd10_title": "Nutritional deficiency, unspecified" }, { "from_icd11": "5B7Z", "icd10_code": "E41", "icd10_title": "Nutritional marasmus" }, { "from_icd11": "5B7Z", "icd10_code": "E539", "icd10_title": "Vitamin B deficiency, unspecified" }, { "from_icd11": "5B7Z", "icd10_code": "E568", "icd10_title": "Deficiency of other vitamins" }, { "from_icd11": "5B7Z", "icd10_code": "E649", "icd10_title": "Sequelae of unspecified nutritional deficiency" }, { "from_icd11": "5B7Z", "icd10_code": "E618", "icd10_title": "Deficiency of other specified nutrient elements" }, { "from_icd11": "5B7Z", "icd10_code": "E40-E46", "icd10_title": "" }, { "from_icd11": "5B7Z", "icd10_code": "E50-E64", "icd10_title": "" }, { "from_icd11": "5B7Z", "icd10_code": "E53", "icd10_title": "Deficiency of other B group vitamins" }, { "from_icd11": "5B7Z", "icd10_code": "E56", "icd10_title": "Other vitamin deficiencies" }, { "from_icd11": "5B7Z", "icd10_code": "E61", "icd10_title": "Deficiency of other nutrient elements" } ]	E43	Unspecified severe protein-calorie malnutrition
A 67-year-old man underwent colonoscopy because of weight loss and nausea. Sigmoid colon cancer with all circumference-related stenosis was found by the examination , and the patient was transferred to our hospital for an additional close inspection and medical treatment. There was no medical history for him. None of his family had a clear history of cancer. Hematological examination showed no elevation of inflammation. The serum carcinoembryonic antigen (CEA) was 5.5 μg/ml (normal range < 5.0 μg/ml) and the serum carbohydrate antigen 19-9 (CA19-9) was 7.8 U/ml (normal range < 37 U/ml). The small intestine ileus caused by the invasion of sigmoid colon cancer to the ileum developed after the transfer . The maximum tumor diameter was 6.5 cm, sized by computed tomography (CT) scan . The decompression of the small intestine was performed before the surgery using a nasal ileus tube. Laparoscopic high anterior resection and extended excision of small intestine segmental resection were performed . A small amount of ascites existed in the pouch of Douglas , and the operative rapid pathologic diagnosis examination showed class I. We firstly divided the small intestine at the point of oral and anal 10 cm distance from the invaded region. Secondly, we dissected the ileal mesentery using an energy device. Thirdly, we performed usual inner approach of high anterior resection for the sigmoid colon cancer. Protective laparoscopic operation was done, and the sigmoid colon cancer with invaded ileum was resected en bloc. Because there was no sign of lymph node metastasis in the ileal mesentery, ileal mesentery resection was performed to a minimum . The operation time was 294 min, and the blood loss volume was 32 ml. Histopathological analysis revealed a pathological diagnosis as pT4b (ileal submucosal invasion) N0 (0/11) M0 f Stage II, moderately differentiated tubular adenocarcinoma, ly1, v2, PN0 . The postoperative course was good, and he was discharged on the 13th postoperative day. Although adjuvant chemotherapy with capecitabine after the operation was planned for half a year, treatment was suspended in the first course due to the patient’s self-judgment. We enforced contrasting CT scan every 6 months. No recurrence was observed for a year after the operation, but metastasis recurrence in the para-aortic lymph node was found by a follow-up abdominal contrasting CT scan one and a half years after the operation . Metastasis in the para-aortic lymph node was not observed by preoperative CT scan . The tumor markers at that time were at normal levels (CEA was 3.4 μg/ml and CA19-9 was 2.6 U/ml). 18 F-fluorodeoxyglucose (FDG) positron emission tomography (PET) revealed that FDG accumulated only in the para-aortic lymph node . Although there was the possibility of dissemination of sigmoidal cancer, we decided to perform surgical resection because the tumor marker level was normal and FDG was not accumulated in any other place. Because there were no findings about small intestine carcinoma in PET–CT and no clinical symptoms of small intestine carcinoma like progression of anemia and elevated tumor markers, we did not review the small intestine for carcinoma by the enteroscopy. Laparoscopic metastasis lymphadenectomy was performed due to the diagnosis of metachronous metastasis to the para-aortic lymph node alone. Intraoperative findings revealed that lymph node metastasis occurred in the mesentery of the ileum where we had performed the partial resection of invaded ileum in the first operation. The lymph node metastasis was located central side of the anastomotic intestine mesentery and near the marginal artery. Protective laparoscopic operation was done and we could preserve the marginal artery . The operation time was 160 min, and the blood loss volume was low. Histopathological analysis was consistent with a lymph node metastasized by colon cancer recurrence as moderately differentiated tubular adenocarcinoma . The postoperative course was good, and he was discharged on the 8th postoperative day. Although we recommended adjuvant chemotherapy after the second surgery, the patient refused. No adjuvant treatment was given after the secondary operation, and he is still alive with no recurrence 1 year and 9 months after the first operation. Fig. 1 Colonoscopy revealed a sigmoid colon cancer with all circumference-related stenosis Fig. 2 a–c CT revealed a 6.5-cm-sized sigmoid colon cancer. Tumor invasion was extended to the ileum and caused the small intestine ileus. The arrowhead shows sigmoidal tumor, and the arrow shows ileal invasion Fig. 3 a, b Tumor invasion was extended to the ileum. The arrow shows ileal invasion caused by sigmoidal tumor. c A small amount of ascites existed in the pouch of Douglas. d The invaded ileum was resected with sigmoid colon cancer. Ileal mesentery resection was performed to a minimum, and the arrow shows ileal mesenteric suture. e, f Laparoscopic high anterior resection with D3 lymph node dissection was performed. The arrow shows inferior mesenteric arterial root Fig. 4 a, b Sigmoid colon cancer with invaded ileum was resected en bloc. Ileal mesentery resection was performed to a minimum Fig. 5 a, b Histopathological analysis revealed that tumor invasion was extended to the ileal submucosa. a H&E, × 1.25, b H&E, × 40 Fig. 6 a Metastasis in the para-aortic lymph node was not observed by the preoperative CT scan. b The para-aortic lymph node metastasis was found by a follow-up abdominal contrasting CT scan one and a half years after the operation. The arrow shows the lymph node metastasis Fig. 7 a, b PET revealed FDG accumulated only into the para-aortic lymph node Fig. 8 a Intraoperative findings revealed that lymph node metastasis occurred in the mesentery of the ileum. The arrow shows the lymph node metastasis in the ileal mesentery. b–d Lymphadenectomy was performed, and the arrowhead shows the defect of ileal mesentery after lymphadenectomy. The arrow shows the anastomosis where the first operation of the partial resection of invaded ileum was done, and it was near the lymph node metastasis location Fig. 9 a Resected specimen. b, c Histopathological analysis was consistent with a metastatic lymph node by colon cancer recurrence as moderately differentiated tubular adenocarcinoma. b H&E, × 1.25, c H&E, × 10	3.861328	0.981934	sec[1]/p[0]	en	0.999996	33492540	https://doi.org/10.1186/s40792-021-01114-w	[ "lymph", "node", "metastasis", "cancer", "ileal", "colon", "small", "ileum", "resection", "mesentery" ]	[ { "code": "BD9Z", "title": "Disorders of lymphatic vessels or lymph nodes, unspecified" }, { "code": "BD90.Z", "title": "Lymphadenitis, unspecified" }, { "code": "BD90.Y", "title": "Other specified lymphadenitis" }, { "code": "BD9Y", "title": "Other specified disorders of lymphatic vessels or lymph nodes" }, { "code": "MA01.Z", "title": "Enlarged lymph nodes, unspecified" }, { "code": "FA20.0", "title": "Seropositive rheumatoid arthritis" }, { "code": "MF30", "title": "Breast lump or mass female" }, { "code": "BB40", "title": "Acute or subacute infectious endocarditis" }, { "code": "FA0Z", "title": "Osteoarthritis, unspecified" }, { "code": "FA85.10", "title": "Localised central endplate defect" } ]	=== ICD-11 CODES FOUND === [BD9Z] Disorders of lymphatic vessels or lymph nodes, unspecified Also known as: Disorders of lymphatic vessels or lymph nodes, unspecified \| Lymphatic system disorders \| lymph disease NOS \| lymph gland disease \| Lymphatic system disease NOS [BD90.Z] Lymphadenitis, unspecified Also known as: Lymphadenitis, unspecified \| Lymphadenitis \| adenitis NOS \| inflammation of gland \| lymphatic gland inflammation [BD90.Y] Other specified lymphadenitis Also known as: Other specified lymphadenitis \| Dermatopathic lymphadenopathy \| lipomelanotic reticulosis \| Infective inguinal bubo \| bubo [BD9Y] Other specified disorders of lymphatic vessels or lymph nodes Also known as: Other specified disorders of lymphatic vessels or lymph nodes \| Chylous cyst \| Mesentery chylous cyst \| Peritoneum chylous cyst \| Lymphocele [MA01.Z] Enlarged lymph nodes, unspecified Also known as: Enlarged lymph nodes, unspecified \| Enlarged lymph nodes \| swollen glands \| Lymphadenopathy \| adenopathy [FA20.0] Seropositive rheumatoid arthritis Also known as: Seropositive rheumatoid arthritis \| high positive rheumatoid factor \| low positive rheumatoid factor \| high positive anticitrullinated protein antibody \| low positive anticitrullinated protein antibody [MF30] Breast lump or mass female Also known as: Breast lump or mass female \| breast irregular nodularity \| breast node \| lump in breast \| lump or mass in breast NOS [BB40] Acute or subacute infectious endocarditis Also known as: Acute or subacute infectious endocarditis \| subacute infective endocarditis NOS \| infective endocarditis NOS \| acute infective endocarditis NOS \| infectious endocarditis Excludes: Infectious myocarditis [FA0Z] Osteoarthritis, unspecified Also known as: Osteoarthritis, unspecified \| osteoarthritis NOS \| arthrosis NOS \| OA - [osteoarthritis] \| Osteoarthritis with determinants [FA85.10] Localised central endplate defect Also known as: Localised central endplate defect \| Schmorl nodes \| schmorl's nodes \| schmorl's nodules === GRAPH WALKS === --- Walk 1 --- [BD9Z] Disorders of lymphatic vessels or lymph nodes, unspecified --PARENT--> [?] Disorders of lymphatic vessels or lymph nodes Def: Disorders due to developmental and acquired disturbances of lymph circulation and drainage and to infective disorders of lymph vessels and nodes.... --RELATED_TO--> [?] Lymphatic malformations Def: Lymphatic malformations (LM), formerly referred to by the term lymphangioma, are malformations of the lymphatic system which result in obstructed lymphatic drainage. There are two types of LM: macrocy... --- Walk 2 --- [BD9Z] Disorders of lymphatic vessels or lymph nodes, unspecified --PARENT--> [?] Disorders of lymphatic vessels or lymph nodes Def: Disorders due to developmental and acquired disturbances of lymph circulation and drainage and to infective disorders of lymph vessels and nodes.... --RELATED_TO--> [?] Lymphatic malformations Def: Lymphatic malformations (LM), formerly referred to by the term lymphangioma, are malformations of the lymphatic system which result in obstructed lymphatic drainage. There are two types of LM: macrocy... --- Walk 3 --- [BD90.Z] Lymphadenitis, unspecified --PARENT--> [BD90] Lymphadenitis --CHILD--> [BD90.2] Chronic lymphadenitis --- Walk 4 --- [BD90.Z] Lymphadenitis, unspecified --PARENT--> [BD90] Lymphadenitis --CHILD--> [BD90.2] Chronic lymphadenitis --- Walk 5 --- [BD90.Y] Other specified lymphadenitis --PARENT--> [BD90] Lymphadenitis --CHILD--> [BD90.2] Chronic lymphadenitis --- Walk 6 --- [BD90.Y] Other specified lymphadenitis --PARENT--> [BD90] Lymphadenitis --CHILD--> [BD90.1] Nonspecific mesenteric lymphadenitis	[ "[BD9Z] Disorders of lymphatic vessels or lymph nodes, unspecified\n --PARENT--> [?] Disorders of lymphatic vessels or lymph nodes\n Def: Disorders due to developmental and acquired disturbances of lymph circulation and drainage and to infective disorders of lymph vessels and nodes....\n --RELATED_TO--> [?] Lymphatic malformations\n Def: Lymphatic malformations (LM), formerly referred to by the term lymphangioma, are malformations of the lymphatic system which result in obstructed lymphatic drainage. There are two types of LM: macrocy...", "[BD9Z] Disorders of lymphatic vessels or lymph nodes, unspecified\n --PARENT--> [?] Disorders of lymphatic vessels or lymph nodes\n Def: Disorders due to developmental and acquired disturbances of lymph circulation and drainage and to infective disorders of lymph vessels and nodes....\n --RELATED_TO--> [?] Lymphatic malformations\n Def: Lymphatic malformations (LM), formerly referred to by the term lymphangioma, are malformations of the lymphatic system which result in obstructed lymphatic drainage. There are two types of LM: macrocy...", "[BD90.Z] Lymphadenitis, unspecified\n --PARENT--> [BD90] Lymphadenitis\n --CHILD--> [BD90.2] Chronic lymphadenitis", "[BD90.Z] Lymphadenitis, unspecified\n --PARENT--> [BD90] Lymphadenitis\n --CHILD--> [BD90.2] Chronic lymphadenitis", "[BD90.Y] Other specified lymphadenitis\n --PARENT--> [BD90] Lymphadenitis\n --CHILD--> [BD90.2] Chronic lymphadenitis", "[BD90.Y] Other specified lymphadenitis\n --PARENT--> [BD90] Lymphadenitis\n --CHILD--> [BD90.1] Nonspecific mesenteric lymphadenitis" ]	BD9Z	Disorders of lymphatic vessels or lymph nodes, unspecified	[ { "from_icd11": "BD9Z", "icd10_code": "I898", "icd10_title": "Other specified noninfective disorders of lymphatic vessels and lymph nodes" }, { "from_icd11": "BD9Z", "icd10_code": "I899", "icd10_title": "Noninfective disorder of lymphatic vessels and lymph nodes, unspecified" }, { "from_icd11": "BD9Z", "icd10_code": "I89", "icd10_title": "Other noninfective disorders of lymphatic vessels and lymph nodes" }, { "from_icd11": "BD90.Z", "icd10_code": "I889", "icd10_title": "Nonspecific lymphadenitis, unspecified" }, { "from_icd11": "BD90.Z", "icd10_code": "I88", "icd10_title": "Nonspecific lymphadenitis" }, { "from_icd11": "BD90.Z", "icd10_code": "I888", "icd10_title": "Other nonspecific lymphadenitis" }, { "from_icd11": "BD90.Z", "icd10_code": "L00-L08", "icd10_title": "" }, { "from_icd11": "MA01.Z", "icd10_code": "R599", "icd10_title": "Enlarged lymph nodes, unspecified" }, { "from_icd11": "MA01.Z", "icd10_code": "R59", "icd10_title": "Enlarged lymph nodes" }, { "from_icd11": "FA20.0", "icd10_code": "M0569", "icd10_title": "Rheumatoid arthritis of multiple sites with involvement of other organs and systems" }, { "from_icd11": "FA20.0", "icd10_code": "M0579", "icd10_title": "Rheumatoid arthritis with rheumatoid factor of multiple sites without organ or systems involvement" }, { "from_icd11": "FA20.0", "icd10_code": "M05612", "icd10_title": "Rheumatoid arthritis of left shoulder with involvement of other organs and systems" }, { "from_icd11": "FA20.0", "icd10_code": "M0570", "icd10_title": "Rheumatoid arthritis with rheumatoid factor of unspecified site without organ or systems involvement" }, { "from_icd11": "FA20.0", "icd10_code": "M0560", "icd10_title": "Rheumatoid arthritis of unspecified site with involvement of other organs and systems" }, { "from_icd11": "FA20.0", "icd10_code": "M0500", "icd10_title": "Felty's syndrome, unspecified site" } ]	I898	Other specified noninfective disorders of lymphatic vessels and lymph nodes
A 16-year-old male who injured his right shoulder in a motorbike accident presented himself to a regional general hospital on the same day of the injury. On initial physical examination, he complained of tenderness of the shoulder and axillary regions. Roentgenograms revealed a non-displaced fracture at the distal end of the right clavicle but neither dislocation of the glenohumeral joint nor humeral neck fracture was observed . Blood supply to the right arm was intact, and the pulses of the radial and ulnar arteries in the affected right arm were palpable. Blood pressure difference between the right and left upper arms was not evaluated at that point. Neurological examination revealed sensory deficit on the lateral aspect of the right upper arm. Although the patient could elevate the upper arm more than 90° against gravity, muscle contraction of the deltoid muscle was not seen under voluntary elevation of the upper arm. There was no other sensory or muscle power deficit. At that point, axillary nerve palsy was diagnosed, and an electrophysiological examination was scheduled for the detailed evaluation of other nerves. However, on the third day after the accident, the patient developed swelling and tenderness in his right upper arm and shoulder, and became paralyzed in his right arm. He was referred to our department. On physical examination, the pulses of the radial and ulnar arteries of the right arm were significantly diminished compared to those in the left arm. Blood pressure was 42/20 mm Hg (systolic/diastolic) in the right arm, and 122/70 mm Hg in the left arm. Widespread ecchymoses were observed on the medial aspect of the upper arm. On neurological examination, muscle power in the right arm included brachialis (M0), biceps (M0), triceps (M0), pectoralis major (M3), pronator teres (M3), supinator (M1), flexor carpi radialis (M3), flexor carpi ulnaris (M3), extensor carpi radialis longus/brevis (M2), flexor digitorum profundus/sublimis (M4), flexor policis longus (M4), extensor digitorum communis (M0), extensor policis longus (M0), and intrinsic muscles (M2) according to the classical medical research council scale. Muscle power of the deltoid and supra/infra spinatus muscles could not be evaluated exactly due to shoulder pain from the fractured clavicle. However, the patient could elevate the upper arm 120° against gravity, indicating that the suprascapular nerve was intact. Complete sensory loss was observed in the lateral aspect of the upper arm and dorsoradial aspects of the forearm and hand, and the volar aspects of the forearm and hand were slightly numbed. The patient complained of slight dull pain (neurostenalgia) at the radial aspect of the forearm. Selective digital subtraction angiography of the axillary artery through the right femoral artery showed complete occlusion of the axillary artery at the site of the coracoid process of the scapula . Collateral blood circulation to the forearm was preserved through the thoracoacromial and posterior circumflex humeral arteries. Magnetic resonance imaging (MRI) demonstrated a mass measuring 4 × 5 cm that was suspected to be a hematoma compressing the axillary artery and brachial plexus in a space between the subscapular muscle and the pectoralis minor muscle . On the same day of referral, surgical explorations of the axillary artery and brachial plexus, and evacuation of the hematoma were performed under general anesthesia. At intraoperative observations, the axillary artery was found to be occluded with thrombus along 5 cm, at the site where the pectoralis minor muscle overlaid the artery; however, the adventitia of the artery was not ruptured. A subscapular artery was ruptured at the site of origin from the axillary artery; the brachial plexus was compressed by the hematoma along a dorsal direction. Following evacuation of the hematoma, the infraclavicular part of the brachial plexus was explored from the origin of the cords to the terminal branches. Macroscopically, the brachial plexus was intact. Intraoperative electrostimulation on the terminal branches of the infraclavicular brachial plexus was performed. Although stimulation of the nerves other than the axillary nerve induced the contraction of the innervated muscles, deltoid muscle contraction didn't occur after the stimulation of the axillary nerve. After exploration of the axillary nerve, it appeared continuous, but tension was slackened. External neurolysis of the nerve was performed to promote spontaneous recovery. The axillary artery was transected to observe the interluminal conditions. The artery was occluded by thrombus; the intima was damaged. The intraluminal thrombus was removed using a 4 French Fogarty catheter in both proximal and distal directions, and revascularization was established by performing an interpositional great saphenous vein graft. The proximal and distal sites of the graft were anastomosed using side-to-end and end-to-end methods, respectively. Care was taken not to sacrifice collateral blood circulation . The ruptured subscapular artery was not repaired but ligated because the artery was stretched and damaged. Heparin and regional thrombolytic therapy with urokinase were administered intravenously to the patient for 5 days after surgery as an anticoagulatory treatment. Just after the surgery, both the radial and ulnar arteries of the right arm became pulsatile, as in the left arm. The sensorimotor functions started to recover from the first day after surgery. All nervous functions except for that of the axillary nerve were sufficiently recovered within 3 days. One year after surgery, muscular powers except for that of the deltoid muscle were completely recovered. Despite of the atrophy of the deltoid muscle, muscle contraction was seen under voluntary elevation of the upper arm against gravity. The patient could abduct his shoulder 180° with a 10-kg load by conjoint function with the supra spinatus muscle. Electromyographic examination of the deltoid muscle revealed motor units under voluntary control, mild decrease in interference patterns and re-innervations, confirming the partial recovery of muscle function. Sensory disturbance on the lateral aspect of the right upper arm also recovered to normal sensation. The patient was satisfied with the function of his right arm .	3.990234	0.977051	sec[1]/p[0]	en	0.999998	18373866	https://doi.org/10.1186/1749-7221-3-9	[ "muscle", "artery", "axillary", "nerve", "that", "deltoid", "brachial", "plexus", "shoulder", "blood" ]	[ { "code": "FB3Z", "title": "Disorders of muscles, unspecified" }, { "code": "FB32.Y", "title": "Other specified disorders of muscles" }, { "code": "8C70.Z", "title": "Muscular dystrophy, unspecified" }, { "code": "FB32.2Z", "title": "Ischaemic infarction of muscle, unspecified" }, { "code": "FB56.2", "title": "Myalgia" }, { "code": "BD5Z", "title": "Diseases of arteries or arterioles, unspecified" }, { "code": "BD52", "title": "Certain specified disorders of arteries or arterioles" }, { "code": "BD52.3", "title": "Rupture of artery" }, { "code": "BD52.2", "title": "Stricture of artery" }, { "code": "BD40.Z", "title": "Atherosclerotic chronic arterial occlusive disease, unspecified" } ]	=== ICD-11 CODES FOUND === [FB3Z] Disorders of muscles, unspecified Also known as: Disorders of muscles, unspecified \| disorder of muscle, unspecified \| muscle disease \| muscular disease \| muscular disorder [FB32.Y] Other specified disorders of muscles Also known as: Other specified disorders of muscles \| Muscle wasting or atrophy, not elsewhere classified \| muscle wasting \| muscle wasting disorder \| Sarcopenia [8C70.Z] Muscular dystrophy, unspecified Also known as: Muscular dystrophy, unspecified \| Muscular dystrophy \| Gower's muscular dystrophy \| progressive musclular dystrophy \| pseudohypertrophic atrophy [FB32.2Z] Ischaemic infarction of muscle, unspecified Also known as: Ischaemic infarction of muscle, unspecified \| Ischaemic infarction of muscle \| muscle infarction [FB56.2] Myalgia Definition: This is a disorder characterised by pain in a muscle or group of muscles. Also known as: Myalgia \| muscle ache \| muscle soreness \| muscular pain \| myalgic Excludes: Chronic primary musculoskeletal pain \| Chronic secondary musculoskeletal pain [BD5Z] Diseases of arteries or arterioles, unspecified Also known as: Diseases of arteries or arterioles, unspecified \| artery disease NOS \| arterial disease NOS \| arteriolar disease NOS \| disorder of artery NOS [BD52] Certain specified disorders of arteries or arterioles Also known as: Certain specified disorders of arteries or arterioles \| Aortic dilatation - joint hypermobility - arterial tortuosity \| Generalised arterial calcification of infancy \| Median arcuate ligament syndrome \| Aortic root abscess Excludes: collagen (vascular) diseases \| Hypersensitivity angiitis \| Acute arterial occlusion [BD52.3] Rupture of artery Also known as: Rupture of artery \| ruptured artery \| artery fistula \| Aortic duodenal fistula \| Aortic colon fistula Excludes: traumatic rupture of artery - see injury of blood vessel by body region [BD52.2] Stricture of artery Also known as: Stricture of artery \| arterial stenosis \| arterial stricture \| artery stricture \| stenosis of artery [BD40.Z] Atherosclerotic chronic arterial occlusive disease, unspecified Also known as: Atherosclerotic chronic arterial occlusive disease, unspecified \| Atherosclerotic chronic arterial occlusive disease \| arteriosclerosis, NOS \| generalised atherosclerosis \| atherosclerosis NOS === GRAPH WALKS === --- Walk 1 --- [FB3Z] Disorders of muscles, unspecified --PARENT--> [?] Disorders of muscles --CHILD--> [FB30] Infectious myositis Def: Infective myositis is an acute, subacute, or chronic infection of skeletal muscle and may be caused by a wide range of infecting organisms. Immunosuppression, particularly as the result of HIV infecti... --- Walk 2 --- [FB3Z] Disorders of muscles, unspecified --PARENT--> [?] Disorders of muscles --CHILD--> [FB30] Infectious myositis Def: Infective myositis is an acute, subacute, or chronic infection of skeletal muscle and may be caused by a wide range of infecting organisms. Immunosuppression, particularly as the result of HIV infecti... --- Walk 3 --- [FB32.Y] Other specified disorders of muscles --PARENT--> [FB32] Certain specified disorders of muscle Def: This is an impairment of health or a condition of abnormal functioning of the muscle that does not fit in another category.... --EXCLUDES--> [?] Cramp or spasm --- Walk 4 --- [FB32.Y] Other specified disorders of muscles --PARENT--> [FB32] Certain specified disorders of muscle Def: This is an impairment of health or a condition of abnormal functioning of the muscle that does not fit in another category.... --EXCLUDES--> [?] Cramp or spasm --- Walk 5 --- [8C70.Z] Muscular dystrophy, unspecified --PARENT--> [8C70] Muscular dystrophy Def: Progressive, hereditary skeletal muscle diseases characterised by muscle weakness, wasting, defects in muscle proteins, necrosis of muscle tissue and replacement of muscle tissue with connective and f... --RELATED_TO--> [?] Epidermolysis bullosa simplex with muscular dystrophy Def: Epidermolysis bullosa simplex with muscular dystrophy (EBS-MD) is an autosomal recessive basal subtype of EBS due to mutations the PLEC gene encoding plectin. It is characterised by generalised bliste... --- Walk 6 --- [8C70.Z] Muscular dystrophy, unspecified --PARENT--> [8C70] Muscular dystrophy Def: Progressive, hereditary skeletal muscle diseases characterised by muscle weakness, wasting, defects in muscle proteins, necrosis of muscle tissue and replacement of muscle tissue with connective and f... --CHILD--> [8C70.0] Becker muscular dystrophy	[ "[FB3Z] Disorders of muscles, unspecified\n --PARENT--> [?] Disorders of muscles\n --CHILD--> [FB30] Infectious myositis\n Def: Infective myositis is an acute, subacute, or chronic infection of skeletal muscle and may be caused by a wide range of infecting organisms. Immunosuppression, particularly as the result of HIV infecti...", "[FB3Z] Disorders of muscles, unspecified\n --PARENT--> [?] Disorders of muscles\n --CHILD--> [FB30] Infectious myositis\n Def: Infective myositis is an acute, subacute, or chronic infection of skeletal muscle and may be caused by a wide range of infecting organisms. Immunosuppression, particularly as the result of HIV infecti...", "[FB32.Y] Other specified disorders of muscles\n --PARENT--> [FB32] Certain specified disorders of muscle\n Def: This is an impairment of health or a condition of abnormal functioning of the muscle that does not fit in another category....\n --EXCLUDES--> [?] Cramp or spasm", "[FB32.Y] Other specified disorders of muscles\n --PARENT--> [FB32] Certain specified disorders of muscle\n Def: This is an impairment of health or a condition of abnormal functioning of the muscle that does not fit in another category....\n --EXCLUDES--> [?] Cramp or spasm", "[8C70.Z] Muscular dystrophy, unspecified\n --PARENT--> [8C70] Muscular dystrophy\n Def: Progressive, hereditary skeletal muscle diseases characterised by muscle weakness, wasting, defects in muscle proteins, necrosis of muscle tissue and replacement of muscle tissue with connective and f...\n --RELATED_TO--> [?] Epidermolysis bullosa simplex with muscular dystrophy\n Def: Epidermolysis bullosa simplex with muscular dystrophy (EBS-MD) is an autosomal recessive basal subtype of EBS due to mutations the PLEC gene encoding plectin. It is characterised by generalised bliste...", "[8C70.Z] Muscular dystrophy, unspecified\n --PARENT--> [8C70] Muscular dystrophy\n Def: Progressive, hereditary skeletal muscle diseases characterised by muscle weakness, wasting, defects in muscle proteins, necrosis of muscle tissue and replacement of muscle tissue with connective and f...\n --CHILD--> [8C70.0] Becker muscular dystrophy" ]	FB3Z	Disorders of muscles, unspecified	[ { "from_icd11": "FB3Z", "icd10_code": "M60831", "icd10_title": "Other myositis, right forearm" }, { "from_icd11": "FB3Z", "icd10_code": "M60869", "icd10_title": "Other myositis, unspecified lower leg" }, { "from_icd11": "FB3Z", "icd10_code": "M60811", "icd10_title": "Other myositis, right shoulder" }, { "from_icd11": "FB3Z", "icd10_code": "M6080", "icd10_title": "Other myositis, unspecified site" }, { "from_icd11": "FB3Z", "icd10_code": "M60851", "icd10_title": "Other myositis, right thigh" }, { "from_icd11": "FB3Z", "icd10_code": "M6010", "icd10_title": "Interstitial myositis of unspecified site" }, { "from_icd11": "FB3Z", "icd10_code": "M6018", "icd10_title": "Interstitial myositis, other site" }, { "from_icd11": "FB3Z", "icd10_code": "M6088", "icd10_title": "Other myositis, other site" }, { "from_icd11": "FB3Z", "icd10_code": "M60862", "icd10_title": "Other myositis, left lower leg" }, { "from_icd11": "FB3Z", "icd10_code": "M60861", "icd10_title": "Other myositis, right lower leg" }, { "from_icd11": "FB3Z", "icd10_code": "M6089", "icd10_title": "Other myositis, multiple sites" }, { "from_icd11": "FB3Z", "icd10_code": "M60852", "icd10_title": "Other myositis, left thigh" }, { "from_icd11": "FB3Z", "icd10_code": "M60821", "icd10_title": "Other myositis, right upper arm" }, { "from_icd11": "FB3Z", "icd10_code": "M60871", "icd10_title": "Other myositis, right ankle and foot" }, { "from_icd11": "FB3Z", "icd10_code": "M60812", "icd10_title": "Other myositis, left shoulder" } ]	M60831	Other myositis, right forearm
The patient was a male, 46 years old. On the morning of March 2, 2023, he worked in an environmental protection energy company in Shandong Province for the first time, which was a waste treatment power plant. His job was to flush the sludge from the inner wall of the sludge treatment tank with a water gun, and the sludge could release hydrogen sulfide gas. According to his and his co-workers, the patient was about to start work after entering the tank for about 1 min, when he suddenly smelt a harsh, pungency smell, and felt dizzy and weak, then the patient suddenly fainted. After hearing the sound of his fainting, the workers waiting at the entrance of the tank immediately called someone to enter the tank and quickly pulled him out, and then sent to the local hospital. At the local hospital, the patient was confused, accompanied by irritability, convulsions and other manifestations, and was treated with sedation (Midazolam and Diprivan) and nutritional support [Structured fat emulsion (20%)/amino acid (16%), glucose injection (13%)]. Two days later , the patient’s condition did not improve. For further diagnosis and treatment, the patient was transferred to the Department of Poisoning and Occupational Diseases in our hospital. The patient awoke in the ambulance while being transferred to this hospital with gradual improvement in consciousness but no recollection of the events that followed. After investigation, the causes of this accident were as follows: the labor unit of the patient did not provide any personal protective equipment, and the patient only wore an ordinary raincoat into the tank; The pipeline around the sludge treatment tank was blocked by sludge, resulting in the accumulation of a large number of high-concentration hydrogen sulfide gas in the tank, causing the patient to faint soon after entering the tank. Although there is an exhaust fan on the tank wall, the volume of the exhaust fan is too small to have any effect. Due to the very short time in the tank, the worker pulled out the patient immediately after entering the tank, and no health abnormalities were found. He was admitted to our hospital with a “disturbance of consciousness for 2 days,” and physical examination findings were as follows: temperature was 37°C; pulse was 95 times/min; respiratory rate was 18 times/min; blood pressure was 131/79 mmHg; and SPO 2 was 96%. The patient had blurred consciousness, poor spirit, and autonomous posture; he was cooperative during cooperation. The skin mucosa of the whole body was not yellow, and the superficial lymph nodes were not large. His bilateral pupils were large and round, reflecting light, and the size of the pupil was 3 mm. There was no cyanosis or congestion in the pharynx. The patient had a soft neck with no resistance, bilateral thorax movement, clear lung sounds when breathing, and no dry and wet rales. The rhythm was normal and there was no pathological murmur in the valve area. The abdomen is soft, there is no tenderness and rebound pain in the whole abdomen, liver, spleen, and ribs, and there is no tapping pain in the liver and kidney areas. No deformity of spine and limbs. The patient had physiological reflex and no pathological reflex. The results of the patient’s blood draw after admission from 2023/3/6 to 2023/3/26, as shown in Table 1 , were basically normal except for a higher number of inflammatory cells (neutrophils ratio was high on Day 1 and Day 3, and the total number of white blood cells was high on Day 1), which indicated an inflammatory response in this patient. Magnetic resonance imaging (MRI) after admission , as shown in Figure 2 , patchy long TI and long T2 signal shadows were seen in the left basal ganglia, and T2 FLAIR showed medium-low peripheral hyperintensity with clear boundaries. The left frontal lobe showed a speckle-like long TI and long T2 signal shadow, T2 FLAIR hyperintensity, and DWI isointensity. The examination concluded that abnormal signals in the left basal ganglia, considering softening foci ; There were a few abnormal signals in the left frontal lobe, considering non-specific signals or degenerative lesions around the perivascular space . And the subsequent reexamination on 2023/3/26 revealed a slight reduction in the extent of the lesion . On CT on 2023/3/6, as shown in Figure 3 , there was a little exudation in the lower lobe of the right lung, with localized interstitial changes , which were relieved on the second examination on 2023/3/26 . There was localized thickening of the right pleura , which were also relieved on the second examination . According to the diagnostic criteria of occupational acute hydrogen sulfide poisoning of the People’s Republic of China ( 12 ), the patient had a history of exposure to a large amount of inhaled hydrogen sulfide in a short period of time, and the clinical manifestations of the central nervous system and respiratory system appeared. The on-site labor hygiene investigation also supported the above exposure history, so the patient was diagnosed with occupational acute hydrogen sulfide poisoning. As a consequence, the following management regimen was applied. Dexamethasone (10 mg, ivdrip, qd) was given to reduce the inflammatory reaction, and the drug was discontinued on the 4th day. Salvianolate (200 mg, ivdrip, qd) to improve microcirculation; Torasemide (20 mg, iv, bid) to reduce edema and speed up the excretion of poison; Nalmefene (0.1 mg, iv, bid) to alleviate the symptoms of consciousness disorder and promoting the recovery of consciousness in the patient and was discontinued on Day 3. Flucloxacillin (1 g, ivdrip, q6h) for infection prevention; In addition, he was treated with sedation (Midazolam and Diprivan) and nutritional support [Structured fat emulsion (20%)/amino acid (16%), glucose injection (13%)]. After comprehensive treatment in our hospital, the patient got better and was discharged on the 7th day of admission , and then came for reexamination on 2023/3/26. Studies involving human subjects were reviewed and approved by the Ethics Committee of Qilu Hospital of Shandong University. The patient provided written informed consent to participate in this study. Personal written informed consent has been obtained for the release of any potentially identifiable images or data contained in this article.	3.619141	0.984863	sec[1]/p[0]	en	0.999994	PMC10641707	https://doi.org/10.3389/fpubh.2023.1226282	[ "tank", "sludge", "hydrogen", "sulfide", "this", "consciousness", "which", "large", "long", "time" ]	[ { "code": "1B21.2Y&XN8RB", "title": "Mycobacterium marinum infection" }, { "code": "NE61", "title": "Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified" }, { "code": "PB36&XM8WA4", "title": "Unintentional exposure to or harmful effects of hydrogen cyanide" }, { "code": "PB36&XM7FL0", "title": "Unintentional exposure to or harmful effects of hydrogen sulfide" }, { "code": "PD05&XM8WA4", "title": "Intentional self-harm by exposure to or harmful effects of hydrogen cyanide" }, { "code": "PH56&XM8WA4", "title": "Exposure to or harmful effects of undetermined intent of hydrogen cyanide" }, { "code": "PD05&XM7FL0", "title": "Intentional self-harm by exposure to or harmful effects of hydrogen sulfide" }, { "code": "PH56&XM7FL0", "title": "Exposure to or harmful effects of undetermined intent of hydrogen sulfide" }, { "code": "PE95&XM7FL0", "title": "Assault by exposure to or harmful effects of hydrogen sulfide" }, { "code": "4A01.03", "title": "Transient hypogammaglobulinaemia of infancy" } ]	=== ICD-11 CODES FOUND === [NE61] Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified Also known as: Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified \| Harmful effects of or exposure to noxious substances chiefly nonmedicinal as to source, alcohols \| alcohol poisoning \| alcohol toxicity \| Harmful effects of or exposure to noxious substances chiefly nonmedicinal as to source, Ethanol Excludes: corrosions \| Bacterial foodborne intoxications [4A01.03] Transient hypogammaglobulinaemia of infancy Also known as: Transient hypogammaglobulinaemia of infancy \| immunoglobulin maturational delay \| THI - [transient hypogammaglobulinaemia of infancy] === GRAPH WALKS === --- Walk 1 --- [NE61] Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified --EXCLUDES--> [?] Bacterial foodborne intoxications Def: Any condition caused by an intoxication due to a bacterial toxin. Intoxication is by ingestion of contaminated food.... --CHILD--> [?] Botulism Def: A disease caused by an infection with the gram-positive bacteria Clostridium botulinum. This disease commonly presents with abdominal pain, vomiting, acute paralysis, blurred vision, diplopia, and may... --- Walk 2 --- [NE61] Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified --EXCLUDES--> [?] Burns Def: A burn is an injury to the tissues caused by a pathological flux of energy which causes cellular destruction and irreversible denaturation of proteins and is primarily caused by thermal or other acute... --EXCLUDES--> [?] Sunburn Def: An injury to the skin causing erythema, tenderness, and sometimes blistering and resulting from excessive exposure to the sun. The reaction is produced by the ultraviolet radiation in sunlight....	[ "[NE61] Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified\n --EXCLUDES--> [?] Bacterial foodborne intoxications\n Def: Any condition caused by an intoxication due to a bacterial toxin. Intoxication is by ingestion of contaminated food....\n --CHILD--> [?] Botulism\n Def: A disease caused by an infection with the gram-positive bacteria Clostridium botulinum. This disease commonly presents with abdominal pain, vomiting, acute paralysis, blurred vision, diplopia, and may...", "[NE61] Harmful effects of or exposure to noxious substances, chiefly nonmedicinal as to source, not elsewhere classified\n --EXCLUDES--> [?] Burns\n Def: A burn is an injury to the tissues caused by a pathological flux of energy which causes cellular destruction and irreversible denaturation of proteins and is primarily caused by thermal or other acute...\n --EXCLUDES--> [?] Sunburn\n Def: An injury to the skin causing erythema, tenderness, and sometimes blistering and resulting from excessive exposure to the sun. The reaction is produced by the ultraviolet radiation in sunlight...." ]	1B21.2Y&XN8RB	Mycobacterium marinum infection	[ { "from_icd11": "NE61", "icd10_code": "T5802XA", "icd10_title": "Toxic effect of carbon monoxide from motor vehicle exhaust, intentional self-harm, initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T550X2A", "icd10_title": "Toxic effect of soaps, intentional self-harm, initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T61781A", "icd10_title": "Other shellfish poisoning, accidental (unintentional), initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T551X2A", "icd10_title": "Toxic effect of detergents, intentional self-harm, initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T5891XA", "icd10_title": "Toxic effect of carbon monoxide from unspecified source, accidental (unintentional), initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T63711A", "icd10_title": "Toxic effect of contact with venomous marine plant, accidental (unintentional), initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T63712A", "icd10_title": "Toxic effect of contact with venomous marine plant, intentional self-harm, initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T63713A", "icd10_title": "Toxic effect of contact with venomous marine plant, assault, initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T63714A", "icd10_title": "Toxic effect of contact with venomous marine plant, undetermined, initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T63791A", "icd10_title": "Toxic effect of contact with other venomous plant, accidental (unintentional), initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T63792A", "icd10_title": "Toxic effect of contact with other venomous plant, intentional self-harm, initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T63793A", "icd10_title": "Toxic effect of contact with other venomous plant, assault, initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T63794A", "icd10_title": "Toxic effect of contact with other venomous plant, undetermined, initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T61771A", "icd10_title": "Other fish poisoning, accidental (unintentional), initial encounter" }, { "from_icd11": "NE61", "icd10_code": "T61772A", "icd10_title": "Other fish poisoning, intentional self-harm, initial encounter" } ]	T5802XA	Toxic effect of carbon monoxide from motor vehicle exhaust, intentional self-harm, initial encounter
CCTA also accurately assesses LVEF . Despite this, concerns related to radiation exposure in women limit the use of CCTA and ERNA to patients with low echogenicity and contraindications to CMR . Also, due to the increasing use of prospective electrocardiogram (ECG) gating, which has resulted in lower radiation dose and better image quality , assessment of LV volumes by CCTA is no longer ubiquitously available. Case 1 Cardiac arrest in a 28-year-old woman. A 28-year-old woman with no medical history other than smoking was admitted to the intensive care unit after an out-of-hospital cardiac arrest with ventricular fibrillation on ECG, return of spontaneous circulation after 8 min of cardiopulmonary resuscitation and external defibrillation. At admission, ECG revealed ST-elevation in the anterior leads. Echocardiography showed a hypokinetic left ventricular anterior wall. ICA was performed to rule out MI, showing normal coronary arteries ( A ) and a hypokinetic apex with mildly reduced LVEF of 45% ( B , end-diastolic ventriculography; C , end-systolic ventriculography), suggestive of TTC. CMR performed 10 days later displayed normalization of LVEF and wall motion abnormalities ( D , end-diastolic and E , end-systolic sequences on balanced SSFP cine sequences in 3-chambers view), and no sign of myocardial scar or edema on LGE and T1-mapping sequences ( F , T1 inversion recovery LGE sequence in 3-chambers view) and was therefore not suggestive of TTC. Given the initial ECG and hypokinetic pattern suggestive of a transient reduced coronary flow in the LAD, coronary vasospasm was hypothesized. A second ICA with assessment of coronary microvascular function and coronary vasoreactivity testing was performed ( G , ICA before pharmacological spasm provocation test). Assessment of coronary microvascular function confirmed normal coronary arteries (index of microcirculatory resistance = 10, N < 25; CFR = 4.5, N > 2). Following intracoronary infusion of 100 μg of acetylcholine, all 3 criteria for vasospastic angina were met, i.e., (i) a marked diffuse vasospasm, most pronounced in the proximal left anterior descending artery ( H , red arrowhead), (ii) angina symptoms provoked by acetylcholine infusion, and (iii) ST-elevation on ECG. All these findings were completely reversed after administration of intracoronary nitroglycerin ( I ). Noteworthy, acetylcholine-induced coronary spasm can be induced at lower acetylcholine doses in women than in men, suggesting a higher sensitivity to vasospasms in the female population which could be related to the high prevalence of CMVD . Upon discharge, a defibrillator was implanted and long-term treatment with calcium-channel blockers and long-acting nitrates was introduced along with advises to cease smoking, which constitutes an important risk factor for coronary vasospasm . Given the fact that up to 80% of MINOCA patients are women, an epicardial origin to angina or, in this case, to cardiac arrest should not be dismissed before ruling out coronary vasospasm . ICA-based criteria are well established to diagnose epicardial vasospastic angina and should be discussed after the acute phase when no clear explanation for an ACS in a woman can be evidenced. Abbreviations : ACS : acute coronary syndrome; CFR : coronary flow reserve; CMR : cardiac magnetic resonance; CMVD : coronary microvascular dysfunction; ECG : electrocardiogram; ICA : invasive coronary angiography; LAD : left anterior descending artery; LGE : late gadolinium enhancement; LVEF : left ventricular ejection fraction; MI : myocardial infarction; MINOCA : myocardial infarction with no obstructive coronary artery disease; N : normal; SSFP : steady-state free precession; TTC : Takotsubo cardiomyopathy Case 2 Chest pain in a 62-year-old woman. A 62-year-old woman with a history of hypertension and polycystic ovary syndrome presented with ongoing episodes of atypical chest pain (high abdominal discomfort) and shortness of breath on exertion. These symptoms had evolved for several years and had lately been increasing in frequency. A submaximal stress ECG test reproduced the symptoms accompanied by negative T-waves in the anterior leads ( A ). Subsequently, 13 N-NH 3 -PET-MPI was performed because myocardial ischemia was suspected. CACS calculated from low-dose CT amounted to 5, due to minimal calcification of the left coronary artery ( B , red arrowhead), indicating a low risk of cardiovascular mortality (score A1N1 of the CACS data and reporting system , with A1 indicating a mildly increased risk and N1 indicating the involvement of one single vessel). Analysis of relative perfusion showed homogenous 13 N-NH 3 uptake in all myocardial segments at rest and stress ( C , horizontal and vertical long axes; D and E : polar maps), with no reversible or non-reversible perfusion defects ( F , polar map), thereby making regional ischemia or scar in an epicardial territory unlikely. The CFR calculated from rest ( G , polar map) and stress ( H , polar map) MBF was diffusively reduced (< 2) in all myocardial segments ( I , polar map). This finding was consistent with the diagnosis of CMVD-related microvascular angina. Subsequently, medical treatments of CMVD including betablockers and nitrates were introduced, alongside the control of CVRFs. Indeed, although to date no standardized treatment of CMVD exists, the current recommendations are to control factors that promote inflammation and thrombosis (such as statin, aspirin, and betablockers) as well as vasomotor dysfunction (such as nitrates) . CMVD is present in about two-thirds of women with angina and non-obstructive CAD and contributes to adverse cardiovascular outcomes in women . Therefore, this diagnosis should always be evoked in women with persisting angina, even in the absence of significant epicardial coronary stenosis. Nuclear imaging techniques, especially PET-MPI, are of paramount importance to establish the diagnosis of CMVD. Abbreviations : 13 N-NH 3 : 13 N-ammonia ; CACS : coronary artery calcium score; CAD : coronary artery disease; CFR : coronary flow reserve; CMVD : coronary microvascular dysfunction; CT : computed tomography; CVRF : cardiovascular risk factor; ECG : electrocardiogram; MBF : myocardial blood flow; MPI : myocardial perfusion imaging; PET: positron emission tomography	4.324219	0.808105	sec[2]/sec[3]/sec[0]/p[2]	en	0.999997	35974185	https://doi.org/10.1007/s00259-022-05914-6	[ "coronary", "myocardial", "cmvd", "angina", "women", "artery", "microvascular", "polar", "lvef", "this" ]	[ { "code": "BA8Z", "title": "Diseases of coronary artery, unspecified" }, { "code": "BA4Z", "title": "Acute ischaemic heart disease, unspecified" }, { "code": "BA41.Z", "title": "Acute myocardial infarction, unspecified" }, { "code": "BA5Z", "title": "Chronic ischaemic heart disease, unspecified" }, { "code": "LA8C.2", "title": "Congenital coronary arterial fistula" }, { "code": "BD1Z", "title": "Heart failure, unspecified" }, { "code": "BA52.Z", "title": "Coronary atherosclerosis, unspecified site" }, { "code": "LA8Z", "title": "Structural developmental anomaly of heart or great vessels, unspecified" }, { "code": "BA6Z", "title": "Ischaemic heart diseases, unspecified" }, { "code": "BA40.Z", "title": "Angina pectoris, unspecified" } ]	=== ICD-11 CODES FOUND === [BA8Z] Diseases of coronary artery, unspecified Also known as: Diseases of coronary artery, unspecified \| coronary artery insufficiency \| coronary artery heart disease \| CAD - [coronary artery disease] \| coronary artery disorder [BA4Z] Acute ischaemic heart disease, unspecified Also known as: Acute ischaemic heart disease, unspecified \| acute coronary syndrome \| ACS - [acute coronary syndrome] \| Silent myocardial ischaemia \| asymptomatic ischemia [BA41.Z] Acute myocardial infarction, unspecified Also known as: Acute myocardial infarction, unspecified \| Acute myocardial infarction \| cardiac attack \| heart attack \| acute cardiac infarction [BA5Z] Chronic ischaemic heart disease, unspecified Also known as: Chronic ischaemic heart disease, unspecified \| Ischaemic heart disease (chronic) NOS \| coronary ischaemia \| coronary damage NOS \| atheroma of heart [LA8C.2] Congenital coronary arterial fistula Definition: A congenital cardiovascular malformation in which a coronary artery communicates, through an anomalous channel, with a cardiac chamber or with any segment of the systemic or pulmonary circulation. Additional information: this communication may be simple and direct or may be tortuous and dilated. In order of frequency the involved coronary artery is the right, the left and, rarely, both coronary arteries. Occasionally multiple fistulas are present. Also known as: Congenital coronary arterial fistula \| coronary fistula \| congenital arteriovenous coronary fistula \| congenital coronary fistula to pulmonary artery \| Congenital coronary arterial fistula to right ventricle Includes: congenital coronary fistula to pulmonary artery Excludes: anomalous origin of coronary artery from pulmonary arterial tree [BD1Z] Heart failure, unspecified Also known as: Heart failure, unspecified \| myocardial failure \| cardiac decompensation \| cardiac failure \| cardiac failure NOS [BA52.Z] Coronary atherosclerosis, unspecified site Also known as: Coronary atherosclerosis, unspecified site \| Coronary atherosclerosis \| cardiac sclerosis \| coronary artery atherosclerosis \| coronary artery sclerosis [LA8Z] Structural developmental anomaly of heart or great vessels, unspecified Also known as: Structural developmental anomaly of heart or great vessels, unspecified \| Heart malformations \| Cardiac malformations \| congenital anomaly of heart \| congenital heart disease [BA6Z] Ischaemic heart diseases, unspecified Also known as: Ischaemic heart diseases, unspecified \| Ischaemic heart disease NOS \| cardiac ischaemia, NOS \| IHD - [ischaemic heart disease], NOS \| cardiac ischemia [BA40.Z] Angina pectoris, unspecified Also known as: Angina pectoris, unspecified \| Angina pectoris \| angor pectoris \| Anginal syndrome \| Ischaemic chest pain === GRAPH WALKS === --- Walk 1 --- [BA8Z] Diseases of coronary artery, unspecified --PARENT--> [?] Diseases of coronary artery Def: Conditions affecting the blood perfusion of the heart.... --RELATED_TO--> [?] Cardiac transplant associated coronary allograft vasculopathy Def: Coronary artery initimal proliferation following cardiac transplantation, defined based on a combination of visual angiographic vessel descriptors in concert with measures of cardiac allograft functio... --- Walk 2 --- [BA8Z] Diseases of coronary artery, unspecified --PARENT--> [?] Diseases of coronary artery Def: Conditions affecting the blood perfusion of the heart.... --RELATED_TO--> [?] Cardiac transplant associated coronary allograft vasculopathy Def: Coronary artery initimal proliferation following cardiac transplantation, defined based on a combination of visual angiographic vessel descriptors in concert with measures of cardiac allograft functio... --- Walk 3 --- [BA4Z] Acute ischaemic heart disease, unspecified --PARENT--> [?] Acute ischaemic heart disease --CHILD--> [BA42] Subsequent myocardial infarction Def: Infarction of any myocardial site, occurring within 4 weeks (28 days) from onset of a previous infarction... --- Walk 4 --- [BA4Z] Acute ischaemic heart disease, unspecified --PARENT--> [?] Acute ischaemic heart disease --PARENT--> [?] Ischaemic heart diseases --- Walk 5 --- [BA41.Z] Acute myocardial infarction, unspecified --PARENT--> [BA41] Acute myocardial infarction Def: The term acute myocardial infarction (MI) should be used when there is evidence of myocardial necrosis in a clinical setting consistent with acute myocardial ischemia. Under these conditions any one o... --EXCLUDES--> [?] Dressler syndrome Def: A condition of postmyocardial infarction (1 to 8 weeks), characterised by a set of associated symptom, including malaise, fever, pericardial discomfort, leukocytosis, an elevated sedimentation rate, a... --- Walk 6 --- [BA41.Z] Acute myocardial infarction, unspecified --PARENT--> [BA41] Acute myocardial infarction Def: The term acute myocardial infarction (MI) should be used when there is evidence of myocardial necrosis in a clinical setting consistent with acute myocardial ischemia. Under these conditions any one o... --EXCLUDES--> [?] Dressler syndrome Def: A condition of postmyocardial infarction (1 to 8 weeks), characterised by a set of associated symptom, including malaise, fever, pericardial discomfort, leukocytosis, an elevated sedimentation rate, a...	