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The patient presented herself at the Division of Rare Diseases for further etiologic examinations. During the first clinical evaluation a very severe attack could be observed. The patient became unconscious and her blood pressure rapidly rose to 230/100 mmHg, with a regular heart rate of 160 to 180/minute . Moreover, focal muscle twitching appeared on her left face, and excessive lacrimation and flushing could also be observed. Because of her unstable clinical condition she was immediately admitted to our ICU. Before the administration of additional medications, the patient’s severe clinical condition improved significantly without assistance. By the end of the paroxysm the frequency of sinus rhythm decreased to 90/minute and her blood pressure was also normalized. After the crisis, no signs of arrhythmia or long-standing neurological defects could be observed. No other significant clinical abnormalities could be found during further clinical examinations. Afterwards, during the first week of hospitalization she had attacks two to four times a day. These paroxysmal hypertensive crises lasted for three to five minutes and then disappeared spontaneously without any medical interventions. Between the paroxysms the patient was asymptomatic. Because of the repeated attacks, a combination of alpha- and beta-adrenoceptor blockers was given, which was able to lower her blood pressure and heart rate during the paroxysms, but not the frequency of the attacks. Holter electrocardiography recordings and twelve-lead surface electrocardiograms revealed sudden onset of episodic sinus tachycardia without any signs of further atrial or ventricular arrhythmias . Renal Doppler ultrasound examination was performed to exclude renovascular disease. It revealed physiological blood flow in both renal arteries, with no significant difference regarding resistive indices (0.67 versus 0.7, respectively). Although previous examinations were not able to prove any endocrinological background of the paroxysms, a repeated laboratory testing of pheochromocytoma and carcinoid was performed. Laboratory results of our patient are shown in Table 2 . An elevated serum chromogranin A level appeared, but it proved to be a false positive result due to concomitant proton-pump inhibitor (PPI) therapy (after cessation of the PPI, the chromogranin A level was in the normal range). Surprisingly, an adenoma could be detected in the left adrenal gland during computed tomography. Due to the repeated severe clinical symptoms, we were obliged to start treatment of the pheochromocytoma; thus, a cardio-selective beta-blocker (bisoprolol 5 mg twice daily) in combination with an alpha-adrenoceptor-blocker (doxazosine 4 mg once daily) was initiated. During this time, urine concentrations of 5-hydroxyindoleacetic acid (5-HIAA), metanephrine, normetanephrine and dopamine were found to be normal. Although, we measured slightly elevated serum concentrations of noradrenalin and dopamine during an attack, the levels did not fulfill the criteria for pheochromocytoma (Table 3 ). To ensure the safe exclusion of pheochromocytoma, an iodine-131-metaiodobenzylguanidine ( 131 I-MIBG) scan was also performed, which did not reveal any abnormalities relating to adrenal gland dysfunction . Furthermore, hyperaldosteronism as a very rare cause of paroxysmal hypertension could also be excluded. Further laboratory tests helped to exclude any hormonal abnormalities; thus the aforementioned adrenal gland adenoma was regarded as an ‘incidentaloma’. Thyroid laboratory tests showed the effective hormonal substitution of hypothyroidism secondary to the previous thyroidectomy. After excluding the possibility of endocrine disorders we focused on anxiolytic medication. For this reason psychiatric examination was performed and alprazolam was re-administered in a daily dose of 1 mg (0.5 mg twice daily). We could demonstrate an immediate clinical improvement; furthermore, the daily dose of alpha- and beta-blockers could also be decreased. During the administration of alprazolam at a daily dose of 1 mg, sleepiness and fatigue occurred, therefore we decreased the daily dose to 0.5 mg. Consequently, the paroxysmal increase in blood pressure reappeared so further therapy of 1 mg was necessary for maintenance. After a four-week period on the ICU, the patient was discharged though still with mild symptoms but with an improved quality of life. The systolic and diastolic blood pressures and heart rate collected after the discharge of our patient were inserted into the manuscript (Table 4 ). Figure 1 During the first clinical evaluation of the patient a very severe attack could be observed. The patient became unconscious, her systolic blood pressure rapidly rose above 230 mmHg. Similar trends were observed during the repeated paroxysms. BP: blood pressure. Figure 2 Holter electrocardiogram revealed a paroxysmal sinus tachycardia during the attack. No other atrial arrhythmias or life-threatening ventricular arrhythmias (ventricular tachycardia and fibrillation) were observed. Table 2 Laboratory data of the patient Laboratory variables Results Laboratory references Na + 144 133 to146 mmol/L K + 4.2 3.5 to 5.3 mmol/L Cl − 107 99 to 111 mmol/L Ca 2+ (total) 2.33 2.1 to 2.6 mmol/L Blood urea nitrogen 4.2 3.6 to 7.2 mmol/L Creatinine 66 44 to 97 μmol/L eGFR (EPI) 89 > 90 mL/minute/1.73 m 2 Glucose 6 3.6 to 6.0 mmol/L HgbA1C 7.8 4.2 to 6.1 % Albumin 42 35 to 52 g/L Total protein 63 60 to 80 g/L AST 26 < 40 U/L ALT 38 < 40 U/L LDH 194 135 to 220 U/L Alkaline phosphatase 74 40 to 115 U/L Amylase 23 < 100 U/L Lipase 17 < 70 U/L CRP 1.9 < 4.6 mg/L WBC 8.66 4.8 to 10.8 Giga/L RBC 3.97 4.2 to 5.4 Tera/L Hemoglobin 123 115 to 150 g/L Hematocrit 0.35 0.35 to 0.47 Platelet 277 150 to 400 Giga/L MCV 88.9 80 to 99 fL MCH 31 27 to 31 pg ALT: alanine transaminase; AST: aspartate transaminase; CRP: C reactive protein; EPI: epidemiology collaboration; GFR: glomerular filtration rate; HgbA1c: hemoglobin A1c; LDH: lactate dehydrogenase; MCH: mean corpuscular hemoglobin; MCH: mean corpuscular hemoglobin; MCV: mean corpuscular volume; MCH: mean corpuscular hemoglobin; MCH: mean corpuscular hemoglobin; RBC: red blood cell; WBC: white blood cell. Table 3 Hormone levels of the studied patient Hormonal variables Results Laboratory references Plasma samples Thyroid stimulating hormone 2.3 0.3 to 4.2 mU/L ACTH (8 hours) < 19 < 75 ng/L Cortisol 245.8 138 to 690 nmol/L Plasma renin Non- detectable 0.5 to 1.9 x hrs μg/L Plasma aldosterone 54.1 28 to 291 pmol/l Chromogranin A (with PPI) 875.4 20 to 100 μg/L Chromogranin A (without PPI) 48.3 20 to 100 μg/L Plasma samples during paroxysm Adrenaline 0.32 < 0.41 nmol/L Noradrenaline 3.37 0.37 to 2.6 nmol/L Dopamine 2.73 < 0.88 nmol/L Urine samples (24-hour collection) Adrenaline 16 3 to109 nmol/die Noradrenaline 187 89 to 473 nmol/die Dopamine 2,171 424 to 1,612 nmol/die Homovanillic acid 31 9.1 to 33.8 μmol/die Vanillyl mandelic acid 31 < 34 μmol/die 5-HIAA 23 3.7 to 42.9 μmol/die Metanephrines 356 375 to 1,506 nmol/die Normetanephrines 1,340 573 to 1,932 nmol/die 3-metoxytyramine 702 < 900 nmol/die 5-HIAA: 5-hydroxyindoleacetic acid; ACTH: adrenocorticotropic hormone; PPI: proton-pump inhibitor. Figure 3 For 131 I- MIBG acquisition, 40 MBq radiopharmaceutical was injected. A whole body (4 cm/minute) and abdominal SPECT/CT acquisition were performed on MEDISO AnyScan SC system (Budapest, Hungary) 72 hours after the injection. SPECT parameters were: 1 minute/projection, 64 views, matrix size 64 × 64. A 16-slice CT was used, with 120 mAs and 120 kV abdominal filter. For the SPECT reconstruction OSEM method was performed. None of the adrenal regions showed abnormal focal uptake. MIBG: metaiodobenzylguanidine, SPECT: Single-Photon Emission Computed Tomography, CT: computed tomography, OSEM: Ordered Subset Expectation Maximization. Table 4 Data on blood pressure and heart rate obtained after the discharge of our patient revealed the lack of reoccurrence of the paroxysms Maximum Minimum Mean BP sys (mmHg) 145 133 140 BP dia (mmHg) 85 79 82 Heart rate (beats/minute) 93 65 74 BP: blood pressure; dia: diastolic; sys: systolic.
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[
{
"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 CLASSIFICATION ═══
【1. Diseases of the blood or blood-forming organs, unspecified (3C0Z)】
Synonyms: Blood, lymph or spleen disease | blood condition NOS | blood disorder NOS | bone marrow disease NOS | haematologic disease NOS
Hierarchy: Diseases of the blood or blood-forming organs (03) → Diseases of the blood or blood-forming organs, unspecified
【2. Haematuria, unspecified (MF50.4Z)】
Synonyms: Haematuria | blood in urine | urinary blood | haematuria NOS | urinary tract haemorrhage NOS
Hierarchy: Symptoms, signs or clinical findings involving the urinary system → Abnormal micturition (MF50) → Haematuria (MF50.4) → Haematuria, unspecified
【3. Finding of cocaine in blood (MA12.1)】
Synonyms: cocaine in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of cocaine in blood
【4. Finding of steroid agent in blood (MA12.4)】
Synonyms: steroid in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of steroid agent in blood
【5. Finding of hallucinogen in blood (MA12.2)】
Synonyms: hallucinogen in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of hallucinogen in blood
|
3C0Z
|
Diseases of the blood or blood-forming organs, unspecified
|
Brain magnetic resonance imaging (MRI) revealed bilateral paraventricular, corona radiata, semioval centre, and right subcortex fluid-attenuated inversion recovery (FLAIR) hyperintensities. Contrast-enhanced scans also showed patchy and linear perivascular radial gadolinium enhancement in these areas . No abnormal findings were detected on nuclear MRI scans of the cervical spinal cord. While urinary incontinence, one of the patient’s symptoms, may be associated with lumbar spinal cord disease, a nuclear MRI of the lumbar spinal cord was not performed. Electroencephalography showed low to medium amplitude alpha waves of 10–11 Hz, and alpha rhythms were observed in both occipital leads when the eyes were open and closed, with poor amplitude and rhythm modulation and basic symmetry bilaterally. When awake and quiet, slightly more medium-wave amplitude 4–7 Hz theta waves were seen in bilateral leads, with significant anterior head, mixed with few low-wave amplitude 14–25 Hz beta waves and basic symmetry bilaterally . The CSF pressure was normal, with leucocytosis of 94 × 106 /L (reference range: < 8 × 106 /L, proportion of neutrophils 22%), elevated protein (0.85 g/L), and normal glucose (2.95 mmol/L). Due to the patient’s psychiatric symptoms, herpes simplex virus (HSV) encephalitis was also considered. However, a CSF polymerase chain reaction (PCR) assay was negative for HSV deoxyribonucleic acid (DNA). Other viral PCRs were performed, including those for varicellovirus, cytomegalovirus, roseolovirus, lymphocryptovirus, and rhadinovirus; the results were all negative. While the patient’s CSF was positive for oligoclonal bands (OCB), his serum was negative for the same. The pattern of OCB was 2. Macroeconomic DNA detection of pathogenic microorganisms in the CSF were negative. Using a cell-based indirect immunofluorescence test , anti-NMDAR antibodies were detected in the CSF (1:10). The microscope used is EUROStar III Plus fluorescence microscope. The camera model for screen imaging is LU375C. The filter system is FITC. The magnification of the microscope eyepiece is 10 times, and the objective lens is 20 times. Acquisition Software is EUROLABOFFICE. The resolution of the camera is 2048 × 1536 pixels. The resolution of the acquired image is 96 dpi. The titre changed to 1:32 when the test was repeated after eight days. Unfortunately, the patient had the test was performed at another hospital and did not provide original pictures. The serum was consistently negative for anti-NMDAR antibodies. Because contrast-enhanced scans showed patchy and linear perivascular gadolinium enhancement in the areas shown in Fig. 1 , a combined GFAP antibody was considered a possibility. Cell-based assays detected anti-GFAP antibodies in the CSF (1:32) , leading to the diagnosis of autoimmune encephalitis. Anti-GFAP antibodies in the CSF were validated using cell-based assays. The CSF was positive for GFAP-IgG (a-c, 1:32). Green color indicates successful transfection of a GFP-tagged plasmid encoding GFAP with a green fluorescent tag, and red represents antibody signals. Specimens were incubated with an Alexa Fluor 546 secondary antibody against human IgG for 1 h at room temperature with red fluorescence. GFAP-IgG was tested by a cell-based assay (CBA). HEK293 cells were co-transfected with full-length human GFAP and pcDNA3.1-EGFP. Thirty-six hours after transfection, the HEK293T cells were fixed with 4% paraformaldehyde for 20 min and permeabilized with 0.1% Triton X-100 in phosphate-buffered saline (PBS) for 20 min. Cells were incubated with the patient's CSF for 2 h and then immunolabeled with an Alexa Fluor 546 secondary antibody against human IgG for 1 h at room temperature. First, use the green light channel to observe cell transfection. If the plasmid transfection is successful, the cell can be observed to have green fluorescence. Then observe with the red channel, if the cell membrane of the successfully transfected cells in the sample well has a more obvious red fluorescence, the sample is positive for the antibody; if the cell membrane of the successfully transfected cells in the sample well does not have a more obvious red fluorescence or the cells that are not successfully transfected have red fluorescence, the sample is negative. The results can be further confirmed by overlapping the green and red channels. Two independent masked assessors classified each sample as positive or negative based on immunofluorescence intensity in direct comparison with non-transfected cells and control samples. Once confirmed, the positive specimens were then serially diluted from 1:10 to 1:1000 to determine the titers. Antibody titer is the highest dilution at which a specimen is tested for a positive reaction after a series of dilutions. The detection of GFAP antibody, cerebrospinal fluid routine non-dilution operation, and then for the positive samples, is the sampling end point dilution (end-point dilution) to determine the final titer. According to the fluorescence intensity of the test, the positive sample is further diluted at 1:10,1:100, and 1:1000 and then detected by the CBA method. Suppose the sample does not detect a positive signal when diluted at 1:100, and a positive signal is detected when diluted at 1:10. In that case, the titer is determined to be 1:10 or 1:32 (the specific situation is judged by the fluorescence intensity that can be seen when diluted at 1:10. If the fluorescence intensity is weak at 1:10 dilution detection, the titer is determined to be 1:10, and if the signal is strong at 1:10 dilution detection, the titer is determined to be 1:32). Images were acquired using a Leica DM IL LED fluorescence microscope. The filter system is L5 ET,s. The magnification of the microscope eyepiece is 10 times, and the objective lens is 20 times. The resolution of the camera is 3072 × 2048 pixels. The resolution of the acquired image is 96 dpi. However, the serum was negative for anti-GFAP antibodies. Other autoimmune antibodies, including anti-α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid 1, anti-α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid 2, anti-gamma-aminobutyric acid β, leucine-rich glioma-inactivated 1, anti-contactin-associated protein-like 2, and anti-glutamic acid decarboxylase 65-kilodalton isoform, were absent in both the CSF and serum. Other abnormal serum indicators were increased anticardiolipin antibodies, antibodies against β2-glycoprotein, human leukocyte antigen-B27, and anti-polymyositis/scleroderma (PM/Scl). A chest computed tomography scan was negative for malignancy. Fig. 1 Magnetic resonance imaging of the patient. Magnetic resonance imaging of the patient. T1 weighted image revealed a low signal in the lesion area ( a - b ). T2-weighted fluid-attenuated inversion recovery (FLAIR) showed elevated signals within bilateral paraventricular, radiate corona, semioval center, and right subcortex ( c - f ), contrast-enhanced scans showed patchy and linear perivascular radial gadolinium enhancement in the above areas ( g - j ) Fig. 2 Electroencephalography of the patient. Electroencephalography ( a - b ) showed that low to medium wave amplitude 10-11 Hz alpha waves and alpha rhythm were seen in both occipital leads when the eyes were awake and closed, with poor amplitude and rhythm modulation and basic symmetry bilaterally. When awake and quiet, slightly more medium-wave amplitude 4-7 Hz theta waves were seen in bilateral leads, with significant anterior head, mixed with a small amount of low-wave amplitude 14-25 Hz beta waves, with basic symmetry bilaterally Fig. 3 NMDAR antibodies in CSF. Anti-N-methyl-D-aspartate receptor (NMDAR) antibodies in CSF validated by a cell-based indirect immunofluorescence test (1:10) Fig. 4 GFAPα-IgG in CSF. GFAPα-IgG test results by GFAP-transfected HEK293 cell-based immunofluorescence assay. A HEK293 cells expressing green fluorescent protein (GFP)-tagged GFAP (green). B HEK293 cells immunostained with human IgG (red if positive): C Merged images (yellow). Images were acquired using a Leica DM IL LED fluorescence microscope. Scale bar = 50 um
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[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Maternal care for other specified fetal problems (JA86.Y)】
Synonyms: Maternal care for other isoimmunization | Isoimmunization NOS | maternal antibodies NOS | pregnancy management affected by incompatibility of blood groups NOS | Maternal care for ABO isoimmunisation
Hierarchy: Pregnancy, childbirth or the puerperium (18) → Maternal care related to the fetus, amniotic cavity or possible delivery problems → Maternal care for other fetal problems (JA86) → Maternal care for other specified fetal problems
【2. Antisocial behaviour (MB23.1)】
Definition: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated.
Synonyms: Child or adolescent antisocial behaviour
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Mental or behavioural symptoms, signs or clinical findings → Symptoms or signs involving appearance or behaviour (MB23) → Antisocial behaviour
【3. Coagulation defects, unspecified (3B4Z)】
Synonyms: blood clotting disturbance | blood clotting defect | blood clotting factor deficiency | clotting abnormality | clotting disorder
Hierarchy: Diseases of the blood or blood-forming organs (03) → Coagulation defects, purpura or other haemorrhagic or related conditions → Coagulation defects → Coagulation defects, unspecified
【4. Antiphospholipid syndrome, unspecified (4A45.Z)】
Synonyms: Antiphospholipid syndrome | Hughes syndrome | Anticardiolipin syndrome
Hierarchy: Diseases of the immune system (04) → Nonorgan specific systemic autoimmune disorders → Antiphospholipid syndrome (4A45) → Antiphospholipid syndrome, unspecified
【5. Other specified overlap non-organ specific systemic autoimmune disease (4A43.Y)】
Synonyms: Antisynthetase syndrome | Reynolds syndrome | Syndromic multisystem autoimmune disease due to ITCH deficiency | Eosinophilia myalgia syndrome | EMS - [eosinophilia myalgia syndrome]
Hierarchy: Diseases of the immune system (04) → Nonorgan specific systemic autoimmune disorders → Overlap or undifferentiated nonorgan specific systemic autoimmune disease (4A43) → Other specified overlap non-organ specific systemic autoimmune disease
|
JA86.Y
|
Maternal care for other specified fetal problems
|
A 42-year-old female patient demonstrated severe OCS with washing and showering compulsions for the past six years. The formal OCD diagnosis was confirmed by SCID-I. The patient’s scores on Y-BOCS and OCI-R were 28 and 30 points, respectively. The patient’s somatic history included migraine with aura. The patient had never experienced focal neurological symptoms, nor did she report experiencing fatigue. Her mother suffered from MS and OCD-like symptoms. The clinical MRI revealed eight single T2w/FLAIR patchy hyperintense lesions in the bilateral periventricular and subcortical white matter as well as one lesion in the pons. Periventricular and brainstem lesions are compatible with demyelinating MS lesions . CSF analyses identified an increased white blood cell count (17 cells/microliter; reference < 5 cells/microliter), intrathecal immunoglobulin (Ig) G and IgM synthesis, as well as CSF-specific oligoclonal bands. The MRZ reaction was positive. All well-characterized antibodies against cell surface, intracellular, and glial antigens were negative . Tissue-based assays detected no novel central nervous system (CNS) autoantibodies. Vitamin B12 and folic acid were within the normal range, while Vitamin D was also within the normal range (31.1 ng/ml; ref. > 20.0 ng/ml), but was substituted in order to achieve highly normal vitamin D levels. Neurotransmitter measurements identified reduced serotonin concentrations of 0.014 μM in CSF (reference range 0.82 ± 0.48 μM ). In addition, the sarcoidosis parameter neopterin was strikingly elevated. In the CT of the chest, no sarcoidosis-associated abnormalities were identified. Further diagnostics with electrophysiological examinations and an MRI of the spine revealed no pathologies (Table 1 ). In the absence of neurological attacks with focal neurological symptoms and no MRI-documented dissemination in time, radiologically isolated syndrome (RIS) was diagnosed because of inflammatory MRI and CSF changes . The patient decided against immunomodulatory treatment, so watchful waiting was initiated. Guideline-based treatment for OCS with 100 mg sertraline (plasma levels of 22 ng/ml, reference: 10–50 ng/ml; higher doses up to 200 mg/day were not tolerated due restlessness, sleep disturbance, and dizziness) and inpatient cognitive behavioral therapy for approximately 10 weeks with exposure and response management resulted in only partial improvement of OCS. The patient’s Y-BOCS score was reduced to 23 (-18%), while her OCI-R score was reduced to 29 (-3%) points. An MRI follow-up after approximately six months showed no subclinical progression of the brain T2 lesion load. Fig. 1 White matter magnetic resonance imaging lesions are marked with arrows. The automated magnetic resonance imaging analysis ( https://www.veobrain.com/?page=veomorph ) did not detect any atrophic changes. The low serotonin levels in cerebrospinal fluid are shown in comparison to known reference values from controls . CSF cerebrospinal fluid, GM grey matter, L left, R right Table 1 Full diagnostic findings Initial diagnostic findings Psychometric scores Y-BOCS 28 OCI-R 30 Serum antibodies, immunological markers and serologies Anti-thyroid antibodies (against TPO, TG and TSH-receptor) Negative ANAs ( on HEp-2 cells ), ANCAs ( on EthOH- /formalin-fixed neutrophils ), APAs ANAs negative, ANCA (EthOH-fixiert, IgG, 1:10): (( +)c), APAs negative Complement factors (C3, C4) Normal IgG, IgM and IgA levels Normal CRP < 3.0 mg/L (ref.: < 5 mg/L) Anti-streptolysin-O Normal Anti-DNaseB Normal Rheumatoid factor Negative Serology for Lyme disease or lues Negative Paraneoplastic IgG antibodies against intracellular antigens Negative Well-characterized neuronal IgG cell surface antibodies Negative Anti-MOG/AQP4-IgG antibodies Negative Tissue based assay on unfixed murine brain tissue (Prof. Prüss) Negative Sarcoidosis parameters (IL-2-R, ACE, neopterin) Neopterin 31.3 nmol/L (ref.: < 10 nm/L), IL-2-R: 328 U/ml (ref. 158–623 U/ml), ACE: 56.4 U/L (ref.: 12–82 U/L) Serologies (CMV, EBV, HBV, HCV, HIV, tuberculosis) EBV positive (Anti-EBNA1-IgG: 18.98 (ref.: < 0.8)), CMV, HBV, HCV, HIV negative Neurotransmitters and precursors from serum* Reduced citrate (25 µM; reference range: 100–150 µM), and elevated concentrations of aromatic amino acids tryptophan (169 µM; reference range 43–89 µM) and phenylalanine (150 µM; reference range 28–85 µM). All other values were normal Cerebrospinal fluid White blood cell count 17/µL (ref.: < 5/µL) Protein concentration 304 mg/L (ref.: < 450 mg/L) Albumin quotient 3.1 (ref.: < 6.5) IgG-index 2.72 (ref.: < 0.7) Oligoclonal bands in serum/CSF Negative/ Positive Well-characterized neuronal IgG cell surface antibodies Negative Local IgG/IgA/IgM synthesis IgG synthesis 76.6% , no IgA synthesis, IgM synthesis 21.2% (ref.: < 10%) MRZ Reaction Positive (ASI Measles-IgG 8.33 (ref.: < 1.5), ASI Rubella-IgG 3.7 (ref.: < 1.5), ASI VZV-IgG 3.6 (ref.: < 15) Tissue based assay on unfixed murine brain tissue (Prof. Prüss) Negative Neurotransmitters and precursors from CSF* Reduced citrate (25 µM; reference range 176 ± 50 µM), succinate (1.1 µM; reference range: 29 ± 5 µM), glutamate (3.5 µM; reference range: 33 ± 7 µM), serine (14 µM; reference range: 42 ± 15 µM), glutamine (113 µM; reference range: 440 ± 80 µM), threonine (12 µM; reference range: 28 ± 5 µM) and serotonin (0.014 µM; reference range 0.82 ± 0.48 µM), as well as low-normal dopamine (0.037 nM; reference range: 0.04–4.5 nM) and slightly elevated GABA . 5-hydroxyinolacetic acid (5-HIAA) concentration was normal (0.103 µM; reference range: 0.055–0.163 µM). All other values were also normal MRI of the neurocranium Visual inspection Periventricular accentuated medullary lesions supratentorial on both sides with involvement of the temporal lobe, which exceeds the age limit. In total, 8 small lesions, two of which two would be compatible with MS (in pons and periventricular) Automated morphometry Normal EEG Visual analyses No intermittent/ generalized slowing, no epileptic activity ICA Normal Electrophysiological investigations VEP Normal SEP Normal MEP Normal OCT Normal Corona (vaccination) status Three doses of a COVID-19 vaccine, no infection ↑ means increased. *The following neurometabolites were measured in serum and CSF: Homocysteine, Cysteine, Cysteamine, Cystathionine, Methionine, Glutathione, Methionine sulfoxide, S-adenosylmethionine, S-adenosylhomocysteine, Creatinine, Argininosuccinic acid, Taurine, Hypotaurine, Homotaurine, Lanthionine, 3-Mercaptopyruvate, Dihydrofolate, 5- Methytetrahydrofolate, Tetrahydrofolate, 5,10-methylene-tetrahydrofolate, Cysteinylglycine,alpha-ketoglutarate, Citrate, Itaconate, Lactate, Malate, Malonate, Methlymalonic acid, Succinate, 2-methylcitrate, Phosphoenolpyruvate, Adenosine, Glucose, Glyceraldehyde-3-phosphate, Glycine, Alanine, Serine, Proline, Valine, Leucine/isoleucine, Aspartic Acid, Lysine, Glutamic Acid, Methionine, Histidine, Arginine, Tryptophan, Tyrosine, Asparagine, Glutamine, Phenylalanine, Threonine, Serotonin, 5-hydroxyinolacetic acid, GABA (gamma-aminobutyric acid), Dopamine, Norepinephrine, Acetylcholine, Choline, and 2-Amino adipic acid ACE angiotensin converting enzyme, ANAs antinuclear antibodies, ANCAs anti-neutrophil cytoplasmic antibodies, MOG myelin oligodendrocyte glycoprotein, APAs antiphospholipid antibodies, AQP4 aquaporin-4, CMV cytomegalovirus, CRP C-reactive protein, CSF cerebrospinal fluid, EBV Epstein-Barr virus, EEG electroencephalography, HBV hepatitis B virus, HCV hepatitis C virus, ICA independent component analysis, IgA/G/M immunoglobulin A/M/G, IL-2-R interleukin-2 receptor, IRDA intermittent rhythmic delta activity, MEP motor evoked potentials, MRI magnetic resonance imaging, MRZ antibody indices against measles, rubella, and varicella zoster virus, OCI-R obsessive–compulsive inventory-revised, ref. reference, SEP somatosensory evoked potentials, TG thyroglobulin, TPO thyroid peroxidase, TSH thyroid-stimulating hormone, VEP visual evoked potential, VZV varicella zoster virus, WBC white blood cell, Y-BOCS Yale-Brown obsessive compulsive scale
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| 0.962891
|
sec[2]/p[0]
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en
| 0.999995
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38289491
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https://doi.org/10.1007/s00702-023-02737-z
|
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[
{
"code": "6B22.Z",
"title": "Olfactory reference disorder, unspecified"
},
{
"code": "MB26.03",
"title": "Delusion of reference"
},
{
"code": "6B22.1",
"title": "Olfactory reference disorder with poor to absent insight"
},
{
"code": "4B00.0Z",
"title": "Neutropaenia, unspecified"
},
{
"code": "3B63.1Z",
"title": "Acquired thrombocytosis, unspecified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Olfactory reference disorder, unspecified (6B22.Z)】
Synonyms: Olfactory reference disorder | Delusions of malodour
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Obsessive-compulsive or related disorders → Olfactory reference disorder (6B22) → Olfactory reference disorder, unspecified
【2. Delusion of reference (MB26.03)】
Definition: A delusion that events, objects, or other people in the person's immediate environment have a particular and unusual personal significance, usually of a negative or pejorative nature.
Hierarchy: Mental or behavioural symptoms, signs or clinical findings → Symptoms or signs involving content of thought (MB26) → Delusion (MB26.0) → Delusion of reference
【3. Olfactory reference disorder with poor to absent insight (6B22.1)】
Definition: All definitional requirements of olfactory reference disorder are met. Most or all of the time, the individual is convinced that the disorder-specific beliefs are true and cannot accept an alternative explanation for their experience. The lack of insight exhibited by the individual does not vary markedly as a function of anxiety level.
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Obsessive-compulsive or related disorders → Olfactory reference disorder (6B22) → Olfactory reference disorder with poor to absent insight
【4. Neutropaenia, unspecified (4B00.0Z)】
Synonyms: Neutropenia | Disorders with decreased neutrophil counts | neutropaenic disorder | neutrophil count below reference range | absence of neutrophils
Hierarchy: Immune system disorders involving white cell lineages → Disorders of neutrophil number (4B00) → Neutropenia (4B00.0) → Neutropaenia, unspecified
【5. Acquired thrombocytosis, unspecified (3B63.1Z)】
Synonyms: Acquired thrombocytosis | Idiopathic haemorrhagic thrombocythaemia | Essential thrombocythaemia | primary haemorrhagic thrombocythaemia | idiopathic thrombocythaemia
Hierarchy: Coagulation defects, purpura or other haemorrhagic or related conditions → Thrombocytosis (3B63) → Acquired thrombocytosis (3B63.1) → Acquired thrombocytosis, unspecified
|
6B22.Z
|
Olfactory reference disorder, unspecified
|
Septic arthritis of the shoulder is relatively rare . In this study, we report the first case of monoarthritis of the shoulder caused by E. cloacae in an immunocompetent patient, with no apparent risk factors. To better understand the characteristics of E. cloacae infected bones and joints in patients without recent post-traumatic or post-operative medical history, a PubMed search was conducted and a total of 13 cases with detailed information, summarized in Table 2 were identified [ 12 – 24 ]. The literature review indicated that, including our patient, the ratio of male/female among the 14 patients without a pertinent history of open procedure was 5:2. Ten of the patients (No.3–6, No.8, No.9, No.11–14) showed apparent risk factors including multifocal infection, immunosuppression, sepsis, or even organ failure. However, 3 of the reported patients (No.2/7/10) had septic osteoarthritis due to trauma or hematogenous seeding at the same site or nearing site several years ago, and they were asymptomatic until the current episodes. These cases remind us that those have undergone invasive procedure may have a subsequent osteoarticular infection, even if they have been asymptomatic after the procedure. Table 2 Summary of E. cloacae osteoarthritis in patients without post-traumatic or post-operative medical history No. Gender Age (years old) The site(s) of osteoarthritis Comorbidities Chief complaints/Symptoms Sample of microbiological test Pathogen (s) Antibiotic treatment Inflammatory markers on admission The isolated or cultured E. cloacae sensitive antibiotics Outcome 1 (our case) F 52 Right shoulder Had right and left surgical kidney stone removal in 7 and 15 years ago. Rotator cuff tear. 6-year history of right shoulder pain and restricted movement, worsened in the last month. Fever, sore throat. Burning sensation, swelling and redness around the right shoulder Joint aspiration E. Cloacae Levofloxacin (300 mg, IV, q12h, 5 days) → 0.5 g, po, qd, 11 days → 300 mg, IV, q12h, 9 days → 0.5 g, po, qd, 17 days WBC:12.7 × 10 9 /L, CRP:41.2 mg/dL, ESR:65 mm/h Details in Table 1 Recovery 2 M 2 Left knee Osteoarticular infection at the same site 2 years earlier (no organism was identified), and he was born via forcep delivery Limp and left knee pain and swelling for 2 weeks Joint aspiration and synovial tissue E. Cloacae Meropenem(20 mg/kg, IV, q8h, 3 weeks) → Sulfamethoxazole–Rimethoprim (4 mg/kg, q12h, po, 6 months) NA Meropenem, cefepime, ciprofloxacin and trimethoprim-sulphamethoxazole Recovery 3 F 36 Right shoulder HIV-positive, sickle cell anemia, latent tuberculosis infection Fever, weight loss, fatigue, 4-months history of right shoulder pain Joint aspiration and surgical specimens E. Cloacae and Mycobacterium tuberculosis Imipenem and Amikacin for 2 weeks, IV CRP:0 Imipenema, latomoxef, amikacin, pefloxacin and ciprofloxacin Recovery 4 M 14 Left sacroiliac joint Sepsis 1-day history of fever and hip pain aggravated by walking, ARDS happened on the third hospital day Blood E. Cloacae Vancomycin(4 g/day) and Ceftriaxone (4 g/day) → Ceftriaxone (4 g/day) and Amikacin (1.5 g/day) for 6 weeks WBC: 6.4 × 10 9 /L, ESR:12 mm/h, CRP:3.1 mg/dL Amikacin, aztreonam, ceftriaxone, ceftazidime, cefotaxime, ciprofloxacin, gentamicin, imipenem, piperacillin/tazobactam; Recovery 5 M 88 T10/T11 Long-term urinary catheter, malignancy Back pain, fever, rigors, weight loss Blood E. Cloacae Meropenum (IV, 3 weeks) and Ciprofloxacin (Long time) WBC: 10.06 × 10 9 /L, CRP: 227 mg/dL NA Failed to therapy 6 M 54 C3-C4 Meningitis and sepsis secondary to urinary tract infection after transrectal ultrasound and biopsies, he had a raised PSA level and acute renal failure 10-day history of headaches, dizziness, neck pain and altered sensation in his upper, limbs Blood E. Cloacae and Klebsiella oxytoca Ciprofloxacin and metronidazole for 5 days → Ceftriaxone (IV, 2 weeks) and a longer course of oral ciprofloxacin NA NA Recovery 7 M 57 leg MSSA infection at the same site after an open fracture 31 years ago. After successful treatment, the facture healed and he remained asymptomatic until the present episode. The implant material was removed many years ago 1-week history of leg pain, swelling and local tenderness but no inflammation of the overlying skin or draining fistula Bone E. Cloacae Garamycin (3 weeks) and Cefepime (6 weeks) NA NA Recovery 8 M 52 L5-S1 Had a extracorporeal shock wave lithotripsy due to right renal lithiasis and hydronephrosis Chills, shaking, high fever, back pain, restricted lumbar movements Blood and urine E. Cloacae Amikacin Indometacin (150 mg/day) for 1 week → Ceftriaxone (2 g, q12h, 1 week) → 1 g/day,3 months) WBC:18 × 10 9 /L, CRP:12.3 mg/dL ESR:110 mm/h Ceftriaxone and amikacin Recovery 9 M 47 Multiple joints Acute pancreatitis, multi-organ failure, ARDS, systemic fatty necrosis High fever, chills, low tension, tachycardia, painful erythematous nodules on the arms, thighs, ankles and fingers Blood and joints aspiration E. Cloacae Pefloxacin, metronidazole and amoxicillin→imipenem-cilastatin WBC: 5.2 × 10 9 /L, CRP:201 mg/dL NA Dead 10 M 50 Cervical spine Hypertension, arthritis, and a gunshot wound to the left chest and birdshot to the head and neck 20 years earlier that resulted in a seizure disorder, and the pellet had not been removed 4-day history of neck pain spreading to right temporal region, right shoulder, right lateral chest, and right upper back from the scapula to the midthoracic spine, mild dysphagia Blood E. Cloacae NA WBC:7.9 × 10 9 /L NA Recovery 11 M 10 weeks old Left proximal tibia Watery stools, malnourished and dehydration 5 weeks earlier. Intraosseous (IO) needle had been placed into the proximal left tibia and a permanent Silastic intravenous catheter had been inserted to start parenteral nutrition and antibiotics Desquamating dermatitis, erythematous nodules on the back skin, the left lower extremity was erythematous and indurated Blood, bone and the serosanguinous aspiration expressed by the IO needle Candida albicans and E. cloacae Ampicillin and cefotaxime(IV) → Ticarcilin-clavulanate(IV) → Fluconazole and aztreonam → amphotericin B and aztreonam NA NA Recovery 12 M 28 L4-L5 HIV-positive, intravenous heroin abused, hepatitis C 2-month history of severe low back pain, fever, night sweats, and weight loss. The lumbar spine was markedly tender with bilateral paravertebral muscle spasm Joint aspiration E. Cloacae Amikacin (1 g/day, intramuscular, 3 weeks) and pefloxacin (800 mg/day, IV, 3 weeks) → pefloxacin ((800 mg/day, po, 14 weeks) WBC:9.2 × 10 9 /L, ESR:50 mm/h Amikacin, pefloxacin, and trimethoprim-sulphamethoxazole Recovery 13 F 68 T8-T9 Gallstones with repeated hepatic colic for 3 years. Intravenous urography, and a barium enema had been done 2-week history of severe pain in the right hypochondrium that increased with motion, general malaise, anorexia, and dystermia. Fever after barium enema Blood and Joint aspiration E. Cloacae trimethoprim(160 mg, q12h, IV, 10 days) and sulphamethoxazole (800 mg, q12h, IV, 10 days) → (trimethoprim-sulphamethoxazole, po) WBC:5.9 × 10 9 /L, ESR:127 mm/h Gentamicin and trimethoprim-sulphamethoxazole Recovery 14 F Prematre neonate (28 weeks) Multiple joints Premature, hyaline membrane disease, sepsis. Umbilical artery catheter was inserted, continuous positive airway pressure using nasal prongs, intravenous nutrition Septic shock, cyanosis, tachypnea, grunting, erythematous left ankle, knee effusion, lost passive and active motion in hips Blood, joints aspiration (Hips, right knee, right ankle) and tip of the catheter E. cloacae (blood, joints aspiration and tip of the catheter) Klebsiella pneumonia (blood) Gentamicin (2 mg, q8h, IV, 6 days) and Methicillin (IV, 6 days) → Furadantin (2.5 mg, q6h, IV, 9 days) → Furadantin (2.5 mg, q6h, IV) and Nalidixic acid NA Furadantin and Nalidixic acid Recovery MSSA Methicillin-sensitive S. aureus, PSA Prostate-specific antigen, WBC White blood cell, CRP C-reactive protein, ESR Erythrocyte sedimentation rate, → From the former treatment changed to the latter, NA Not available
| 4.179688
| 0.606934
|
sec[2]/p[1]
|
en
| 0.999998
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33407223
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https://doi.org/10.1186/s12879-020-05699-9
|
[
"cloacae",
"recovery",
"blood",
"pain",
"aspiration",
"shoulder",
"years",
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[
{
"code": "LB17.2",
"title": "Persistent cloaca"
},
{
"code": "GC04.19",
"title": "Combined urinary and rectal fistula including cloaca with severe scar or extensive tissue loss"
},
{
"code": "6B80.2",
"title": "Anorexia Nervosa in recovery with normal body weight"
},
{
"code": "3C0Z",
"title": "Diseases of the blood or blood-forming organs, unspecified"
},
{
"code": "MF50.4Z",
"title": "Haematuria, unspecified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Persistent cloaca (LB17.2)】
Definition: A congenital anomaly in which the intestinal, urinary, and reproductive ducts open into a common cavity, a result of the failure of the urorectal septum to form during prenatal development. They occur exclusively in girls and comprise the most complex defect in the spectrum of anorectal malformations.