[ "[BA8Z] Diseases of coronary artery, unspecified\n --PARENT--> [?] Diseases of coronary artery\n Def: Conditions affecting the blood perfusion of the heart....\n --RELATED_TO--> [?] Cardiac transplant associated coronary allograft vasculopathy\n Def: Coronary artery initimal proliferation following cardiac transplantation, defined based on a combination of visual angiographic vessel descriptors in concert with measures of cardiac allograft functio...", "[BA8Z] Diseases of coronary artery, unspecified\n --PARENT--> [?] Diseases of coronary artery\n Def: Conditions affecting the blood perfusion of the heart....\n --RELATED_TO--> [?] Cardiac transplant associated coronary allograft vasculopathy\n Def: Coronary artery initimal proliferation following cardiac transplantation, defined based on a combination of visual angiographic vessel descriptors in concert with measures of cardiac allograft functio...", "[BA4Z] Acute ischaemic heart disease, unspecified\n --PARENT--> [?] Acute ischaemic heart disease\n --CHILD--> [BA42] Subsequent myocardial infarction\n Def: Infarction of any myocardial site, occurring within 4 weeks (28 days) from onset of a previous infarction...", "[BA4Z] Acute ischaemic heart disease, unspecified\n --PARENT--> [?] Acute ischaemic heart disease\n --PARENT--> [?] Ischaemic heart diseases", "[BA41.Z] Acute myocardial infarction, unspecified\n --PARENT--> [BA41] Acute myocardial infarction\n Def: The term acute myocardial infarction (MI) should be used when there is evidence of myocardial necrosis in a clinical setting consistent with acute myocardial ischemia. Under these conditions any one o...\n --EXCLUDES--> [?] Dressler syndrome\n Def: A condition of postmyocardial infarction (1 to 8 weeks), characterised by a set of associated symptom, including malaise, fever, pericardial discomfort, leukocytosis, an elevated sedimentation rate, a...", "[BA41.Z] Acute myocardial infarction, unspecified\n --PARENT--> [BA41] Acute myocardial infarction\n Def: The term acute myocardial infarction (MI) should be used when there is evidence of myocardial necrosis in a clinical setting consistent with acute myocardial ischemia. Under these conditions any one o...\n --EXCLUDES--> [?] Dressler syndrome\n Def: A condition of postmyocardial infarction (1 to 8 weeks), characterised by a set of associated symptom, including malaise, fever, pericardial discomfort, leukocytosis, an elevated sedimentation rate, a..." ]	BA8Z	Diseases of coronary artery, unspecified	[ { "from_icd11": "BA4Z", "icd10_code": "I248", "icd10_title": "Other forms of acute ischemic heart disease" }, { "from_icd11": "BA4Z", "icd10_code": "I256", "icd10_title": "Silent myocardial ischemia" }, { "from_icd11": "BA4Z", "icd10_code": "I249", "icd10_title": "Acute ischemic heart disease, unspecified" }, { "from_icd11": "BA4Z", "icd10_code": "I24", "icd10_title": "Other acute ischemic heart diseases" }, { "from_icd11": "BA41.Z", "icd10_code": "I21A1", "icd10_title": "Myocardial infarction type 2" }, { "from_icd11": "BA41.Z", "icd10_code": "I21A9", "icd10_title": "Other myocardial infarction type" }, { "from_icd11": "BA41.Z", "icd10_code": "I2109", "icd10_title": "ST elevation (STEMI) myocardial infarction involving other coronary artery of anterior wall" }, { "from_icd11": "BA41.Z", "icd10_code": "I2119", "icd10_title": "ST elevation (STEMI) myocardial infarction involving other coronary artery of inferior wall" }, { "from_icd11": "BA41.Z", "icd10_code": "I2111", "icd10_title": "ST elevation (STEMI) myocardial infarction involving right coronary artery" }, { "from_icd11": "BA41.Z", "icd10_code": "I2102", "icd10_title": "ST elevation (STEMI) myocardial infarction involving left anterior descending coronary artery" }, { "from_icd11": "BA41.Z", "icd10_code": "I2129", "icd10_title": "ST elevation (STEMI) myocardial infarction involving other sites" }, { "from_icd11": "BA41.Z", "icd10_code": "I2121", "icd10_title": "ST elevation (STEMI) myocardial infarction involving left circumflex coronary artery" }, { "from_icd11": "BA41.Z", "icd10_code": "I2101", "icd10_title": "ST elevation (STEMI) myocardial infarction involving left main coronary artery" }, { "from_icd11": "BA41.Z", "icd10_code": "I214", "icd10_title": "Non-ST elevation (NSTEMI) myocardial infarction" }, { "from_icd11": "BA41.Z", "icd10_code": "I213", "icd10_title": "ST elevation (STEMI) myocardial infarction of unspecified site" } ]	I248	Other forms of acute ischemic heart disease
A 40 years old man, was injured in a car accident as a front passenger, occurred during a frontal collision with another vehicle passing illegally in continuous white line down the center of a national road, the impact speed was 100 km per hour. Unfortunately, the patient was not restrained by seatbelt and sustained lower extremity and head impacts without loss of consciousness, he experienced a severe pain in his hips. On presentation to the emergency department, patient was hemodynamically stable and conscious, examination of the chest and abdomen was normal. However, both hips were deformed in flexion, internal rotation and adduction , without neurovascular deficit or skin lesions of the lower limbs. Radiographs displayed bilateral posterior hip dislocation combined with right femoral head fracture, and small posterior wall fracture of the left acetabulum. Prompt hips reduction was performed within one hour of presentation through closed manipulation below general anesthesia, curarisation and controlled by fluoroscopy, patient was maintained in supine position on the ground and the aide applied a hand pression on the iliac wings of the pelvis, while the operator exerted a traction on the leg then flexed the hip with adduction and external rotation, the same technique was applied successfully to the second hip. The Left hip was stable up to 100° flexion and 45° internal and external rotation after reduction. However, the right hip was unstable, for this reason, we have positioned a posterior knee splint for temporary stabilization. Reduction of each hip was checked by antero-posterior pelvic radiograph and CT-scan . X-ray showed an irreducible right femoral head fracture, but in the other side it showed a concentric reduction of the left hip joint. CT scan showed in the right side a one-third of femoral head suprafoveal fracture Pipkin type II that was anterior, rotated and incarcerated associated with two small fragments; one was superior and the second inferior. In the left side we have detected a minor posterior wall fracture of the left acetabulum without any intra-articular fragments. Surgical treatment was planned next day to accomplish anatomic open reduction of the right femoral head fracture and its internal fixation (ORIF) using a modified Hardinge approach that was chosen because the Pipkin II fragment was switched, therefore its reduction need surgical dislocation of the hip and a wide access to the femoral head. Moreover, this approach is known to have less risk of sciatic nerve injury, preserve the pelvitrochanteric muscles and have less risk of limping in comparison with the conventional Hardinge approach, because we spread the fibers of the gluteus medius just at its anterior middle one-third, preserving a great portion of this muscle insertions and function . In the operational room, under general anesthesia the patient was placed in the full lateral position with pubic and sacral support, left leg was maintained in extension to stabilize ipsilateral hip, then a modified external approach of Hardinge was performed exposing the fascia lata that was incised in line with skin incision and retracted anteriorly while the gluteus maximus was retracted posteriorly to expose the common tendon of vastus lateralis and the gluteus medius, it was split longitudinally at the anterior third and sharply separated from the greater trochanter. Without extension more than 3 cm proximally to the insertion of the great trochanter to avoid inferior branch of the superior gluteal nerve injury. An anterior flap was formed by the anterior portion of the gluteus medius, the underlying gluteus minimus, and the anterior portion of the vastus lateralis. A T-shaped capsulotomy was performed to release the anterior capsule. The hip joint was then dislocated, and the femoral head fracture was exposed, we have explored a rotated pipkin II fragment and extracted two small parts that had footprints in the not weight bearing area in the distal part of the femoral head . After cleaning and washing hip joint, we have reduced the Pipkin fragment and fixed them by two small pins that was over-drilled with a cannulated drill bit, followed by osteosynthesis with two Herbert screws, then hip was reduced and the flaps was repaired by transosseuses sutures to the great trochanter layer by layer using a vicryl number 2. Postoperative examination revealed no neurological deficit and the postoperative imaging of the pelvis showed an anatomic reduction of the femoral head fracture and a good position of the two Herbert screws . The patient was kept on bed rest for three weeks, followed by a further six weeks of right non-weight bearing. At 6 months there was a good fracture healing, the patient had no limited hip motion, without limping and normal return to his daily activity . Then at one and two years follow up there was no signs of osteoarthritis or avascular necrosis detected on the pelvic X-rays. However, a right hip non-bridging Brooker type I heterotopic ossification was noticed . Fig. 1 Clinical photograph of the patient, with hip flexion and internal rotation. Fig. 1 Fig. 2 Anteroposterior radiograph of the pelvis showing bilateral posterior hip dislocation with right Pipkin type II fracture. Fig. 2 Fig. 3 Immediate pelvic X-ray showing a non-reduction of right hip because of a non-reduced Pipkin II fragment (blue arrow) with a small superior fragment (red arrow). Fig. 3 Fig. 4 3D CT-scan (a) with transversal view (b) showing a non-anatomic reduction of the right hip because of incarcerated and rotated femoral head fragment (blue arrow) and presence of two small fragments one superior (red arrow) and the second inferior (green arrow), and small posterior wall fracture of left acetabulum (yellow star). Fig. 4 Fig. 5 Hardinge approach to the right hip allowing osteosynthesis of Pipkin’s fracture with two Herbert screws. Fig. 5 Fig. 6 pelvic post-operative X-ray showing the Herbert screws fixation and good anatomic hip reduction. Fig. 6 Fig. 7 clinical examination of lower limbs and the skin incision of modified Hardinge approach. Fig. 7 Fig. 8 two years pelvic post-operative X-ray showing absence of osteoarthritis or avascular necrosis signs, but a right hip non-bridging Brooker type I heterotopic ossification was noticed. Fig. 8	4.007813	0.971191	sec[1]/p[0]	en	0.999999	31352316	https://doi.org/10.1016/j.ijscr.2019.07.017	[ "fracture", "head", "reduction", "femoral", "small", "pipkin", "approach", "fragment", "without", "pelvic" ]	[ { "code": "ND56.2", "title": "Fracture of unspecified body region" }, { "code": "ND32", "title": "Fractures involving multiple body regions" }, { "code": "NB52.Z", "title": "Fracture of lumbar spine or pelvis, unspecified" }, { "code": "FB80.B", "title": "Pathological fracture" }, { "code": "FB80.Y", "title": "Other specified disorders of bone density or structure" }, { "code": "NA63", "title": "Traumatic amputation at neck level" }, { "code": "MB4D", "title": "Headache, not elsewhere classified" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "MB48.3", "title": "Light-headedness" }, { "code": "FB3Z", "title": "Disorders of muscles, unspecified" } ]	=== ICD-11 CODES FOUND === [ND56.2] Fracture of unspecified body region Also known as: Fracture of unspecified body region \| avulsion fracture of unspecified body site \| comminuted fracture of unspecified body site \| compression fracture of unspecified body site \| fracture dislocation of unspecified body site Excludes: multiple fractures NOS [ND32] Fractures involving multiple body regions Also known as: Fractures involving multiple body regions \| multiple skeletal fractures \| multiple fractures \| multiple compression fractures \| fracture of multiple bone sites [NB52.Z] Fracture of lumbar spine or pelvis, unspecified Also known as: Fracture of lumbar spine or pelvis, unspecified \| Fracture of lumbar spine or pelvis \| Fracture of pelvis, not elsewhere classified \| fracture pelvis NOS \| pelvic fracture [FB80.B] Pathological fracture Also known as: Pathological fracture \| pathological bone fracture \| Pathological fracture NOS \| spontaneous fracture \| spontaneous fracture with dislocation Excludes: Collapsed vertebra, not elsewhere classified [FB80.Y] Other specified disorders of bone density or structure Also known as: Other specified disorders of bone density or structure \| Bone dysplasia \| Inherited bone dysplasia \| Acquired bone dysplasia \| Drug-induced bone dysplasia [NA63] Traumatic amputation at neck level Also known as: Traumatic amputation at neck level \| complete head avulsion \| Decapitation \| decapitation of head at neck level \| severed head Includes: Decapitation [MB4D] Headache, not elsewhere classified Definition: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above. Also known as: Headache, not elsewhere classified \| cephalalgia \| cephalgia \| cephalodynia \| pain in head NOS Excludes: Trigeminal neuralgia \| Atypical facial pain \| Acute headache, not elsewhere classified [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [MB48.3] Light-headedness Also known as: Light-headedness \| light headed [FB3Z] Disorders of muscles, unspecified Also known as: Disorders of muscles, unspecified \| disorder of muscle, unspecified \| muscle disease \| muscular disease \| muscular disorder === GRAPH WALKS === --- Walk 1 --- [ND56.2] Fracture of unspecified body region --PARENT--> [ND56] Injury of unspecified body region Def: Damage inflicted on the body in an unspecified area as the direct or indirect result of an external force, with or without disruption of structural continuity.... --EXCLUDES--> [?] Injuries involving multiple body regions --- Walk 2 --- [ND56.2] Fracture of unspecified body region --EXCLUDES--> [?] Fractures involving multiple body regions --CHILD--> [?] Fractures involving multiple regions of one arm --- Walk 3 --- [ND32] Fractures involving multiple body regions --RELATED_TO--> [?] Fractures involving multiple body regions due to birth injury Def: A condition characterised by the presence of skeletal fractures in more than one body region due to physical pressure or injury during delivery.... --PARENT--> [?] Birth injury to skeleton Def: A condition characterised by the presence of damage to the skeleton due to physical pressure or injury during delivery.... --- Walk 4 --- [ND32] Fractures involving multiple body regions --RELATED_TO--> [?] Fractures involving multiple body regions due to birth injury Def: A condition characterised by the presence of skeletal fractures in more than one body region due to physical pressure or injury during delivery.... --PARENT--> [?] Fractures involving multiple body regions --- Walk 5 --- [NB52.Z] Fracture of lumbar spine or pelvis, unspecified --PARENT--> [NB52] Fracture of lumbar spine or pelvis Def: Broken bone in the lumbar spine or pelvis.... --CHILD--> [NB52.2] Fracture of the pelvic ring with incomplete disruption of posterior arch --- Walk 6 --- [NB52.Z] Fracture of lumbar spine or pelvis, unspecified --PARENT--> [NB52] Fracture of lumbar spine or pelvis Def: Broken bone in the lumbar spine or pelvis.... --CHILD--> [NB52.0] Fracture of lumbar vertebra	[ "[ND56.2] Fracture of unspecified body region\n --PARENT--> [ND56] Injury of unspecified body region\n Def: Damage inflicted on the body in an unspecified area as the direct or indirect result of an external force, with or without disruption of structural continuity....\n --EXCLUDES--> [?] Injuries involving multiple body regions", "[ND56.2] Fracture of unspecified body region\n --EXCLUDES--> [?] Fractures involving multiple body regions\n --CHILD--> [?] Fractures involving multiple regions of one arm", "[ND32] Fractures involving multiple body regions\n --RELATED_TO--> [?] Fractures involving multiple body regions due to birth injury\n Def: A condition characterised by the presence of skeletal fractures in more than one body region due to physical pressure or injury during delivery....\n --PARENT--> [?] Birth injury to skeleton\n Def: A condition characterised by the presence of damage to the skeleton due to physical pressure or injury during delivery....", "[ND32] Fractures involving multiple body regions\n --RELATED_TO--> [?] Fractures involving multiple body regions due to birth injury\n Def: A condition characterised by the presence of skeletal fractures in more than one body region due to physical pressure or injury during delivery....\n --PARENT--> [?] Fractures involving multiple body regions", "[NB52.Z] Fracture of lumbar spine or pelvis, unspecified\n --PARENT--> [NB52] Fracture of lumbar spine or pelvis\n Def: Broken bone in the lumbar spine or pelvis....\n --CHILD--> [NB52.2] Fracture of the pelvic ring with incomplete disruption of posterior arch", "[NB52.Z] Fracture of lumbar spine or pelvis, unspecified\n --PARENT--> [NB52] Fracture of lumbar spine or pelvis\n Def: Broken bone in the lumbar spine or pelvis....\n --CHILD--> [NB52.0] Fracture of lumbar vertebra" ]	ND56.2	Fracture of unspecified body region	[ { "from_icd11": "ND56.2", "icd10_code": "T142", "icd10_title": "" }, { "from_icd11": "ND32", "icd10_code": "T02", "icd10_title": "" }, { "from_icd11": "ND32", "icd10_code": "T020", "icd10_title": "" }, { "from_icd11": "ND32", "icd10_code": "T021", "icd10_title": "" }, { "from_icd11": "ND32", "icd10_code": "T022", "icd10_title": "" }, { "from_icd11": "ND32", "icd10_code": "T023", "icd10_title": "" }, { "from_icd11": "ND32", "icd10_code": "T024", "icd10_title": "" }, { "from_icd11": "ND32", "icd10_code": "T025", "icd10_title": "" }, { "from_icd11": "ND32", "icd10_code": "T026", "icd10_title": "" }, { "from_icd11": "ND32", "icd10_code": "T027", "icd10_title": "" }, { "from_icd11": "ND32", "icd10_code": "T028", "icd10_title": "" }, { "from_icd11": "ND32", "icd10_code": "T029", "icd10_title": "" }, { "from_icd11": "NB52.Z", "icd10_code": "S329XXD", "icd10_title": "Fracture of unspecified parts of lumbosacral spine and pelvis, subsequent encounter for fracture with routine healing" }, { "from_icd11": "NB52.Z", "icd10_code": "S32601A", "icd10_title": "Unspecified fracture of right ischium, initial encounter for closed fracture" }, { "from_icd11": "NB52.Z", "icd10_code": "S329XXG", "icd10_title": "Fracture of unspecified parts of lumbosacral spine and pelvis, subsequent encounter for fracture with delayed healing" } ]	T142
The patient was then sent to our hospital’s emergency department. Figure 4 shows the results of the ECG examination on admission: elevated ST-segment returning to baseline; Q-wave deepening in lead III; Q-wave forming in lead IVF; and inverted T-waves in leads V3, V4, V5, and V6. The patient’s medical history included 3 years of paroxysmal hypertension (maximum blood pressure of 230/146 mm Hg) and 10 months of undiagnosed hyperglycemia. Assessment of his vital signs revealed a temperature of 36.5°C, heart rate of 94 beats/minute, respiratory rate of 20 breaths/minute, and blood pressure of 100/64 mmHg. Physical examination revealed left lower abdominal pain, cyanosis of the lips, low breath tones in both lungs, and a slight amount of moist rales. Figure 5 shows the emergency plain and enhanced abdominal computed tomography scans, which revealed masses and mixed low-density shadows on both sides of the abdominal aorta. The larger mass was located on the left side and measured 4.01×3.94 cm, had visibly thin walls and partitions, and had a computed tomography value of approximately 14 HU. The enhanced scan revealed annular enhancement of this mass, which was classified as PGL. Emergency coronary angiography, shown in Fig. 6 , revealed the following: Left main (LM) had no obvious stenosis; proximal and middle segment of left anterior descending (LAD) artery and left circumflex (LCX) artery had diffuse ectasia with rough endangium, forward blood flow grade TIMI 2, conformed to Markis type I diagnosis standard . The obtuse marginal (OM) artery had irregular stenosis and forward blood flow grade TIMI 2. The patient ultimately received thrombolysis agents, antiplatelet therapy, atorvastatin, and myocardial protection agents. His glycosylated hemoglobin A1c concentration was 10.3%, and an oral glucose tolerance test revealed that his plasma glucose level was 15.0 mmol/L after 2 hours (Table 3 ). As a result, we were able to determine that he had diabetes mellitus, though it was likely secondary. The results of the laboratory tests for PGL are shown in Table 4 . The patient’s free normetanephrine level was significantly elevated in both plasma and 24-hour urine, his normetanephrine + metanephrine level was elevated in plasma, and his 3-methoxytyramine and vanillylmandelic acid levels were elevated in urine. PGL was diagnosed and treated by phenoxybenzamine; surgery was recommended, but the patient declined. Table 5 shows the results of myocardial enzymology performed while the patient was hospitalized. The patient’s health gradually but clearly improved. His systolic blood pressure ranged from 104 to 136 mmHg, and his diastolic blood pressure fluctuated between 66 and 82 mmHg during the 6-month post-discharge follow-up. His fasting plasma glucose level varied from 4.2 to 5.8 mmol/L, and another oral glucose tolerance test showed that his plasma glucose level after 2 hours was <11.0 mmol/L. Another physical examination revealed no cyanosis of the lips, normal breath tones in both lungs, no moist rales, no discomfort in the left lower abdomen, and no other bothersome symptoms, all indicating a good overall clinical condition. Another echocardiography examination after 6 months revealed normal systolic motion of the apex, inferior wall, left ventricular anterior wall, and interventricular septum; a left ventricular end-diastolic diameter of 50 mm; and a left ventricular ejection fraction of 66%. Therefore, according to the European guidelines , the patient had no red flags of acute infectious myocarditis, and was retrospectively diagnosed with TTS combined with the echocardiographical findings at the cardiovascular hospital and the after 6 months of follow-up. Finally, we were able to validate the efficacy of phenoxybenzamine for the PGL; thrombolysis, antiplatelet therapy, and lipid modulation for the CAE; and myocardial protection for the TTS. Consent for all treatments was obtained from the patient. He was ultimately diagnosed with TTS produced by PGL combined with Markis type I CAE. Fig. 4 Ectrocardiography at Emergency Department of our hospital elevated ST- segment returning to baseline, Q-wave deepening in lead III, Q-wave forming in lead aVF, and inverted T-waves in leads V3, V4, V5, and V6 Fig. 5 A and B Abdominal computer tomography plain and enhanced scan. Masses and mixed low-density shadow on both sides of the abdominal aorta, and marked with a red arrow. The larger mass was located on the left side and measured 4.01×3.94cm; it was visible thin walls and partitions; the CT value was approximately 14HU; and the enhanced scan revealed annular enhancement. CT: computer tomography Fig. 6 A and B Coronary angiography on the fourth day of hospitalization. Left main (LM) had no obvious stenosis; Proximal and middle segment of left anterior descending (LAD) artery and left circumflex (LCX) artery had diffuse ectasia with rough endangium, forward blood flow grade TIMI 2, conformed to Markis type I diagnosis standard; The obtuse marginal (OM) artery had irregular stenosis and forward blood flow grade TIMI 2 Table 3 Results of the OGTT Time(h) Fasting 1h 2h 3h GLU ( mmol/L) 5.2 10.2 15.0 16.5 OGTT Oral Glucose Tolerance Test, GLU Glucose Table 4 Laboratory examination of paraganglioma Samples Plasma (Reference range) Urine (Reference range) Free NMN >1000 ↑ (<145pg/ml) 3070.7 ↑ (125-510ug/24h) Free MN 13.1 (<62pg/ml) 85.4 (62-270ug/24h) NMN+MN >1013.1 ↑ (<207pg/ml) Free E / 5.3 Free NE / 733.6 Free DA / 545 3-MT / 320.3 ↑ (≤306ug/24h) VMA / 12mg ↑ (0-7.5mg/24h) HVA / 5.9 (0-10mg/24h) Urine volume in 24 hours / 2660mL NMN Normetanephrine, MN Metanephrine, E Epinephrine, NE Norepinephrine; DA : Dopamine, 3-MT 3-methoxytyramine, VMA Vanillylmandelice acid, HVA Homovanillic acid. Above normal range of indices were indicated by an arrow Table 5 Results of myocardial enzymology examination during hospitalization CK (50-310)U/L CK-MB <24U/L Myo <70ng/ml cTNI <0.023ng/ml NT-proBNP <450ng/L D-dimer (0-1)mg/L ED / 43 63 1.8 1320 2.16 January 16 347 34 31 1.5 2060 1.5 January 17 131 23 45 0.55 1100 0.6 January 18 65 13 44 0.23 612 / January 20 37 18 21 / / 0.7 CK CreatineKinase, CK-MB CreatineKinase-MB, Myo Myohemoglobin, cTNI Cardiac troponin-I, NT-proBNP N-terminal pro-B-type natriuretic peptide, ED Emergency department	4.050781	0.96875	sec[1]/p[2]	en	0.999996	PMC10625213	https://doi.org/10.1186/s12872-023-03577-1	[ "blood", "glucose", "artery", "plasma", "free", "emergency", "abdominal", "both", "segment", "wave" ]	[ { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" }, { "code": "MA12.1", "title": "Finding of cocaine in blood" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "MA12.2", "title": "Finding of hallucinogen in blood" }, { "code": "5A40.Z", "title": "Intermediate hyperglycaemia, unspecified" }, { "code": "5C61.Y", "title": "Other specified disorders of carbohydrate absorption or transport" }, { "code": "5A40.1", "title": "Impaired glucose tolerance" }, { "code": "5A40.0", "title": "Impaired fasting glucose" }, { "code": "5C61.5", "title": "Disorders of facilitated glucose transport" } ]	=== ICD-11 CODES FOUND === [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS [MA12.1] Finding of cocaine in blood Also known as: Finding of cocaine in blood \| cocaine in blood [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [MA12.2] Finding of hallucinogen in blood Also known as: Finding of hallucinogen in blood \| hallucinogen in blood [5A40.Z] Intermediate hyperglycaemia, unspecified Also known as: Intermediate hyperglycaemia, unspecified \| Intermediate hyperglycaemia \| Impaired glucose regulation \| prediabetes \| latent diabetes [5C61.Y] Other specified disorders of carbohydrate absorption or transport Also known as: Other specified disorders of carbohydrate absorption or transport \| Other disorders of intestinal carbohydrate absorption \| Glucose malabsorption \| Isomaltose malabsorption \| Sucrose malabsorption [5A40.1] Impaired glucose tolerance Definition: Impaired glucose tolerance (IGT) is a metabolic disorder, which is characterised by 2-h postload glucose 140–199 mg/dl (7.8–11.1 mmol/l). Also known as: Impaired glucose tolerance \| IGT - [impaired glucose tolerance] \| Impaired glucose tolerance with unspecified complication \| Impaired glucose tolerance without complication \| abnormal glucose tolerance [5A40.0] Impaired fasting glucose Definition: Impaired fasting tolerance is a metabolic disorder with Fasting Plasma Glucose (FPG) 110–125 mg/dl (6.1–6.9 mmol/l). Also known as: Impaired fasting glucose \| IFG - [impaired fasting glucose] \| impaired fasting glycaemia \| elevated fasting glucose \| high fasting blood sugar [5C61.5] Disorders of facilitated glucose transport Also known as: Disorders of facilitated glucose transport \| Encephalopathy due to GLUT1 deficiency \| Glucose transporter defect, blood-brain barrier \| Facilitated glucose transporter protein type 1 deficiency \| Familial renal glucosuria === GRAPH WALKS === --- Walk 1 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --EXCLUDES--> [?] Diseases of the immune system --- Walk 2 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --RELATED_TO--> [?] Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system --- Walk 3 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --EXCLUDES--> [?] Recurrent or persistent glomerular haematuria Def: The presence in the urine of glomerular origin red blood cells (RBCs). In microscopic haematuria the urine appears normal to the naked eye, but examination with a microscope shows a high number of RBC... --- Walk 4 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --PARENT--> [MF50] Abnormal micturition --- Walk 5 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.0] Finding of opiate drug in blood --- Walk 6 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --PARENT--> [?] Clinical findings in blood, blood-forming organs, or the immune system	[ "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --EXCLUDES--> [?] Diseases of the immune system", "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --RELATED_TO--> [?] Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --EXCLUDES--> [?] Recurrent or persistent glomerular haematuria\n Def: The presence in the urine of glomerular origin red blood cells (RBCs). In microscopic haematuria the urine appears normal to the naked eye, but examination with a microscope shows a high number of RBC...", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --PARENT--> [MF50] Abnormal micturition", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.0] Finding of opiate drug in blood", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --PARENT--> [?] Clinical findings in blood, blood-forming organs, or the immune system" ]	3C0Z	Diseases of the blood or blood-forming organs, unspecified	[ { "from_icd11": "3C0Z", "icd10_code": "D75A", "icd10_title": "Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia" }, { "from_icd11": "3C0Z", "icd10_code": "D7581", "icd10_title": "Myelofibrosis" }, { "from_icd11": "3C0Z", "icd10_code": "D7582", "icd10_title": "Heparin induced thrombocytopenia (HIT)" }, { "from_icd11": "3C0Z", "icd10_code": "D7589", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D759", "icd10_title": "Disease of blood and blood-forming organs, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "D763", "icd10_title": "Other histiocytosis syndromes" }, { "from_icd11": "3C0Z", "icd10_code": "Q899", "icd10_title": "Congenital malformation, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "III", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D70-D77", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D75", "icd10_title": "Other and unspecified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D77", "icd10_title": "Other disorders of blood and blood-forming organs in diseases classified elsewhere" }, { "from_icd11": "3C0Z", "icd10_code": "D758", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D76", "icd10_title": "Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue" }, { "from_icd11": "MF50.4Z", "icd10_code": "R310", "icd10_title": "Gross hematuria" }, { "from_icd11": "MF50.4Z", "icd10_code": "R312", "icd10_title": "Other microscopic hematuria" } ]	D75A	Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia
Patient, male, 65 years old, was admitted to our hospital on 5 May 2022 with a chief complaint of “pain in the right maxillary sinus”. Physical examination on admission: slight swelling on the right side of the nasal cheek, no redness or swelling of the skin, palpable subcutaneous hard mass, no mobility, slight tenderness to pressure, chronic nasal congestion on both sides, no evidence of neoplasm or foreign body in the nasal cavity, slightly deviated nasal septum, bilateral hypertrophy of the inferior turbinate. Enhanced computed tomography (CT) scan of the paranasal sinuses showed a mild soft tissue density shadow in the anterior wall of the right maxillary sinus with adjacent bone destruction and resorption, mild deviation of the nasal septum, and bilateral hypertrophy of the nasal turbinate . An extended chest CT scan showed: 1. Multiple small nodules in both lungs, suggestive of pneumoconiosis or infectious lesions; 2. Enlarged mediastinal lymph nodes and mild bilateral pleural thickening. On 7 May 2022, the patient underwent endoscopic-assisted excision of the mass in the right maxillary sinus. Post-surgical pathology revealed an infiltrative growth pattern of the tumor, with tumor cells arranged in glandular and papillary structures, partially organized in small nests, and individual tumor cell infiltration observed in the fibrous stroma. The glands were composed of single-layer ciliated columnar cells. The tumor cells were oval or short spindle-shaped, with an increased nuclear-cytoplasmic ratio, eosinophilic cytoplasm, and significant nuclear atypia, manifesting as irregular nuclear shapes, dark staining, vesicular nuclei, prominent nucleoli in some cells, and visible mitotic figures. No significant necrosis was observed. Fibrous tissue hyperplasia with a small amount of lymphocytic infiltration was present in the tumor stroma, and the tumor was seen invading the surrounding normal bone tissue. Cancer emboli were detected within the blood vessels. Immunohistochemical staining showed CK7 (+), CK20 ( – ), CDX2 ( – ), SATB2 ( – ), MUC2 ( – ), MLH1 ( +), MSH2 ( +), MSH6 (+), PMS2 (+), Ki67 (+ , 20–30%). Based on the morphological and immunohistochemical findings, the diagnosis of high-grade non-intestinal-type adenocarcinoma was established . One month after surgery, a follow-up magnetic resonance imaging (MRI) of the head showed a local absence of the anterior wall of the right maxillary sinus without abnormal enhancement after contrast administration . Based on the auxiliary examinations, radiotherapy was administered to the tumor bed and lymphatic drainage area 1 month after surgery. During the course of radiotherapy , the patient developed back pain. Subsequent bone scan showed osteolytic bone destruction and abnormal bone metabolism in the right scapula, T8 vertebra and left skeletal bone, suggesting bone metastasis. Palliative radiotherapy was administered to the bone metastatic lesions with a prescription dose of 30 GY/10f. After radiotherapy, the pain improved significantly and six cycles of immunocombination chemotherapy were administered from 2022-10-11 to 2023-02-06, the specific regimen was carilizumab + docetaxel + cisplatin. Stable disease (SD) after five cycles of chemotherapy combined with immunotherapy post assessment, and then continued with carilizumab immuno-maintenance therapy. Review of head and chest CT : 1. Localized increased bone density in the anterior wall of the right superior collateral sinus. 2. Scattered multinodular nodular shadows in both lungs along the lung texture, considering lung metastases, which were increased and enlarged compared with the previous ones. 3. Bone destruction was seen in the thoracic 8 and 11 vertebrae and accessories, and the splenic bone of the right shoulder, with increased bone density in the left division of the cervical 5 vertebrae, considering metastasis, and the cervical 5 vertebrae was the new lesion. In combination with the adjuvant examination, progressive disease (PD) was considered. Genetic testing showed EGFR and TP53 mutations, and gefitinib targeted therapy was started on 2023-06-01. 2023-08-11 Repeat Head and Chest CT: 1. Localized increased bone density in the anterior wall of the right frontal sinus, unchanged compared to previous. 2. Multiple scattered spots and nodules of varying sizes in both lungs, suggestive of lung metastases. Decreased in number and size compared to previous images. 3. Increased bone density in multiple vertebrae and the right shoulder blade and spleen bone, suggestive of metastases. 2023-11-21 Repeat CT: Multiple small nodular opacities in both lungs, similar to previous findings. The patient is still undergoing oral treatment with gefitinib targeted therapy . Fig. 1 Imaging findings: A CT scan of the paranasal sinuses shows a slight soft tissue density shadow in the anterior wall area of the right maxillary sinus with adjacent bone destruction and resorption. The nasal septum is slightly deviated to the right and both nasal turbinates are enlarged. B MRI scan of the head shows: 1. Local absence of the anterior wall of the right maxillary sinus, no abnormal enhancement seen after contrast administration Fig. 2 Pathological findings: The tumor demonstrates an infiltrative growth pattern, with tumor cells arranged in glandular and papillary structures, partially organized in small nests. Individual tumor cells can be seen infiltrating the fibrous stroma ( A HE × 100). The glands are composed of single-layer ciliated columnar cells. The tumor cells are oval or short spindle-shaped, with an increased nuclear-cytoplasmic ratio, eosinophilic cytoplasm, and significant nuclear atypia, manifesting as irregular nuclear shapes, dark staining, vesicular nuclei, prominent nucleoli in some cells, and visible mitotic figures ( B : HE × 200), no significant necrosis is observed. The tumor stroma exhibits fibrous tissue hyperplasia with a small amount of lymphocytic infiltration, and the tumor is seen invading the surrounding normal bone tissue ( C : HE × 200). Intravascular cancer emboli are observed, with CD31 staining indicating endothelial cells ( D : EnVision × 200)。 E :CK7( +), F :CK20(–), G :MLH1 ( +)， H :MSH2( +)， I :MSH6( +), J :PMS2( +), K :Ki67(+ , 20–30%) Fig. 3 Diagnosis and treatment flowchart	4.035156	0.977051	sec[1]/p[0]	en	0.999996	39097562	https://doi.org/10.1007/s00432-024-05744-z	[ "bone", "tumor", "cells", "sinus", "nasal", "maxillary", "both", "tissue", "wall", "small" ]	[ { "code": "FC0Z", "title": "Diseases of the musculoskeletal system or connective tissue, unspecified" }, { "code": "FB84.Z", "title": "Osteomyelitis or osteitis, unspecified" }, { "code": "FB80.Z", "title": "Disorder of bone density or structure, unspecified" }, { "code": "FB86.11", "title": "Hypertrophy of bone" }, { "code": "FB86.1Z", "title": "Bone hyperplasias, unspecified" }, { "code": "2F9Z", "title": "Neoplasms of unknown behaviour of unspecified site" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "2E6Z", "title": "Carcinoma in situ of unspecified site" }, { "code": "2F91.1", "title": "Neoplasms of unknown behaviour of trachea, bronchus or lung" }, { "code": "2F92", "title": "Neoplasms of unknown behaviour of skin" } ]	=== ICD-11 CODES FOUND === [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified Also known as: Diseases of the musculoskeletal system or connective tissue, unspecified \| bone disease NOS \| bone disorder NOS \| bone lesion NOS \| musculoskeletal complications NOS [FB84.Z] Osteomyelitis or osteitis, unspecified Also known as: Osteomyelitis or osteitis, unspecified \| Osteomyelitis or osteitis \| bone inflammation \| bone ulcer \| bone inflammatory disease [FB80.Z] Disorder of bone density or structure, unspecified Also known as: Disorder of bone density or structure, unspecified \| Certain specified disorders of bone density or structure [FB86.11] Hypertrophy of bone Also known as: Hypertrophy of bone \| bone thickening \| periosteum thickening \| Infantile cortical hyperostoses \| Post traumatic subperiosteal ossification [FB86.1Z] Bone hyperplasias, unspecified Also known as: Bone hyperplasias, unspecified \| Bone hyperplasias [2F9Z] Neoplasms of unknown behaviour of unspecified site Also known as: Neoplasms of unknown behaviour of unspecified site \| neoplasia \| neoplasm growth NOS \| tumour of unspecified site \| tumourlet of unspecified site [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [2E6Z] Carcinoma in situ of unspecified site Also known as: Carcinoma in situ of unspecified site \| carcinoma in situ of unspecified site \| carcinoma in situ NOS \| carcinoma in situ \| in situ neoplasm [2F91.1] Neoplasms of unknown behaviour of trachea, bronchus or lung Also known as: Neoplasms of unknown behaviour of trachea, bronchus or lung \| trachea, bronchus or lung tumour NOS \| Intravascular bronchial alveolar tumour of unspecified site \| Bronchial adenoma of unknown behaviour of unspecified site \| Intravascular bronchial alveolar tumour unknown behaviour of unspecified site [2F92] Neoplasms of unknown behaviour of skin Also known as: Neoplasms of unknown behaviour of skin \| skin tumour NOS === GRAPH WALKS === --- Walk 1 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --EXCLUDES--> [?] Endocrine, nutritional or metabolic diseases Def: This chapter includes endocrine diseases, nutritional diseases as well as metabolic diseases.... --- Walk 2 --- [FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue.... --RELATED_TO--> [?] Nonorgan specific systemic autoimmune disorders --- Walk 3 --- [FB84.Z] Osteomyelitis or osteitis, unspecified --PARENT--> [FB84] Osteomyelitis or osteitis --EXCLUDES--> [?] Inflammatory conditions of jaws --- Walk 4 --- [FB84.Z] Osteomyelitis or osteitis, unspecified --PARENT--> [FB84] Osteomyelitis or osteitis --EXCLUDES--> [?] Infection of vertebra Def: A condition of the vertebrae, caused by an infection with a bacterial, viral, fungal, or parasitic source. This condition commonly presents with fever, chills, headache, weight loss, or may be asympto... --- Walk 5 --- [FB80.Z] Disorder of bone density or structure, unspecified --PARENT--> [FB80] Certain specified disorders of bone density or structure --CHILD--> [FB80.0] Fibrous dysplasia of bone Def: Fibrous dysplasia of bone is a congenital non-hereditary benign bone disease, where normal bone is replaced by a fibrous-like tissue with immature osteogenesis. Bone lesions are mono- or polyostotic a... --- Walk 6 --- [FB80.Z] Disorder of bone density or structure, unspecified --PARENT--> [FB80] Certain specified disorders of bone density or structure --CHILD--> [FB80.1] Skeletal fluorosis	[ "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --EXCLUDES--> [?] Endocrine, nutritional or metabolic diseases\n Def: This chapter includes endocrine diseases, nutritional diseases as well as metabolic diseases....", "[FC0Z] Diseases of the musculoskeletal system or connective tissue, unspecified\n --PARENT--> [15] Diseases of the musculoskeletal system or connective tissue\n Def: This chapter contains diseases of musculoskeletal system and diseases of connective tissue....\n --RELATED_TO--> [?] Nonorgan specific systemic autoimmune disorders", "[FB84.Z] Osteomyelitis or osteitis, unspecified\n --PARENT--> [FB84] Osteomyelitis or osteitis\n --EXCLUDES--> [?] Inflammatory conditions of jaws", "[FB84.Z] Osteomyelitis or osteitis, unspecified\n --PARENT--> [FB84] Osteomyelitis or osteitis\n --EXCLUDES--> [?] Infection of vertebra\n Def: A condition of the vertebrae, caused by an infection with a bacterial, viral, fungal, or parasitic source. This condition commonly presents with fever, chills, headache, weight loss, or may be asympto...", "[FB80.Z] Disorder of bone density or structure, unspecified\n --PARENT--> [FB80] Certain specified disorders of bone density or structure\n --CHILD--> [FB80.0] Fibrous dysplasia of bone\n Def: Fibrous dysplasia of bone is a congenital non-hereditary benign bone disease, where normal bone is replaced by a fibrous-like tissue with immature osteogenesis. Bone lesions are mono- or polyostotic a...", "[FB80.Z] Disorder of bone density or structure, unspecified\n --PARENT--> [FB80] Certain specified disorders of bone density or structure\n --CHILD--> [FB80.1] Skeletal fluorosis" ]	FC0Z	Diseases of the musculoskeletal system or connective tissue, unspecified	[ { "from_icd11": "FC0Z", "icd10_code": "XIII", "icd10_title": "" }, { "from_icd11": "FB84.Z", "icd10_code": "M86672", "icd10_title": "Other chronic osteomyelitis, left ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86172", "icd10_title": "Other acute osteomyelitis, left ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86171", "icd10_title": "Other acute osteomyelitis, right ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M86671", "icd10_title": "Other chronic osteomyelitis, right ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X7", "icd10_title": "Other osteomyelitis, ankle and foot" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X8", "icd10_title": "Other osteomyelitis, other site" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X6", "icd10_title": "Other osteomyelitis, lower leg" }, { "from_icd11": "FB84.Z", "icd10_code": "M868X9", "icd10_title": "Other osteomyelitis, unspecified sites" }, { "from_icd11": "FB84.Z", "icd10_code": "M8668", "icd10_title": "Other chronic osteomyelitis, other site" }, { "from_icd11": "FB84.Z", "icd10_code": "M86662", "icd10_title": "Other chronic osteomyelitis, left tibia and fibula" }, { "from_icd11": "FB84.Z", "icd10_code": "M86151", "icd10_title": "Other acute osteomyelitis, right femur" }, { "from_icd11": "FB84.Z", "icd10_code": "M86141", "icd10_title": "Other acute osteomyelitis, right hand" }, { "from_icd11": "FB84.Z", "icd10_code": "M86641", "icd10_title": "Other chronic osteomyelitis, right hand" }, { "from_icd11": "FB84.Z", "icd10_code": "M8669", "icd10_title": "Other chronic osteomyelitis, multiple sites" } ]	XIII