Synonyms: anal and urogenital canal fusion | anal fusion | Cloaca NOS
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the digestive tract → Structural developmental anomalies of anal canal (LB17) → Persistent cloaca
【2. Combined urinary and rectal fistula including cloaca with severe scar or extensive tissue loss (GC04.19)】
Definition: A condition characterised by the presence of extensive amounts of fibrous tissue (fibrosis) that have replaced normal tissue associated with an abnormal connection or passageway between the rectum, including cloaca, and a location within the urinary system.
Synonyms: Combined urinary and rectal fistula with severe scar or extensive tissue loss
Hierarchy: Certain specified diseases of urinary system → Fistula of the genitourinary tract (GC04) → Fistulae involving female genital tract (GC04.1) → Combined urinary and rectal fistula including cloaca with severe scar or extensive tissue loss
【3. Anorexia Nervosa in recovery with normal body weight (6B80.2)】
Definition: Among individuals who are recovering from Anorexia Nervosa and whose body weight is more than 18.5 kg/m2 for adults or over the fifth percentile for BMI-for-age for children and adolescents, the diagnosis should be retained until a full and lasting recovery is achieved, as indicated by the maintenance of a healthy weight and the cessation of behaviours aimed at reducing body weight independent of ...
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Feeding or eating disorders → Anorexia Nervosa (6B80) → Anorexia Nervosa in recovery with normal body weight
【4. Diseases of the blood or blood-forming organs, unspecified (3C0Z)】
Synonyms: Blood, lymph or spleen disease | blood condition NOS | blood disorder NOS | bone marrow disease NOS | haematologic disease NOS
Hierarchy: Diseases of the blood or blood-forming organs (03) → Diseases of the blood or blood-forming organs, unspecified
【5. Haematuria, unspecified (MF50.4Z)】
Synonyms: Haematuria | blood in urine | urinary blood | haematuria NOS | urinary tract haemorrhage NOS
Hierarchy: Symptoms, signs or clinical findings involving the urinary system → Abnormal micturition (MF50) → Haematuria (MF50.4) → Haematuria, unspecified
|
LB17.2
|
Persistent cloaca
|
A 45-year-old man was admitted to the Neurology Department at the local hospital in January 2017 due to verbal communication disturbances, nausea, vomiting, headaches, disequilibrium and a walking problem. Computer tomography (CT) of the head revealed small bilateral calcifications in the ventricles as well as reduced density of white matter. A lumbar puncture (LP) was performed. Analysis of the cerebrospinal fluid (CSF) yielded the following findings: protein 33 mg/dL, glucose 66 mg/dL, pleocytosis 70/μL – neutrophils (70%). Neuroinfection was considered, and empirical antibiotic therapy with ceftazidime was given to the patient, who was then transferred to the Department of Infectious Diseases. In the absence of the desired effect, the antibiotic was changed to ceftriaxone, and acyclovir and steroids (a daily dose of 12 mg dexamethasone for 10 days) were added. After taking these drugs, the patient reported a slight improvement and headache reduction, which were probably due to the action of the steroids. No antibody tests were carried out to determine the cause of infectious disease. The patient was discharged home. One week later, epileptic seizures appeared, and the patient was admitted to the Department of Infectious Diseases in another hospital. A head CT scan was performed, yielding results comparable to those of the previous scan. Another LP was also performed, with the following CSF findings: protein 302 mg/dL, glucose 30 mg/dL, pleocytosis 160/μL - neutrophils 80%, and negative cerebrospinal fluid culture. Blood culture, urine culture and respiratory tract culture were also negative. The liver enzyme levels were elevated, and slight hyponatraemia (up to 130 mmol/l) was observed, but the C-reactive protein (CRP) level was normal. Brain MRI revealed slight enhancement of the lining of the leptomeninx , mainly the ventricular ependyma, after administration of gadolinium contrast. According to the CSF findings, tuberculosis infection was the most likely cause of the symptoms, but the Bactec and QuantiFERON tests were negative. Human immunodeficiency virus (HIV), cytomegalovirus (CMV), Lyme disease, hepatitis B (HBV), and hepatitis C (HCV) infections were also excluded. A test for CMV antibodies was performed using serum and CSF samples. All of the test results were negative. The patient’s condition deteriorated; he could not walk or communicate verbally any longer, and he could only make minimal active movements with his limbs. A re-examination of CSF yielded the following findings: protein 496 mg/dL, glucose 88 mg/dL, pleocytosis 35/μL – neutrophils 80%. In May 2017, the patient was admitted to the Neurology Department in our hospital. He was conscious but unable to communicate verbally, and he had stiffness of the neck for 4 fingers, tremor of the upper limbs, and a high degree of paresis of the lower limbs. Brain MRI revealed cerebral oedema, enlarged ventricles containing blood, and strong enhancement of the ventricular lining with gadolinium contrast . We noticed the presence of hydrocephalus, which was probably a consequence of disease progression. Generalized seizures intensified, and the patient’s EEG was abnormal, with mainly slow waves but without status epilepticus. A number of tests for pathogenic microorganisms were carried out repeatedly. HBV, HCV, HIV, Francisella tularensis , Leptospira , CMV, EBV, Borrelia and Toxoplasma infections were excluded. The tests for tuberculosis (Bactec and QuantiFERON) were repeated, and their results were negative as well. An LP was performed again (xanthochromia, protein 705 mg/dL, glucose 6 mg/dL, pleocytosis 90/μL – neutrophils 90%). Atypical cells characterized by a polymorphic nucleus and heterochromatin cytoplasm were found. Elevated levels of lactate dehydrogenase were found in the serum and the CSF. Because of the presence of atypical cells in the CSF, meningeal biopsy was performed, and the specimen was sent for histopathological examination. Unfortunately, the specimen did not show any abnormalities. That was probably due to a lack of noticeable changes at the biopsy site: the specimen was taken from the frontal vault, but most of the melanomatotic changes were present in the base of brain. Nevertheless, the neoplastic process was highly probable; therefore, we tried to identify the source of the tumour. The diagnostics were extended to include abdominal and chest computer tomography scans, and ophthalmoscopy of the fundus oculi as well as abdominal, thyroid and testicular ultrasound examinations. Colonoscopy was planned, but the patient’s general condition was serious. The patient was examined by specialists in cardiology, internal medicine, pulmonology, ophthalmology, infectious diseases, anaesthesiology and neurosurgery. Non-infective diseases, such as sarcoidosis or vasculitis, were taken into account. The patient’s skin was evaluated several times, but there was no suspected area requiring further analysis. Drugs were used only for symptomatic treatment. The patient’s condition worsened, and epileptic seizures were observed several times a day. Moreover, he had a fever (38–39 degrees Celsius) and heart arrhythmia. The patient died 4 weeks after admission to our hospital. The patient survived 7 months after the onset of symptoms. After his death, an autopsy was performed. There was a small amount of fuscous or brown contents under the arachnoid part of the base of the brain and the cerebellum. The ventricular system was slightly enlarged and filled with a cloudy, red-brown liquid. In the lumens of the posterior ventricles, there were soft, flabby, loosely attached beige masses measuring 3–4 cm. The lining of the ventricles was beige or honey-coloured, sometimes spreading and dull. There was a slight degree of cerebral oedema. The histopathological examination of the meninges showed infiltrations of histiocyte-like cells [CD68(−), S-100(+), Vim(+), PanCK(−), LCA(−)] containing deposits of a brown pigment in the cytoplasm, with local polymorphic features and enlarged cell nuclei . The infiltrations were observed beneath the arachnoid membrane of the brain and the cerebellum and lined the ventricles . There were small points of necrotic tissue in the subependymal regions, along with congestion and cerebral oedema. There was neoplasm of the CNS, probably melanoma. This finding was supported by another histopathological examination of the meninges in a centre with a higher degree of reference and allowed us to recognize the meningeal melanomatosis. Based on the clinical examination, the results of additional tests and the autopsy, we could diagnose the patient with primary meningeal melanomatosis presenting as melanocytic meningitis. The above clinical case shows that despite extensive diagnosis and a number of tests, the diagnosis was extremely difficult to establish. Fig. 1 Cervical spinal cord MRI T1-weighted imaging without ( a ) and with ( b ) gadolinium contrast enhancement. Hyper-intensity on T1 was due to the paramagnetic effect of melanin, which had stable organic free radicals inside it, resulting in shortened T1 relaxation times in typical melanotic melanoma Fig. 2 Brain MRI T1-weighted imaging without ( c ) and with ( d ) gadolinium contrast enhancement. Hydrocephalus as a consequence of disease progression. Hyper-intensity on T1 was due to the paramagnetic effect of melanin, which had stable organic free radicals inside it, resulting in shortened T1 relaxation times in typical melanotic melanoma Fig. 3 Staining with haematoxylin and eosin, enlargement 200x ( e ) Polymorphic cells infiltrating the stroma Fig. 4 Staining of haematoxylin and eosin, enlargement 400x ( f ). There were many large, polymorphic cells with large nuclei and coarse chromatin. There was brown staining in the cytoplasm Fig. 5 Neoplastic cells have a positive reaction in S100 staining, confirming the neuroectodermal origin; enlargement 200x ( g ) Fig. 6 Most neoplastic cells are positive for Melano-A staining, confirming the melanocytic character of the neoplastic change; enlargement 200x (H)
| 3.992188
| 0.978516
|
sec[1]/p[0]
|
en
| 0.999997
|
31690266
|
https://doi.org/10.1186/s12883-019-1460-x
|
[
"cells",
"tests",
"brain",
"ventricles",
"protein",
"staining",
"department",
"findings"
] |
[
{
"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 CLASSIFICATION ═══
【1. Other specified clinical findings on examination of urine, without diagnosis (MF9Y)】
Synonyms: Methaemoglobinuria | Other and unspecified abnormal findings in urine | Calciuria | Cells and casts in urine | casts in urine
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings of the genitourinary system → Clinical findings on examination of urine, without diagnosis → Other specified clinical findings on examination of urine, without diagnosis
【2. Mucolipidosis (5C56.20)】
Synonyms: Mucolipidosis type 3 | Pseudo-Hurler polydystrophy | Pseudo-Hurler disease | Mucolipidosis type 2 | N-acetyl-glucosamine 1-phosphotransferase deficiency
Excludes: Sialidosis (mucolipidosis type 1)
Hierarchy: Inborn errors of metabolism → Lysosomal diseases (5C56) → Glycoproteinosis (5C56.2) → Mucolipidosis
【3. Sickle cell disease without crisis (3A51.1)】
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.
Synonyms: Hb-SS disease without crisis | HbSS without crisis | Sickle-cell anaemia without crisis | SCD - [sickle cell disease] | SCA - [sickle cell anaemia]
Hierarchy: Diseases of the blood or blood-forming organs (03) → Anaemias or other erythrocyte disorders → Sickle cell disorders or other haemoglobinopathies (3A51) → Sickle cell disease without crisis
【4. Primary anterior uveitis (9A96.3)】
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.
Synonyms: anterior chamber cell
Hierarchy: Disorders of the eyeball - anterior segment → Disorders of the anterior uvea → Anterior uveitis (9A96) → Primary anterior uveitis
【5. Acquired pure red cell aplasia, unspecified (3A61.Z)】
Synonyms: Acquired pure red cell aplasia | acquired red cell aplasia | red cell aplasia NOS | pure red cell aplastic anaemia | red cell aplastic anaemia
Hierarchy: Anaemias or other erythrocyte disorders → Pure red cell aplasia → Acquired pure red cell aplasia (3A61) → Acquired pure red cell aplasia, unspecified
|
MF9Y
|
Other specified clinical findings on examination of urine, without diagnosis
|
A 36-year-old gravid2 live1 pregnant Iranian woman at the 36th week of her gestation came to Imam Khomeini (Tehran, Iran) Obstetric Emergency Department in March 2021 complaining of subacute visual disturbance. She had well-controlled gestational diabetes (on diet) in her current pregnancy and history of hypothyroidism since 10 years ago, soon after her first delivery, which was well controlled before and during pregnancy by taking 150 microgram oral levothyroxine daily. She also denied any cigarette smoking or alcohol consumption, and she did not take any other medication except for daily levothyroxine, multi-prenatal, and ferrous sulfate tablets (each one tablet per day). She was a housewife living in a small county in Tehran Province with middle socioeconomical status. Regarding her family history, no thromboembolic event or similar visual or neurological disorders had been evident in her first- or second-degree relatives. She had been suffering from decreased vision that she described as central scotoma since 5 days ago. Her first visual attack had lasted for nearly 15 minutes, but thereafter, similar attacks had reoccurred frequently, finally leading to persistent decreased vision since 2 days before her arrival. She had been hospitalized in another center for 2 days. Brain magnetic resonance imaging (MRI) and neurologic consult were done there, and the first impression was papillophelebitis at that center, but the patient had discharged home at her own request and referred to our hospital for further assessment. Upon her admission, she was completely conscious, her vital signs were all within normal ranges (blood pressure: 115/75 mmHg, pulse rate: 88 beats/minute, respiratory rate: 14/minute, oral temperature: 36.9 °C), and she had no complaints of poor signs (headache, nausea, vomiting, and/or epigastric pain) other than visual disturbance. Her body mass index was 24.9 kg/m 2 , and obstetrical examination was unremarkable (no evidence of uterine contraction or hypertonicity, rupture of membranes, vaginal bleeding, or any abnormalities in fetal heart rate tracing). Moreover, reviewing different body systems, sensorimotor neurological examination, gate assessment, lung and heart auscultation, musculoskeletal and dermatological examination, and also liver and spleen palpation (as far as doable due to presence of a large gravid uterus), no pathological findings were revealed. On ophthalmological examination, uncorrected visual acuity was counting fingers at 3-meter distance and 10/10 in her right and left eye, respectively. Direct fundoscopy of the right eye revealed evidence of retinal ischemia and scattered hemorrhages as well as optic disc swelling. Previous brain MRI was reviewed by another radiologist, and no pathologic findings were documented. Furthermore, no abnormalities were found on carotid sonographic examination or transthoracic echocardiography (TTE). Subsequently, we consulted with an ophthalmologist in Farabi Eye Hospital. Intraocular pressure (IOP) was 13 mmHg in both eyes, and there was no abnormal lesion in anterior segment, as well as no vitritis in both eyes. Posterior segment assessment in her right eye revealed evidence of retinal ischemia, cotton-wool spots, and scattered flame-shaped hemorrhages, as well as optic disc swelling, in favor of CRVO and CLRAO . The next day, spectral-domain ocular coherence tomography (SD-OCT) confirmed our clinically suspected diagnosis . According to the ophthalmologist's opinion, as no macular edema was evident, no further interventions or early delivery was necessitated. The ophthalmologist highly recommended us to check thrombophilia disorders or other relevant systemic diseases as potential predisposing factors and also to rule out preeclampsia syndrome. Additionally, they emphasized continuing prophylactic dose of low-molecular-weight heparin (LMWH) 40 mg subcutaneously daily, which had been initiated on her admission, and they arranged an outpatient follow-up a week later. As our investigations ruled out potential comorbidities and preeclampsia syndrome (all blood pressure measurements were normal, and there was no abnormality in laboratory tests except for D-dimer and CRP) (Table 1 ), she was discharged home and no drugs except for LMWH were prescribed for an additional 7 days. There were no changes in her symptoms, and visual acuity remained stable at 3-meter counting fingers at 1-week follow-up. Six days later, she experienced labor pain and thus was admitted again at Imam Khomeyni Hospital; due to establishment of active labor, she subsequently underwent an uncomplicated emergency cesarean section through a Pfannesteil skin incision under regional anesthesia, and a 3300-g female newborn with Apgar score of 9 and 10 at the first and fifth minute was delivered. The baby’s height and head circumference were also 51 and 35.5 cm, respectively. Finally, both mother and baby were discharged home 48 hours later without any complications. We strongly recommended her to organize an ophthalmologist’s appointment as soon as possible. Finally, she visited her ophthalmologist 2 weeks after delivery; the follow-up examination and imaging results are shown in Figs. 1 B and 2 C, D. She had improvement in visual acuity to 4/10 and somewhat decrease in retinal findings. At 8-week postpartum follow-up, her visual acuity was 8/10 in the right eye and ocular coherence tomography angiography (OCT-A) was performed, revealing no edema as well as a decrease in vascular density in cilioretinal artery supply in superior of the macula . She had no complications such as IOP rise or neovascularization. One-year follow-up examination was done by the same ophthalmologist, showing that all hemorrhage and exudate had resolved and cilioretinal artery occlusion was barely visible at the superior macula, while on imaging, no macular edema was seen . Moreover, thyroid stimulating hormone, complete blood cell count, and 75-g glucose tolerance test were all within normal limits. Fig. 1 Fundus photo of the patient. A Fundus photo of the right eye revealed optic nerve edema and diffuse peripapillary flame-shape hemorrhage and cotton-wool spots in the peripapillary region as well as tortuous and dilated veins, and whitening in superior of the macula in favor of cilioretinal artery occlusion. B 2-Week postpartum follow-up and C 8-week postpartum follow-up revealed a decrease in hemorrhage and cotton-wool spots and discrete exudate in the macula. D At 1-year follow-up, all hemorrhage and exudate had resolved and cilioretinal artery occlusion was barely visible visible at the superior macula Fig. 2 A Spectral-domain optical coherence tomography (SD-OCT) of the right eye in fovea showed hyperreflectivity in the middle retinal layer and no apparent edema, and B in superior macula revealed thickening and hyperreflectivity of the inner retina and shadowing in cilioretinal artery occlusion area. C and D At 2-week postpartum follow-up. E OCT angiography (OCTA) of the right eye at 8-week postpartum follow-up showed a decrease in vascular density in superficial and deep capillary plexus and no macular edema. F OCTA follow-up at 1-year revealed a stable nonperfused area and no macular edema Table 1 Patient’s laboratory tests Lab Result Lab Result WBC 8400/mL (75% neutrophils) AST 33 IU/L Hb 10.9 g/dL ALT 31 IU/L Plt 212 × 10 3 /mL LDH 400 IU/L D-Dimer 3.5 mg/L (high) TSH 0.6 mIU/mL ESR 40 mm/H (high) ANA Neg Anti-dsDNA 8.6 (Neg) APS pannel Neg Plasma homocysteine level 4.75 (Nl) 24-hour urinary protein excretion 175 Protein C level 81 (Nl) Protein S level 53 (minimum of 42 is normal in the laboratory reference’s range) Prothrombin gene mutation Wilde type Factor Leiden mutation Wild type WBC white blood cell, Hb hemoglobin, Plt platelet, ESR erythrocyte sedimentation rate, anti-dsDNA anti-double strand deoxyribonucleic acid, AST aspartate aminotransferase, ALT alanine aminotransferase, LDH lactate dehydrogenase, TSH thyroid stimulating hormone, ANA antinuclear antibody, APS antiphospholipid syndrome
| 3.880859
| 0.982422
|
sec[1]/p[0]
|
en
| 0.999996
|
35568885
|
https://doi.org/10.1186/s13256-022-03421-8
|
[
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[
{
"code": "QA07.Z",
"title": "Follow-up examination after treatment for conditions other than malignant neoplasms, unspecified"
},
{
"code": "QB8Y",
"title": "Contact with health services for other specified surgical interventions"
},
{
"code": "PK81.0",
"title": "Ventilation associated with injury or harm in therapeutic use"
},
{
"code": "CA82.1",
"title": "Chronic or other pulmonary manifestations due to radiation"
},
{
"code": "PK80.4Z",
"title": "Endocrine procedure associated with injury or harm in therapeutic use, unspecified approach"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Follow-up examination after treatment for conditions other than malignant neoplasms, unspecified (QA07.Z)】
Synonyms: Follow-up examination after treatment for conditions other than malignant neoplasms | medical surveillance following treatment for conditions other than malignant neoplasms | follow-up examination NOS | examination following treatment NOS
Hierarchy: Reasons for contact with the health services → Contact with health services for purposes of examination or investigation → Follow-up examination after treatment for conditions other than malignant neoplasms (QA07) → Follow-up examination after treatment for conditions other than malignant neoplasms, unspecified
【2. Contact with health services for other specified surgical interventions (QB8Y)】
Synonyms: Follow-up care involving removal of external fixation device | Orthopaedic follow-up care, unspecified | orthopedic aftercare | unspecified orthopedic aftercare | Surgical follow-up care, unspecified
Hierarchy: Factors influencing health status or contact with health services (24) → Reasons for contact with the health services → Contact with health services for specific surgical interventions → Contact with health services for other specified surgical interventions
【3. Ventilation associated with injury or harm in therapeutic use (PK81.0)】
Synonyms: complication during or following ventilation | Ventilator associated pneumonia | VAP - [ventilator associated pneumonia] | respirator associated pneumonia
Excludes: Circumstances associated with a surgical or other medical procedure influencing the episode of care, without injury or harm
Hierarchy: Causes of healthcare related harm or injury → Surgical or other medical procedures associated with injury or harm in diagnostic or therapeutic use → Certain medical procedures associated with injury or harm in therapeutic use (PK81) → Ventilation associated with injury or harm in therapeutic use
【4. Chronic or other pulmonary manifestations due to radiation (CA82.1)】
Definition: A chronic inflammatory reaction of the lung ultimately resulting in fibrosis in response to repeated or high dose radiation exposure.
Synonyms: Fibrosis of lung following radiation | radiation fibrosis of lung | Chronic radiation pulmonary fibrosis
Hierarchy: Diseases of the respiratory system (12) → Lung diseases due to external agents → Respiratory conditions due to other external agents (CA82) → Chronic or other pulmonary manifestations due to radiation
【5. Endocrine procedure associated with injury or harm in therapeutic use, unspecified approach (PK80.4Z)】
Synonyms: Endocrine procedure associated with injury or harm in therapeutic use | Complication during or following endocrine procedure | Complication during or following endocrine medical or surgical endocrine intervention | complication of endocrine procedure without mention of misadventure at the time of the procedure
Hierarchy: Surgical or other medical procedures associated with injury or harm in diagnostic or therapeutic use → Medical or surgical procedure associated with injury or harm in therapeutic use (PK80) → Endocrine procedure associated with injury or harm in therapeutic use (PK80.4) → Endocrine procedure associated with injury or harm in therapeutic use, unspecified approach
|
QA07.Z
|
Follow-up examination after treatment for conditions other than malignant neoplasms, unspecified
|
A previously healthy man in his early forties developed mild COVID-19 in March 2021, with persistent fatigue and transient hyposmia and hypogeusia being the only reported symptoms. His previous history was significant for atopic dermatitis and saphenous vein stripping due to chronic venous insufficiency. His family history was reported to be negative for neurological diseases. However, his father died at 33 years of age, apparently due to myocardial infarction, while his mother had been rescued from Shoah and adopted in her infancy (she was reported to be of Spanish origin, possibly Sephardim). In the second half of May 2021 he started seeing black shadows when closing his eyes, followed by dizziness, difficulty reading and worsening of balance. In June 2021 he noticed loss of coordination of the left arm. Due to gradual progression, he was admitted to a local neurological ward in late June: the neurological exam showed ataxia of the left limbs, right-beating nystagmus and absent lower-limb reflexes. Brain Magnetic Resonance Imaging (MRI) was reportedly normal; lumbar puncture showed normal cerebrospinal fluid (CSF) protein and cells and absence of oligoclonal bands. Serological testing was negative for onconeural antibodies, neuronal surface antibodies, anti-ganglioside antibodies, Treponema pallidum, Borrelia, Human Immunodeficiency Virus and Hepatitis B and C Viruses. He was discharged, to be readmitted shortly thereafter due to the appearance of clonic movements of the left hand. A second brain MRI was again reportedly normal, including diffusion-weighted sequences. Electroencephalographic (EEG) recordings showed frequent diffuse theta-delta slowing with epileptiform discharges on the right hemisphere. Carbamazepine proved ineffective and was therefore replaced with valproic acid, which was in turn substituted with levetiracetam due to excessive drowsiness. Nevertheless, symptoms progressed and repeat EEGs showed an increase in slow activity and generalized discharges described as spike-and-wave. A second onconeural and neuronal surface antibody panel was negative, as well as antinuclear and anti-neutrophil cytoplasmic antibodies. A total-body Positron Emission Tomography-Computed Tomography was normal. A trial of intravenous immunoglobulin was associated with a transient arrest of progression; in the following days, a 5-day high-dose intravenous methylprednisolone was accompanied with an abrupt worsening of incoordination of the left arm and the appearance of dystonia of both upper limbs. At the end of July 2021, the patient was transferred to our ward. On admission, he was drowsy but responsive and partially oriented, his speech was effortful and telegraphic in English (native language untestable). Eye movements were preserved, although saccadic movements were slow in all directions. He could not sit, stand or walk due to diffuse dystonic posturing, more evident on the left side, and diffuse myoclonus. His left upper limb showed non-stereotyped and irregular involuntary movements. A complete strength testing could not be performed due to the inability to adequately perform voluntary movements; however, no major deficits were noted. Similarly, finger-to-nose and heel-to-shin tests could not be performed due to the dystonic and myoclonic components. A repeat brain MRI showed mild T2 hyperintensity of the caudate nuclei and putamina and diffusion restriction within the right fronto-temporo-insular cortex, left fronto-parietal cortex and bilateral occipital cortex . Prolonged EEG recordings showed diffuse theta slowing with generalized triphasic 1–2 Hz periodic sharp-wave complexes . As shown in Table 1 , an increase in serum interleukin (IL)-1β, IL-6 and IL-8 and neurofilament light chain (NfL) was observed, with normal C-reactive protein and mid-regional proadrenomedullin (MR-ProADM) levels; extended autoimmune and infectious panels were negative. SARS-CoV-2 serology showed positive anti-Receptor Binding Domain (RBD) Spike 1 total antibodies at high titre, with negative IgM antibodies; multiple nasal swabs were negative for SARS-CoV-2. CSF analysis showed normal protein, cells and MR-ProADM, with moderately elevated IL-8 and C-X-C motif chemokine Ligand 10 (CXCL10) and very high NfL levels. A neurodegenerative biomarker profile showed extremely high total Tau levels, slightly reduced beta-amyloid 1-42 and normal phospho-Tau-181 and beta-amyloid 42/40 ratio. Real-Time Quaking-Induced Conversion (RT-QuIC) CSF testing for PrP was positive. Figure 1. A-D: magnetic resonance imaging of the brain showing typical features of Creutzfeldt-Jakob disease: hyperintensity of the caudate nuclei and putamina on fluid-attenuated inversion recovery imaging (panel a, arrowheads) and diffusion restriction within the bilateral striatum, right fronto-temporo-insular cortex, left fronto-parietal cortex and bilateral occipital cortex on diffusion-weighted imaging (panels b-d, arrows). e: electroencephalographic recording showing typical features of Creutzfeldt-Jakob disease. a longitudinal montage is depicted. Subcontinuous generalized triphasic periodic sharp-wave complexes can be seen on this segment, with a 1–2 Hz discharge rate (a); during the short interruptions in periodic sharp-wave complex firing, a diffusely slowed background activity in the theta range can be observed (b). Table 1. Relevant laboratory analyses on blood and cerebrospinal fluid samples. Feature Value Reference range Inflammation Pro-adrenomedullin Serum CSF CSF/blood ratio 0.46 nMol/l 0.55 nMol/l 1.2 <0.56 Interleukin 1β Serum CSF 0.2 pg/ml <0.1 pg/ml <0.16 <0.1 Interleukin 6 Serum CSF 42.5 pg/ml 4.4 pg/ml 0.8–6.4 1.0–3.1 Interleukin 8 Serum CSF 32.7 pg/ml 49.6 pg/ml 6.7–16.2 15.2–38.4 TNFα Serum CSF 10.9 pg/ml 0.4 pg/ml 7.8–12.2 <0.5 CXCL10 Serum CSF 76.4 pg/ml 223 pg/ml 37.2–222 6-132 C-reactive protein 0.3 mg/l <5 Autoimmunity ANA Absent Absent ENA (Ro/SSA, La/SSB, SM, RNP, Scl70, Jo1) Absent Absent Anti-MPO 1 UA/ml <11 Anti-PR3 0 UA/ml <11 Serum and CSF onconeural antibodies* Absent Absent Serum neuronal surface antigen antibodies † Absent Absent IgLON5 antibodies Absent Absent Immunofluorescence on monkey cerebellum Negative Negative SARS-CoV-2 RT-PCR on nasal swab (multiple tests) Negative Negative Serum antibodies IgG (CLIA) IgM (CLIA) Total (ECLIA) 43.1 UA/ml 0.5 UA/ml 275 U/ml <8 <8 <0.79 Cerebrospinal fluid CSF/serum glucose ratio 82% 50–90 Total protein 334 mg/l 150–450 Cells 0.8 /µl (100% lymphocytes) <3 PCR for HSV 1–2, VZV, Adeno-, Paraecho-, Enterovirus Negative Negative Borrelia and tick-borne encephalitis antibodies Absent Absent Biomarkers of neuronal degeneration or injury Total Tau >2000 pg/ml <404 Phospho-Tau-181 27 pg/ml <56.5 β-Amyloid 1-42 319 pg/ml >599 β-Amyloid 42/40 ratio 0.088 >0.069 Neurofilament light chain Serum CSF 171 pg/ml 5978 pg/ml 6.3–22.2 155–1757 RT-QuIC for misfolded PrP Positive Negative Abnormal results are shown in bold. Reference ranges for interleukins in cerebrospinal fluid were obtained by our Laboratory based on 100 cerebrospinal fluid samples. *: Onconeural antibody panel: amphiphysin, CV2, Ma2/Ta, Ri, Yo, Hu, recoverin, Sox1, titin, Zic4, GAD, Tr. †: Neuronal surface antigen antibody panel: N-methyl-D-aspartate receptor (NMDA-R), leucine rich glioma inactivated 1 (LGI1), contactin-associated protein-like 2 (CASPR2), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor 1 and 2 (AMPA-1-R and AMPA-2-R), γ-aminobutyric acid receptor B (GABA-B-R), dipeptidyl aminopeptidase-like protein (DPPX). Abbreviations: ANA, antinuclear antibodies; CLIA, chemiluminescence immunoassay; CSF, cerebrospinal fluid; CXCL10, C-X-C Motif Chemokine Ligand 10; ECLIA, electrochemiluminescence immunoassay; ENA, extractable nuclear antigens; HSV, herpes simplex virus; MPO, myeloperoxidase; PCR, polymerase chain reaction; PR3, proteinase 3; PrP, prion protein; RT-PCR, reverse transcriptase polymerase chain reaction; RT-QuIC, real-time quaking-induced conversion; TNFα, tumour necrosis factor α; VZV, varicella zoster virus.
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sec[1]/sec[0]/p[0]
|
en
| 0.999996
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PMC9255144
|
https://doi.org/10.1080/19336896.2022.2095185
|
[
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"serum",
"protein",
"fluid",
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"total",
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[
{
"code": "8A68.Y",
"title": "Other specified type of seizure"
},
{
"code": "LA75.1",
"title": "Agenesis of lung"
},
{
"code": "8A66.1Z",
"title": "Non-convulsive status epilepticus, unspecified"
},
{
"code": "LB20.0Y",
"title": "Other specified structural developmental anomalies of liver"
},
{
"code": "QF01.Y",
"title": "Other specified acquired absence of organs"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Other specified type of seizure (8A68.Y)】
Synonyms: Absence episode | Absence seizure episode | Pseudotetanus | Clonic seizure disorder
Hierarchy: Diseases of the nervous system (08) → Epilepsy or seizures → Types of seizures (8A68) → Other specified type of seizure
【2. Agenesis of lung (LA75.1)】
Definition: This refers to the absence or rudimentary residua of an undeveloped lung.
Synonyms: Pulmonary agenesis | absence of lung | aplasia of lung | apulmonism | congenital absence of lung
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the respiratory system → Structural developmental anomalies of lungs (LA75) → Agenesis of lung
【3. Non-convulsive status epilepticus, unspecified (8A66.1Z)】
Synonyms: Non-convulsive status epilepticus | Epileptic absence status | Petit mal status epilepticus | Petit-mal status | absence status
Hierarchy: Epilepsy or seizures → Status epilepticus (8A66) → Non-convulsive status epilepticus (8A66.1) → Non-convulsive status epilepticus, unspecified
【4. Other specified structural developmental anomalies of liver (LB20.0Y)】
Synonyms: Alagille syndrome | Alagille-Watson syndrome | Arteriohepatic dysplasia | Syndromic bile duct paucity | Alagille syndrome type 1
Hierarchy: Structural developmental anomalies of the liver, biliary tract, pancreas or spleen → Structural developmental anomalies of gallbladder, bile ducts or liver (LB20) → Structural developmental anomalies of liver (LB20.0) → Other specified structural developmental anomalies of liver
【5. Other specified acquired absence of organs (QF01.Y)】
Synonyms: Acquired absence of part of head or neck | Acquired absence of eye | absence of eye | absence of eyeball | acquired anophthalmos
Hierarchy: Factors influencing health status → Acquired absence of body structure → Acquired absence of organs (QF01) → Other specified acquired absence of organs
|
8A68.Y
|
Other specified type of seizure
|
Our patient satisfied the American College of Rheumatology criteria for diagnosis of SLE as well as criteria for autoimmune hepatitis. Although earlier necropsy studies have estimated the prevalence of myocarditis in SLE to be 50–80%, most of those cases were subclinical. Symptomatic SLE myocarditis is rare and occurs in up to 9% of cases . Endomyocardial biopsy remains the gold standard for diagnosis, but it has its pitfalls—an invasive process with associated complications—and there is a low sensitivity and specificity associated with it (due to the patchy nature of myocardial involvement in lupus myocarditis) [ 3 – 5 ]. Thus, for urgent treatment-related decision making, clinical diagnosis of SLE myocarditis remains an important tool as untreated cases may develop abrupt and life-threatening complications including arrhythmias, dilated cardiomyopathy, and heart failure . Heart failure is the most common presenting feature of myocarditis . Pulmonary infection, ischemic heart disease, pulmonary embolism, alveolitis, pulmonary hemorrhage, and pulmonary hypertension can mimic the symptoms of lupus myocarditis-related heart failure like shortness of breath, chest pain, palpitations, pedal edema, and exertion intolerance. Echocardiography, a noninvasive and highly useful adjunct in the proper clinical setting, has many parameters that reportedly have a high index of sensitivity and specificity for diagnosis of acute myocarditis. These include decreased ejection fraction, increased chamber size, decreased diastolic descent rate of anterior mitral leaflet, decreased ratio of mean systolic to mean diastolic velocity of left ventricular posterior wall, reduced early to late diastolic flow velocity ( E/A ) ratio, lower deceleration rate of early diastolic flow velocity, prolonged isovolumetric relaxation time, and atrial ejection force . Our patient met with many of the above mentioned criteria. Viral and ischemic cardiomyopathy were the two other important differential diagnoses considered and then discarded in our case, although viral serology (except for the hepatitis viruses) and angiography were not done. Our patient was a young girl without having any obvious risk factors for atherosclerosis and with an SLE disease activity index of 24 at presentation. In a resource-poor country like ours, we drew inspiration from the same approach in an earlier landmark Asian case series . Anti-Ro, anti-ds-DNA, and APLA positivity has been variably associated with SLE myocarditis . Anti-Ro and anti-ds-DNA were positive, and APLA workup negative in our patient. Acute mode of onset, presence of renal and hematological involvement with low complement (C3 and C4) levels, hypoalbuminemia, and raised ESR associated with clinical myocarditis, as found in our case, has been documented rarely . The first presentation of SLE in the form of heart failure, overlapping with autoimmune hepatitis and immune hemolytic anemia as found in our case, is rarer . Anti-Ro is very rarely positive in normal individuals or hospitalized patients with nonrheumatic disorders . Anti-Ro antibody may be associated with cardiac disorders in adults, as well as in neonates . Troponin T, a serum marker with high sensitivity and specificity for cardiac myocyte injury, was negative in our case, as was CPK-MB fraction and troponin T. Troponin T positivity occurs in only 34% of cases and that too, in early cases of autoimmune myocarditis, presenting within a month. CPK-MB is inferior as it is positive in only 5.7% of biopsy-proven cases . This young girl presented to us after her symptoms were present for more than a month, possibly explaining the negativity of CPK-MB and troponin T. Blockade of small vessels of the heart as part of widespread microvascular occlusion in the setting of APLA positivity is another possibility of cardiac dysfunction in SLE. Widespread microvascular thrombosis of cardiac vessels and other organs, culminating into diffuse cardiomyopathy, heart failure, or even cardiac arrest, can occur acute or as chronic manifestation without signs of inflammation or large vessel involvement in patients of SLE. Diagnosis is possible only with an endomyocardial biopsy . However, in the light of APLA and lupus anticoagulant negativity and prompt response to immunosuppression, this diagnosis was less likely in our case on clinical grounds, even though biopsy was not performed. The jaundice and hepatic derangements in a case like ours may be due to SLE activity itself, due to autoimmune hepatitis with other nonhepatic autoimmune manifestations or SLE with coexistent autoimmune hepatitis. Patients with AIH are more prone to develop systemic autoimmune diseases and viceversa . Both SLE and AIH have many autoimmune features in common, namely, polyarthralgia, hypergammaglobulinemia, and ANA positivity . It is difficult, but important the to distinguish between SLE with hepatic dysfunction and AIH from, therapeutic point of view as SLE will lead to renal end organ damage while AIH patients will have hepatic failure as the terminal event. There are some clinical, serological, and, most importantly, histological pointers, which differentiates the two conditions. Previously thought to be rare, hepatic involvement in SLE is now considered to be more clinically significant . Hepatomegaly is common, and elevated liver enzymes can be found in 23.5% cases . The most common histological findings in SLE are fatty infiltration followed by atrophy and/or necrosis of central hepatic cells. In most cases, this concomitant hepatic dysfunction is subclinical . The histological hallmark of AIH is interface hepatitis and portal inflammation with plasma cell infiltration . Concomitant periportal piecemeal necrosis, variable lobular hepatitis, and rosette formation of the hepatic cells further supports the diagnosis of AIH but does not exclude SLE. Presence of only lobular hepatitis tilts the diagnostic scale more towards SLE . Anti-ribosomal-P antibody positivity occurs in 44% of patients with SLE-associated hepatic dysfunction but is absent in AIH, making it a useful serological differentiator between the two . Anti-ds-DNA may be transiently elevated in AIH, though more recent data emphasizes its presence to be associated with active lupus rather than AIH, where it is negative . Anti-Smith antibody may also be positive in SLE-AIH overlap in some cases . In our patient, presence of low serum complement (C3 and C4), DCT-positive hemolytic anemia, ANA positivity, and high titres of ds-DNA and anti-Ro fulfilled the revised ACR diagnostic criteria and pointed strongly towards SLE. Likewise, our patient had jaundice with high AST/ALT ratio, hypergammaglobulinemia, ANA, and anti-smooth muscle antibody positivity with supportive evidence of liver biopsy showing interface hepatitis, periportal piecemeal necrosis, rosette formation, and lobular hepatitis, strongly suggesting the diagnosis of autoimmune hepatitis . Portal plasma cell and lymphocyte infiltration is said to be characteristic of untreated AIH as both lupus hepatitis and treated AIH may have lymphocytic infiltration of the portal tracts in common. A good response to corticosteroids further bolstered the diagnosis of autoimmune hepatitis in our case. AIH-SLE overlap has rarely been reported before [ 24 , 27 , 29 – 32 ]. This disease entity responds rapidly to steroid therapy with the hepatic dysfunction improving in parallel with stabilization of other systemic manifestations, and the prognosis is generally good . Some researchers have reported less favorable steroid response in AIH-SLE overlap syndrome . We believe that our patient had an overlap of SLE and AIH which is very rare at the outset. A presentation of lupus myocarditis with AIH has not been reported before to the best of our knowledge. Lupus myocarditis must be considered in suspected lupus patients with unexplained tachycardia and acute episodes of breathlessness, as timely intervention with systemic steroid therapy is often life salvaging.
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|
sec[2]/p[0]
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en
| 0.999997
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22937445
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[
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[
{
"code": "DB97.Z",
"title": "Inflammatory liver disease, unspecified"
},
{
"code": "1E50.Z",
"title": "Acute viral hepatitis, unspecified"
},
{
"code": "DB97.2",
"title": "Chronic hepatitis, not elsewhere classified"
},
{
"code": "1E5Z",
"title": "Viral hepatitis, unspecified"
},
{
"code": "1E51.0Z",
"title": "Chronic hepatitis B, unspecified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Inflammatory liver disease, unspecified (DB97.Z)】
Synonyms: Certain specified inflammatory liver diseases | Nonspecific reactive hepatitis | inflammatory liver disease | hepatitis NOS | inflammation of liver
Hierarchy: Diseases of the digestive system (13) → Diseases of liver → Certain specified inflammatory liver diseases (DB97) → Inflammatory liver disease, unspecified
【2. Acute viral hepatitis, unspecified (1E50.Z)】
Synonyms: Acute viral hepatitis | acute anicteric hepatitis | Acute hepatitis NOS | acute viral hepatitis non-A non-B NEC | non A non B viral hepatitis
Hierarchy: Certain infectious or parasitic diseases (01) → Viral hepatitis → Acute viral hepatitis (1E50) → Acute viral hepatitis, unspecified
【3. Chronic hepatitis, not elsewhere classified (DB97.2)】
Synonyms: Chronic hepatitis, unspecified | Chronic active hepatitis NEC | Other specified chronic hepatitis | Chronic persistent hepatitis NEC | Chronic lobular hepatitis NEC
Excludes: hepatitis (chronic): granulomatous NEC | Drug-induced or toxic liver disease | hepatitis (chronic): viral | hepatitis (chronic): alcoholic
Hierarchy: Diseases of the digestive system (13) → Diseases of liver → Certain specified inflammatory liver diseases (DB97) → Chronic hepatitis, not elsewhere classified
【4. Viral hepatitis, unspecified (1E5Z)】
Synonyms: anicteric hepatitis
Hierarchy: Certain infectious or parasitic diseases (01) → Viral hepatitis → Viral hepatitis, unspecified
【5. Chronic hepatitis B, unspecified (1E51.0Z)】
Synonyms: Chronic hepatitis B | Chronic hepatitis B without delta agent | chronic HBV - [hepatitis B virus] infection | hepatitis B NOS | chronic type B viral hepatitis
Hierarchy: Viral hepatitis → Chronic viral hepatitis (1E51) → Chronic hepatitis B (1E51.0) → Chronic hepatitis B, unspecified
|
DB97.Z
|
Inflammatory liver disease, unspecified
|
Based on the history, physical examination and laboratory results with the findings in the peritoneal dialysis fluid, a diagnosis of peritoneal dialysis-related peritonitis was confirmed. Besides related symptomatic treatments, the empiric antibiotic treatment was initiated with levofloxacin (0.5 g intravenously) every day, ceftazidime (0.25 g intraperitoneally) in a 3-h dwell four times every day and vancomycin (1.0 g intraperitoneally) in an 8-h dwell once at night every 5 days. Final culture of initial peritoneal effluent results indicated the organism was P. multocida, which was found to be sensitive to ampicillin/sulbactam, cefazolin, cefotaxime, cefoxitin, levofloxacin, ampicillin, cefuroxime, imipenem, ciprofloxacin, ceftazidime, meropenem, and cefoperazone/sulbactam, while in the blood there were no bacterial infections were having been found. According to the drug-sensitive test, the intravenously antibiotic treatment was switched to meropenem (0.5 g intravenously) every 12 h, with peritoneal antibiotic treatment still. After one-day treatment, the symptoms disappeared but the WBC count of peritoneal effluent was still above the normal level of the WBC counts, which reminded the infection remained existed. Therefore, Cefazidime was switched to amikacin (200 mg intraperitoneally) in a 3-h dwell four times every day and on Aug. 18th, intravenously meropenem was switched to cefoperazone/sulbactam (1.5 g intravenously) every 12 h. And for the reason that the CAPD was noneffective and the peritoneal infection was not controlled, the patient was undergone temporary hemodialysis four times every week with imipenem/cilastatin (500 mg intraperitoneally) in a 6-h dwell every day for continuing peritoneal antibiotic treatment. After 12-day antibiotic treatment, the WBC count of peritoneal effluent was not significantly improved and the peritoneal effluent was still cloudy. When it was the deadline of the ISPD guideline recommending to hemodialysis, the patient firmly refused the lasting hemodialysis and asked for a further treatment. According to the drug-sensitive results and reviewed case reports (Table 1 ), the patient was switched to ampicillin/sulbactam (3 g intravenously) twice every day. The WBC count of peritoneal effluent was markedly improved and the antibiotic treatment was continued until the WBC count of the peritoneal effluent was below 100 × 106/L and the PD effluent was clear, which revealed the infection was controlled . During the treatment, the patient was undergone temporary hemodialysis 12 times to maintain the function of excretion. When the infection was controlled and the function of CAPD recovered, the temporary hemodialysis was stopped and the patient was returned to CAPD. The patient discharged after using ampicillin/sulbactam for 17 days and continued amoxicillin (0.25 g orally) three times a day for another 6 days. The detail key dates of altering antibiotics and methods have been listed (Table 2 ). Table 1 Review of the cases in the previous literatures Case Reference Sex Age(Yr) Main complains PD effluent characters Animal exposure Effective treatments Results 1 Rondon-Berrios, H. Male 38 Severe and diffuse abdominal pain Cloudy Household cat Piperacillin/tazobactam (IV) Vancomycin (IV) Hemodialysis 2 Campos, A. Male 8 Diffuse abdominal pain Cloudy Household hamster Tobramycin (IP) Peritoneal dialysis 3 Sol, P. M. Female 7 Abdominal pain and vomiting Cloudy Household cat Ampicillin (IP) Peritoneal dialysis 4 Paul, R. V. Female 55 Severe abdominal pain Milk-colored Household cat Vancomycin (IV) gentamicin (IV) Peritoneal dialysis 5 Cooke, F. J. Female 73 Abdominal pain Cloudy Household cat Gentamicin (IP) Ciprofloxacin (PO) Peritoneal dialysis 6 Dresselaars, H. F. Female 62 Mild abdominal discomfort Turbid Household cat Cotrimoxazole (IV) Cefalotin (IP) Peritoneal dialysis 7 Giron, F. F. Male 72 Abdominal pain Turbid Household cat vancomycin (IP) ceftazidime (IP) Peritoneal dialysis 8 Satomura, A. Male 58 Abdominal discomfort Unknown Household cat cefazolin (IP) ceftazidime (IP) Peritoneal dialysis 9 Joh, J. Male 55 Abdominal pain, nausea and vomiting Cloudy Household cat Gentamicin (IP) Ampicillin/sulbactam (PO) Peritoneal dialysis 10 Kim, I. Female 25 Diffuse abdominal pain Cloudy Household cat Cefazolin (IP) Gentamicin (IP) Peritoneal dialysis 11 Loghman-Adham, M. Female 12 Mild abdominal pain Clear Household cat Cephapirin (IP) Gentamicin (IP) Peritoneal dialysis 12 MacKay, K. Male 73 Mild abdominal discomfort Cloudy Household cat Vancomycin (IP) Ceftazadime (IP) Peritoneal dialysis 13 Nishina, M. Male 45 Abdominal pain Cloudy Household cat Vancomycin (IV) Ceftazidime (IP) Peritoneal dialysis 14 Freeman, A. F. Female 14 Abdominal pain Cloudy Household hamster Vancomycin (IP) Ceftazadime (IP) Ampicillin/sulbactam (IV) Peritoneal dialysis 15 Kanaan, N. Female 24 Diffuse abdominal pain and nausea Turbid Household cat Vancomycin (IV) Ciprofloxacin (PO) Peritoneal dialysis 16 Sillery, J. Female 48 General abdominal discomfort Unknown Household cat Ampicillin (IV) Peritoneal dialysis 17 Elsey, R. M. Male 25 Abdominal pain and nausea Cloudy Household cat Cephradine (IP) Gentamicin (IP) Peritoneal dialysis 18 London, R. D. Male 54 Abdominal pain, nausea and vomiting Cloudy Household cat Vancomycin (IV) Gentamicin (IV) Peritoneal dialysis 19 Mugambi, S. M. Female 36 Abdominal pain, nausea and vomiting Cloudy Household cat Vancomycin (IV, IP) Gentamicin (IV, IP) Peritoneal dialysis 20 Poliquin, P. G. Female 28 Severe abdominal pain Cloudy Household cat Cefazolin (IP) Tobramycin (IP) Ceftazidime (IP) Peritoneal dialysis 21 Poliquin, P. G. Male 37 Abdominal pain Cloudy Household cat Cefazolin (IP) Tobramycin (IP) Peritoneal dialysis 22 Poliquin, P. G. Male 41 Abdominal pain, nausea, vomiting and diarrhea Cloudy Household cat Cefazolin (IP) Tobramycin (IP) Peritoneal dialysis 23 Poliquin, P. G. Female 51 Abdominal pain, nausea and vomiting. Cloudy Household cat Cefazolin (IP) Tobramycin (IP) Peritoneal dialysis 24 Poliquin, P. G. Female 37 Abdominal pain, chills and diarrhea. Cloudy Household cat Cefazolin (IP) Ceftazidime (IP) Peritoneal dialysis 25 Poliquin, P. G. Female 59 Abdominal pain, nausea and vomiting Unknown Household cat Cefazolin (IP) Tobramycin (IP) Peritoneal dialysis 26 Poliquin, P. G. Female 69 Abdominal pain Cloudy Household cat Cefazolin (IP) Tobramycin (IP) Peritoneal dialysis 27 Van Langenhove, G. Female 22 Heavy abdominal pain Cloudy Household cat Vancomycin (IP) Amikacin (IP) Ciprofloxacin (PO) Peritoneal dialysis 28 Weiss, G. A. Male 57 Diffuse abdominal pain Cloudy Household cat Vancomycin (IP) Ceftazadime (IP) Peritoneal dialysis 29 This case Male 75 Abdominal pain Cloudy Household cat Ampicillin/sulbactam (IV) Peritoneal dialysis IV Intravenously, IP Intraperitoneal, PO per os Table 2 Key altered dates of changing selected antibiotics and methods Key Altered Dates Selected Antibiotics Orally Intraperitoneally Intravenously 2019/8/9 – Vancomycin (1.0 g intraperitoneally) in an 8-h dwell once at night and ceftazidime (0.25 g intraperitoneally) in a 3-h dwell four times every day Levofloxacin (0.5 g intravenously) every day 2019/8/15 – Vancomycin (1.0 g intraperitoneally) in an 8-h dwell once at night – 2019/8/18 – – Stop levofloxacin and change to meropenem (0.5 g intravenously) every 12 h 2019/8/19 – Vancomycin (1.0 g intraperitoneally) in an 8-h dwell once at night, stop ceftazidime and change to Amikacin (200 mg intraperitoneally) in a 3-h dwell four times every day – 2019/8/22 – Stop Amikacin – 2019/8/23 – – Stop meropenem and change to cefoperazone/sulbactam (1.5 g intravenously) every 12 h 2019/8/26 – Imipenem/cilastatin (500 mg intraperitoneally) in a 6-h dwell every day – 2019/8/30 – – Stop cefoperazone/sulbactam and change to ampicillin/sulbactam (3 g intravenously) twice every day 2019/9/11 – Stop imipenem/cilastatin – 2019/9/16 Discharge and continue amoxicillin (0.25 g orally) three times a day for another 6 days – –
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| 0.959961
|
sec[1]/p[3]
|
en
| 0.999998
|
32192435
|
https://doi.org/10.1186/s12882-020-01765-1
|
[
"peritoneal",
"dialysis",
"abdominal",
"household",
"pain",
"cloudy",
"vancomycin",
"intravenously"
] |
[
{
"code": "DC5Z",
"title": "Diseases of peritoneum, unspecified"
},
{
"code": "DC50.Z",
"title": "Peritonitis, unspecified"
},
{
"code": "2F94",
"title": "Neoplasms of unknown behaviour of peritoneum"
},
{
"code": "DC51.Y",
"title": "Other specified disorders of peritoneum or retroperitoneum"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Diseases of peritoneum, unspecified (DC5Z)】
Synonyms: peritoneal disease
Hierarchy: Diseases of the digestive system (13) → Diseases of peritoneum → Diseases of peritoneum, unspecified
【2. Peritonitis, unspecified (DC50.Z)】
Synonyms: Peritonitis | peritoneum inflammation | peritonitis of undetermined cause | peritonitis of unspecified cause | pelviperitonitis
Hierarchy: Diseases of the digestive system (13) → Diseases of peritoneum → Peritonitis (DC50) → Peritonitis, unspecified
【3. Neoplasms of unknown behaviour of peritoneum (2F94)】
Synonyms: peritoneum tumour NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of peritoneum
【4. Other specified disorders of peritoneum or retroperitoneum (DC51.Y)】
Synonyms: Abdominal granuloma | Peritoneal granuloma | Epiploic appendagitis | Male frozen pelvis | Mesenteric cyst
Hierarchy: Diseases of the digestive system (13) → Diseases of peritoneum → Certain specified disorders of peritoneum or retroperitoneum (DC51) → Other specified disorders of peritoneum or retroperitoneum
|
DC5Z
|
Diseases of peritoneum, unspecified
|
Under general anesthesia, the patient was placed in supine position. Mobilization of the stomach and en bloc systematic lymph node dissection were performed via five trocars under a pneumoperitoneum . Sufficient lymphadenectomy is performed, and the stomach is transected. The resected specimen is removed through the extended umbilical incision, using a large plastic bag. An approximately 3–4-cm longitudinal incision was made to remove the specimen. The extended umbilical incision normally shrinks well within a few months . The detailed lymphadenectomy and resection procedure was described in our previously published articles [ 8 – 10 ]. Fig. 1 Trocar placement and incision. a Location of trocar placement and incision. b Postoperative view of the abdominal wound Methods of intracorporeal gastrointestinal reconstruction after total gastrectomy (Roux-en-Y) Mechanical stapler methods: conventional circular stapler-anvil method (type A): The stomach was lifted up, and a purse-string suture was placed at 1 cm above the predetermined transected line . A hole was made at the esophagogastric junction using the Harmonic scalpel. The anvil was introduced into the esophageal stump through the hole, and the purse-string suture was tied . The esophagogastric junction was divided, and the stomach was extracted. The circular stapler was introduced into the jejunum through the jejunal stump . The circular stapler attached with the anvil and fired . The jejunal stump was closed with endoscopic linear staplers. Linear stapler method (type B): A small opening was made 10 cm from the stump on the distal jejunum , and the latter was then pulled up to the esophagus, in which a small side opening was also made . A side-to-side antiperistaltic esophagojejunostomy was then performed using linear staplers , and then, the entry hole and esophagus were closed using staplers . Fig. 2 Intracorporeal conventional circular stapler-anvil end-to-side esophagojejunostomy. a The purse-string suture was placed on the esophagus. b The anvil was introduced into the esophageal stump. c The circular stapler was introduced into the jejunum through the jejunal stump and attached with the anvil. d The circular stapler fired and completed the esophagojejunostomy Fig. 3 Intracorporeal linear stapler side-to-side esophagojejunostomy. a One hole was created on the posterior wall of the esophageal stump. b The other hole was created on the antimesenteric side of the efferent jejunal. c Each jaw of the linear stapler was inserted into the holes on the esophageal stump and the jejunum, and then, the linear stapler fired. d The entry hole and esophagus were closed using staplers Hand-sewn methods (type C): The jejunal loop was brought up to reach the esophageal stump. The jejunum was anchored to the esophageal stump by several serosal muscularis interrupted sutures placed to the posterior layer of the esophageal stump . Two small holes were created: one on the antimesenteric side of the jejunum and the other on the esophageal stump . The posterior wall was closed by several full-thickness interrupted sutures , and closure of the anterior wall was carried out by a full-thickness continuous suture . The seromuscular layer was strengthened with interrupted sutures to reduce tension . Fig. 4 Intracorporeal hand-sewn end-to-side esophagojejunostomy. a The jejunum and esophageal stump attached to each other with seromuscular sutures. b A 2-cm-wide incision at the antimesenteric side of the jejunum. c Suture of the posterior wall using interrupted sutures. d Suture of the anterior wall using a continuous suture. e Strengthening of the seromuscular layer with interrupted sutures. f Complete esophagojejunostomy Methods of intracorporeal gastrointestinal reconstruction after distal gastrectomy Mechanical stapler methods: linear stapler delta-shaped method (Billroth I, type D): Small holes were then created along the edge of the gastric stump and duodenal stump . Then, they were approximated and joined with the endoscopic linear stapler . The staple line was then inspected for any defects, and hemostasis was verified. Stay sutures were placed to lift the common opening, which was then closed with two applications of the linear stapler . Linear stapler side-to-side method (Billroth II, type E): Two access openings were created: one on the posterior wall of the gastric stump 2 cm towards the cutting margin and the other on the antimesenteric side of the efferent jejunal (15 cm distal to the ligament of Treitz) . One of the endoscopic linear stapler legs was inserted into the jejunum opening to draw the jejunum to the rear of the gastric stump. Then, the second leg was inserted into the stomach opening and fired . The common opening was closed with a continuous hand-sewn suture . Fig. 5 Linear stapler delta-shaped gastroduodenostomy. a Two small holes were created for jaw inserting. b The gastric stump and duodenal stump were approximated and joined with the endoscopic linear stapler. c The common opening was closed with two applications of the linear stapler. d Completed gastroduodenostomy Fig. 6 Linear stapler side-to-side gastrojejunostomy. a One hole was created on the posterior wall of the gastric stump. b The other hole was created on the antimesenteric side of the efferent jejunal. c Endoscopic linear stapler completing the anastomosis. d Laparoscopically closed common opening sewn by hand Hand-sewn methods: gastrojejunostomy (Roux-en-Y, type F): A detachable laparoscopic intestinal clamp was placed at the greater curvature side of the gastric stump and transected with ultrasonic coagulating shears . The jejunal loop was introduced to approach the gastric stump. The details of hand-sewn gastrojejunostomy were similar to those of type C . Finally, a side-to-side jejunojejunostomy was performed through the enlarged umbilical incision. Gastroduodenostomy (Billroth I, type G): Two detachable laparoscopic intestinal clamps were placed at the pylorus and duodenum to avoid contamination. The duodenum was divided perpendicularly with ultrasonic coagulating shears between the two detachable clamps . The gastric stump was introduced to approach the duodenal stump. Then, several serosal muscularis interrupted sutures were made which are located at the rear part of the gastric and duodenal stump. A 3–4-cm-wide incision was made at the greater curvature side of the gastric stump for end-to-end gastroduodenostomy . The posterior wall of the esophagojejunostomy was sutured using interrupted sutures, and the anterior wall was sutured using a continuous suture . The seromuscular layer was strengthened with interrupted sutures to reduce tension . Gastrojejunostomy (Billroth II, type H): The jejunum loop 15 cm distal to the ligament of Treitz was introduced to approach the gastric stump. Then, several serosal muscularis interrupted sutures were made which are located at the rear part of the jejunum and gastric stump. A 3–4-cm-wide incision was made at the antimesenteric side of the jejunum for end-to-side gastrojejunostomy. The details of hand-sewn gastrojejunostomy were similar to those described above . Fig. 7 Intracorporeal hand-sewn end-to-side gastrojejunostomy. a Transection of the gastric stump with ultrasonic coagulating shears. b Suture of the posterior wall using interrupted sutures. c Suture of the anterior wall using a continuous suture. d Completed gastrojejunostomy Fig. 8 Intracorporeal hand-sewn end-to-end gastroduodenostomy. a Transection of the duodenum with ultrasonic coagulating shears between two clamps. b Ready for anastomosis after transection of the gastric stump. c Suture of the anterior wall using a continuous suture. d Completed gastroduodenostomy Fig. 9 Intracorporeal hand-sewn end-to-side gastrojejunostomy. a Transection of the gastric stump with ultrasonic coagulating shears. b Suture of the posterior wall using interrupted sutures. c Suture of the anterior wall using a continuous suture. d Completed gastrojejunostomy
| 4.113281
| 0.671387
|
sec[1]/sec[1]/p[0]
|
en
| 0.999995
|
27094509
|
https://doi.org/10.1186/s12957-016-0868-7
|
[
"stump",
"side",
"stapler",
"suture",
"linear",
"gastric",
"using",
"wall"
] |
[
{
"code": "2F72.1",
"title": "Spitzoid tumour of uncertain malignant potential"
},
{
"code": "NE85.4",
"title": "Infection of amputation stump"
},
{
"code": "8A0Y",
"title": "Other specified movement disorders"
},
{
"code": "NE85.6",
"title": "Other or unspecified complications of amputation stump"
},
{
"code": "NE85.5",
"title": "Necrosis of amputation stump"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Spitzoid tumour of uncertain malignant potential (2F72.1)】
Definition: A spindle cell and epithelioid cell melanocytic neoplasm in which there are sufficient features distinguishing it from a benign Spitz naevus to cast doubt on its benign nature. These atypical features include development in adult life, asymmetry, large diameter (>6 and especially >10 mm), significant thickness (particularly subcutaneous extension), lack of “maturation” and nodule formation, cytolo...
Synonyms: STUMP - [Spitzoid tumour of uncertain malignant potential] | Atypical Spitz naevus | atypical Spitz tumour | atypical Spitz tumour of uncertain malignant potential
Hierarchy: Neoplasms (02) → Neoplasms of uncertain behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of uncertain behaviour of skin (2F72) → Spitzoid tumour of uncertain malignant potential
【2. Infection of amputation stump (NE85.4)】
Synonyms: stump infection | infection or inflammation of amputation stump
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injury or harm arising from surgical or medical care, not elsewhere classified → Complications peculiar to reattachment or amputation (NE85) → Infection of amputation stump
【3. Other specified movement disorders (8A0Y)】
Synonyms: Other movement disorders, not elsewhere classified | chorea NOS | Familial congenital mirror movements | Primary progressive freezing gait | Ramsay Hunt cerebellar syndrome
Hierarchy: Diseases of the nervous system (08) → Movement disorders → Other specified movement disorders
【4. Other or unspecified complications of amputation stump (NE85.6)】
Synonyms: Abnormal or painful amputation stump | Amputation stump contracture | Amputation stump oedema | Oedema of amputation stump | Contracture of next proximal joint of amputation stump
Excludes: Phantom limb syndrome
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injury or harm arising from surgical or medical care, not elsewhere classified → Complications peculiar to reattachment or amputation (NE85) → Other or unspecified complications of amputation stump
【5. Necrosis of amputation stump (NE85.5)】
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injury or harm arising from surgical or medical care, not elsewhere classified → Complications peculiar to reattachment or amputation (NE85) → Necrosis of amputation stump
|
2F72.1
|
Spitzoid tumour of uncertain malignant potential
|
Patient 2, male, 52 years old, in August 2019, had slurred speech, difficulty swallowing, and general weakness without obvious reasons or incentives. On August 3, 2019, he was admitted to the Affiliated Hospital of Weifang Medical College and was diagnosed with myasthenia gravis. The effect of treatment was considered acceptable. During this period, CT was performed to identify a thymic tumor. The patient was admitted to our hospital on August 13, 2019. After completing relevant examinations, the patient underwent anterior mediastinal tumor resection. During surgery, there was a hard mass in the left anterior mediastinum, approximately 5 × 3 × 3 cm, with an incomplete capsule and a relatively fixed base. In addition, there was obvious external invasion on the surface of the left lung pleura, and the surface of the visceral pleura was scattered with small nodules. A piece of the pleural nodule was taken during the operation. Rapid pathological examination revealed malignant tumors, and the possibility of an invasive thymoma and malignant mesothelioma was high. Intraoperative diagnosis of thymic carcinoma with extensive pleural metastasis. Partial resection of the primary tumor in the mediastinum was performed and the pleural nodules were coagulated, cauterized, and washed with distilled water and lobaplatin (50 mg). Postoperative pathology revealed a thymoma (type B3, volume 5 × 3 × 0.5 cm). The postoperative recovery was good, and myasthenia gravis was significantly relieved. Subsequently, the patient underwent local radiotherapy from October 2019 to November 2019. After irregular reexamination, the patient’s condition was stable. In June 2021, the patient experienced persistent pain and discomfort in the left quarter-rib area. On August 5, 2021, an outpatient CT scan of the chest, abdomen, and pelvis showed that after thymic carcinoma, the left anterior mediastinal nodule and left pleura showed nodular thickening and metastasis. After admission to our department, CT-guided pleural puncture biopsy was performed on August 16, 2021. The postoperative pathological report showed: (left pleural puncture tissue) combined with a medical history consistent with thymoma, type B3. Immunohistochemical results: CK broad (+), Vimentin (partial +), P63 (+), CK5/6 (+), TdT (partial +), CD5 (partial +), CD117 (A little +), CK7 (individual +), CD3 (partial +), CD20 (a small amount +), CD1a (partial +), CD99 (partial +), Calretinin (a little +), MLH1 (+), MSH2 (+), MSH6 (+), PMS2 (+), Ki-67 index (60%). PD-L1(22C3)CPS = number of PD-L1 positive cells (tumor cells, lymphocytes, and macrophages)/total number of viable tumor cells × 100 = 55. Subsequently, according to the patient’s condition, diagnosis, and treatment guidelines, after discussion in the department, it was recommended that the patient combine immunotherapy with chemotherapy; however, this patient refused. From August 18, 2021, the patient was treated with one cycle of docetaxel and cisplatin, and the process proceeded smoothly. In the second cycle, the patients and their families requested additional immunotherapy; therefore, from September 11, 2021, they were treated with docetaxel d1, cisplatin d2, and tislelizumab 200 mg d3 in one cycle. Myocardial enzyme spectrum, hs-CTNI, BNP, and liver function were all normal before drug administration. On September 13, 2021, the laboratory test BNP 188.00 pg/mL, myocardial enzyme spectrum: LDH 245 U/L, HBDH 192 U/L, hs-CTNI and liver function were normal. On October 1, 2021, the patient underwent cervicothoracic, abdominal, and pelvic CT and cranial MRI to evaluate the condition, and the efficacy was evaluated by partial response (PR). The lung lesions and left pleura gradually reduced. The patient complained of chest tightness and suffocation after reexamination, and he did not experience any other discomfort. The laboratory examination results after admission were October 4, 2021. ALT 205 U/L, AST 605 U/L, LDH 1388 U/L, HBDH 1339 U/L, CK 9290 U/L, CK-MB 146.87 ng/mL, hs-CTNI 18585.3 pg/mL, BNP 145.00 pg/mL, the patient was given an ECG showing complete right branch block. Urgently seek consultation from the Department of Cardiology and Critical Care Medicine, consider immune myocarditis and immune hepatitis, give liver-protective drugs such as glutathione and magnesium isoglycyrrhizinate; give vitamin C, coenzyme Q10, and creatine phosphate sodium to nourish the myocardium; and recombinant human brain natriuresis peptide and isoproterenol pump therapy. According to the 2020 version of the Chinese expert consensus on the monitoring and management of immune checkpoint inhibitor-related myocarditis, the patient was given methylprednisolone 1 g qd pulse therapy for 3 to 5 days , and human immunoglobulin were given at the same time . To prevent gastrointestinal bleeding, secondary fungi, bacteria, pneumocystis pneumonia, osteoporosis, deep vein thrombosis caused by high-dose hormone application, compound trimoxazole, cimetidine, and potassium citrate were added. October 6, 2021 Myocardial enzyme spectrum: LDH 1458 U/L, HBDH 1400 U/L, CK 3365 U/L, CK-MB 136.46 ng/mL, hs-CTNI 11594.2 pg/mL, BNP 295.00 pg/mL. October 7, 2021 Myocardial enzyme spectrum: AST 164 U/L, LDH 1212 U/L, HBDH 1303 U/L, CK 1842 U/L, CK-MB 105.07 ng/mL, hs-CTNI 9713.2 pg/mL, BNP 1091.00 pg/mL, 2021-10-07 ECG: accelerated junctional escape rhythm, atrioventricular block (occasionally sinus), intraventricular block, QTC interval > 470 ms, and increasing r-wave in the chest leads bad. The patient’s myocardial enzyme spectrum was lower than before, and the BNP level was significantly increased. The patient was considered to have acute heart failure, and torsemide 20 mg bid was added to diuretic treatment. October 8, 2021 Liver function: ALT 222 U/L, AST 160 U/L, BNP 1293.00 pg/mL, myocardial enzyme spectrum: LDH 1109 U/L, HBDH 1214 U/L, CK 1879 U/L, CK-MB 110.26 ng/mL, hs-CTNI 12802.5 pg/mL. The patient’s myocardial enzyme spectrum continued to rise over the past 2 days, and the patient’s symptoms did not improve significantly. Considering that the current treatment effect is is poor, mycophenolate mofetil 1.0 g bid was added according to the guidelines. October 10, 2021 Liver function: ALT 195 U/L, AST 171 U/L, LDH 1070 U/L, HBDH 1075 U/L, CK 1244 U/L, CK-MB 115.92 ng/mL, hs-CTNI 16632.9 pg/mL. After symptomatic treatment, the patient’s myocardial enzyme spectrum decreased in a short time, and liver function improved slightly. From October 8, 2021, the patient’s myocardial enzyme spectrum and BNP increased sharply again, and he was administered mycophenolate mofetil 1 g bid orally several times during the period. Dynamic monitoring of the ECG changes indicated that the atrioventricular block had worsened. After active continuous ECG monitoring, oxygen inhalation, myocardial nutrition, cardiotonics, diuretics, liver protection, and other drug treatments, the patient remained palpitated, chest tight, and suffocated. Progressive aggravation, inability to lie down, right eyelid drooping. On October 10, 2021, the patient’s family requested automatic discharge after careful consideration. After discharge, the patient received oral methylprednisolone 150 mg/day, a weekly dose reduction of 20 mg, mycophenolate mofetil 1 g bid, coenzyme Q10 nutrition for cardiac muscle, spironolactone diuretic treatment, glycyrrhizic acid diamine enteric-coated capsules, and bicyclol tablets for liver protection. The patient stopped mycophenolate mofetil on November 1, 2021, and the hormone was gradually reduced. On January 17, 2022, the patient’s liver function returned to normal. Since the patient did not reexamine troponin level, his specific condition was unknown, and reexamination CT showed that the tumor was in a stable condition. However, to date, the patient remains alive and in good physical condition, with an ECOG score of 2. In Figure 2 , we show the ALT, AST, BNP, hs-CTNI, and ECG of patient 2 during treatment.