The patient, a 52-year-old male, was admitted to the Department of Neurology of our hospital on October 25, 2022, mainly due to involuntary limb jitter for >1 month and aggravated for 1 day. One month before admission, the patient showed involuntary limb shaking without obvious inducement, mainly in the upper limbs, at night, with no obvious pattern, accompanied by sleepiness, accompanied by dizziness, visual rotation, and double vision when both eyes were in the right and left. During this period, he was admitted to the local hospital and given conservative treatment (specific details are unknown). His family complained that the patient’s involuntary limb-shaking did not improve significantly. The involuntary vibration of the limbs was worse than before, accompanied by weakness of the lower limbs. During the course of the disease, the patient had poor diet and sleep, dry stools, and normal urination. The patient had no symptoms such as fever, cough, sore throat, and diarrhea before onset. He had a 1-year history of hypertension with a maximum of 160/115 mm Hg. He regularly took antihypertensive drugs and controlled them at 140/110 mm Hg. He had a history of brain stem hemorrhage 5 months before admission, underwent tracheotomy, left a speech problem, could not eat on his own (indwelling gastric tube), was bedridden, and could not stand. Denial of diabetes, coronary heart disease, and other medical history; no history of tuberculosis and other infectious diseases; no history of trauma; denial of allergies to drugs, food, etc. Physical examination: body temperature 36.2ºC, heart rate 66 times/min, breathing 18 times/min, blood pressure 131/91 mm Hg. Double lungs breathing sound coarse, can hear phlegm sound, regular heart rhythm, and each valve auscultation area did not hear and murmur. Abdomen is flat and soft, no obvious tenderness or rebound pain. Lethargy, slurred speech, slow reaction, uncooperative physical examination, double pupils large and equal round, sensitive light reflex, both eyes can move in all directions, left and right visual objects in pairs, horizontal and vertical eye tremors, bilateral soft palate involuntary tremor, uvula centered, and disappearance of pharyngeal reflex. Bilateral frontal line symmetry, nasolabial groove symmetry, indicating the mouth angle is not biased, extended tongue center, and tongue muscle fibrillation. Head-turning and shoulder-hunching are strong, bilateral limb muscle tone is slightly low, double upper limb muscle strength level 5, double lower limb muscle strength level 5−. Involuntary tremor of limbs, ataxia movement: bilateral rotation test, finger nose test, heel-knee-shin test and other physical examination did not cooperate, and pathological signs and meningeal stimulation signs were negative. Auxiliary examination: after admission, blood gas analysis was improved for 2 times (Table 1 ), complete abnormalities were found in 2 emergencies, routine abnormalities were found in 2 urinalysis, and video electroencephalogram (EEG) (1 day after admission) indicated abnormal EEG. Onset: Dozens of episodes were detected. EEG: No epileptic discharge was observed, and kinetic artifacts were observed. Intermittent period: diffuse slow wave activity increased in each lead. Episode: an episodic event. Reexamination of EEG (7 days after admission): abnormal EEG, EEG topographic map, multiple medium amplitude θ-wave unit δ-wave in each lead, fast wave: a small amount of low amplitude β-wave. Respiratory sleep monitoring (7 days after admission): moderate OSAHS. Imaging examination: cranial magnetic resonance angiography (MRA) (at the local hospital 1 month before admission): formation of pons softening lesion and abnormal signal shadow in the anterior medulla oblongata. Craniocerebral MRA: cerebral atherosclerotic changes (M2 segment of both middle cerebral arteries, right posterior cerebral artery). Head computed tomography scan (15 days before admission): left basal ganglia ischemic lesion was considered; brain atrophy. Head computed tomography + 3D reconstruction indicated (1 day after admission): bilateral lacunar cerebral infarction in basal ganglia area, small softening lesion in basal ganglia area and left pons. Cranial diffusion-weighted imaging (DWI) + MRA: No obvious abnormally high signal was observed on cranial DWI imaging. The right anterior cerebral artery A1 segment and the right middle cerebral artery M1 segment were localized stenosis. Head magnetic resonance imaging (MRI) + susceptibility weighted imaging : patchy abnormal signal shadow in the pontine, considering hemorrhagic malacia foci; multiple small softening foci in bilateral basal ganglia and left thalamus. Treatment process: the patient had an acute onset and a history of hypertension and cerebral hemorrhage. This time, the patient had bilateral involuntary tremor of the soft palate, tongue muscle fibrillation, binocular nystagmus, diplopia, involuntary shaking of the limbs and disturbance of consciousness. Imaging suggested that the lesion was located in the brain stem, so it was diagnosed as a degenerative disease of the nervous system: hyperplasia of inferior olivine degeneration. Symptomatic treatment after admission, antiepileptic drugs valproate sustained-release tablets and levetiracetam were given to control limb vibration, but the effect of limb shaking was not good on the 3rd day after admission. After topiramate 25 mg was added once every 12 hours, the symptoms were relieved, levetiracetam was reduced, benzoxol hydrochloride 2 mg was added once every 24 hours, and the spirit was significantly improved. On the 12th day after admission, the patient still had intermittent involuntary shaking at night. After the addition of clonazepam tablets before going to bed, the shaking during the sleep period was significantly reduced, which supported hypertrophic olivary degeneration. And give antihypertensive, open the body to wake up the brain, protect the stomach, promote collateral circulation and other treatments. Supplementary diagnosis: OSAHS. The patient’s family members purchased portable ventilators by themselves and were given noninvasive assisted ventilation. After treatment, oxygen partial pressure increased significantly and dizziness and other symptoms were relieved.	3.849609	0.983887	sec[1]/p[0]	en	0.999997	36637934	https://doi.org/10.1097/MD.0000000000032681	[ "involuntary", "limb", "cerebral", "shaking", "imaging", "limbs", "double", "muscle", "wave", "obvious" ]	[ { "code": "MF50.2Z", "title": "Urinary incontinence, unspecified" }, { "code": "ME07.Z", "title": "Faecal incontinence, unspecified" }, { "code": "MB47.5", "title": "Fasciculation" }, { "code": "MB46.Z", "title": "Abnormal involuntary movements, unspecified" }, { "code": "MB46.Y", "title": "Other specified abnormal involuntary movements" }, { "code": "ND56.1", "title": "Open wound of unspecified body region" }, { "code": "LB9Z", "title": "Structural developmental anomalies of the skeleton, unspecified" }, { "code": "FB56.6", "title": "Other specified soft tissue disorders" }, { "code": "5B51&XS25", "title": "Severe wasting in infants, children or adolescents" }, { "code": "ND55", "title": "Other injuries of leg, level unspecified" } ]	=== ICD-11 CODES FOUND === [MF50.2Z] Urinary incontinence, unspecified Also known as: Urinary incontinence, unspecified \| Urinary incontinence \| urinary incontinence, NOS \| bladder incontinence NOS \| absence of bladder continence [ME07.Z] Faecal incontinence, unspecified Also known as: Faecal incontinence, unspecified \| Faecal incontinence \| bowel incontinence \| involuntary stool \| faecal incontinence NOS [MB47.5] Fasciculation Also known as: Fasciculation \| flickering muscles \| fluttering muscles \| muscle fasciculation \| muscular fasciculation [MB46.Z] Abnormal involuntary movements, unspecified Also known as: Abnormal involuntary movements, unspecified \| Abnormal involuntary movements \| shaking \| shaking all over \| the shakes [MB46.Y] Other specified abnormal involuntary movements Also known as: Other specified abnormal involuntary movements \| Reflex convulsions \| Dystonic movements [ND56.1] Open wound of unspecified body region Also known as: Open wound of unspecified body region \| cut NOS \| open wound NOS \| penetrating wound NOS \| Puncture wound with foreign body unspecified body region Excludes: Traumatic amputations involving multiple body regions \| Open wounds involving multiple body regions \| traumatic amputation NOS [LB9Z] Structural developmental anomalies of the skeleton, unspecified Also known as: Structural developmental anomalies of the skeleton, unspecified \| Abnormal bone development \| skeletal anomaly NOS [FB56.6] Other specified soft tissue disorders Also known as: Other specified soft tissue disorders \| Fat necrosis \| fatty necrosis \| Profichet's disease \| Sloughing of fascia [ND55] Other injuries of leg, level unspecified Also known as: Other injuries of leg, level unspecified \| other injuries of lower limb, level unspecified \| Superficial injury of leg, level unspecified \| Abrasion of leg, level unspecified \| Contusion of leg, level unspecified Excludes: Fracture of leg, level unspecified \| Injuries involving multiple body regions === GRAPH WALKS === --- Walk 1 --- [MF50.2Z] Urinary incontinence, unspecified --PARENT--> [MF50.2] Urinary incontinence Def: Any condition of the urinary system, caused by determinants arising during the antenatal period or after birth, leading to loss of voluntary control or support of the urethra. These conditions are cha... --CHILD--> [MF50.22] Mixed incontinence --- Walk 2 --- [MF50.2Z] Urinary incontinence, unspecified --PARENT--> [MF50.2] Urinary incontinence Def: Any condition of the urinary system, caused by determinants arising during the antenatal period or after birth, leading to loss of voluntary control or support of the urethra. These conditions are cha... --EXCLUDES--> [?] Recurrent or persistent glomerular haematuria Def: The presence in the urine of glomerular origin red blood cells (RBCs). In microscopic haematuria the urine appears normal to the naked eye, but examination with a microscope shows a high number of RBC... --- Walk 3 --- [ME07.Z] Faecal incontinence, unspecified --PARENT--> [ME07] Faecal incontinence Def: Failure of voluntary control of the anal sphincters, with involuntary passage of faeces and flatus.... --EXCLUDES--> [?] Encopresis Def: Encopresis is the repeated passage of faeces in inappropriate places. Encopresis should be diagnosed if inappropriate passage of faeces occurs repeatedly (e.g., at least once per month over a period o... --- Walk 4 --- [ME07.Z] Faecal incontinence, unspecified --PARENT--> [ME07] Faecal incontinence Def: Failure of voluntary control of the anal sphincters, with involuntary passage of faeces and flatus.... --CHILD--> [ME07.0] Faecal smearing --- Walk 5 --- [MB47.5] Fasciculation --PARENT--> [MB47] Abnormality of tonus or reflex --CHILD--> [MB47.1] Abnormal posture --- Walk 6 --- [MB47.5] Fasciculation --PARENT--> [MB47] Abnormality of tonus or reflex --CHILD--> [MB47.0] Abnormal reflex	[ "[MF50.2Z] Urinary incontinence, unspecified\n --PARENT--> [MF50.2] Urinary incontinence\n Def: Any condition of the urinary system, caused by determinants arising during the antenatal period or after birth, leading to loss of voluntary control or support of the urethra. These conditions are cha...\n --CHILD--> [MF50.22] Mixed incontinence", "[MF50.2Z] Urinary incontinence, unspecified\n --PARENT--> [MF50.2] Urinary incontinence\n Def: Any condition of the urinary system, caused by determinants arising during the antenatal period or after birth, leading to loss of voluntary control or support of the urethra. These conditions are cha...\n --EXCLUDES--> [?] Recurrent or persistent glomerular haematuria\n Def: The presence in the urine of glomerular origin red blood cells (RBCs). In microscopic haematuria the urine appears normal to the naked eye, but examination with a microscope shows a high number of RBC...", "[ME07.Z] Faecal incontinence, unspecified\n --PARENT--> [ME07] Faecal incontinence\n Def: Failure of voluntary control of the anal sphincters, with involuntary passage of faeces and flatus....\n --EXCLUDES--> [?] Encopresis\n Def: Encopresis is the repeated passage of faeces in inappropriate places. Encopresis should be diagnosed if inappropriate passage of faeces occurs repeatedly (e.g., at least once per month over a period o...", "[ME07.Z] Faecal incontinence, unspecified\n --PARENT--> [ME07] Faecal incontinence\n Def: Failure of voluntary control of the anal sphincters, with involuntary passage of faeces and flatus....\n --CHILD--> [ME07.0] Faecal smearing", "[MB47.5] Fasciculation\n --PARENT--> [MB47] Abnormality of tonus or reflex\n --CHILD--> [MB47.1] Abnormal posture", "[MB47.5] Fasciculation\n --PARENT--> [MB47] Abnormality of tonus or reflex\n --CHILD--> [MB47.0] Abnormal reflex" ]	MF50.2Z	Urinary incontinence, unspecified	[ { "from_icd11": "MF50.2Z", "icd10_code": "N39498", "icd10_title": "Other specified urinary incontinence" }, { "from_icd11": "MF50.2Z", "icd10_code": "N3941", "icd10_title": "Urge incontinence" }, { "from_icd11": "MF50.2Z", "icd10_code": "N3946", "icd10_title": "Mixed incontinence" }, { "from_icd11": "MF50.2Z", "icd10_code": "N3945", "icd10_title": "Continuous leakage" }, { "from_icd11": "MF50.2Z", "icd10_code": "N3944", "icd10_title": "Nocturnal enuresis" }, { "from_icd11": "MF50.2Z", "icd10_code": "N39490", "icd10_title": "Overflow incontinence" }, { "from_icd11": "MF50.2Z", "icd10_code": "N3943", "icd10_title": "Post-void dribbling" }, { "from_icd11": "MF50.2Z", "icd10_code": "N3942", "icd10_title": "Incontinence without sensory awareness" }, { "from_icd11": "MF50.2Z", "icd10_code": "R32", "icd10_title": "Unspecified urinary incontinence" }, { "from_icd11": "MF50.2Z", "icd10_code": "N394", "icd10_title": "Other specified urinary incontinence" }, { "from_icd11": "ME07.Z", "icd10_code": "R159", "icd10_title": "Full incontinence of feces" }, { "from_icd11": "ME07.Z", "icd10_code": "R152", "icd10_title": "Fecal urgency" }, { "from_icd11": "ME07.Z", "icd10_code": "R151", "icd10_title": "Fecal smearing" }, { "from_icd11": "ME07.Z", "icd10_code": "R150", "icd10_title": "Incomplete defecation" }, { "from_icd11": "ME07.Z", "icd10_code": "R15", "icd10_title": "Fecal incontinence" } ]	N39498	Other specified urinary incontinence
A 7-year-old boy was hospitalized for the evaluation of multiple progressive complaints over the previous 2 years, including frontal headache, fatigue, tics, leg pain, nocturnal sweating, constipation, and poor food intake. He had a normal birthweight/height following an unremarkable pregnancy, and his family history was normal. His growth curve showed normal growth until the age of 3 years, followed by a marked decrease to about −2 standardised deviation scores (SDS) at the age of 6 years . Thereafter, his growth increased rapidly compared with the Dutch national standards. His parents were of modest stature (father, 170 cm and mother, 164 cm) by the current Dutch median height standards (men, 182.9 cm and women, 169.3 cm). A sellar tumor with an enlarged sella turcica was discovered . Laboratory analysis showed an insulin-like growth factor I (IGF-I) level of 56.5 nmol/L (normal range [NR], 8.3-38.2; SDS, +3.49), prolactin level of 0.5 nmol/L (NR, <0.36 nmol/L), TSH level of 1.07 mU/L (NR, 0.6-5.6 mU/L), free thyroxine level of 20.2 pmol/L (NR, 13-26 nmol/L), afternoon cortisol level of 270 nmol/L (NR, <700 nmol/L), and undetectable luteinizing hormone and follicle-stimulating hormone (normal for prepubertal state). 10 Over time, the growth rate accelerated further , in parallel with rising IGF-I (71.2 nmol/L; +4.53 SDS), a random GH of 30.8 μg/L (NR, <4.0 μg/L), and prolactin increase to 0.75 nmol/L. The nadir GH value during an oral glucose tolerance test was 26.7 μg/L. He complained of vomiting and loss of appetite. Treatment of the GH-secreting macroadenoma was initiated with lanreotide 120 mg once in 4 weeks, which resulted in no biochemical response (IGF-I level, 76.6 nmol/L and GH level, 28.0 μg/L) or inhibition of tumor growth after 4 doses. Lanreotide was switched to pasireotide 60-mg long-acting release (LAR) once in 4 weeks. One month after switching, he developed a new onset of bitemporal field defects and headaches that indicated symptomatic optic chiasmal compression, and he underwent transsphenoidal surgery for the first time. Two months postoperatively (3 months after the initiation of pasireotide LAR), IGF-I (70.3 nmol/L) and GH (23.4 μg/L) levels remained elevated. Pasireotide LAR showed no hormonal or tumoral effects, and the GH receptor antagonist pegvisomant was started with a weekly dose of 40 mg. Although IGF-I levels dropped to 34.1 nmol/L (NR, 10.9-47.3 nmol/L; 0.87 SDS), the local GH assay, which does not detect pegvisomant, continued to show an elevated random GH level (48.6 μg/L). After 1 month of pegvisomant, severe headaches returned, and bitemporal hemianopsia reoccurred due to an increase in tumor volume . Pegvisomant was stopped, and a second transsphenoidal resection followed . The histopathologic report revealed a pituitary adenoma staining positive for GH and negative for prolactin . One month after the second transsphenoidal surgery, his IGF-I level declined to 29.3 nmol/L (0.4 SDS), GH level was 2.7 μg/L, and prolactin level declined from 0.60 to 0.38 nmol/L (NR, 0.1-0.5 nmol/L). Five months after surgery, the headaches returned, and magnetic resonance imaging 1 month later showed a small remnant lateral to the right internal carotid artery . IGF-I increased again to +2.9 SDS. A third transsphenoidal surgery was performed, leading to the normalization of GH and IGF-I levels. Thirteen months after his last surgery, he received stereotactic radiotherapy (54 Gy), and 4 months after radiotherapy, his last IGF-I was −0.8 SDS. Fig. 1 Growth chart of the patient with incipient gigantism. The initial normal growth of the patient declined from 3 to 6 years of age, but then deflected markedly upward. The patient was diagnosed at the age of 7 years. The blue arrow corresponds with LAN treatment, the purple arrow corresponds with PAS treatment, and the red arrow corresponds with PEGV surgery. One month after switching to pasireotide, the first transsphenoidal resection was performed. Two months thereafter, pegvisomant was started. After 1 month of pegvisomant, the tumor volume increased; pegvisomant was stopped, and a second resection followed. Six months thereafter, the third surgery was performed. LAN = lanreotide; PAS = pasireotide; PEGV surgery = pegvisomant and surgery. Fig. 2 Sequential magnetic resonance imaging over the clinical course of the patient between 2018 and 2020. Contrast-enhanced T1-weighted sequences in coronal ( A, C, E, G, I, K, M ) and sagittal ( B, D, F, H, J, L, N ) planes were chosen and corrected for gray scale and magnification. The timing of the scans were as follows: at clinical presentation ( A, B ); before the first operation (lanreotide was switched to pasireotide; after this, magnetic resonance imaging was performed because of tumor growth, and surgery was performed because of visual field defects due to chiasmic compression) ( C, D ); postoperatively after the first operation ( E, F ); before the second operation ( G, H ); postoperatively after the second operation (1 month after intiating pegvisomant treatment) ( I, J ); before the third operation ( K, L ); and postoperatively after the third operation ( M, N ). There was no inhibition of tumor growth after the use of somatostatin analogs in terms of tumor size and extent. Correspondingly, the growth hormone secretion was normalized after the respective tumor resections. The tumor was medial to the intracavernous intercarotid line (Knosp status grade II); however, on direct vision during the last surgery, there was invasion of the cavernous sinus wall. Fig. 3 Histopathologic features of the tumor at the second and third surgery. The tissue from the first surgery was unavailable. A-F , the second surgery; G-L , the third surgery. A and G , Hematoxylin and eosin staining show a pituitary adenoma with interspersed mitoses in both surgeries (black arrows). B and H , Growth hormone expression. C and I , PanCK immunohistochemistry shows a few fibrous bodies in both specimens. In both specimens, there is an increased proliferation activity (Ki67 staining in D and J ). The tissue from both the surgeries had a homogeneous expression of SSTR2 ( E, K ), whereas SSTR5 was moderately expressed in the specimen of the second surgery ( F ) and absent in the tissue of the third surgery ( L ).	4.199219	0.966797	sec[1]/p[0]	en	0.999997	PMC9123570	https://doi.org/10.1016/j.aace.2021.12.003	[ "nmol", "growth", "tumor", "pegvisomant", "pasireotide", "transsphenoidal", "prolactin", "hormone", "lanreotide", "postoperatively" ]	[ { "code": "2F9Z", "title": "Neoplasms of unknown behaviour of unspecified site" }, { "code": "MG44.1Z", "title": "Lack of expected normal physiological development, unspecified" }, { "code": "5A61.3", "title": "Growth hormone deficiency" }, { "code": "8C7Y", "title": "Other specified primary disorders of muscles" }, { "code": "FB86.Z", "title": "Disorders associated with bone growth, unspecified" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "2E6Z", "title": "Carcinoma in situ of unspecified site" }, { "code": "2F91.1", "title": "Neoplasms of unknown behaviour of trachea, bronchus or lung" }, { "code": "2F92", "title": "Neoplasms of unknown behaviour of skin" }, { "code": "2F00.Y", "title": "Other specified benign neoplasm of middle ear or respiratory system" } ]	=== ICD-11 CODES FOUND === [2F9Z] Neoplasms of unknown behaviour of unspecified site Also known as: Neoplasms of unknown behaviour of unspecified site \| neoplasia \| neoplasm growth NOS \| tumour of unspecified site \| tumourlet of unspecified site [MG44.1Z] Lack of expected normal physiological development, unspecified Also known as: Lack of expected normal physiological development, unspecified \| Lack of expected normal physiological development \| delayed physiological development \| unspecified delay in development \| development arrest [5A61.3] Growth hormone deficiency Definition: Deficiency of growth hormone in children, adolescents and adults. Includes deficiency of growth hormone releasing hormone (GHRH) and excess of central somatostatin, leading to growth hormone deficiency. Includes idiopathic, inborn and acquired forms of growth hormone deficiency. Also known as: Growth hormone deficiency Excludes: Hypopituitarism [8C7Y] Other specified primary disorders of muscles Also known as: Other specified primary disorders of muscles \| Certain specified primary disorders of muscles \| Myopathy due to calsequestrin or SERCA1 protein overload \| Delayed muscle maturation \| delayed muscle growth [FB86.Z] Disorders associated with bone growth, unspecified Also known as: Disorders associated with bone growth, unspecified \| Disorders associated with bone growth [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [2E6Z] Carcinoma in situ of unspecified site Also known as: Carcinoma in situ of unspecified site \| carcinoma in situ of unspecified site \| carcinoma in situ NOS \| carcinoma in situ \| in situ neoplasm [2F91.1] Neoplasms of unknown behaviour of trachea, bronchus or lung Also known as: Neoplasms of unknown behaviour of trachea, bronchus or lung \| trachea, bronchus or lung tumour NOS \| Intravascular bronchial alveolar tumour of unspecified site \| Bronchial adenoma of unknown behaviour of unspecified site \| Intravascular bronchial alveolar tumour unknown behaviour of unspecified site [2F92] Neoplasms of unknown behaviour of skin Also known as: Neoplasms of unknown behaviour of skin \| skin tumour NOS [2F00.Y] Other specified benign neoplasm of middle ear or respiratory system Also known as: Other specified benign neoplasm of middle ear or respiratory system \| Benign neoplasm of middle ear, nasal cavity or accessory sinuses \| Transphenoidal adenoma \| Benign neoplasm of accessory sinus \| Benign neoplasm of cartilage of nose === GRAPH WALKS === --- Walk 1 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour --- Walk 2 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour --- Walk 3 --- [MG44.1Z] Lack of expected normal physiological development, unspecified --PARENT--> [MG44.1] Lack of expected normal physiological development Def: Lack of expected normal physiological development includes delayed milestone of development and other lack of expected normal physiological development including gross and fine motor development, lang... --EXCLUDES--> [?] Disorders of intellectual development Def: Disorders of intellectual development are a group of etiologically diverse conditions originating during the developmental period characterised by significantly below average intellectual functioning ... --- Walk 4 --- [MG44.1Z] Lack of expected normal physiological development, unspecified --PARENT--> [MG44.1] Lack of expected normal physiological development Def: Lack of expected normal physiological development includes delayed milestone of development and other lack of expected normal physiological development including gross and fine motor development, lang... --CHILD--> [MG44.10] Delayed milestone --- Walk 5 --- [5A61.3] Growth hormone deficiency Def: Deficiency of growth hormone in children, adolescents and adults. Includes deficiency of growth hormone releasing hormone (GHRH) and excess of central somatostatin, leading to growth hormone deficienc... --EXCLUDES--> [?] Hypopituitarism Def: A disorder manifesting a deficiency or decrease of one or more pituitary hormones, which is caused by a variety of diseases such as tumour, trauma/surgery, irradiation, inflammation and haemorrhage/in... --CHILD--> [?] Argonz-del Castillo Syndrome --- Walk 6 --- [5A61.3] Growth hormone deficiency Def: Deficiency of growth hormone in children, adolescents and adults. Includes deficiency of growth hormone releasing hormone (GHRH) and excess of central somatostatin, leading to growth hormone deficienc... --PARENT--> [5A61] Hypofunction or certain other specified disorders of pituitary gland Def: Clinical status with disordered function of the pituitary gland without excessive pituitary hormone production, which is caused by a variety of diseases... --CHILD--> [5A61.1] Adrenocorticotropic hormone deficiency Def: Deficiency of adrenocorticotropic hormone (ACTH) resulting in functional hypocortisolism. Includes deficiency of corticotropin releasing hormone (CRH, CRF)....	[ "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour", "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour", "[MG44.1Z] Lack of expected normal physiological development, unspecified\n --PARENT--> [MG44.1] Lack of expected normal physiological development\n Def: Lack of expected normal physiological development includes delayed milestone of development and other lack of expected normal physiological development including gross and fine motor development, lang...\n --EXCLUDES--> [?] Disorders of intellectual development\n Def: Disorders of intellectual development are a group of etiologically diverse conditions originating during the developmental period characterised by significantly below average intellectual functioning ...", "[MG44.1Z] Lack of expected normal physiological development, unspecified\n --PARENT--> [MG44.1] Lack of expected normal physiological development\n Def: Lack of expected normal physiological development includes delayed milestone of development and other lack of expected normal physiological development including gross and fine motor development, lang...\n --CHILD--> [MG44.10] Delayed milestone", "[5A61.3] Growth hormone deficiency\n Def: Deficiency of growth hormone in children, adolescents and adults. Includes deficiency of growth hormone releasing hormone (GHRH) and excess of central somatostatin, leading to growth hormone deficienc...\n --EXCLUDES--> [?] Hypopituitarism\n Def: A disorder manifesting a deficiency or decrease of one or more pituitary hormones, which is caused by a variety of diseases such as tumour, trauma/surgery, irradiation, inflammation and haemorrhage/in...\n --CHILD--> [?] Argonz-del Castillo Syndrome", "[5A61.3] Growth hormone deficiency\n Def: Deficiency of growth hormone in children, adolescents and adults. Includes deficiency of growth hormone releasing hormone (GHRH) and excess of central somatostatin, leading to growth hormone deficienc...\n --PARENT--> [5A61] Hypofunction or certain other specified disorders of pituitary gland\n Def: Clinical status with disordered function of the pituitary gland without excessive pituitary hormone production, which is caused by a variety of diseases...\n --CHILD--> [5A61.1] Adrenocorticotropic hormone deficiency\n Def: Deficiency of adrenocorticotropic hormone (ACTH) resulting in functional hypocortisolism. Includes deficiency of corticotropin releasing hormone (CRH, CRF)...." ]	2F9Z	Neoplasms of unknown behaviour of unspecified site	[ { "from_icd11": "2F9Z", "icd10_code": "D487", "icd10_title": "Neoplasm of uncertain behavior of other specified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D482", "icd10_title": "Neoplasm of uncertain behavior of peripheral nerves and autonomic nervous system" }, { "from_icd11": "2F9Z", "icd10_code": "D37-D48", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D377", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D48", "icd10_title": "Neoplasm of uncertain behavior of other and unspecified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D489", "icd10_title": "Neoplasm of uncertain behavior, unspecified" }, { "from_icd11": "MG44.1Z", "icd10_code": "R6250", "icd10_title": "Unspecified lack of expected normal physiological development in childhood" }, { "from_icd11": "MG44.1Z", "icd10_code": "R6259", "icd10_title": "Other lack of expected normal physiological development in childhood" }, { "from_icd11": "MG44.1Z", "icd10_code": "R6251", "icd10_title": "Failure to thrive (child)" }, { "from_icd11": "MG44.1Z", "icd10_code": "R6252", "icd10_title": "Short stature (child)" }, { "from_icd11": "MG44.1Z", "icd10_code": "R62", "icd10_title": "Lack of expected normal physiological development in childhood and adults" }, { "from_icd11": "MG44.1Z", "icd10_code": "R628", "icd10_title": "" }, { "from_icd11": "MG44.1Z", "icd10_code": "R629", "icd10_title": "" }, { "from_icd11": "FB86.Z", "icd10_code": "M89761", "icd10_title": "Major osseous defect, right lower leg" }, { "from_icd11": "FB86.Z", "icd10_code": "M89772", "icd10_title": "Major osseous defect, left ankle and foot" } ]	D487	Neoplasm of uncertain behavior of other specified sites
MR images of the brain and cervical spine were acquired using a 1.5-Tesla scanner (MAGNETOM AVANTO, Siemens, Germany) with head and neck knee coils, respectively. The MRI sequences of the brain and cervical spine used in the present case are shown in Table 1 . On T2-weighted images, ill-defined, heterogeneously hyperintense areas in the spinal cord were identified eccentrically in the central-to-left dorsal aspect of the second cervical (C2) spinal cord, leading to focal spinal cord swelling showing a loss of normal parenchymal architecture and circumferential attenuation of the cerebrospinal fluid (CSF) line on T2-weighted half-Fourier acquisition single-shot turbo spin-echo sequence . The lesion appeared iso-to-hypointense on T1-weighted images and iso-to-hyperintense on T2-weighted multi-echo data image combination sequence images . Post-contrast T1-weighted images revealed an ovoid, well-defined mass with homogeneously marked contrast enhancement . The mass contained multiple small cavitary lesions suggestive of cystic or necrotic areas, causing severe left-sided compression of the spinal cord . In the dorsal subarachnoid space just caudal to the C2 mass, although not typical, a structure resembling the “golf-tee” sign, a characteristic sign of an intradural extramedullary lesion ( 4 , 6 , 10 , 11 ), and syringomyelia were noted . Additionally, an appearance mimicking the “dural tail sign” in the meninges adjacent to the thickened C2 left nerve root with contrast enhancement, mild atrophy with contrast enhancement of the paraspinal muscle adjacent to the lesion, and ventral meningeal enhancement were identified . However, broad-based dural attachment of the mass was not evident in any sequence. Other MRI findings included multiple cervical intervertebral disc degeneration with protrusion, bilateral ventriculomegaly, and Chiari-like malformations. Based on MRI findings, we concluded that the C2 spinal mass was the cause of the patient’s symptoms. Considering the MRI characteristics of the mass (single, well-defined, marked contrast enhancement, and mass effect), a spinal tumor concurrent with gliosis, left-sided neuritis, and myositis was strongly suspected rather than inflammatory or vascular diseases. However, the origin of the C2 mass could not be determined because of its extensive distribution throughout the spinal parenchyma in all images. An intradural extramedullary tumor (e.g., meningioma, nerve sheath tumor, or nephroblastoma) was considered a primary differential diagnosis, and other possibilities were an intramedullary tumor or intramedullary invasion by an extramedullary tumor. Given its clear margins, location, size, and the progression of clinical signs, surgical removal of the mass was planned via dorsal laminectomy of C2 combined with durotomy. A high-speed burr and bone-cutting forceps were used to create a hinged osteotomy of the C2 dorsal arch involving the cranial 75% of the C2 spinous process . The bone flap was rotated dorsally and cranially on the preserved attachment of the cranial aspect of the C2 spinous process to the dorsal arch of C1 to visualize the dorsal dura. A midline durotomy revealed a capsulated mass. Most tumor parts were easily separated and removed from the adjacent spinal cord parenchyma; however, the ventral part adhered locally to the spinal cord meninges adjacent to the left nerve root with fibrotic tissues . After careful removal of these ventral tumor tissues, mild hemorrhage occurred but was promptly controlled. No abnormalities were observed in the spinal cord. A biological, absorbable dura substitute (Lyoplant Onlay; B Braun Co., Germany) was used to cover the dural defect and sutured to the adjacent dura before the spinous process was returned to its normal position. Subsequently, the dorsal arch of the axis was rotated back over the exposed vertebral canal, and the spinous process was stabilized with 2–0 non-absorbable sutures (PROLENE Polypropylene Suture; ETHICON Co., United States) through one predrilled hole. The excised mass, characterized by a red, well-circumscribed, and elastic appearance , was histopathologically examined (hematoxylin and eosin staining). Spindle round-to-polygonal cells containing small-to-moderate amounts of pale, eosinophilic, wispy-to-granular cytoplasm were observed. The nuclei were heterochromatic, with one or two variably distinct nucleoli, along with moderate anisocytosis and anisokaryosis . These findings were most consistent with glioma, in which oligodendroglioma may be the most consistent. The histopathological identification of an intramedullary tumor did not match the MRI or surgical findings. Thus, a genomic tumor test was performed using the same tissue sample as the histopathology; immunohistochemical staining for glial cell tumor markers could not be performed because of the owner’s financial constraints. This genomic tumor test is a tumor-only, next-generation sequencing, hybrid-capture test involving canine gene panel covering 482,000 base pairs of 120 genes associated with canine or human cancer. Genetic data revealed copy number loss of CDKN2N and gain of MYC , supporting the diagnosis of a canine glioma. Three days postoperatively, the dog was able to stand up and walk in the cage, and its appetite returned to normal. Approximately 2 weeks postoperatively, all neurological signs returned to normal. Although postoperative MRI and computed tomography (CT) examinations were recommended to confirm the residual tissue of the glioma and assess metastasis, including “CSF drop metastasis” from the glioma ( 12–14 ), only a 16-channel multi-detector CT (Emotion 16, Siemens Healthcare, Forchheim, Germany) scan was performed due to the owner’s financial constraints. On postoperative CT images, regional bone defects in the C2 lamina due to surgery were identified. However, no significant CT findings were identified in the whole body, including tumor metastasis, lymph node enlargement, or contrast-enhanced lesions around the surgical site. Although chemotherapy was recommended based on the genetic data and considering possible recurrence and local adhesion or invasion of the tumor, the owner rejected this suggestion. Ten months after discharge, no recurrence of neurological signs had been reported.	4.207031	0.590332	sec[1]/p[1]	en	0.999996	38840642	https://doi.org/10.3389/fvets.2024.1400139	[ "tumor", "spinal", "cord", "contrast", "weighted", "enhancement", "adjacent", "cervical", "however", "spinous" ]	[ { "code": "2F9Z", "title": "Neoplasms of unknown behaviour of unspecified site" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "2E6Z", "title": "Carcinoma in situ of unspecified site" }, { "code": "2F91.1", "title": "Neoplasms of unknown behaviour of trachea, bronchus or lung" }, { "code": "2F92", "title": "Neoplasms of unknown behaviour of skin" }, { "code": "FB1Z", "title": "Conditions associated with the spine, unspecified" }, { "code": "FA7Z", "title": "Structural disorders of spine, unspecified" }, { "code": "FA9Z", "title": "Inflammation of spine, unspecified" }, { "code": "LB73.2Z", "title": "Structural developmental anomalies of spine, unspecified" }, { "code": "FA82", "title": "Spinal stenosis" } ]	=== ICD-11 CODES FOUND === [2F9Z] Neoplasms of unknown behaviour of unspecified site Also known as: Neoplasms of unknown behaviour of unspecified site \| neoplasia \| neoplasm growth NOS \| tumour of unspecified site \| tumourlet of unspecified site [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [2E6Z] Carcinoma in situ of unspecified site Also known as: Carcinoma in situ of unspecified site \| carcinoma in situ of unspecified site \| carcinoma in situ NOS \| carcinoma in situ \| in situ neoplasm [2F91.1] Neoplasms of unknown behaviour of trachea, bronchus or lung Also known as: Neoplasms of unknown behaviour of trachea, bronchus or lung \| trachea, bronchus or lung tumour NOS \| Intravascular bronchial alveolar tumour of unspecified site \| Bronchial adenoma of unknown behaviour of unspecified site \| Intravascular bronchial alveolar tumour unknown behaviour of unspecified site [2F92] Neoplasms of unknown behaviour of skin Also known as: Neoplasms of unknown behaviour of skin \| skin tumour NOS [FB1Z] Conditions associated with the spine, unspecified Also known as: Conditions associated with the spine, unspecified \| dorsopathies \| disorder of spine \| spinal disorder [FA7Z] Structural disorders of spine, unspecified Also known as: Structural disorders of spine, unspecified \| spinal disease [FA9Z] Inflammation of spine, unspecified Also known as: Inflammation of spine, unspecified \| spinal inflammation \| discitis, unspecified [LB73.2Z] Structural developmental anomalies of spine, unspecified Also known as: Structural developmental anomalies of spine, unspecified \| Structural developmental anomalies of spine \| Malformations of spine \| maldevelopment of spine [FA82] Spinal stenosis Definition: This is a condition characterised by narrowing of the spinal canal. Also known as: Spinal stenosis \| spinal canal stenosis \| Spinal stenosis with no determinant \| primary spinal stenosis \| Spinal stenosis with determinant === GRAPH WALKS === --- Walk 1 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour --- Walk 2 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2F90] Neoplasms of unknown behaviour of oral cavity or digestive organs --- Walk 3 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Enlarged lymph nodes Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes.... --EXCLUDES--> [?] Chronic lymphadenitis --- Walk 4 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Enlarged lymph nodes Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes.... --EXCLUDES--> [?] Nonspecific mesenteric lymphadenitis --- Walk 5 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --PARENT--> [02] Neoplasms Def: An abnormal or uncontrolled cellular proliferation which is not coordinated with an organism's requirements for normal tissue growth, replacement or repair.... --- Walk 6 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E60] Carcinoma in situ of oral cavity, oesophagus or stomach	[ "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour", "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2F90] Neoplasms of unknown behaviour of oral cavity or digestive organs", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Enlarged lymph nodes\n Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes....\n --EXCLUDES--> [?] Chronic lymphadenitis", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Enlarged lymph nodes\n Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes....\n --EXCLUDES--> [?] Nonspecific mesenteric lymphadenitis", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --PARENT--> [02] Neoplasms\n Def: An abnormal or uncontrolled cellular proliferation which is not coordinated with an organism's requirements for normal tissue growth, replacement or repair....", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E60] Carcinoma in situ of oral cavity, oesophagus or stomach" ]	2F9Z	Neoplasms of unknown behaviour of unspecified site	[ { "from_icd11": "2F9Z", "icd10_code": "D487", "icd10_title": "Neoplasm of uncertain behavior of other specified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D482", "icd10_title": "Neoplasm of uncertain behavior of peripheral nerves and autonomic nervous system" }, { "from_icd11": "2F9Z", "icd10_code": "D37-D48", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D377", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D48", "icd10_title": "Neoplasm of uncertain behavior of other and unspecified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D489", "icd10_title": "Neoplasm of uncertain behavior, unspecified" }, { "from_icd11": "ME61", "icd10_code": "R2240", "icd10_title": "Localized swelling, mass and lump, unspecified lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2232", "icd10_title": "Localized swelling, mass and lump, left upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2242", "icd10_title": "Localized swelling, mass and lump, left lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2231", "icd10_title": "Localized swelling, mass and lump, right upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2241", "icd10_title": "Localized swelling, mass and lump, right lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2233", "icd10_title": "Localized swelling, mass and lump, upper limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2243", "icd10_title": "Localized swelling, mass and lump, lower limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2230", "icd10_title": "Localized swelling, mass and lump, unspecified upper limb" }, { "from_icd11": "ME61", "icd10_code": "R220", "icd10_title": "Localized swelling, mass and lump, head" } ]	D487	Neoplasm of uncertain behavior of other specified sites
A 6-year-old White/Caucasian male (M6) with no significant family or past medical history presented with an intra- and suprasellar mass measuring 2.2 cm in diameter in the suprasellar component that compressed the optic chiasm and displaced the supraclinoid carotid arteries and the floor of the 3rd ventricle . The tumor was resected via transsphenoidal approach and pathology was consistent with a macroadenoma with patchy lymphoid inflammatory infiltrates . The tumor was composed of relatively monomorphic epithelial cells with abundant light eosinophilic (chromophobe) cytoplasm and large nuclei containing prominent nucleoli . Mitotic figures were conspicuous, in average 4 per 10 high power fields. Normal-appearing glandular structures were scattered within the tumor, and were lined by a monolayer of epithelial polarized cells labeled by NHERF1, a polarity marker structuring microvilli at the apical plasma membrane of epithelial cells . These glandular cells were also labeled by cytokeratin but not by synaptophysin , suggesting derivation from Rathke’s pouch. In contrast, tumor cells were diffusely and strongly labeled by both cytokeratin and synaptophysin. IHC with the six adenohypophysis hormones showed positivity only for prolactin, with a peripheral subplasmalemmal distribution . This peripheral pattern is novel, and is not characteristic of the two described histologic types of lactotroph adenoma/prolactinoma, sparsely or densely granulated, which show paranuclear or diffuse cytoplasmic staining, respectively . The pre-operative levels of prolactin were only mildly elevated, at 50 ng/ml (normal levels 3.2–20 ng/ml). Electron microscopy was performed and peripherally placed small secretory vesicles were observed; however, “misplaced exocytosis”, an ultrastructural characteristic of prolactinoma, could not be visualized due to poor tissue preservation (not shown). Another unusual histologic finding was the lack of estrogen receptor labeling , a staining generally positive in prolactinoma . The Ki-67 proliferation index was highly elevated, at 16.7%, and correlated with the mitotic count . IHC for p53 showed strong labeling of rare scattered nuclei, but the large majority of the cells were negative. Due to the atypical histologic features and high mitotic and Ki-67 proliferation indices, the tumor was diagnosed as atypical/high-risk prolactinoma, and frequent follow-up was recommended. Three months later, the tumor recurred and a second resection was performed . To prevent future recurrence, opposed lateral proton pencil beam radiation (54 CGE (Cobalt Gray Equivalent) in 30 fractions over 6 weeks) was administered resulting in efficient control of the pituitary tumor. Three months later, a new, rapidly growing, non-homogeneously contrast-enhancing, 4 cm cerebellar mass was detected and underwent gross total resection . Histologic examination showed a high-grade “blue” embryonal tumor exhibiting necrosis and vascular proliferation. The neoplastic cells showed hyperchromatic pleomorphic nuclei displaying molding and numerous mitotic figures, in average 22 per high-power field . IHC showed focal synaptophysin and GFAP, strong diffuse nuclear p53, patchy nuclear Olig2 expression, and a very high Ki-67 proliferation index, over 70%. The two IHC markers that are usually positive in the MB-SHH tumors, YAP and GAB1, showed an unusual pattern with patchy strong expression, including nuclear, for YAP, and weak and very focal expression for GAB1. The tumor was diagnosed as large cell/anaplastic histologic variant, suspicious for MB-SHH/TP53-mutant molecular subgroup, pending molecular characterization. Based on anaplastic histology, the tumor was treated as high-risk MB, with craniospinal irradiation (54 CGE in 30 fractions over 6 weeks) and concomitant weekly vincristine, followed by chemotherapy (6 × 28-day cycles of vincristine, cyclophosphamide, cisplatin). The patient was tumor-free for 10 months after therapy completion, being treated only for panhypopituitarism, when he developed a third, right posterior fossa, dural, bone-lytic mass . Histologic examination showed a high-grade pleomorphic sarcoma with very high mitotic index (30 mitoses per 10 high-power fields) and numerous atypical mitotic figures , suggestive for a radiation-induced sarcoma. IHC showed positive staining in subsets of neoplastic cells for h-caldesmon and CD163, weak for SMA, and strong labeling of multinucleated osteoclast-like giant cells by CD68 ; negative stains included desmin, myogenin, S100 and EMA. The differential diagnosis of pleomorphic leiomyosarcoma versus undifferentiated pleomorphic sarcoma was formulated for this high-grade sarcoma, and a methylation array test performed for further tumor classification was non-contributory. Fig. 2 LFS MB and sarcoma progressing after radiotherapy for atypical prolactinoma. A MRI showing a macroadenoma compressing the optic chiasm and the floor of the 3rd ventricle. B H&E of the macroadenoma shows patchy lymphocytic inflammatory infiltrates (blue arrow), mitotic figures (green arrowhead) and glandular structures (red arrow). C , D IHC with indicated antibodies distinguishes the neoplastic cells from non-neoplastic inflammatory infiltrates (blue arrows) and glands (red arrows). Note NHERF1 labeling of glandular epithelial apical membrane and small T lymphocytes. The neoplastic cells show Cam 5.2 diffuse and strong cytoplasmic staining, synaptophysin and prolactin diffuse peripheral staining (insets with magnification), negative estrogen receptor (ER) staining, increased Ki-67 proliferation index and lack of p53 diffuse staining. E Timeline of tumor progression and treatment for the LFS M6 patient. Red rhomboid arrows indicate intracranial surgeries; green arrows (up – growth; down – decrease) indicate tumor progression observed on MRI (MRI progr); yrs, years; RT, radiotherapy (proton beam therapy); chemo, chemotherapy. F MRI of the two posterior fossa masses: left hemispheric cerebellar mass, and right dural mass (red arrow). G H&E and IHC with p53 antibody of the cerebellar mass show the large cell/anaplastic MB variant. H High-grade pleomorphic sarcoma shows numerous atypical mitotic figures (green arrowheads) and multinucleated osteoclast-like giant cells (blue arrows)	4.277344	0.935059	sec[2]/sec[0]/sec[1]/p[0]	en	0.999997	PMC9382778	https://doi.org/10.1186/s12964-022-00930-3	[ "tumor", "cells", "mitotic", "staining", "histologic", "sarcoma", "figures", "prolactinoma", "proliferation", "strong" ]	[ { "code": "2F9Z", "title": "Neoplasms of unknown behaviour of unspecified site" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "2E6Z", "title": "Carcinoma in situ of unspecified site" }, { "code": "2F91.1", "title": "Neoplasms of unknown behaviour of trachea, bronchus or lung" }, { "code": "2F92", "title": "Neoplasms of unknown behaviour of skin" }, { "code": "MF9Y", "title": "Other specified clinical findings on examination of urine, without diagnosis" }, { "code": "5C56.20", "title": "Mucolipidosis" }, { "code": "3A51.1", "title": "Sickle cell disease without crisis" }, { "code": "9A96.3", "title": "Primary anterior uveitis" }, { "code": "3A61.Z", "title": "Acquired pure red cell aplasia, unspecified" } ]	=== ICD-11 CODES FOUND === [2F9Z] Neoplasms of unknown behaviour of unspecified site Also known as: Neoplasms of unknown behaviour of unspecified site \| neoplasia \| neoplasm growth NOS \| tumour of unspecified site \| tumourlet of unspecified site [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [2E6Z] Carcinoma in situ of unspecified site Also known as: Carcinoma in situ of unspecified site \| carcinoma in situ of unspecified site \| carcinoma in situ NOS \| carcinoma in situ \| in situ neoplasm [2F91.1] Neoplasms of unknown behaviour of trachea, bronchus or lung Also known as: Neoplasms of unknown behaviour of trachea, bronchus or lung \| trachea, bronchus or lung tumour NOS \| Intravascular bronchial alveolar tumour of unspecified site \| Bronchial adenoma of unknown behaviour of unspecified site \| Intravascular bronchial alveolar tumour unknown behaviour of unspecified site [2F92] Neoplasms of unknown behaviour of skin Also known as: Neoplasms of unknown behaviour of skin \| skin tumour NOS [MF9Y] Other specified clinical findings on examination of urine, without diagnosis Also known as: Other specified clinical findings on examination of urine, without diagnosis \| Methaemoglobinuria \| Other and unspecified abnormal findings in urine \| Calciuria \| Cells and casts in urine [5C56.20] Mucolipidosis Also known as: Mucolipidosis \| Mucolipidosis type 3 \| Pseudo-Hurler polydystrophy \| Pseudo-Hurler disease \| Mucolipidosis type 2 Excludes: Sialidosis (mucolipidosis type 1) [3A51.1] Sickle cell disease without crisis Definition: A disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may present with fatigue, shortness of breath, dizziness, headaches, pallor of skin or mucous membranes, and jaundice. This disorder is confirmed by identification of HbS mutation by genetic testing. Also known as: Sickle cell disease without crisis \| Hb-SS disease without crisis \| HbSS without crisis \| Sickle-cell anaemia without crisis \| SCD - [sickle cell disease] [9A96.3] Primary anterior uveitis Definition: This refers to primary inflammation of the uvea. The uvea consists of the middle, pigmented, vascular structures of the eye and includes the iris, ciliary body, and choroid. Also known as: Primary anterior uveitis \| anterior chamber cell [3A61.Z] Acquired pure red cell aplasia, unspecified Also known as: Acquired pure red cell aplasia, unspecified \| Acquired pure red cell aplasia \| acquired red cell aplasia \| red cell aplasia NOS \| pure red cell aplastic anaemia === GRAPH WALKS === --- Walk 1 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour --- Walk 2 --- [2F9Z] Neoplasms of unknown behaviour of unspecified site --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2F91] Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs --- Walk 3 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Enlarged lymph nodes Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes.... --PARENT--> [?] General symptoms --- Walk 4 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Localised adiposity Def: A condition characterised by accumulation of adipose tissue in specific regions of the body.... --CHILD--> [?] Benign symmetrical lipomatosis Def: Benign symmetrical lipomatosis is an uncommon condition characterised by progressive symmetrical accumulation during adult life of adipose mass at the level of the head, neck and upper trunk . The con... --- Walk 5 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs --- Walk 6 --- [2E6Z] Carcinoma in situ of unspecified site --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues --PARENT--> [02] Neoplasms Def: An abnormal or uncontrolled cellular proliferation which is not coordinated with an organism's requirements for normal tissue growth, replacement or repair....	[ "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --RELATED_TO--> [?] Pathological fracture in neoplastic disease of unknown behaviour", "[2F9Z] Neoplasms of unknown behaviour of unspecified site\n --PARENT--> [?] Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2F91] Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Enlarged lymph nodes\n Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes....\n --PARENT--> [?] General symptoms", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Localised adiposity\n Def: A condition characterised by accumulation of adipose tissue in specific regions of the body....\n --CHILD--> [?] Benign symmetrical lipomatosis\n Def: Benign symmetrical lipomatosis is an uncommon condition characterised by progressive symmetrical accumulation during adult life of adipose mass at the level of the head, neck and upper trunk . The con...", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --CHILD--> [2E61] Carcinoma in situ of other or unspecified digestive organs", "[2E6Z] Carcinoma in situ of unspecified site\n --PARENT--> [?] In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues\n --PARENT--> [02] Neoplasms\n Def: An abnormal or uncontrolled cellular proliferation which is not coordinated with an organism's requirements for normal tissue growth, replacement or repair...." ]	2F9Z	Neoplasms of unknown behaviour of unspecified site	[ { "from_icd11": "2F9Z", "icd10_code": "D487", "icd10_title": "Neoplasm of uncertain behavior of other specified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D482", "icd10_title": "Neoplasm of uncertain behavior of peripheral nerves and autonomic nervous system" }, { "from_icd11": "2F9Z", "icd10_code": "D37-D48", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D377", "icd10_title": "" }, { "from_icd11": "2F9Z", "icd10_code": "D48", "icd10_title": "Neoplasm of uncertain behavior of other and unspecified sites" }, { "from_icd11": "2F9Z", "icd10_code": "D489", "icd10_title": "Neoplasm of uncertain behavior, unspecified" }, { "from_icd11": "ME61", "icd10_code": "R2240", "icd10_title": "Localized swelling, mass and lump, unspecified lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2232", "icd10_title": "Localized swelling, mass and lump, left upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2242", "icd10_title": "Localized swelling, mass and lump, left lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2231", "icd10_title": "Localized swelling, mass and lump, right upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2241", "icd10_title": "Localized swelling, mass and lump, right lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2233", "icd10_title": "Localized swelling, mass and lump, upper limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2243", "icd10_title": "Localized swelling, mass and lump, lower limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2230", "icd10_title": "Localized swelling, mass and lump, unspecified upper limb" }, { "from_icd11": "ME61", "icd10_code": "R220", "icd10_title": "Localized swelling, mass and lump, head" } ]	D487	Neoplasm of uncertain behavior of other specified sites