| 3.90625
| 0.983887
|
sec[1]/sec[1]/p[0]
|
en
| 0.999996
|
36401466
|
https://doi.org/10.1097/MD.0000000000031873
|
[
"myocardial",
"october",
"enzyme",
"spectrum",
"liver",
"partial",
"ctni",
"august"
] |
[
{
"code": "BD1Z",
"title": "Heart failure, unspecified"
},
{
"code": "BA41.Z",
"title": "Acute myocardial infarction, 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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Heart failure, unspecified (BD1Z)】
Synonyms: myocardial failure | cardiac decompensation | cardiac failure | cardiac failure NOS | heart failure NOS
Hierarchy: Diseases of the circulatory system (11) → Heart failure → Heart failure, unspecified
【2. Acute myocardial infarction, unspecified (BA41.Z)】
Synonyms: Acute myocardial infarction | cardiac attack | heart attack | acute cardiac infarction | acute MI - [myocardial infarction]
Hierarchy: Ischaemic heart diseases → Acute ischaemic heart disease → Acute myocardial infarction (BA41) → Acute myocardial infarction, unspecified
【3. Coronary atherosclerosis, unspecified site (BA52.Z)】
Synonyms: Coronary atherosclerosis | cardiac sclerosis | coronary artery atherosclerosis | coronary artery sclerosis | atherosclerotic coronary artery disease
Hierarchy: Ischaemic heart diseases → Chronic ischaemic heart disease → Coronary atherosclerosis (BA52) → Coronary atherosclerosis, unspecified site
【4. Structural developmental anomaly of heart or great vessels, unspecified (LA8Z)】
Synonyms: Heart malformations | Cardiac malformations | congenital anomaly of heart | congenital heart disease | congenital malformation of heart
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the circulatory system → Structural developmental anomaly of heart or great vessels → Structural developmental anomaly of heart or great vessels, unspecified
【5. Ischaemic heart diseases, unspecified (BA6Z)】
Synonyms: Ischaemic heart disease NOS | cardiac ischaemia, NOS | IHD - [ischaemic heart disease], NOS | cardiac ischemia | myocardial ischaemia
Hierarchy: Diseases of the circulatory system (11) → Ischaemic heart diseases → Ischaemic heart diseases, unspecified
|
BD1Z
|
Heart failure, unspecified
|
A 65-year-old woman was diagnosed with bladder cancer 6 years earlier, for which she had undergone transurethral resection six times. Oophorectomy for a right ovarian cyst and total hysterectomy for endometriosis had been performed under general anesthesia at the ages of 30 and 35 years, respectively. During the current surgery, she underwent radical cystectomy with creation of an ileal conduit and removal of pelvic lymph nodes. Her past history of multiple laparotomies resulted in intestinal adhesions and massive intraoperative bleeding. The total blood loss during surgery was 5340 ml. She received 1200 ml of autologous blood transfusion, and subsequent transfusion of 560 ml of red cell concentrates and 1200 ml of fresh-frozen plasma in the operation room. The surgical time was 6 h and 49 min. Administration of the antibiotic isepamicin (ISP: 200 mg) was started to treat a fever of 40 °C immediately after moving her to the ward. However, she developed a shock state 16 h after the operation . Her systolic blood pressure decreased to approximately 70 mmHg and urine output was less than 25 ml/h. Infusions of Ringer’s solution, albumin preparations, immunoglobulins, and vasopressors were started because we suspected septic shock. Administration of imipenem/cilastatin sodium (IPM/CS: 500 mg) was added to ISP because we thought that more intensive empiric antimicrobial therapy was necessary. Two days after the operation, she was transferred to the intensive care unit (ICU) because of deterioration in her respiratory and circulatory condition. Soon after moving her to the ICU, a subcutaneous hemorrhage-like skin rash appeared and extended rapidly over her left side . On admission to the ICU, blood tests indicated severe metabolic acidosis, liver and renal dysfunction, and signs of disseminated intravascular coagulation (DIC) (Table 1 ). Her APACHE II (Acute physiology and chronic health evaluation) and SOFA (Sequential organ failure assessment) scores at this time were 24 and 14, respectively. An X-ray examination and computed tomography (CT), which was performed on postoperative day 2, indicated uninterrupted massive emphysematous tissue from her left chest to lower abdomen . Suspecting necrotizing fasciitis or gas gangrene, we performed emergency fasciotomy. Subsequently, multidisciplinary treatment, including mechanical ventilation, hyperbaric oxygen therapy (HBOT), polymyxin B-immobilized fiber column direct hemoperfusion (PMX-DHP), and continuous hemodiafiltration (CHDF), was started. Antibiotic therapy was changed to clindamycin (CLDM: 900 mg), vancomycin , and IPM/CS . Gram-positive bacilli, but not Gram-negative bacteria, were detected by microscopic examination of blister fluid aspirated from the skin rash. Moreover, a culture test detected Clostridium perfringens ( C. perfringens ) in a wound abscess that was removed during the emergency fasciotomy. Based on these observations, she was diagnosed with gas gangrene. HBOT was performed on the first and second ICU days. We had to abandon plans for a second fasciotomy because the area that required treatment was too large. CHDF was continued through her ICU stay, although PMX-DMP was performed only once on the first ICU day. Her APACHE II and SOFA scores continued to be flat during the first 2 weeks, indicating that the initial treatment prevented further clinical deterioration . However, she suffered from secondary infection from the 3rd week onward due to infection with multiple pathogenic bacteria, including Candida albicans and Pseudomonas aeruginosa , as shown in Table 2 . Finally, she died of sepsis 38 days after the operation in spite of prompt diagnosis and intensive therapy for the gas gangrene. Fig. 1 Vital signs of the patient on the day of and after the surgery. The black and brown lines indicate systolic and diastolic blood pressure, respectively. Closed circles and open squares indicate body temperature and heart rate, respectively. DOA dopamine hydrochloride Fig. 2 Images of the patient. a A diffuse skin rash developed over the left side of her trunk 2 days after the surgery. b Abdominal X-ray taken immediately after ICU admission showed soft tissue swelling with the density of air on the left abdominal wall ( arrows ). c Computed tomography of the abdomen taken 2 days after the surgery showed subcutaneous emphysema over the left side of the trunk ( arrows ). d Enhanced computed tomography of the abdomen taken 1 day after the surgery. The rectangle indicates the area shown in e . e The arrows indicate air-densities between the subcutaneous fat tissue and muscle layer in the vicinity of the left drainage tube Table 1 Results of blood tests performed immediately after ICU admission Blood count Hct (%) 28.5 Hb (g/dl) 9.2 WBC (/μl) 7500 Plt (/μl) 7.1 × 10 4 Blood coagulation tests PT (%) 37 APTT (s) 59.3 Fibrinogen (mg/dl) 385 FDP (μg/ml) 47.3 d -dimer (μg/ml) 39.1 TAT (ng/ml) 36.1 PIC (μg/ml) 1.3 Biochemical tests T-bil (mg/dl) 6.2 AST (IU/l) 376 ALT (IU/l) 41 LDH (IU/l) 3067 BUN (mg/dl) 49 Cr (mg/dl) 2.23 CRP (mg/dl) 24.24 Blood gas analysis pH 7.265 pCO 2 14.3 pO 2 (FM 8L) 91.7 HCO 3 − (mmol/l) 10.5 BE (mmol/l) −20.6 Hct hematocrit, Hb hemoglobin, WBC white blood cells, Plt platelets, PT prothrombin time, APTT activated partial thromboplastin time, FDP fibrin/fibrinogen degradation products, TAT thrombin-antithrombin complex, PIC plasmin-α2 plasmin inhibitor complex, T-bil total bilirubin, AST aspartate aminotransferase, ALT alanine aminotransferase, LDH lactate dehydrogenase, BUN blood urea nitrogen, Cr creatinine, CRP C-reactive protein, pCO 2 carbon dioxide partial pressure, pO 2 oxygen partial pressure, BE base excess Fig. 3 The clinical course of the patient. FOM fosfomycin, ISP isepamicin, IPM/CS imipenem/cilastatin, CLDM clindamycin, VCM vancomycin, FCZ fluconazole, CPFX ciprofloxacin, MEPM meropenem, CAZ ceftazidime, HBOT hyperbaric oxygen therapy, HD hemodialysis, CHDF continuous hemodiafiltration, APACHE acute physiology and chronic health evaluation, SOFA sequential organ failure assessment Table 2 List of the bacteria that were isolated and results of antimicrobial susceptibility testing Species Bacteria count Sampling site Date of collection Reporting date Antimicrobial susceptibility testing Susceptible Intermediate Resistant Clostridium perfringens 2+ Closed abscess POD2 POD6 ABPC, ABPC/SBT, CMZ, FMOX, IPM, MINO LVFX CLDM Small number Drain POD2 POD9 ABPC/SBT, IPM, MINO ABPC, CMZ, FMOX, LVFX CLDM Pseudomonas aeruginosa 3+ Debris POD14 POD17 PIPC, CPZ, CAZ, CZOP, CFS, CFPM, MEPM, AZT, S/C, TOB, AMK, LVFX, CPFX IPM, GM FOM 3+ Open abscess POD24 POD28 Pseudomonas aeruginosa with metallo-β-lactamase 3+ Open abscess POD33 POD38 PIPC, CAZ, CZOP, S/C, TOB, AMK CPZ, CFS, CFPM, GM IPM, MEPM, AZT, LVFX, CPFX, FOM Candida albicans 2+ Debris POD14 POD17 N/A N/A N/A 2+ Open abscess POD24 POD28 N/A N/A N/A Staphylococcus epidermidis N/A CV catheter POD8 POD15 VCM, TEIC, LZD N/A PCG, ABPC, MPIPC, CEZ, CTM, CPR, FMOX, IPM, GM, EM, CLDM, LVFX, ST, FOM N/A Venous blood, CV catheter POD14 POD17 VCM, TEIC N/A PCG, ABPC, MPIPC, CEZ, CTM, CPR, FMOX, IPM, GM, EM, CLDM, LVFX, ST, FOM Stenotrophomonas maltophilia 3+ Open abscess POD24 POD28 MINO, ST LVFX CAZ 3+ Open abscess POD33 POD38 MINO, ST N/A CAZ, LVFX Enterococcus casseliflavus 3+ Open abscess POD33 POD38 VCM, TEIC, LZD N/A LVFX, ABPC, PCG, EM POD post-operative days, ABPC ampicillin, ABPC/SBT ampicillin/sulbactam, CMZ cefmetazole, FMOX flomoxef, IPM imipenem, MINO minocycline, PIPC piperacillin, CPZ cefoperazone, CAZ ceftazidime, CZOP cefozopran, CFS cefsulodin, CFPM cefepime, MEPM meropenem, AZT aztreonam, S/C sulbactam/cefoperazone, TOB tobramycin, AMK amikacin, LVFX levofloxacin, VCM vancomycin, TEIC teicoplanin, LZD linezolid, ST sulfamethoxazole–trimethoprim, GM gentamicin, CLDM clindamycin, FOM fosfomycin, CPFX ciprofloxacin, PCG benzylpenicillin, MPIPC oxacillin, CEZ cephazolin, CTM cefotiam, CPR cefpirome, EM erythromycin
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https://doi.org/10.1186/s13104-016-2194-0
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[
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[
{
"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 CLASSIFICATION ═══
【1. Diseases of the blood or blood-forming organs, unspecified (3C0Z)】
Synonyms: Blood, lymph or spleen disease | blood condition NOS | blood disorder NOS | bone marrow disease NOS | haematologic disease NOS
Hierarchy: Diseases of the blood or blood-forming organs (03) → Diseases of the blood or blood-forming organs, unspecified
【2. Haematuria, unspecified (MF50.4Z)】
Synonyms: Haematuria | blood in urine | urinary blood | haematuria NOS | urinary tract haemorrhage NOS
Hierarchy: Symptoms, signs or clinical findings involving the urinary system → Abnormal micturition (MF50) → Haematuria (MF50.4) → Haematuria, unspecified
【3. Finding of cocaine in blood (MA12.1)】
Synonyms: cocaine in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of cocaine in blood
【4. Finding of steroid agent in blood (MA12.4)】
Synonyms: steroid in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of steroid agent in blood
【5. Finding of hallucinogen in blood (MA12.2)】
Synonyms: hallucinogen in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of hallucinogen in blood
|
3C0Z
|
Diseases of the blood or blood-forming organs, unspecified
|
Subject 1A was first examined in our clinic at the age of 11 years. He was born after uncomplicated pregnancy and underwent pyloromyotomy due to hypertrophic pyloric stenosis in the newborn period. Otherwise, he was a healthy boy with a normal development. The family history was positive for arthritis/gout but negative for aortic aneurysms/dissections and sudden cardiac deaths. At the age of 9 years, he developed painless swelling of the metacarpophalangeal joints (MCP) of the right hand. Juvenile idiopathic arthritis (JIA) was suspected by the age of 10 years. An MRI scan of the hand revealed synovitis and effusion of the MCP3 joint. Antinuclear antibodies (ANA) were positive (maximum titer 1:2,560), whereas rheumatoid factors and Human Leukocyte Antigen (HLA) B27 could not be detected. The patient showed progressive arthritis of further MCP joints of both hands and the wrist of the right hand. He was treated with nonsteroidal anti-inflammatory drugs (NSAID) and methotrexate. Further MRI scans showed necrosis of the medial sesamoid bone of the left first toe and bursitis trochanterica as well as mild synovialitis of the left hip joint. At the age of 11 years, the clinical examination revealed arachnodactyly with positive wrist and thumb signs. X-ray of his hands detected arthritis-associated destruction of the metacarpophalangeal joints of the fingers and the distal radioulnar joint. Subluxation of the carpometacarpal joints of both thumbs was observed . In consequence, the therapy was extended by Adalimumab (TNF-α monoclonal antibody). One year later, no clinical improvement was observed and the treatment was changed to Etanercept (TNF-α inhibitor). Furthermore, at the age of 13 years the clinical examination revealed kyphoscoliosis and pectus excavatum. His height exceeded the 99th percentile in the toddler period and reached 178 cm (+ 1.8 SD, > 95P) by the age of 13 years 19 . His arm span to height ratio was 1.07. An MRI scan of the spine revealed dural ectasia, anterospondylolisthesis, and vertebral osteochondrosis. Significant aortic root dilatation was excluded by transthoracic echocardiography . Repeated ophthalmologic investigations remained unremarkable. The diagnosis of hereditary connective tissue disorder (HCTD) was considered and discussed. According to the revised Ghent nosology 12 , this patient reaches a systemic score of 7, which led to the diagnosis of a MASS-like phenotype (Table 1 ). Gene panel sequencing revealed the heterozygous missense variant c.3936G > T p. in COL2A1 (Table 1 ), which was classified as LPV (class 4) according to the ACMG/AMP guidelines 17 . Rules, considerations and data for assigning classification criteria are outlined in the Supplementary Information (Supplementary Results and Table S2 ). Figure 2 Selected clinical features of the presented patients. ( a ) Photographs, X-ray image and transthoracic echocardiography in parasternal long-axis of subject 1A. Note the long and slender fingers (arachnodactyly) and camptodactyly of digit V of the right hands (upper left image) as well as the subluxation of the proximal phalanx of the right thumb (black circle, upper right image) and erosive changes of the distal epiphysis of the 3 rd and 4 th metacarpal bone (white circle, upper right image). The lower image shows normal aortic root diameter of 29 mm (Z-Score 1.3) and intact aortic shape. ( b ) Photographs of subject 1B. Note the long and slender fingers (arachnodactyly) with camptodactyly of the fifth finger. ( c ) Pedigrees of the families of subjects 1, 2 and 3. Affected individuals show a MASS-like phenotype and carry a putative disease-relevant COL2A1 variant. Identified heterozygous variants in COL2A1 are given. Parents of subjects 2 and 3 were not clinically examined and segregation analysis was not possible in the parents of subject 2. + , wild-type allele; n.d., not determined. (d) Magnetic resonance images of the lower spine and spinal cord of subject 3. Note the lumbosacral dural ectasia (black arrows). Table 1 Patients, variant classification and clinical features. Patient Subject 1A Subject 1B Subject 2 Subject 3 Sex Male Female Female Male Age at last examination 13 years 44 years 53 years 22 years Family history Positive: grandfather with arthritis/gout Positive: father with arthritis/gout Uncertain: father with suspected arthritis Uncertain: parents n.a. for clinical examination Variant classification COL2A1 variant (c. notation, p. notation) c.3936G > T (het) p. c.3936G > T (het) p. c.193G > A (het) p.(Asp65Asn) c.4013G > A (het) p. gnomAD v.2.1.1 total population allele frequency (allele count/allele number/hom) 0.000003976 (1/251,478/0) 0.000003976 (1/251,478/0) 0 (0/280,928/0) 0 (0/251,496/0) VarSome pathogenicity prediction (damaging/uncertain/tolerated) 13/1/2 13/1/2 11/1/4 3/2/11 HGMD/ClinVar N.l./N.l. N.l./N.l. N.l./VUS* N.l./N.l. ACMG/AMP classification (Criteria) LPV (PM1, PM2 † , PP2, PP3) LPV (PM1, PM2 † , PP2, PP3) LPV (PM1, PM2, PP2, PP3) VUS (PM1, PM2, PP2, BP4) Clinical features Height 178 cm (+ 1.8 SD, > 95P) 172 cm (+ 1.3 SD, > 90P) 179 cm (+ 2.4 SD, > 95P) 198 cm (+ 3.0 SD, > 95P) Tall stature Yes Yes Yes Yes Arm span/arm span to height ratio 191 cm/1.07 185 cm/1.07 N.a. N.a. Arachnodactyly Yes Yes Yes Yes Wrist and thumb sign Both positive (SySc. 3) Positve wrist sign (SySc. 1) Both positive (SySc. 3) Positive thumb sign (SySc. 1) Dural ectasia Yes (SySc. 2) N.a. Yes (SySc. 2) Yes (SySc. 2) Pectus deformity P. excavatum (SySc. 1) P. excavatum (SySc. 1) No P. carinatum (SySc. 2), Asymmetric thorax Spinal deformity Kyphosis (SySc. 1) Kyphosis (SySc. 1) Scoliosis (SySc. 1) Scoliosis (SySc. 1) Foot deformity No Pes planus (SySc. 1) Pes planus (SySc. 1) Hindfoot deformity (SySc. 2) Aortopathy No (ARD 29 mm, Z-score 1.3) No (ARD 32 mm, Z-Score 0.4) No (ARD 33 mm, Z-score 0.68) No (ARD 36 mm, Z-Score 1.2) Craniofacial features High arched palate Dolichocephaly, enophthalmus malar hypoplasia , high arched palate (SySc. 1) No Dolichocephaly, malar hypoplasia, retrognathia , high arched palate (SySc. 1) Osteoarthritis Yes Yes No No Other features anterospondylolisthesis, vertebral osteochondrosis bone odema of both feet hypermobility of knee and elbow joints, varicosis suspected bicuspid aortic valve, myopia (< 3dpt) Diagnosis according to revised Ghent nosology MASS-like (Systemic score 7) MASS-like (Systemic score 5) MASS-like (Systemic score 7) MASS-like (Systemic score 9) Patients, variant classification and clinical features. Variant details and clinical features are listed by patient. Nucleotide numbering uses + 1 as the A of the ATG translation initiation codon in the reference sequence, with the initiation codon as codon 1. gnomAD, Genome Aggregation Database (v2.1.1); allele frequency in total population is given; allele count/allele number/hom, total number of alleles/total number of analysed alleles/number of homozygous carriers. HGMD, Human Gene Mutation Database. ClinVar, database on the relationships between human variations and phenotypes. Variants were classified as recommended by ACMG/AMP: VUS, variant of uncertain significance or with conflicting interpretations of pathogenicity; LPV, likely pathogenic variant. Clinical features supporting a MASS-like phenotype are shown in bold and the respective systemic score (SySc.) according to the revised Ghent nosology is given in parentheses 12 . het, heterozygous; N.a., not available; N.l., not listed; SD, standard deviation; ARD, aortic root diameter; MASS, Myopia, mitral valve prolapse, borderline and non-progressive aortic root dilatation, skeletal findings and striae. *, variant c.193G > A p.(Asp65Asn) has previously been reported in the ClinVar database and classified as VUS. The patient’s condition and the inheritance were not recorded. † , VarSome 10.2 assigns PM2 for dominant genes if the allele count of the variant is less than 5 in the gnomAD database (for details see Supplementary Results).
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[
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[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Birth depression with 5 minute Apgar score 0-3 (KD30.0)】
Definition: A condition characterised by cardiorespiratory and neurological depression, defined as an Apgar score between 0 to 3 at 5 minutes following birth.
Hierarchy: Certain conditions originating in the perinatal period (19) → Certain disorders originating in the perinatal period → Birth depression (KD30) → Birth depression with 5 minute Apgar score 0-3
【2. Birth depression with 5 minute Apgar score 4-6 (KD30.1)】
Definition: A condition characterised by cardiorespiratory and neurological depression, defined as an Apgar score between 4 and 6 at 5 minutes following birth.
Hierarchy: Certain conditions originating in the perinatal period (19) → Certain disorders originating in the perinatal period → Birth depression (KD30) → Birth depression with 5 minute Apgar score 4-6
【3. Coma (MB20.1)】
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 ...
Synonyms: comatose | exanimation | Coma, NOS | Unconsciousness, NOS | unconsciousness
Excludes: Diabetic coma | Hepatic coma | Neonatal coma | Nondiabetic hypoglycaemic coma | chronic uremic coma
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Mental or behavioural symptoms, signs or clinical findings → Symptoms, signs or clinical findings involving consciousness (MB20) → Coma
【4. Severe birth asphyxia (KB21.0)】
Definition: Pulse less than 100 per minute at birth and falling or steady, respiration absent or gasping, colour poor, tone absent.
Synonyms: severe perinatal hypoxia | asphyxia pallida of newborn | Asphyxia with 5-minute Apgar score 0-3 | newborn severe asphyxia | newborn severe asphyxiation
Hierarchy: Certain conditions originating in the perinatal period (19) → Respiratory disorders specific to the perinatal or neonatal period → Birth asphyxia (KB21) → Severe birth asphyxia
|
KD30.0
|
Birth depression with 5 minute Apgar score 0-3
|
On 16 August 2011, a 64-year-old male physician with no relevant co-morbidity was suspected for transversal colon cancer by colonoscopy. On 26 August 2011, seven likely secondary lesions were detected by abdominal ultrasound in different hepatic segments (the biggest lesion was 42 mm in the largest diameter) and 15 ng/mL was the serum CEA level. On 29 August 2011, he underwent hemicolectomy of the transversal colon, and adeno-carcinoma, G2/G4 was found in post-operative histology. pT3 N2a (6 regional lymph-nodes involved of 22 examined) M1 was the post-operative TNM classification. Kras was wild type. From 14 October 2011 to 20 July 2012, he received 1000 sqm/day capecitabine given orally and 130 mg/sqm oxaliplatin by i.v. infusion (the Xelox regimen) on days 1–14 every 21 days (one cycle) for six cycles, plus 5 mg/kg bevacizumab by i.v. infusion every two weeks followed by maintenance therapy with capecitabine and bevacizumab. His serum CEA level, which was 52 ng/mL at the beginning of chemotherapy, decreased to 25 ng/mL in July 2012, and the WB CT showed slight decreases in all hepatic metastases. On 11 September 2012, all detectable liver metastases were surgically removed following his demand and, in October 2012, his serum CEA level was in the normal range. In December 2012, a radiological assessment documented a suspected secondary lung nodule in addition to the recovery of metastatic liver disease; the serum CEA value was 5.9 ng/mL, which increased to 66 ng/mL in January 2013. From January to April 2013, he received seven cycles of 130 mg/sqm irinotecan by i.v. infusion and 500 mg/sqm cetuximab by i.v. infusion every two weeks. On 2 July 2013, he underwent surgical removal of the left liver lobe; contemporaneously, other secondary lesions were enucleated (overall, ten liver metastases were removed); post-operative histology confirmed the secondary origin of the liver lesions, while a subsequent abdominal MRI signaled three regions with necrotic features. WB positron emission tomography (PET) was negative for residual metastatic liver disease. On 28 February 2013 and 1 July 2013, the serum CEA level was in the normal range. On 21 August 2013, thoracic CT showed an increase in the previously detected nodule placed at the inferior left lung lobe, while liver ultra-sonography showed two small hypo-echogenic areas suspected to be surgical scars. On 29 August 2013, the patient underwent atypical resection of the inferior left lung lobe and post-operative histology confirmed that it was a secondary lesion compatible with primary colorectal cancer. The serum CEA level was 9 ng/mL at the end of September/beginning of October. On 24 October 2013, WB CT signaled a new focal liver lesion that was 57 mm in the largest diameter and a 16 mm nodule at the lower-basal region of the right lung: both were suspected for relapse. Following oncologic consultation, from November 2013 to January 2014, he received cycles with FOLFIRI plus cetuximab. In January 2014, the serum CEA level was in the normal range. In March 2014, he underwent surgical removal of the liver nodule and of the other two nodules in the right lung (one of them was a pleural lesion); they were all determined to be secondary lesions in the post-operative histology. He continued to receive the same FOLFIRI treatment for a total of six cycles until June 2014. In July 2014, WB CT showed multiple new bilateral lung nodules. On 19 September 2014, he accepted the invitation to be included in an experimental clinical trial that was recruiting previously treated patients who had progressed under cetuximab after an initial benefit. He was selected because of his high MET level, as assessed in a biologic sample; therefore, he was given 120 mg of tivantinib (a MET-inhibitor) orally twice daily and cetuximab until 25 October 2016, with an initial clinical-radiological partial response (PR) followed by a stable disease as documented by WB PET carried out on 27 May 2016. In addition, WB PET showed a likely metastatic lesion of the lateral eighth right rib; therefore, from 13 June 2016 to 17 June 2016, in addition to tivantinib and cetuximab, he underwent RT at the rib lesion (25 Gy as a total dose by five fractionated doses). During treatment with tivantinib plus cetuximab, the serum CEA value, which was measured every 2–3 months, was in the normal range until 21 September 2016, when it was found to be 12 ng/mL. On 04 November 2016, WB PET documented the progression of the secondary lesion of the eighth right rib. On 2 December 2016, the patient underwent palliative surgical removal of the lateral arch of the VIII rib; post-operative histology confirmed the metastatic origin of the rib lesion with infiltration of the surgical edges. On 27 December 2016, surgical intervention for soft rib tissue radicalization and the removal of the middle lobe of the right lung was carried out; post-operative histology confirmed the metastatic origin of the residual disease. On 15 February 2017, WB CT showed multiple bilateral suspected secondary pulmonary lesions; the biggest was 7 mm in the largest diameter in the right lung. On 15 February 2017, he was given regorafenib, which he interrupted on 23 May 2017 because of strong side effects and the rising serum CEA level. On 17 August 2017, WB CT documented the progression of the disease at the level of the previously treated eighth rib lesion and the appearance of two new suspected lung lesions on the thoracic wall and lower right lobe (29 × 13 mm), respectively. The remaining multiple bilateral pulmonary lesions, the biggest of which was 8 mm in the largest diameter, were substantially stable. On 15 September 2017, WB PET signaled at least four suspected lung lesions and pathological activity of the VII, VIII, and IX right ribs. On 21 September 2017 and 20 November 2017, serum CEA values were 11.2 and 96 ng/mL, respectively. In November 2017, he again started treatment with the Xelox regimen plus cetuximab. After two cycles, oxaliplatin was interrupted because of strong side effects and he continued with capecitabine and cetuximab. On 13 February 2018, the serum CEA value was 46.3 ng/mL. In March 2018, WB PET confirmed the progression of metastatic disease in the right lung and right chest wall; on 20 March 2018, following transient ChT interruption, the patient underwent surgical removal of the lower right pulmonary lobe, multiple resections of the residual left lung, and thoracotomy of the VII, VIII, and IX right ribs with subsequent plastic surgery of the thoracic wall. The control with WB CT, which was carried out soon after surgery, showed that one metastatic lesion remained on the right lobe, in addition to the multiple repetitions of the left lung lobe. He started with capecitabine and cetuximab therapy, which he continued until March 2019. Serum CEA values, which were 4.1, 4.5, 3.6, 4, and 5.1 ng/mL on 6 April 2018, 24 May 2018, 1 August 2018, 10 October 2018, and 27 October 2018, respectively, increased to 12 ng/mL in December 2018. WB CT carried out in January 2019 signaled three new hepatic repetitions, the largest of which was about 2 cm, and two of them were close to the hepatic and cava veins, respectively, while the multiple lung repetitions were stable. At the beginning of April 2019, he underwent surgical removal of the hepatic lesions. Yet another WB CT carried out soon after surgery showed the persistence of metastatic liver disease in addition to the stable multiple lung repetitions. At the beginning of July 2019, he entered an experimental protocol with temozolomide that was interrupted at the end of August after two cycles because of the unresponsive metastatic disease. On 16 August, the serum CEA value was 86 ng/mL. On 2 September, following an oncologic consultation, he was given tas-102 therapy, which is ongoing. Although this patient is not disease-free, he is doing well with 0-1 ECOG PS 98 months after primary surgery.
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|
sec[1]/sec[8]/sec[0]/p[0]
|
en
| 0.999997
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31795079
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https://doi.org/10.3390/ijms20235986
|
[
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"august",
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[
{
"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 CLASSIFICATION ═══
【1. Other specified diseases of the respiratory system (CB40.Y)】
Synonyms: Secondary respiratory disorders | Respiratory disorders in diseases classified elsewhere | Diseases of bronchus, not elsewhere classified | Acquired bronchial diverticulum | acquired bronchus diverticulum
Hierarchy: Diseases of the respiratory system (12) → Certain diseases of the respiratory system (CB40) → Other specified diseases of the respiratory system
【2. Agenesis of lung (LA75.1)】
Definition: This refers to the absence or rudimentary residua of an undeveloped lung.
Synonyms: Pulmonary agenesis | absence of lung | aplasia of lung | apulmonism | congenital absence of lung
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the respiratory system → Structural developmental anomalies of lungs (LA75) → Agenesis of lung
【3. Pneumonia, organism unspecified (CA40.Z)】
Synonyms: Pneumonia | infectious pneumonia | PN - [pneumonia] | lobar pneumonia NOS | multifocal pneumonia
Hierarchy: Diseases of the respiratory system (12) → Lung infections → Pneumonia (CA40) → Pneumonia, organism unspecified
【4. Respiratory failure (CB41)】
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.
Synonyms: lung failure NOS | pulmonary failure
Excludes: Acute respiratory distress syndrome | Respiratory arrest | Respiratory distress of newborn
Hierarchy: Diseases of the respiratory system (12) → Respiratory failure
【5. Other injury of lung (NB32.3Y)】
Synonyms: Haematoma of lung | Traumatic hydropneumothorax | Acute traumatic lung congestion | Rupture of lung
Hierarchy: Injuries to the thorax → Injury of other or unspecified intrathoracic organs (NB32) → Certain injuries of lung (NB32.3) → Other injury of lung
|
CB40.Y
|
Other specified diseases of the respiratory system
|
Clinical indications in oncology Fig. 47 68 Ga-DOTA peptide, follow-up of NET, comparison 68 Ga-DOTANOC, 68 Ga-DOTATATE. Clinical history : 68 y.o. woman with midgut NET and liver metastases treated with octreotide. PET/CT findings : 68 Ga-DOTANOC ( a ) and 68 Ga-DOTATATE ( b ) performed 8 months apart show similar uptake in the metastatic lesions, but due to the higher liver 68 Ga-DOTATATE uptake, more metastases are identified in the 68 Ga-DOTANOC study Fig. 48 68 Ga-DOTA peptide, suspected lesion of the pancreas, specificity. Clinical history : 50 y.o. woman; a hypervascular lesion in the pancreas was incidentally found on CT ( a ). PET/CT findings : focal area of high expression of somatostatin receptor analogues in the pancreas corresponding to the CT finding ( b MIP, c fused). Surgery confirmed the presence of a well differentiated neuroendocrine tumour (Ki67 2%) Fig. 49 68 Ga-DOTA peptide, suspected lesion of the pancreas, false negative. Clinical history : 48 y.o. man with lesion in the pancreatic body ( a MRI). Clinical suspicion of insulinoma (hypoglycaemias). PET/CT findings : no uptake in the lesion ( b MIP, c CT, PET, and fused images). The patient underwent surgery, which confirmed the presence of an insulinoma (frequently false negative with 68 Ga-DOTA peptide) Fig. 50 68Ga-DOTA peptide, suspected lesion of the liver, specificity. Clinical history : 52 y.o. male with an incidental detection of a single liver lesion in the left lobe. The lesion is suspect to be a metastasis on ultrasound and CT from an unknown primary site. PET/CT findings : increased focal uptake in a loop of the ileum, suspect to be a primary tumour. Increased uptake in the left liver lobe consistent with a secondary lesion ( a MIP, b CT and fused images). After surgery, the lesions were diagnosed to be a well-differentiated NET grade 1 (Ki67 2%) Fig. 51 68 Ga-DOTA peptide, staging of endobronchial carcinoid tumour, specificity. Clinical history : 33 y.o. man. Persistent cough and wheezing not responsive to bronchodilators. CT showed endobronchial node in left main bronchus. Bronchoscopy biopsy: NET. PET/CT findings : uptake in node protruding into the left main bronchus ( a MIP, b CT and fused images). Focal uptake in the left adrenal gland ( c ). MRI confirmed the presence of an adrenal adenoma Fig. 52 68 Ga-DOTA peptide, staging of NET lung primary, comparison with FDG. Clinical history : 65 y.o. man. Staging of a lung mass with FDG PET and biopsy indicated a moderately differentiated NET (grade 2, Ki67 8%). Consequently, the patient underwent a second PET using 68 Ga-DOTANOC to stage the disease more accurately. PET/CT findings : increased focal uptake in FDG PET in the right lung, without any other findings ( a MIP). 68 Ga-DOTANOC showed intense uptake in the lung and in the thyroid due to a known De Quervain thyroiditis ( b MIP) Fig. 53 68 Ga-DOTA peptide, staging NET of the pancreas, comparison with FDG. Clinical history : 68 y.o. man with moderately differentiated multi-metastatic NET of pancreas (Ki67 8%). PET/CT findings : 68 Ga-DOTANOC PET/CT shows intense pathologic uptake of somatostatin receptor analogue by the known pancreatic tumour, as well as in lymph nodes, multiple liver lesions, and previously unknown bone lesions ( a ). FDG-PET/CT confirms pathologic uptake of the tracer in pancreas and lymph nodes and in some of the known liver and bone lesions ( b ) Fig. 54 68Ga-DOTA peptide, NET of the pancreas, suspicion of relapse, comparison with FDG. Clinical history : 63 y.o. man with known well differentiated pancreatic NET (Ki67:2%); routine follow-up CT detected a suspicious lesion in the liver ( a ); PET/CT was requested to restage the patient. PET/CT findings : 68 Ga-DOTANOC PET/CT shows only physiological uptake of somatostatin receptor analogue ( b MIP and fused PET/CT). FDG-PET/CT shows a mild pathologic uptake of the tracer in the liver and confirms liver involvement ( c MIP and fused PET/CT) Fig. 55 68 Ga-DOTA peptide, staging NET of the pancreas, sensitivity. Clinical history : 60 y.o. man. Incidental finding of a NET of the pancreas grade 2 (Ki67 5%). PET/CT findings : multiple areas of increased tracer uptake in the pancreas, liver, and abdominal lymph nodes ( a MIP). Note the uptake in a left supraclavicular lymph node 5 mm in size ( b CT and fused images) Fig. 56 68 Ga-DOTA peptide, restaging after surgery for lung carcinoid, specificity. Clinical history : 60 y.o. man; restaging after surgery for lung carcinoid. PET/CT findings : focal increased 68 Ga-DOTATOC uptake in a benign rib fracture ( a MIP, b PET and fused PET/CT, blue marker) Fig. 57 68 Ga-DOTA peptide, suspicion of NET, specificity. Clinical history : 17 y.o. boy with known Von Hippel-Lindau disease. Increased level of pancreatic polypeptide. PET/CT findings : 68 Ga-DOTANOC PET shows an intramedullary focus of increased uptake at the level of T8 ( a MIP, b PET and fused PET/CT), corresponding to a hemangioblastoma Fig. 58 68 Ga-DOTA peptide, follow-up of midgut NET, specificity. Clinical history : 69 y.o. woman with NET treated by surgery the year before. Right upper lobe lung adenocarcinoma treated by chemo- and radiation therapy 4 years prior. PET/CT findings : in addition to multifocal recurrent disease ( a MIP), 68 Ga-DOTANOC PET shows moderately increased uptake in the previously irradiated lung parenchyma ( b ) Fig. 59 68 Ga-DOTA peptide, staging NET of the pancreas, sensitivity. Clinical history : 54 y.o. man with known pancreatic NET, candidate for surgery. PET/CT findings : very high uptake in the known pancreatic lesion and as well as in previously unknown lymph nodes and bone lesions ( a MIP, b CT and fused imaging) Fig. 60 68 Ga-DOTA peptide, suspected NET of the pancreas, false positive (inflammation), compared with FDG. Clinical history : 65 y.o. man with suspected NET of the pancreas. PET/CT findings : FDG: there is no significant uptake in the pancreas. Intense symmetric uptake in mediastinal lymph nodes ( a MIP). 68 Ga-DOTANOC: there is increased symmetrical uptake in mediastinal lymph nodes but no significant uptake in the pancreas ( b MIP) Fig. 61 68 Ga-DOTA peptide, suspected recurrence of paraganglioma. Clinical history : 39 y.o. man with a previous history of paragangliomas. During follow-up CT suspected a relapse in the thorax. PET/CT findings : intense focal uptake in a para-caval round shaped lesion ( a MIP, b CT and fused PET/CT) consistent with a paraganglioma Fig. 62 68Ga-DOTA peptide, staging medullary thyroid cancer (MTC). Clinical history : 70 y.o. man with newly diagnosed MTC. Suspicion of distant metastases on CT. PET/CT findings : moderately increased uptake in the known tumour ( a MIP, b CT, PET, and fused imaging). No other suspicious lesions are seen Fig. 63 68 Ga-DOTA peptide, recurrent medullary thyroid cancer (MTC). Clinical history : 56 y.o. man with previous history of MTC, treated by surgery. Progressive increase in calcitonin . PET/CT findings : increased uptake in cervical LN ( a ) and bone lesion ( b right scapula). Additional hepatic metastases are seen on the diagnostic CT, but due to the high background they are not evident on PET images ( c ) Fig. 64 68 Ga-DOTA peptide, meningioma. Clinical history : 70 y.o. man with meningioma of the skull basis. PET/CT findings : 68 Ga-DOTATATE shows highly increased uptake in the right sphenoidal bone area with an extension to the orbital cavity, corresponding to the meningioma ( a CT; d , h MRI; d , e , i PET; c fused PET/CT; f , j fused PET and MRI) Fig. 65 68 Ga-DOTA peptide, meningioma. Clinical history : 57 y.o. woman with history of a NET of the tail of the pancreas (grade 1) treated with surgery. Suspect relapse in a peripancreatic lymph node. PET/CT findings : increased uptake in a peripancreatic lymph node consistent with relapse ( a MIP). Intense uptake in a large lesion in the base of the cranium consistent with a meningioma ( b CT and fused imaging)
| 4.097656
| 0.425049
|
sec[1]/sec[9]/p[7]
|
en
| 0.999997
|
34191163
|
https://doi.org/10.1186/s41824-019-0066-2
|
[
"uptake",
"dota",
"peptide",
"findings",
"pancreas",
"fused",
"lesion",
"increased"
] |
[
{
"code": "5C52.00",
"title": "Disorders of carnitine transport or the carnitine cycle"
},
{
"code": "5C50.FZ",
"title": "Disorders of peptide metabolism, unspecified"
},
{
"code": "5C50.FY",
"title": "Other specified disorders of peptide metabolism"
},
{
"code": "LD27.6Z",
"title": "Genetic lipodystrophy, unspecified"
},
{
"code": "2C10.1",
"title": "Neuroendocrine neoplasms of pancreas"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Disorders of carnitine transport or the carnitine cycle (5C52.00)】
Synonyms: Carnitine transporter deficiency | Carnitine brain transporter deficiency | Carnitine uptake deficiency | Primary carnitine deficiency | Carnitine palmitoyltransferase I deficiency
Hierarchy: Inborn errors of metabolism → Inborn errors of lipid metabolism (5C52) → Inborn errors of fatty acid oxidation or ketone body metabolism (5C52.0) → Disorders of carnitine transport or the carnitine cycle
【2. Disorders of peptide metabolism, unspecified (5C50.FZ)】
Synonyms: Disorders of peptide metabolism
Hierarchy: Inborn errors of metabolism → Inborn errors of amino acid or other organic acid metabolism (5C50) → Disorders of peptide metabolism (5C50.F) → Disorders of peptide metabolism, unspecified
【3. Other specified disorders of peptide metabolism (5C50.FY)】
Hierarchy: Inborn errors of metabolism → Inborn errors of amino acid or other organic acid metabolism (5C50) → Disorders of peptide metabolism (5C50.F) → Other specified disorders of peptide metabolism
【4. Genetic lipodystrophy, unspecified (LD27.6Z)】
Synonyms: Genetic lipodystrophy | Hereditary lipodystrophy | Lipodystrophy due to peptidic growth factors deficiency | Hoepffner-Dreyer-Reimers syndrome | Combined insulin, insulin-like growth factor 1 (IGF1) and epidermal growth factor (EGF) deficiency
Hierarchy: Multiple developmental anomalies or syndromes → Syndromes with skin or mucosal anomalies as a major feature (LD27) → Genetic lipodystrophy (LD27.6) → Genetic lipodystrophy, unspecified
【5. Neuroendocrine neoplasms of pancreas (2C10.1)】
Definition: A neoplasm with neuroendocrine differentiation that arises from the pancreas. It includes neuroendocrine tumours (low and intermediate grade) and neuroendocrine carcinomas (high grade).
Synonyms: neuroendocrine pancreatic cancer | carcinoma of islet cell of pancreas | malignant islet cell tumour of unspecified site | malignant tumour, islet cell of pancreas | Neuroendocrine tumours of pancreas
Hierarchy: Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues → Malignant neoplasms of digestive organs → Malignant neoplasm of pancreas (2C10) → Neuroendocrine neoplasms of pancreas
|
5C52.00
|
Disorders of carnitine transport or the carnitine cycle
|
We present the case of a 65-year-old Caucasian male who was diagnosed with stage IV NSCLC in September 2021. The patient initially presented with symptoms of progressive dyspnea, exercise intolerability and recurring thorax pain. As the patient had a known history of cardiovascular disease, cardiac magnetic resonance imaging (MRI) was performed, revealing an incidental nodule of the left posterior inferior lung lobe. He was then referred to our lung cancer center. The patient teaches law as a professor at a university and there was no known family history of cancer. However, the patient presented with 40 pack years of cigarette smoking. The patient did not show any further risk factors for lung cancer such as exposition to asbestos, radiation or other potential hazards. Initial workup included a bronchoscopy with endobronchial ultrasound and transbronchial needle aspiration (EBUS-TBNA). However, two consecutive bronchoscopies failed to deliverer a malignant cytology sample of the tumor for further workup. A subsequent fluorodeoxyglucose positron emission tomography-computed tomography (FDG-PET-CT) revealed a hypermetabolic tumor of the left lung, various bone metastases of the spine and a singular metastasis of the left adrenal gland . Finally, the histology of the tumor was obtained through drainage of a pleural effusion of the left lung. Pathological examination revealed an adenocarcinoma of the lung and the initial staging of the patient resulted in cT1 cN1 cM1c and UICC IVB. Comprehensive molecular diagnostics fulfilling standards of the national Network for Genomic Medicine (Germany) were performed. Targeted next generation sequencing (NGS) with a TSO500 panel (Illumina) was performed to detect single nucleotide variants and small insertions or deletions in 523 genes recurrently affected by mutations in various cancer types. This analysis further evaluated copy number variants of 59 genes, microsatellite instability and tumor mutation burden. Additionally, the Archer FusionPlex Lung panel was used to detect fusion transcripts of 17 genes including ALK, ROS1, RET and NTRK1-3. Fluorescence in situ hybridization (FISH) was performed to detect MET amplifications. These studies revealed an EGFR Exon 18 mutation (p.Glu709_Thr710delinsAsp), a neomorph U2AF1 mutation and a likely inactivating mutation in PPC6, a negative regulator of MEK. Further, likely and known inactivating mutations in ATM, AR, DDX41 and variants of unknown significance in six further genes were detected ( Table 1 ). No ALK, ROS1, RET or NTRK1/2/3 fusion transcripts and no MET amplifications were found. Tumor mutation burden was 8.6 variants/megabase pair (Mbp). At the same time, there was no expression of programmed death-ligand 1 (PD-L1) on tumor cells. After primary diagnosis of the NSCLC in September 2021, we initiated standard of care first-line treatment. The initial regimen was Cisplatin (75 mg/m²) and Pemetrexed (500 mg/m²) administered every three weeks, starting mid October 2021. The patient received two cycles of therapy in total without any major side effects. The bone metastases were additionally treated with intravenous infusions of zolendronic acid every other month, commencing in October 2021. The decision to waive radiation therapy in this patient was based on the absence of significant symptom burden associated with bone metastases such as pain or hypercalcemia. Furthermore, there were no osteolytic lesions at risk of fracturing detectable. The patient exhibited good tolerance to zolendronic acid, which was utilized as an adjunctive therapy alongside all systemic treatments thereafter. To monitor therapeutic success, we conducted a computed tomography (CT) scan in December 2021. Unfortunately, this follow-up scan revealed a progression of the primary tumor according to RECIST criteria. Although none of the distant metastases progressed, the patient’s pleural effusion required more frequent drainage. As the patient furthermore suffered from severe nausea and vomiting from cisplatin, we decided to end chemotherapy and initiate TKI-therapy with Osimertinib. This decision was based on case reports previously describing the use of TKIs for this particular EGFR mutation with variable success ( 9 – 12 ). We began treatment with Osimertinib at the beginning of December 2021, starting with 80 mg taken orally once daily. The patient tolerated the administration of Osimertinib well and did not have any clinical signs of side effects or toxicities at first follow-up. Nevertheless, it was necessary to reduce Osimertinib dosing to 40 mg daily as the patient developed worsening thrombocytopenia (nadir of 114 giga/l) three weeks in to his TKI treatment. After dose reduction, the thrombocyte count remained stable at >120 giga/l. We conducted a short-term CT follow-up examination in January 2022, which revealed comprehensive therapeutic response of the NSCLC to Osimertinib therapy. The various bone metastases displayed increasing sclerosis compatible with a notable therapeutic response. The aforementioned pleural effusion likewise regressed. Additionally, the patient continued to tolerate Osimertinib without any further notable toxicities. Follow-up CT scans were conducted in March and August of 2022, which showed stable disease based on Response Evaluation Criteria in Solid Tumors (RECIST). However, the patient again developed a progressive pleural effusion in August 2022. The effusion was initially solely monitored using ultrasound. Regrettably, tumor progression was eventually noted on a further follow-up CT scan in November 2022 and Osimertinib therapy was discontinued. The pleural effusion was now treated with pleurodesis. Again, malignant NSCLC cells were detectable in the pleural fluid and we repeated a comprehensive pathological and molecular workup using NGS (TSO500). Here, PD-L1 status could be assessed to 5% on tumor cells in the newly acquired sample but otherwise the mutation pattern was identical to the initial analysis we conducted. As the patient reported a history of 40 pack years and no prior treatment with immunotherapy, the decision was made for a chemo-immunotherapy re-induction third line therapy regimen. The therapy was initiated in late November 2022 and consisted of Carboplatin AUC 5 (550mg absolute dose), Pemetrexed 500 mg/m² and Pembrolizumab 200mg administered every three weeks . The patient received two cycles of this treatment and tolerated it well. In January 2023, a follow-up CT scan revealed a mixed response to the applied cycles of chemo-immunotherapy. The patient again exhibited progressive pleural effusions on both sides, while the primary tumor in the left lung remained constant. The size of mediastinal lymph nodes was decreasing, but some lymph nodes in the retroperitoneal and mediastinal regions showed minor progression. At the same time, bone lesions remained stable compared to previous CT scans and no further distant metastases were detectable. The CT examination was classified as stable disease based on RECIST. As the patient consistently showed good therapy tolerance, two additional cycles of chemo-immunotherapy were administered with unchanged dosage. In February 2023, another follow-up CT scan yet again showed a stable disease state based on RECIST. Both the primary lung tumor and lymph nodes displayed no significant changes in size. Notably, the bone metastases demonstrated progressive sclerosis further indicating therapy response. After completing four cycles of chemo-immunotherapy, Carboplatin and Pemetrexed were discontinued, while Pembrolizumab monotherapy was continued every three weeks. As of May 2023, the patient underwent another CT follow-up examination, which once more showed a stable disease state with a minor reduction of primary lung tumor size. No new distant metastases or other irregularities were observed. As of June 2023, the patient is currently continuing Pembrolizumab monotherapy.
| 4.230469
| 0.95459
|
sec[1]/p[0]
|
en
| 0.999997
|
37655099
|
https://doi.org/10.3389/fonc.2023.1182391
|
[
"tumor",
"lung",
"therapy",
"further",
"metastases",
"follow",
"pleural",
"mutation"
] |
[
{
"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 CLASSIFICATION ═══
【1. Neoplasms of unknown behaviour of unspecified site (2F9Z)】
Synonyms: neoplasia | neoplasm growth NOS | tumour of unspecified site | tumourlet of unspecified site | tumour mass NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of unspecified site
【2. Subcutaneous swelling, mass or lump of uncertain or unspecified nature (ME61)】
Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature
Synonyms: localised swelling, mass or lump of skin and subcutaneous tissue | localised subcutaneous nodules | subcutaneous nodules | superficial subcutaneous nodules | localised swelling
Excludes: localized adiposity | mass and lump: breast | enlarged lymph nodes | mass and lump: intra-abdominal or pelvic | oedema
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings involving the skin → Symptoms or signs involving the skin → Subcutaneous swelling, mass or lump of uncertain or unspecified nature
【3. Carcinoma in situ of unspecified site (2E6Z)】
Synonyms: carcinoma in situ of unspecified site | carcinoma in situ NOS | carcinoma in situ | in situ neoplasm
Hierarchy: Neoplasms (02) → In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues → Carcinoma in situ of unspecified site
【4. Neoplasms of unknown behaviour of trachea, bronchus or lung (2F91.1)】
Synonyms: 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 | Lung hemangiopericytoma of unknown behaviour
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs (2F91) → Neoplasms of unknown behaviour of trachea, bronchus or lung
【5. Neoplasms of unknown behaviour of skin (2F92)】
Synonyms: skin tumour NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of skin
|
2F9Z
|
Neoplasms of unknown behaviour of unspecified site
|
A 76-year-old overweight man (body mass index of 29) with osteoarthrosis, who had already successfully undergone left hip and knee surgeries some years previously, also underwent right knee arthroplasty in another hospital, developing polymicrobial PJI ( Enterococcus spp , Klebsiella pneumonia , Staphylococcus aureus ), within a few days of surgery. Since the conditions to perform DAIR were not met, he was treated with two-stage exchange and antibiotic therapy (daptomycin and meropenem), suffering side effects (such as anemia and leukopenia) and developing carbapenem resistance. After one year of continued antibiotic treatment with glycopeptides, cephalosporin, and quinolones, a knee prosthesis reimplantation was attempted without success, being complicated by patellar tendon rupture, mixed infection of the wound, and dehiscence. As anemia, leukopenia, and high serum inflammatory markers still persisted and wound dehiscence did not heal with negative pressure wound therapy, the patient was admitted to our hospital. On admission, he was tired, pale, had knee pain that was treated with non-steroid anti-inflammatory drugs, and his inflammatory markers were increased. Laboratory analysis showed: erythrocyte sedimentation rate (ESR) 79 mm/h (normal value <37mm/h), C reactive protein (CRP) 13.6 mg/dL (normal value <0.5mg/dL), white blood cells count 3550 cells/µL, neutrophils count 1750 cells/µL (49%), red blood cells count 3,160,000 cells/µL, hemoglobin 11.1 g/dL, platelets count 286,000/µL, creatinine 0.83 mg/dL. His American Society of Anesthesiologists (ASA) score was 2. The prosthesis was promptly removed, and an antibiotic-impregnated spacer was put. Antibiotic therapy was adjusted according to the microbial cultures collected from (a) the knee surgical wound (positive for Staphylococcus hominis ) and (b) the knee joint (positive for extended-spectrum β-lactamases (ESBL) Klebsiella pneumoniae , and multidrug-resistant (MDR) Acinetobacter baumannii , only sensitive to colistin). Tigecycline (50 mg every 12 h), daptomycin (500 mg a day), and colistin (4.5 MU every 12 h) were started. Due to the progressive worsening of renal function (creatinine values oscillating between 1.32 and 2.8 mg/dL), the dosage of the latter two antibiotics was gradually reduced within one month to 300 mg a day and to 2 MU every 12 h, respectively, based on estimated creatinine clearance. Rifampin was also orally administered two hours after meals at a dosage of 600 mg a day. Iron deficiency and anemia were corrected with intravenous iron load, intramuscular vitamin B12, erythropoietin administration (10.000 IU twice a week), and, when necessary, red blood cell transfusions (if hemoglobin was less than 7 g/dL). Vitamin D deficiency and low potassium and magnesium were also restored. Reconstructive surgery of the knee wound was attempted, and 40 cycles of hyperbaric treatment were performed with only partial benefit. A number of microbial cultures collected from (a) the knee wound and (b) the knee joint during the change of the first spacer with another one were positive for MDR Pseudomonas aeruginosa (sensitive only to colistin) and Staphylococcus capitis . Inflammatory markers were increased: ESR 52 mm/h, CRP 1.10 mg/dL, D-dimer 1762 ng/mL (normal value <500 ng/mL). Other laboratory analyses showed: white blood cells count 2780 cells/µL, neutrophils count 1290 cells/µL (46%), red blood cell count 2,100,000 cells/µL, hemoglobin 7.2 g/dL, platelets count 216,000/µL, creatinine 1.83 mg/dL. Tygecicline and daptomycin were discontinued, while an antibiotic combination (ceftolozane/tazobactam, 500 mg/250 mg in 100 mL of saline (0.9%) solution intravenously three times a day, according to estimated creatinine clearance) was given in addition to colistin and rifampin; the latter were stopped after two months because of the persistence of high serum inflammatory markers , while fosfomicin (4 g intravenously, three times a day) was added to ceftolozane/tazobactam, with benefit against Staphylococcus capitis . Due to the persistence of MDR and carbapenem-resistant Pseudomonas aeruginosa in cultures collected from the knee wound, the spacer was removed, an external fixator was put in, and a daily antibiotic treatment was continued in a rehabilitation center. Three months later, healing of the knee wound and normalization of all serum inflammatory markers occurred. During this period, Corynebacterium striatum was isolated from a skin purulent fluid collected near the fixator pin of the thigh and treated, with benefit, by administering dalbavancin at a dosage of 1.5 g intravenously (two times, one week apart). After a further three months, thanks to the persistent normal values of serum inflammatory markers, ceftolozane/tazobactam and fosfomicin were stopped; two months later, the patient underwent labeled leucocyte scintigraphy and positron emission tomography, which excluded bone and joint pathogenic processes. Hence, the patient underwent new right knee arthroplasty. Some days after implantation, there was dehiscence of the surgical wound, and MDR Klebsiella pneumoniae was isolated from the surgical wound. The patient had fever and showed a decrease in blood pressure. Blood analysis showed electrolyte disturbances (potassium 1.8 mmol/L (normal value 3.5–5.1 mmol/L), chlorine 85 mmol/L (normal value 98–107 mmol/L), calcium 7.5 mg/dL (normal value 8.4–10.2 mg/dL)), phosphorus 1.7 mg/dL (normal value 2.3–4.7 mg/dL), and high serum inflammatory markers . A solution for intravenous infusion of 500 mL glucose (5%) combined with potassium 80 mEqs was promptly administered, and calcium and phosphorus were restored orally. Although blood culture and microbial culture collection from the knee joint were negative, antibiotic treatment was started using an antibiotic combination (ceftazidime/avibactam 2/0.5 g in 100 mL of saline (0.9%) solution, administered intravenously three times a day; after a few days, reduced to 1/0.25 g, three times a day) and fosfomicin (4 g in 100 mL water for injection, administered intravenously three times a day). After one week, the clinical conditions of the patient improved; there was normalization of electrolyte balance and reduction of serum inflammatory markers (ESR 34 mm/h, CRP 1.09 mg/dL, D-dimer 830 ng/mL). Unfortunately, the wound showed signs of necrosis; a tomography revealed severe cellulitis of soft tissue near the prosthesis and, during surgical curettage, a severe detachment of the skin, extending over several cm 2 ; the presence of a fistula was noted. The prosthesis was removed, a new external fixator was put in, and, after fifteen days, due to the worsening of the wound, leg amputation was performed . After two weeks, the wound of the amputation had healed, and he was transferred in good clinical condition to a clinical rehabilitation center. Fifteen days later, the patient developed a severe infection from Clostridium difficile , which quickly caused profuse, intense diarrhea and, after two days, toxic megacolon and septic shock despite treatment with oral vancomycin (125 mg orally, four times daily) and metronidazole (500 mg, three times daily). Therefore, he was urgently transferred to an intensive care unit of a general hospital, where vancomycin and metronidazole were given via rectal Foley and intravenously (500 mg in 100 mL normal saline solution every 6 h and 500 mg every 8 h, respectively) and supplemental oxygen, intravenous fluids (albumin, balanced salt solutions), medications (particularly vasopressors), and blood products were also promptly administered without success, thus making it necessary to perform a colectomy. The postoperative course was complicated by electrolytic disturbances, anemia, acidosis, and malnutrition, which were treated with salt solutions, red blood cells transfusions, bicarbonate, and nutrition support; however, after four months of intensive care, he died.
| 4.011719
| 0.975098
|
sec[1]/p[0]
|
en
| 0.999995
|
34501775
|
https://doi.org/10.3390/ijerph18179186
|
[
"knee",
"wound",
"blood",
"cells",
"inflammatory",
"antibiotic",
"markers",
"count"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Inflammatory arthropathies, unspecified (FA2Z)】
Synonyms: polyarthritis NOS | inflammatory joint disease NOS | nonpyogenic arthritis NOS | arthritic nodosa | polyarthrosis NOS
Hierarchy: Diseases of the musculoskeletal system or connective tissue (15) → Arthropathies → Inflammatory arthropathies → Inflammatory arthropathies, unspecified
【2. Other specified superficial injury of knee or lower leg (NC90.Y)】
Synonyms: 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 | Knee haematoma
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injuries to the knee or lower leg → Superficial injury of knee or lower leg (NC90) → Other specified superficial injury of knee or lower leg
【3. Ankylosis of joint (FA34.4)】
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.
Synonyms: ankylosis | ankylosis of joint, site unspecified | frozen joint | fusion of joint | joint ankylosis
Excludes: stiffness of joint without ankylosis | Ankylosis of spinal joint
Hierarchy: Arthropathies → Certain specified joint disorders or deformities of limbs → Certain specified joint derangements (FA34) → Ankylosis of joint
【4. Chronic instability of knee, unspecified (FA33.4Z)】
Synonyms: Chronic instability of knee | instability of knee | old disruption of ligament of knee
Hierarchy: Certain specified joint disorders or deformities of limbs → Internal derangement of knee (FA33) → Chronic instability of knee (FA33.4) → Chronic instability of knee, unspecified
【5. Abrasion of knee (NC90.0)】
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injuries to the knee or lower leg → Superficial injury of knee or lower leg (NC90) → Abrasion of knee
|
FA2Z
|
Inflammatory arthropathies, unspecified
|
A 66-year-old male patient was admitted to ICU at the University Hospital, in Sassari, Italy, on March 26, 2020, with a diagnosis of SARS-CoV2 infection. Due to a rapid and progressive deterioration of oxygenation, the patient was intubated after a short period of non-invasive respiratory support. He had a history of arterial hypertension treated with ACE-inhibitors and had recently been diagnosed with urinary tract infection. The beginning of COVID-19 symptoms reportedly started one week before admission. A therapy with hydroxychloroquine and lopinavir-ritonavir was administered for the first 10 days. At ICU admission, the patient was deeply sedated, underwent protective mechanical ventilation, according to the new evidence described for such pulmonary damage phenotype, to avoid ventilator-induced lung injury (tidal volume = 6–7 ml kg −1 *PBW, positive end expiratory pressure (PEEP) = 12 cmH 2 O; PaO 2 /FiO 2 = 262); he also required circulatory support with vasopressor (norepinephrine = 0.2 mcg kg −1 min). In addition, the patient had multiple organ dysfunction syndrome with sequential organ failure assessment (SOFA) = 14. A few days after admission, respiratory parameters progressively worsened: a lower oxygenation (PaO 2 /FiO 2 = 174), increase of radiological infiltrates and a parenchymal thickening of the left lower lobe were observed. Table 1 describes the main clinical and laboratory data, which include crucial biomarkers reported at ICU admission and during the whole hospitalization. The patient did not present with fever, the white blood count (WBC) and neutrophils increased while lymphocytes progressively decreased (lymphocytes [Nadir] = 400 μl −1 ; neutrophil/lymphocyte [N/L] ratio = 19.2). As C-reactive protein CRP (= 14.3 mg dl −1 ) and procalcitonin PCT (= 6.91 ng ml −1 ) values were elevated he was administered empirical antibiotic therapy (piperacillin-tazobactam 18 gr IV infusion, 24 h a day and levofloxacin 700 mg a day). As a result of a reduced kidney function, a renal replacement therapy (RRT) was required for several days. However, after 2 weeks of empirical therapy, neither were pathogens isolated on microbiological work-up of samples, nor an improvement of oxygenation (PaO 2 /FiO 2 = 130) was observed. Inflammatory biomarkers showed higher values (CRP = 25 mg dl −1 ; PCT = 13.1 ng ml −1 ) and a further worsening of pulmonary infiltrates with an increase of parenchymal thickening of the whole left lung was observed by imaging techniques. Empirical therapy was replaced by meropenem 1 gr IV infusion every 24 h (dose-adjusted for renal dysfunction) and linezolid 600 mg every 12 h and a bronchial aspirate (BAS) was repeated to confirm SARS-CoV-2 and detect any additional co-infections. Two weeks after ICU admission a surgical tracheostomy was performed at bedside for prolonged mechanical ventilation. A first BAS negative for SARS-CoV-2 was obtained, while the second BAS tested for co-infections showed rapidly growing cotton-candy like colonies on sabouraud dextrose agar (SDA) at 30 °C. Microscopic examination with lactophenol cotton blue preparation showed aseptate broad hyphae, sporangia containing sporangiospores. The mould was identified as Rhizopus spp. based on the phenotype features, however no susceptibility test could be performed at our laboratory. A cranial and thoracic computed tomography (CT) scan was performed to search for specific lesions. Non-encephalic lesions were found, opacification of the left maxillary sinus and thickening with sclerosis of sinus walls were observed and the thoracic scans were suggestive of buried cavitary lesions in the lingula of the left lung upper lobe . Treatment with liposomal Amphotericin B, 5 mg kg −1 IV was commenced, according to guidelines and after discussing with an infectious disease consultant . A second and a third BAS were consecutively positive for Rhizopus spp . A biopsy of the left maxillary sinus was performed to find the source of mould infection, but samples were positive only for Candida glabrata . A transbronchial biopsy was excluded because of severe hypoxia and high risk of airway bleeding. A probable pulmonary mucormycosis was then hypothesized. After 16 days of antifungal treatment, a slow improvement of gas exchange was noticed. Since the bronchoalveolar lavage (BAL) was still positive for Rhizopus spp., a surgical evaluation was requested, and thoracic CT scan was repeated. The scan revealed a rupture of the cavities previously observed in the pleural space and bilateral pleural effusion was observed. Therefore, a thoracentesis was performed at bedside. Samples of pleural effusion were tested for microbiological and histopathological examination, but neither moulds nor other fungi were isolated. At that stage, surgery was not performed, being regarded as high-risk intervention. Finally, 40 days after ICU admission and 20 days after the beginning of liposomal Amphotericin B treatment, even though Rhizopus spp growth was still observed in BAL samples, the patient clinically improved and recovered from lymphopenia (lymphocyte = 1,800 μl −1 , N / L = 3.5). Since persistency of the positive culture on BAL for Rhizopus spp was still occurring, a surgical consultation was planned to eradicate the necrotic lesions from the left lung. At the same time, the antifungal treatment was shifted to Isavuconazole and the liposomal Amphotericin B treatment was suspended . However, since following thoracentesis the oxygenation considerably improved (PaO 2 /FiO 2 > 300), surgery was postponed and also the surgeon evaluated the patient as being at extremely high risk for surgery. Sedation was then suspended, and the patient began a ventilatory weaning process. Furthermore, an improvement of other organ functions was observed, such as in the kidneys, as the urine volume started to increase gradually. The following week, a new clinical deterioration was observed, showing fever, an increase of PCT, severe haemodynamic instability and worsening of kidney and liver functions, probably due to a bacterial co-infection. Although an antifungal treatment with Isavuconazole was maintained together with a prompt empirical antibiotic therapy, the patient died at day 62 after ICU admission due to refractory shock and liver failure. Unfortunately, the autopsy could not be performed due to the lack of a negative pressure room required to execute safe procedures. Table 1 Daily clinical and laboratory variables during ICU stay Variables Reference range Day 1 admission Day 3 Day 7 Day 10 Day14 Day21 Day 28 Day 40 SOFA 13 16 16 16 17 16 15 13 White blood counts (per μl) 5,700 11,710 9990 8760 17,070 27,930 23,750 10,710 Lymphocytes(per μl) 1000 700 800 400 800 1,200 800 1800 Neutrophils (per μl) 4,300 10,300 8,500 7700 12,600 23,600 21,100 6300 N/L 4.3 14.7 10.6 19.2 15.7 19.6 26.3 3.5 CRP (mg/dl) (0–1) 17.7 31.9 18.6 14.3 25 17.1 8.65 8.80 Procalcitonin(ng/ml) (0–0.5) 1.9 13 7.92 6.91 13.1 10 6.06 5.75 Glycemia (mg/dl) (60–99) 124 96 96 70 92 96 86 96 D-dimer (mg/l) (0–0.5) 1.9 4.3 0.8 1 2.6 2.7 6.1 7.64 Ferritin (ng/ml) (26–388) 3216 3755 2012 1395 2128 2737 2084 – NT-proBNP (pg/ml) (0–125) 653 2147 2869 2801 3028 12,541 26,116 19,692 PaO 2 /FiO 2 190 197 208 174 130 161 189 260 SARS-CoV-2 RT-PCR Positive Positive Positive Positive Negative Negative after 24 h Negative BAS/BAL Rhizopus spp. Rhizopus spp. R hizopus spp. Rhizopus spp. Pleural effusion Histo-pathology Negative Microbio-logy Negative SOFA sequential organ failure assessment, N/L neutrophils/lymphocyte ratio, CRP C-reactive protein Fig. 1 Microbiological sample from bronchial aspirate: morphology of Rizhopus spp.. Lactophenol cotton blue preparation showed aseptate broad hyphae, sporangia and sporangiospores Fig. 2 a Thoracic computed tomography (CT) scan showed buried cavitary lesions in the left lung; b cranial CT scan showed corpuscular material in the left maxillary sinus
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https://doi.org/10.1007/s15010-020-01561-x
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[
{
"code": "QB82",
"title": "Contact with health services for routine or ritual circumcision"
},
{
"code": "QA01.Y",
"title": "Other specified examination or encounter for administrative purposes"
},
{
"code": "QA01.0",
"title": "Examination for admission to educational institution"
},
{
"code": "QB95.6",
"title": "Orthoptic training"
},
{
"code": "QA30.10",
"title": "Contact with health services for in vitro fertilisation"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Contact with health services for routine or ritual circumcision (QB82)】
Synonyms: admission for circumcision | ritual circumcision
Hierarchy: Factors influencing health status or contact with health services (24) → Reasons for contact with the health services → Contact with health services for specific surgical interventions → Contact with health services for routine or ritual circumcision
【2. Other specified examination or encounter for administrative purposes (QA01.Y)】
Synonyms: Encounter for paternity testing | Examination for admission to prison | medical examination for admission to prison | medical examination of prisoners for entrance into prison | Examination for admission to summer camp
Hierarchy: Reasons for contact with the health services → Contact with health services for purposes of examination or investigation → Examination or encounter for administrative purposes (QA01) → Other specified examination or encounter for administrative purposes
【3. Examination for admission to educational institution (QA01.0)】
Synonyms: medical examination for admission to school | examination for admission to preschool | medical examination of preschool children for admission to school
Hierarchy: Reasons for contact with the health services → Contact with health services for purposes of examination or investigation → Examination or encounter for administrative purposes (QA01) → Examination for admission to educational institution
【4. Orthoptic training (QB95.6)】
Synonyms: admission for orthoptic training
Hierarchy: Reasons for contact with the health services → Contact with health services for nonsurgical interventions not involving devices → Care involving use of rehabilitation procedures (QB95) → Orthoptic training
【5. Contact with health services for in vitro fertilisation (QA30.10)】
Synonyms: admission for in vitro fertilisation
Hierarchy: Contact with health services for procreative management → Contact with health services for medically assisted reproduction (QA30) → Contact with health services for assisted reproductive technology (QA30.1) → Contact with health services for in vitro fertilisation
|
QB82
|
Contact with health services for routine or ritual circumcision
|
A (positron emission tomography) PET scan revealed mediastinal adenopathy with increased fluorodeoxyglucose (FDG) uptake. The hematologists found no indication for chemotherapy. The patient had circulating IgG, but no IgM, specific for (EBNA, concentration: 47.5 U/mL, cut-off: > 20 U/mL) and viral capsid antigen (VCA, concentration: 577 U/mL, cut-off: 20 U/mL). EBV plasma DNA levels were repeatedly (5x over a period of three years) moderately elevated (from < 1000 copies/ml (positive) to 3905 copies/ml). By serology and PCR, she tested negative for human immunodeficiency virus, hepatitis B and C virus, cytomegalovirus, parvovirus, varicella zoster virus, and syphilis. High titer anti-nuclear antibodies (ANA) were present (homogeneous nuclear staining, titer > 1280 (+++), normal range: 0–160) (Table 1 ). Auto-antibody specificities (anti- Cyclic Citrullinated Peptide (CCP), IgM rheumatoid factor, SS-A Ro52, SS-A Ro60, SS-B, Sm, ribo-nuclear protein (RNP), Scl-70 and Jo-1, Table 1 ) were negative. Markers for auto-immune hepatitis (soluble liver antigen (SLA) antibodies, liver cytosol (LC1) antibodies, antimitochondrial antibodies (AMA), ACTIN, liver-kidney microsomes (LKM) antibodies) were also negative and clinical signs of autoimmunity were absent. Patient 25-Hydroxy –Vitamin D3 levels were repeatedly (69 and 105 nmol/L) normal. A pan human leukocyte antigen (HLA)-I class specific antibody (e-bioscience clone W6/32) demonstrated that the patient’s leukocytes expressed HLA-class I similar to that of a control (data not shown). HLA-genotyping revealed homozygosity for HLA-A*01, 01; HLA-B*08, 08 and HLA-C*07, 07 but not for HLA-DRB1*03, 13 or HLA-DQB1*02, 06. The patient displayed prolonged elevated lactate dehydrogenase (> 255 U/L), alkaline phosphatase (> 105 U/L) and alanine transaminase (> 45 U/L) levels, consistent with hepatitis. Due to markedly reduced IgA, IgG and IgM levels (Table 1 ), intravenous immunoglobulin substitution was initiated. Lately, the patient has experienced a 30–40 kg weight loss, her pulmonary function is rapidly deteriorating and she constantly relies on supplemental oxygen . In agreement with informed written consent and the study protocols , immunologic work-up was initiated. A peripheral blood count revealed monocytosis (Table 1 ) and f low cytometry revealed reduced CD19 + B cell concentrations, reduced frequencies of CD19 + CD27 + IgD − (GC derived) memory B cells, normal CD4 + T cell concentrations but extremely low CD8 + T cell concentrations (Table 1 ). Patient CD3 − CD16 + /CD56 + natural killer (NK) cell concentrations (> 130 × 10 6 /L) were normal. Next generation sequencing found no variants in a targeted panel of genes associated with EBV disease: SH2D1A, PRF1, XIAP, CD27, CTPS1, RASGRP1, CD70, RLTPR, ITK, MAGT1, PRKCD, UNC13D, STX11, STXBP2, FAAP24 and CORO1A , with CVID: ICOS, TNFRSF13B (TACI), TNFSF13 (APRIL), TNFRSF13C (BAFF-R),TNFSF12 (TWEAK), CD19, CD81, CR2 (CD21), MS4A1(CD20), LRBA, CTLA4, PLCG2, NFKB1, NFKB2, PIK3CD, PIK3R1, VAV1, RAC2, BLK, IKZF1 (IKAROS) and IRF2BP2 and genes of interest: BLIMP-1 (PRDM1), BCL-6, CD8A, IL21, IL21R and STAT3. Sanger sequencing of the IL-21 gene promoter revealed no mutations. The tridecavalent pneumococcal conjugate vaccine (PCV) was used to vaccinate two age and gender matched controls and the patient. For all three subjects, this was their first PCV vaccination. Three weeks post-vaccination, our patient’s PCV titers were significantly diminished compared to age and gender matched controls (Table 1 ) and only the patient failed to generate protective antibody levels to some PCV serotypes: 4, 5, 6B and 18C (data not shown). After vaccination, the patient, in contrast to three controls, responded with a 5-fold increase in CD4 + IL-21 + frequencies but with no induction of CD4 + CD45RA − CXCR5 + CCR7 low PD-1 high (peripheral) T follicular helper cells ((p)T FH ) . CD4 + IL-21 + T cell frequency determination on a patient sample (sampled > 1 year after vaccination) and an additional non-vaccinated control sample, accentuated the difference in peripheral CD4 + IL-21 + T cell frequencies (patient vs. all controls: Mann-Whitney U -test p < 0.01). Contrary to four control sera (2 are shown in Table 1 ), only patient sera (2 of 4) were positive for IL-21 (30.1 and 37.8 pg/mL). Peripheral T FH and CD4 + IL-21 + frequencies were negatively correlated among vaccinated subjects . Patient CD4 + IL-21 + T cells were predominantly CXCR5 − (approximately 82%, data not shown). Three days of stimulation with anti-CD3/anti-CD28/IL-2 and subsequent intracellular staining for B-cell lymphoma 6 protein (Bcl-6) revealed that patient peripheral CD4 + T cells tended to be less often Bcl-6 positive ( n = 2 different time points, 31.5%; 26.6–36.6%) than peripheral controls CD4 + T cells ( n = 5 controls, 65.0%; 48.4–83.7%, p = 0.05). Among ex-vivo peripheral IL-21 + CD4 + T cells, only those of the patient were dominated by a chemokine receptor profile (CCR7 − ) consistent with tissue homing (CCR7- / CCR7+ fraction: 1.3 vs. two age and gender matched healthy controls: both 0.8, Table 1 ). The patient’s CD4 + CD25 high FoxP3 + T reg frequencies were reduced (0.9% of CD4 + T cells) compared to those of 4 healthy adult controls (median: 2.5%; range: 1.7–3.1% of CD4 + T cells). The patient’s healthy son (23 years younger than the patient) had normal CD4 + and CD8 + T cell concentrations, positive IgG anti-EBNA but no detectable EBV copies in his blood (data not shown). Table 1 Immunological characteristics associated with chronic EBV reactivation. *In-house (age adjusted) normal range. ** Protective antibody levels: PCV: 0.35 μg/mL, C. diphteria: > 0.1 IU/mL and C. tetani: > 0.01 IU/mL (Statens Serum Institut, Copenhagen, Denmark), HD healthy donor, ND not determined. # Mann-Whitney U-test Fig. 2 Time line of clinical history. 2012: pulmonary infiltrates are detected. 2014: An open lung biopsy is performed revealing pronounced EBV activity and histologic signs of B cell transformation. Elevated peripheral EBV copy numbers, mediastinal adenopathy, elevated liver enzymes, CD8 + lymphopenia and hypogammaglobulinemia are also documented and immunologic and genetic work-up commences. 2019: rapidly deteriorating lung function and 30–40 kg weight loss Fig. 3 Frequencies of CD4 + IL-21 + T cells and pT FH prior to and after vaccination. PBMC cultures (1 × 10 6 /mL), sampled pre-, seven and 21 days post-vaccination, were stimulated for three hours with PMA (20 ng/mL) and ionomycin (300 ng/mL). Staining for surface CD4 + and intra-cellular IL-21 was performed on PBMC derived from the patient a and three (2 of whom were age matched) female controls b (patient vs. controls (all time points), p = 0.01, Mann- Whitney U test). Frequencies of CD4 + CD45RA − CXCR5 + CCR7 low PD-1 high p (eripheral) T FH were ascertained ex-vivo, using the gating strategy devised by He et al. , in PBMC (1 × 10 6 /mL), sampled pre-, seven and 21 days post-vaccination. Frequencies of CCR7 low and PD-1 high expression among patient c and controls d CD4 + CD45RA − CXCR5 + T cells are shown (patient vs. controls (all time points), p = 0.01, Mann-Whitney U test). Median and minimum-maximum values are shown. The dot-plots for the PE-conjugated isotype control (mouse IgG1 K ) antibody in PMA and inonomycin stimulated CD4 + T cells from e the patient and f a control. FI: fluorescence intensity, PE: phycoerythrin, BV: Brilliant Violet, PerCP: Peridinin-Chlorophyll-Protein Fig. 4 Correlation between frequencies of pT FH and CD4 + IL-21 + T cells. The inverse correlation (Spearman’s rho: − 0.86, p < 0.001) between frequencies of CCR7 low and PD-1 high pT FH (among CD4 + CD45RA − CXCR5 + T cells) and CD4 + IL-21 + T cells, collected prior to, seven and 21 days after vaccination ( n = 5 subjects and 15 time points). The three time points for the patient are depicted as triangles
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[
{
"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 CLASSIFICATION ═══
【1. Other specified clinical findings on examination of urine, without diagnosis (MF9Y)】
Synonyms: Methaemoglobinuria | Other and unspecified abnormal findings in urine | Calciuria | Cells and casts in urine | casts in urine
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings of the genitourinary system → Clinical findings on examination of urine, without diagnosis → Other specified clinical findings on examination of urine, without diagnosis
【2. Mucolipidosis (5C56.20)】
Synonyms: Mucolipidosis type 3 | Pseudo-Hurler polydystrophy | Pseudo-Hurler disease | Mucolipidosis type 2 | N-acetyl-glucosamine 1-phosphotransferase deficiency
Excludes: Sialidosis (mucolipidosis type 1)
Hierarchy: Inborn errors of metabolism → Lysosomal diseases (5C56) → Glycoproteinosis (5C56.2) → Mucolipidosis
【3. Sickle cell disease without crisis (3A51.1)】
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.
Synonyms: Hb-SS disease without crisis | HbSS without crisis | Sickle-cell anaemia without crisis | SCD - [sickle cell disease] | SCA - [sickle cell anaemia]
Hierarchy: Diseases of the blood or blood-forming organs (03) → Anaemias or other erythrocyte disorders → Sickle cell disorders or other haemoglobinopathies (3A51) → Sickle cell disease without crisis
【4. Primary anterior uveitis (9A96.3)】
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.