A 64-year-old female patient, with a medical history of axial spondylarthropathy, diabetes mellitus type II, hypertension, morbid obesity and laparoscopic Roux en Y bypass, received a left TKA in 2015 for end-stage osteoarthritis. The surgical procedure and follow-up were uneventful. In May 2021, the patient returned with pain in both shoulders, upper arms, the right wrist and, most prominently, in the left knee. She reported that she had felt ill for a day and had 39 degrees fever nine days before she visited the hospital. The physical examination showed a non-acute ill patient with a large swelling of the left knee, and a heavily impaired function with a flexion of 50 degrees, and extension deficit of 30 degrees. The blood examination showed a C-reactive protein (CRP) of 156 mg/L and a white blood cell count (WBC) of 13.1 × 10 9 /L per liter. A sterile puncture was performed and samples were taken and analyzed for gram stain, culture and uric crystals, and blood cultures were taken. A rheumatologist was consulted and the aspirate of the knee was analyzed for uric crystals, which were not found. The gram staining was negative but was positive for leukocytes. Initially, the clinical diagnosis was made of reactive arthritis due to systemic infection with an unknown primary location. The patient was hospitalized for close monitoring, while antibiotics were withheld. On the second day of admission, the blood cultures turned positive for a gram-negative rod. Intravenous cefuroxime was started at 1.5 g four times a day. Due to minor clinical improvement with a decrease in knee pain and a drop of CRP to 120 mg/L, no surgery was performed the second day. The third day after admission, the CRP, however, rose to 149 mg/L again, and the patient reported more pain in the knee. Therefore, the decision was made to perform a Debridement, Antibiotics and Implant Retention (DAIR) procedure. Preoperatively, two grams of cefazolin was administered to prevent secondary infections. An open arthrotomy was performed. Perioperatively, there was no collection of pus but cloudy synovium was observed. Four cultures were taken of synovial fluid, periprosthetic tissue, joint capsule and interface tissue around the prosthesis, each with a clean instrument. The prosthesis insert could not be removed due to unavailability of the insert because this type of prosthesis was discontinued by the manufacturer. If it had been available, the insert would have been exchanged for a new insert. Extensive debridement, with all “suspicious” tissues excised, was performed, by using a diathermic knife. The prosthesis and surrounding tissues were then brushed manually with a scrubbing sponge and povidone-iodine for at least two minutes. This was followed by extensive flushing of the knee by pulsating lavage with six liters of 0.9% saline solution. Subsequently, a new extremity drape was placed around the knee, and gloves were changed and the knee was closed in layers using new instruments. Immediately after surgery, the patient improved clinically. A trans-esophageal ultrasound was performed on the request of the infectious disease specialist and showed no evidence of infectious endocarditis. The blood cultures (BD Bactec) became positive after two incubation days and they were cultured aerobically and anaerobically on solid media. The bacteria were seen after five days on blood agar with 5% sheep blood and on chocolate agar media. A Vitek MS matrix-assisted laser desorption/ionization time-of-flight mass spectrometer (MALDITOF) (bioMérieux) was used based on mass spectrometry principle for the identification of the grown colonies, but the identification failed repeatedly. The identification with Vitek MS MALDITOF (bioMérieux) depends on the database of the analyzer which can impose limitations. For this reason, molecular diagnostics using the 16S rRNA gene polymerase chain reaction technique (PCR) were chosen to help the identification and eventually S. moniliformis was found. The knee aspirate was also cultured and after 7 days of incubation. S. moniliformis was also identified in the knee aspirate by 16S rRNA gene sequencing. Unfortunately, the susceptibility of S. moniliformis could not be determined despite all the efforts to use microaerophilic conditions and Schaedler anaerobe agar with sheep blood with haemin and vitamin K1 as advised in the literature . Knowing the etiological agent of bacteremia, the antibiotics were switched to ceftriaxone based upon literature review . Supplementary history taking was performed to trace the origin of the infection. The patient reported having no memory of a rat bite, nor living close to rats, nor living close to open water or having eaten spoiled food. She reported having two dogs. Possibly the dogs could have been infected by S. moniliformis by either eating rats, being bitten by rats or by swimming in water polluted by rat feces. Therefore, both dogs were swabbed and samples were taken of their saliva. S. moniliformis could not be cultured and recovered from the dogs. Sixteen days after the DAIR, the patient could be discharged home with intravenous antibiotics in good clinical condition. The CRP at time of discharge was 20 mg/L and the WBC was normal at 3.9 × 10 9 /L. A total antibiotic regimen of twelve weeks was planned according to clinical guidelines . After multidisciplinary consultation with a medical microbiologist, an infectious diseases specialist and an orthopedic surgeon, the following antibiotic regimen was agreed upon: six weeks intravenous ceftriaxone two grams once daily because of bacteremia and another six weeks of oral doxycycline 200 mg once daily to complete the treatment of the PJI . Due to penicillin allergy confirmed with skin testing, the patient could not be treated with oral amoxicillin, which normally would be the oral antibiotic of choice. At three, six and thirteen weeks after surgery, the patient was seen for follow-up. At the most recent follow-up at thirteen weeks when the patient was one week after the last administration of doxycycline, the pain had diminished. The function of the knee was improved to normal walking with a flexion of 120 degrees and full extension to zero degrees. Laboratory results showed CRP < 1 mg/L and WBC of 2.9 × 10 9 /L.	3.878906	0.980957	sec[1]/p[0]	en	0.999997	PMC8976379	https://doi.org/10.1186/s42836-022-00114-x	[ "knee", "blood", "degrees", "moniliformis", "pain", "cultures", "antibiotics", "prosthesis", "insert", "identification" ]	[ { "code": "FA2Z", "title": "Inflammatory arthropathies, unspecified" }, { "code": "NC90.Y", "title": "Other specified superficial injury of knee or lower leg" }, { "code": "FA34.4", "title": "Ankylosis of joint" }, { "code": "FA33.4Z", "title": "Chronic instability of knee, unspecified" }, { "code": "NC90.0", "title": "Abrasion of knee" }, { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" }, { "code": "MA12.1", "title": "Finding of cocaine in blood" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "MA12.2", "title": "Finding of hallucinogen in blood" } ]	=== ICD-11 CODES FOUND === [FA2Z] Inflammatory arthropathies, unspecified Also known as: Inflammatory arthropathies, unspecified \| polyarthritis NOS \| inflammatory joint disease NOS \| nonpyogenic arthritis NOS \| arthritic nodosa [NC90.Y] Other specified superficial injury of knee or lower leg Also known as: Other specified superficial injury of knee or lower leg \| Nonthermal blister of other or unspecified parts of lower leg \| Nonvenomous insect bite of other or unspecified parts of lower leg \| Superficial foreign body in other or unspecified parts of lower leg \| Splinter in other or unspecified parts of lower leg [FA34.4] Ankylosis of joint Definition: The term ankylosis denotes restricted movement in the joint, and it can be bony or fibrous. Most cases are caused by trauma, infection, radiotherapy, or severe arthritic condition. Also known as: Ankylosis of joint \| ankylosis \| ankylosis of joint, site unspecified \| frozen joint \| fusion of joint Excludes: stiffness of joint without ankylosis \| Ankylosis of spinal joint [FA33.4Z] Chronic instability of knee, unspecified Also known as: Chronic instability of knee, unspecified \| Chronic instability of knee \| instability of knee \| old disruption of ligament of knee [NC90.0] Abrasion of knee Also known as: Abrasion of knee [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS [MA12.1] Finding of cocaine in blood Also known as: Finding of cocaine in blood \| cocaine in blood [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [MA12.2] Finding of hallucinogen in blood Also known as: Finding of hallucinogen in blood \| hallucinogen in blood === GRAPH WALKS === --- Walk 1 --- [FA2Z] Inflammatory arthropathies, unspecified --PARENT--> [?] Inflammatory arthropathies --CHILD--> [FA22] Polymyalgia rheumatica Def: Polymyalgia rheumatica (PMR) is a syndrome characterised by aching of the proximal portions of the extremities and torso. Provisional classification criteria for PMR by the European League Against Rhe... --- Walk 2 --- [FA2Z] Inflammatory arthropathies, unspecified --PARENT--> [?] Inflammatory arthropathies --RELATED_TO--> [?] Peripheral spondyloarthritis Def: Experts from the Assessment of SpondyloArthritis international Society (ASAS) developed classification criteria for axSpA and peripheral SpA. These criteria were developed for patients with peripheral... --- Walk 3 --- [NC90.Y] Other specified superficial injury of knee or lower leg --PARENT--> [NC90] Superficial injury of knee or lower leg --PARENT--> [?] Injuries to the knee or lower leg --- Walk 4 --- [NC90.Y] Other specified superficial injury of knee or lower leg --PARENT--> [NC90] Superficial injury of knee or lower leg --CHILD--> [NC90.2] Abrasion of other or unspecified parts of lower leg --- Walk 5 --- [FA34.4] Ankylosis of joint Def: The term ankylosis denotes restricted movement in the joint, and it can be bony or fibrous. Most cases are caused by trauma, infection, radiotherapy, or severe arthritic condition.... --EXCLUDES--> [?] Stiffness of joint Def: Lack of range of motion of a joint secondary to pain, disease process or congenital malformation not detailed in or used in conjunction with other codes.... --CHILD--> [?] Stiffness of joint, multiple sites --- Walk 6 --- [FA34.4] Ankylosis of joint Def: The term ankylosis denotes restricted movement in the joint, and it can be bony or fibrous. Most cases are caused by trauma, infection, radiotherapy, or severe arthritic condition.... --PARENT--> [FA34] Certain specified joint derangements --CHILD--> [FA34.2] Recurrent instability of joint	[ "[FA2Z] Inflammatory arthropathies, unspecified\n --PARENT--> [?] Inflammatory arthropathies\n --CHILD--> [FA22] Polymyalgia rheumatica\n Def: Polymyalgia rheumatica (PMR) is a syndrome characterised by aching of the proximal portions of the extremities and torso. Provisional classification criteria for PMR by the European League Against Rhe...", "[FA2Z] Inflammatory arthropathies, unspecified\n --PARENT--> [?] Inflammatory arthropathies\n --RELATED_TO--> [?] Peripheral spondyloarthritis\n Def: Experts from the Assessment of SpondyloArthritis international Society (ASAS) developed classification criteria for axSpA and peripheral SpA. These criteria were developed for patients with peripheral...", "[NC90.Y] Other specified superficial injury of knee or lower leg\n --PARENT--> [NC90] Superficial injury of knee or lower leg\n --PARENT--> [?] Injuries to the knee or lower leg", "[NC90.Y] Other specified superficial injury of knee or lower leg\n --PARENT--> [NC90] Superficial injury of knee or lower leg\n --CHILD--> [NC90.2] Abrasion of other or unspecified parts of lower leg", "[FA34.4] Ankylosis of joint\n Def: The term ankylosis denotes restricted movement in the joint, and it can be bony or fibrous. Most cases are caused by trauma, infection, radiotherapy, or severe arthritic condition....\n --EXCLUDES--> [?] Stiffness of joint\n Def: Lack of range of motion of a joint secondary to pain, disease process or congenital malformation not detailed in or used in conjunction with other codes....\n --CHILD--> [?] Stiffness of joint, multiple sites", "[FA34.4] Ankylosis of joint\n Def: The term ankylosis denotes restricted movement in the joint, and it can be bony or fibrous. Most cases are caused by trauma, infection, radiotherapy, or severe arthritic condition....\n --PARENT--> [FA34] Certain specified joint derangements\n --CHILD--> [FA34.2] Recurrent instability of joint" ]	FA2Z	Inflammatory arthropathies, unspecified	[ { "from_icd11": "FA2Z", "icd10_code": "M1389", "icd10_title": "Other specified arthritis, multiple sites" }, { "from_icd11": "FA2Z", "icd10_code": "M1380", "icd10_title": "Other specified arthritis, unspecified site" }, { "from_icd11": "FA2Z", "icd10_code": "M13862", "icd10_title": "Other specified arthritis, left knee" }, { "from_icd11": "FA2Z", "icd10_code": "M13872", "icd10_title": "Other specified arthritis, left ankle and foot" }, { "from_icd11": "FA2Z", "icd10_code": "M13871", "icd10_title": "Other specified arthritis, right ankle and foot" }, { "from_icd11": "FA2Z", "icd10_code": "M13861", "icd10_title": "Other specified arthritis, right knee" }, { "from_icd11": "FA2Z", "icd10_code": "M13879", "icd10_title": "Other specified arthritis, unspecified ankle and foot" }, { "from_icd11": "FA2Z", "icd10_code": "M13842", "icd10_title": "Other specified arthritis, left hand" }, { "from_icd11": "FA2Z", "icd10_code": "M13841", "icd10_title": "Other specified arthritis, right hand" }, { "from_icd11": "FA2Z", "icd10_code": "M13811", "icd10_title": "Other specified arthritis, right shoulder" }, { "from_icd11": "FA2Z", "icd10_code": "M13162", "icd10_title": "Monoarthritis, not elsewhere classified, left knee" }, { "from_icd11": "FA2Z", "icd10_code": "M13869", "icd10_title": "Other specified arthritis, unspecified knee" }, { "from_icd11": "FA2Z", "icd10_code": "M1388", "icd10_title": "Other specified arthritis, other site" }, { "from_icd11": "FA2Z", "icd10_code": "M13171", "icd10_title": "Monoarthritis, not elsewhere classified, right ankle and foot" }, { "from_icd11": "FA2Z", "icd10_code": "M13152", "icd10_title": "Monoarthritis, not elsewhere classified, left hip" } ]	M1389	Other specified arthritis, multiple sites
A 70-year-old Japanese man who complained of dysphagia was admitted to our hospital. He had a medical history of using medications for type 2 diabetes mellitus, percutaneous coronary intervention for coronary artery disease, and radiotherapy for laryngeal cancer. Upper gastrointestinal endoscopy revealed an ulcerative and infiltrative type of tumor in the middle of the thoracic esophagus. Chest computed tomography (CT) scan did not reveal the presence of any lymph nodes and distant metastases. The diagnosis was T3N0M0 esophageal squamous cell cancer. The patient received two courses of adjuvant chemotherapy comprising 5-fluorouracil (5-FU) and docetaxel plus cisplatin (DCF regimen; 5-FU 600 mg/m 2 : days 1–5, docetaxel 60 mg/m 2 : day 1, and cisplatin 60 mg/body: day 1). Thoracoscopic esophagectomy, gastric tube reconstruction via the retrosternal route, and cervical esophagogastrostomy with circular-stapled end-to-side anastomosis were performed. On the 4th postoperative day (POD), flare, pus discharge, and saliva outflow from the cervical wound were observed, and cervicotomy with a wide opening was performed. Upper gastrointestinal endoscopy revealed a partial defect in the necrotic wall of the gastric tube at the anastomosis, and air bubbles were appearing in the cervical wound. The patient was initially treated conservatively with drainage tube placement and antibiotic intravenous (i.v.) administration of tazobactam/piperacillin (4.5 g) three times a day, for establishing of a controlled infection around the anastomotic leakage. The patient received nutritional management with a combination of central venous and tube feeding, and blood glucose levels were controlled with insulin administration. Body weight and serum albumin levels were 50 kg and 3.1 g/dL on the 30th POD and recovered to 50.5 kg and 3.4 g/dL after 4 weeks, respectively. At the same time, HbA1c was unchanged from the preoperative level (6.6%) and serum C-reactive protein was close to normal level. However, the patient developed refractory anastomotic fistula, and Enterobacter cloacae was isolated from the culture of the cervical wound. On the 60th day following the initial surgery, the left sternoclavicular joint was partially resected to widen the surgical field, and necrotized tissue debridement and free jejunal autograft transfer were performed as salvage reconstruction. The jejunum was transected at the level of the second jejunal vessel, and was joined to the distal end of the cervical esophagus and the proximal end of the residual gastric tube in an end-to-end anastomosis. The jejunal artery and vein were anastomosed to the right transverse cervical artery and the right internal jugular vein, respectively. Further, the free jejunal autograft was covered with the pectoralis major muscle flap . In addition, a blood-rich pectoralis major muscle flap and two suction drain placements eliminated dead space to prevent osteomyelitis. A feeding jejunostomy tube was placed for nutrition. However, on the 15th day following the second surgery, the patient complained of gradual worsening of lumbago. Upon clinical examination, the patient was febrile a temperature of 38.2 °C, and he was experiencing pain in the lumbar spinal area. Neurological examination revealed weakness in both the legs. However, the sensibility and perception of the patient did not change. The following laboratory examination results were obtained: white blood cell count (WBC) count 9200 /μL and C-reactive protein (CRP) level 9.86 mg/dL (normal range < 0.03 mg/dL). The presence of inflammatory foci was not observed on chest radiography and abdominal ultrasonography. E. cloacae was isolated from the arterial blood cultures. Magnetic resonance imaging (MRI) of the lumbar spine at the L4–5 revealed an obvious decrease in the signal intensity on T1-weighted images and an increase in the signal intensity on T2-weighted and short tau inversion recovery (STIR) images, as confirmed based on the typical appearance of pyogenic spondylodiscitis . The infection might be attributed to the hematogenous spread of the infection from the refractory anastomotic fistula in the neck. After intensive treatment with intravenous antibiotics, tazobactam/piperacillin 4.5 g (i.v.) three times a day for 4 weeks and cefepime 1 g (i.v.) twice a day for the succeeding 4 weeks, oral antibiotic (trimethoprim and sulfamethoxazole 4 g/day for the last 4 weeks) was administered until the levels of the laboratory markers, including WBC and CRP, normalized. Spinal immobilization via lumbar fixation using a corset was continued. The patient’s lumbago and pyrexia gradually diminished, and the CRP and WBC levels decreased. Oral intake was started on the 14th POD, which was the time when the anastomotic fistula closed. The patient took small amounts of food orally. However, he remained dependent on the feeding jejunostomy for most of his nutritional intake. After 12 weeks of antibiotic administration, the patient was free from pain, and no signs of infection were observed. Thus, the administration of antibiotics was discontinued. The patient was discharged from our hospital and was receiving maintenance therapy for cancer. Eventually, he recovered. However, pleural disseminations were observed on the chest CT scan that was conducted during the follow-up 2 years after the initial surgery. The patient died 50 months after the initial surgery. However, the symptoms of infectious and neurological disorders did not recur until the patient’s death. Fig. 1 a Refractory anastomotic fistula was observed on the 60th day after esophagectomy. b Debridement of the necrotized tissues was performed. c Salvage reconstruction with the free jejunal autograft (arrows) was performed. The jejunal artery and vein were anastomosed to the right transverse cervical artery and the right internal jugular vein, respectively. d The pectoralis major muscle flap (arrows) was used. These figures have been previously published Fig. 2 Magnetic resonance images. T1-weighted image ( a ), T2-weighted image ( b ), and STIR image ( c ) revealed pathological changes in the L4 and L5. The signal intensity in the diseased area was low on the T1-weighted image ( a , arrowheads) and high on the T2-weighted and STIR images ( b , c )	3.933594	0.977051	sec[1]/p[0]	en	0.999997	32607876	https://doi.org/10.1186/s40792-020-00922-w	[ "cervical", "tube", "however", "jejunal", "weighted", "artery", "anastomotic", "administration", "infection", "blood" ]	[ { "code": "GA04", "title": "Cervicitis" }, { "code": "GA1Z&XA5WW1", "title": "Noninflammatory disorders of cervix uteri" }, { "code": "FB1Y", "title": "Other specified conditions associated with the spine" }, { "code": "GA04&XT5R", "title": "Acute cervicitis" }, { "code": "GA04&XT8W", "title": "Chronic cervicitis" }, { "code": "GA07.Z&XA3EF0", "title": "Inflammatory disease of fallopian tube" }, { "code": "JA01.1", "title": "Tubal pregnancy" }, { "code": "GB90.Y", "title": "Other specified disorders of kidney or ureter" }, { "code": "GB54", "title": "Tubulo-interstitial nephritis, not specified as acute or chronic" }, { "code": "AB10.Z", "title": "Disorders of Eustachian tube, unspecified" } ]	=== ICD-11 CODES FOUND === [GA04] Cervicitis Also known as: Cervicitis \| inflammation of cervix \| inflammation of cervix uteri \| Ulcer of cervix with cervicitis \| Acute cervicitis [FB1Y] Other specified conditions associated with the spine Also known as: Other specified conditions associated with the spine \| Other recurrent vertebral subluxation \| Interspinous ligament syndrome \| Spondylitis muscularis \| Posterior longitudinal ligament calcification [JA01.1] Tubal pregnancy Definition: A condition characterised by implantation of the embryo within the fallopian tube (ampullary, isthmus, interstitium) during pregnancy. Also known as: Tubal pregnancy \| Fallopian pregnancy \| fallopian tube pregnancy \| Tubal abortion \| Rupture of fallopian tube due to pregnancy Includes: Fallopian pregnancy \| Tubal abortion [GB90.Y] Other specified disorders of kidney or ureter Also known as: Other specified disorders of kidney or ureter \| Other secondary disorders of kidney or ureter \| Other disorders of kidney and ureter NEC \| Inflammatory diseases of the renal pelvis or the ureter without tubulo-interstitial nephritis \| Infectious diseases of the renal pelvis or the ureter without tubulo-interstitial nephritis [GB54] Tubulo-interstitial nephritis, not specified as acute or chronic Definition: A disease characterised by inflammation of and damage to tubules or the interstitium of the kidney while sparing the glomeruli secondary to immune reaction or toxic agent. Also known as: Tubulo-interstitial nephritis, not specified as acute or chronic \| tubulo-interstitial nephritis \| renal disease with interstitial nephritis \| Congenital pyelitis \| Cystopyelitis Excludes: calculous pyelonephritis [AB10.Z] Disorders of Eustachian tube, unspecified Also known as: Disorders of Eustachian tube, unspecified \| Disorders of Eustachian tube \| auditory tube disorder \| disease of Eustachian tube \| Eustachian tube dysfunction === GRAPH WALKS === --- Walk 1 --- [GA04] Cervicitis --PARENT--> [?] Inflammatory disorders of the female genital tract --EXCLUDES--> [?] Infections of genitourinary tract in pregnancy --- Walk 2 --- [GA04] Cervicitis --RELATED_TO--> [?] Tuberculosis of cervix uteri Def: This is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. This diagnosis is of the cervix uteri.... --PARENT--> [?] Tuberculosis of female reproductive organs Def: This is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. This diagnosis is of the female reproductive organs.... --- Walk 3 --- [FB1Y] Other specified conditions associated with the spine --PARENT--> [?] Conditions associated with the spine Def: This is a group of conditions in which there is a deviation from or interruption of the normal structure or function of the spine.... --CHILD--> [?] Inflammation of spine --- Walk 4 --- [FB1Y] Other specified conditions associated with the spine --PARENT--> [?] Conditions associated with the spine Def: This is a group of conditions in which there is a deviation from or interruption of the normal structure or function of the spine.... --CHILD--> [?] Degenerative condition of spine Def: This is a disease characterised by degenerative changes in the intervertebral disc, vertebral end-plates and spinal joints due to aging or structural change....	[ "[GA04] Cervicitis\n --PARENT--> [?] Inflammatory disorders of the female genital tract\n --EXCLUDES--> [?] Infections of genitourinary tract in pregnancy", "[GA04] Cervicitis\n --RELATED_TO--> [?] Tuberculosis of cervix uteri\n Def: This is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. This diagnosis is of the cervix uteri....\n --PARENT--> [?] Tuberculosis of female reproductive organs\n Def: This is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. This diagnosis is of the female reproductive organs....", "[FB1Y] Other specified conditions associated with the spine\n --PARENT--> [?] Conditions associated with the spine\n Def: This is a group of conditions in which there is a deviation from or interruption of the normal structure or function of the spine....\n --CHILD--> [?] Inflammation of spine", "[FB1Y] Other specified conditions associated with the spine\n --PARENT--> [?] Conditions associated with the spine\n Def: This is a group of conditions in which there is a deviation from or interruption of the normal structure or function of the spine....\n --CHILD--> [?] Degenerative condition of spine\n Def: This is a disease characterised by degenerative changes in the intervertebral disc, vertebral end-plates and spinal joints due to aging or structural change...." ]	GA04	Cervicitis	[ { "from_icd11": "FB1Y", "icd10_code": "M5126", "icd10_title": "Other intervertebral disc displacement, lumbar region" }, { "from_icd11": "JA01.1", "icd10_code": "O00102", "icd10_title": "Left tubal pregnancy without intrauterine pregnancy" }, { "from_icd11": "JA01.1", "icd10_code": "O0010", "icd10_title": "Tubal pregnancy without intrauterine pregnancy" }, { "from_icd11": "JA01.1", "icd10_code": "O00101", "icd10_title": "Right tubal pregnancy without intrauterine pregnancy" }, { "from_icd11": "JA01.1", "icd10_code": "O00111", "icd10_title": "Right tubal pregnancy with intrauterine pregnancy" }, { "from_icd11": "JA01.1", "icd10_code": "O001", "icd10_title": "Tubal pregnancy" }, { "from_icd11": "GB54", "icd10_code": "N12", "icd10_title": "Tubulo-interstitial nephritis, not specified as acute or chronic" }, { "from_icd11": "AB10.Z", "icd10_code": "H6991", "icd10_title": "Unspecified Eustachian tube disorder, right ear" }, { "from_icd11": "AB10.Z", "icd10_code": "H6981", "icd10_title": "Other specified disorders of Eustachian tube, right ear" }, { "from_icd11": "AB10.Z", "icd10_code": "H6980", "icd10_title": "Other specified disorders of Eustachian tube, unspecified ear" }, { "from_icd11": "AB10.Z", "icd10_code": "H6982", "icd10_title": "Other specified disorders of Eustachian tube, left ear" }, { "from_icd11": "AB10.Z", "icd10_code": "H6990", "icd10_title": "Unspecified Eustachian tube disorder, unspecified ear" }, { "from_icd11": "AB10.Z", "icd10_code": "H68", "icd10_title": "Eustachian salpingitis and obstruction" }, { "from_icd11": "AB10.Z", "icd10_code": "H69", "icd10_title": "Other and unspecified disorders of Eustachian tube" }, { "from_icd11": "AB10.Z", "icd10_code": "H698", "icd10_title": "Other specified disorders of Eustachian tube" } ]	M5126	Other intervertebral disc displacement, lumbar region
A 37-year-old Chinese woman (gravida 4, para 1) with a regular menstrual cycle presented to her local doctor with a complaint of mild abdominal distension. Abdominal ultrasonography suggested an ovarian cyst with an increasing mean diameter from 3 to 8 cm in 3 months. Thereafter, she was referred to our hospital. She had no abnormal vaginal bleeding or discharge. She had a history of cesarean section 10 years ago and uterine myoma (diameter: ~2 cm) 3 years ago. Transvaginal ultrasound showed that the size of the uterus was 64 × 36 mm, and fibroids measuring 20 × 14 mm were found in the posterior wall. A 95 × 80 mm cyst was observed below the back of the uterus. The cyst had multiple partitions with viscous internal fluid. The left ovary was normal, and the right ovary was not visible . Three-phase enhanced whole abdomen computed tomography (CT) showed an 8.8 × 8.5 cm multilocular cyst between the cervix and right ovary . The radiologists considered that the cyst might have originated from the cervix, and no enhancement was observed around the cyst wall. Serum cancer antigen (CA)125, CA199, and carcinoembryonic antigen levels were within the normal range. Physical examination revealed that the exocervix was small and flat, with a very deep position due to the traction of adhesion after cesarean section. A large cyst was palpable at the Douglas fossa, and the uterus moved with the cyst. The appearance of the cervix on speculum examination was unremarkable. Human papillomavirus (HPV)-E6 and E7 mRNA tests showed HPV-16 positivity. A thin-layer liquid-based cytological test (TCT) of the cervix revealed negative findings. The patient with a high-risk HPV infection was diagnosed with no tumor lesions on cervical biopsy histopathology. A giant cyst of the cervix is rare, and although the CT report was assessed without detailed view of CT images, owing to the patient's long history of pelvic ultrasonography, the initial diagnosis was a right ovarian cyst and uterine myoma. The patient underwent laparoscopic surgery the day after admission. Intraoperatively, the bilateral ovaries and fallopian tubes were normal. A cyst measuring around a child's head was observed extending from the lower posterior wall of the uterus to the posterior lip of the cervix. The cyst surface was smooth and intact. A myoma with a diameter of 1.5 cm in the left posterior wall was observed. Since the cervical cyst did not show malignant growth patterns, we decided to strip the cyst and excise the uterine myoma. We first removed the hysteromyoma and sewed the muscle wall, and then cut the serous layer on the cyst surface layer by layer to expose the thin cyst wall. The boundary between the cyst and the surrounding cervical tissue was unclear. During stripping, the cyst ruptured, and the transparent and viscous cyst fluid flowed out. A few partitions were observed in the cyst. The root of the cyst was located at the posterior lip of the cervix and reached the internal orifice of the cervix. To completely remove the cyst tissue, we cut off the upper part of the cyst tissue in the abdominal cavity, and the root of the cyst was removed from the cervical canal through the vagina. The sample was sent for frozen section analysis, and abnormal glands were observed in the cervical tissues, which could be malignant. The uterus was sutured, and peritoneal lavage fluid was left for cytological examination. Histological examination revealed a cervical adenocarcinoma. The tumor invaded the smooth muscle fiber stroma without vessels and nerves. The uterine tumor was a leiomyoma. The carcinoma components comprised lobular, tubular, and villous growth patterns distributed in the capsule wall, smooth muscle, and fibrous tissue. The immunohistochemical staining results showed PAX-8, P16, and Ki-67 (50%) positivity and vimentin, ER, PR, and WT-1 negativity, further verifying the diagnosis of cervical adenocarcinoma . Pathological examination was performed at the Pathology Center of Peking University Third Hospital, and the results were consistent with those of HPV infection-related cervical adenocarcinoma. Cytological examination of peritoneal lavage fluid revealed no tumor cells. Pelvic magnetic resonance imaging revealed a suspicious lesion in the cervix. However, no ligament involvement was observed, except in the cervix . Her serum squamous cell carcinoma-related antigen (SCC) level was normal. After further discussion with the oncology team, exploratory laparotomy was performed with the intent of extensive hysterectomy, bilateral salpingectomy, and pelvic lymphadenectomy. After frozen sections of bilateral ovarian biopsies were found to be negative, we reserved the bilateral ovaries. The overall histological and histochemical features of the specimen obtained during laparotomy revealed no adenocarcinoma components in the resected specimen. No tumor was found in the uterus, bilateral fallopian tubes, or pelvic lymph nodes. Based on the two pathological results and cyst size, the final diagnosis was stage IB3 cervical adenocarcinoma according to the International Federation of Gynecology and Obstetrics staging system. Since all the tumors were located in the cervical cyst, the depth of invasion could not be determined. However, the cyst was 9 cm in diameter, protruded into the abdominal cavity, and ruptured intraoperatively. After a detailed discussion by gynecologists, medical oncologists, and radiologists, postoperative radiotherapy and chemotherapy were recommended to prevent recurrence. However, the patient refused radiotherapy because of the fear of ovarian dysfunction. The treatment included intravenous paclitaxel at 175 mg/m 2 administered over 3 h, followed by carboplatin at a dose giving an area under the time–concentration curve of 5 mg × min/mL (based on the calculated creatinine clearance) diluted in 500 mL of 5% glucose administered over 1 h. Both drugs were administered once every 3 weeks and two courses were administered. Follow-up visits every 3 months consisted of clinical assessment for any signs of recurrence, together with the HPV E6 and E7 mRNA and TCT at the vaginal stumps, CA125 test, SCC assay, and chest and pelvic CT. After 21 months of follow-up, no clinical or radiological evidence of recurrence was found.	3.955078	0.981445	sec[1]/p[0]	en	0.999997	PMC8959486	https://doi.org/10.3389/fsurg.2022.841255	[ "cyst", "cervix", "cervical", "wall", "uterus", "tumor", "pelvic", "adenocarcinoma", "abdominal", "ovarian" ]	[ { "code": "FB80.5", "title": "Solitary bone cyst" }, { "code": "EK70.Z", "title": "Cutaneous cysts, unspecified" }, { "code": "FB4Y", "title": "Other specified disorders of synovium or tendon" }, { "code": "CA0C", "title": "Cyst or mucocele of nose or nasal sinus" }, { "code": "9A7Y", "title": "Other specified disorders of the cornea" }, { "code": "GA1Z&XA5WW1", "title": "Noninflammatory disorders of cervix uteri" }, { "code": "GA04", "title": "Cervicitis" }, { "code": "GA15.1", "title": "Erosion or ectropion of cervix uteri" }, { "code": "JA01.Y", "title": "Other specified ectopic pregnancy" }, { "code": "GA15.7", "title": "Low grade squamous intraepithelial lesion of cervix uteri" } ]	=== ICD-11 CODES FOUND === [FB80.5] Solitary bone cyst Definition: A solitary bone cyst is a benign non-epithelial bone cavity that is asymptomatic and that is found most commonly in the second decade of life by chance. The long bones are most often affected, but cases involving the jaw bone have been reported. Also known as: Solitary bone cyst \| cyst of bone \| local cyst of bone \| simple bone cyst \| solitary bone cyst, unspecified site Excludes: solitary cyst of jaw [EK70.Z] Cutaneous cysts, unspecified Also known as: Cutaneous cysts, unspecified \| Cutaneous cysts \| Follicular cysts of skin and subcutaneous tissue [FB4Y] Other specified disorders of synovium or tendon Also known as: Other specified disorders of synovium or tendon \| Shortening of tendon \| short tendon \| Shortening of tibialis anterior \| Contracture of tendon [CA0C] Cyst or mucocele of nose or nasal sinus Definition: A condition which refers to diseases of the nose and nasal sinus that cause a cyst or mucocele. A mucocele is any dilatation (typically pathologic) with accumulation of mucus. Mucoceles are benign, epithelium-lined cysts filled with mucus, which can form in the paranasal sinuses. These structures may cause symptoms if sufficiently large or if exerting pressure on surrounding anatomic structures. Symptomatic mucoceles typically require surgical intervention. Mucoceles should be differentiated fro Also known as: Cyst or mucocele of nose or nasal sinus \| cyst of sinus \| mucocele of sinus \| Cyst of maxillary sinus \| cyst of maxillary antrum [9A7Y] Other specified disorders of the cornea Also known as: Other specified disorders of the cornea \| Secondary disorders of sclera or cornea \| Disorders of sclera and cornea in diseases classified elsewhere \| Secondary keratitis or keratoconjunctivitis \| Keratitis and keratoconjunctivitis in other diseases classified elsewhere [GA04] Cervicitis Also known as: Cervicitis \| inflammation of cervix \| inflammation of cervix uteri \| Ulcer of cervix with cervicitis \| Acute cervicitis [GA15.1] Erosion or ectropion of cervix uteri Definition: A condition of the cervix uteri, caused by an increase in the total estrogen level in the body. This condition is characterised by protrusion and transformation of the endocervical columnar epithelium to stratified squamous epithelium on the cervix uteri. This condition may also present with non-purulent vaginal discharge, post-coital bleeding, or may be asymptomatic. Also known as: Erosion or ectropion of cervix uteri \| Cervical ectropion \| cervical eversion \| eversion of cervix \| ectropion of cervix Excludes: Cervicitis [JA01.Y] Other specified ectopic pregnancy Also known as: Other specified ectopic pregnancy \| Cornual gestation or pregnancy \| cornual gestation \| cornual pregnancy \| Cervical pregnancy [GA15.7] Low grade squamous intraepithelial lesion of cervix uteri Definition: A condition of the cervix uteri caused by chronic infection. This condition is characterised by premalignant transformation and abnormal cell growth and behaviour of the cervical squamous epithelial tissue. Also known as: Low grade squamous intraepithelial lesion of cervix uteri \| cervical dysplasia \| cervical dysplasia nos \| intraepithelial neoplasia of cervix \| intraepithelial neoplasia of the cervix uteri Excludes: Carcinoma in situ of cervix uteri \| High grade squamous intraepithelial lesion of cervix uteri \| Cervical Intraepithelial neoplasia grade III === GRAPH WALKS === --- Walk 1 --- [FB80.5] Solitary bone cyst Def: A solitary bone cyst is a benign non-epithelial bone cavity that is asymptomatic and that is found most commonly in the second decade of life by chance. The long bones are most often affected, but cas... --PARENT--> [FB80] Certain specified disorders of bone density or structure --EXCLUDES--> [?] Osteopoikilosis --- Walk 2 --- [FB80.5] Solitary bone cyst Def: A solitary bone cyst is a benign non-epithelial bone cavity that is asymptomatic and that is found most commonly in the second decade of life by chance. The long bones are most often affected, but cas... --EXCLUDES--> [?] Other cysts of jaw Def: This is mostly odontogenic cysts but may be also of non-odontogenic source. The mandible and maxilla are the bones with the highest prevalent of cysts in the human body owing to odontogenic and develo... --CHILD--> [?] Aneurysmal cyst of jaw --- Walk 3 --- [EK70.Z] Cutaneous cysts, unspecified --PARENT--> [EK70] Cutaneous cysts --CHILD--> [EK70.0] Epidermoid cyst Def: A cutaneous cyst with an epidermoid wall filled with keratin and its breakdown products. It most commonly forms as the result of squamous metaplasia in a damaged sebaceous gland but may result from tr... --- Walk 4 --- [EK70.Z] Cutaneous cysts, unspecified --PARENT--> [EK70] Cutaneous cysts --CHILD--> [EK70.1] Trichilemmal cyst Def: A trichilemmal (pilar) cyst is a common, typically non-tender, intradermal or subcutaneous cyst. The cysts are typically confined to the scalp and are often multiple. They usually occur sporadically b... --- Walk 5 --- [FB4Y] Other specified disorders of synovium or tendon --PARENT--> [?] Disorders of synovium or tendon Def: This is a group of disorders which affect the synovial joint lining (synovium) and also tendons.... --PARENT--> [?] Soft tissue disorders --- Walk 6 --- [FB4Y] Other specified disorders of synovium or tendon --PARENT--> [?] Disorders of synovium or tendon Def: This is a group of disorders which affect the synovial joint lining (synovium) and also tendons.... --CHILD--> [FB42] Certain specified disorders of synovium or tendon	[ "[FB80.5] Solitary bone cyst\n Def: A solitary bone cyst is a benign non-epithelial bone cavity that is asymptomatic and that is found most commonly in the second decade of life by chance. The long bones are most often affected, but cas...\n --PARENT--> [FB80] Certain specified disorders of bone density or structure\n --EXCLUDES--> [?] Osteopoikilosis", "[FB80.5] Solitary bone cyst\n Def: A solitary bone cyst is a benign non-epithelial bone cavity that is asymptomatic and that is found most commonly in the second decade of life by chance. The long bones are most often affected, but cas...\n --EXCLUDES--> [?] Other cysts of jaw\n Def: This is mostly odontogenic cysts but may be also of non-odontogenic source. The mandible and maxilla are the bones with the highest prevalent of cysts in the human body owing to odontogenic and develo...\n --CHILD--> [?] Aneurysmal cyst of jaw", "[EK70.Z] Cutaneous cysts, unspecified\n --PARENT--> [EK70] Cutaneous cysts\n --CHILD--> [EK70.0] Epidermoid cyst\n Def: A cutaneous cyst with an epidermoid wall filled with keratin and its breakdown products. It most commonly forms as the result of squamous metaplasia in a damaged sebaceous gland but may result from tr...", "[EK70.Z] Cutaneous cysts, unspecified\n --PARENT--> [EK70] Cutaneous cysts\n --CHILD--> [EK70.1] Trichilemmal cyst\n Def: A trichilemmal (pilar) cyst is a common, typically non-tender, intradermal or subcutaneous cyst. The cysts are typically confined to the scalp and are often multiple. They usually occur sporadically b...", "[FB4Y] Other specified disorders of synovium or tendon\n --PARENT--> [?] Disorders of synovium or tendon\n Def: This is a group of disorders which affect the synovial joint lining (synovium) and also tendons....\n --PARENT--> [?] Soft tissue disorders", "[FB4Y] Other specified disorders of synovium or tendon\n --PARENT--> [?] Disorders of synovium or tendon\n Def: This is a group of disorders which affect the synovial joint lining (synovium) and also tendons....\n --CHILD--> [FB42] Certain specified disorders of synovium or tendon" ]	FB80.5	Solitary bone cyst	[ { "from_icd11": "FB80.5", "icd10_code": "M85412", "icd10_title": "Solitary bone cyst, left shoulder" }, { "from_icd11": "FB80.5", "icd10_code": "M85441", "icd10_title": "Solitary bone cyst, right hand" }, { "from_icd11": "FB80.5", "icd10_code": "M8548", "icd10_title": "Solitary bone cyst, other site" }, { "from_icd11": "FB80.5", "icd10_code": "M8540", "icd10_title": "Solitary bone cyst, unspecified site" }, { "from_icd11": "FB80.5", "icd10_code": "M854", "icd10_title": "Solitary bone cyst" }, { "from_icd11": "EK70.Z", "icd10_code": "L729", "icd10_title": "Follicular cyst of the skin and subcutaneous tissue, unspecified" }, { "from_icd11": "EK70.Z", "icd10_code": "L728", "icd10_title": "Other follicular cysts of the skin and subcutaneous tissue" }, { "from_icd11": "EK70.Z", "icd10_code": "L60-L75", "icd10_title": "" }, { "from_icd11": "EK70.Z", "icd10_code": "L72", "icd10_title": "Follicular cysts of skin and subcutaneous tissue" }, { "from_icd11": "CA0C", "icd10_code": "J341", "icd10_title": "Cyst and mucocele of nose and nasal sinus" }, { "from_icd11": "GA15.1", "icd10_code": "N86", "icd10_title": "Erosion and ectropion of cervix uteri" }, { "from_icd11": "GA15.7", "icd10_code": "N879", "icd10_title": "Dysplasia of cervix uteri, unspecified" }, { "from_icd11": "GA15.7", "icd10_code": "N870", "icd10_title": "Mild cervical dysplasia" }, { "from_icd11": "GA15.7", "icd10_code": "N871", "icd10_title": "Moderate cervical dysplasia" }, { "from_icd11": "GA15.7", "icd10_code": "N87", "icd10_title": "Dysplasia of cervix uteri" } ]	M85412	Solitary bone cyst, left shoulder