Synonyms: anterior chamber cell
Hierarchy: Disorders of the eyeball - anterior segment → Disorders of the anterior uvea → Anterior uveitis (9A96) → Primary anterior uveitis
【5. Acquired pure red cell aplasia, unspecified (3A61.Z)】
Synonyms: Acquired pure red cell aplasia | acquired red cell aplasia | red cell aplasia NOS | pure red cell aplastic anaemia | red cell aplastic anaemia
Hierarchy: Anaemias or other erythrocyte disorders → Pure red cell aplasia → Acquired pure red cell aplasia (3A61) → Acquired pure red cell aplasia, unspecified
|
MF9Y
|
Other specified clinical findings on examination of urine, without diagnosis
|
On admission, physical examination found rashes mainly in face, trunk and limbs, liver and spleen just palpable, no edema of palms, no periungual or perianal desquamation, no cervical lymphadenopathy, no conjunctival congestion, no strawberry tongue, erythema of the oropharyngeal mucosa or cracking of the lip. No positive neurological sign was found. Laboratory tests showed an increase in leukocytes (20.1 × 10 9 /L) and neutrophils (12.82 × 10 9 /L), erythrocyte sedimentation rate (33 mm/h), positive antinuclear antibody and antineutrophil cytoplasmic antibody, mild anemia (hemoglobin 89 g/L) and slightly elevation of C-reactive protein (19.5 mg/L). Liver function, lymphocyte subtypes, immunoglobulin isotypes, serum ferritin, coagulation function test and blood fat level were normal; Serology for human immunodeficiency virus; widal test; bacterial culture of peripheral blood, bone marrow and urine; hepatitis A, B, and C; mycoplasma pneumoniae (IgM and DNA); tests for tuberculosis; cytomegalovirus (IgM, IgG, and DNA); Human Herpes Virus; Toxopasma (IgM); Rubella (IgM); influenza virus A and B; parainfluenza virus; respiratory syncytial virus; adenovirus; legionella; rickettsia; and Epstein Barr Virus (IgM, IgG and DNA) were all negative. Echocardiography was normal. A diagnosis of acute inflammatory response syndrome and autoimmune vasculitis was entertained on clinical grounds and laboratory findings. Bacterial infection could not be ruled out, then antibiotics (linezolid and cefuroxime) and methylprednisolone was used for 3 days;the high fever subsided and the skin rash disappeared thereafter. 4 days later, high fever recurred and generalized skin eruption was noted. Intravenous immunoglobulin (IVIG) with a dosage of 400 mg/Kg was administered. 8 hours later, his condition deteriorated rapidly to present with a decrease of oxygen saturation, hypotension, tachycardia, tachypnea, irritability and cyanosis. He was transferred to the pediatric intensive care unit (PICU), and administration of fluids, inotropes (dopamine, norepinephrine) and mechanical ventilation was started. Blood routine test showed leukocyte count 10 x 10 9 /L (neutrophil 3 %; eosinophil 40.1 %), erythrocyte count 3.21 x 10 12 /L, hemoglobin 83 g/L, platelet count 46 x 10 9 /L. Total IgE (104.4 KIU/L) was elevated significantly (Table 1 ). Hypersensitive to IVIG was considered and high-dose intravenous methylprednisolone (30 mg/Kg) was given. During his hospitalization in PICU, the patient experienced several shocks each time after transfusion of blood and blood products, including IVIG, albumin, plasma, washed red blood cells. He developed progressive hepatosplenomegaly, severe pancytopenia (leukocyte count 0.2 x 10 9 /L, erythrocyte count 1.11 x 10 12 /L, hemoglobin 29 g/L, platelet count 9 x 10 9 /L). He presented with neck stiffness, convulsion and light coma, and his eyes couldn't follow light. Serum ferritin , lactate dehydrogenase and triglycerides (4.53 mmol/L) were elevated significantly, but lactic acid was normal and plasma fibrinogen was decreased apparently (0.49 g/L). Both the prothrombin time and the activated partial thromboplastin time were above limit of detection. Lumbar puncture revealed the initial pressure of 125 mmH 2 O and clear cerebrospinal fluid containing white blood cells 0 cells/L, red blood cells 28 cell/L, protein 513 mg/L. The fifth bone marrow aspiration displayed large amount of hemophagocytic histiocytes . Brain magnetic resonance imaging (MRI) suggested ANEC . DNA samples were obtained from the peripheral blood of the patient by standard procedures, and the sequences of HLH-associated genes were analyzed. The result verified 1 variation in the PRF1 gene, 10 variations in the UNC13D gene, 8 variations in the STXBP2 gene, 2 variations in the XIAP gene and no variations in STX11 and SH2D1A (Table 2 ). Table 1 The main laboratory parameters detected in this patient Laboratory parameters Reference value Results Rheumatoid factor 0–20 IU/ml <20 Anti-streptolysin “O” 0–200 IU/ml <25 C-reactive protein 0–10 mg/L 19.5 Anti-cyclic-citrullinated peptide antibody – Negative Autoantibodies Antinuclear antibody – Positive Antineutrophil cytoplasmic antiboby – Positive Anti jo-1 antibody – Negative Anti-centromere antibody – Negative Anti-double-stranded dna antibody – Negative Anti-histone antibody – Negative Antismith antibody – Negative Anti-rnp antibody – Negative Anti-SS A antibody – Negative Anti-SS B antibody – Negative Anti-ribosomal antibody – Negative Anti-Sc1-70 antibody Negative Anti-nucleosome antibody – Negative Lymphocyte subsets (Flow cytometry) CD3+ T cell 68 % ± 10.7 % 66.1 % CD4+ T cell 31.5 % ± 8.8 % 34.2 % CD8+ T cell 25.7 % ± 6.5 % 19.66 % CD19 B cell 15.6 % ± 5.8 % 19.1 % NK cell 5.6 % - 31 % 9 % CD4+/CD8+ 1.5 ± 0.5 1.74 Ig isotypes IgG 3.22 – 14.0 g/L 6.39 IgM 0.57 – 1.41 g/L 0.82 IgA 0.24 – 1.79 g/L 0.43 Complement C3 0.8 – 1.6 g/L 0.74 Complement C4 0.1 – 0.4 g/L 0.18 Total IgE 0 – 20 KIU/L 104.4 Ammonia 9 – 30 umol/L 19 Lactic acid 0.7 – 2.1 mmol/L 1.22 Coagulation test Activated partial thromboplastin time 26.1 – 40.73 s 29.7 a , over the upper limit of detection b Prothrombin time 9.3 –12.9 s 12.8 a , over the upper limit of detection b International normalized ratio 0.72 – 1.15 1.09 a , over the upper limit of detection b Thrombin time 13.2 – 20.1 s 20 a , over the upper limit of detection b Fibrinogen 1.57 – 3.93 g/L 2.43 a , 0.49 b Blood lipid levels Total cholesterol 3.1 – 5.8 mmol/L 1.78 a , 3.62 b Triglyceride 0.23 – 1.7 mmol/L 2.54 a , 4.53 b High density lipoprotein 0.9 – 1.8 g/L 0.47 a , 1.05 b Low density lipoprotein 2.07 – 4.1 g/L 1.1 a , 1.8 b Serum ferritin 22 – 322 ng/ml 15.4 a , 18699 b Values of eosinophils 0.02 – 0.5 × 10 9 /L 0.26 a , 4.01 c , 0.01 d Note: a - on admission, b - peak period of disease, c - the 2nd day after first IVIG, d - when severe pancytopenia Fig. 1 Hemophagocytic histiocytes in bone marrow. Bone marrow aspiration showing a normal histiocyte ( a ), a hemophagocytic histiocyte containing aphagocytosed neutrophil and platelets ( b ), a hemophagocytic histiocyte containing aphagocytosed erythroblast and platelets ( c ). Fig. 2 Neurological images. Brain magnetic resonance imaging (MRI) on admission revealed extensive edema ( a ); after 4 weeks, it showed long T2 signal ( b ) and hyperintensity on fluid-attenuated inversion recovery (FLAIR, c ) mainly in thalamus, basal ganglia, brainstem. 1 year later, the neurological image (MRI) showed slightly abnormal shape of bi-lateral ventricular, not any other obvious abnormal changes noticed ( d ) Table 2 Variations of the HLH related genes and the references Gene Exons Function Variation rs number MAF Clinical significance PRF1 EXON3 Synonymous c.900C > T p.His300His rs885822 0.3041 UNC13D EEXON1 Intron c.117 + 59C > T rs3744010 0.2847 EXON4 Synonymous c.279C > T p.Pro93Pro rs3744007 0.1000 EXON5 Intron c.388 + 122C > T rs3744006 0.4930 EXON11 Synonymous c.888G > C p.Pro296Pro rs7223416 0.4902 EXON18 Intron c.1596 + 36A > G rs3744026 0.3083 EEXON19 Intron c.1728-48 T > C rs3744024 0.3025 EXON21 Synonymous c.1977C > T p.Thr659Thr rs2290770 0.0799 EXON24 Intron c.2299-46C > T rs7212635 0.2542 EXON28 Intron c.2709 + 48C > T rs2290768 0.2530 EEXON32 Synonymous c.3198A > G.Glu1066Glu rs7210574 0.4738 STX11 No mutation STXBP2 Exon2 Intron c.38-7C > T rs113939878 NA acceptor Exon10 Synonymous c.816 C > T p.Ser272Ser rs78010345 0.0034 EXON15 Intron c.1247-43 T > C rs929807 0.4730 EXON15 Intron c.1356 + 18A > G rs889187 0.4836 Exon16 Synonymous c.1443 T > C .Asp481Asp rs10001 0.4958 Exon18 Intron c.1696 + 28G > C rs34976997 0.3157 Exon18 Intron c.1696 + 77G > A rs794074 0.4615 Exon19 Intron c.1697-26 T > G rs794073 0.465 XIAP Exon5 Intron c.1099 + 264G > C rs28382732 0.2638 Exon7a 3' UTR c.*12A > G rs28382740 0.2673 SH2D1A No mutation Note: MAF - minor allele frequency; UTR , untranslated region
| 4.046875
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sec[1]/p[1]
|
en
| 0.999998
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27576518
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https://doi.org/10.1186/s13052-016-0286-z
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[
"antibody",
"intron",
"anti",
"blood",
"synonymous",
"virus",
"count",
"time"
] |
[
{
"code": "JA86.Y",
"title": "Maternal care for other specified fetal problems"
},
{
"code": "MA14.14",
"title": "Anti-nuclear antibody positive"
},
{
"code": "MA14.13",
"title": "Anti-nuclear antibody negative"
},
{
"code": "JA86.0",
"title": "Maternal care for red cell antibodies"
},
{
"code": "MA14.1C",
"title": "Raised antibody titre"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Maternal care for other specified fetal problems (JA86.Y)】
Synonyms: Maternal care for other isoimmunization | Isoimmunization NOS | maternal antibodies NOS | pregnancy management affected by incompatibility of blood groups NOS | Maternal care for ABO isoimmunisation
Hierarchy: Pregnancy, childbirth or the puerperium (18) → Maternal care related to the fetus, amniotic cavity or possible delivery problems → Maternal care for other fetal problems (JA86) → Maternal care for other specified fetal problems
【2. Anti-nuclear antibody positive (MA14.14)】
Synonyms: ANA - [anti-nuclear antibody] positive
Hierarchy: Clinical findings in blood, blood-forming organs, or the immune system → Immunological findings in blood, blood-forming organs, or the immune system (MA14) → Certain specified immunological findings (MA14.1) → Anti-nuclear antibody positive
【3. Anti-nuclear antibody negative (MA14.13)】
Synonyms: ANA - [anti-nuclear antibody] negative
Hierarchy: Clinical findings in blood, blood-forming organs, or the immune system → Immunological findings in blood, blood-forming organs, or the immune system (MA14) → Certain specified immunological findings (MA14.1) → Anti-nuclear antibody negative
【4. Maternal care for red cell antibodies (JA86.0)】
Definition: Maternal care for rhesus or other isoimmunization
Synonyms: Maternal care for rhesus isoimmunization | Rh factor immunization affecting management of pregnancy | Rh incompatibility | Rh incompatibility with hydrops fetalis | Anti-D [Rh] antibodies
Hierarchy: Pregnancy, childbirth or the puerperium (18) → Maternal care related to the fetus, amniotic cavity or possible delivery problems → Maternal care for other fetal problems (JA86) → Maternal care for red cell antibodies
【5. Raised antibody titre (MA14.1C)】
Synonyms: antibody titre above reference range | high antibody titre | increased antibody titre
Excludes: isoimmunization, in pregnancy affecting fetus or newborn
Hierarchy: Clinical findings in blood, blood-forming organs, or the immune system → Immunological findings in blood, blood-forming organs, or the immune system (MA14) → Certain specified immunological findings (MA14.1) → Raised antibody titre
|
JA86.Y
|
Maternal care for other specified fetal problems
|
An 18-year-old man was admitted to our hospital with proteinuria identified in a medical checkup. He had no personal or familial medical history. His height was 176.5 cm, weight was 126 kg, and body mass index (BMI) was 40 kg/m 2 . He was taking no medication and had never consumed alcohol. A physical examination demonstrated no findings. Laboratory investigation results are shown in Table 1 . Hepatic dysfunction, dyslipidemia, and slight increases in C-reactive protein were noted in the laboratory findings. All hepatitis viral markers were negative. An elevated serum protein level induced by vitamin K absence or antagonist-II (PIVKA-II) level of 92 mAU/mL was observed. Abdominal ultrasonography showed a liver tumor approximately 7 cm in diameter protruding forward with a hypoechoic and smooth surface on the left lobe . Abdominal plain computed tomography showed an isodense tumor in the left lobe . Contrast-enhanced computed tomography (CECT) showed two tumors in Couinaud’s segments 3/4 and 8 that showed enhancement in the early phase and prolonged enhancement in the late phase. The two tumors expressed hyperintensity on T1-weighted magnetic resonance imaging (MRI) and mild hyperintensity on T2-weighted MRI . Gadoxetic acid-enhanced (EOB)-MRI showed that the two tumors had similar contrast attitudes on CECT in the early and late phases . EOB-MRI also revealed three tumors with low signal intensity in the hepatocellular phase that were 68 mm, 16 mm, and 9 mm in size in segments 3/4, 8, and 1, respectively. They were suspected as either HCAs, focal nodular hyperplasia, lymphoma, or hepatocellular carcinoma (HCC) based on the imaging findings. A percutaneous needle biopsy was performed on the largest tumor. Microscopic pathological examination revealed that the tumor had slightly larger hepatocyte proliferation, expanded muscle-type arteries, scattered sinusoids, and inflammatory cell invasion but no nuclear atypia. An immunohistochemical examination revealed that the tumor cells were negative for serum amyloid A protein and β-catenin and partially positive for glutamine synthetase. Downregulation of liver fatty acid-binding protein was not observed. The cells also tested negative for cytokeratin 7 and MIB1. Accordingly, the tumor was diagnosed as an unclassified type HCA. Although the patient attempted to lose weight with diet and exercise before surgery, his weight had not decreased and the tumor size was unchanged 6 months after the diagnosis. The patient was scheduled to undergo laparoscopic partial liver resection for the largest HCA, which was larger than 5 cm and carried potential risks of rupture and malignant transformation. Finally, laparoscopic partial liver resection was performed on all three HCAs as we found using ultrasound that the other two small tumors were located near the liver surface. The intraoperative findings are shown in Fig. 3 a–d. The operative time was 397 min, and a blood loss of 32 mL. A blood transfusion was not needed. A gross pathological examination revealed that the tumors were yellowish, well-defined, and 57 × 45 mm, 9 mm, and 5 mm in size, in segments 3/4, 1, and 8, respectively . The largest HCA had a hemorrhage. A pathological examination revealed slightly larger hepatocyte proliferation, expanded muscle-type arteries, and scattered sinusoids within the tumors . It also revealed a hematoma with hemosiderosis in the red areas of the tumors. The background of the liver was steatotic (70%) with mild fibrosis . Immunohistochemistry revealed that the tumor cells were positive for serum amyloid A protein, negative for β-catenin, negative for glutamine synthetase, and negative for cytokeratin 7 without downregulation of fatty acid-binding protein . Based on the findings, all the tumors were diagnosed as inflammatory type HCAs. The postoperative course was uneventful, and the patient was discharged 12 days after the procedure. The serum PIVKA-II decreased to 27 mAU/mL 8 months after discharge. As of 21 months postoperative follow-up, no recurrence was noted. Table 1 Patient’s laboratory data at admission Laboratory test Value Normal range White blood cells 7100/μL 4000–8500/μL Red blood cells 583 × 10 4 /μL 4.15–5.50 × 10 4 /μL Hemoglobin 16.5 g/dL 13.5–17.5 g/dL Platelets 24.1 × 10 4 /μL 12–36 × 10 4 /μL Prothrombin time 88.7% 80–125% Sodium 140.9 mEq/L 136–147 mEq/L Potassium 4.01 mEq/L 3.5–5.0 mEq/L Chloride 102.5 mEq/L 98–108 mEq/L Total protein 8.0 g/dL 6.5–8.2 g/dL Albumin 4.5 g/dL 3.8–5.3 g/dL Total bilirubin 1.00 mg/dL 0.3–1.2 mg/dL Aspartate aminotransferase 38 U/L 8–40 U/L Alanine aminotransferase 105 U/L 5–45 U/L Alkaline phosphatase 475 U/L 100–340 U/L Gamma-glutamyl transpeptidase 127 U/L 0–75 U/L Lactate dehydrogenase 205 U/L 115–245 U/L Cholinesterase 467 U/L 239–485 U/L Total cholesterol 254 mg/dL 130–219 mg/dL High-density lipoprotein cholesterol 49 mg/dL 40–85 mg/dL Low-density lipoprotein cholesterol 168 mg/dL 70–139 mg/dL Triglyceride 212 mg/dL 30–149 mg/dL Blood urea nitrogen 11.5 mg/dL 8.0–23.0 mg/dL Creatinine 0.70 mg/dL 0.61–1.08 mg/dL C-reactive protein 1.32 mg/dL 0–0.30 mg/dL Hemoglobin A1c 6.2% < 6.0% Hepatatis B surface antigen Negative Negative Hepatitis C virus antibody Negative Negative Anti-nuclear antibody < × 40 < × 40 Anti-mitochondrial M2 antibody < 1.5 INDEX 0–6.99 INDEX Alfa-fetoprotein < 2.00 ng/mL 0–10 ng/mL Carcinoembryonic antigen 1.8 ng/mL 0–5 ng/mL Carbohydrate antigen 19-9 4.58 U/mL 0–2 U/mL Protein induced by vitamin K absence or antagonist-II 92 mAU/mL < 40 mAU/mL Neuron-specific enolase 10.4 ng/mL < 16.3 ng/mL Soluble interleukin-2 receptor 397 U/mL 145–519 U/mL Indocyanine green 15-min retention rate 15.0% < 10% Fig. 1 Preoperative ultrasonography and computed tomography images showing the presence of tumors. a Abdominal ultrasonography image showing a hypoechoic tumor approximately 7 cm in diameter protruding forward on the surface of the left lobe. b Plain computed tomography image showing an isodense tumor in the left lobe. c Contrast-enhanced computed tomography (CECT) image showing two tumors in segments 3/4 and 8 that were enhanced in the arterial phase. d CECT showing the two tumors with prolonged enhancement in the late phase Fig. 2 Preoperative magnetic resonance imaging showing the presence of tumors. a T1-weighted magnetic resonance image (MRI) showing the two hyperintense tumors. b T2-weighted MRI showing the two tumors with mild hypertensity. c Gadoxetic acid-enhanced (EOB)-MRI showing the two tumors with enhancement in the arterial phase. d EOB-MRI showing the two tumors with prolonged partial enhancement in the late phase. e EOB-MRI showing the two tumors in segments 3/4 and 8 with low signal intensity in the hepatocellular phase. f EOB-MRI showing the one tumor in segment 1 with low signal intensity in the hepatocellular phase Fig. 3 Intraoperative findings during the laparoscopic partial liver resection. a Laparoscopy showing the largest tumor, which protruded out of the left lobe. b Gross appearance of the cut surface of segment 3/4. c Gross appearance of the cut surface of segment 8. d Gross appearance of the cut surface of segment 1 Fig. 4 Histopathological findings of the resected specimen. a Macroscopically, the tumors were yellowish and well-defined with hemorrhage (white arrows). b , c Pathological examination revealed slightly enlarged hepatocyte proliferations without nuclear atypia or mitosis. (Hematoxylin and eosin [H&E] staining, original magnification: b H&E, × 20; c H&E, × 100) d The background liver was steatotic with mild fibrosis (H&E, × 100). e – i Immunohistochemical examination revealed the following findings of the tumor cells (immunohistochemical staining, × 200): positive for serum amyloid A protein ( e ), negative for β-catenin ( f ), negative for glutamine synthetase ( g ), negative for cytokeratin 7 ( h ), and positive for fatty acid-binding protein ( i )
| 4.109375
| 0.971191
|
sec[1]/p[0]
|
en
| 0.999997
|
31410698
|
https://doi.org/10.1186/s40792-019-0689-3
|
[
"tumors",
"tumor",
"showing",
"protein",
"phase",
"findings",
"liver",
"surface"
] |
[
{
"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 CLASSIFICATION ═══
【1. Neoplasms of unknown behaviour of unspecified site (2F9Z)】
Synonyms: neoplasia | neoplasm growth NOS | tumour of unspecified site | tumourlet of unspecified site | tumour mass NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of unspecified site
【2. Subcutaneous swelling, mass or lump of uncertain or unspecified nature (ME61)】
Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature
Synonyms: localised swelling, mass or lump of skin and subcutaneous tissue | localised subcutaneous nodules | subcutaneous nodules | superficial subcutaneous nodules | localised swelling
Excludes: localized adiposity | mass and lump: breast | enlarged lymph nodes | mass and lump: intra-abdominal or pelvic | oedema
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings involving the skin → Symptoms or signs involving the skin → Subcutaneous swelling, mass or lump of uncertain or unspecified nature
【3. Carcinoma in situ of unspecified site (2E6Z)】
Synonyms: carcinoma in situ of unspecified site | carcinoma in situ NOS | carcinoma in situ | in situ neoplasm
Hierarchy: Neoplasms (02) → In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues → Carcinoma in situ of unspecified site
【4. Neoplasms of unknown behaviour of trachea, bronchus or lung (2F91.1)】
Synonyms: 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 | Lung hemangiopericytoma of unknown behaviour
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs (2F91) → Neoplasms of unknown behaviour of trachea, bronchus or lung
【5. Neoplasms of unknown behaviour of skin (2F92)】
Synonyms: skin tumour NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of skin
|
2F9Z
|
Neoplasms of unknown behaviour of unspecified site
|
On April 9, 2023, a 23-year-old woman was admitted to the hospital with a history of abnormal mood episodes for 4 years and weakness in the extremities for over 1 week. The patient had experienced intestinal obstruction in 2018, which was conservatively treated with satisfactory results, without any significant personal or family medical history. The patient began to exhibit mood abnormalities 4 years ago, manifesting as signs of depression and irritability. Over the years, the patient has frequently experienced abdominal and lumbar pain, leading her to engage in habitual jumping to alleviate these discomforts. These physical symptoms were accompanied by panic attacks, chest tightness, and difficulty sleeping. These symptoms often preceded menstruation and resolved spontaneously post-menstruation. Despite being treated with citalopram, olanzapine, and quetiapine at a hospital, the symptoms persisted. One month ago, the patient again became irritable and experienced insomnia, accompanied by unresponsiveness. Consequently, she was admitted to an external hospital, though the details of the consultation and treatment remain unknown. The symptoms did not improve significantly. One week prior to admission, the patient developed weakness in all four limbs, particularly the upper limbs, and progressively became bedridden. She also experienced dysphagia, shortness of breath, urination difficulties, and mental depression. Physical examination revealed moderate growth, malnutrition, a passive position, emaciation, and cyanosis. The patient had a urinary catheter and gastric tube in place. The remaining examination findings were unremarkable. The neurological examination showed that the patient was unconscious, non-verbal, and could only open her eyes in response to stabbing pain. The bilateral pupils were equal in size and round, with a diameter of approximately 4.0 mm, and the direct and indirect light reflexes were sensitive. The muscle strength of the limbs was weak, and there was a slight avoidance response to stabbing pain. Additionally, the patient exhibited decreased muscle tone in the extremities, absent tendon reflexes in the extremities, absent bilateral baroreflexes, neck tenderness, and kyphosis. Blood gas analysis indicated an oxygen partial pressure of 27 mmHg and carbon dioxide partial pressure of 68 mmHg, suggestive of type II respiratory failure. Despite being admitted to the hospital and receiving cardiac monitoring, high-concentration oxygen therapy, and sputum suction, the patient's SpO2 remained below 70%. As a result, she was intubated and ventilated with ventilator-assisted ventilation. Laboratory test results revealed a significant elevation in the white blood cell count at 24.52 * 109/L, a significantly reduced sodium ion concentration at 115.5 mmol/L, and an elevated calcitoninogen level at 0.530↑ng/mL. BNP, troponin I, coagulation routine, hepatic function, renal function, thyroid function, ANCA, and rheumatoid immune series did not exhibit any significant abnormalities. The results of infection markers, aspergillus serology test, and fungal D-glucan were within normal limits. On April 11, 2023, cranial magnetic resonance imaging (MRI) revealed high signal intensity in the bilateral parieto-occipital lobe and right temporal lobe in T2-FLAIR, local high signal intensity in the diffusion-weighted imaging (DWI) sequence, and a slightly low signal intensity in the corresponding ADC. No obvious abnormality of low signal intensity was observed in the brain parenchyma on the susceptibility-weighted imaging (SWI). Based on these imaging findings, reversible posterior white matter encephalopathy was considered . Electromyography showed peripheral nerve damage in the upper and lower extremities involving both motor and sensory fibers, as well as axonal damage. The cell count in the cerebrospinal fluid was normal, while the protein concentration was elevated; ganglioside antibodies were negative in both blood and cerebrospinal fluid. Guillain-Barré syndrome (GBS) was suspected due to the acute onset and rapid progression of spinal and cranial nerve damage in the extremities, along with the presence of albuminocytological dissociation in the cerebrospinal fluid. Therefore, we administered human immunoglobulin (0.4 g/kg.d) for 5 days, methylcobalamin and lipoic acid to nourish the nerves, antibiotics to combat infection, and 3% hypertonic saline to correct hyponatremia, among other treatments. However, the patient's limb weakness did not show any significant improvement, and she still had difficulty with extrication and quadriplegia. Nine days after admission, the patient in the ICU experienced repeated loss of consciousness, failure to call out, and limb twitching. These symptoms could be terminated after administering sedative diazepam. Seizures were considered, so levetiracetam and sodium phenobarbital were added for antiepileptic purposes. Electroencephalogram showed a slowing of the background rhythm. At that time, we carefully considered the possibility of a misdiagnosis and further summarized the patient's condition. The patient had a history of intestinal obstruction and subsequently developed intermittent psychiatric abnormalities and abdominal pain related to the menstrual cycle. Recently, the patient had experienced acutely worsening peripheral neurological symptoms, which were consistent with the classic triad of acute intermittent porphyria. We observed that the patient's urine darkened to a dark brown color when exposed to sunlight . Additionally, we collected urine samples from the patient's parents and found that the father's urine did not significantly change color after exposure to sunlight, while the mother's urine exhibited a mild darkening. Further genetic analysis of the patient's family line revealed that the patient carried a heterozygous missense variant in the HMBS gene (c.518G>A:p.R173Q), which was inherited from the patient's mother and was not present in the patient's father or younger brother. Ultimately, a definitive diagnosis of acute intermittent porphyria was made. Subsequently, we adjusted the patient's treatment regimen to include progesterone injections of 20 mg once daily (QD) intramuscularly to delay menstruation, dextrose injections of 150 g twice daily (BID) intranasally, and discontinued the use of aggravating medications such as sodium phenobarbital. On April 28, 2023, the patient's repeat cranial brain MRI revealed a significant decrease in the extent of the abnormal signal in the bilateral parietal-occipital lobes and right temporal lobe compared to the previous scan . Despite being hospitalized for 32 days, the patient was still unable to be discharged, and her family requested an automatic discharge. At the time of discharge, she was coherent but mentally impaired, with normal comprehension and attention span. Her bilateral pupils were equal in size and round, measuring approximately 3 mm in diameter, and light reflexes were present. Both eyes were incompletely closed, and her bilateral frontal lines and nasolabial folds were symmetrical. The muscle strength of both upper limbs was grade 0, the distal muscle strength of both lower limbs was grade 1, and the muscle tone of all limbs was reduced. Tendon reflexes of the extremities were not elicited, and Babinski's sign was not present on both sides. The patient exhibited no signs of meningeal irritation, and the remaining portions of the examination could not be coordinated. On follow-up 10 days after discharge, the patient tolerated being weaned off the ventilator but still had weakness in all four limbs. Three months after discharge, the patient was on an intermittent high-sugar diet, and her limbs had significantly improved in strength, allowing her to stand on her own. Although she could breathe on her own, she still exhibited emotional irritability.
| 3.943359
| 0.97998
|
sec[1]/p[0]
|
en
| 0.999996
|
38274883
|
https://doi.org/10.3389/fneur.2023.1334743
|
[
"limbs",
"extremities",
"experienced",
"symptoms",
"muscle",
"signal",
"weakness",
"pain"
] |
[
{
"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": "ND55",
"title": "Other injuries of leg, level unspecified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Open wound of unspecified body region (ND56.1)】
Synonyms: cut NOS | open wound NOS | penetrating wound NOS | Puncture wound with foreign body unspecified body region | Laceration of extremity, not elsewhere classified
Excludes: Traumatic amputations involving multiple body regions | Open wounds involving multiple body regions | traumatic amputation NOS
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injuries to unspecified part of trunk, limb or body region → Injury of unspecified body region (ND56) → Open wound of unspecified body region
【2. Structural developmental anomalies of the skeleton, unspecified (LB9Z)】
Synonyms: Abnormal bone development | skeletal anomaly NOS
Hierarchy: Developmental anomalies (20) → Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the skeleton → Structural developmental anomalies of the skeleton, unspecified
【3. Other specified soft tissue disorders (FB56.6)】
Synonyms: Fat necrosis | fatty necrosis | Profichet's disease | Sloughing of fascia | Swelling of arm
Hierarchy: Soft tissue disorders → Miscellaneous specified soft tissue disorders → Specified soft tissue disorders, not elsewhere classified (FB56) → Other specified soft tissue disorders
【4. Other injuries of leg, level unspecified (ND55)】
Synonyms: other injuries of lower limb, level unspecified | Superficial injury of leg, level unspecified | Abrasion of leg, level unspecified | Contusion of leg, level unspecified | contusion of lower limb, level unspecified
Excludes: Fracture of leg, level unspecified | Injuries involving multiple body regions
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injuries to unspecified part of trunk, limb or body region → Other injuries of leg, level unspecified
|
ND56.1
|
Open wound of unspecified body region
|
Multiple times during her adolescence, P asked her mother how she put up with her father’s charming behavior and received answers such as “Oh, let him.” P repeatedly felt a tightness in her head, neck, and shoulders, and sometimes suffered from shortness of breath associated with now diagnosed gluten intolerance. Food always got stuck in her, combined with a feeling of fullness and the general impression that “once everything is in the cells, it does not want to come out any time soon.” As a child, she often felt the need to hide in her father’s wardrobe, a small, dark room where she could “hear” the “sound of silence,” completely undisturbed. The allergies kept changing, with an allergy to food turned into an allergy to pollen, and stress aggravating the symptoms. Toward the end of the initial session, P was informed about hypnotic trance and its corresponding phenomena and was casually asked if she wanted to experience it for a few more minutes before coming back. She was very happy to do so, with O’s appropriate invitation already being delivered slowly. During this brief 10-min trance, she was invited to experience autonomic ideomotor limb movements (twitches and arm raises) of early childhood that were familiar to her body from her memory, with the accompanying comment of “being perceived by everyone else as an autonomous human being with boundaries.” In other words, a reference to her childhood ability of setting autonomous behavior , and thus her dignity of “being human” and not a “heteronomous function of other people’s needs,” was implicitly emphasized. In the next session, which took place about 7 weeks after the first one, P reported that she was already feeling better. She cried that her father always presented her as a “decorated little horse.” During this recollection, she felt pressure in her throat and a feeling of “thickening of breath” coupled with the account of her dreams, in which she could neither speak nor scream and her voice could not “get out” of her gullet. With the instruction to continue from this topic the next time, symptoms currently arising in the context of her memories disappeared. In the next session, she reported that when she was overwhelmed at work, during which she would function like a robot, and she would always have a similar dream at night. The physical overload caused by exceeding her stress limit often triggered violent anger and abdominal pain in her; she looked in the toilet mirror at work and said to herself, “that is not you!” Thus, she was asked by O whether she would like to “remember this sort of gut feeling” that she just talked about during trance “to make new discoveries from there.” With her consent obtained, she was told that she is not alone with her feelings, is in a safe environment, and O can be trusted. When P was in a trance with her eyes closed, O triggered a memory by asking P what she wants to start remembering first . “Is it a picture, a sound, a feeling, a smell, or a taste?” (i.e., requesting sub-modalities to trigger the memory). She did not have to answer; it was just a matter of finding out how it made her feel, and when she was ready to rehearse, she could confirm this to O by, for example, moving her left index finger (ideomotor activity as evidence of intrinsic activity). Once she moved her finger, O asked, “What is it now?”; P responded, “pressure in the belly.” Then, O replied, “Please try to make the feeling stronger, let time slow down until it almost stops. Your space becomes wide, wider, and wider, so that you can see very far, very comfortably into the inner vastness. (Transitive state of “slowing down of time and expansion of space ”) [Pause for several seconds] What happens now?” P said, “It has moved upward, suddenly in the throat” [P is choking now]. O replied, “Please hold on, take your time, I am with you, and you are safe, wait and see what comes, slowly but surely.” P stopped choking and sat in amazement. O asked, “What is it now?” P said, “I see a tall woman with long black hair, a white face, her hair fluttering in the air, floating above the ground.” [P was crying at this moment]. O responded, “Take your time, that is right, it is good to let it all come, more and more, just let it come, let it get stronger and stronger” [pause, no further speaking]. As soon as O saw her body relaxing, he invited her to dehypnotize with the instruction, “Remember any experience that your subconscious mind wants you to remember now after you wake up. And once you are fully awake, please stretch your whole body so that you feel as relaxed, fresh, and strong again as if you had slept well all night” [pause]. After waking up, P said, “Crazy, that was intense!” O asked, “What do you mean, pleasant or unpleasant, or unclassifiable, but just intense?” P replied, “Violent, yes, enormous, strange” [pause, as P was still completely preoccupied with her feelings]. O said, “Your body will surprise you now every other day, almost imperceptibly, [pause] as if your perspective on all events changes, especially on all challenges [pause] that you have experienced again and again in the past, with—little—changed perspective [pause]. At the same time, your perspective has changed by 0.347 or 0.591 degrees [a metaphor for arbitrarily low numbers that gift-wrap the mediated notion of minute and therefore initially imperceptible changes], and a little more each day [pause].” As soon as P began to smile (which is often the case after such a procedure), O asked, “What are you going to do today?” This question typically induced amnesia to distract P from the previous trance, bring her completely out of trance, and make her feel like everything is done for today, that she can now go about her daily activities with peace of mind. The next session was scheduled 4 weeks later because, in O’s experience, reflections on the perceived changes need some configuration time to emerge fully. At the next meeting a month later, P reported that a lot had happened. Enormous anger, but also strength, emerged in her. She had outbursts of anger in many situations, but also had corresponding feelings of guilt. She must now learn to be careful with the boundaries she feels, so as not to hurt anyone. She was also suddenly able to distinguish mendacity and feigned kindness from genuine kindness, which gave her considerable symptom relief. Stomach pain and sleeping problems (which she initially only mentioned in passing) were no longer an issue, and her tinnitus had improved by around 40%. In further sessions, her constantly new discoveries in dealing with herself and topics such as her father’s death that had taken place in the meantime, were taken up. These investigations were conducted during trance or conventional exploration, and were integrated into P’s current horizon of experience, according to her directly conveyed images or feelings that accompanied these themes. Ultimately—according to the last meeting in November 2019—P found that any food, “even foods high in histamine, such as eggplant or radishes,” was tolerable. Her breathing also improved, allowing her to breathe much deeper. She effortlessly distanced herself from her family problems and could now concentrate much better on her further plans. Stressful statements that she had learned in her childhood, such as “Nothing is free in life, my child” or “You have to pay for everything in life, be it freedom, happiness or ultimately even with life,” were now regarded as attitudes or points of view that did not belong to her and had very limited meaning. In accordance with the previous considerations, this brief example shows that the sufferer P experiences herself in a position where she can find verbal symbolizations and events related to her suffering. This has previously been described based on typical cases of somatoform “chronic pelvic pain disorder” .
| 3.318359
| 0.648926
|
sec[3]/sec[1]/p[1]
|
en
| 0.999996
|
PMC9616690
|
https://doi.org/10.3389/fpsyg.2022.821566
|
[
"time",
"your",
"pause",
"asked",
"trance",
"feeling",
"experience",
"next"
] |
[
{
"code": "PL13.52",
"title": "Incorrect timing of drug or medicament, as mode of injury"
},
{
"code": "QF2A",
"title": "Difficulty or need for assistance with community participation"
},
{
"code": "MF50.1",
"title": "Pollakiuria"
},
{
"code": "JA25.3",
"title": "Eclampsia, time period unspecified"
},
{
"code": "KD3B.Z",
"title": "Unspecified time of fetal death, cause not specified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Incorrect timing of drug or medicament, as mode of injury (PL13.52)】
Synonyms: wrong timing of drug | timing error in giving drug | timing mistake in administration of drug | administration error involving timing of drug | medication error involving timing of drug
Excludes: Problem with delayed treatment | Overdose of substance, as mode of injury or harm
Hierarchy: Causes of healthcare related harm or injury → Mode of injury or harm associated with exposure to a drug, medicament or biological substance (PL13) → Incorrect administration of drug or medicament, as mode of injury (PL13.5) → Incorrect timing of drug or medicament, as mode of injury
【2. Difficulty or need for assistance with community participation (QF2A)】
Synonyms: difficulty with community participation | need for assistance with community participation | need for assistance with community, social and civic life | difficulty with community, social and civic life | Lack of relaxation or leisure
Hierarchy: Factors influencing health status or contact with health services (24) → Factors influencing health status → Difficulty or need for assistance with activities → Difficulty or need for assistance with community participation
【3. Pollakiuria (MF50.1)】
Synonyms: pollakisuria | Daytime frequency of micturition
Hierarchy: Symptoms, signs or clinical findings of the genitourinary system → Symptoms, signs or clinical findings involving the urinary system → Abnormal micturition (MF50) → Pollakiuria
【4. Eclampsia, time period unspecified (JA25.3)】
Definition: Onset of convulsions in a woman with pre-eclampsia not attributable to other causes without a specific onset time.
Synonyms: Eclampsia NOS | eclamptic coma | eclamptic toxaemia | toxaemia with convulsions | Convulsions following gestational [pregnancy-induced] hypertension with significant proteinuria
Hierarchy: Pregnancy, childbirth or the puerperium (18) → Oedema, proteinuria, or hypertensive disorders in pregnancy, childbirth, or the puerperium → Eclampsia (JA25) → Eclampsia, time period unspecified
【5. Unspecified time of fetal death, cause not specified (KD3B.Z)】
Synonyms: Fetal death, cause not specified | stillbirth NOS | stillborn NOS | intrauterine fetal demise | osteopedion fetus
Hierarchy: Certain conditions originating in the perinatal period (19) → Certain disorders originating in the perinatal period → Fetal death, cause not specified (KD3B) → Unspecified time of fetal death, cause not specified
|
PL13.52
|
Incorrect timing of drug or medicament, as mode of injury
|
The patient came to the Hospital de Día de Pie Diabético at the Polo Sanitario of Los Polvorines, in Malvinas Argentinas, Province of Buenos Aires, Argentina, (Diagnostics Table 1 , Timeline Table 2 ), on October 23, 2020, to see a doctor about necrosis on the fifth toe of the right foot. A biopsy was performed on the remaining bone, testing positive for Pseudomonas aeruginosa . Lab results came back with high inflammatory markers. A targeted antibiotic treatment was prescribed. Table 1 Diagnostics. Table 1 Type Value Unit 17/10/2020 ALP (alkaline phosphatase) 173 IU/L ALT (alanine aminotransferase) 16 IU/L AST (aspartate aminotransferase) 40 U/L Cholesterol, HDL 47 mg/dL Cholesterol, LDL 161 mg/dL Cholesterol, total 226 mg/dL Creatinine 1.25 mg/dL CRP (C-reactive protein) 7 mg/dL ESR (erythrocyte sedimentation rate) 18 mm/1st hour Glucose 104 mg/dL Hb (hemoglobin) 11.67 g/dL HbA1C (hemoglobin A1C) 6.5 % Hct (hematocrit) 39 % Platelet count 193,000 cells/μL Serum urea 36 mg/dL Triglycerides 90 mg/dL 12/2/2021 Creatinine 1.08 mg/dL CRP (C-reactive protein) 48 mg/dL CSF white blood cell count 15,000 cells/μL ESR (erythrocyte sedimentation rate) 85 mm/1st hour Glucose 118 mg/dL Hb (hemoglobin) 11.8 g/dL HbA1C (hemoglobin A1C) 6.6 % Hct (hematocrit) 36.8 % Serum urea 32 mg/dL 15/3/2021 Creatinine 1.49 mg/dL CRP (C-reactive protein) 12 mg/dL CSF white blood cell count 8400 cells/μL Glucose 119 mg/dL Hb (hemoglobin) 95 g/dL HbA1C (hemoglobin A1C) 6.5 % Hct (hematocrit) 33.4 % Platelet count 276.600 cells/μL Serum urea 35 mg/dL 31/5/2021 ALP (alkaline phosphatase) 202 IU/L ALT (alanine aminotransferase) 14 IU/L AST (aspartate aminotransferase) 45 IU/L Cholesterol, HDL 40 mg/dL Cholesterol, LDL 81 mg/dL Creatinine 1.13 mg/dL CRP (C-reactive protein) 12 mg/dL CSF red blood cell count 9700 cells/μL ESR (erythrocyte sedimentation rate) 10 mm/1st hour Glucose 110 mg/dL Hb (hemoglobin) 93 g/dL HbA1C (hemoglobin A1C) 6 % Hct (hematocrit) 311 % Platelet count 359,900 cells/μL Serum urea 23 mg/dL Triglycerides 46 mg/dL Table 2 Timeline. Table 2 22/10/2020 Consultation due to necrosis of the fifth toe, right foot, bone cannula, iodoformedgauze healing, bone bx requested, empirical treatment started VO AMC –TMS, laboratories requested, nutrition and education ic 3/11/2020 Poor evolution ATB is rotated to the DELABAXI protocol (delafloxacin 450 mg) PPS culture is taken and right foot X-ray control, management with 26 IU I Giargina sc C/24 h arterial Doppler of lower limbs is requested 15/2/2021 Arterial Doppler result on 02/16/21 right side tib before and after decreased velocities, monophasic, compatible with moderate obstructive compromise 22/2/2021 Negative pps culture results from 4/11/2020 AMC and TMS The patient's treatment regimen included cilostazol (100 mg every 12 h), rosuvastatin (20 mg), aspirin (100 mg), and clopidogrel (75 mg). In addition, the evolution of the wound was monitored in collaboration with the diabetic foot care team. 23/2/2021 Spontaneous amputation of the fifth finger, dry necrosis of the fourth toe on the right foot, healing of iodine-formed gauzes. RX right foot osteomyelitis, fourth and fifth toe 2/3/2021 Lower limb arteriography is requested, fourth, third and finger necrosis is delimited with alcohol, second finger with cyanosis alcohol is indicated 4/4/2021 Vascular surgery indicates 5/4/2021 Result of angiography performed on 30/3/2021 right side proximal occluded anterior tibial artery, occluded proximal peroneal artery, occluded posterior tibial artery. 5/4/2021 Left side proximal occluded anterior tibial artery, slow flow peroneal artery, occludes at the distal level, occluded posterior tibial artery, continuous cures with alcohol 5/4/2021 Angiography 30/3/2021 Right proximal occluded anterior tibial artery, occluded proximal fibular artery and posterior tibial artery. Left proximal tibial anterior artery occluded, peroneal slow flow, occluded distally, posterior tibial artery occluded. We immediately consulted with the vascular surgery area, where it was determined that revascularization was not a possibility in this case, given the moderate-to-severe nature of the stenosis. 21/4/2021 Digital necrosis delimited with alcohol, that day vascular surgery indicates digital amputation, IC with O and T from the Diabetic Foot team is requested. AMG 90–120 with symptoms of hypoglycemia is indicated I Giargin 20 IU 26/4/2021 Due to poor evolution and cyanosis of the hallux, transmetatarsal amputation was decided in the operating room, samples of the remaining bone were taken for culture. 28/4/2021 Postoperative control of transmetatarsal amputation with bleeding is indicated flat cure with alcohol control with O and T 28/5/2021 Positive bone biopsy result: Citrobacter freundii , Pseudomona aeruginosa, Enterococcus faecalis 28/5/2021 It presents with necrosis of the edges, distal points are removed, healing with iodoformed gauzes, necrosis of the lateral edges is observed, poor evolution Treatment of delafloxacin 450 mg iv every 12 h at home 8/6/2021 Transmetarsal amputation ulcer devitalized tissue in the amputation site bed, moderate to severe pain in treatment with Delafloxacin IV, Toilette and bone bx taken in the operating room by O and T. Healing with iodine-formed gauzes and collagenase 11/6/2021 Healing with pocket iodine-formed gauzes and granulating base with collagenase 15/6/2021 Amputation stump ulcer with abundant exudate and fibrin, iodine-formed gauzes indicated moderate pain at the time of healing, the appointment for control with alarm guidelines. Bone bx culture result received from 12/6/2021 IM penicillin is indicated 15/6/2021 Bone biopsy culture result received on 12/6/21 was positive for: Pseudomona aeuriginosa , Enterococcus Faecalis , Escherichia Coli 22/6/2021 Poor evolution and contamination of the wound. Cleaning with soap and water and treatment every 12 h at home with gauze soaked in Silver Sulfadiazine, Lidocaine and Vitamin A and Silver Sulfadiazine, Lidocaine and Vitamin A spray are indicated. 5/7/2021 He attends control with improvement in control of exudate, decrease in pain, mechanical debridement is performed with a continuous scalpel, cures with soaked gauzes and silver sulfadiazine spray, lidocaine, and vitamin A every 12 h. 2/8/2021 Concurs healing with marked improvement in pain and good evolution background of amputation stump ulcer with decreased fibrin, continuous healing at home with aerosol and gauze soaked in silver sulfadiazine, lidocaine, vitamin A 2/8/2021 Wound with good evolution. Lesion area: 164.42 cm 2 ; perimeter: 52.17 cm; necrotic tissue area: 52.01 %; Granulation area percentage: 31.6 % 16/9/2021 Clopidogrel 75 mg every 24 h is added, amputation of stump ulcer with fibrinogranulating bottom, diameter decrease and pain control, continuous healing at home with aerosol and gauze soaked with silver sulfadiazine, vitamin lidocaine 5/10/2021 Wound with good evolution. Lesion area: 22.31 cm 2 ; perimeter: 23.67 cm; necrotic tissue area: 14.07 %; Granulation area percentage: 85.85 % 5/10/2021 Amputation stump ulcer with fibrinogranulating background, a notable decrease in diameter, patient reports no pain even at the time of healing. Continuous home treatment with aerosol and gauze soaked with silver sulfadiazine, lidocaine, vitamin A 8/11/2021 Good evolution, currently without pain without antibiotic therapy and decrease in diameter, continues with cures at home with aerosol and gauze soaked with silver sulfadiazine, lidocaine, vitamin A 8/11/2021 Wound with good evolution. Lesion area: 18.015 cm 2 ; perimeter: 28.319 cm; necrotic tissue area %: 1.729; Granulation area percentage: 96.0 % 21/12/2021 Ulcer instead of amputation stump, with good evolution lesion area 1.18 cm 2 , perimeter 7.623 cm, necrotic tissue area 1.159 %, granulation percentage 98.2 %
| 3.914063
| 0.945801
|
sec[1]/p[7]
|
en
| 0.999996
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38219509
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https://doi.org/10.1016/j.ijscr.2023.109180
|
[
"area",
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"amputation",
"artery",
"healing",
"occluded",
"bone",
"hemoglobin"
] |
[
{
"code": "QF29",
"title": "Difficulty or need for assistance with major areas of life"
},
{
"code": "EH40.1Y",
"title": "Other specified infantile napkin dermatoses"
},
{
"code": "GA90",
"title": "Hyperplasia of prostate"
},
{
"code": "2B66.Z",
"title": "Malignant neoplasms of other or unspecified parts of mouth, unspecified"
},
{
"code": "EG63.Z",
"title": "Sacrococcygeal pilonidal disease, unspecified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Difficulty or need for assistance with major areas of life (QF29)】
Synonyms: difficulty with major areas of life | need for assistance with major areas of life | Difficulty or need for assistance with education | Difficulty or needs for assistance with work and economic life
Hierarchy: Factors influencing health status or contact with health services (24) → Factors influencing health status → Difficulty or need for assistance with activities → Difficulty or need for assistance with major areas of life
【2. Other specified infantile napkin dermatoses (EH40.1Y)】
Synonyms: Infections of the napkin area
Hierarchy: Skin disorders associated with pregnancy, the neonatal period or infancy → Dermatoses of infancy (EH40) → Infantile napkin dermatoses (EH40.1) → Other specified infantile napkin dermatoses
【3. Hyperplasia of prostate (GA90)】
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.
Synonyms: Adenofibromatous hypertrophy of prostate | benign prostatic hyperplasia | prostate hyperplasia | prostatic area hypertrophy | prostatic hypertrophy
Excludes: Benign neoplasms of prostate
Hierarchy: Diseases of the genitourinary system (16) → Diseases of the male genital system → Diseases of prostate → Hyperplasia of prostate
【4. Malignant neoplasms of other or unspecified parts of mouth, unspecified (2B66.Z)】
Synonyms: Malignant neoplasms of other or unspecified parts of mouth | cancer of buccal mucosa | cancer of cheek mucosa | internal cheek cancer | malignant neoplasm of buccal mucosa NOS
Hierarchy: Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues → Malignant neoplasms of lip, oral cavity or pharynx → Malignant neoplasms of other or unspecified parts of mouth (2B66) → Malignant neoplasms of other or unspecified parts of mouth, unspecified
【5. Sacrococcygeal pilonidal disease, unspecified (EG63.Z)】
Synonyms: Sacrococcygeal pilonidal disease | pilonidal disease of sacrococcygeal area | sacrococcygeal pilonidal disease NOS
Hierarchy: Skin disorders involving the genital and perianal regions → Dermatoses of the anus, perianal area or perineum → Sacrococcygeal pilonidal disease (EG63) → Sacrococcygeal pilonidal disease, unspecified
|
QF29
|
Difficulty or need for assistance with major areas of life
|
On admission, the patient’s height, weight, body mass index, waist circumference, and arm span were 161.1 cm, 97.1 kg, 37.4 kg/m 2 , 111.2 cm, and 160.0 cm, respectively. Her visceral fat area estimated via bioelectrical impedance analysis using EW-FA90 (Panasonic Corporation, Osaka, Japan) was 166 cm 2 . She had no history of head trauma or head surgery, and neither drank alcohol nor took any medications. Although her maternal grandmother had obesity and diabetes, she had no other family members with obesity, diabetes, or endocrinological disorders, and there were no consanguineous marriages in her family. She had primary amenorrhea and was at Tanner stage 2 for both pubic hair and breast development. Her intelligence quotient, as determined by the Wechsler Adult Intelligence Scale, fourth edition, was 60, indicating mild intellectual disability. Her laboratory findings are presented in Table 1 . Her hemoglobin A1c level was 9.7%. She had thrombocytopenia, prolonged prothrombin time, and elevated liver fibrosis marker levels, suggestive of liver cirrhosis. Viral hepatitis, autoimmune hepatitis, primary biliary cholangitis, Wilson’s disease, and alcoholic hepatitis were unlikely differential diagnoses, suggesting that her liver dysfunction was caused by nonalcoholic steatohepatitis (NASH). Her endocrinological findings are presented in Tables 1 and 2 . HH and growth hormone deficiency (GHD) were confirmed by comparing baseline hormonal levels and the results of stimulation tests. Central hypothyroidism (CH) was diagnosed due to low free thyroxine level and normal or mildly elevated TSH levels at the baseline, although the TSH level responded normally to thyrotropin-releasing hormone (TRH) stimulation. Plasma ACTH levels showed normal responses in both the insulin tolerance test (ITT) and corticotropin-releasing hormone (CRH) stimulation test. Cortisol response was almost normal in the rapid ACTH stimulation test but blunted in the ITT and CRH stimulation test. Based on these results and the absence of clinical signs of adrenal insufficiency, central adrenal insufficiency was not apparent. Her prolactin level responded normally to TRH stimulation. Thus, she was diagnosed with CPHD (combined HH, GHD, and CH). Brain magnetic resonance imaging (MRI) revealed anterior pituitary hypoplasia . There were no other abnormal intracranial findings, including those of the posterior pituitary and pituitary stalk. Abdominal MRI showed morphological features of cirrhosis and esophagogastroduodenoscopy (EGD) showed esophageal varices . Liver biopsy revealed severe fibrosis . Although there was no histological evidence of NASH, such as severe steatosis or ballooning, the clinical course and laboratory findings shown in Table 1 suggested that the liver cirrhosis was caused by burnt-out NASH. Based on these results, we diagnosed her with CPHD (combined HH, GHD, and CH) with comorbid diabetes, severe obesity, and liver cirrhosis probably due to NASH. She started treatment with recombinant GH, levothyroxine sodium, and estrogen/progesterone therapy. Her glycemic control was remarkably improved by treatment with linagliptin and empagliflozin. Table 1 Laboratory characteristics of the patient at the time of admission Hematologic characteristics CPR 1.5 ng/mL (1.0–1.6) WBC 3,900 /µL (3,500–9,800) GADAb < 5.0 U/mL (< 5.0) RBC 435 × 10 4 /µL (376–500) HBsAg 0.00 IU/mL (0.00–0.04) Hemoglobin 13.1 g/dL (11.3–15.2) HCVAb 0.1 S/CO (< 1.0) Platelet 5.5 × 10 4 /µL (13.0–36.9) AMA2 < 1.5 (< 7) Biochemical characteristics ANA 20 (< 40) Total cholesterol 161 mg/dL (139–220) Iron 103 µg/dL (40–188) Triglycerides 113 mg/dL (36–149) TIBC 265 µg/dL (246–410) HDL-C 48 mg/dL (40–87) Ferritin 121 ng/mL (5–152) LDL-C 88 mg/dL (59–139) Ceruloplasmin 25 mg/dL (21–37) Urea nitrogen 9.7 mg/dL (8.4–20.4) M2BPGi 4.27 (< 1.0) Creatinine 0.50 mg/dL (0.40–0.74) 4C7S 12.2 ng/mL (< 4.4) eGFR 112.2 mL/min/1.73 m 2 Endocrinological characteristics Total protein 6.7 g/dL (6.7–8.2) LH < 0.10 mIU/mL Albumin 3.6 g/dL (4.0–4.8) FSH < 0.10 mIU/mL Total bilirubin 2.0 mg/dL (0.2–1.2) Estradiol 24.0 pg/mL AST 43 U/L (10–33) Growth hormone < 0.03 ng/mL (0.13–9.88) ALT 22 U/L (6–35) IGF-1 < 7 ng/mL (119–283) ALP 100 U/L (38–113) TSH 5.18 µU/mL (0.61–4.23) γ-GTP 152 U/L (8–60) FT3 1.33 pg/mL (1.68–3.67) PT 54.9 % (70–120) FT4 0.63 ng/dL (0.7–1.48) APTT 38.4 sec (23.0–39.0) ACTH 28.8 pg/mL (7.2–63.3) Uric acid 3.9 mg/dL (2.2–6.7) Cortisol 5.0 µg/dL (3.7–19.4) Sodium 142 mEq/L (135–147) DHEA-S 33 µg/dL (58–327) Potassium 3.6 mEq/L (3.6–5.0) Prolactin 12.60 ng/mL (6.12–30.54) Chloride 105 mEq/L (98–108) Urinalysis findings Calcium 8.8 mg/dL (8.8–10.2) U-CPR 172 µg/day (29.2–167) FPG 115 mg/dL (70–110) U-Albumin 9 mg/day (< 30) Hemoglobin A1c 9.7 % (4.6–6.2) U-Copper 13.5 µg/day (2.5–20.0) IRI 10.6 µU/mL (5–10) U-Cortisol 27.6 µg/day (5.5–66.7) Reference ranges are shown in parentheses WBC white blood cell count, RBC red blood cell count, HDL-C high-density lipoprotein cholesterol, LDL-C low-density lipoprotein cholesterol, eGFR estimated glomerular filtration rate, AST aspartate aminotransferase, ALT alanine aminotransferase, ALP alkaline phosphatase, γ-GTP γ-glutamyl transpeptidase, PT prothrombin time, APTT activated partial thromboplastin time, FPG fasting plasma glucose, IRI immunoreactive insulin, CPR C-peptide immunoreactivity, GADAb anti-glutamic acid decarboxylase antibody, HBsAg hepatitis B surface antigen, HCVAb hepatitis C virus antibody, AMA2 anti-mitochondrial M2 antibody, ANA antinuclear antibody, TIBC total iron binding capacity, M2BPGi Mac-2 binding protein glycosylated isomers, 4C7S type IV collagen 7S, LH luteinizing hormone, FSH follicle-stimulating hormone, IGF-1 insulin-like growth factor-1, TSH thyroid-stimulating hormone, FT3 free 3,5,3′-triiodothyronine, FT4 free thyroxine, ACTH adrenocorticotropic hormone, DHEA-S dehydroepiandrosterone sulfate Table 2 Results of patient’s pituitary stimulation tests LHRH stimulation test 0 min 30 min 60 min 90 min 120 min LH, mIU/mL < 0.10 0.15 0.13 0.13 0.13 FSH, mIU/mL < 0.10 0.16 0.19 0.25 0.28 Insulin tolerance test 0 min 15 min 30 min 45 min 60 min 75 min 90 min 120 min GH, ng/mL (0.13–9.88) < 0.03 0.04 0.07 0.10 0.08 0.05 0.05 0.05 ACTH, pg/mL (7.2–63.3) 25.3 25.2 26.3 112.2 253.1 128.9 79.9 47.1 Cortisol, µg/dL (3.7–19.4) 4.1 5.3 4.2 7.1 13.7 14.7 13.8 10.6 Glucose, mg/dL (70–110) 103 85 50 37 127 89 55 66 GHRP-2 stimulation test 0 min 15 min 30 min 45 min 60 min GH, ng/mL (0.13–9.88) < 0.03 0.05 0.04 < 0.03 < 0.03 TRH stimulation test 0 min 30 min 60 min 90 min 120 min TSH, µU/mL (0.61–4.23) 3.83 36.23 36.80 35.22 29.06 PRL, ng/mL (6.12–30.54) 12.83 31.73 19.84 17.86 15.82 CRH stimulation test 0 min 30 min 60 min 90 min 120 min ACTH, pg/mL (7.2–63.3) 17.7 95.5 62.8 39.0 33.5 Cortisol, µg/dL (3.7–19.4) 6.5 14.8 14.3 12.1 10.8 Rapid ACTH test 0 min 30 min 60 min Cortisol, µg/dL (3.7–19.4) 4.6 15.3 17.6 Reference ranges are shown in parentheses LHRH luteinizing hormone releasing hormone, LH luteinizing hormone, FSH follicle-stimulating hormone, GH growth hormone, ACTH adrenocorticotropic hormone, GHRP-2 growth hormone releasing peptide-2, TRH thyrotropin-releasing hormone, TSH thyroid-stimulating hormone, PRL prolactin, FT4 free thyroxine, CRH corticotropin-releasing hormone Fig. 1 Gadolinium-enhanced brain magnetic resonance imaging showing anterior pituitary hypoplasia (circle) Fig. 2 a : Abdominal magnetic resonance imaging showing morphological features characteristic of cirrhosis such as irregularity of the liver surface, enlargement of the left lobe, and splenomegaly (arrowhead). b : Esophagogastroduodenoscopy showing esophagus varices (arrowhead). c : Histological analysis of the liver with Masson’s trichrome staining showing severe fibrosis (arrow) and mild steatosis (arrowhead)
| 4.1875
| 0.966797
|
sec[1]/p[1]
|
en
| 0.999997
|
PMC10210328
|
https://doi.org/10.1186/s12902-023-01373-8
|
[
"hormone",
"stimulation",
"test",
"liver",
"acth",
"releasing",
"cortisol",
"findings"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Endocrine diseases, unspecified (5B3Z)】
Synonyms: endocrine disorder NOS | disorder of endocrine gland | disease of endocrine gland | disorder of endocrine system | disorder of hormones
Hierarchy: Endocrine, nutritional or metabolic diseases (05) → Endocrine diseases → Endocrine diseases, unspecified
【2. Harmful pattern of use of non-psychoactive substances, unspecified (6C4H.1Z)】
Synonyms: 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 | Abuse of hormones
Hierarchy: Disorders due to substance use → Disorders due to use of non-psychoactive substances (6C4H) → Harmful pattern of use of non-psychoactive substances (6C4H.1) → Harmful pattern of use of non-psychoactive substances, unspecified
【3. Growth hormone deficiency (5A61.3)】
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.
Excludes: Hypopituitarism
Hierarchy: Endocrine diseases → Disorders of the pituitary hormone system → Hypofunction or certain other specified disorders of pituitary gland (5A61) → Growth hormone deficiency
【4. Hypopituitarism (5A61.0)】
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.
Synonyms: subpituitarism | hypophyseal dystrophy | hypohypophysism | anterior pituitary insufficiency (in part) | deficient secretion of one or more pituitary hormones
Hierarchy: Endocrine diseases → Disorders of the pituitary hormone system → Hypofunction or certain other specified disorders of pituitary gland (5A61) → Hypopituitarism
【5. Testicular hypofunction (5A81.1)】
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).
Synonyms: hypofunction testicle | gonadal insufficiency of testis | Testicular hypogonadism NOS | undeveloped testis | testicular hypogonadism
Hierarchy: Endocrine diseases → Disorders of the gonadal hormone system → Testicular dysfunction or testosterone-related disorders (5A81) → Testicular hypofunction
|
5B3Z
|
Endocrine diseases, unspecified
|
A non-contrast brain MRI scan at the First Affiliated Hospital, Sun Yat-sen University, 2 months prior revealed slight ischemia in the bilateral frontal lobes. Color ultrasound at the First Affiliated Hospital, Sun Yat-sen University 2 months prior revealed a hypoechoic lesion in the left testicle with abundant blood supply, a high probability of inflammation, and a cyst in the head of the right epididymis; no abnormalities of the right testicle or left epididymis were found on ultrasound. Electronic gastroscopy at Shenzhen People’s Hospital 1 month previously suggested chronic superficial gastritis with erosion. Pathology revealed chronic active inflammation in the gastric antrum. No significant abnormalities were detected via routine blood tests, routine urine tests, routine stool tests, coagulation panels (4 items), thyroid panels (3 items), erythrocyte sedimentation rate, fasting blood glucose levels, glycosylated hemoglobin levels, renal function tests, myocardial damage, C-reactive protein levels, electrolyte levels, or liver function examinations performed upon admission. Vitamin B12 was 917 ↑ pmol/L, uric acid was 450 ↑ µmol/L, total blood cholesterol was 2.60 ↓ mmol/L, high-density lipoprotein was 0.77 ↓ mmol/L, and low-density lipoprotein was 1.44 ↓ mmol/L. No obvious abnormalities were detected in the urine Bence-Jones protein or chorionic gonadotropin. Immunoglobulin and complement: β2-microglobulin 2.65 ↑ mg/L. Among tumor markers, no significant abnormalities in alpha-fetoprotein, carcinoembryonic antigen, CA125, CA19-9, CA153, CA72-4, total prostate-specific antigen or free prostate-specific antigen were detected. Tests of rheumatic immune antibodies revealed that anti-CCP antibodies, anti-ENA antibodies, antinuclear antibodies, vasculitis antibody series, antiphospholipid antibody series, and rheumatoid arthritis antibody series were negative. Demyelinating disease examination of the central nervous system revealed that the anti-AQP4 antibody IgG, anti-MOG antibody IgG, and anti-MBP antibody IgG were negative. Paraneoplastic syndrome examination revealed that anti-Hu antibody IgG, anti-Yo antibody IgG, anti-Ri antibody IgG, anti-CV2 antibody IgG, anti-Ma2 antibody IgG, anti-amphiphysin antibody IgG, anti-ANNA-3 antibody IgG, anti-PCA-2IgG antibody, and anti-GAD antibody IgG were negative. Parasite panels of the liver, lungs, and brain showed that antibodies for liver fluke, lung fluke, hydatid, Toxoplasma gondii, cerebral cysticercus, sparganum, and Schistosoma japonicum were negative. In the peripheral blood smear , the morphology of the granulocytes, lymphocytes and platelets was basically normal. Mild anisocytosis was observed in some mature erythrocytes. Some monocytes had intracytoplasmic vacuoles. No nucleated erythrocytes, plasmodium, or microfilaria were observed. Bone marrow puncture revealed the following findings : active bone marrow hyperplasia; hyperplasia of granulocytes, erythrocytes and megakaryocytes; slightly high levels of plasma cells; and individual atypical lymphocytes. In the cerebrospinal fluid, pressure was 165 mm H 2 O, glucose was 3.09 ↓ mmol/L, protein was 0.757 ↑ g/L, globulin (qualitative) was positive (+), and cell count was 4/µL. Bacterial, Mycobacterium tuberculosis and fungal cultures were negative. Cytopathologic diagnosis of cerebrospinal fluid revealed visible individual lymphocytes in the smear. Electrocardiogram was normal. A noncontrast brain CT scan revealed no obvious abnormalities. CT of the nasopharynx, neck, chest and whole abdomen (pelvis included) revealed multiple small lymph nodes in the bilateral neck and groin and mild fatty liver; no definite abnormal signs observed on the noncontrast CT scan of the remaining regions of the nasopharynx, neck, chest, or whole abdomen. On transcranial Doppler, the blood flow velocity of the right vertebral artery was slightly decreased. Color ultrasound of the cervical vessels showed no abnormal echoes or images in the bilateral carotid or vertebral arteries. Color ultrasound of the superficial mass revealed a subcutaneous solid lesion behind the neck. Color ultrasound of extremity vessels showed no obviously abnormal echoes or images in the arteries or veins of the bilateral lower extremities. Heart color ultrasound revealed no significant abnormalities in the morphology, structure or valve mobility of the heart. No obvious abnormalities were observed in ventricular wall motion at rest. The left ventricle showed normal systolic function. Scrotum color ultrasound revealed abnormal echoes and images in the left testicle, and an inflammatory lesion was suspected. No abnormal echoes or images were observed in the right testicle. No abnormal echoes or images were noted in the bilateral epididymides. Left spermatic corditis was observed. There was no obvious abnormality in the right spermatic cord. On electromyography, incomplete damage to the right facial nerve, left facial nerve damage(manifestation during the recovery period of chronic lesions) and mild facial muscle synkinesis (left side) were observed. The distal motor and sensory conduction functions of the peripheral nerves of both lower extremities and the right upper extremity were normal or somewhat poor (sensory nerve action potential of the left superficial peroneal nerve was significantly decreased). The proximal motor conduction F wave was significantly impaired (the F wave of the tibial nerve of the right lower extremity was significantly slowed, with a markedly reduced occurrence rate) or slightly impaired (the F wave occurrence rate of the median and ulnar nerves of the right upper extremity was lower). H reflex examination of the left and right tibial nerves (soleus muscle) revealed no definite H reflex, indicating poor nerve conduction function at the proximal end of S1. The medial vastus muscle, tibialis anterior muscle, and gastrocnemius muscle of the right lower extremity and the left and right digit abductor muscles exhibited mild neurogenic lesions on electromyography, which were more significant in the terminal muscles. Several evoked potential tests were performed. Pattern reversal visual evoked potentials: The visual pathways of the eyes showed normal conduction function. Brainstem auditory evoked potential: there was slow conduction in the superior segment of the brainstem in the auditory pathway of the ears (III–V wave peak interval > I–III wave peak interval). Somatosensory evoked potential: The somatosensory pathway of both lower extremities showed normal conduction function upon stimulation of the medial malleolus segment of the tibial nerve of the lower extremities (no abnormalities in P40 and N9 latency). Brain + non-contrast MRA + spine and spinal cord MRI revealed the following: no abnormal signs on non-contrast MR scans or MRAs of the head; abnormal signals at T10 and T11, suggesting fat deposition; and mild backward herniation of the C4/5-C6/7 intervertebral disc, cervical spine degeneration, a high possibility of small hemangioma in C7, and backward herniation of the L4/5 intervertebral disc. Lymph node histopathology revealed high-grade B-cell lymphoma, which was consistent with double-hit lymphoma . Immunohistochemistry (IHC): CD3(−), CD5(−), CD20(+), CD79a(+), Bcl2(weakly+), Bc16(+), CD10(−), CD21(FDC+), Muml(partially +), Ki67(appr. 90%+), PAX5(+), CD23(FDC+), CyclinD1(−), Bc12(+/−), and MPO(−). In situ hybridization revealed EBERs(−). The fluorescence in situ hybridization results were as follows: IgH/Bcl2 t (14:18)(q32;q21) chromosomal translocation: negative; Bc16 gene translocation: positive; Myc(8;q24) chromosomal translocation: positive. The clonal gene rearrangement test for B-cell lymphoma was positive, and the clonal gene rearrangement test for T-cell lymphoma was negative.