The proband was a 52-year-old male. His first symptom 30 years ago was a simple headache. He went to the local hospital for imaging examination and found multiple intracranial cavernous hemangioma with a small amount of bleeding (Unfortunately, the specific inspection method and lesion location are unknown). At that time, the cavernous hemangioma at the bleeding site was surgically removed. After the resection, he took Yangjiao granules for 2 to 3 years, and the pain symptoms basically disappeared. However, 4 years ago, he accidentally bumped his head at work and got a headache again without any other symptoms, another imaging examination revealed that a cavernous hemangioma in his skull had slight bleeding. He was resurgically removed the cavernous blood vessels at the bleeding site and recovered well after the operation, and except for numbness on the left face after a short period of time after the operation, he had no other discomfort, finally, the numbness on the left face gradually relieved on its own without any special treatment. The doctor considered that the postoperative symptom was the microbleeding caused by overwork and finally absorbed by itself, or the short-term sequelae caused by the operation, and recommended him to follow-up regularly. Since then, he has not had head symptoms again. The imaging examination of this trial shows multiple cavernous hemangioma in both cerebral hemispheres, as shown in Figure 2 (1–5). The proband's father was 76 years old. He hit his head at work when he was 65 years old and underwent craniocerebral imaging, and multiple cavernous hemangiomas were found in the brain (the specific location and manifestations of the lesions were unclear). So far, he has no obvious symptoms, and according to the doctor's recommendation, he has never given any special treatment to the intracranial cavernous hemangioma. Later, after 5 years of regular follow-up (Simple telephone follow-up), no obvious changes in the lesions were found. Due to his hunchback and some personal reasons, he did not have the latest MRI examination during this study, and the results of the previous cranial imaging examinations were not found. The daughter of the proband is 32 years old and has had no obvious symptoms so far, and her latest MRI examination does not reveal any abnormalities, and the son of the proband son has never had obvious head discomfort, but he once had multiple intracranial cavernous hemangioma in a cranial MRI when he needed a physical examination due to work. Based on his clinical manifestations and imaging data, the doctor recommended no special treatment for hemangioma. He has not usually received MRI of the brain. The results of this imaging examination suggests that he has multiple cavernous hemangioma in the right cerebellar hemisphere and right prefrontal lobe, as shown in the 22–27 diagram of Figure 2 . The eldest sister of the proband is 50 years old and, last year, experienced stutter and salivation when the right side of her head was tilted without an obvious cause, she therefore underwent an MRI examination, prompting the diagnosis of CCM. Following her diagnosis, she received conservative treatment at the local hospital and was observed carefully, she gradually recovered, and was discharged from the hospital. Now she is being followed up with regularly. Her latest MRI examination mainly reveals multiple cavernous hemangioma in the brain stem and bilateral cerebral hemispheres, as shown in the 6–10 diagram of Figure 2 . The son of the proband's eldest sister has never had obvious head discomfort, nor has he undergone a cranial examination. His latest MRI examination revealed multiple cavernous hemangiomas in the right cerebellar hemisphere and right prefrontal lobe, as shown in the 28–31 diagram of Figure 2 . The second sister of the proband is now 47 years old, and 2 years ago, she experienced blurred vision on the right side without any obvious cause, after conservative treatment, her vision improved, and she is currently taking vincamine orally and being followed up with regularly. Her latest MRI results mainly indicate multiple cavernous hemangiomas in the cerebellum, brain stem, and bilateral cerebral hemispheres, as shown in the 11–16 diagram of Figure 2 . Her son is 28 years old, and experienced a seizure when he was 13 years old, which was stiff and convulsive, at the time, an imaging examination revealed CCM, then he was treated conservatively and taken carbamazepine orally. The drug was eventually stopped because his symptoms did not relapse, however, 2 years ago, he experienced another epileptic seizure with the same symptoms as the previous one, and an imaging examination revealed multiple cavernous hemangiomas in the brain, one of which was bleeding, so he underwent surgery and the cavernous hemangioma was removed. Since the operation, he has been taking sodium valproate sustained-release tablets regularly to prevent seizures. He experienced epilepsy three months after surgery and once in May 2019, but is now asymptomatic and is followed up with regularly. His latest cranial MRI examination reveals multiple cavernous hemangiomas in the brain stem and bilateral cerebral hemispheres, as shown in the 32–35 diagram of Figure 2 . The third sister of the proband is 44 years old, at the age of 27 years, she had no obvious cause for general fatigue, and an imaging examination revealed multiple intracranial cavernous hemangiomas, one of which had ruptured and was bleeding. She underwent surgery to remove the cavernous hemangioma at the lesion, and now she walks with a slight limp, favoring the left half of her body. Her latest MRI examination reveals multiple cavernous hemangiomas in both cerebellar hemispheres, brainstem, bilateral thalamus, and bilateral cerebral hemispheres, as shown in the 17–21 diagram of Figure 2 . The genetic analysis of the two daughters of the proband's third sister did not find the sequence of the mutant gene, and the previous MRI results of the brain did not indicate abnormalities, so MRI was not performed in this test. Table 2 shows the incidence, diagnosis, treatment, and prognosis of the family members. Representative MRIs of the family members with imaging changes can be found in Figure 2	3.841797	0.973633	sec[3]/sec[0]/p[1]	en	0.999996	PMC9013744	https://doi.org/10.3389/fneur.2022.795514	[ "cavernous", "multiple", "imaging", "hemangioma", "proband", "obvious", "shown", "hemangiomas", "brain", "latest" ]	[ { "code": "9C81.4", "title": "Cavernous sinus syndromes" }, { "code": "GB06.Y", "title": "Other specified disorders of penis" }, { "code": "LA90.10", "title": "Macrocystic lymphatic malformation" }, { "code": "8B22.43", "title": "Carotid cavernous fistula" }, { "code": "NA07.Y", "title": "Other specified intracranial injury" }, { "code": "6B64", "title": "Dissociative identity disorder" }, { "code": "JA80.Z", "title": "Maternal care related to unspecified multiple gestation" }, { "code": "QA46.Z", "title": "Outcome of delivery, unspecified" }, { "code": "8A40.Z", "title": "Multiple sclerosis, unspecified" }, { "code": "ND31", "title": "Open wounds involving multiple body regions" } ]	=== ICD-11 CODES FOUND === [9C81.4] Cavernous sinus syndromes Also known as: Cavernous sinus syndromes [GB06.Y] Other specified disorders of penis Also known as: Other specified disorders of penis \| Other inflammatory disorders of penis \| cavernositis \| Abscess of corpus cavernosum and penis \| abscess of corpus cavernosum [LA90.10] Macrocystic lymphatic malformation Definition: A condition caused by failure of the lymphatic system to correctly develop during the antenatal period. This condition is characterised by large, soft, smooth clear masses under normal or bluish skin. This condition may be associated with cellulitis, bleeding within the malformation, pain, or leakage of lymphatic fluid internally. Also known as: Macrocystic lymphatic malformation \| Cavernous lymphangioma \| Cavernous lymphatic malformation \| Macrocystic lymphangioma \| Circumscribed lymphatic malformation [8B22.43] Carotid cavernous fistula Definition: A carotid-cavernous fistula results from an abnormal communication between the arterial and venous systems within the cavernous sinus in the skull. Also known as: Carotid cavernous fistula [NA07.Y] Other specified intracranial injury Also known as: Other specified intracranial injury \| Traumatic intracranial haemorrhage, not elsewhere classified \| Traumatic cranium cavity haemorrhage \| traumatic intracranium haemorrhage \| Intracranial haematoma [6B64] Dissociative identity disorder Definition: Dissociative identity disorder is characterised by disruption of identity in which there are two or more distinct personality states (dissociative identities) associated with marked discontinuities in the sense of self and agency. Each personality state includes its own pattern of experiencing, perceiving, conceiving, and relating to self, the body, and the environment. At least two distinct personality states recurrently take executive control of the individual’s consciousness and functioning i Also known as: Dissociative identity disorder \| Multiple personality \| Multiple personality disorder [JA80.Z] Maternal care related to unspecified multiple gestation Also known as: Maternal care related to unspecified multiple gestation \| Maternal care related to multiple gestation \| multiple gestation, unspecified, unspecified trimester \| multiple pregnancy \| Multiple pregnancy NOS [QA46.Z] Outcome of delivery, unspecified Also known as: Outcome of delivery, unspecified \| Outcome of delivery \| Multiple birth, unspecified \| Single birth, unspecified [8A40.Z] Multiple sclerosis, unspecified Also known as: Multiple sclerosis, unspecified \| Multiple sclerosis \| cerebrospinal sclerosis \| disseminated sclerosis \| generalised multiple sclerosis [ND31] Open wounds involving multiple body regions Also known as: Open wounds involving multiple body regions \| Open wounds involving head with neck \| Open wounds of sites classifiable as open wounds to the head or open wounds of the neck \| Nasopharyngeal laceration \| Open wounds involving thorax with abdomen, lower back or pelvis Excludes: Traumatic amputations involving multiple body regions === GRAPH WALKS === --- Walk 1 --- [9C81.4] Cavernous sinus syndromes --PARENT--> [9C81] Ocular motor nerve palsies --EXCLUDES--> [?] Internuclear ophthalmoplegia Def: This is a disorder of conjugate lateral gaze in which the affected eye shows impairment of adduction.... --- Walk 2 --- [9C81.4] Cavernous sinus syndromes --PARENT--> [9C81] Ocular motor nerve palsies --PARENT--> [?] Strabismus or ocular motility disorders Def: Disorder due to abnormalities of extraocular muscles or ocular motor abnormalities.... --- Walk 3 --- [GB06.Y] Other specified disorders of penis --PARENT--> [GB06] Certain specified disorders of penis --PARENT--> [?] Diseases of the male genital system Def: Any disease characterised by pathological changes to the male genital system.... --- Walk 4 --- [GB06.Y] Other specified disorders of penis --PARENT--> [GB06] Certain specified disorders of penis --CHILD--> [GB06.2] Penile fibromatosis Def: A condition characterised by induration of the corpora cavernosa of the penis producing a painful fibrous chordee within the soft tissue of the penis and inflammation of the tunica albuginea. This con... --- Walk 5 --- [LA90.10] Macrocystic lymphatic malformation Def: A condition caused by failure of the lymphatic system to correctly develop during the antenatal period. This condition is characterised by large, soft, smooth clear masses under normal or bluish skin.... --PARENT--> [LA90.1] Lymphatic malformations Def: Lymphatic malformations (LM), formerly referred to by the term lymphangioma, are malformations of the lymphatic system which result in obstructed lymphatic drainage. There are two types of LM: macrocy... --CHILD--> [LA90.12] Lymphatic malformations of certain specified sites --- Walk 6 --- [LA90.10] Macrocystic lymphatic malformation Def: A condition caused by failure of the lymphatic system to correctly develop during the antenatal period. This condition is characterised by large, soft, smooth clear masses under normal or bluish skin.... --PARENT--> [LA90.1] Lymphatic malformations Def: Lymphatic malformations (LM), formerly referred to by the term lymphangioma, are malformations of the lymphatic system which result in obstructed lymphatic drainage. There are two types of LM: macrocy... --CHILD--> [LA90.11] Microcystic lymphatic malformation Def: Microcystic lymphatic malformations consist of clusters of dilated lymphatic vessels which have developed without connection to the systemic lymphatic circulation. They present with grouped clear or h...	[ "[9C81.4] Cavernous sinus syndromes\n --PARENT--> [9C81] Ocular motor nerve palsies\n --EXCLUDES--> [?] Internuclear ophthalmoplegia\n Def: This is a disorder of conjugate lateral gaze in which the affected eye shows impairment of adduction....", "[9C81.4] Cavernous sinus syndromes\n --PARENT--> [9C81] Ocular motor nerve palsies\n --PARENT--> [?] Strabismus or ocular motility disorders\n Def: Disorder due to abnormalities of extraocular muscles or ocular motor abnormalities....", "[GB06.Y] Other specified disorders of penis\n --PARENT--> [GB06] Certain specified disorders of penis\n --PARENT--> [?] Diseases of the male genital system\n Def: Any disease characterised by pathological changes to the male genital system....", "[GB06.Y] Other specified disorders of penis\n --PARENT--> [GB06] Certain specified disorders of penis\n --CHILD--> [GB06.2] Penile fibromatosis\n Def: A condition characterised by induration of the corpora cavernosa of the penis producing a painful fibrous chordee within the soft tissue of the penis and inflammation of the tunica albuginea. This con...", "[LA90.10] Macrocystic lymphatic malformation\n Def: A condition caused by failure of the lymphatic system to correctly develop during the antenatal period. This condition is characterised by large, soft, smooth clear masses under normal or bluish skin....\n --PARENT--> [LA90.1] Lymphatic malformations\n Def: Lymphatic malformations (LM), formerly referred to by the term lymphangioma, are malformations of the lymphatic system which result in obstructed lymphatic drainage. There are two types of LM: macrocy...\n --CHILD--> [LA90.12] Lymphatic malformations of certain specified sites", "[LA90.10] Macrocystic lymphatic malformation\n Def: A condition caused by failure of the lymphatic system to correctly develop during the antenatal period. This condition is characterised by large, soft, smooth clear masses under normal or bluish skin....\n --PARENT--> [LA90.1] Lymphatic malformations\n Def: Lymphatic malformations (LM), formerly referred to by the term lymphangioma, are malformations of the lymphatic system which result in obstructed lymphatic drainage. There are two types of LM: macrocy...\n --CHILD--> [LA90.11] Microcystic lymphatic malformation\n Def: Microcystic lymphatic malformations consist of clusters of dilated lymphatic vessels which have developed without connection to the systemic lymphatic circulation. They present with grouped clear or h..." ]	9C81.4	Cavernous sinus syndromes	[ { "from_icd11": "6B64", "icd10_code": "F449", "icd10_title": "Dissociative and conversion disorder, unspecified" }, { "from_icd11": "6B64", "icd10_code": "F44", "icd10_title": "Dissociative and conversion disorders" }, { "from_icd11": "JA80.Z", "icd10_code": "O30", "icd10_title": "Multiple gestation" }, { "from_icd11": "JA80.Z", "icd10_code": "O308", "icd10_title": "Other specified multiple gestation" }, { "from_icd11": "JA80.Z", "icd10_code": "O309", "icd10_title": "Multiple gestation, unspecified" }, { "from_icd11": "QA46.Z", "icd10_code": "Z379", "icd10_title": "Outcome of delivery, unspecified" }, { "from_icd11": "QA46.Z", "icd10_code": "Z37", "icd10_title": "Outcome of delivery" }, { "from_icd11": "8A40.Z", "icd10_code": "G35", "icd10_title": "Multiple sclerosis" }, { "from_icd11": "8A40.Z", "icd10_code": "G370", "icd10_title": "Diffuse sclerosis of central nervous system" }, { "from_icd11": "8A40.Z", "icd10_code": "G375", "icd10_title": "Concentric sclerosis [Balo] of central nervous system" }, { "from_icd11": "ND31", "icd10_code": "T01", "icd10_title": "" }, { "from_icd11": "ND31", "icd10_code": "T010", "icd10_title": "" }, { "from_icd11": "ND31", "icd10_code": "T011", "icd10_title": "" }, { "from_icd11": "ND31", "icd10_code": "T012", "icd10_title": "" }, { "from_icd11": "ND31", "icd10_code": "T013", "icd10_title": "" } ]	F449	Dissociative and conversion disorder, unspecified
In our case report, we presented a patient who had already been diagnosed for Klein Levin syndrome as a teenager. Exhaustive and comprehensive neurological workout did not find the cause of his disease, as in many patients with this syndrome, the cause has not been found. During the diagnostic workout, we paid great attention to the hypothalamus as the basic center in the control of sleep, appetite and sexual behavior . Although no pathology were found, our patient was well with symptomatic therapy, we very successfully controlled his key symptoms and after a certain period of time therapy was gradually discontinued. Evaluation of the cerebrospinal fluid (CSF) and serological inflammatory markers are un-remarkable, electroencephalography (EEG) shows slowing in most patients during episodes without epileptic activity , but in our patient there were no pathological changes on the EEG. Single photon emission CT scanning during patients’ symptomatic periods can demonstrate hypoperfusion in the thalamus, hypothalamus, temporal lobes, orbito-frontal and parasagittal frontal lobes, and basal ganglia , but we were unable to do that test after our patient overcame a COVID infection. Fluorodeoxyglucose-positron emission tomography (FDG-PET) scanning in some patients may indicate asymmetric hypometabolism in the thalamus and hypothalamus , but not in our patient. Viral causative factors have been suggested, on the basis of the frequent report of flu-like symptoms at onset, and the most frequent precipitating factor is preceding infection . The disease itself most often occurs in the colder periods of the year, especially in autumn and winter, when the number of respiratory infections is increased in the general population. The most common predisposing factors, in addition to those infectious, are psychological and physical stress, alcohol use or use of illicit drugs (marijuana, cocaine), sleep deprivation, physical exertion, traveling and head trauma. We did not find any predisposing factor in our patient at the time the disease was first diagnosed, but also at the most recent relapse of the disease. Only moderate SARS-CoV-2 infection coincided with the new relapse of his symptoms. The SARS-CoV-2 virus is a single stranded RNA virus which primarily causes respiratory symptoms, but an increasing number of patients also have neurological symptoms or neurological consequences of overcoming infection . There is almost no neurological symptom that has not been described in the literature as a possible consequence of SARS-CoV-2 infection. Many COVID patients experienced non-specific neurological complications such as headache, dizziness, ageusia (loss of taste), anosmia (loss of smell), myalgia and fatigue. In moderate or severe forms of the COVID disease, can occur serious neurological symptoms such as prolonged headache, disturbance in consciousness, acute cerebrovascular disease (ischemic stroke, cerebral or subarachnoid hamorrhage), acute encephalopathy, encephalitis or meningitis, polyneuropathy, multiple sclerosis spectrum of disease and seizures. Direct viral invasion of the neural and endothelial cell by ACE2 receptors, diffuse endothelial inflammation, which impair cerebral vasoreactivity , coagulopathy and hyperactivity of the host immune system are the mechanisms by which the virus causes neurological dysfunction. The pathophysiological mechanism by which SARS-CoV-2 virus can trigger Klein Levin syndrome is unclear. It is probably a disorder at the level of the hypothalamus, either a direct invasion of neural and glial cells, or damage to the endothelial cells of the blood vessels essential for the perfusion of the hypothalamus. The way in which the SARS-CoV2 virus causes changes in the brain is most commonly associated with changes in the endothelium of the small blood vessels of the brain. The endothelium of small blood vessels has a large expression of ACE2 receptors which form an excellent channel for virus entry. Changes in the endothelium lead to diffuse endothelium damage which in turn causes changes in cerebral circulation. The new changes on the brain MRI in our patient are nothing but microcirculatory scars as a consequence of hypoperfusion, they are of vascular etiology, not demyelinating. Impaired vasoreactivity that we detected on TCCD in our patient also confirms that his cerebral endothelium is diffusely damaged after infection, but only some vascular lesion we have noticed on brain MR. Hypothalamic hypoperfusion may be the trigger for a new relapse of the disease. The most common findings on MR brain images after COVID infection were hyperintensive nonconfluent white matter lesions in the FLAIR sequence with postcontrast imbibition (such lesions are seen on MRI of the brain in our patient), hemorrhagic lesions, and diffuse microbleeds within the white matter in the SWI sequence in the medial temporal lobe area. Laminar cortical lesions are also common, which may indicate a disorder of vasomotor reactivity, but also numerous periventricular and juxtacortical hypertensive lesions in the FLAIR sequence with in-creased signal intensity on the DWI map or in the area of the corpus callosum . The symptoms of Kleine-Levin syndrome are characterized by their intermittent and periodic nature . The episodes themselves usually last between 1 and 3 weeks and cycle length can last from 2 to 4 months. Recurrences vary in frequency and in patients with adult onset, the disorder is less likely to resolve. As the disorder progresses and the patient ages, the cycle length increases. Symptoms tend to wane in severity as the disorder progresses and eventually spontaneously resolve in patients with adolescent onset . Viral infections can be a common cause of exacerbations. The classic triad of hypersomnia, hyperphagia, and hypersexuality is not always present, and like our patient, they often exhibit some form of cognitive or mood impairment. As a rule, exacerbations of Klein Levin’s syndrome symptoms often present with the same pattern, so each new relapse of the disease can have identical clinical picture as the previous one. Our patient had the same clinical expression of the disease at the time he was diagnosed with the disease as well after SARS-CoV-2 infection.	4.128906	0.96582	sec[2]/p[0]	en	0.999997	34294673	https://doi.org/10.3390/neurolint13030033	[ "patients", "infection", "which", "neurological", "changes", "sars", "virus", "brain", "hypothalamus", "endothelium" ]	[ { "code": "PL14.C", "title": "Patient received diagnostic test or treatment intended for another patient" }, { "code": "QB14", "title": "Unavailability or inaccessibility of health care facilities" }, { "code": "PL14.2", "title": "Problem associated with physical transfer of patient" }, { "code": "QB12.0", "title": "Organ transplant candidate" }, { "code": "QA15.1", "title": "Counselling related to sexual behaviour and orientation or sexual relationships of the person" }, { "code": "1H0Z", "title": "Infection, unspecified" }, { "code": "1G40", "title": "Sepsis without septic shock" }, { "code": "FA10.Z", "title": "Direct infections of joint, unspecified" }, { "code": "1D9Z", "title": "Unspecified viral infection of unspecified site" }, { "code": "1A40.Z", "title": "Infectious gastroenteritis or colitis without specification of infectious agent" } ]	=== ICD-11 CODES FOUND === [PL14.C] Patient received diagnostic test or treatment intended for another patient Also known as: Patient received diagnostic test or treatment intended for another patient \| wrong patient \| incorrect patient Excludes: Procedure undertaken at wrong site or wrong side, as mode of injury or harm [QB14] Unavailability or inaccessibility of health care facilities Also known as: Unavailability or inaccessibility of health care facilities \| unavailability of medical facilities \| Unavailability of outpatient clinic \| Unavailability or inaccessibility of residential aged care service Excludes: bed unavailable [PL14.2] Problem associated with physical transfer of patient Also known as: Problem associated with physical transfer of patient [QB12.0] Organ transplant candidate Also known as: Organ transplant candidate \| patient waiting for organ availability \| health services provided because of need for organ transplant \| organ transplant candidate awaiting organ availability \| person on organ transplant waiting list [QA15.1] Counselling related to sexual behaviour and orientation or sexual relationships of the person Also known as: Counselling related to sexual behaviour and orientation or sexual relationships of the person \| advice on sexual behaviour or orientation \| counselling on sexual behaviour or orientation \| promiscuity counselling \| patient concerned regarding sexual orientation [1H0Z] Infection, unspecified Also known as: Infection, unspecified \| infection NOS \| infectious disease NOS \| infection unknown \| infection process NOS [1G40] Sepsis without septic shock Definition: Sepsis is defined as a life-threatening organ dysfunction caused by a dysregulated host response to infection. Also known as: Sepsis without septic shock \| sepsis without septic shock with known organism \| Sepsis-associated hypotension \| Unspecified sepsis \| general septic intoxication Excludes: Septicaemia \| Sepsis of fetus or newborn [FA10.Z] Direct infections of joint, unspecified Also known as: Direct infections of joint, unspecified \| Direct infections of joint \| septic arthritis \| pyogenic arthritis \| arthritis due to infection [1D9Z] Unspecified viral infection of unspecified site Also known as: Unspecified viral infection of unspecified site \| viral infection NOS \| viral disorder NOS \| disease caused by virus \| unspecified viremia [1A40.Z] Infectious gastroenteritis or colitis without specification of infectious agent Also known as: Infectious gastroenteritis or colitis without specification of infectious agent \| Gastroenteritis or colitis without specification of infectious agent \| diarrhoea and gastroenteritis of presumed infectious origin \| diarrhoeal enteritis \| GE - [gastroenteritis] === GRAPH WALKS === --- Walk 1 --- [PL14.C] Patient received diagnostic test or treatment intended for another patient --EXCLUDES--> [?] Procedure undertaken at wrong site or wrong side, as mode of injury or harm --EXCLUDES--> [?] Patient received diagnostic test or treatment intended for another patient --- Walk 2 --- [PL14.C] Patient received diagnostic test or treatment intended for another patient --PARENT--> [PL14] Mode of injury or harm associated with other health care related causes --EXCLUDES--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance --- Walk 3 --- [QB14] Unavailability or inaccessibility of health care facilities --EXCLUDES--> [?] Person awaiting admission to adequate facility elsewhere --CHILD--> [?] Person awaiting admission to mental health facility or unit --- Walk 4 --- [QB14] Unavailability or inaccessibility of health care facilities --EXCLUDES--> [?] Person awaiting admission to adequate facility elsewhere --CHILD--> [?] Person awaiting admission to mental health facility or unit --- Walk 5 --- [PL14.2] Problem associated with physical transfer of patient --PARENT--> [PL14] Mode of injury or harm associated with other health care related causes --EXCLUDES--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance --- Walk 6 --- [PL14.2] Problem associated with physical transfer of patient --PARENT--> [PL14] Mode of injury or harm associated with other health care related causes --EXCLUDES--> [?] Mode of injury or harm associated with a surgical or other medical procedure	[ "[PL14.C] Patient received diagnostic test or treatment intended for another patient\n --EXCLUDES--> [?] Procedure undertaken at wrong site or wrong side, as mode of injury or harm\n --EXCLUDES--> [?] Patient received diagnostic test or treatment intended for another patient", "[PL14.C] Patient received diagnostic test or treatment intended for another patient\n --PARENT--> [PL14] Mode of injury or harm associated with other health care related causes\n --EXCLUDES--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance", "[QB14] Unavailability or inaccessibility of health care facilities\n --EXCLUDES--> [?] Person awaiting admission to adequate facility elsewhere\n --CHILD--> [?] Person awaiting admission to mental health facility or unit", "[QB14] Unavailability or inaccessibility of health care facilities\n --EXCLUDES--> [?] Person awaiting admission to adequate facility elsewhere\n --CHILD--> [?] Person awaiting admission to mental health facility or unit", "[PL14.2] Problem associated with physical transfer of patient\n --PARENT--> [PL14] Mode of injury or harm associated with other health care related causes\n --EXCLUDES--> [?] Mode of injury or harm associated with exposure to a drug, medicament or biological substance", "[PL14.2] Problem associated with physical transfer of patient\n --PARENT--> [PL14] Mode of injury or harm associated with other health care related causes\n --EXCLUDES--> [?] Mode of injury or harm associated with a surgical or other medical procedure" ]	PL14.C	Patient received diagnostic test or treatment intended for another patient	[ { "from_icd11": "QB14", "icd10_code": "Z753", "icd10_title": "Unavailability and inaccessibility of health-care facilities" }, { "from_icd11": "QA15.1", "icd10_code": "F66", "icd10_title": "Other sexual disorders" }, { "from_icd11": "QA15.1", "icd10_code": "F660", "icd10_title": "" }, { "from_icd11": "QA15.1", "icd10_code": "F661", "icd10_title": "" }, { "from_icd11": "QA15.1", "icd10_code": "F662", "icd10_title": "" }, { "from_icd11": "QA15.1", "icd10_code": "F668", "icd10_title": "" }, { "from_icd11": "QA15.1", "icd10_code": "F669", "icd10_title": "" }, { "from_icd11": "QA15.1", "icd10_code": "Z701", "icd10_title": "Counseling related to patient's sexual behavior and orientation" }, { "from_icd11": "1H0Z", "icd10_code": "B999", "icd10_title": "Unspecified infectious disease" }, { "from_icd11": "1H0Z", "icd10_code": "A312", "icd10_title": "Disseminated mycobacterium avium-intracellulare complex (DMAC)" }, { "from_icd11": "1H0Z", "icd10_code": "B998", "icd10_title": "Other infectious disease" }, { "from_icd11": "1H0Z", "icd10_code": "A249", "icd10_title": "Melioidosis, unspecified" }, { "from_icd11": "1H0Z", "icd10_code": "R6511", "icd10_title": "Systemic inflammatory response syndrome (SIRS) of non-infectious origin with acute organ dysfunction" }, { "from_icd11": "1H0Z", "icd10_code": "R6510", "icd10_title": "Systemic inflammatory response syndrome (SIRS) of non-infectious origin without acute organ dysfunction" }, { "from_icd11": "1H0Z", "icd10_code": "A318", "icd10_title": "Other mycobacterial infections" } ]	Z753	Unavailability and inaccessibility of health-care facilities
The patient was discharged from hospital at the age of one month. The disease course after discharge is shown in Table 4 . During the first months after discharge, she was clinically and metabolically stable. At the age of four months, convulsion-like symptoms appeared. Sandifer syndrome was considered as the convulsion like symptoms seemed to be mostly related to nutrition, but was later confirmed as epilepsy by electroencephalography. Brain MRI showed no abnormalities. From the age of 4.5 months old, our patient showed mild developmental delay on important milestones. At 5 months she rolled over form back to abdomen for the first time. At 9 months old developmental level was assessed by a physiotherapist using the BSID-III-NL. Fine motor skills were scored normal, however gross motor skills were delayed scoring an overall percentile of 12/100. Delay on gross motor function was mostly based on not being fully able to maintain the abdominal position. Furthermore, she had mild active hypertonia in her legs. Table 4 Disease course. Table 4 T1 T2 T3 T4 T5 T6 T7 Metabolic Decompensation No No Admission respiratory tract infection Admission metabolic decompensation No Admission heart failure Admission acute heart failure Laboratory investigations CK 156 U/l Acylcarnitine profile: see Table 3 CK 128 U/l CK 7387 U/l lactate 1.8, pH 7.4, CO2 5.1, glucose 5.3 CK 26.654 U/l lactate 4.7, pH 7.3, CO2 7.3, glucose 4.9 Not done CK 20037 U/l, lactate 4.0, pH 7.4, CO2 4.5, glucose 7.1 First CK level 212 U/l, after 1 day hospitalization: CK 3371 U/l. Lactate 4.9, pH 7.29, CO2 5.4, glucose 6.2 Medication Hydroxybutarate 400 mg/kg/day Hydroxybutarate 470 mg/kg/day Hydroxybutarate 470 mg/kg/day Hydroxybutyrate to 500 mg/kg/day Hydroxybutarate 500 mg/kg/day Hydroxybutarate 500 mg/kg/day Hydroxybutarate 500 mg/kg/day Cardiac ECG Sinus tachycardia, mild abnormalities of T-waves. No pathological signs Sinus tachycardia, mild abnormalities of T-waves. No pathological signs Sinus tachycardia, negative T-waves in the inferior, lateral and V4-V6 leads Sinus tachycardia, negative T-waves in the inferior, lateral and V4-V6 leads Echocardiography Normal sizes of both ventricles and atria (LVEDD 20 mm; z-score + 0.2); no hypertrophy. Normal systolic LV (LV FS 42%) and RV function. Normal sizes of both ventricles and atria (LVEDD 24 mm; z-score + 0.9); no hypertrophy. Normal systolic LV (LV FS 34%) and RV function. Not done First episode of acute deterioration of cardiac function. Poor systolic LV function (LV FS 18%, normal >29%) and moderate systolic RV function. Dilation of the LV (LVEDD 35 mm; z-score + 3.1). Within 24 h after continuous i.v. glucose infusion recovery of systolic LV (LV FS 33%) and RV function. Not done Second episode of acute deterioration of cardiac function. Poor systolic LV function (LV FS 21%) and moderate systolic RV function. Dilation of the LV (LVEDD 36 mm; z-score + 3.0). Mild LV hypertrophy. Within 72 h after continuous i.v. glucose infusion recovery of systolic LV (LV FS 33%) and RV function. Third episode of acute deterioration of cardiac function. Poor systolic LV function (LV FS 14%) and moderate systolic RV function. Dilation of the LV (LVEDD 39 mm; z-score + 3.3). Mild LV hypertrophy. Laboratory investigations NT-proBNP 150 pg/ml NT-proBNP 110 pg/ml NT-proBNP 190 pg/ml NT-proBNP 6600 pg/ml Nt Pro BNP 6200 pg/ml NT pro-BNP 9700 pg/ml (later rose to 24.000 pg/ml) Medication Captopril Hydrochlorothiazide Spironolactone Stop captopril Hydrochlorothiazide Spironolactone Hydrochlorothiazide Spironolactone Furosemide Hydrochlorothiazide Spironolactone Furosemide Hydrochlorothiazide Spironolactone Enalapril Furosemide Hydrochlorothiazide Spironolactone Enalapril Furosemide Milrinone, Dobutamine, Furosemide Neurologic Development Laughing, alert, motor functions active, normal reflexes Stagnation of gross motor skills Assessment of development: delayed gross motor skills Normal vision Epilepsy No convulsions Convulsion-like symptoms No convulsions No convulsions Recurrence of epilepsy No convulsions No convulsions Medication Start levetiracetam Switch to Zonisamide MRI MRI at 4 months old: Symmetrical aspect of the myelination pattern, normal for age. No areas of tissue loss. Normal aspect of the basal ganglia. No structural abnormalities demonstrated Gastrointestinal Partly drinking, partly tube feeding Reflux: start Nexium Limited to tube feeding Vomiting Laparoscopic gastrostomy, obstipation and gagging Obstipation Frequent gagging and vomiting Diet Exclusively Monogen© 16.8 g/100 ml) - 99 kcal/kg - 134 ml/kg - 8 feeds per day Maximum fasting: 4 h Exclusively Monogen© (16.8 g/100 ml) - 96 kcal/kg - 129 ml/kg - 6 feeds per day Maximum fasting: 4 h. Extension to 5 h during the night. Emergency regime: during admission: Monogen (17 g/100 ml) + 5 g maltodextrine per bottle. Iv glucose 10% 8 mg/kg/min 6 feeds per day Emergency regime during admission: Iv glucose 10% 8 mg/kg/min Iv glucose 10% 5.8 mg/kg/min (fluid restriction of 850 ml/day) 850 ml Monogen© (18,5 g/100 ml) with maltodextrin 2,9 g/100 ml. Maximum fasting 1 h at a maximum of twice per day. Followed by: iv. glucose 50% + TPV base 2 proteins/carbohydrates Growth Length 53.5 cm (−1.5SD) Weight 3876 g (−1.9SD w/a; −0,45SD w/h) Length: 60 cm (−1.0SD) Weight: 5560 g (−1.0SD w/a; +0,1SD w/h) Weight 6300 g (−0.7SD w/a) Length: 71 cm (−0,3SD) Weight: 9100 g (+0,6SD w/a; +1,0SD w/h) Weight 10,000 g (+0,91SD) Length 73 (−0,3SD) Length: 73 cm (−0.3SD) Weight: 10.080 g (+0.9SD w/a; +1.5SD w/h) Length 78 cm (+0.9SD) Weight 10,400 g(+0,7SD w/a; +0.1w/h) Table 4 : Disease course from 2 months to 13 months is presented and divided by metabolic, cardiac, neurologic, gastrointestinal, diet and growth records. Legend Table 4 : T1 = 2 months old: Visit outpatient clinic 4 weeks after discharge. T2 = 4 months old: Start epilepsy. T3 = 6 months old: First hospital admission with respiratory tract infection and vomiting leading to metabolic decompensation. T4 = 9 months old: Metabolic decompensation. T5 = 10 months: Recurrence of epilepsy. T6 = 11 months: Metabolic decompensation, admission from outpatient clinic with severe cardiac decompensation. T7 = 12-13 months: Hospital admission severe cardiac decompensation.	4.109375	0.947754	sec[2]/sec[0]/p[9]	en	0.999996	PMC9248206	https://doi.org/10.1016/j.ymgmr.2022.100873	[ "function", "glucose", "systolic", "decompensation", "cardiac", "weight", "motor", "metabolic", "hydroxybutarate", "hydrochlorothiazide" ]	[ { "code": "DD91.Z", "title": "Irritable bowel syndrome or functional bowel disorders, unspecified" }, { "code": "9E1Z", "title": "Diseases of the visual system, unspecified" }, { "code": "6B60.Z", "title": "Dissociative neurological symptom disorder, with unspecified symptoms" }, { "code": "6B60.8Y", "title": "Dissociative neurological symptom disorder, with other specified movement disturbance" }, { "code": "6B60.3", "title": "Dissociative neurological symptom disorder, with other sensory disturbance" }, { "code": "5A40.Z", "title": "Intermediate hyperglycaemia, unspecified" }, { "code": "5C61.Y", "title": "Other specified disorders of carbohydrate absorption or transport" }, { "code": "5A40.1", "title": "Impaired glucose tolerance" }, { "code": "5A40.0", "title": "Impaired fasting glucose" }, { "code": "5C61.5", "title": "Disorders of facilitated glucose transport" } ]	=== ICD-11 CODES FOUND === [DD91.Z] Irritable bowel syndrome or functional bowel disorders, unspecified Also known as: Irritable bowel syndrome or functional bowel disorders, unspecified \| Irritable bowel syndrome or certain specified functional bowel disorders \| Functional intestinal disorders NOS [9E1Z] Diseases of the visual system, unspecified Also known as: Diseases of the visual system, unspecified \| eye diseases NOS \| disorder of vision \| visual disorder [6B60.Z] Dissociative neurological symptom disorder, with unspecified symptoms Also known as: Dissociative neurological symptom disorder, with unspecified symptoms \| Dissociative neurological symptom disorder \| Functional neurological disorders \| Functional neurological symptom disorder \| Conversion disorder [6B60.8Y] Dissociative neurological symptom disorder, with other specified movement disturbance Also known as: Dissociative neurological symptom disorder, with other specified movement disturbance \| Functional movement disorder \| Other functional hyperkinetic movements [6B60.3] Dissociative neurological symptom disorder, with other sensory disturbance Definition: Dissociative neurological symptom disorder, with other sensory disturbance is characterised by sensory symptoms not identified in other specific categories in this grouping such as numbness, tightness, tingling, burning, pain, or other symptoms related to touch, smell, taste, balance, proprioception, kinesthesia, or thermoception. The symptoms are not consistent with a recognised disease of the nervous system, other mental, behavioural or neurodevelopmental disorder, or other medical condition a Also known as: Dissociative neurological symptom disorder, with other sensory disturbance \| Functional neurological symptom disorder, with alteration of sensation \| Functional sensory disorder \| Functional neurological symptom disorder, with other sensory disturbance [5A40.Z] Intermediate hyperglycaemia, unspecified Also known as: Intermediate hyperglycaemia, unspecified \| Intermediate hyperglycaemia \| Impaired glucose regulation \| prediabetes \| latent diabetes [5C61.Y] Other specified disorders of carbohydrate absorption or transport Also known as: Other specified disorders of carbohydrate absorption or transport \| Other disorders of intestinal carbohydrate absorption \| Glucose malabsorption \| Isomaltose malabsorption \| Sucrose malabsorption [5A40.1] Impaired glucose tolerance Definition: Impaired glucose tolerance (IGT) is a metabolic disorder, which is characterised by 2-h postload glucose 140–199 mg/dl (7.8–11.1 mmol/l). Also known as: Impaired glucose tolerance \| IGT - [impaired glucose tolerance] \| Impaired glucose tolerance with unspecified complication \| Impaired glucose tolerance without complication \| abnormal glucose tolerance [5A40.0] Impaired fasting glucose Definition: Impaired fasting tolerance is a metabolic disorder with Fasting Plasma Glucose (FPG) 110–125 mg/dl (6.1–6.9 mmol/l). Also known as: Impaired fasting glucose \| IFG - [impaired fasting glucose] \| impaired fasting glycaemia \| elevated fasting glucose \| high fasting blood sugar [5C61.5] Disorders of facilitated glucose transport Also known as: Disorders of facilitated glucose transport \| Encephalopathy due to GLUT1 deficiency \| Glucose transporter defect, blood-brain barrier \| Facilitated glucose transporter protein type 1 deficiency \| Familial renal glucosuria === GRAPH WALKS === --- Walk 1 --- [DD91.Z] Irritable bowel syndrome or functional bowel disorders, unspecified --PARENT--> [DD91] Irritable bowel syndrome or certain specified functional bowel disorders Def: This group incorporates functional bowel disorders which principally present symptoms attributable to the intestinal tract in the absence of specific and unique organic pathology in the small and larg... --CHILD--> [DD91.2] Functional diarrhoea Def: Functional diarrhoea is a continuous or recurrent syndrome characterised by the passage of loose (mushy) or watery stools without abdominal pain or discomfort.... --- Walk 2 --- [DD91.Z] Irritable bowel syndrome or functional bowel disorders, unspecified --PARENT--> [DD91] Irritable bowel syndrome or certain specified functional bowel disorders Def: This group incorporates functional bowel disorders which principally present symptoms attributable to the intestinal tract in the absence of specific and unique organic pathology in the small and larg... --CHILD--> [DD91.0] Irritable bowel syndrome Def: Irritable bowel syndrome (IBS) is a functional bowel disorder in which abdominal pain or discomfort is associated with defecation or a change in bowel habit, and with features of disordered defecation... --- Walk 3 --- [9E1Z] Diseases of the visual system, unspecified --PARENT--> [09] Diseases of the visual system Def: This refers to any diseases of the visual system, which includes the eyes and adnexa, the visual pathways and brain areas, which initiate and control visual perception and visually guided behaviour.... --CHILD--> [?] Disorders of the eyeball - posterior segment Def: This refers to disorders of the back two-thirds of the eye that includes the anterior hyaloid membrane and all of the optical structures behind it: the vitreous humour, retina, choroid, and optic nerv... --- Walk 4 --- [9E1Z] Diseases of the visual system, unspecified --PARENT--> [09] Diseases of the visual system Def: This refers to any diseases of the visual system, which includes the eyes and adnexa, the visual pathways and brain areas, which initiate and control visual perception and visually guided behaviour.... --EXCLUDES--> [?] Certain infectious or parasitic diseases Def: This chapter includes certain conditions caused by pathogenic organisms or microorganisms, such as bacteria, viruses, parasites or fungi.... --- Walk 5 --- [6B60.Z] Dissociative neurological symptom disorder, with unspecified symptoms --PARENT--> [6B60] Dissociative neurological symptom disorder Def: Dissociative neurological symptom disorder is characterised by the presentation of motor, sensory, or cognitive symptoms that imply an involuntary discontinuity in the normal integration of motor, sen... --EXCLUDES--> [?] Factitious disorders Def: Factitious disorders are characterised by intentionally feigning, falsifying, inducing, or aggravating medical, psychological, or behavioural signs and symptoms or injury in oneself or in another pers... --- Walk 6 --- [6B60.Z] Dissociative neurological symptom disorder, with unspecified symptoms --PARENT--> [6B60] Dissociative neurological symptom disorder Def: Dissociative neurological symptom disorder is characterised by the presentation of motor, sensory, or cognitive symptoms that imply an involuntary discontinuity in the normal integration of motor, sen... --CHILD--> [6B60.0] Dissociative neurological symptom disorder, with visual disturbance Def: Dissociative neurological symptom disorder, with visual disturbance is characterised by visual symptoms such as blindness, tunnel vision, diplopia, visual distortions or hallucinations that are not co...	[ "[DD91.Z] Irritable bowel syndrome or functional bowel disorders, unspecified\n --PARENT--> [DD91] Irritable bowel syndrome or certain specified functional bowel disorders\n Def: This group incorporates functional bowel disorders which principally present symptoms attributable to the intestinal tract in the absence of specific and unique organic pathology in the small and larg...\n --CHILD--> [DD91.2] Functional diarrhoea\n Def: Functional diarrhoea is a continuous or recurrent syndrome characterised by the passage of loose (mushy) or watery stools without abdominal pain or discomfort....", "[DD91.Z] Irritable bowel syndrome or functional bowel disorders, unspecified\n --PARENT--> [DD91] Irritable bowel syndrome or certain specified functional bowel disorders\n Def: This group incorporates functional bowel disorders which principally present symptoms attributable to the intestinal tract in the absence of specific and unique organic pathology in the small and larg...\n --CHILD--> [DD91.0] Irritable bowel syndrome\n Def: Irritable bowel syndrome (IBS) is a functional bowel disorder in which abdominal pain or discomfort is associated with defecation or a change in bowel habit, and with features of disordered defecation...", "[9E1Z] Diseases of the visual system, unspecified\n --PARENT--> [09] Diseases of the visual system\n Def: This refers to any diseases of the visual system, which includes the eyes and adnexa, the visual pathways and brain areas, which initiate and control visual perception and visually guided behaviour....\n --CHILD--> [?] Disorders of the eyeball - posterior segment\n Def: This refers to disorders of the back two-thirds of the eye that includes the anterior hyaloid membrane and all of the optical structures behind it: the vitreous humour, retina, choroid, and optic nerv...", "[9E1Z] Diseases of the visual system, unspecified\n --PARENT--> [09] Diseases of the visual system\n Def: This refers to any diseases of the visual system, which includes the eyes and adnexa, the visual pathways and brain areas, which initiate and control visual perception and visually guided behaviour....\n --EXCLUDES--> [?] Certain infectious or parasitic diseases\n Def: This chapter includes certain conditions caused by pathogenic organisms or microorganisms, such as bacteria, viruses, parasites or fungi....", "[6B60.Z] Dissociative neurological symptom disorder, with unspecified symptoms\n --PARENT--> [6B60] Dissociative neurological symptom disorder\n Def: Dissociative neurological symptom disorder is characterised by the presentation of motor, sensory, or cognitive symptoms that imply an involuntary discontinuity in the normal integration of motor, sen...\n --EXCLUDES--> [?] Factitious disorders\n Def: Factitious disorders are characterised by intentionally feigning, falsifying, inducing, or aggravating medical, psychological, or behavioural signs and symptoms or injury in oneself or in another pers...", "[6B60.Z] Dissociative neurological symptom disorder, with unspecified symptoms\n --PARENT--> [6B60] Dissociative neurological symptom disorder\n Def: Dissociative neurological symptom disorder is characterised by the presentation of motor, sensory, or cognitive symptoms that imply an involuntary discontinuity in the normal integration of motor, sen...\n --CHILD--> [6B60.0] Dissociative neurological symptom disorder, with visual disturbance\n Def: Dissociative neurological symptom disorder, with visual disturbance is characterised by visual symptoms such as blindness, tunnel vision, diplopia, visual distortions or hallucinations that are not co..." ]	DD91.Z	Irritable bowel syndrome or functional bowel disorders, unspecified	[ { "from_icd11": "DD91.Z", "icd10_code": "K598", "icd10_title": "Other specified functional intestinal disorders" }, { "from_icd11": "DD91.Z", "icd10_code": "K599", "icd10_title": "Functional intestinal disorder, unspecified" }, { "from_icd11": "DD91.Z", "icd10_code": "K592", "icd10_title": "Neurogenic bowel, not elsewhere classified" }, { "from_icd11": "DD91.Z", "icd10_code": "K55-K64", "icd10_title": "" }, { "from_icd11": "DD91.Z", "icd10_code": "K59", "icd10_title": "Other functional intestinal disorders" }, { "from_icd11": "9E1Z", "icd10_code": "H5500", "icd10_title": "Unspecified nystagmus" }, { "from_icd11": "9E1Z", "icd10_code": "H5509", "icd10_title": "Other forms of nystagmus" }, { "from_icd11": "9E1Z", "icd10_code": "H5581", "icd10_title": "Saccadic eye movements" }, { "from_icd11": "9E1Z", "icd10_code": "H5501", "icd10_title": "Congenital nystagmus" }, { "from_icd11": "9E1Z", "icd10_code": "H5502", "icd10_title": "Latent nystagmus" }, { "from_icd11": "9E1Z", "icd10_code": "H5589", "icd10_title": "Other irregular eye movements" }, { "from_icd11": "9E1Z", "icd10_code": "H5503", "icd10_title": "Visual deprivation nystagmus" }, { "from_icd11": "9E1Z", "icd10_code": "H5504", "icd10_title": "Dissociated nystagmus" }, { "from_icd11": "9E1Z", "icd10_code": "H44522", "icd10_title": "Atrophy of globe, left eye" }, { "from_icd11": "9E1Z", "icd10_code": "H3552", "icd10_title": "Pigmentary retinal dystrophy" } ]	K598	Other specified functional intestinal disorders
By the time of her first outpatient visit at the Unit of Psychiatry, the patient was 18 years old. She was well kept, fully cooperative, and able to provide detailed medical history information. Her mood was euthymic. No signs of anxiety were observed. No hallucinatory behavior was observed. The patient showed a ruminative process of thinking and the thought content revealed worries about her own body image and weight control. She also reported recent episodes of binge eating and subsequent verbalization of suicidal intent, showing poor insight and flattened affect. Her sleep/wake rhythm was described as normal. She did not show any neurological signs or deficits. She did not appear grossly impaired on a cognitive basis, with both recent and remote memory preserved. Orientation in space, time, and person was intact. A thorough psychological assessment was then carried out using the Eating Disorder Inventory (EDI)-3, the Body Uneasiness Test (BUT), the Symptom Checklist (SCL)-90, the Binge Eating Scale (BES), the Minnesota Multiphasic Personality Inventory (MMPI)-2, the Structured Clinical Interview for Diagnostic and Statistical Manual of Mental Disorders, -Fifth Edition, -Clinician Version (SCID-5-CV), and the SCID-5, -Personality Disorders (SCID-5-PD), converging towards the diagnosis of other specified feeding or eating disorder (OSFED) and prior major depressive episode, according to the Diagnostic and Statistical Manual of Mental Disorders, -Fifth Edition (DSM-5): in particular, characteristics of drive for thinness, weight phobia, compulsive control of body image, tendency to perfectionism, personal insecurity, somatization, and emotional dysregulation emerged. On the contrary, the presence of a personality disorder was ruled out. Ongoing pharmacotherapy with sertraline was maintained at first and olanzapine was progressively titrated to 7.5 mg/day and then tapered off and discontinued due to excessive sedation. Psychotherapy intervention and monitoring of the eating disorder were initiated and continued for about one year. At a follow-up visit planned one year after her first evaluation, a good control over aberrant eating behaviors was reported, whereas egodystonic blasphemous thoughts persisted, often followed by the urge to perform specific movements or acts (e.g., running to exhaustion, screaming, beating the wall, beating her chest), of which the patient felt ashamed. Also, motor and vocal tics including echopraxia (i.e., repeatedly writing down sentences pronounced by others), echolalia (i.e., verbally repeating sentences pronounced by others), and tongue clicking emerged at that time. During the next 3 years, the clinical picture was monitored on an almost monthly basis. Despite good compensation of affective symptoms, the eating disorder, and early perceptual distortions, monitoring indicated that the intrusive thoughts, repetitive behaviors, and tics kept fluctuating. A brain MRI scan taken when she was 19 years old revealed consistent findings with the previous one, confirming the presence of DWV. A neuropsychological assessment was performed using the Wechsler Adult Intelligence Scale, -Fourth Edition (WAIS-IV). The patient’s full-scale intelligence quotient (FSIQ) was in the average range as per the normative data (FSIQ = 95), with “Verbal Comprehension” hard to interpret due to high internal discrepancy, “Perceptual Reasoning” and “Verbal Memory” in the average range, and “Processing Speed” in the very low average range. Further assessment using the Raven’s Standard Progressive Matrices (SPM) confirmed an average intelligence. Moderate deficits in executive function and spatial cognition were highlighted with the Cerebellar Cognitive Affective syndrome (CCAS) scale. The reported level of personal autonomy was investigated through the World Health Organization Disability Assessment Schedule (WHODAS) 2.0, which revealed mildly impaired general functioning. Individual difficulties were reported in the domains of Comprehension and Communication, Activities of Daily Living, and Social Interaction. A differential diagnosis with full-blown psychotic disorders was considered. However, the patient easily dismissed the puzzling auditory experiences occurring prior to her presentation to the Unit, which were recognized as intrusive thoughts that were just her imagination. Also, repetitive behaviors were perceived as egodystonic and not enacted in response to the delusional content of hallucinations. No other potential psychotic symptoms were detected for the entire duration of the healthcare path. Consequently, a final diagnosis of obsessive–compulsive disorder (OCD) associated with persistent motor and vocal tic disorder was made, according to the DSM-5. Throughout the years, several attempts were made to adjust pharmacotherapy, never resulting in complete remission of symptoms. First, sertraline as monotherapy was titrated to 200 mg/day, but obsessive–compulsive symptoms and tics did not subside. Aripiprazole at a dosage of 10 mg/day was then added to sertraline, with beneficial effects over intrusive thoughts and tics, but with early occurrence of constipation and a sudden drop in white blood cell count and platelet count, so it was reduced to a dosage of 5 mg/day with relapsing symptoms. Adjunctive therapy with haloperidol up to a dosage of 4 mg/day was also beneficial on intrusive thoughts but led to the occurrence of increasing anxiety and daytime sedation, so it was suspended concurrently with aripiprazole. Quetiapine extended release (ER; 50 mg/day) and subsequent lurasidone (74 mg/day) add-on attempts were not effective and quickly switched to risperidone (up to 3 mg/day), which the patient asked to stop after about one year due to excessive weight gain, although a good control over symptoms was observed. Ongoing sertraline was then cross-titrated with clomipramine (150 mg/day) as a third-line treatment for OCD, leading to a rapid improvement in obsessive thinking, but suspended shortly after due to the occurrence of iatrogenic acute hepatitis. The patient was then started on brexpiprazole (up to 4 mg/day, then lowered to the present dosage of 2 mg/day), which has been continued for over one year and a half at the time of writing.	4.078125	0.969238	sec[1]/sec[1]/p[0]	en	0.999996	PMC11048094	https://doi.org/10.3390/brainsci14040362	[ "eating", "thoughts", "which", "time", "control", "scale", "disorders", "sertraline", "over", "tics" ]	[ { "code": "6B8Z", "title": "Feeding or eating disorders, unspecified" }, { "code": "MG43.1", "title": "Overeating" }, { "code": "MG43.40", "title": "Refusal of food, not elsewhere classified" }, { "code": "MG43.Y", "title": "Other specified symptoms or signs concerning food or fluid intake" }, { "code": "6B8Y", "title": "Other specified feeding or eating disorders" }, { "code": "MB26.12", "title": "Thought withdrawal" }, { "code": "MB25.3", "title": "Thought blocking" }, { "code": "MB26.11", "title": "Thought insertion" }, { "code": "MB21.B", "title": "Racing thoughts" }, { "code": "MB25.02", "title": "Disorganised thinking" } ]	=== ICD-11 CODES FOUND === [6B8Z] Feeding or eating disorders, unspecified Also known as: Feeding or eating disorders, unspecified \| Eating disorder, not elsewhere classified \| eating disorder NOS [MG43.1] Overeating Definition: The consumption of excess food in relation to energy and nutritional requirements. Also known as: Overeating \| Excessive eating \| gluttony \| hyperalimentation \| Hyperalimentation NOS Includes: Excessive eating Excludes: Bipolar or related disorders \| Depressive disorders \| Feeding or eating disorders [MG43.40] Refusal of food, not elsewhere classified Also known as: Refusal of food, not elsewhere classified \| stopped eating \| refusal to eat Excludes: Intentional self-harm by lack of food \| Anorexia [MG43.Y] Other specified symptoms or signs concerning food or fluid intake Also known as: Other specified symptoms or signs concerning food or fluid intake \| Eating problem in child [6B8Y] Other specified feeding or eating disorders Also known as: Other specified feeding or eating disorders [MB26.12] Thought withdrawal Definition: The experience that one's thoughts are being removed by an outside person or force. Also known as: Thought withdrawal [MB25.3] Thought blocking Definition: A phenomenon usually manifested by the person's speech being suddenly interrupted by silences, experienced as a quick and total emptying of the mind. Also known as: Thought blocking [MB26.11] Thought insertion Definition: The experience that certain thoughts are being placed in one's mind by others. Also known as: Thought insertion [MB21.B] Racing thoughts Definition: Subjective perception of accelerated thought processes. Also known as: Racing thoughts [MB25.02] Disorganised thinking Definition: A disturbance in the associative thought process typically manifested in speech in which the person shifts suddenly from one topic to another that is unrelated or minimally related to the first. The individual gives no indication of being aware of the disconnectedness or illogicality of their thinking. Also known as: Disorganised thinking \| thought derailment \| loose associations \| disorganised speech === GRAPH WALKS === --- Walk 1 --- [6B8Z] Feeding or eating disorders, unspecified --PARENT--> [?] Feeding or eating disorders Def: Feeding and Eating Disorders involve abnormal eating or feeding behaviours that are not explained by another health condition and are not developmentally appropriate or culturally sanctioned. Feeding ... --CHILD--> [6B81] Bulimia Nervosa Def: Bulimia Nervosa is characterised by frequent, recurrent episodes of binge eating (e.g. once a week or more over a period of at least one month). A binge eating episode is a distinct period of time dur... --- Walk 2 --- [6B8Z] Feeding or eating disorders, unspecified --PARENT--> [?] Feeding or eating disorders Def: Feeding and Eating Disorders involve abnormal eating or feeding behaviours that are not explained by another health condition and are not developmentally appropriate or culturally sanctioned. Feeding ... --CHILD--> [6B82] Binge eating disorder Def: Binge eating disorder is characterised by frequent, recurrent episodes of binge eating (e.g. once a week or more over a period of several months). A binge eating episode is a distinct period of time d... --- Walk 3 --- [MG43.1] Overeating Def: The consumption of excess food in relation to energy and nutritional requirements.... --EXCLUDES--> [?] Feeding or eating disorders Def: Feeding and Eating Disorders involve abnormal eating or feeding behaviours that are not explained by another health condition and are not developmentally appropriate or culturally sanctioned. Feeding ... --PARENT--> [?] Mental, behavioural or neurodevelopmental disorders Def: Mental, behavioural and neurodevelopmental disorders are syndromes characterised by clinically significant disturbance in an individual's cognition, emotional regulation, or behaviour that reflects a ... --- Walk 4 --- [MG43.1] Overeating Def: The consumption of excess food in relation to energy and nutritional requirements.... --EXCLUDES--> [?] Depressive disorders Def: Depressive disorders are characterised by depressive mood (e.g., sad, irritable, empty) or loss of pleasure accompanied by other cognitive, behavioural, or neurovegetative symptoms that significantly ... --CHILD--> [?] Recurrent depressive disorder Def: Recurrent depressive disorder is characterised by a history of at least two depressive episodes separated by at least several months without significant mood disturbance. A depressive episode is chara... --- Walk 5 --- [MG43.40] Refusal of food, not elsewhere classified --PARENT--> [MG43.4] Insufficient intake of food or water due to self neglect --CHILD--> [MG43.40] Refusal of food, not elsewhere classified --- Walk 6 --- [MG43.40] Refusal of food, not elsewhere classified --EXCLUDES--> [?] Anorexia Def: Anorexia is a pathological lack or loss of appetite.... --EXCLUDES--> [?] Decreased appetite Def: Intermittent or persistent decreased motivation or desire to eat food as compared to what is typical for the individual....	[ "[6B8Z] Feeding or eating disorders, unspecified\n --PARENT--> [?] Feeding or eating disorders\n Def: Feeding and Eating Disorders involve abnormal eating or feeding behaviours that are not explained by another health condition and are not developmentally appropriate or culturally sanctioned. Feeding ...\n --CHILD--> [6B81] Bulimia Nervosa\n Def: Bulimia Nervosa is characterised by frequent, recurrent episodes of binge eating (e.g. once a week or more over a period of at least one month). A binge eating episode is a distinct period of time dur...", "[6B8Z] Feeding or eating disorders, unspecified\n --PARENT--> [?] Feeding or eating disorders\n Def: Feeding and Eating Disorders involve abnormal eating or feeding behaviours that are not explained by another health condition and are not developmentally appropriate or culturally sanctioned. Feeding ...\n --CHILD--> [6B82] Binge eating disorder\n Def: Binge eating disorder is characterised by frequent, recurrent episodes of binge eating (e.g. once a week or more over a period of several months). A binge eating episode is a distinct period of time d...", "[MG43.1] Overeating\n Def: The consumption of excess food in relation to energy and nutritional requirements....\n --EXCLUDES--> [?] Feeding or eating disorders\n Def: Feeding and Eating Disorders involve abnormal eating or feeding behaviours that are not explained by another health condition and are not developmentally appropriate or culturally sanctioned. Feeding ...\n --PARENT--> [?] Mental, behavioural or neurodevelopmental disorders\n Def: Mental, behavioural and neurodevelopmental disorders are syndromes characterised by clinically significant disturbance in an individual's cognition, emotional regulation, or behaviour that reflects a ...", "[MG43.1] Overeating\n Def: The consumption of excess food in relation to energy and nutritional requirements....\n --EXCLUDES--> [?] Depressive disorders\n Def: Depressive disorders are characterised by depressive mood (e.g., sad, irritable, empty) or loss of pleasure accompanied by other cognitive, behavioural, or neurovegetative symptoms that significantly ...\n --CHILD--> [?] Recurrent depressive disorder\n Def: Recurrent depressive disorder is characterised by a history of at least two depressive episodes separated by at least several months without significant mood disturbance. A depressive episode is chara...", "[MG43.40] Refusal of food, not elsewhere classified\n --PARENT--> [MG43.4] Insufficient intake of food or water due to self neglect\n --CHILD--> [MG43.40] Refusal of food, not elsewhere classified", "[MG43.40] Refusal of food, not elsewhere classified\n --EXCLUDES--> [?] Anorexia\n Def: Anorexia is a pathological lack or loss of appetite....\n --EXCLUDES--> [?] Decreased appetite\n Def: Intermittent or persistent decreased motivation or desire to eat food as compared to what is typical for the individual...." ]	6B8Z	Feeding or eating disorders, unspecified	[ { "from_icd11": "6B8Z", "icd10_code": "F5082", "icd10_title": "Avoidant/restrictive food intake disorder" }, { "from_icd11": "6B8Z", "icd10_code": "F5089", "icd10_title": "Other specified eating disorder" }, { "from_icd11": "6B8Z", "icd10_code": "F5081", "icd10_title": "Binge eating disorder" }, { "from_icd11": "6B8Z", "icd10_code": "F9829", "icd10_title": "Other feeding disorders of infancy and early childhood" }, { "from_icd11": "6B8Z", "icd10_code": "F9821", "icd10_title": "Rumination disorder of infancy" }, { "from_icd11": "6B8Z", "icd10_code": "F508", "icd10_title": "Other eating disorders" }, { "from_icd11": "6B8Z", "icd10_code": "F509", "icd10_title": "Eating disorder, unspecified" }, { "from_icd11": "6B8Z", "icd10_code": "F50", "icd10_title": "Eating disorders" }, { "from_icd11": "6B8Z", "icd10_code": "F504", "icd10_title": "" }, { "from_icd11": "6B8Z", "icd10_code": "F505", "icd10_title": "" }, { "from_icd11": "6B8Z", "icd10_code": "F982", "icd10_title": "Other feeding disorders of infancy and childhood" }, { "from_icd11": "MG43.1", "icd10_code": "R632", "icd10_title": "Polyphagia" }, { "from_icd11": "MB25.3", "icd10_code": "R4689", "icd10_title": "Other symptoms and signs involving appearance and behavior" }, { "from_icd11": "MB25.3", "icd10_code": "R4681", "icd10_title": "Obsessive-compulsive behavior" }, { "from_icd11": "MB25.3", "icd10_code": "R468", "icd10_title": "Other symptoms and signs involving appearance and behavior" } ]	F5082	Avoidant/restrictive food intake disorder
A 50-year-old man with pharyngalgia and right neck mass presented to the hospital in December 2020. The patient had previously been diagnosed with hepatitis B infection and diabetes, had long-term work experience with chemical drugs and had no history of smoking, alcohol consumption and history of cancers. Physical examination showed an exophytic yellow-white cauliflower-like mass on the surface of the right tonsil, partially extending beyond the midline, but not invading other tissues around the oropharynx . Several swollen lymph nodes were palpable on both sides of the neck. Head and neck magnetic resonance imaging (MRI) showed a mass of 32 mm × 16 mm in the right tonsil and multiple enlarged lymph nodes around the bilateral carotid arteries. The largest lymph nodes were 40 mm × 23 mm (right) and 36 mm × 31 mm (left). Whole-body Positron Emission Tomography-Computed Tomography (PET/CT) showed multiple enlarged lymph nodes in the mediastinum and hilum, with a maximum of 21 × 18 mm . Biopsy of cervical lymph nodes and the tonsil mass revealed diffuse growth of medium to large lymphocyte tumor cells, indicating lymphoma and requiring further assessment. The biopsy specimens were delivered to the central laboratory for analysis (Guangzhou, China). Due to severe pharyngalgia, dysphagia and wheezing, the patient was administered two cycles of chemotherapy (doxorubicin liposomal at 40 mg on d1, vincristine at 2 mg on d1 and prednisone at 100 mg on d1-d5) from December 31, 2020. However, head and neck MRI showed a 36 × 25 mm mass in the right tonsil, and the largest cervical lymph nodes were 45 × 37 mm (right) and 36 × 39 mm (left). Chest CT showed multiple enlarged lymph nodes in the mediastinum and hilus, with a maximum of 29 mm × 23 mm . According to Response Evaluation Criteria in Solid Tumors (RECIST) version 1.1, the efficacy was evaluated as progressive disease (PD). The pathological results of the central laboratory were confirmed on March 5, 2021. Tonsil mass was demonstrated as malignancy by hematoxylin-eosin (H&E) staining which showed small foci of heterotypic tumor cells, some cells with light stained and obvious large nucleoli cell . Immunohistochemistry (IHC) revealed that the tumor cells were positive for Ki-67 (95%), CD138 and Vimentin, PD-L1 (90%) , and negative for CK, p16, CD20, CD3, CD30, CD4, CD8, LCA, CD5, CD7, ALK, CD56, CD31, ERG, CD21, CD23, CD38, CAM5.2, CD117, EMA, S-100, SOX-10 and Mum-1, κ, λ. Epstein-Barr encoding region (EBER) in situ hybridization was negative. The final diagnosis of this patient was based on a thorough review of gene rearrangements analysis, histopathology and IHC analysis. Lymphoma was excluded by the gene rearrangement assay (lymphoma biomarkers IGH, IGK and IGL were negative, S1). According to the H&E staining, IHC and PET/CT results, the patient was finally diagnosed with undifferentiated tonsillar carcinoma with multiple cervical and mediastinal lymph nodes metastases [cT2N2M1, stage IVC, American Joint Committee on Cancer (AJCC) 8 th edition]. Next generation sequencing (NGS, Geneseeq Technology Inc) identified germline BRCA1 gene P.S1374Rfs * 3 exon 12 frameshift mutation and a high TMB of 30.7 mutations/MB in the tumor tissue. Considering the high risk of operative hemorrhage and asphyxia, surgical oncologist believed that the risks of surgery outweigh the benefits. After carefully evaluating the patient’s performance status, treatment tolerance, tumor imaging and the feasibility of intravascular interventional therapy, the multidisciplinary team developed a focused treatment plan individualized to the patient. The patient received bilateral external carotid artery branch embolization on March 15, 2021 and March 18, 2021 because of the obvious enlargement of the tonsil mass and the high risk of airway obstruction. Intravascular interventional therapy was less invasive and had a low risk of surgical complications. After locoregional operation, the patient had only mild pain around the neck, the tonsil mass was reduced to 20×15 mm, and the largest cervical lymph nodes were 32 × 20 mm (right) and 27 × 17 mm (left) . According to the germline BRCA1 mutation, high TMB and the PD-L1 positive status, the patient was administered 300 mg niraparib QD and 200 mg tislelizumab Q3W from March 25, 2021. Follow-up MRI and CT revealed a striking decrease in tumor burden after the second treatment cycle. The tonsil mass basically disappeared, and the largest cervical lymph nodes shrank to 21 × 18 mm (right) and 18 × 13 mm (left). The hilar lymph node was reduced to 20 × 14 mm . The patient achieved a PR under treatment of niraparib combined with tislelizumab according to the RESISIT 1.1 criteria. Surveillance imaging showed continuous partial remission for 12 months . The patient developed mild fatigue, leukopenia (2.26×10 9 /L) and anemia (HGB 83g/L) in the first month of niraparib administration. These adverse events were rated as levels 1-2 (CTCAE 5.0) and resolved after niraparib was reduced to 200 mg QD. The patient resumed normal work with a high quality of life. In March 2022, the patient was admitted to the hospital with hematochezia and anemia (HGB 67 g/L). Colonoscopy revealed a mass in the ascending colon, accounting for about 2/3 of the intestinal lumen, with superficial fragility and hemorrhage . Whole-body CT and barium meal examination of the digestive tract showed a mass of 43mm x 22mm in the proximal ascending colon, with no signs of new lesions elsewhere . The patient underwent ascending colectomy with lymph node dissection on April 28, 2022. Postoperative pathology showed that the colonic mass was metastatic undifferentiated tonsillar carcinoma, with no lymph node metastasis . Re-examination of MRI and CT revealed no evidence of progression to the tonsils, cervical lymph nodes, and mediastinal and hilar lymph nodes . However, owing to the worse performance status after intestinal surgery, the patient declined any further treatment, except palliative care. Eventually, he died of multiple organ failure in July 2022. The patient’s PFS and OS were 12 months and 19 months, respectively, after treatment with niraparib and tislelizumab. The timeline of the relevant information is shown in Figure 3 .	4.148438	0.962891	sec[1]/p[0]	en	0.999996	PMC10234503	https://doi.org/10.3389/fonc.2023.1078814	[ "lymph", "nodes", "tonsil", "cervical", "tumor", "neck", "multiple", "march", "niraparib", "largest" ]	[ { "code": "BD9Z", "title": "Disorders of lymphatic vessels or lymph nodes, unspecified" }, { "code": "BD90.Z", "title": "Lymphadenitis, unspecified" }, { "code": "BD90.Y", "title": "Other specified lymphadenitis" }, { "code": "BD9Y", "title": "Other specified disorders of lymphatic vessels or lymph nodes" }, { "code": "MA01.Z", "title": "Enlarged lymph nodes, unspecified" }, { "code": "FA20.0", "title": "Seropositive rheumatoid arthritis" }, { "code": "MF30", "title": "Breast lump or mass female" }, { "code": "BB40", "title": "Acute or subacute infectious endocarditis" }, { "code": "FA0Z", "title": "Osteoarthritis, unspecified" }, { "code": "FA85.10", "title": "Localised central endplate defect" } ]	=== ICD-11 CODES FOUND === [BD9Z] Disorders of lymphatic vessels or lymph nodes, unspecified Also known as: Disorders of lymphatic vessels or lymph nodes, unspecified \| Lymphatic system disorders \| lymph disease NOS \| lymph gland disease \| Lymphatic system disease NOS [BD90.Z] Lymphadenitis, unspecified Also known as: Lymphadenitis, unspecified \| Lymphadenitis \| adenitis NOS \| inflammation of gland \| lymphatic gland inflammation [BD90.Y] Other specified lymphadenitis Also known as: Other specified lymphadenitis \| Dermatopathic lymphadenopathy \| lipomelanotic reticulosis \| Infective inguinal bubo \| bubo [BD9Y] Other specified disorders of lymphatic vessels or lymph nodes Also known as: Other specified disorders of lymphatic vessels or lymph nodes \| Chylous cyst \| Mesentery chylous cyst \| Peritoneum chylous cyst \| Lymphocele [MA01.Z] Enlarged lymph nodes, unspecified Also known as: Enlarged lymph nodes, unspecified \| Enlarged lymph nodes \| swollen glands \| Lymphadenopathy \| adenopathy [FA20.0] Seropositive rheumatoid arthritis Also known as: Seropositive rheumatoid arthritis \| high positive rheumatoid factor \| low positive rheumatoid factor \| high positive anticitrullinated protein antibody \| low positive anticitrullinated protein antibody [MF30] Breast lump or mass female Also known as: Breast lump or mass female \| breast irregular nodularity \| breast node \| lump in breast \| lump or mass in breast NOS [BB40] Acute or subacute infectious endocarditis Also known as: Acute or subacute infectious endocarditis \| subacute infective endocarditis NOS \| infective endocarditis NOS \| acute infective endocarditis NOS \| infectious endocarditis Excludes: Infectious myocarditis [FA0Z] Osteoarthritis, unspecified Also known as: Osteoarthritis, unspecified \| osteoarthritis NOS \| arthrosis NOS \| OA - [osteoarthritis] \| Osteoarthritis with determinants [FA85.10] Localised central endplate defect Also known as: Localised central endplate defect \| Schmorl nodes \| schmorl's nodes \| schmorl's nodules === GRAPH WALKS === --- Walk 1 --- [BD9Z] Disorders of lymphatic vessels or lymph nodes, unspecified --PARENT--> [?] Disorders of lymphatic vessels or lymph nodes Def: Disorders due to developmental and acquired disturbances of lymph circulation and drainage and to infective disorders of lymph vessels and nodes.... --CHILD--> [BD92] Lymphangiectasia --- Walk 2 --- [BD9Z] Disorders of lymphatic vessels or lymph nodes, unspecified --PARENT--> [?] Disorders of lymphatic vessels or lymph nodes Def: Disorders due to developmental and acquired disturbances of lymph circulation and drainage and to infective disorders of lymph vessels and nodes.... --EXCLUDES--> [?] Enlarged lymph nodes Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes.... --- Walk 3 --- [BD90.Z] Lymphadenitis, unspecified --PARENT--> [BD90] Lymphadenitis --EXCLUDES--> [?] Enlarged lymph nodes Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes.... --- Walk 4 --- [BD90.Z] Lymphadenitis, unspecified --PARENT--> [BD90] Lymphadenitis --EXCLUDES--> [?] Human immunodeficiency virus disease Def: A case of HIV infection is defined as an individual with HIV infection irrespective of clinical stage including severe or stage 4 clinical disease (also known as AIDS) confirmed by laboratory criteria... --- Walk 5 --- [BD90.Y] Other specified lymphadenitis --PARENT--> [BD90] Lymphadenitis --PARENT--> [?] Disorders of lymphatic vessels or lymph nodes Def: Disorders due to developmental and acquired disturbances of lymph circulation and drainage and to infective disorders of lymph vessels and nodes.... --- Walk 6 --- [BD90.Y] Other specified lymphadenitis --PARENT--> [BD90] Lymphadenitis --EXCLUDES--> [?] Enlarged lymph nodes Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes....	[ "[BD9Z] Disorders of lymphatic vessels or lymph nodes, unspecified\n --PARENT--> [?] Disorders of lymphatic vessels or lymph nodes\n Def: Disorders due to developmental and acquired disturbances of lymph circulation and drainage and to infective disorders of lymph vessels and nodes....\n --CHILD--> [BD92] Lymphangiectasia", "[BD9Z] Disorders of lymphatic vessels or lymph nodes, unspecified\n --PARENT--> [?] Disorders of lymphatic vessels or lymph nodes\n Def: Disorders due to developmental and acquired disturbances of lymph circulation and drainage and to infective disorders of lymph vessels and nodes....\n --EXCLUDES--> [?] Enlarged lymph nodes\n Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes....", "[BD90.Z] Lymphadenitis, unspecified\n --PARENT--> [BD90] Lymphadenitis\n --EXCLUDES--> [?] Enlarged lymph nodes\n Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes....", "[BD90.Z] Lymphadenitis, unspecified\n --PARENT--> [BD90] Lymphadenitis\n --EXCLUDES--> [?] Human immunodeficiency virus disease\n Def: A case of HIV infection is defined as an individual with HIV infection irrespective of clinical stage including severe or stage 4 clinical disease (also known as AIDS) confirmed by laboratory criteria...", "[BD90.Y] Other specified lymphadenitis\n --PARENT--> [BD90] Lymphadenitis\n --PARENT--> [?] Disorders of lymphatic vessels or lymph nodes\n Def: Disorders due to developmental and acquired disturbances of lymph circulation and drainage and to infective disorders of lymph vessels and nodes....", "[BD90.Y] Other specified lymphadenitis\n --PARENT--> [BD90] Lymphadenitis\n --EXCLUDES--> [?] Enlarged lymph nodes\n Def: Enlarged lymph node is called lymphadenopathy which means the abnormal enlargement of lymph nodes...." ]	BD9Z	Disorders of lymphatic vessels or lymph nodes, unspecified	[ { "from_icd11": "BD9Z", "icd10_code": "I898", "icd10_title": "Other specified noninfective disorders of lymphatic vessels and lymph nodes" }, { "from_icd11": "BD9Z", "icd10_code": "I899", "icd10_title": "Noninfective disorder of lymphatic vessels and lymph nodes, unspecified" }, { "from_icd11": "BD9Z", "icd10_code": "I89", "icd10_title": "Other noninfective disorders of lymphatic vessels and lymph nodes" }, { "from_icd11": "BD90.Z", "icd10_code": "I889", "icd10_title": "Nonspecific lymphadenitis, unspecified" }, { "from_icd11": "BD90.Z", "icd10_code": "I88", "icd10_title": "Nonspecific lymphadenitis" }, { "from_icd11": "BD90.Z", "icd10_code": "I888", "icd10_title": "Other nonspecific lymphadenitis" }, { "from_icd11": "BD90.Z", "icd10_code": "L00-L08", "icd10_title": "" }, { "from_icd11": "MA01.Z", "icd10_code": "R599", "icd10_title": "Enlarged lymph nodes, unspecified" }, { "from_icd11": "MA01.Z", "icd10_code": "R59", "icd10_title": "Enlarged lymph nodes" }, { "from_icd11": "FA20.0", "icd10_code": "M0569", "icd10_title": "Rheumatoid arthritis of multiple sites with involvement of other organs and systems" }, { "from_icd11": "FA20.0", "icd10_code": "M0579", "icd10_title": "Rheumatoid arthritis with rheumatoid factor of multiple sites without organ or systems involvement" }, { "from_icd11": "FA20.0", "icd10_code": "M05612", "icd10_title": "Rheumatoid arthritis of left shoulder with involvement of other organs and systems" }, { "from_icd11": "FA20.0", "icd10_code": "M0570", "icd10_title": "Rheumatoid arthritis with rheumatoid factor of unspecified site without organ or systems involvement" }, { "from_icd11": "FA20.0", "icd10_code": "M0560", "icd10_title": "Rheumatoid arthritis of unspecified site with involvement of other organs and systems" }, { "from_icd11": "FA20.0", "icd10_code": "M0500", "icd10_title": "Felty's syndrome, unspecified site" } ]	I898	Other specified noninfective disorders of lymphatic vessels and lymph nodes
A 51-year-old patient presented with severe restlessness, insomnia, impaired concentration, increased aggressiveness, redness of the face and body hair growth, all gradually developing over the last six months. Fifteen years previously, he had undergone an orchidectomy for LCT, and thirteen years later, excision of a retroperitoneal mass, confirmed on histology as LCT metastasis. Imaging revealed multiple lesions consistent with liver, lung and retroperitoneal metastases. Immunohistochemistry of a tissue biopsy confirmed vimentin-positive, inhibin-negative metastatic LCT. Serum testosterone was very high at 93 nmol/L (normal male reference range 7–27 nmol/L). Urinary steroid profiling by gas chromatography–mass spectrometry (GC–MS) showed increased androgen metabolite excretion as well as increased excretion of DHEA, metabolites of pregnenolone, progesterone, 17-hydroxypregnenolone, 17-hydroxyprogesterone and cortisol (230 µg/24 h; normal <130) . Prognosis was assessed as poor and the patient declined chemotherapy. However, he agreed to the initiation of mitotane treatment in an attempt to improve the clinical signs and symptoms of tumor-related androgen excess that were significantly limiting his quality of life. Mitotane dose was gradually titrated to 3 g per day, with concurrent hydrocortisone replacement (20 mg tid). Within a few weeks, androgen excretion decreased from 101,476 to 12,827 µg/24 h, with evidence of significant inhibition of 5α-reductase activity and normalization of other steroids that were increased at baseline ( Table 1 ). Plasma mitotane concentrations considered therapeutic (anti-proliferative) in the context of adrenocortical carcinoma (14–20 mg/L) ( 16 ) were reached after 5 months of treatment ( Supplementary Table 1 , see section on supplementary data given at the end of this article). Follow-up imaging still showed progressive disease at two months, but stable disease according to RECIST 1.1 criteria after six months of mitotane treatment . Alongside the decrease in androgens, the patient reported a significant improvement of his previously debilitating clinical signs and symptoms. He returned to full-time work and enjoyed good quality of life. After 10 months of mitotane treatment, he died suddenly of a suspected myocardial infarction; no post-mortem examination was carried out. Figure 1 (Panel A) Steroid synthesis in the two patients with metastatic testicular Leydig cell tumor as assessed by mass spectrometry-based 24-h urinary steroid profiling before initiation of mitotane treatment (log scale; closed circles, patient 1; open triangles, patient 2). Box plots represent medians and interquartile ranges from a group of 24 healthy male volunteers (age: 40–60 years); whiskers represent the full range. (Panel B) Immunohistochemical staining for sterol-O-acyltransferase 1 (SOAT1) using formalin-fixed paraffin-embedded tissue from the recurrent tumor of patient 2, demonstrating high (60% of cells) to moderate (30% of cells) expression of SOAT1 in the tumor tissue. Table 1 24-h urine steroid metabolite excretion (µg/24 h) in the two patients with metastatic Leydig cell tumor before (= baseline) and during mitotane treatment. The male reference range is derived from the 24-h urine steroid excretion observed in 24 healthy men aged 40–60 years. The numbers of the steroid metabolites relate to the numbers in Fig. 1A . The total glucocorticoid metabolites were calculated as the sum of metabolites 20, 22–25 and 27–30. Median (min–max) steroid excretion in healthy men (µg/24 h) Patient 1 Patient 2 Baseline Mitotane Baseline Mitotane Month 1 Month 2 Month 4 Month 6 Month 9 Month 4 Androgen and androgen precursor metabolites 1 Androsterone 1684 44,744 13,833 11,823 9790 4448 4440 38,092 597 2 Etiocholonaolone 1668 56,732 30,283 37,617 22,408 9798 8387 31,016 2697 3 11β-Hydroxy-androsterone 609 2066 1414 2318 301 169 174 13,351 643 4 Dehydroepiandrosterone (DHEA) 202 1939 1034 434 294 194 311 47,344 359 5 16α-Hydroxy-DHEA 269 4404 9179 4098 3510 2533 6250 23,569 1126 6 5-Pregnenetriol 181 (38–951) 1558 2574 2484 2600 1575 2483 20,891 1053 7 5-Pregnenediol 326 (64–801) 19,972 27,844 24,732 25,548 9545 15,450 168,192 11,304 Mineralocorticoids and mineralocorticoid precursor metabolites 8 Tetrahydro-11-deoxycorticosterone 94 (22–290) 445 195 162 188 29 128 308 22 9 5α-Tetrahydro-11-deoxycorticosterone 107 (50–360) 62 100 87 52 32 49 74 18 10 Tetraydrocorticosterone 97 (24–258) 300 154 263 169 41 105 177 18 11 5α-Tetrahydrocorticosterone 193 130 261 489 0 0 0 90 0 12 3α,5β-Tetrahydroaldosterone 30 (12–64) n.m. n.m. n.m. n.m. 13 28 293 25 13 Tetrahydrodeoxycorticosterone 13 (5–36) n.m. n.m. n.m. n.m. 93 343 216 37 Glucocorticoid precursor metabolites 14 Pregnanediol 157 (32–336) 3249 1857 1474 1171 455 646 4832 199 15 3α,5α-17-Hydroxy-pregnanolone 14 (6–89) n.m. n.m. n.m. n.m. 13 18 809 8 16 17-Hydroxypregnanolone 133 (41–537) 7163 1940 1589 1538 817 998 39,306 551 17 Pregnanetriol 576 9562 5701 4295 4366 2128 2677 28,349 1495 18 Pregnanetriolone 13 (5–58) 20 2 0 3 5 0 1822 10 19 Tetrahydro-11-deoxycortisol 61 (21–159) 594 525 690 492 314 911 116 322 Glucocorticoid metabolites 20 Cortisol 57 (22–224) 252 735 497 495 399 813 414 201 21 6β-Hydroxy-cortisol 114 (63–504) n.m. n.m. n.m. n.m. 7657 23 193 393 3578 22 Tetrahydrocortisol 1694 4260 9578 7936 5087 2115 3001 2779 1391 23 5α-Tetrahydrocortisol 1408 477 344 161 114 37 66 702 40 24 α-Cortol 319 1665 2566 1880 1256 524 831 597 286 25 β-Cortol 513 957 378 306 207 108 153 467 60 26 11β-Hydroxy-etiocholanolone 315 (23–899) 257 147 91 95 37 50 1092 89 27 Cortisone 93 (39–348) 198 400 389 286 309 480 671 102 28 Tetrahydrocortisone 3333 3978 2391 2113 1564 763 1124 5597 807 29 α-Cortolone 1228 3892 2661 2222 1166 410 539 1623 479 30 β-Cortolone 696 1110 300 303 216 108 213 1130 42 31 11-Oxo-etiocholanolone 464 (74–997) 1059 734 736 868 844 1196 3144 267 Total glucocorticoid metabolite excretion 9665 16,789 19,353 15,807 10,391 4773 7220 13,980 3408 Steroid ratios indicative of 5α-reductase Androsterone/etiochaolanolone 1.13 (0.05–3.00) 0.79 0.46 0.31 0.44 0.45 0.53 1.23 0.22 5α-Tetrahydrocortisol/tetrahydrocortisol 0.92 (0.05–2.27) 0.11 0.04 0.02 0.02 0.02 0.02 0.25 0.03 n.m., not measured.	4.253906	0.808594	sec[2]/sec[0]/p[0]	en	0.999998	29330226	https://doi.org/10.1530/EJE-17-0542	[ "mitotane", "steroid", "metabolites", "excretion", "androgen", "tumor", "hydroxy", "baseline", "glucocorticoid", "tetrahydrocortisol" ]	[ { "code": "6C4H.Y", "title": "Other specified disorders due to use of non-psychoactive substances" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "6C4H.1Z", "title": "Harmful pattern of use of non-psychoactive substances, unspecified" }, { "code": "MF99", "title": "Elevated urine levels of drugs, medicaments and biological substances" }, { "code": "2F32.Y", "title": "Other specified benign neoplasm of ovary" }, { "code": "5C6Z", "title": "Disorders of metabolite absorption or transport, unspecified" }, { "code": "5C6Y", "title": "Other specified disorders of metabolite absorption or transport" }, { "code": "NE60", "title": "Harmful effects of drugs, medicaments or biological substances, not elsewhere classified" }, { "code": "PB28", "title": "Unintentional exposure to or harmful effects of other or unspecified drug, medicament or biological substance" }, { "code": "PC98&XE13E", "title": "Intentional self-harm by exposure to or harmful effects of antineoplastic antimetabolites" } ]	=== ICD-11 CODES FOUND === [6C4H.Y] Other specified disorders due to use of non-psychoactive substances Also known as: Other specified disorders due to use of non-psychoactive substances \| Steroid dependence [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified Also known as: Harmful pattern of use of non-psychoactive substances, unspecified \| Harmful pattern of use of non-psychoactive substances \| harmful use of nonprescribed drugs, non-dependence producing \| Abuse of antacids \| Abuse of herbal or folk remedies [MF99] Elevated urine levels of drugs, medicaments and biological substances Definition: Elevated urine levels of drugs, medicaments and biological substances mean that the levels of drugs, medicaments, and biological substances have elevated on the urine examination. Also known as: Elevated urine levels of drugs, medicaments and biological substances \| Elevated urine levels of 17-ketosteroids \| Elevated urine levels of catecholamines \| Elevated urine levels of indoleacetic acid \| Elevated urine levels of steroids [2F32.Y] Other specified benign neoplasm of ovary Also known as: Other specified benign neoplasm of ovary \| Serous or mucinous ovarian cystadenoma of childhood \| Benign androblastoma of ovary \| tubular androblastoma of unspecified site, female \| Adenofibroma of ovary [5C6Z] Disorders of metabolite absorption or transport, unspecified Also known as: Disorders of metabolite absorption or transport, unspecified [5C6Y] Other specified disorders of metabolite absorption or transport Also known as: Other specified disorders of metabolite absorption or transport [NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified Also known as: Harmful effects of drugs, medicaments or biological substances, not elsewhere classified \| drugs, medicaments or biological substances, toxicity not elsewhere classified \| adverse drug effects \| drug reaction NOS \| drug allergy NOS Excludes: Alcohol intoxication \| pathological drug intoxication \| hypersensitivity reaction to correctly administered drug [PB28] Unintentional exposure to or harmful effects of other or unspecified drug, medicament or biological substance Also known as: Unintentional exposure to or harmful effects of other or unspecified drug, medicament or biological substance \| accidental overdose of other or unspecified drug, medicament or biological substance \| accidental poisoning by other or unspecified drug, medicament or biological substance \| other or unspecified drug, medicament or biological substance taken in error \| accidental drug overdose === GRAPH WALKS === --- Walk 1 --- [6C4H.Y] Other specified disorders due to use of non-psychoactive substances --PARENT--> [6C4H] Disorders due to use of non-psychoactive substances Def: Disorders due to use of non-psychoactive substances are characterised by the pattern and consequences of non-medical use of non-psychoactive substances. Non-psychoactive substances include laxatives, ... --CHILD--> [6C4H.1] Harmful pattern of use of non-psychoactive substances Def: A pattern of use of non-psychoactive substances that has caused clinically significant harm to a person’s physical or mental health. The pattern of use is evident over a period of at least 12 months i... --- Walk 2 --- [6C4H.Y] Other specified disorders due to use of non-psychoactive substances --PARENT--> [6C4H] Disorders due to use of non-psychoactive substances Def: Disorders due to use of non-psychoactive substances are characterised by the pattern and consequences of non-medical use of non-psychoactive substances. Non-psychoactive substances include laxatives, ... --CHILD--> [6C4H.Y] Other specified disorders due to use of non-psychoactive substances --- Walk 3 --- [MA12.4] Finding of steroid agent in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.1] Finding of cocaine in blood --- Walk 4 --- [MA12.4] Finding of steroid agent in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.2] Finding of hallucinogen in blood --- Walk 5 --- [6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified --PARENT--> [6C4H.1] Harmful pattern of use of non-psychoactive substances Def: A pattern of use of non-psychoactive substances that has caused clinically significant harm to a person’s physical or mental health. The pattern of use is evident over a period of at least 12 months i... --PARENT--> [6C4H] Disorders due to use of non-psychoactive substances Def: Disorders due to use of non-psychoactive substances are characterised by the pattern and consequences of non-medical use of non-psychoactive substances. Non-psychoactive substances include laxatives, ... --- Walk 6 --- [6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified --PARENT--> [6C4H.1] Harmful pattern of use of non-psychoactive substances Def: A pattern of use of non-psychoactive substances that has caused clinically significant harm to a person’s physical or mental health. The pattern of use is evident over a period of at least 12 months i... --EXCLUDES--> [?] Episode of harmful use of non-psychoactive substances Def: An episode of use of a non-psychoactive substance that has caused damage to a person’s physical or mental health. Harm to health of the individual occurs due to direct or secondary toxic effects on bo...	[ "[6C4H.Y] Other specified disorders due to use of non-psychoactive substances\n --PARENT--> [6C4H] Disorders due to use of non-psychoactive substances\n Def: Disorders due to use of non-psychoactive substances are characterised by the pattern and consequences of non-medical use of non-psychoactive substances. Non-psychoactive substances include laxatives, ...\n --CHILD--> [6C4H.1] Harmful pattern of use of non-psychoactive substances\n Def: A pattern of use of non-psychoactive substances that has caused clinically significant harm to a person’s physical or mental health. The pattern of use is evident over a period of at least 12 months i...", "[6C4H.Y] Other specified disorders due to use of non-psychoactive substances\n --PARENT--> [6C4H] Disorders due to use of non-psychoactive substances\n Def: Disorders due to use of non-psychoactive substances are characterised by the pattern and consequences of non-medical use of non-psychoactive substances. Non-psychoactive substances include laxatives, ...\n --CHILD--> [6C4H.Y] Other specified disorders due to use of non-psychoactive substances", "[MA12.4] Finding of steroid agent in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.1] Finding of cocaine in blood", "[MA12.4] Finding of steroid agent in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.2] Finding of hallucinogen in blood", "[6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified\n --PARENT--> [6C4H.1] Harmful pattern of use of non-psychoactive substances\n Def: A pattern of use of non-psychoactive substances that has caused clinically significant harm to a person’s physical or mental health. The pattern of use is evident over a period of at least 12 months i...\n --PARENT--> [6C4H] Disorders due to use of non-psychoactive substances\n Def: Disorders due to use of non-psychoactive substances are characterised by the pattern and consequences of non-medical use of non-psychoactive substances. Non-psychoactive substances include laxatives, ...", "[6C4H.1Z] Harmful pattern of use of non-psychoactive substances, unspecified\n --PARENT--> [6C4H.1] Harmful pattern of use of non-psychoactive substances\n Def: A pattern of use of non-psychoactive substances that has caused clinically significant harm to a person’s physical or mental health. The pattern of use is evident over a period of at least 12 months i...\n --EXCLUDES--> [?] Episode of harmful use of non-psychoactive substances\n Def: An episode of use of a non-psychoactive substance that has caused damage to a person’s physical or mental health. Harm to health of the individual occurs due to direct or secondary toxic effects on bo..." ]	6C4H.Y	Other specified disorders due to use of non-psychoactive substances	[ { "from_icd11": "MA12.4", "icd10_code": "R786", "icd10_title": "Finding of steroid agent in blood" }, { "from_icd11": "6C4H.1Z", "icd10_code": "F558", "icd10_title": "Abuse of other non-psychoactive substances" }, { "from_icd11": "6C4H.1Z", "icd10_code": "F552", "icd10_title": "Abuse of laxatives" }, { "from_icd11": "6C4H.1Z", "icd10_code": "F553", "icd10_title": "Abuse of steroids or hormones" }, { "from_icd11": "6C4H.1Z", "icd10_code": "F551", "icd10_title": "Abuse of herbal or folk remedies" }, { "from_icd11": "6C4H.1Z", "icd10_code": "F55", "icd10_title": "Abuse of non-psychoactive substances" }, { "from_icd11": "MF99", "icd10_code": "R825", "icd10_title": "Elevated urine levels of drugs, medicaments and biological substances" }, { "from_icd11": "NE60", "icd10_code": "T50A95A", "icd10_title": "Adverse effect of other bacterial vaccines, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50Z15A", "icd10_title": "Adverse effect of immunoglobulin, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50Z95A", "icd10_title": "Adverse effect of other vaccines and biological substances, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50A95S", "icd10_title": "Adverse effect of other bacterial vaccines, sequela" }, { "from_icd11": "NE60", "icd10_code": "T50B95A", "icd10_title": "Adverse effect of other viral vaccines, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50A25A", "icd10_title": "Adverse effect of mixed bacterial vaccines without a pertussis component, initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T50A91A", "icd10_title": "Poisoning by other bacterial vaccines, accidental (unintentional), initial encounter" }, { "from_icd11": "NE60", "icd10_code": "T498X5A", "icd10_title": "Adverse effect of other topical agents, initial encounter" } ]	R786	Finding of steroid agent in blood
We here report the case of a 21-year-old Somali woman, who was delivered by emergency caesarean section at 35 weeks of gestational age with acute dyspnea, placental abruption and gross edema due to severe PE/HELLP syndrome. This was her first pregnancy, which had been uneventful up to the 34th gestational week. Her soluble fms-like tyrosine kinase-1/placental growth factor ratio 2 days prior was 211.4 . After surgery, the patient was immediately transferred to Intensive Care Unit because of lung edema. The laboratory analysis revealed anemia of 7.4 g/dL, thrombocytopenia of 50 G/L, a negative coombs test, increased serum lactate dehydrogenase of 690 U/L, increased bilirubin of 2.2 mg/dL, elevated aspartate transaminase of 150 U/L, elevated alanine transaminase of 140, creatinine of 1.19 mg/dL, and no detectable haptoglobin levels (< 0.09 g/L). The peripheral blood smear showed manifold schistocytes (2.8%) and the activated prothrombin time was 38.2 s (Additional file 1 : Table S1). The PLASMIC score was high indicating a high pretest probability for TTP (> 90%) . The patient displayed elevated systolic blood pressure between 160 and 200 mmHg despite of intensive blood pressure lowering medication including urapidil, nifedipin, furosemide, and dihydralazine. As concern for the diagnosis TTP was strong, we immediately initiated plasma exchange therapy (PEX) and glucocorticoid medication, and proceeded with further diagnostic evaluation over the next days . While undergoing PEX, the renal retention parameters slowly increased over the next 4 days, reaching a serum creatinine level of 2.09 mg/dL and an estimated glomerular filtration rate of 33 mL/min/1. 73m 2 . In parallel, fibrinogen levels decreased to a nadir of 103 mg/dL, and the thrombocyte count was still as low as 35 G/L on the 4th postoperative day. This decrease was associated with a peak of D-dimer level (26.27 mg/L) on the 6th postoperative day. The lack of early response to PEX prompted us to discuss the need for anti-complement therapy with eculizumab and to seek for other causes of the patient’s symptoms. There was no retained placental rest after delivery. In the meantime, ADAMTS13 activity had been measured and was found to be only slightly decreased to 39–63%, thus excluding the diagnosis of TTP . Shiga-toxin, malaria parasites, and HIV antigen/antibodies were not detectable, and the hepatitis B and C serology tests were negative. The screening test for antibodies to extractable nuclear antigens (ENA), antinuclear, antiphospholipid and anticardiolipin antibodies, as well as serum C3c (0.917 g/L) and serum C4 levels (0.113 g/L) were within normal ranges. Urinary analyses revealed an albuminuria of 4.2 g/g and 40% acanthocytes, respectively. Since the patient presented with anasarca, somnolence, partial respiratory insufficiency due to lung edema and pleural effusions as well as still poorly controlled hypertension, we initiated a continuous renal replacement therapy with ultrafiltration on the 4th postoperative day, reducing the body weight of the patient from 70 kg to 49.5 kg in four days. Furthermore, PEX was daily continued. Along with the negative fluid balance of 20.5 L, the patient drastically improved both clinically and with the laboratory parameters. In parallel, kidney biopsy was performed on the 6th postoperative day, which revealed a residual thrombotic microangiopathy and signs of malignant hypertension such as doubling of the basal membrane as well as mild tubular necrosis. Immune complex nephritis, e.g. lupus nephritis, was excluded . Therefore, we stopped PEX on the 6th day postpartum, after having reached a thrombocyte threshold of >100G/L. Dose and number of antihypertensive medication were drastically decreased. In a serum sample drawn prior to PEX, we found no evidence of complement activation using an in-vitro assay for complement deposition on non-activated endothelial cells . C3c and C5b-9 complement deposition assays were performed as described previously by Noris et al. Moreover, there were neither anti-complement factor H antibodies as determined by ELISA, nor mutations of atypical hemolytic uremic syndrome-related genes ADAMTS13, C3, CFB, CFB, CFH, CFHR1, CFHR2, CFHR3, CFHR4, CFHR5, CFI, DGKE, MCP/CD46, MMACHC, and THBD as detected by next-generation sequencing . The patient completely recovered without further need for renal replacement therapy and/or PEX. Thrombocyte count increased to 240 G/L, and creatinine serum levels decreased to 0.99 mg/dL on the 12th day after delivery. The patient was discharged from Intensive Care Unit after 10 days, and dismissed from Hospital on the 16th day postpartum without any chronic impairment of glomerular filtration rate. Twenty-eight days after delivery, the glomerular filtration rate was 127.7 mL/min/1. 73m 2 , and serum creatinine was 0.64 mg/dL. Moreover, urinary albumin/creatinine ratio recovered from 4.2 g/g to 0.55 g/g within 4 weeks. Fig. 1 Time course showing laboratory parameters and therapeutic interventions: Platelet count (PLT, closed circles), serum lactate dehydrogenase (LDH, open triangles), hemoglobin (Hb, open squares) and serum creatinine (closed diamonds) are given on the ordinate as a function of time [days]. Day 0 is defined as the day of delivery (dotted black line). Red bars indicate infusion of erythrocyte concentrates (EC), blue arrows show treatment with plasma exchange (PEX), green arrows renal replacement therapy (RRT), and the orange arrows stands for kidney biopsy (KB), respectively Fig. 2 Representative renal biopsy pictures: a Periodic acid-Schiff reaction-(PAS) stained section showing glomerulopathy with thickened glomerular basement membranes, roundish capillary lumina and thrombotic obliteration of a capillary lumen. b Silver stained section illustrating segmental double contours of the capillary loops. Magnification × 400 Fig. 3 Complement deposition on endothelial cells: Endothelial cells were incubated with a serum from the index patient and b control serum from a patient with acute complement-mediated TMA due to complement factor H mutation. Serum of the index patient caused no deposition of C3c (FITC) and C5b-9 (Rhodamine) on non-activated endothelial cells. Magnification × 40	4.082031	0.975586	sec[1]/p[0]	en	0.999996	30871486	https://doi.org/10.1186/s12882-019-1286-1	[ "serum", "complement", "creatinine", "renal", "glomerular", "postoperative", "delivery", "antibodies", "deposition", "endothelial" ]	[ { "code": "NE80.3", "title": "Other serum reactions" }, { "code": "5D0Y", "title": "Other specified metabolic disorders" }, { "code": "5B91.0", "title": "Hypercalcaemia" }, { "code": "4A84.Y", "title": "Other specified anaphylaxis" }, { "code": "5C50.F2", "title": "Homocarnosinosis" }, { "code": "4A00.1Z", "title": "Defects in the complement system, unspecified" }, { "code": "4A00.1Y", "title": "Other specified defects in the complement system" }, { "code": "4A00.11", "title": "Immunodeficiency with a late component of complement deficiency" }, { "code": "4A00.10", "title": "Immunodeficiency with an early component of complement deficiency" }, { "code": "4A00.13", "title": "Immunodeficiency with factor D anomaly" } ]	=== ICD-11 CODES FOUND === [NE80.3] Other serum reactions Also known as: Other serum reactions \| Allergic reaction to serum \| serum allergy \| Complications of vaccination, protein sickness \| Protein sickness Excludes: serum hepatitis [5D0Y] Other specified metabolic disorders Also known as: Other specified metabolic disorders \| Disorders of plasma-protein metabolism, not elsewhere classified \| abnormal protein transport \| dysproteinaemia \| Absence of albumin in blood [5B91.0] Hypercalcaemia Definition: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused by dehydration secondary to urinary losses of calcium, water and other electrolytes, and to an increase in membrane potential caused by the elevation in extracellular fluid ionized calcium concentration. Patients with moderate to severe hypercalcaemia often complain of nausea and vomiting, symptoms Also known as: Hypercalcaemia \| Calcium excess \| elevated serum calcium \| hypercalcaemic crisis \| hypercalcaemic syndrome [4A84.Y] Other specified anaphylaxis Also known as: Other specified anaphylaxis \| Latex-induced anaphylaxis \| Anaphylaxis due to latex \| Latex anaphylaxis \| Anaphylactic shock due to serum [5C50.F2] Homocarnosinosis Definition: Homocarnosinosis is a metabolic defect characterised by progressive spastic diplegia, intellectual deficit and retinitis pigmentosa. This extremely rare disorder has been reported in only one family, namely a woman and three of her children. The latter showed but their mother was symptom free. It is therefore uncertain whether there is a relationship between the biochemical defect and the clinical symptoms. Inheritance in the reported family seems to be autosomal dominant. Also known as: Homocarnosinosis \| Homocarnosinase deficiency \| Serum carnosinase deficiency [4A00.1Z] Defects in the complement system, unspecified Also known as: Defects in the complement system, unspecified \| Defects in the complement system \| Immunodeficiency with a complement cascade protein anomaly \| complement deficiency disease \| complement system defect [4A00.1Y] Other specified defects in the complement system Also known as: Other specified defects in the complement system \| Complement component C3 deficiency \| Recurrent Neisseria infections due to factor D deficiency \| Complement component C4b-binding protein deficiency \| Immunodeficiency with CD46 deficiency [4A00.11] Immunodeficiency with a late component of complement deficiency Also known as: Immunodeficiency with a late component of complement deficiency \| Deficiency of complement terminal pathway \| Complement component C5 deficiency \| Complement component C6 deficiency \| Complement component C7 deficiency [4A00.10] Immunodeficiency with an early component of complement deficiency Also known as: Immunodeficiency with an early component of complement deficiency \| Deficiency of complement initial pathway \| Complement component C1q deficiency \| Complement component C1r/C1s deficiency \| Complement component C2 deficiency [4A00.13] Immunodeficiency with factor D anomaly Definition: Factor D deficiency is an autosomal recessive immunologic disorder characterised by increased susceptibility to bacterial infections, particularly Neisseria infections, due to a defect in the alternative complement pathway. Also known as: Immunodeficiency with factor D anomaly \| CFDD - [Complement factor D deficiency] === GRAPH WALKS === --- Walk 1 --- [NE80.3] Other serum reactions --PARENT--> [NE80] Injury or harm arising following infusion, transfusion or therapeutic injection, not elsewhere classified --EXCLUDES--> [?] Endophthalmitis --- Walk 2 --- [NE80.3] Other serum reactions --EXCLUDES--> [?] Acute hepatitis B without Hepatitis D virus co-infection Def: Acute liver injury related with hepatitis B virus (HBV). Acute hepatitis B is suspected based on positive HBsAg and high-titer IgM anti-HBc. However, other causes of acute viral hepatitis may not be f... --CHILD--> [?] Transfusion hepatitis --- Walk 3 --- [5D0Y] Other specified metabolic disorders --PARENT--> [?] Other metabolic disorders --EXCLUDES--> [?] Langerhans cell histiocytosis Def: A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognised: eosinophilic granuloma, Letterer-Siwe di... --- Walk 4 --- [5D0Y] Other specified metabolic disorders --PARENT--> [?] Other metabolic disorders --EXCLUDES--> [?] Langerhans cell histiocytosis Def: A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognised: eosinophilic granuloma, Letterer-Siwe di... --- Walk 5 --- [5B91.0] Hypercalcaemia Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ... --RELATED_TO--> [?] Myopathy due to hypercalcaemia --PARENT--> [?] Neurological disorders due to an excess of micro or macro nutrients --- Walk 6 --- [5B91.0] Hypercalcaemia Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ... --RELATED_TO--> [?] Myopathy due to hypercalcaemia --PARENT--> [?] Hypercalcaemia Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ...	[ "[NE80.3] Other serum reactions\n --PARENT--> [NE80] Injury or harm arising following infusion, transfusion or therapeutic injection, not elsewhere classified\n --EXCLUDES--> [?] Endophthalmitis", "[NE80.3] Other serum reactions\n --EXCLUDES--> [?] Acute hepatitis B without Hepatitis D virus co-infection\n Def: Acute liver injury related with hepatitis B virus (HBV). Acute hepatitis B is suspected based on positive HBsAg and high-titer IgM anti-HBc. However, other causes of acute viral hepatitis may not be f...\n --CHILD--> [?] Transfusion hepatitis", "[5D0Y] Other specified metabolic disorders\n --PARENT--> [?] Other metabolic disorders\n --EXCLUDES--> [?] Langerhans cell histiocytosis\n Def: A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognised: eosinophilic granuloma, Letterer-Siwe di...", "[5D0Y] Other specified metabolic disorders\n --PARENT--> [?] Other metabolic disorders\n --EXCLUDES--> [?] Langerhans cell histiocytosis\n Def: A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognised: eosinophilic granuloma, Letterer-Siwe di...", "[5B91.0] Hypercalcaemia\n Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ...\n --RELATED_TO--> [?] Myopathy due to hypercalcaemia\n --PARENT--> [?] Neurological disorders due to an excess of micro or macro nutrients", "[5B91.0] Hypercalcaemia\n Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ...\n --RELATED_TO--> [?] Myopathy due to hypercalcaemia\n --PARENT--> [?] Hypercalcaemia\n Def: Hypercalcaemia is a condition caused by increased calcium levels. The higher the calcium levels and the faster its level rises, the more severe will be the symptoms. When present, symptoms are caused ..." ]	NE80.3	Other serum reactions	[ { "from_icd11": "NE80.3", "icd10_code": "T880XXA", "icd10_title": "Infection following immunization, initial encounter" }, { "from_icd11": "NE80.3", "icd10_code": "T8061XA", "icd10_title": "Other serum reaction due to administration of blood and blood products, initial encounter" }, { "from_icd11": "NE80.3", "icd10_code": "T8069XA", "icd10_title": "Other serum reaction due to other serum, initial encounter" }, { "from_icd11": "NE80.3", "icd10_code": "T8062XA", "icd10_title": "Other serum reaction due to vaccination, initial encounter" }, { "from_icd11": "NE80.3", "icd10_code": "T806", "icd10_title": "Other serum reactions" }, { "from_icd11": "NE80.3", "icd10_code": "T880", "icd10_title": "Infection following immunization" }, { "from_icd11": "5C50.F2", "icd10_code": "E7281", "icd10_title": "Disorders of gamma aminobutyric acid metabolism" }, { "from_icd11": "5C50.F2", "icd10_code": "E728", "icd10_title": "Other specified disorders of amino-acid metabolism" }, { "from_icd11": "4A00.1Z", "icd10_code": "D841", "icd10_title": "Defects in the complement system" } ]	T880XXA	Infection following immunization, initial encounter
More detailed examples of DRPs identified among these patients are listed in Table 3 . Table 3 Examples of DRP Type of DRP Comment Adverse drug reaction A 78-year-old man with Alzheimer’s disease, hypertension and hypokalemia was admitted to the hospital because of hypertension (205/115 mmHg). The doctor initially suspected that the patient’s symptom was an ADR related to galantamine, and so discontinued the treatment. However, hypertension secondary to primary aldosteronism was then diagnosed, and ten days later galantamine was restarted at the same dosage as at admission (i.e., 24 mg daily). The patient was ready to be discharged, but got nauseous and vomited and had to stay on the ward. The clinical pharmacist suspected an ADR and suggested to decrease the dose of galantamine, which was done. The symptoms resolved and the patient could be discharged. Dosage too high A 71-year-old man was admitted to the hospital because of a history of falls. His chronic medical problems included schizophrenia, diabetes mellitus type II, mental retardation and a suspected dementia. He had a catheter because of urinary retention. A UTI was diagnosed. His schizophrenia was treated with zuclopenthixol decanoate intramuscular injections every fourth week, and for side effects with trihexyphenidyl 20 mg daily. The dosage of zuclopenthixole had been lowered by more than 75% over recent years whilst the dosage of trihexyphenidyl was unchanged. The clinical pharmacist questioned the dose of the anticholinergic drug that might have been a contributory factor to suspected dementia, history of falls and urinary retention. The dose of trihexyphenidyl was gradually lowered and finally discontinued, and the injection switched to risperidone tablets. Dosage too low An 89-year-old man with cognitive impairment was admitted to the hospital because of urosepsis. His medical history included stroke and abdominal pain, which was treated with sustained-release morphine 30 mg twice daily, and sodium picosulfate PRN for prevention of opioid-associated constipation. Examination on the ward revealed severe constipation, which was treated with methylnaltrexone. The patient’s MMSE score several weeks before hospital admission was 13/30. Because of his low MMSE score and the fact that he was living at home on his own, it was unclear whether the patient understood the importance/need of taking the laxative in time. The clinical pharmacist suggested regular dosing of sodium picosulfate, a recommendation that was followed by the physician. Osmotic laxatives were also prescribed. Ineffective/Inappropriate drug A 90-year-old woman with cognitive impairment was admitted to the hospital because of excessive daytime sleepiness. A medication review performed by the clinical pharmacist revealed that medication with propiomazine 25 mg at bedtime was started by primary care 8 days prior to admission to the hospital. Propiomazine can cause daytime sleepiness and is classified as an inappropriate drug by the quality indicator developed by the Swedish National Board of Health and Welfare. Propiomazine was discontinued. Interaction An 86-year-old man with Alzheimer’s disease was admitted to the hospital because of bursitis. Two months before admission, he was prescribed fluconazole 50 mg daily as a seven-day treatment, but due to a transcription error, it was added to the medication list as an ongoing prescription. The patient also had an ongoing treatment with citalopram. On the ward, he got more and more agitated, and hallucinated. Haloperidol was prescribed. The patient’s symptoms might have been a result of increased concentrations of citalopram due to an interaction between citalopram and fluconazole. The clinical pharmacist recommended discontinuation of fluconazole and haloperidol. Fluconazole was discontinued and haloperidol was prescribed PRN, and since the hallucinations disappeared, haloperidol was no longer needed. Needs additional drug therapy An 87-year-old woman was admitted to the hospital because of deteriorating heart failure. She had a medical history of atrial fibrillation, angina pectoris, heart failure, stroke and vascular dementia, with an MMSE score of 14/30. She was agitated and aggressive to the staff and it was assumed that she suffered from pain, which was treated with oxycodone PRN. A medication review performed by the clinical pharmacist revealed that gabapentin was discontinued for unclear reasons just a week prior to admission to the hospital. The indication for gabapentin use was not only neuropathic pain but also post-stroke epilepsy, of which the physician was unaware. Gabapentin was the only antiepileptic drug treatment the patient had been prescribed. Gabapentin was reinitiated. Noncompliance One patient admitted to the ward for dyspnea had been prescribed a multidrug treatment for COPD (stage III) with dry powder inhalers. According to the medical record, the patient required full support to cope with activities of daily living and could not follow instructions. It is therefore possible that the patient was unable to use the inhaler devices properly prior to readmission, leading to ineffective drug treatment. The pharmacist recommended the use of a pressurized metered-dose inhaler together with a spacer instead. Unnecessary drug therapy An 89-year-old woman with vascular dementia, diabetes mellitus, previous stroke and angina pectoris was admitted to the hospital because of headache and abnormal motor function; meningitis was diagnosed. The patient was also nauseous and had been so for a long time. In 2005, she had been prescribed haloperidol for the treatment of her nausea, and she was still treated with this at the time of admission . Her diabetes was treated with metformin, which could be the cause of her nausea. Because of decreased renal function and an HbA1c fluctuating between 46–58 mmol/mol during the last two years, the clinical pharmacist suggested that both haloperidol and metformin should be discontinued (with monitoring of HbA1c later on), which was done. Abbreviations : ADR adverse drug reaction, COPD chronic obstructive pulmonary disease, DRP drug-related problem, MMSE Mini Mental State Examination, PRN Pro Re Nata, UTI urinary tract infection	3.931641	0.941895	sec[2]/p[2]	en	0.999995	28655357	https://doi.org/10.1186/s40360-017-0157-2	[ "drug", "because", "that", "pharmacist", "which", "prescribed", "discontinued", "treated", "haloperidol", "daily" ]	[ { "code": "QE11.Z", "title": "Hazardous drug use, unspecified" }, { "code": "6C4G.2Z", "title": "Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified" }, { "code": "NE60", "title": "Harmful effects of drugs, medicaments or biological substances, not elsewhere classified" }, { "code": "4A85.0Z", "title": "Drug hypersensitivity of unspecified type" }, { "code": "6C4G.3", "title": "Intoxication due to unknown or unspecified psychoactive substance" }, { "code": "QC10", "title": "Procedure not carried out because of contraindication" }, { "code": "QB12.0", "title": "Organ transplant candidate" }, { "code": "QC04.5", "title": "Immunization not carried out because of patient refusal" }, { "code": "QC04.Z", "title": "Immunization not carried out for unspecified reason" }, { "code": "QC04.6", "title": "Immunization not carried out because of caregiver refusal" } ]	=== ICD-11 CODES FOUND === [QE11.Z] Hazardous drug use, unspecified Also known as: Hazardous drug use, unspecified \| Hazardous drug use \| chronic drug use NOS \| chronic IV substance use \| drug use nos [6C4G.2Z] Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified Also known as: Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified \| Unknown or unspecified psychoactive substance dependence \| Drug dependence NOS [NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified Also known as: Harmful effects of drugs, medicaments or biological substances, not elsewhere classified \| drugs, medicaments or biological substances, toxicity not elsewhere classified \| adverse drug effects \| drug reaction NOS \| drug allergy NOS Excludes: Alcohol intoxication \| pathological drug intoxication \| hypersensitivity reaction to correctly administered drug [4A85.0Z] Drug hypersensitivity of unspecified type Also known as: Drug hypersensitivity of unspecified type \| Drug or pharmacological agents hypersensitivity \| medicinal hypersensitivity \| drug sensitivity NOS [6C4G.3] Intoxication due to unknown or unspecified psychoactive substance Definition: Intoxication due to unknown or unspecified psychoactive substance is a transient condition that develops during or shortly after the administration of an unknown or unspecified psychoactive substance that is characterised by disturbances in level of consciousness, cognition, perception, affect or behaviour, or other psychophysiological functions and responses. This diagnosis should be made only when there is strong evidence that an unidentified substance has been taken and the features cannot be Also known as: Intoxication due to unknown or unspecified psychoactive substance \| psychoactive substance abuse \| trance and possession disorders in psychoactive substance intoxication \| drug intoxication NOS [QC10] Procedure not carried out because of contraindication Also known as: Procedure not carried out because of contraindication \| intervention not carried out because of contraindication \| Procedure cancelled due to contraindication [QB12.0] Organ transplant candidate Also known as: Organ transplant candidate \| patient waiting for organ availability \| health services provided because of need for organ transplant \| organ transplant candidate awaiting organ availability \| person on organ transplant waiting list [QC04.5] Immunization not carried out because of patient refusal Also known as: Immunization not carried out because of patient refusal [QC04.Z] Immunization not carried out for unspecified reason Also known as: Immunization not carried out for unspecified reason \| Immunization not carried out \| vaccination not done \| Immunization not carried out because of contraindication, not otherwise specified [QC04.6] Immunization not carried out because of caregiver refusal Also known as: Immunization not carried out because of caregiver refusal === GRAPH WALKS === --- Walk 1 --- [QE11.Z] Hazardous drug use, unspecified --PARENT--> [QE11] Hazardous drug use Def: A pattern of use of psychoactive substance(s) other than nicotine or alcohol that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent... --CHILD--> [QE11.0] Hazardous use of opioids Def: A pattern of opioid use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health profe... --- Walk 2 --- [QE11.Z] Hazardous drug use, unspecified --PARENT--> [QE11] Hazardous drug use Def: A pattern of use of psychoactive substance(s) other than nicotine or alcohol that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent... --EXCLUDES--> [?] Disorders due to substance use Def: Disorders due to substance use include disorders that result from a single occasion or repeated use of substances that have psychoactive properties, including certain medications. Disorders related to... --- Walk 3 --- [6C4G.2Z] Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified --PARENT--> [6C4G.2] Unknown or unspecified psychoactive substance dependence Def: Unknown or unspecified psychoactive substance dependence is a disorder of regulation of use of an unknown or unspecified substance arising from repeated or continuous use of the substance. The charact... --EXCLUDES--> [?] Harmful pattern of use of unknown or unspecified psychoactive substance Def: A pattern of use of an unknown or unspecified psychoactive substance that has caused damage to a person’s physical or mental health or has resulted in behaviour leading to harm to the health of others... --- Walk 4 --- [6C4G.2Z] Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified --PARENT--> [6C4G.2] Unknown or unspecified psychoactive substance dependence Def: Unknown or unspecified psychoactive substance dependence is a disorder of regulation of use of an unknown or unspecified substance arising from repeated or continuous use of the substance. The charact... --CHILD--> [6C4G.20] Unknown or unspecified psychoactive substance dependence, current use Def: Current dependence on an unknown or unspecified psychoactive substance, with use of the substance within the past month.... --- Walk 5 --- [NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified --PARENT--> [?] Harmful effects of substances --CHILD--> [NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified --- Walk 6 --- [NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified --EXCLUDES--> [?] Allergic or hypersensitivity conditions Def: Allergy is a hypersensitivity reaction initiated by proven immunologic mechanisms. Hypersensitivity is defined as conditions clinically resembling allergy that cause objectively reproducible symptoms... --CHILD--> [?] Allergic or hypersensitivity disorders involving the eye Def: Allergic or hypersensitivity disorders involving the eye includes several clinically different conditions that can be considered as hypersensitivity disorders of the ocular surface. The classification...	[ "[QE11.Z] Hazardous drug use, unspecified\n --PARENT--> [QE11] Hazardous drug use\n Def: A pattern of use of psychoactive substance(s) other than nicotine or alcohol that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent...\n --CHILD--> [QE11.0] Hazardous use of opioids\n Def: A pattern of opioid use that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent that warrants attention and advice from health profe...", "[QE11.Z] Hazardous drug use, unspecified\n --PARENT--> [QE11] Hazardous drug use\n Def: A pattern of use of psychoactive substance(s) other than nicotine or alcohol that appreciably increases the risk of harmful physical or mental health consequences to the user or to others to an extent...\n --EXCLUDES--> [?] Disorders due to substance use\n Def: Disorders due to substance use include disorders that result from a single occasion or repeated use of substances that have psychoactive properties, including certain medications. Disorders related to...", "[6C4G.2Z] Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified\n --PARENT--> [6C4G.2] Unknown or unspecified psychoactive substance dependence\n Def: Unknown or unspecified psychoactive substance dependence is a disorder of regulation of use of an unknown or unspecified substance arising from repeated or continuous use of the substance. The charact...\n --EXCLUDES--> [?] Harmful pattern of use of unknown or unspecified psychoactive substance\n Def: A pattern of use of an unknown or unspecified psychoactive substance that has caused damage to a person’s physical or mental health or has resulted in behaviour leading to harm to the health of others...", "[6C4G.2Z] Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified\n --PARENT--> [6C4G.2] Unknown or unspecified psychoactive substance dependence\n Def: Unknown or unspecified psychoactive substance dependence is a disorder of regulation of use of an unknown or unspecified substance arising from repeated or continuous use of the substance. The charact...\n --CHILD--> [6C4G.20] Unknown or unspecified psychoactive substance dependence, current use\n Def: Current dependence on an unknown or unspecified psychoactive substance, with use of the substance within the past month....", "[NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified\n --PARENT--> [?] Harmful effects of substances\n --CHILD--> [NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified", "[NE60] Harmful effects of drugs, medicaments or biological substances, not elsewhere classified\n --EXCLUDES--> [?] Allergic or hypersensitivity conditions\n Def: Allergy is a hypersensitivity reaction initiated by proven immunologic mechanisms.\n\nHypersensitivity is defined as conditions clinically resembling allergy that cause objectively reproducible symptoms...\n --CHILD--> [?] Allergic or hypersensitivity disorders involving the eye\n Def: Allergic or hypersensitivity disorders involving the eye includes several clinically different conditions that can be considered as hypersensitivity disorders of the ocular surface. The classification..." ]	QE11.Z	Hazardous drug use, unspecified	[ { "from_icd11": "QE11.Z", "icd10_code": "Z722", "icd10_title": "" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F1920", "icd10_title": "Other psychoactive substance dependence, uncomplicated" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F19239", "icd10_title": "Other psychoactive substance dependence with withdrawal, unspecified" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F1921", "icd10_title": "Other psychoactive substance dependence, in remission" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F19221", "icd10_title": "Other psychoactive substance dependence with intoxication delirium" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F1924", "icd10_title": "Other psychoactive substance dependence with psychoactive substance-induced mood disorder" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F19229", "icd10_title": "Other psychoactive substance dependence with intoxication, unspecified" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F19220", "icd10_title": "Other psychoactive substance dependence with intoxication, uncomplicated" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F19280", "icd10_title": "Other psychoactive substance dependence with psychoactive substance-induced anxiety disorder" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F19259", "icd10_title": "Other psychoactive substance dependence with psychoactive substance-induced psychotic disorder, unspecified" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F19231", "icd10_title": "Other psychoactive substance dependence with withdrawal delirium" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F19232", "icd10_title": "Other psychoactive substance dependence with withdrawal with perceptual disturbance" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F19222", "icd10_title": "Other psychoactive substance dependence with intoxication with perceptual disturbance" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F19281", "icd10_title": "Other psychoactive substance dependence with psychoactive substance-induced sexual dysfunction" }, { "from_icd11": "6C4G.2Z", "icd10_code": "F19288", "icd10_title": "Other psychoactive substance dependence with other psychoactive substance-induced disorder" } ]	Z722
Patient: Mr. Niu; gender: male; age: 70 years old; occupation: retired medical staff. The chief complaint was intermittent facial and bilateral lower extremity edema. The patient developed facial and bilateral lower limb edema without any known cause one year ago. Increased nocturia was reported, although there was no hematuria, no fever, no malaise, no skin rash, and no photosensitivity. The patient was found to be positive for proteinuria several times. The patient was administered “Bailing Capsules and Shenyankangfu Tablets” orally, but the edema varied between mild and (at times) severe. The outpatient examination results were as follows : urine protein quantification 5.54 g/24 h/2300 ml, blood glucose (BG) 7.5 mmol/L, glycosylated albumin 21.3%. The patient had a previous history of DM for 26 years with a maximum BG of 16 mmol/L. The patient was treated with Novolin 16 U bid and glargine insulin 12 U qn, together with oral administration of “Metformin”. Fasting BG (FBG) was within the range of 10–15 mmol/L with basically normal postprandial blood glucose (PBG). The patient had a 20-year history of hypertension with maximum blood pressure (BP) of 160/90mmHg. He was medicated with oral administration of Norvasc 5 mg QD and BP was maintained at around 140/80 mmHg. He had a 20-year history of coronary heart disease (CHD) with long-term oral administration of Atorvastatin. A pacemaker had been implanted for eight months due to bradycardia. Patient had undergone cataract surgery in the right eye six months earlier, and suffered from blurred vision in both eyes at time of examination. Personal history: reported no history of smoking and drinking. Marital history: patient married at 29 years of age; spouse had hypertension and DM. Patient had one son, who was healthy. Family history: patient’s father died of unknown cause; mother died of cerebral hemorrhage. Patient’s two brothers died of renal failure and esophageal cancer, respectively. Patient’s two sisters had DM and hypertension respectively before death. Results of physical examination at admission: T 36.5℃, P 66/min, R 18 times/min, BP 153/92 mmHg. The patient was conscious and communicated fluently. The bilateral breath sound was clear without dry and moist rales. The pacemaker rate was 66 times/min. The abdomen was flat, and the liver and spleen were impalpable. Mild pit edema of both lower extremities was observed. Ultrasonography results: normal size of both kidneys, sclerosis plaque formation in the bilateral carotid artery. Chest computed tomography (CT) results: multiple micronodules in both lungs with partial calcification, which were considered to be benign. Post cardiac surgery, aortic and coronary artery sclerosis. No significant changes compared to the examination results on 3 Mar 2021. Electrocardiography (ECG) results: dual-chamber pacing rhythm with normal electrical axis. Routine blood test results: white blood cell (WBC) 5.78×10 9 , red blood cell (RBC) 4.62×10 12 , platelet (PLT) 154×10 9 , Neutrophil (N) 65%. Routine urine test results: urine protein 3+, occult blood, RBC 0.61/Hp, glucose (GLU)2+. Renal function test results: blood urea nitrogen (BUN) 7.63 mmol/L, creatinine (Cr) 57 μmol/L, uric acid (UA) 336 μmol/L. Liver function test results: albumin (ALB) 30 g/L, lipid:total cholesterol (TCH) 5.60 mmol/L, triglyceride (TG) 2.13 mmol/L. The levels of serum albumin, serum creatinine, and urine protein of this patient on different dates are show in Table 1 . Glycosylated hemoglobin (HBA1c) 7.2%; albumin creatinine ratio (ACR) 5871.94 mg/g. No abnormalities in the following: four items of coagulation, three items of cardiac enzymes, eight items of infection, antineutrophil cytoplasmic antibody (ANCA), anti-phospholipase A2 antibody (PLA2R), antinuclear antibody profile, autoantibodies, blood and urine immunofixation electrophoresis, immunoglobulins, and complements. Impressions at admission: 1. nephrotic syndrome (NS); 2. hypertension, grade 2 with very high risk; 3. DM; 4. CHD; 5. post-pacemaker implantation; 6. post-operation of right cataract surgery. The treatments administered were as follows: 1. monitoring of BP and BG, with a low-salt, low-fat diabetic diet; 2. Atorvastatin for lipid-lowering, Novolin + glargine insulin + Metformin for BG lowering and amlodipine besylate for BP lowering; 3. plan to conduct a renal biopsy to confirm the pathological diagnosis. On 10 Nov 2021, following the completion of relevant examinations, a renal biopsy was conducted. On 12 Nov 2021, the T cell results for tuberculosis were found to be positive, and the department of tuberculosis was consulted. The suggestions were as follows: taking into account the calcific density nodule in the right middle lobe in lung CT and TB-IGRA (+), the status of tuberculosis infection should be considered, and old tuberculosis of the right lung could not be excluded; prophylactic anti-tuberculosis therapy should be conducted through the administration of hormones or immunosuppressants. Results following consultation with the department of ophthalmology were as follows: macular degeneration in both eyes, cataract in the left eye, post-operation of cataract surgery in the right eye. Calcium hydroxybenzene sulfonate was recommended along with regular review. Immunofluorescence (IF): IgA+ IgG± IgM- C3+ C1q- FRA- Alb± κ± λ± IgG 1- IgG 2- IgG 3- IgG 4- ; Light microscopy showed moderate to severe diffuse proliferation of mesangial cells and stroma. The periodic acid-Schiff staining showed moderate to diffuse proliferation of mesangial cells and stroma , and the periodic acid silver methenamine staining and Masson’s staining showed severe diffuse proliferation of mesangial cells and stroma . It was accompanied by diffuse thickening of the basement membrane, small eosinophilic deposits in the subendothelial segment, and microangioma-like expansion of segmental capillary loops. There were vacuoles and granular degeneration of renal tubular epithelial cells, focal atrophy, and a few protein casts were observed in the lumen. Renal interstitial focal lymphocyte and monocyte infiltration with fibrosis, wall thickening of small arteries, intimal fibrosis and sclerosis, and lumen stenosis were also observed.	3.677734	0.98584	sec[1]/p[0]	en	0.999998	PMC9816133	https://doi.org/10.3389/fendo.2022.992933	[ "blood", "mmol", "renal", "urine", "both", "tuberculosis", "edema", "times", "follows", "protein" ]	[ { "code": "3C0Z", "title": "Diseases of the blood or blood-forming organs, unspecified" }, { "code": "MF50.4Z", "title": "Haematuria, unspecified" }, { "code": "MA12.1", "title": "Finding of cocaine in blood" }, { "code": "MA12.4", "title": "Finding of steroid agent in blood" }, { "code": "MA12.2", "title": "Finding of hallucinogen in blood" }, { "code": "GB42.1", "title": "Albuminuria, Grade A3" }, { "code": "GB42.0", "title": "Albuminuria, Grade A2" }, { "code": "MA18.0Y", "title": "Other specified elevated blood glucose level" }, { "code": "GC2Z&XA6KU8", "title": "Disease of kidney, not elsewhere classified" }, { "code": "GB6Z", "title": "Kidney failure, unspecified" } ]	=== ICD-11 CODES FOUND === [3C0Z] Diseases of the blood or blood-forming organs, unspecified Also known as: Diseases of the blood or blood-forming organs, unspecified \| Blood, lymph or spleen disease \| blood condition NOS \| blood disorder NOS \| bone marrow disease NOS [MF50.4Z] Haematuria, unspecified Also known as: Haematuria, unspecified \| Haematuria \| blood in urine \| urinary blood \| haematuria NOS [MA12.1] Finding of cocaine in blood Also known as: Finding of cocaine in blood \| cocaine in blood [MA12.4] Finding of steroid agent in blood Also known as: Finding of steroid agent in blood \| steroid in blood [MA12.2] Finding of hallucinogen in blood Also known as: Finding of hallucinogen in blood \| hallucinogen in blood [GB42.1] Albuminuria, Grade A3 Definition: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted to the urine creatinine concentration to correct for variations in overall urine concentration. When persistent and of moderate or greater severity usually indicates overt glomerular disease such as caused by diabetic glomerulosclerosis, glomerulonephritis or amyloid. Also known as: Albuminuria, Grade A3 \| albuminuria >30 mg/mmol creatinine \| macroalbuminuria \| overt albuminuria \| overt nephropathy [GB42.0] Albuminuria, Grade A2 Definition: Presence of excessive albumin in the urine, indicating abnormal permeability glomerular filtration. Can be quantitated by either timed collections or spot urine samples with the concentration adjusted to the urine creatinine concentration to correct for variations in overall urine concentration. When persistent and of moderate or greater severity usually indicates overt glomerular disease such as caused by diabetic glomerulosclerosis, glomerulonephritis or amyloid. Also known as: Albuminuria, Grade A2 \| microalbuminuria \| incipient nephropathy \| mild to moderate albuminuria \| albuminuria 3-30 mg/mmol creatinine [MA18.0Y] Other specified elevated blood glucose level Also known as: Other specified elevated blood glucose level \| Blood glucose between 8.0 - 11.9 mmol/L pre-meal or fasting \| Blood glucose between 12.0 - 13.9 mmol/L pre-meal or fasting \| Blood glucose greater than or equal to 14.0 mmol/L pre-meal or fasting \| Blood glucose between 12.0 - 13.9 mmol/L post-meal or not otherwise specified [GB6Z] Kidney failure, unspecified Also known as: Kidney failure, unspecified \| nontraumatic kidney injury \| renal failure NOS \| kidney block \| renal impairment NOS === GRAPH WALKS === --- Walk 1 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --EXCLUDES--> [?] Diseases of the immune system --- Walk 2 --- [3C0Z] Diseases of the blood or blood-forming organs, unspecified --PARENT--> [03] Diseases of the blood or blood-forming organs Def: This chapter includes diseases of the blood as well as diseases of blood forming organs.... --RELATED_TO--> [?] Neoplasms of haematopoietic or lymphoid tissues Def: A neoplasm arising from hematopoietic or lymphoid cells found in the bone marrow, peripheral blood, lymph nodes and spleen (organs of the hematopoietic or lymphoid system). Hematopoietic or lymphoid c... --- Walk 3 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --CHILD--> [MF50.41] Microscopic haematuria --- Walk 4 --- [MF50.4Z] Haematuria, unspecified --PARENT--> [MF50.4] Haematuria Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th... --PARENT--> [MF50] Abnormal micturition --- Walk 5 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.1] Finding of cocaine in blood --- Walk 6 --- [MA12.1] Finding of cocaine in blood --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system --CHILD--> [MA12.1] Finding of cocaine in blood	[ "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --EXCLUDES--> [?] Diseases of the immune system", "[3C0Z] Diseases of the blood or blood-forming organs, unspecified\n --PARENT--> [03] Diseases of the blood or blood-forming organs\n Def: This chapter includes diseases of the blood as well as diseases of blood forming organs....\n --RELATED_TO--> [?] Neoplasms of haematopoietic or lymphoid tissues\n Def: A neoplasm arising from hematopoietic or lymphoid cells found in the bone marrow, peripheral blood, lymph nodes and spleen (organs of the hematopoietic or lymphoid system). Hematopoietic or lymphoid c...", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --CHILD--> [MF50.41] Microscopic haematuria", "[MF50.4Z] Haematuria, unspecified\n --PARENT--> [MF50.4] Haematuria\n Def: Hematuria means presence of blood or red blood cells (RBCs) in the urine. There are two types of hematuria: gross hematuria (when one can see the blood in the urine) and microscopic hematuria (when th...\n --PARENT--> [MF50] Abnormal micturition", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.1] Finding of cocaine in blood", "[MA12.1] Finding of cocaine in blood\n --PARENT--> [MA12] Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system\n --CHILD--> [MA12.1] Finding of cocaine in blood" ]	3C0Z	Diseases of the blood or blood-forming organs, unspecified	[ { "from_icd11": "3C0Z", "icd10_code": "D75A", "icd10_title": "Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia" }, { "from_icd11": "3C0Z", "icd10_code": "D7581", "icd10_title": "Myelofibrosis" }, { "from_icd11": "3C0Z", "icd10_code": "D7582", "icd10_title": "Heparin induced thrombocytopenia (HIT)" }, { "from_icd11": "3C0Z", "icd10_code": "D7589", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D759", "icd10_title": "Disease of blood and blood-forming organs, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "D763", "icd10_title": "Other histiocytosis syndromes" }, { "from_icd11": "3C0Z", "icd10_code": "Q899", "icd10_title": "Congenital malformation, unspecified" }, { "from_icd11": "3C0Z", "icd10_code": "III", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D70-D77", "icd10_title": "" }, { "from_icd11": "3C0Z", "icd10_code": "D75", "icd10_title": "Other and unspecified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D77", "icd10_title": "Other disorders of blood and blood-forming organs in diseases classified elsewhere" }, { "from_icd11": "3C0Z", "icd10_code": "D758", "icd10_title": "Other specified diseases of blood and blood-forming organs" }, { "from_icd11": "3C0Z", "icd10_code": "D76", "icd10_title": "Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue" }, { "from_icd11": "MF50.4Z", "icd10_code": "R310", "icd10_title": "Gross hematuria" }, { "from_icd11": "MF50.4Z", "icd10_code": "R312", "icd10_title": "Other microscopic hematuria" } ]	D75A	Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia
The patient was an 18-year-old woman, 2 gravida, nullipara. She had no remarkable medical or family history. She had a natural pregnancy and did not visit a hospital during the first trimester of pregnancy. The gestational age at the time of her initial hospital visit was estimated to be 15 weeks via measurement of the fetus on an ultrasound. Fetal biometry and estimated fetal weight were measured and evaluated according to the standardization committee of fetal measurement of the Japanese Society of Ultrasound in Medicine . Head circumference (HC) was calculated from the diameter of biparietal diameter (BPD) and occipito-frontal diameter using the formula for an eclipse and evaluated according to the Hadlock et al. . The BPD and femur length were 33 mm and 21 mm, respectively . At that time, there were no major anomalies, including the size and morphology of the fetal head. The estimated date of confinement was determined via ultrasound because of an unknown last menstrual period. At 23 weeks and 0 days of gestation, an ultrasound revealed a BPD of 40 mm (-5.3 standard deviations, SD), and the measured section of the BPD was poorly visualized. Additionally, observation of the fetal head using transvaginal ultrasound did not provide useful information about the cranial and cerebral defects. At 27 weeks and 5 days of gestation, an ultrasound revealed a BPD of 48 mm (-6.7 SD), however, there was difficulty visualizing the skull above the orbit and the overall growth, except for the head, was within the normal size range. At 29 weeks and 0 days of gestation, an ultrasound revealed a BPD of 43.6 mm (-8.9 SD) and a HC of 15.19 mm (-12.4 SD) . At 31 weeks and 6 days of gestation, we explained to the patient and the family that there was a possibility of microencephaly or anencephaly, and that anencephaly would result in a poor prognosis. We proposed magnetic resonance imaging (MRI) to the patient for a definitive diagnosis of the fetal head anomaly. However, the patient and the family refused further examination. At 34 weeks and 4 days of gestation, the patient was admitted for induction of labor due to the patient preferences. Elevation of the fetal head due to cervical dilatation by insertion of a metreurynter permitted confirmation of the structure of the fetal skull. MRI and 3-dimensional computed tomography (3D-CT) were performed to confirm the absence of anencephaly. 3D-CT revealed the presence of a fetal skull, a prominent occipital bone, and a sloping forehead . MRI showed a cerebral sickle, cerebellar tent, cerebral defects, a hypoplastic cerebellum, and a normal brain stem . The presence of the skull, cerebellum, and brainstem allowed us to diagnose the fetus with microhydranencephaly, rather than anencephaly. This diagnosis indicated that the fetus may be able to survive after delivery. Thus, the induction of labor was stopped, and the patient and the family were counseled for treatment. Vaginal delivery was scheduled with an exception for a cesarean section in the case of maternal indication. For the infant, noninvasive resuscitation treatment, except for tracheal intubation and chest compression, was scheduled for after delivery. Because the patient had cancelled many scheduled visits, she was hospitalized until delivery. At 36 weeks and 6 days of gestation, she had a premature rupture of the membranes, and delivered vaginally the following day without shoulder dystocia due to the small fetal head. The baby was a male weighing 2342 g, with a HC of 24 cm (-5.4 SD according to the Japanese neonatal data ) and Apgar scores of 1, 5, and 8 after 1, 5, and 10 min, respectively. Umbilical artery blood pH, pCO 2 , pO 2 , and base excess were 7.34, 42.7 mm Hg, 22.5 mm Hg, and − 3.5 mmol/L, respectively. After birth, the baby had spontaneous respiration. Both eyes and the nose had a normal appearance, but the head was flat above the forehead, with a suspected partial head defect with a sloping forehead . Skin defects were found in the parietal region , and encephalocele was suspected. The skin lesion was taped, and healing was confirmed after 12 days, with no signs of infection or leakage of cerebrospinal fluid. Although the sucking reflex was observed, swallowing movements appeared difficult. On day 11 after birth, a gastric tube was inserted, and tube feeding was started. On day 32 after birth, an MRI revealed the absence of cerebral tissue; instead, a membranous structure was present and hydrocephalus was evident , which was consistent with microhydranencephaly. Screenings for rubella virus, herpesvirus, cytomegalovirus, and toxoplasma infections causing hydrocephalus were all negative. Chromosomal analysis with G-banding yielded 46, XY, with and inv (9) (p12 q13) harboring less clinically significant abnormalities. The baby received desmopressin acetate due to central diabetes insipidus 6 months after birth. Fig. 1 Prenatal fetal ultrasonographic findings. a Image of the fetal head at 15 weeks and 3 days of gestation. b Sagittal and c transverse images of the fetus at 29 weeks and 0 days of gestation. d A BPD growth chart. The magnitude of BPD measured by ultrasound was plotted using the x-labels. BPD, biparietal diameter Fig. 2 Prenatal fetal 3-dimensional computed tomography (3D-CT) and magnetic resonance imaging (MRI) scans. a and b 3D-CT images at 34 weeks of gestation showing the presence of a fetal skull, a prominent occipital bone, and a sloping forehead. c T2-weighted sagittal image of an MRI scan showing a cerebral sickle, cerebellar tent, a defect of the cerebrum, a hypoplastic cerebellum, a normal brain stem, and excess cerebrospinal fluid Fig. 3 Images of the head and facial features of the infant after birth. a A skin defect without scalp rugae. b Front view of the face and c lateral view of the head. Both eyes and the nose appeared normal, but the head was flat above the forehead with a suspected partial head defect. d demonstrates the sloping forehead Fig. 4 Magnetic resonance imaging (MRI) findings of the infant’s head. a T2-weighted sagittal image. b T2-weighted coronal image. On day 32 after birth, an MRI scan showed the absence of a cerebrum and a membranous structure in the region of the cerebrum with hydrocephalus	4.027344	0.976563	sec[1]/p[0]	en	0.999996	33176733	https://doi.org/10.1186/s12884-020-03400-1	[ "head", "fetal", "gestation", "ultrasound", "forehead", "birth", "cerebral", "skull", "family", "fetus" ]	[ { "code": "NA63", "title": "Traumatic amputation at neck level" }, { "code": "MB4D", "title": "Headache, not elsewhere classified" }, { "code": "ME61", "title": "Subcutaneous swelling, mass or lump of uncertain or unspecified nature" }, { "code": "MB48.3", "title": "Light-headedness" }, { "code": "FB3Z", "title": "Disorders of muscles, unspecified" }, { "code": "LD9Z", "title": "Developmental anomalies, unspecified" }, { "code": "KB20.Z", "title": "Intrauterine hypoxia, unspecified" }, { "code": "3A50.4", "title": "Hereditary persistence of fetal haemoglobin" }, { "code": "KB42", "title": "Persistent pulmonary hypertension of the newborn" }, { "code": "LD2Z", "title": "Multiple developmental anomalies or syndromes, unspecified" } ]	=== ICD-11 CODES FOUND === [NA63] Traumatic amputation at neck level Also known as: Traumatic amputation at neck level \| complete head avulsion \| Decapitation \| decapitation of head at neck level \| severed head Includes: Decapitation [MB4D] Headache, not elsewhere classified Definition: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above. Also known as: Headache, not elsewhere classified \| cephalalgia \| cephalgia \| cephalodynia \| pain in head NOS Excludes: Trigeminal neuralgia \| Atypical facial pain \| Acute headache, not elsewhere classified [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature Also known as: Subcutaneous swelling, mass or lump of uncertain or unspecified nature \| localised swelling, mass or lump of skin and subcutaneous tissue \| localised subcutaneous nodules \| subcutaneous nodules \| superficial subcutaneous nodules Excludes: localized adiposity \| mass and lump: breast \| enlarged lymph nodes [MB48.3] Light-headedness Also known as: Light-headedness \| light headed [FB3Z] Disorders of muscles, unspecified Also known as: Disorders of muscles, unspecified \| disorder of muscle, unspecified \| muscle disease \| muscular disease \| muscular disorder [LD9Z] Developmental anomalies, unspecified Also known as: Developmental anomalies, unspecified \| congenital malformations, deformations and chromosomal abnormalities \| congenital malformation NOS \| developmental abnormality NOS \| fetal abnormality NOS [KB20.Z] Intrauterine hypoxia, unspecified Also known as: Intrauterine hypoxia, unspecified \| Intrauterine hypoxia \| fetal distress \| fetal distress syndrome \| intrauterine distress [3A50.4] Hereditary persistence of fetal haemoglobin Definition: Hereditary persistence of fetal haemoglobin (HPFH) associated with beta-thalassaemia is a haemoglobinopathy characterised by high haemoglobin (Hb)F levels and an increased number of fetal-Hb-containing cells. The association of HPFH with beta-thalassaemia mitigates the clinical manifestations which vary from a normal state to beta-thalassaemia intermedia. Also known as: Hereditary persistence of fetal haemoglobin \| HPFH - [Hereditary persistence of fetal haemoglobin] \| fetal haemoglobin \| persistence of fetal haemoglobin \| persistent haemoglobin F [KB42] Persistent pulmonary hypertension of the newborn Definition: Persistent pulmonary hypertension of the newborn is a cardiopulmonary disorder characterised by systemic arterial hypoxemia secondary to pulmonary hypertension and extrapulmonary right-to-left shunting across the foramen ovale and ductus arteriosus. Also known as: Persistent pulmonary hypertension of the newborn \| persistent fetal circulation syndrome \| fetal circulation \| PFC - [persistent fetal circulation] syndrome \| PPHN - [Persistent pulmonary hypertension of the newborn] [LD2Z] Multiple developmental anomalies or syndromes, unspecified Also known as: Multiple developmental anomalies or syndromes, unspecified \| multiple congenital birth defects NOS \| multiple congenital birth deformities NOS \| multiple fetal abnormalities NOS \| severe birth deformities NOS === GRAPH WALKS === --- Walk 1 --- [NA63] Traumatic amputation at neck level --PARENT--> [?] Injuries to the neck --CHILD--> [NA21] Open wound of neck --- Walk 2 --- [NA63] Traumatic amputation at neck level --PARENT--> [?] Injuries to the neck --EXCLUDES--> [?] Other injuries of spine or trunk, level unspecified --- Walk 3 --- [MB4D] Headache, not elsewhere classified Def: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above.... --EXCLUDES--> [?] Trigeminal neuralgia Def: Trigeminal neuralgia is a manifestation of orofacial neuropathic pain restricted to one or more divisions of the trigeminal nerve. The pain is recurrent, abrupt in onset and termination, triggered by ... --CHILD--> [?] Idiopathic trigeminal neuralgia Def: Idiopathic trigeminal neuralgia is a persistent facial pain that does not have the characteristics of cranial neuralgias and cannot be attributed to a different disorder... --- Walk 4 --- [MB4D] Headache, not elsewhere classified Def: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above.... --EXCLUDES--> [?] Acute headache, not elsewhere classified --PARENT--> [?] Acute pain Def: Pain with a duration of less than 3 months. This code should be used only when there is no further specification of site.... --- Walk 5 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --PARENT--> [?] Symptoms or signs involving the skin Def: This category allows the capture of imprecise data where a more specific diagnosis cannot be made or to supplement information about a specific diagnosis.... --CHILD--> [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --- Walk 6 --- [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature... --EXCLUDES--> [?] Localised adiposity Def: A condition characterised by accumulation of adipose tissue in specific regions of the body.... --CHILD--> [?] Fatty apron	[ "[NA63] Traumatic amputation at neck level\n --PARENT--> [?] Injuries to the neck\n --CHILD--> [NA21] Open wound of neck", "[NA63] Traumatic amputation at neck level\n --PARENT--> [?] Injuries to the neck\n --EXCLUDES--> [?] Other injuries of spine or trunk, level unspecified", "[MB4D] Headache, not elsewhere classified\n Def: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above....\n --EXCLUDES--> [?] Trigeminal neuralgia\n Def: Trigeminal neuralgia is a manifestation of orofacial neuropathic pain restricted to one or more divisions of the trigeminal nerve. The pain is recurrent, abrupt in onset and termination, triggered by ...\n --CHILD--> [?] Idiopathic trigeminal neuralgia\n Def: Idiopathic trigeminal neuralgia is a persistent facial pain that does not have the characteristics of cranial neuralgias and cannot be attributed to a different disorder...", "[MB4D] Headache, not elsewhere classified\n Def: Headache with characteristic features suggesting that it is a unique diagnostic entity, a finding or complaint, but not fulfilling criteria for any of the headache disorders described above....\n --EXCLUDES--> [?] Acute headache, not elsewhere classified\n --PARENT--> [?] Acute pain\n Def: Pain with a duration of less than 3 months.\n\nThis code should be used only when there is no further specification of site....", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --PARENT--> [?] Symptoms or signs involving the skin\n Def: This category allows the capture of imprecise data where a more specific diagnosis cannot be made or to supplement information about a specific diagnosis....\n --CHILD--> [ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...", "[ME61] Subcutaneous swelling, mass or lump of uncertain or unspecified nature\n Def: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature...\n --EXCLUDES--> [?] Localised adiposity\n Def: A condition characterised by accumulation of adipose tissue in specific regions of the body....\n --CHILD--> [?] Fatty apron" ]	NA63	Traumatic amputation at neck level	[ { "from_icd11": "NA63", "icd10_code": "S18", "icd10_title": "" }, { "from_icd11": "ME61", "icd10_code": "R2240", "icd10_title": "Localized swelling, mass and lump, unspecified lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2232", "icd10_title": "Localized swelling, mass and lump, left upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2242", "icd10_title": "Localized swelling, mass and lump, left lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2231", "icd10_title": "Localized swelling, mass and lump, right upper limb" }, { "from_icd11": "ME61", "icd10_code": "R2241", "icd10_title": "Localized swelling, mass and lump, right lower limb" }, { "from_icd11": "ME61", "icd10_code": "R2233", "icd10_title": "Localized swelling, mass and lump, upper limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2243", "icd10_title": "Localized swelling, mass and lump, lower limb, bilateral" }, { "from_icd11": "ME61", "icd10_code": "R2230", "icd10_title": "Localized swelling, mass and lump, unspecified upper limb" }, { "from_icd11": "ME61", "icd10_code": "R220", "icd10_title": "Localized swelling, mass and lump, head" }, { "from_icd11": "ME61", "icd10_code": "R221", "icd10_title": "Localized swelling, mass and lump, neck" }, { "from_icd11": "ME61", "icd10_code": "R222", "icd10_title": "Localized swelling, mass and lump, trunk" }, { "from_icd11": "ME61", "icd10_code": "R229", "icd10_title": "Localized swelling, mass and lump, unspecified" }, { "from_icd11": "ME61", "icd10_code": "R20-R23", "icd10_title": "" }, { "from_icd11": "ME61", "icd10_code": "R22", "icd10_title": "Localized swelling, mass and lump of skin and subcutaneous tissue" } ]	S18
The patient, woman, 23 years old, height 170 cm, weight 137 kg, body mass index (BMI) 47.4 kg/m 2 , entered our hospital on November 7, 2018 to perform laparoscopic bariatric surgery under general anesthesia. She was evaluated class III risk using the American Society of Anesthesiologists (ASA) criteria and did not have respiratory, circulatory diseases, liver, kidney, or other organ dysfunction. She had a history of penicillin allergy, appendectomy under local anesthesia, and cesarean section under spinal anesthesia. She has smoked for 7 years, 7 cigarettes/d, and has stopped smoking for 4 days before the operation. With preoperative prohibition of drinking for 6 hours, fasting for 12 hours, she has been monitored routinely and has been measured radial artery pressure when entered the operating room at 8:30 am on November 12, 2018. We provided the patient with 8 L/min high-flow mask oxygen. The arterial blood pressure (ABP) was 158/76 mm Hg. The heart rate (HR) was 78 beats/min. The peripheral oxygen saturation (SpO 2 ) measured using pulse oximetry was 99%. The results of arterial blood gas analysis were as follows: arterial partial pressure of carbon dioxide (PaCO 2 ), 44.3 mm Hg, arterial partial pressure of oxygen (PaO 2 ), 260 mm Hg; and arterial oxygen saturation (SaO 2 ) 100%. After 10 minutes of oxygen inhalation, anesthesia was induced with intravenous (IV) penehyclidine 1 mg , prednisolone 20 mg , midazolam 10 mg , propofol 150 mg , cis-atracurium 40 mg , and Sufentanil 20 μg . After tracheal intubation, breathing sounds were not heard in both lungs, and end tidal CO 2 (ETCO 2 ) can be seen continuously waveform. Auscultating again, we could hear the weak breath of both lungs, and a lot of dry and wet rales, then we used negative pressure to suck sputum with only a small amount of clear secretions. A lot of dry and wet rales could still been heard at this time. With hand-controlled ventilation, airway pressure (Paw) was 40 cmH 2 O, and tidal volume ( V t ) was 350 mL. At this point PaCO 2 was 50.6 mmHg, PaO 2 81 mm Hg, SaO 2 was 95%, SpO 2 was 95%, and fraction inspired O 2 (FiO 2 ) was 90%. We considered bronchospasm, followed by intravenous injection of aminophylline 250 mg, methylprednisolone 80 mg. After 15 minutes, the results of arterial blood gas analysis were as follows: PaCO 2 was 46.3 mm Hg, PaO 2 was 80 mm Hg, SaO 2 was 95%, SpO 2 was 95%, and FiO 2 was 90%. Hemodynamics showed no significant fluctuation and respiratory compliance improved. After 10 minutes, PaCO 2 was 40.6 mm Hg, PaO 2 was 103 mm Hg, SaO 2 was 98%, SpO 2 was 96%, and FiO 2 was 90%. Pressure controlled ventilation was used after intubation, Paw was 38 cmH 2 O, V t was 350 mL, respiratory rate (RR) was 12 breath/min, positive end-expiratory pressure (PEEP) was 5 cmH 2 O, and slope was 1.0 seconds. Please consult the Department of Respiratory Medicine. The consultation opinion indicates that the patient has a long history of smoking, and the airway sensitivity is likely to cause airway spasm after endotracheal intubation. We informed the patient's family related risks fully and obtained the consent of the patient's family to suspend the operation. After 1 hour, Paw was 35 cmH 2 O, V t was 450 mL, RR was 12 breath/min, PEEP was 5 cmH 2 O, slope was 1.0 seconds, and respiratory compliance was significantly improved. Return to intensive care unit safely under moderate sedation. Respiratory function recovered completely after 2 hours. We removed the tracheal tube. At this time, PaCO 2 was 40.1 mmHg, PaO 2 was 96 mm Hg, SaO 2 was 98%, SpO 2 was 96%, FiO 2 was 40%. The next day, the patient reported tracheal burning sensation, which was considered by the pressure injury of the airway. After preparation by atomization and oxygen therapy, the operation was performed again on November 21, 2018. Intramuscular injection of atropine 1 mg, intravenous drip of aminophylline 250 mg, and methylprednisolone 80 mg to prevent airway spasm. At this time, PaCO 2 was 44.2 mmHg, PaO 2 was 243 mmHg, SaO 2 was 100%, SpO 2 was 99%, FiO 2 was 50%. The drug and dose for anesthesia induction were the same as the first time, and the Hand-controlled ventilation was given after the spontaneous breathing disappears. During ventilation, airway resistance was high, skin of anterior chest and neck was flushed, and there was no obvious hemodynamic fluctuation. After tracheal intubation, anesthesia was maintained with sevoflurane in 50% oxygen in air. A large number of dry and wet rales could been heard in both lungs during auscultation. The airway resistance increased significantly. After 5 minutes, the symptoms of skin flushing gradually disappeared. At this moment, PaCO 2 was 41.6 mmHg, PaO 2 was 98 mmHg, SaO 2 was 98%, SPO 2 was 98%, FiO 2 was 90%. Then, the pressure ventilation mode was given, P plat was 28 cmH 2 O, RR was 12 breath/min, PEEP was 7 cmH 2 O, slope was 1.0 seconds, V t was 522 mL. After 1 hour, the rales gradually disappeared and the breath sound was clear. Re-examination of arterial blood gas analysis were as follows: PaCO 2 was 49.5 mm Hg, PaO 2 was 135 mm Hg, SaO 2 was 99%, SpO 2 was 99%, FiO 2 was 90%. At this time, the patient's breathing gradually recovered, and 5 mg of cis-atracurium was given again. The airway pressure increased immediately. Auscultation of the lungs again showed a large amount of dry rales, considering anaphylactoid reaction caused by cis-atracurium which induced the bronchial spasm. Respiratory consultation pointed out that under general anesthesia tracheal intubation, the left lung breath sounds weak, the right lower lung was covered with biphasic high-pitched dry sounds. Symptoms such as skin flushing and bronchospasm appeared immediately after induction of this patient, and cis-atracurium was considered to cause anaphylactoid reaction. When the symptoms were gradually relieved, we gave cisatracurium again with bronchospasm symptoms immediately developed, while bronchospasm did not occur again when rocuronium was given. It was equivalent to carry out drug provocation test without knowing it. Drug provocation test is the golden standard for finding allergens, so it was determined that the bronchospasm was caused by cis-atracurium.	3.917969	0.975586	sec[1]/p[0]	en	0.999998	33847670	https://doi.org/10.1097/MD.0000000000025516	[ "pressure", "paco", "airway", "anesthesia", "respiratory", "oxygen", "arterial", "this", "breath", "mmhg" ]	[ { "code": "EH90.Z", "title": "Pressure ulcer of unspecified grade" }, { "code": "MB23.L", "title": "Pressured speech" }, { "code": "MD30.Z", "title": "Chest pain, unspecified" }, { "code": "CB22.Y", "title": "Other specified diseases of mediastinum, not elsewhere classified" }, { "code": "BA2Z", "title": "Hypotension, unspecified" }, { "code": "CB40.Y", "title": "Other specified diseases of the respiratory system" }, { "code": "CA22.Z", "title": "Chronic obstructive pulmonary disease, unspecified" }, { "code": "7A41", "title": "Obstructive sleep apnoea" }, { "code": "CB60", "title": "Tracheostomy malfunction" }, { "code": "MD2Z", "title": "Haemorrhage from respiratory passages, unspecified" } ]	=== ICD-11 CODES FOUND === [EH90.Z] Pressure ulcer of unspecified grade Also known as: Pressure ulcer of unspecified grade \| Pressure ulceration \| pressure injury \| pressure ulcer \| decubitus ulcer [MB23.L] Pressured speech Definition: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person talks without any social stimulation and may continue to talk even though no one is listening. Also known as: Pressured speech Excludes: Schizophrenia or other primary psychotic disorders \| Bipolar or related disorders [MD30.Z] Chest pain, unspecified Also known as: Chest pain, unspecified \| Pain in throat or chest \| chest pain NOS \| pain in chest \| chest pressure [CB22.Y] Other specified diseases of mediastinum, not elsewhere classified Also known as: Other specified diseases of mediastinum, not elsewhere classified \| Hernia of mediastinum \| mediastinal hernia \| mediastinal herniation \| Infectious mediastinitis [BA2Z] Hypotension, unspecified Also known as: Hypotension, unspecified \| hypopiesis \| low blood pressure \| arterial hypotension NOS \| decreased blood pressure [CB40.Y] Other specified diseases of the respiratory system Also known as: Other specified diseases of the respiratory system \| Secondary respiratory disorders \| Respiratory disorders in diseases classified elsewhere \| Diseases of bronchus, not elsewhere classified \| Acquired bronchial diverticulum [CA22.Z] Chronic obstructive pulmonary disease, unspecified Also known as: Chronic obstructive pulmonary disease, unspecified \| Chronic obstructive pulmonary disease \| COPD - [chronic obstructive pulmonary disease] \| COAD - [chronic obstructive airways disease] \| COLD - [chronic obstructive lung disease] [7A41] Obstructive sleep apnoea Definition: Obstructive sleep apnoea is characterised by repetitive episodes of apnoea or hypopnea that are caused by upper airway obstruction occurring during sleep. These events often result in reductions in blood oxygen saturation and are usually terminated by brief arousals from sleep. Excessive sleepiness is a major presenting complaint in many but not all cases. Reports of insomnia, poor sleep quality, and fatigue are also common. Upper airway resistance syndrome shares the same pathophysiology and sh Also known as: Obstructive sleep apnoea \| obstructive sleep apnoea syndrome \| obstructive sleep apnoea, adult \| OSA - [obstructive sleep apnoea] \| obstructive sleep apnoea, paediatric Excludes: Obstructive neonatal apnoea [CB60] Tracheostomy malfunction Also known as: Tracheostomy malfunction \| tracheostomy dysfunction \| status of malfunctioning tracheostomy \| functional disturbance of tracheostomy \| tracheostomy complications [MD2Z] Haemorrhage from respiratory passages, unspecified Also known as: Haemorrhage from respiratory passages, unspecified \| haemorrhage from respiratory tract \| respiratory system haemorrhage \| airway haemorrhage NOS === GRAPH WALKS === --- Walk 1 --- [EH90.Z] Pressure ulcer of unspecified grade --PARENT--> [EH90] Pressure ulceration Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th... --EXCLUDES--> [?] Erosion or ectropion of cervix uteri Def: A condition of the cervix uteri, caused by an increase in the total estrogen level in the body. This condition is characterised by protrusion and transformation of the endocervical columnar epithelium... --- Walk 2 --- [EH90.Z] Pressure ulcer of unspecified grade --PARENT--> [EH90] Pressure ulceration Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th... --CHILD--> [EH90.2] Pressure ulceration grade 3 Def: Pressure ulcer with full thickness skin loss. Subcutaneous fat may be visible but bone, tendon or muscle are not exposed. Slough may be present but does not obscure the depth of tissue loss. There may... --- Walk 3 --- [MB23.L] Pressured speech Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t... --EXCLUDES--> [?] Schizophrenia or other primary psychotic disorders Def: Schizophrenia and other primary psychotic disorders are characterised by significant impairments in reality testing and alterations in behaviour manifest in positive symptoms such as persistent delusi... --PARENT--> [?] Mental, behavioural or neurodevelopmental disorders Def: Mental, behavioural and neurodevelopmental disorders are syndromes characterised by clinically significant disturbance in an individual's cognition, emotional regulation, or behaviour that reflects a ... --- Walk 4 --- [MB23.L] Pressured speech Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t... --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour --RELATED_TO--> [?] Speech dysfluency Def: Speech dysfluency is characterised by the frequent or pervasive disruption of the rhythmic flow of speech that arises subsequent to the developmental period (i.e., adult onset) and is outside the limi... --- Walk 5 --- [MD30.Z] Chest pain, unspecified --PARENT--> [MD30] Pain in throat or chest Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx.... --RELATED_TO--> [?] Pain in throat Def: Pain in throat means having pain sensation in throat. Throat is a tube that carries food to oesophagus and air to windpipe and larynx.... --- Walk 6 --- [MD30.Z] Chest pain, unspecified --PARENT--> [MD30] Pain in throat or chest Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx.... --EXCLUDES--> [?] Acute pharyngitis Def: Acute pharyngitis is defined as an infection or irritation of the pharynx and/or tonsils and is a part of the common cold symptoms. The etiology is usually infectious, with most cases being of viral o...	[ "[EH90.Z] Pressure ulcer of unspecified grade\n --PARENT--> [EH90] Pressure ulceration\n Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th...\n --EXCLUDES--> [?] Erosion or ectropion of cervix uteri\n Def: A condition of the cervix uteri, caused by an increase in the total estrogen level in the body. This condition is characterised by protrusion and transformation of the endocervical columnar epithelium...", "[EH90.Z] Pressure ulcer of unspecified grade\n --PARENT--> [EH90] Pressure ulceration\n Def: Pressure ulcers result from localised injury and ischaemic necrosis of skin and underlying tissues due to prolonged pressure, or pressure in combination with shear; bony prominences of the body are th...\n --CHILD--> [EH90.2] Pressure ulceration grade 3\n Def: Pressure ulcer with full thickness skin loss. Subcutaneous fat may be visible but bone, tendon or muscle are not exposed. Slough may be present but does not obscure the depth of tissue loss. There may...", "[MB23.L] Pressured speech\n Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t...\n --EXCLUDES--> [?] Schizophrenia or other primary psychotic disorders\n Def: Schizophrenia and other primary psychotic disorders are characterised by significant impairments in reality testing and alterations in behaviour manifest in positive symptoms such as persistent delusi...\n --PARENT--> [?] Mental, behavioural or neurodevelopmental disorders\n Def: Mental, behavioural and neurodevelopmental disorders are syndromes characterised by clinically significant disturbance in an individual's cognition, emotional regulation, or behaviour that reflects a ...", "[MB23.L] Pressured speech\n Def: Speech in which the person feels undue pressure to get the words out. The person’s speech is usually rapid, loud, and emphatic and may be difficult or impossible to interrupt. Frequently, the person t...\n --PARENT--> [MB23] Symptoms or signs involving appearance or behaviour\n --RELATED_TO--> [?] Speech dysfluency\n Def: Speech dysfluency is characterised by the frequent or pervasive disruption of the rhythmic flow of speech that arises subsequent to the developmental period (i.e., adult onset) and is outside the limi...", "[MD30.Z] Chest pain, unspecified\n --PARENT--> [MD30] Pain in throat or chest\n Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx....\n --RELATED_TO--> [?] Pain in throat\n Def: Pain in throat means having pain sensation in throat. Throat is a tube that carries food to oesophagus and air to windpipe and larynx....", "[MD30.Z] Chest pain, unspecified\n --PARENT--> [MD30] Pain in throat or chest\n Def: Pain in throat and chest means having pain sensation in throat or chest. Throat is a tube that carries food to oesophagus and air to windpipe and larynx. The technical name for throat is pharynx....\n --EXCLUDES--> [?] Acute pharyngitis\n Def: Acute pharyngitis is defined as an infection or irritation of the pharynx and/or tonsils and is a part of the common cold symptoms. The etiology is usually infectious, with most cases being of viral o..." ]	EH90.Z	Pressure ulcer of unspecified grade	[ { "from_icd11": "EH90.Z", "icd10_code": "L89623", "icd10_title": "Pressure ulcer of left heel, stage 3" }, { "from_icd11": "EH90.Z", "icd10_code": "L89621", "icd10_title": "Pressure ulcer of left heel, stage 1" }, { "from_icd11": "EH90.Z", "icd10_code": "L89899", "icd10_title": "Pressure ulcer of other site, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L89620", "icd10_title": "Pressure ulcer of left heel, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89622", "icd10_title": "Pressure ulcer of left heel, stage 2" }, { "from_icd11": "EH90.Z", "icd10_code": "L89892", "icd10_title": "Pressure ulcer of other site, stage 2" }, { "from_icd11": "EH90.Z", "icd10_code": "L89519", "icd10_title": "Pressure ulcer of right ankle, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L89891", "icd10_title": "Pressure ulcer of other site, stage 1" }, { "from_icd11": "EH90.Z", "icd10_code": "L89610", "icd10_title": "Pressure ulcer of right heel, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89893", "icd10_title": "Pressure ulcer of other site, stage 3" }, { "from_icd11": "EH90.Z", "icd10_code": "L89890", "icd10_title": "Pressure ulcer of other site, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89629", "icd10_title": "Pressure ulcer of left heel, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L89619", "icd10_title": "Pressure ulcer of right heel, unspecified stage" }, { "from_icd11": "EH90.Z", "icd10_code": "L8945", "icd10_title": "Pressure ulcer of contiguous site of back, buttock and hip, unstageable" }, { "from_icd11": "EH90.Z", "icd10_code": "L89894", "icd10_title": "Pressure ulcer of other site, stage 4" } ]	L89623	Pressure ulcer of left heel, stage 3
A 63-year-old patient suffering from advanced chronic periodontitis came under our observation to resolve his dental problems with a specific request to be rehabilitated with fixed prostheses. The patient was in good health general state. Intraoral examination showed tartar deposits and various mobility degrees of the upper and lower teeth in the masticatory region. In addition, there were no adequate fixed restorations on natural teeth numbers 4-5-28-29-30-31. In Figure 1 the initial periodontal charting and radiographic status of the patient are shown. The panoramic radiograph presented by the patient before avulsion of the left side elements indicated widespread bone resorption. After taking alginate impressions, models were developed with diagnostic wax-up of the case. A TC Dental Scan to evaluate the available bone volume for the prosthetic rehabilitation of periodontally compromised sites has been prescribed. After careful oral examination and periodontal charting, nonsurgical periodontal therapy was carried out and reevaluation at 4 weeks was done. At this time point, the patient had severe periodontal bone loss in the posterior right regions. The images demonstrate the clinical healing at 10 weeks after the extractions of left posterior teeth. Periodontal charting at reevaluation is shown in Figure 3 . On tooth number 21 we proceeded to remove the incongruous fixed prosthetic crown and we cemented a provisional resin restoration. The patient throughout the course of therapy was maintained periodontally stable and at each control was motivated to oral hygiene; this is in accordance with the guidelines set out recently in the seventh European Workshop on Periodontology . After the reduction of local irritative factors implant treatment was undertaken . Surgery was performed under local anesthesia using 4% articaine solution combined with a vasoconstrictor (Ubistesin forte, 3M ESPE). The incision was extended from the edentulous distal crest to the sulcus of the mesial tooth, the canine. A full thickness flap was carefully elevated. It was decided to proceed with the placement of implants in sites numbers 12-13-14 (OsseoSpeed Astra Tech AB, Mölndal, Sweden) and numbers 21-20-19 with a type 3 implant placement (large bone healing and full maturity of soft tissue/T, 10 weeks). Before surgery, the patient has performed for 2 minutes rinse with chlorhexidine 0.20% (Curasept, Curadent Healthcare SPA, Saronno VA, Italy). The prosthetic-guided implant positioning was obtained with the aid of a surgical template developed by the initial diagnostic wax-up . Sites numbers 12-13-14 were prepared to accommodate 2 implants, respectively, 3.5 × 11 mm and 1 implant 4.0 × 11 mm. In the mandible 3 implants were inserted: 3.5 × 9 mm (number 21), 3.5 × 11 mm (number 20), and 4.0 × 9 mm (number 19), respectively. The wound was closed with E-PTFE mattress and simple sutures . After surgery, the patient received antibiotic prophylaxis with amoxicillin 2 g/day and rinsed 0.2% chlorhexidine digluconate 3 times/day for 4 weeks. The patient was shown how to perform a roll-stroke brushing technique and was motivated to control oral hygiene. The patient did not report specific symptoms and showed no adverse clinical signs. During implant osseointegration implant, the patient did not apply on the edentulous ridges any provisional prosthetic rehabilitation in order to avoid trauma and wound dehiscence in the early stages of healing. After 5 months of healing, we proceeded to expose the implants with a small incision using a miniblade. The cover screw was replaced with a healing abutment. We proceeded at 7 days to take the polyether impression (Impregum Penta 3M ESPE). Clinical examination at the delivery of the prosthesis revealed clinically healthy peri-implant soft tissue and no signs of complication. We proceeded to cement with temporary device (Temp Bond, Kerr, Italy) carefully controlling cement excess to avoid inflammation of peri-implant tissues . After 4 weeks, we proceeded to rehabilitate the posterior right regions. For the maxillary arch implant placement was made at six weeks from the extractions (type 2 placement). Soft tissue healing made easier the surgery but the partial bone healing has forced us to a recountourning of the crest with a xenograft (Bio-Oss, Geistlich, Wolhusen, Switzerland) at the gap created between the implant surface and the residual alveolar extraction cavity . In fact, literature demonstrates a spontaneous healing for 4-wall defects with bone gap of 1.5–2 mm . In cases of osseous irregularity or big defects is necessary to use a bone graft to counteract the peri-implant tissue remodeling. The width of the ridge was well maintained, showing postoperative ridge width between 5 and 6 mm at the most coronal portion of the alveolar bone. Two 3.5/13 mm implants were inserted in the number 5-4 sites and 1 implant 4.0/9 mm in site number 3. One month after the surgery we removed incongruous fixed prosthesis on numbers 31-30-29, grinded stumps, and delivered provisional bridges number 31 to number 29. After two weeks, number 28 was extracted because fractured and an immediate implant was placed in fresh socket (type 1) . In Table 1 all implant dimensions and a brief surgical and prosthetic schematic overview are shown. Here we have opted for a transmucosal healing for an easier management of the flaps and tissue closure. Literature does not demonstrate statistically significant differences in terms of survival and success rates for implants inserted with two-stage or one-stage technique and transmucosal healing . The osseointegration period was, respectively, 6 months for the maxilla and 4 months for the mandibula. All postextraction implants were inserted by placing implant shoulder 1 mm under the crestal bone level providing the physiological ridge alterations after extraction, and were anchored to the lingual/palatal cortical plate of the post-extraction sites [ 15 – 18 ]. In Figure 7 is shown a schematic overview of timing of implant placement relative to tooth extraction. Clinical control at the delivery of the prothesis shows the appearance of a great aesthetic result and good quality of peri-implant soft tissues .	3.919922	0.976563	sec[1]/p[0]	en	0.999997	25949833	https://doi.org/10.1155/2015/217895	[ "implant", "healing", "bone", "number", "implants", "numbers", "periodontal", "shown", "prosthetic", "sites" ]	[ { "code": "GC7A", "title": "Disorders of breast augmentation" }, { "code": "QB51.7", "title": "Presence of orthopaedic joint implants" }, { "code": "QB51.Y", "title": "Presence of other specified devices other than cardiac or vascular implants" }, { "code": "QB51.5", "title": "Presence of endocrine implants" }, { "code": "QB51.6", "title": "Presence of tooth-root or mandibular implants" }, { "code": "BA50", "title": "Old myocardial infarction" }, { "code": "DA63.Y&XT5R", "title": "Acute healed duodenal ulcer" }, { "code": "BD74.32", "title": "Healed venous leg ulcer" }, { "code": "EC20.02", "title": "Autosomal recessive congenital ichthyosis" }, { "code": "EB90.1Y", "title": "Other specified forms of cutaneous mucinosis" } ]	=== ICD-11 CODES FOUND === [GC7A] Disorders of breast augmentation Definition: A group of disorders that may arise in concert with or subsequent to the surgical placement of breast implants. Also known as: Disorders of breast augmentation \| Capsule contraction or scarring \| Implant rupture [QB51.7] Presence of orthopaedic joint implants Also known as: Presence of orthopaedic joint implants \| presence of joint implant \| replacement of joint by artificial or mechanical device or prosthesis \| Presence of shoulder-joint implant \| presence of shoulder joint replacment prosthesis [QB51.Y] Presence of other specified devices other than cardiac or vascular implants Also known as: Presence of other specified devices other than cardiac or vascular implants \| Presence of bone or tendon implants other than orthopaedic joint implants \| replacement of tendon by artificial or mechanical device or prosthesis \| presence of tendon implant \| Presence of skull plate [QB51.5] Presence of endocrine implants Also known as: Presence of endocrine implants \| presence of insulin pump Includes: presence of insulin pump [QB51.6] Presence of tooth-root or mandibular implants Also known as: Presence of tooth-root or mandibular implants \| presence of tooth root implant \| presence of mandibular implant [BA50] Old myocardial infarction Definition: Past myocardial infarction diagnosed by electrocardiogram (ECG) or other special investigation, but currently presenting no symptoms. Also known as: Old myocardial infarction \| past myocardial infarction \| healed myocardial infarction \| myocardial scar \| myocardial scarring Includes: healed myocardial infarction [BD74.32] Healed venous leg ulcer Also known as: Healed venous leg ulcer [EC20.02] Autosomal recessive congenital ichthyosis Definition: A heterogeneous group of genetically-determined ichthyoses with autosomal recessive inheritance. Also known as: Autosomal recessive congenital ichthyosis \| Congenital non-bullous ichthyosiform erythroderma \| Congenital ichthyotic ichthyosis \| Lamellar ichthyosis \| Collodion baby [EB90.1Y] Other specified forms of cutaneous mucinosis Also known as: Other specified forms of cutaneous mucinosis \| Focal primary cutaneous mucinosis \| Idiopathic follicular mucinosis \| Alopecia mucinosa \| Focal cutaneous mucinosis Includes: Idiopathic follicular mucinosis === GRAPH WALKS === --- Walk 1 --- [GC7A] Disorders of breast augmentation Def: A group of disorders that may arise in concert with or subsequent to the surgical placement of breast implants.... --PARENT--> [?] Postprocedural disorders of genitourinary system Def: Any disorder caused by or subsequent to any intervention of the genitourinary system.... --EXCLUDES--> [?] States associated with artificial menopause Def: Any condition caused by the artificial cessation of menstruation induced by surgical or pharmacological effects.... --- Walk 2 --- [GC7A] Disorders of breast augmentation Def: A group of disorders that may arise in concert with or subsequent to the surgical placement of breast implants.... --PARENT--> [?] Postprocedural disorders of genitourinary system Def: Any disorder caused by or subsequent to any intervention of the genitourinary system.... --CHILD--> [GC71] Prolapse of vaginal vault after hysterectomy Def: A condition of the vagina, caused by or subsequent to hysterectomy. This condition is characterised by descensus of the vaginal vault that may also lead to weakening of the vaginal walls.... --- Walk 3 --- [QB51.7] Presence of orthopaedic joint implants --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants --EXCLUDES--> [?] Fitting, adjustment or management of devices --- Walk 4 --- [QB51.7] Presence of orthopaedic joint implants --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants --CHILD--> [QB51.1] Presence of urogenital implants --- Walk 5 --- [QB51.Y] Presence of other specified devices other than cardiac or vascular implants --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants --PARENT--> [?] Presence of device, implants or grafts --- Walk 6 --- [QB51.Y] Presence of other specified devices other than cardiac or vascular implants --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants --EXCLUDES--> [?] Fitting, adjustment or management of devices	[ "[GC7A] Disorders of breast augmentation\n Def: A group of disorders that may arise in concert with or subsequent to the surgical placement of breast implants....\n --PARENT--> [?] Postprocedural disorders of genitourinary system\n Def: Any disorder caused by or subsequent to any intervention of the genitourinary system....\n --EXCLUDES--> [?] States associated with artificial menopause\n Def: Any condition caused by the artificial cessation of menstruation induced by surgical or pharmacological effects....", "[GC7A] Disorders of breast augmentation\n Def: A group of disorders that may arise in concert with or subsequent to the surgical placement of breast implants....\n --PARENT--> [?] Postprocedural disorders of genitourinary system\n Def: Any disorder caused by or subsequent to any intervention of the genitourinary system....\n --CHILD--> [GC71] Prolapse of vaginal vault after hysterectomy\n Def: A condition of the vagina, caused by or subsequent to hysterectomy. This condition is characterised by descensus of the vaginal vault that may also lead to weakening of the vaginal walls....", "[QB51.7] Presence of orthopaedic joint implants\n --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants\n --EXCLUDES--> [?] Fitting, adjustment or management of devices", "[QB51.7] Presence of orthopaedic joint implants\n --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants\n --CHILD--> [QB51.1] Presence of urogenital implants", "[QB51.Y] Presence of other specified devices other than cardiac or vascular implants\n --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants\n --PARENT--> [?] Presence of device, implants or grafts", "[QB51.Y] Presence of other specified devices other than cardiac or vascular implants\n --PARENT--> [QB51] Presence of devices other than cardiac or vascular implants\n --EXCLUDES--> [?] Fitting, adjustment or management of devices" ]	GC7A	Disorders of breast augmentation	[ { "from_icd11": "QB51.7", "icd10_code": "Z96652", "icd10_title": "Presence of left artificial knee joint" }, { "from_icd11": "QB51.7", "icd10_code": "Z96649", "icd10_title": "Presence of unspecified artificial hip joint" }, { "from_icd11": "QB51.7", "icd10_code": "Z96641", "icd10_title": "Presence of right artificial hip joint" }, { "from_icd11": "QB51.7", "icd10_code": "Z96653", "icd10_title": "Presence of artificial knee joint, bilateral" }, { "from_icd11": "QB51.7", "icd10_code": "Z96651", "icd10_title": "Presence of right artificial knee joint" }, { "from_icd11": "QB51.7", "icd10_code": "Z96643", "icd10_title": "Presence of artificial hip joint, bilateral" }, { "from_icd11": "QB51.7", "icd10_code": "Z96642", "icd10_title": "Presence of left artificial hip joint" }, { "from_icd11": "QB51.7", "icd10_code": "Z96611", "icd10_title": "Presence of right artificial shoulder joint" }, { "from_icd11": "QB51.7", "icd10_code": "Z96619", "icd10_title": "Presence of unspecified artificial shoulder joint" }, { "from_icd11": "QB51.7", "icd10_code": "Z96612", "icd10_title": "Presence of left artificial shoulder joint" }, { "from_icd11": "QB51.7", "icd10_code": "Z96659", "icd10_title": "Presence of unspecified artificial knee joint" }, { "from_icd11": "QB51.7", "icd10_code": "Z96629", "icd10_title": "Presence of unspecified artificial elbow joint" }, { "from_icd11": "QB51.7", "icd10_code": "Z96661", "icd10_title": "Presence of right artificial ankle joint" }, { "from_icd11": "QB51.7", "icd10_code": "Z96691", "icd10_title": "Finger-joint replacement of right hand" }, { "from_icd11": "QB51.7", "icd10_code": "Z96698", "icd10_title": "Presence of other orthopedic joint implants" } ]	Z96652	Presence of left artificial knee joint
An 11-year-old female patient with no previous history presented with right conjunctival injection and photophobia. The patient had previously been treated with fluorometholone 0.1% eye drops; however, the same symptoms recurred twice in 1 year. At presentation, her best-corrected decimal visual acuity (BCVA) was 0.4 in the right eye and 1.2 in the left eye. Intraocular pressures (IOPs) of the right and left eyes were 17 and 16 mmHg, respectively (Normal range: 10–21 mmHg). Slit-lamp examination showed ciliary injection and diffuse fine keratic precipitates. Micro-hypopyon and an anterior chamber cell grade of 3+ (based on the Standardization of Uveitis Nomenclature Working Group classification ) were observed; posterior synechiae were also present in the right eye. Fundus examination of the right eye was hazy and lacked clarity. The left eye exhibited no apparent abnormalities in the anterior chamber or fundus. Fluorescein angiography (FA) of the right eye revealed diffuse vascular leakage and optic disc leakage. The patient did not complain of arthralgia or genital ulcers, but had a history of recurrent oral ulcers. On the basis of these findings, the patient was diagnosed with unilateral panuveitis. The differential diagnosis was as follows: Behçet’s disease, juvenile idiopathic arthritis-related uveitis, HLA-B27-related uveitis, A20 haploinsufficiency, and sarcoidosis. Dexamethasone eye drops (0.1%, instilled hourly), tropicamide/phenylephrine eye drops (four times/day), 1% atropine eye drops (once/day), and prednisolone (15 mg/day orally) therapies were initiated for inflammation of the right eye. Further investigation revealed ileocecal ulcers and HLA-B51 positivity. Interferon-gamma release assay and tuberculin tests for tuberculosis infection, raid plasma regain assay, and Treponema pallidum antibody hemagglutination test for syphilis were negative; angiotensin-converting enzyme, antinuclear antibody, matrix metalloproteinase-3, and anti-citrullinated protein antibody levels were within the normal range. A20 haploinsufficiency was thought to be less likely in this patient, due to the absence of a family history of autoimmune diseases, genital ulcers, and fever spikes . In Behçet’s disease, oral ulcers heal without scars, uveitis typically involves the posterior chamber, and retinal vasculitis manifests with a fern-like pattern; in contrast, A20 haploinsufficiency is characterised by anterior uveitis . Because of the presence of typical ocular symptoms and recurrent oral ulcers, the patient was diagnosed with the incomplete type of Behçet’s disease, in accordance with the Japanese diagnostic criteria for Behçet’s disease . Inflammation in the anterior chamber and BCVA gradually improved after the treatment. However, a relapse occurred in the right eye and new-onset uveitis appeared in the left eye during the tapering of prednisolone. Adalimumab was administered subcutaneously to avoid the side effects of systemic corticosteroid. The patient was 12-year-old and weighed 36 kg when adalimumab was started. Since there is no indication regarding the dose of adalimumab for paediatric Behçet’s disease, we administered 40 mg every 2 weeks without a loading dose, in accordance with the recommended dose for treatment of juvenile idiopathic arthritis-associated uveitis. The duration of uveoretinitis prior to starting adalimumab was 11 months. After beginning administration of adalimumab, the patient complained of transient abdominal pain, which resolved spontaneously. BCVA improved to 1.5 in both eyes and the anterior chamber cell grade improved to < 0.5+ within 2 weeks. Within 4 weeks, laser flare photometry values dramatically improved from the peak value of 48 ph/ms (physiological value approximately 3 ph/ms) in both eyes, to 3–4 ph/ms in the right eye and 2–3 ph/ms in the left eye. FA revealed improvement of retinal vasculitis. Oral ulcers healed without scars after adalimumab administration. Ileocecal ulcers were completely resolved on the follow-up colonoscopy, which was performed 3 months after initiation of the therapy. The inflammation has remained well-controlled by administration of adalimumab without local or systemic corticosteroid for 17 months. No side effects of adalimumab have been observed. Fig. 1 Clinical appearance of the right eye of Case 1 at presentation. Slit-lamp examination showed ciliary injection and diffuse non-granulomatous keratic precipitates in the right eye. Micro-hypopyon and anterior chamber cell grading of 3+ (based on the Standardization of Uveitis Nomenclature Working Group classification) were observed, as were posterior synechiae in the right eye Fig. 2 Fluorescein angiography (FA) of Case 1 before and after adalimumab treatment. a , b FA of the right and left eyes at presentation, respectively, revealed fern-like diffuse vascular leakage and optic disc hyperfluorescence in the right eye. FA of the left eye at presentation did not show any vasculitis. c , d FA of the right and left eyes at 5 months after adalimumab treatment, respectively. There were no signs of vasculitis in either eye Fig. 3 Changes in anterior chamber cell grade ( a ) and prednisolone dose ( b ) in Case 1. Anterior chamber cell grading in the right eye was 3+ (based on the Standardization of Uveitis Nomenclature Working Group classification) at presentation. After prednisolone 15 mg/day was initiated, ocular inflammation and best-corrected visual acuity (BCVA) gradually improved; however, relapse occurred in both eyes during tapering of prednisolone. Adalimumab was administered to avoid the side effects of systemic corticosteroid. BCVA improved to 1.5 in both eyes and the anterior chamber cell grade improved to less than 0.5+ within 2 weeks. Both local and systemic corticosteroid were discontinued, and complete control of inflammation was achieved with adalimumab Fig. 4 Change in laser flare photometry values in Case 1. Laser flare values were recorded from day 63. Increased laser flares were observed during two relapses under the influence of corticosteroid. After adalimumab was introduced, laser flare values dramatically improved and reached the normal range within 4 weeks, without local or systemic corticosteroid	4.203125	0.96875	sec[1]/sec[0]/p[0]	en	0.999998	31182107	https://doi.org/10.1186/s12969-019-0333-6	[ "adalimumab", "chamber", "uveitis", "ulcers", "improved", "eyes", "cell", "corticosteroid", "bcva", "prednisolone" ]	[ { "code": "9A8Z", "title": "Disorders of the anterior chamber, unspecified" }, { "code": "9A96.3", "title": "Primary anterior uveitis" }, { "code": "9A90.0", "title": "Disorders of chamber angle" }, { "code": "9A8Y", "title": "Other specified disorders of the anterior chamber" }, { "code": "9A80", "title": "Hyphaema" }, { "code": "9A96.Z", "title": "Anterior uveitis, unspecified" }, { "code": "9B65.Z", "title": "Posterior uveitis, unspecified" }, { "code": "9C20.Z", "title": "Panuveitis, unspecified" }, { "code": "9B66.Z", "title": "Intermediate uveitis, unspecified" }, { "code": "1C1G.12", "title": "Ophthalmic Lyme borreliosis" } ]	=== ICD-11 CODES FOUND === [9A8Z] Disorders of the anterior chamber, unspecified Also known as: Disorders of the anterior chamber, unspecified [9A96.3] Primary anterior uveitis Definition: This refers to primary inflammation of the uvea. The uvea consists of the middle, pigmented, vascular structures of the eye and includes the iris, ciliary body, and choroid. Also known as: Primary anterior uveitis \| anterior chamber cell [9A90.0] Disorders of chamber angle Definition: This refers to the change of tissue to a lower or less functionally active form, of the fluid-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium. Also known as: Disorders of chamber angle [9A8Y] Other specified disorders of the anterior chamber Also known as: Other specified disorders of the anterior chamber [9A80] Hyphaema Also known as: Hyphaema \| blood in anterior chamber \| haemorrhage of anterior chamber of eye \| hyphaemia \| anterior chamber haemorrhage Excludes: traumatic hyphaema [9A96.Z] Anterior uveitis, unspecified Also known as: Anterior uveitis, unspecified \| Anterior uveitis \| uveokeratitis \| keratouveitis \| iridocyclitis [9B65.Z] Posterior uveitis, unspecified Also known as: Posterior uveitis, unspecified \| Posterior uveitis \| Chorioretinitis \| Retinochoroiditis \| Choroiditis [9C20.Z] Panuveitis, unspecified Also known as: Panuveitis, unspecified \| Panuveitis \| diffuse uveitis [9B66.Z] Intermediate uveitis, unspecified Also known as: Intermediate uveitis, unspecified \| Intermediate uveitis [1C1G.12] Ophthalmic Lyme borreliosis Also known as: Ophthalmic Lyme borreliosis \| Lyme oculopathy \| Borrelia burgdorferi dacryoadenitis \| Borrelia burgdorferi orbital myositis \| Lyme iridocyclitis === GRAPH WALKS === --- Walk 1 --- [9A8Z] Disorders of the anterior chamber, unspecified --PARENT--> [?] Disorders of the anterior chamber --CHILD--> [9A81] Parasites in the anterior chamber of the eye --- Walk 2 --- [9A8Z] Disorders of the anterior chamber, unspecified --PARENT--> [?] Disorders of the anterior chamber --RELATED_TO--> [?] Retained foreign body in anterior chamber of eye --- Walk 3 --- [9A96.3] Primary anterior uveitis Def: This refers to primary inflammation of the uvea. The uvea consists of the middle, pigmented, vascular structures of the eye and includes the iris, ciliary body, and choroid.... --PARENT--> [9A96] Anterior uveitis --CHILD--> [9A96.1] Anterior uveitis associated with systemic conditions --- Walk 4 --- [9A96.3] Primary anterior uveitis Def: This refers to primary inflammation of the uvea. The uvea consists of the middle, pigmented, vascular structures of the eye and includes the iris, ciliary body, and choroid.... --PARENT--> [9A96] Anterior uveitis --CHILD--> [9A96.1] Anterior uveitis associated with systemic conditions --- Walk 5 --- [9A90.0] Disorders of chamber angle Def: This refers to the change of tissue to a lower or less functionally active form, of the fluid-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium.... --PARENT--> [9A90] Degeneration of iris or ciliary body --CHILD--> [9A90.0] Disorders of chamber angle Def: This refers to the change of tissue to a lower or less functionally active form, of the fluid-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium.... --- Walk 6 --- [9A90.0] Disorders of chamber angle Def: This refers to the change of tissue to a lower or less functionally active form, of the fluid-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium.... --PARENT--> [9A90] Degeneration of iris or ciliary body --CHILD--> [9A90.0] Disorders of chamber angle Def: This refers to the change of tissue to a lower or less functionally active form, of the fluid-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium....	[ "[9A8Z] Disorders of the anterior chamber, unspecified\n --PARENT--> [?] Disorders of the anterior chamber\n --CHILD--> [9A81] Parasites in the anterior chamber of the eye", "[9A8Z] Disorders of the anterior chamber, unspecified\n --PARENT--> [?] Disorders of the anterior chamber\n --RELATED_TO--> [?] Retained foreign body in anterior chamber of eye", "[9A96.3] Primary anterior uveitis\n Def: This refers to primary inflammation of the uvea. The uvea consists of the middle, pigmented, vascular structures of the eye and includes the iris, ciliary body, and choroid....\n --PARENT--> [9A96] Anterior uveitis\n --CHILD--> [9A96.1] Anterior uveitis associated with systemic conditions", "[9A96.3] Primary anterior uveitis\n Def: This refers to primary inflammation of the uvea. The uvea consists of the middle, pigmented, vascular structures of the eye and includes the iris, ciliary body, and choroid....\n --PARENT--> [9A96] Anterior uveitis\n --CHILD--> [9A96.1] Anterior uveitis associated with systemic conditions", "[9A90.0] Disorders of chamber angle\n Def: This refers to the change of tissue to a lower or less functionally active form, of the fluid-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium....\n --PARENT--> [9A90] Degeneration of iris or ciliary body\n --CHILD--> [9A90.0] Disorders of chamber angle\n Def: This refers to the change of tissue to a lower or less functionally active form, of the fluid-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium....", "[9A90.0] Disorders of chamber angle\n Def: This refers to the change of tissue to a lower or less functionally active form, of the fluid-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium....\n --PARENT--> [9A90] Degeneration of iris or ciliary body\n --CHILD--> [9A90.0] Disorders of chamber angle\n Def: This refers to the change of tissue to a lower or less functionally active form, of the fluid-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium...." ]	9A8Z	Disorders of the anterior chamber, unspecified	[ { "from_icd11": "9A8Z", "icd10_code": "H211X2", "icd10_title": "Other vascular disorders of iris and ciliary body, left eye" }, { "from_icd11": "9A8Z", "icd10_code": "H211X9", "icd10_title": "Other vascular disorders of iris and ciliary body, unspecified eye" }, { "from_icd11": "9A8Z", "icd10_code": "H219", "icd10_title": "Unspecified disorder of iris and ciliary body" }, { "from_icd11": "9A8Z", "icd10_code": "H211", "icd10_title": "Other vascular disorders of iris and ciliary body" }, { "from_icd11": "9A80", "icd10_code": "H2102", "icd10_title": "Hyphema, left eye" }, { "from_icd11": "9A80", "icd10_code": "H2101", "icd10_title": "Hyphema, right eye" }, { "from_icd11": "9A80", "icd10_code": "H2100", "icd10_title": "Hyphema, unspecified eye" }, { "from_icd11": "9A80", "icd10_code": "H2103", "icd10_title": "Hyphema, bilateral" }, { "from_icd11": "9A80", "icd10_code": "H210", "icd10_title": "Hyphema" }, { "from_icd11": "9A96.Z", "icd10_code": "H20051", "icd10_title": "Hypopyon, right eye" }, { "from_icd11": "9A96.Z", "icd10_code": "H2012", "icd10_title": "Chronic iridocyclitis, left eye" }, { "from_icd11": "9A96.Z", "icd10_code": "H2000", "icd10_title": "Unspecified acute and subacute iridocyclitis" }, { "from_icd11": "9A96.Z", "icd10_code": "H20052", "icd10_title": "Hypopyon, left eye" }, { "from_icd11": "9A96.Z", "icd10_code": "H2013", "icd10_title": "Chronic iridocyclitis, bilateral" }, { "from_icd11": "9A96.Z", "icd10_code": "H2010", "icd10_title": "Chronic iridocyclitis, unspecified eye" } ]	H211X2	Other vascular disorders of iris and ciliary body, left eye

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