| 4.148438
| 0.67041
|
sec[1]/p[2]
|
en
| 0.999996
|
PMC11688034
|
https://doi.org/10.1097/MD.0000000000041097
|
[
"antibody",
"anti",
"abnormalities",
"ultrasound",
"nerve",
"color",
"blood",
"tests"
] |
[
{
"code": "JA86.Y",
"title": "Maternal care for other specified fetal problems"
},
{
"code": "MA14.14",
"title": "Anti-nuclear antibody positive"
},
{
"code": "MA14.13",
"title": "Anti-nuclear antibody negative"
},
{
"code": "JA86.0",
"title": "Maternal care for red cell antibodies"
},
{
"code": "MA14.1C",
"title": "Raised antibody titre"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Maternal care for other specified fetal problems (JA86.Y)】
Synonyms: Maternal care for other isoimmunization | Isoimmunization NOS | maternal antibodies NOS | pregnancy management affected by incompatibility of blood groups NOS | Maternal care for ABO isoimmunisation
Hierarchy: Pregnancy, childbirth or the puerperium (18) → Maternal care related to the fetus, amniotic cavity or possible delivery problems → Maternal care for other fetal problems (JA86) → Maternal care for other specified fetal problems
【2. Anti-nuclear antibody positive (MA14.14)】
Synonyms: ANA - [anti-nuclear antibody] positive
Hierarchy: Clinical findings in blood, blood-forming organs, or the immune system → Immunological findings in blood, blood-forming organs, or the immune system (MA14) → Certain specified immunological findings (MA14.1) → Anti-nuclear antibody positive
【3. Anti-nuclear antibody negative (MA14.13)】
Synonyms: ANA - [anti-nuclear antibody] negative
Hierarchy: Clinical findings in blood, blood-forming organs, or the immune system → Immunological findings in blood, blood-forming organs, or the immune system (MA14) → Certain specified immunological findings (MA14.1) → Anti-nuclear antibody negative
【4. Maternal care for red cell antibodies (JA86.0)】
Definition: Maternal care for rhesus or other isoimmunization
Synonyms: Maternal care for rhesus isoimmunization | Rh factor immunization affecting management of pregnancy | Rh incompatibility | Rh incompatibility with hydrops fetalis | Anti-D [Rh] antibodies
Hierarchy: Pregnancy, childbirth or the puerperium (18) → Maternal care related to the fetus, amniotic cavity or possible delivery problems → Maternal care for other fetal problems (JA86) → Maternal care for red cell antibodies
【5. Raised antibody titre (MA14.1C)】
Synonyms: antibody titre above reference range | high antibody titre | increased antibody titre
Excludes: isoimmunization, in pregnancy affecting fetus or newborn
Hierarchy: Clinical findings in blood, blood-forming organs, or the immune system → Immunological findings in blood, blood-forming organs, or the immune system (MA14) → Certain specified immunological findings (MA14.1) → Raised antibody titre
|
JA86.Y
|
Maternal care for other specified fetal problems
|
The patient was a 49-year-old self-employed male in good health who liked to tend to flowers and plants and was found to show evidence of mouse bites. The patient developed a cough at home 4 days prior to admission. Occasionally, the sputum was tinged with red blood, which was accompanied by pharyngeal and knee pain; his highest temperature was 39.2 °C and was accompanied by chills. He did not experience night sweats, herpes of the mouth, chest pain, abdominal pain, diarrhea, hoarseness, headache, vomiting, etc. He visited our hospital because of chest tightness after activity. Upon admission, the patient was conscious, with a temperature of 37.5 °C, a pulse rate of 105 beats/min, a respiration rate of 18 breaths/min, and a blood pressure of 100/49 mmHg. The following observations were recorded: the bulbous conjunctiva was free of congestion, his lips were not bluish, wet rales were heard in both lungs, his heart rate was 105 beats/minute. The laboratory tests showed a c-reactive protein level of 90.87 mg/L, and white blood cell count, 9.6 × 10 9 /L; hemoglobin, 117 g/L,platelet count, 96 × 10 9 /L. The results of the blood gas analysis revealed a PO 2 of 119 mmHg, pH of 7.43; PCO 2 of 38 mmHg, and procalcitonin (PCT) level of 4 pg/mL (Table 1 ). The chest CT revealed bilateral pulmonary infiltrations . After admission, piperacillin tazobactam was administered at a dose of 4.5 g iv q8h due to moderate severe pneumonia, a 40 mg methylprednisolone injection was administered to control body temperature, and supportive nasal catheter oxygenation was provided (5 L/min). The patient’s condition worsened after 4 h, with a temperature increased to 39 °C, a heart rate of 150 beats/min, a respiratory rate of 26 breaths/min, a blood pressure of 85/55 mmHg, shortness of breath, but no chills; additionally, the patient’s SpO 2 level through the nasal catheter decreased to 70%. The patient’s SpO 2 increased to 86%, when switched to a venturi mask, but his shortness of breath persisted. The patient was transferred to ICU for adequate fluid resuscitation and blood culture is collected. The patient soon exhibited hemoptysis, his SpO 2 decreased to 67%, the PO 2 of 43 mmHg, blood pressure was 96/56 mmHg, and wet rales in both lungs had significantly increased. A pulse index continuous cardiac output examination was performed, which resulted in a extravascular lung water index of 36.9 mL/kg (normal range 3.0–7.0) and pulmonary vascular permeability index of 6.0 dn.s.m. 2 /cm 5 (normal range 1.0–3.0). The patient’s heart function was normal, his lung permeability increased, and the presence of extravascular fluid in the lung increased. After 10 h in the ICU, the patient’s condition deteriorated again. He was agitated under noninvasive mechanical ventilation, and the SpO 2 level was difficult to maintain within the normal range. Mechanical ventilation via tracheal intubation was performed. During the process of endotracheal intubation, a large amount of bloody secretions sprouted from the airway, the heart rate rapidly decreased to 40 beats/min, and the arterial pulse and SpO 2 were not detected; Cardiopulmonary resuscitation was implemented immediately. After 1 min of cardiopulmonary resuscitation, the patient recovered spontaneous circulation but was still agitated and in respiratory distress with an SpO 2 of 80%, despite full mechanical ventilator support. Five hours after the patient was sedated using midazolam and fentanyl through continuous micropumping, his shortness of breath was still not relieved. The ventilator provided a small tidal volume ventilation with a frequency of 50–60 breaths/min. The blood gas analysis indicated a pH of 7.19, pCO 2 of 65 mmHg, PO 2 of 61 mmHg, and hemoglobin concentration of 68 g/dL. The patient showed significant pulmonary hemorrhage and clinical manifestations of ARDS. After a discussion among the staff of the cardiology department, thoracic surgery department, ICU, respiratory department and other experts in our hospital, v-vECMO was initiated at 4.5 L/min, with sweeping gas flow through the oxygenator at 4.5 L/ min of 100% oxygen. After the procedure, the patient’s SpO 2 was 100%. The partial thromboplastin time (PTT) was maintained at 40–50 s by administering a heparin infusion, and the PTT was monitored every 4 h to avoid aggravating the pulmonary hemorrhage. The ECMO blood flow was maintained at 4.5 L/min during the first 3 days. Because general community-acquired pneumonia was considered an unlikely diagnosis, 4.5 g of piperacillin tazobactam iv q8h and 0.5 g of azithromycin iv qd as anti-infection medications and 40 mg of methylprednisolone q8h were continued. A tracheoscopic examination prompted diffuse bleeding in the airway and alveolar lavage fluid was collected and for second-generation gene sequencing on the next day. The hemoglobin concentration decreased to 61 g/dL after an infusion with 3 units of red blood cells, and the platelet count decreased to 57 × 10 9 /L. Six units of a red blood cell suspension were applied to correct the anemia, and 10 units of platelets and 600 mL of fresh frozen plasma were re-administered to supplement the clotting factors. The laboratory tests revealed an increase in the PCT level to 77 ng/mL, and the antibiotics were upgraded to 0.5 g of intravenous meropenem q6h for a suspected severe infection. Four days later, second-generation gene sequencing of the patient’s alveolar lavage fluid was positive for Leptospira interrogans . Although the blood culture and urine culture did not detect leptospirosis, prior to discharge, the results showing strongly positive Leptospira serology with an IgM titer of 1:1280 detected using an enzyme-linked immunosorbent assay (ELISA). Leptospirosis was considered, and the patient was administered an injection of 800,000 U of penicillin q8h (with a first dose of 400,000 U) as anti-infective treatment. The patient was sedated using midazolam and fentanyl during the ECMO supportive treatment, and no obvious complications occurred. After 6 days, the chest radiograph of the patient was significantly improved and ECMO was stopped . On day 9, the endotracheal tube was removed, and the patient was transferred to the general ward on day 10. Table 1 Laboratory examination results during ICU hospitalization Days Normal range 0 1 2 3 4 5 6 7 8 9 10 11 WBCx109/L 3.5–9.5 6.4 7.8 10.3 9.7 9.9 11.7 14.2 20.1 23.3 25.3 30.7 20 hemoglobin (g/L) 115–150 117 64 90 102 81 93 99 121 118 117 128 127 platelet (× 109/L) 125–350 96 57 102 118 170 170 197 164 174 186 231 250 PCT (ng/mL) 0.02–0.05 4.7 74 29 12.8 12.8 3.28 1.96 0.95 0.62 0.57 0.4 0.22 CRP (mg/dL) 0–8.0 93 119.6 119 23 16 14 6.69 4.07 6.31 3.51 26.14 53.2 CREA (umol/L) 41–81 90 108 79 87 62 54 46 42 46 42 39 48 AST(u/L) 13–35 26 24 25 28 27 43 51 62 38 28 16 14 ALT(u/L) 7–40 98 93 115 132 150 169 173 135 73 52 37 38 TBIL (umol/L) 3.1–17.1 14.1 41.3 34.7 29 20.3 27.7 24.2 21.2 19.6 17.6 12.3 14.3 PT (s) 9.2–13.1 14.4 29.4 18.4 19.3 23.5 31.3 140 16.6 14.8 12.7 16.5 13.1 APTT (s) 25.4–40.9 27.5 28.9 61.5 40.4 46.4 38.4 38.4 33.3 32.6 32.1 27.5 26.9 INR 0.82–1.15 1.1 1 1.12 1.11 1.14 1.09 1.14 1.23 1.17 1.1 1.01 1.14 FIB (g/l) 2.0–4.0 1.93 1.94 1.66 1.74 1.96 1.83 2.4 2.92 2,.43 1.93 3.4 3.7 Fig. 1 Chest CT on the day of admission. Both lungs presented exudative lesions, which are obvious in the image of the right lung Fig. 2 a : Chest X-ray before ECMO treatment. Diffuse exudation was observed in both lungs, presenting as a large white lung. b : Chest X-ray on day 6 of ECMO treatment. The diffuse exudation in both lungs was significantly absorbed compared with the image captured at admission. c : Chest X-ray on the 9th day after the removal of ECMO. The pneumonia was completely absorbed without residual disease
| 3.822266
| 0.980469
|
sec[1]/p[0]
|
en
| 0.999998
|
33109122
|
https://doi.org/10.1186/s12879-020-05518-1
|
[
"blood",
"chest",
"mmhg",
"rate",
"ecmo",
"admission",
"lungs",
"level"
] |
[
{
"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 CLASSIFICATION ═══
【1. Diseases of the blood or blood-forming organs, unspecified (3C0Z)】
Synonyms: Blood, lymph or spleen disease | blood condition NOS | blood disorder NOS | bone marrow disease NOS | haematologic disease NOS
Hierarchy: Diseases of the blood or blood-forming organs (03) → Diseases of the blood or blood-forming organs, unspecified
【2. Haematuria, unspecified (MF50.4Z)】
Synonyms: Haematuria | blood in urine | urinary blood | haematuria NOS | urinary tract haemorrhage NOS
Hierarchy: Symptoms, signs or clinical findings involving the urinary system → Abnormal micturition (MF50) → Haematuria (MF50.4) → Haematuria, unspecified
【3. Finding of cocaine in blood (MA12.1)】
Synonyms: cocaine in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of cocaine in blood
【4. Finding of steroid agent in blood (MA12.4)】
Synonyms: steroid in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of steroid agent in blood
【5. Finding of hallucinogen in blood (MA12.2)】
Synonyms: hallucinogen in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of hallucinogen in blood
|
3C0Z
|
Diseases of the blood or blood-forming organs, unspecified
|
A 49-year-old patient was admitted to the hospital due to “the discovery of uterine cavity space-occupying and pelvic mass for over 20 days” on August 30, 2020. The patient had regular menstruation at normal times, with the chief complaint of increased vaginal discharge (transparent in color with no odor) with the same volume as that of menstruation over the last 10 years. The patient underwent “hysteroscopic resection of the uterine cavity lesion” 3 years ago due to a “uterine cavity space-occupying mass”, with “adenomyoma” indicated by postoperative pathology, and the patient showed improvement in vaginal discharge symptoms postoperatively. In the last year, the patient experienced symptoms of increased vaginal discharge again with no obvious inducement. The discharge volume was approximately twice that of the normal menstrual volume, and there was occasionally blood in leucorrhea, without odor, accompanied by abdominal distension; no abdominal pain, frequent urination, urgent urination, dysuria, fever, nausea, vomiting, or other discomfort were reported. The patient visited the local hospital many times, received a diagnosis of vaginitis and was provided with drug therapy with poor effects. On August 10, 2020, the patient underwent color Doppler ultrasound in the local hospital with the discovery of an “abnormal echo in the uterine cavity (3.9 cm × 3.4 cm × 1.6 cm) and cystic mass in bilateral adnexa areas”. The local hospital recommended a further examination, and the patient refused due to personal reasons. For further diagnosis and treatment, the patient visited our hospital on August 30, 2020, and was admitted to our hospital due to an “abnormal echo in the uterine cavity, pelvic infection, adnexal tumor, and hydrosalpinx?”. The personal history and family history of the patient were unremarkable. Gynecological examination results were as follows: No abnormality in the development of the vulva; no obstruction in the vagina; an increase in vaginal discharge, which was odorless; cervical congestion; bleeding on touch; tough texture; large size of the uterine body, with normal activity and tenderness pain; and tenderness pain in the bilateral appendages. On July 15, 2020, the detection result of HPV-DNA typing in the cervix was negative; however, there were abnormalities in the ThinPrep Cytology Test. Re-examination by transvaginal ultrasound on August 30, 2020, suggested a mixed-echo mass in the uterine cavity, a bilateral adnexal cystic mass, a left adnexal cystic mass, an isoechoic mass in the right adnexal area (with the possibility of teratoma to be confirmed), multiple cystic nodules of the cervix, and pelvic effusion. Tumor marker detection showed no abnormalities in human epididymis protein 4, carbohydrate antigen 125, the premenopausal ROMA index, carcinoembryonic antigen, alpha fetoprotein, or specific β human chorionic gonadotropin. Furthermore, carbohydrate antigen 199 was measured to be 170.43 U/mL (reference range: 0–37 U/mL). Laparoscopic hysteroscopy was planned. However, considering the presence of a space-occupying lesion in the uterine cavity, a pelvic magnetic resonance imaging (MRI) plain scan and diffusion-weighted imaging were performed on August 31, 2020 . As indicated by the results, the posterior wall of the cervix was thickened, with a mass-like shadow (approximately 2.8 cm × 1.1 cm × 1.4 cm in size) of slightly low signal intensity on T1WI and slightly high signal intensity on T2WI, with unclear boundaries and an uneven distribution of signals; in addition, diffusion was limited slightly in DWI and showed a high signal (no further description of the remaining imaging results). The imaging diagnosis was as follows: abnormal signal of the cervical posterior wall, with the consideration of a neoplastic lesion and the possibility of cervical cancer; abnormal signal nodule in the right adnexal area, with the consideration of the possibility of tumor or a chocolate cyst, which remain to be differentiated; an intrauterine mass-like abnormal signal shadow with a close relationship with the cervix locally, with the consideration of the possibility of cervical lesions (polyps or endometrial hyperplasia) protruding into the uterine cavity; bilateral hydrosalpinx and dilation and complicated hematocele in the right fallopian tube; cervical myometrium signal disorder, multiple cysts, and a bleeding focus, with the possibility of endometriosis to be determined; and a small amount of pelvic effusion. Consequently, the patient underwent cervical colposcopy + cervical biopsy + uterine curettage. On September 5, 2020, pathological examination findings based on cervical biopsy + uterine curettage suggested the following: (uterine cavity) proliferative endometrium accompanied by a few papillary structures; (cervical canal) the detection of abnormal glands with a papillary structure in the bleeding tissues, immunohistochemistry: P53 (+, 20%–30%), Ki-67 (+, 30%–40% partially), Vimentin (–), ER (–), PR (–), and P16 (–) in the heterotypic gland; and (at 3, 6, 9, and 12 o’clock in the cervix) heterotypic glands in the tissues tested, and adenocarcinoma in situ at least observed by immunohistochemistry, immunohistochemistry: P16 (–), and Ki-67 (+, 60%–70% partially). The pathological examination results showed cervical cancer, which was at stage IB2 on the basis of pre-operative clinical diagnosis. After full informed consent was obtained, “robot-assisted laparoscopic radical hysterectomy + bilateral salpingectomy-ovariectomy + pelvic lymph node dissection + pelvic adhesiolysis” were performed on September 6, 2020. The intra-operative rapid pathological examination showed that the cervical canal was approximately 3 cm in length, with a diameter of the opening of 2.5 cm and severe erosion. At the location of the incision, an invasive tumor was found, which was approximately 3 × 2 × 1.5 cm in size, showing a grayish white cutting section, hard texture, and unclear boundary. The following were suggested by the results: (left side) cystic dilatation with atypical epithelial hyperplasia in the fallopian tube; (right ovary) atypical hyperplastic glands in the stroma with the specific type to be determined by paraffin-embedded section examination; and (cervix) adenocarcinoma. On September 13, 2020, postoperative routine pathological examination findings suggested the following: (cervix) invasive gastric adenocarcinoma with a borderline serous tumor, a mass size of 3 × 2 × 1.5 cm, the mass invaded over 1/2 of the cervical wall, and multiple-focal mucous metaplasia; borderline serous tumor of bilateral fallopian tubes; borderline clear cell tumor of the right ovary, with the size of 2.5 × 2 cm; no cancer at the cutting edge of the vaginal wall; proliferative endometrium; and (left pelvic cavity, right pelvic cavity, adjacent area of left common iliac artery, and adjacent area of right common iliac artery) no cancer in the lymph nodes, which were (0/8; 0/8; 0/1; 0/9), respectively. The immunohistochemistry results were as follows: gastric adenocarcinoma, ER, PR, P16, and Vim (–), with P53 (+, positive cell count of 50%–60%); borderline serous tumor, ER, PR, P16, Vim, and WT-1 (–), P53 (+, positive cell count of 3%–5%); and borderline clear cell tumor, ER, PR, P16 (–), P504S (+), and NapsinA (+ partially). One month after the operation, the patient came and received local radiotherapy (DT = 48.6 Gy/27f) in the Department of Oncology of our hospital. During radiotherapy, the patient was given concurrent chemotherapy by using cisplatin (40 mg). After radiotherapy and chemotherapy, the patient was in good condition. No obvious abnormality was found in the test results, and no recurrence was observed. The patient is currently under further follow-up.
| 3.84375
| 0.984375
|
sec[1]/p[0]
|
en
| 0.999997
|
34941092
|
https://doi.org/10.1097/MD.0000000000028239
|
[
"uterine",
"cervical",
"cavity",
"mass",
"pelvic",
"tumor",
"results",
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Inflammatory disorders of the uterus, except cervix, unspecified (GA01.Z)】
Synonyms: Inflammatory disorders of the uterus, except cervix | inflammatory disease of the uterus | uterine inflammatory disease | uterus inflammation | syncytioma
Hierarchy: Diseases of the female genital system → Inflammatory disorders of the female genital tract → Inflammatory disorders of the uterus, except cervix (GA01) → Inflammatory disorders of the uterus, except cervix, unspecified
【2. Other specified acquired abnormalities of uterus, except cervix (GA16.Y)】
Synonyms: Polyp of corpus uteri | intrauterine polyp | polyp of body of uterus | polyp of uterus | uterine polyp
Hierarchy: Diseases of the female genital system → Noninflammatory disorders of female genital tract → Acquired abnormalities of uterus, except cervix (GA16) → Other specified acquired abnormalities of uterus, except cervix
【3. Injury of uterus (NB92.6)】
Synonyms: uterine injury | intrauterine injury NOS | Injury of uterus without open wound into cavity | Injury of uterus with open wound into cavity | Laceration of uterus
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injuries to the abdomen, lower back, lumbar spine or pelvis → Injury of urinary or pelvic organs (NB92) → Injury of uterus
【4. Other specified fistulae involving female genital tract (GC04.1Y)】
Synonyms: Other female intestinal-genital tract fistulae | Intestinouterine fistula | enterouterine fistula | Cervicosigmoidal fistula | Rectouterine fistula
Hierarchy: Certain specified diseases of urinary system → Fistula of the genitourinary tract (GC04) → Fistulae involving female genital tract (GC04.1) → Other specified fistulae involving female genital tract
|
GA01.Z
|
Inflammatory disorders of the uterus, except cervix, unspecified
|
To the best of our knowledge, this is the first report in which a long-term follow-up was performed in patients who underwent toe pulp reconstruction surgery in scarred and atrophic outcomes via a neurocutaneous composite GTP flap. Only one other author has shown that chronic injuries can be treated with good results by using a neurocutaneous composite free flap from the foot. In this report, however, the second toe pulp was used as the donor site . Nowadays, performing conservative treatment of digital pulp injuries with occlusive dressings or through the use of local flaps results in excellent healing in the majority of cases, making the digital pulp reconstruction procedure with more sophisticated methods necessary only in a small percentage of patients. Therefore, in view of the vast arsenal of digital pulp reconstruction techniques, foot-free flaps are a relatively rare indication and only in cases where the loss of substance is greater than 3 cm 2 with flexor tendon and bone exposure . In other cases, occlusive dressing with bone shortening or a dermal matrix makes for optimal results . While this debate is open regarding acute injuries, nothing is reported in the literature about the treatment of retracting scars or pulp atrophy. For three patients the initial extent of the trauma was not clear, and therefore it was unclear which strategy was adopted for their treatment. It can be considered that an immediate local flap or appropriate wound management with early motion could have been beneficial for proper healing. The body’s attempt to repair the injury site resulted in a retracting scar that limited joint extension. When managing the loss of substance, it resulted in a bigger loss of tissue than would have been expected. So, after debridement and full extension of the PIP or IP joint, a loss of more than 3 cm 2 resulted, requiring a free tissue transfer to reconstruct such a defect. In patient 3, the GTP flap solved two tasks. Firstly, the chronic ulcer could have been secondary to daily usage of the forearm skin for pinching, something that it is not made for. With the free flap, a specific skin designed for pressure bearing has been transferred into the fingertip position. Secondly a retracting scar limiting thumb abduction was released and substituted with the supple tissue of the flap tail. It is clear that, when dealing with a dystrophic fingertip, a flexion contracture of adjacent joints should be managed contemporarily, so that the physician has to figure out a bigger flap than one would expect, looking only at the skin. Patient 4 is midway between an acute and a chronic situation. In fact, a sub-amputation led to progressive necrosis of the pulp, with tendon and bone exposure without any chance of using a local flap due to gap extension. Such a situation could have resulted in a dystrophic scar, as for previous patients. The use of a GTP flap in a subacute setting prevented this. In one of our cases an acute arterial thrombosis occurred, and revision surgery was required to save the flap. This meant a longer hospital stay for the patient, but the outcome was not affected by this event. No venous graft was required for arterial anastomosis, showing that even in chronic cases digital arteries are available to efficiently revascularize the flap, even with a short pedicle, and it is not necessary to reach vessels in the palm or anatomic snuffbox for the thumb. Moreover, the complete survival rate of flaps in our series confirmed what is said in the literature . Other than providing skin, a pulp flap is able to bring a sensate skin. The patients in our series presented values of the 2-PD test and the SM-MF in line with the results obtained by other authors who performed the acute reconstruction procedure. Yuan et al. , in their series of 24 cases, reported flap necrosis as a complication in four patients; the mean S-2PD test result was 5 mm with a mean follow-up of 20 months. In 2014, Gu et al. , in their series of pulp reconstructions using a free pulp flap of the big or second toe, reported a mean value of 4.8 mm at re-evaluation of patients at 20 months in the S-2PD test, and 4.12 in the SW-MF test. Subsequently, another Chinese author published his series of 32 finger pulp reconstructions with a mean value of 6.17 mm in the S-2PD test and 3.675 in the SW-MF test at the mean follow-up of 22.8 months. Balan reported a series of five acutely treated cases, in which he obtained a median value of 6.5 mm for the S-2PD and 4.31 for the SW-MF test at 9-month follow-up. Wang et al. , during a 34.8-month follow-up in a series of 34 patients, found a median value of 7.13 mm in the S-2PD test and 3.48–4.71 in the SW-MF test. Recently, an Italian group in their series of 37 cases with a mean follow-up of one year obtained a mean S-2PD value of 9.41 mm; they concluded that, especially for more distal lesions, a technique without nerve suturing can be used. In our series, the recovery was comparable to previous reports, even when a complication occurred. Even in chronic cases, sensory recovery is expected to be good, providing an S3+ or S4 sensation. One should consider that several factors affecting sensation recovery such as age, follow-up period, and sensory re-education are known in the literature; in particular, a properly executed sensory rehabilitation program is essential for sensation restoration . What nerve repair could be most useful for is the treatment and prevention of cold intolerance. In fact, it seems that nerve repair associated with vascular repair is able to reduce this symptom, sustaining nerve repair . From this case series, it emerged that patients present only lesser symptoms related to cold intolerance, as presented in other studies . Pulp reconstruction allowed patients to reach a normal hand function without any impediment to their work and daily activities. One of the main aspects of this reconstruction was the ability to solve a flexion contracture, greatly increasing the ROM of the affected finger and hand. Our results are limited due to the low number of cases in the study. But this fact could be explained by the relatively rare indication that this procedure has. In most cases, the appropriate debridement and treatment of fingertip injuries may prevent inappropriate healing. Furthermore, the dystrophic healing of the fingertip of an ulnar finger rarely required such a complex reconstruction, since it is better tolerated than those involving thumb and radial fingers. What can be deduced from our case series is that immediate and appropriate treatment of fingertip injuries should be planned to prevent complications and reduce the need for a free flap. Four of the patients that have been treated presented a dystrophic pulp as a consequence of a second intention healing in an injury that probably would have been successfully treated by an immediate local or free flap reconstruction. When dystrophic healing occurs, it is associated with a retracting scar that induces a flexion contracture in the adjacent joints. When debridement is performed and a normal extension of the IP is obtained, more soft tissue is to be provided to maintain a complete ROM. This excludes, in most of cases, at least in our series, the use of local flaps. Their dimensions could be limited compared to soft tissue loss and, not secondarily, most of them are non-innervated. Furthermore, the use of a distant flap has the advantage of not injuring the recipient digit that received multitissutal damage already. The main drawback consists of the risk of microsurgical failure, but this is low in specialized microsurgical centers. Moreover, local flaps should not be demonized and kept as plan B in the event of free flap failure.
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sec[3]/p[0]
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en
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39997321
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https://doi.org/10.3390/jpm15020044
|
[
"flap",
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[
{
"code": "EM0Y",
"title": "Other specified diseases of the skin"
},
{
"code": "EL51",
"title": "Cutaneous flap necrosis"
},
{
"code": "EL52",
"title": "Myocutaneous flap necrosis"
},
{
"code": "DA09.4",
"title": "Pulp degeneration"
},
{
"code": "DA09.0",
"title": "Pulpitis"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Other specified diseases of the skin (EM0Y)】
Synonyms: 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 | Postprocedural cutaneous complications of surgical procedures
Hierarchy: Diseases of the skin (14) → Other specified diseases of the skin
【2. Cutaneous flap necrosis (EL51)】
Definition: Necrosis of surgical skin flap
Synonyms: Cutaneous flap necrosis, partial | Cutaneous flap necrosis, total
Hierarchy: Diseases of the skin (14) → Postprocedural disorders of the skin → Cutaneous flap necrosis
【3. Myocutaneous flap necrosis (EL52)】
Definition: Necrosis of a surgical flap containing both skin and muscle
Synonyms: Myocutaneous flap necrosis, partial | Myocutaneous flap necrosis, total
Hierarchy: Diseases of the skin (14) → Postprocedural disorders of the skin → Myocutaneous flap necrosis
【4. Pulp degeneration (DA09.4)】
Synonyms: degeneration of dental pulp | Denticles | Dental pulp calcification | pulp calcification | Dental pulp stone
Hierarchy: Diseases of the digestive system (13) → Diseases or disorders of orofacial complex → Diseases of pulp or periapical tissues (DA09) → Pulp degeneration
【5. Pulpitis (DA09.0)】
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.
Synonyms: Pulpitis NOS | Suppurative pulpitis | Acute pulpitis | Chronic pulpitis | Chronic hyperplastic pulpitis
Hierarchy: Diseases of the digestive system (13) → Diseases or disorders of orofacial complex → Diseases of pulp or periapical tissues (DA09) → Pulpitis
|
EM0Y
|
Other specified diseases of the skin
|
The vignette (Table 1 ) was developed to be representative of a typical presentation of dementia in community-based primary care settings (i.e. expressing symptoms of Alzheimer’s disease, vascular dementia, and mixed Alzheimer’s and vascular dementia). It was developed to contribute to building a regionalized dementia network in eastern Ontario, Canada, with a focus on phrases of transition for improved patient-centered care . It was also developed to provide a framework for medical education regarding transition points for patients with dementia. The vignette was developed by an expert advisory committee consisting of a family caregiver, clinicians, and representatives from key organizations providing services for people with dementia and their family caregivers. The vignette illustrated 13 time points from mild cognitive impairment (i.e. ≤ time point 4) to a moderate dementia stage (i.e. time points 5 to 8) and later/severe dementia stages (i.e. ≥ time point 8) of the disease trajectory. Table 1 The dementia case vignette Time Description Medical background Mrs. G.C. is a 76 year old married woman with Grade 12 education. She had a mother who developed Alzheimer’s Disease onset age 84. Her medical history including hypertension, hyperlipidemia and osteoporosis. Her medications are Hydrochlorothiazide, Adalat XL, Lipitor, Calcium, Vitamin D, and Fosamax. 0 months (T1) Warning signs In the last six months her husband noted that she seemed to be a little bit forgetful, having some problems with names, “not quite as sharp” as one year previously, having a little more difficulty planning the bigger family social events and being a little less interested in leisure activities. She was still driving, shopping, cooking, independent in all her IADL’s although she occasionally needed a reminder to take her medication. 6 months (T2) Screening results and early recognition While at the local Pharmacy her husband noticed that the Pharmacist was offering a 2 min Dementia Screening Test so he and Mrs. G.C. did the test. He was fine but his wife had difficulties in animal naming (9 in one minute) and clock drawing. He realized that this was a significant issue which needed medical attention. 7 months (T3) Mild cognitive impairment Her husband was now worried that this was more than normal ageing and did in fact arrange an appointment with the family physician. The family physician tested first with the MMSE on which her score was 25/30. Laboratory testing was negative. The conceptualization was that Mrs. G.C. was not as “sharp with her memory” as she was six months previously but no other areas of cognitive function or functional abilities were affected. The Family Physician explained the concepts of mild cognitive impairment and gave advice about being physically, mentally and socially active. He explained that it could progress to more problems with memory and said that he would see her in one year or earlier if there was greater concern about memory or function. The patient’s hypertension and hyperlipidemia were well controlled and enteric coated aspirin was started at 81 mg daily. 1 year and 7 months (T4) Annual follow-up One year later there didn’t seem to be any progression of symptoms or functional loss. Her MMSE was now 24/30. 2 years and 7 months (T5) Diagnosis One year later the husband was more concerned because she got lost once while out driving the car back from her sister’s home 30 miles away, and because he noticed that she was having more trouble with cooking more complicated meals, being more forgetful about medications and occasionally having angry outbursts. He was a little bit worried about leaving her alone for a weekend to go to his big curling bonspiels in the winter. Her MMSE was now 20/30. Her family physician did further evaluation which showed poor visual spatial function (clock drawing) and poor performance of Trails A and Trails B. A CT scan was done which showed periventricular white matter changes and two old lacunar infarcts. The family physician made the diagnosis of mild mixed Alzheimer’s and vascular dementia and she was started on cholinesterase inhibitor treatment. Based on her overall assessment he advised her that she needed to stop driving. 2 years and 10 months (T6) 3 month follow-up Three months later she was seen and she had improved. She was more active, more attune to social situations and conversation and more like her old self. Her MMSE had improved to 22. At this stage she only needed a little bit of cueing for finances and shopping. She was referred to a Day Centre at a Senior’s Centre for increased stimulation and socialization and to provide her husband with some respite. 3 years and 7 months (T7) Increased support for IADL Nine months later she was about the same, though a little more forgetful. Her husband had hired a maid to do some of the simple cleaning services through the local community for-profit support agency and he also needed to become more involved in cooking simple meals, shopping and finances. Her MMSE was now 20/30. 4 years and 7 months (T8) IADL, ADL, and behavioral and psychological symptoms One year later she was more forgetful, was unable to cook on the stove but still could use the microwave and do simple cold meals. She needed help with laundry and help with shopping. She was independent in her personal ADL’s and only occasionally needed some cueing with respect to clothes selection. She did need help with respect to bathing and homecare became involved. Her MMSE was 16/30. She was more emotionally labile, apathetic and became very anxious if left alone. She was also having episodic bouts of agitation and occasionally aggressive behavior. Memantine (Ebixa) was started and there was some improvement in terms of cognition, (MMSE 18/30), ADL, agitation and anxiety. 5 years and 7 months (T9) Increased support in ADL and caregiver stress One year later her MMSE had declined to 15/30. Her husband was doing all the instrumental activities of daily living. She needed help with bathing, hygiene and toileting and there was considerable caregiver stress in that she could only be left alone for approximately an hour. Homecare was providing more services in terms of bathing and personal care. She was occasionally incontinent. Her gait was unsteady and her fall risk was increased, and thus she needed to use a walker. A day program helped with respect to daytime respite and there was an increase in paid services by the husband to lessen caregiver stress. 6 years and 7 months (T10) Stroke and hospitalization One year later she had a small stroke leaving her with some weakness on the right side. Her incontinence was worse. She was admitted to the hospital where she became much more confused. 6 years and 8 months (T11) Transition from home to long-term care Following a conference attended by phone by their daughter in Florida who felt that her parents should be together, she was discharged home. She developed a tendency towards wandering about the house and once wandered outside. Her husband was no longer able to look after her. It was decided that she would re-locate to residential care. This move was very positive for the husband. 7 years and 8 months (T12) Increase in behavioral and psychological symptoms One year later her communication skills were markedly affected. Her mobility was decreased. The nursing staff noted that she began having increasing hallucinations and angry outbursts. 8 years and 8 months (T13) Death One year later after receiving appropriate end of life care she was found deceased on morning nursing rounds. ADL activities of daily living, IADL instrumental activities of daily living, MMSE mini-mental state examination, T time point. Adopted from
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sec[1]/sec[1]/p[0]
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| 0.999994
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27184962
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https://doi.org/10.1186/s12913-016-1435-1
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[
"dementia",
"later",
"husband",
"mmse",
"years",
"family",
"care",
"time"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Dementia, unknown or unspecified cause (6D8Z)】
Synonyms: Dementia NOS | Dementia, unspecified | senile melancholia | senile depression | senile paranoid reaction
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Neurocognitive disorders → Dementia → Dementia, unknown or unspecified cause
【2. Dementia due to cerebrovascular disease (6D81)】
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 consid...
Synonyms: arteriosclerotic dementia | Strategic-infarct dementia | Post stroke dementia | vascular cognitive impairment | vascular dementia
Excludes: Alzheimer disease dementia, mixed type, with cerebrovascular disease
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Neurocognitive disorders → Dementia → Dementia due to cerebrovascular disease
【3. Dementia, other specified cause (6D8Y)】
Synonyms: Degenerative dementia | primary degenerative dementia NOS
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Neurocognitive disorders → Dementia → Dementia, other specified cause
【4. Dementia due to use of volatile inhalants (6D84.2)】
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 sy...
Synonyms: inhalant dementia | volatile solvents dementia
Hierarchy: Neurocognitive disorders → Dementia → Dementia due to psychoactive substances including medications (6D84) → Dementia due to use of volatile inhalants
【5. Dementia due to diseases classified elsewhere (6D85)】
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Neurocognitive disorders → Dementia → Dementia due to diseases classified elsewhere
|
6D8Z
|
Dementia, unknown or unspecified cause
|
The patient had a history of admission to the Department of Thoracic Surgery on April 9th, 2022, due to dyspnea and pleuritic chest pain. On April 17th, 2022, the patient had a chest CT scan, revealing pleural effusion plus pyopneumothorax and consolidation, causing the collapse of the right lower lobe (RLL) in the right hemithorax . The patient was diagnosed with tuberculosis (Tb), which had caused right pleural empyema. The standard four‐drug regimen was followed for treatment, and the patient was discharged. After being discharged, the patient only reported experiencing minor dyspnea, which did not affect her daily functioning. As a result, she was started on anti‐TB medications and did not require further workups.On admission, she declared a 2‐day constipation with abdominal pain but denied any associated hematemesis, fevers, chills, or urinary symptoms. During the first episode 5 years ago, she was evaluated at an outlying health center and diagnosed with peptic ulcer disease. She was managed with omeprazole intermittently. She had no allergies and denied alcohol intake or tobacco use. Her HIV serostatus was negative approximately 1 year prior to presentation. On examination, her general appearance was toxic; she was afebrile, with a heart rate of 120 beats/min, blood pressure of 135/78 mmHg, and respiratory rate of 22/min. The lungs were clear with no rales, rhonchi, wheezes, or rubs. Upon abdominal examination, mild distension was observed with guarding and marked rebound tenderness in the epigastrium. Bowel sounds were absent, and no palpable masses were detected. She was immediately transferred to the general surgical department. On August 10th, 2022, the patient had an abdominal and pelvic CT scan performed, showing pneumoperitoneum, ascites, diffuse mesenteric stranding, and engorged mesenteric vessels. Thereupon, the patient was diagnosed with peritonitis as a result of a perforated peptic ulcer. Also, pleural effusion plus a right pyopneumothorax causing the collapse of the RLL, in the right hemithorax, were reported on the observable lung portions of abdominal and pelvic CT consistent with the prior CT scan . Therefore, a chest tube was inserted along with treatment with broad‐spectrum antibiotics. On August 10th, 2022, the patient was taken to the operating room (OR); after undergoing anesthesia, her abdomen was prepped and draped in sterile fashion; a midline incision was made above and under the umbilicus; upon entrance, 1 L of pus and bile secretions was discharged. On examination, the liver, small intestine, and colons appeared normal. A 2*2 cm perforated peptic ulcer was identified on the anterior wall of the stomach body near the lesser curvature. A biopsy from the edge of the ulcer was obtained, which later indicated no results consistent with neoplasia. The perforated ulcer was repaired, and an omental patch was placed. Finally, the abdomen was irrigated with saline. She was immediately transferred to the Internal Medicine Department's intensive care unit (ICU). On August 13th, 2022, the patient underwent a second look surgery, which showed no postoperational complications. During the ICU stay, clinical workups began to diagnose pulmonary issues. A thoracocentesis sample was sent for lab on August 15th, 2022, which showed hemorrhagic fluid with inflammatory clusters but no atypical cells on microscopy. The patient's condition was not stable throughout the admission; on August 23rd, 2022, despite receiving antibiotics, she was intubated. On August 26th, 2022, a chest tube was inserted to aspirate symptomatic pleural effusion. On August 28th, 2022, a bronchoscopy with bronchoalveolar lavage (BAL) was performed. No abnormal findings were detected. A biopsy and bronchial secretion from the BAL were obtained and sent to the lab. Later, the biopsy indicated chondroid mesenchymal hamartoma as the primary diagnosis. Bronchial secretions were negative for acid‐fast and other microbial pathogens. Also, cytology showed no abnormal findings. A CT scan for the abdomen and pelvis was conducted on September 1st, 2022, which showed no postoperation complications. Also, the right perihilar mass‐like lesion causing the collapse of the RLL, pleural thickening with pleural effusion, and nodular opacities were reported, which were inconsistent with malignancy. On account of mild fever, tachycardia, and increased airway mucus hypersecretion, a bronchoscopy with BAL was performed on September 18th, 2022, showing the same results as the previous bronchoscopy with BAL. Bronchial cultures from BAL were negative for acid‐fast and fungal pathogens. Sputum cultures were positive for Enterococcus the first time and Pseudomonas the second time. The patient continued to be on broad‐spectrum antibiotics. Blood culture was negative. Often, chest tube secretions were bloody. Further control CTs consistently showed pleural effusion, collapse consolidation, hypodense foci with hyperdensity, and increased pleural thickness. On September 24th, 2022, a transbronchial lung biopsy (TBLB) was performed in the right superior lobar bronchus during flexible bronchoscopy with BAL, indicating hemangioendothelioma plus chronic inflammation along with negative polymerase chain reactions (PCRs) for TB and nontuberculous mycobacterium (NTM) from bronchial secretion collected from BAL. Computed angiography (CTA) ruled out pulmonary embolism. Eventually, the case was presented to the joint meeting of the Pulmonary Department and Department of Thorax Surgery, and a consensus decision was made to perform thoracotomy and invasive pulmonary surgery; consequently, on October 3rd, 2022, the patient underwent surgery. The surgeon reported that due to intrathoracic adhesions causing an unexpandable lung, its resection could not be operated, although a biopsy was obtained and sent for a frozen section. Necrotic tissues were removed as much as possible, together with decortication . According to the frozen section biopsy results, there was a malignant tumor suspected of mesothelioma. On further examination, Clusters of Differentiation 31 (CD31), CD34, vimentin, and epithelial membrane antigen (EMA) were all positive on immunohistochemistry (IHC) along with negative cytokeratin . Microscopy indicated that pleural tissue involved by neoplastic proliferation, atypical epitheloid cells with marked nuclear atypia, mitosis, and vacuolated cytoplasm and focal vascular lumen in a desmoplastic stroma was seen . Taking everything into account, the pathologist reported pulmonary angiosarcoma. On October 15th, 2022, whole body scan and single‐photon emission CT (SPECT/CT) were carried out, demonstrating increased tracer uptake in the 4th–12th right ribs on whole‐body images suggesting local invasion of the tumor and focal involvement with the collapse of the right lung on CT component of the study suggesting PPA. On October 24th, 2022, the patient was started on pazopanib (two 200 mg tablets) on the first day and IV paclitaxel (four 120 mg/m 2 injectable solutions diluted in 500 ccs of normal saline) on the first, 8th, and 15th day of treatment. Despite being under intensive care, the patient's clinical status continued to deteriorate. The exacerbation of dyspnea necessitated the initiation of bilevel positive airway pressure (BiPAP) to manage the increased respiratory effort. Given the patient's critical state and following consultation with the oncologist, it was concluded that an escalation in chemotherapy dosage was untenable. Consequently, the prognosis was deemed terminal. Eventually, the patient was deceased on November 11th, 2022, due to multiple complications from different organs, recurrent infections, several admissions, and delay of treatment.
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sec[1]/p[1]
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39107956
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|
[
"pleural",
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"surgery",
"chest",
"scan",
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] |
[
{
"code": "CB2Z",
"title": "Pleural, diaphragm or mediastinal disorders, unspecified"
},
{
"code": "LA76",
"title": "Structural developmental anomalies of pleura"
},
{
"code": "MD31",
"title": "Pleurisy"
},
{
"code": "NB32.60",
"title": "Laceration of pleura"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Pleural, diaphragm or mediastinal disorders, unspecified (CB2Z)】
Hierarchy: Diseases of the respiratory system (12) → Pleural, diaphragm or mediastinal disorders → Pleural, diaphragm or mediastinal disorders, unspecified
【2. Structural developmental anomalies of pleura (LA76)】
Definition: Anomalies of the lining of the lung (visceral pleura) and thoracic cavity (parietal pleura)
Synonyms: Malformations of pleura | anomaly of pleura | abnormal pleura | pleural anomaly | Developmental anomaly of pleura, unilateral
Hierarchy: Developmental anomalies (20) → Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the respiratory system → Structural developmental anomalies of pleura
【3. Pleurisy (MD31)】
Definition: Pleurisy or Pleuritis is the medical term for inflammation of the pleura. The most common cause of pleuritis is infection, but it can also be caused by lupus, rheumatoid arthritis, and certain medicines. Pleurisy or pleuritis usually accumulates exudative pleural effusions.
Synonyms: pleuritis | pleurisy NOS | double pleurisy | pleurisy without effusion | dry pleurisy
Excludes: pleurisy with effusion
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings of the respiratory system → Symptoms or signs involving the respiratory system → Pleurisy
【4. Laceration of pleura (NB32.60)】
Hierarchy: Injuries to the thorax → Injury of other or unspecified intrathoracic organs (NB32) → Injury of pleura (NB32.6) → Laceration of pleura
|
CB2Z
|
Pleural, diaphragm or mediastinal disorders, unspecified
|
A 76-year-old female was brought to the Emergency Department (ED) after being found unconscious at home with a recorded room air saturation of 86%. She had a past medical history significant for arterial hypertension, hypercholesterolemia, transient ischemic attack, and non-valvular atrial fibrillation. Home medications included apixaban, digoxin, atorvastatin, nebivolol, perindopril, moxonidine, and esomeprazole. There was no previous smoking, alcohol, or illicit drug use. According to her relatives, she was last seen well 3 h earlier. She had presented no fever, cough, dyspnea, or preceding illness. At ED presentation, her blood pressure was 83/47 mmHg; heart rate, 90 beats per minute; respiratory rate, 12 breaths per minute; oxygen saturation, 100% on high flow oxygen via a non-rebreather mask; and temperature, 35°C. An arterial blood gas indicated a type 1 respiratory failure (Table 1 ). On examination, she was completely unresponsive (Glasgow Coma Scale score of 3) with no tonic-clonic activity. Deep tendon reflexes were weak, and Babinski’s sign was bilaterally present. Chest auscultation revealed bibasilar coarse crackles. The remainder of her physical examination was non-contributory. Brain computed tomography (CT) and subsequent CT angiography were normal. CT of the chest and abdomen showed ground glass opacities, predominantly peripheral and involving all five lung lobes, compatible with COVID-19 pneumonia . Baseline blood analysis was notable for lymphopenia, rhabdomyolysis, crush-related acute kidney injury, and elevated prolactin level (Table 1 ). A nasopharyngeal swab real-time reverse-transcriptase-polymerase-chain-reaction (rRT-PCR) test was positive for SARS-CoV-2. The blood and urine cultures were negative. Cerebrospinal fluid examination was unremarkable, except for elevated lactate (Table 1 ). To secure her airway and assure adequate oxygenation, rapid sequence intubation using ketamine 1–2 mg/kg and succinylcholine 1.5 mg/kg was performed and mechanical ventilation started. A bolus of 10 mg midazolam was administered. Norepinephrine titrated up to 0.4 mcg/kg/min was added for refractory hypotension after 30 mL/kg of intravenous crystalloid fluid resuscitation, targeting a mean arterial pressure of 65 mmHg. She received intravenous ceftriaxone 2g and dexamethasone 6mg as recommended for severe COVID-19 pneumonia. As urgent electroencephalography (EEG) is not routinely available at our ED, EEG could not be obtained. With NCSE as presumed etiology of the patient’s coma, she was empirically treated with intravenous valproic acid 40 mg/kg and admitted to the intensive care unit (ICU). The EEG at day 1 showed non-epileptiform generalized background slowing, consistent with antiepileptic drug (AED) treatment. The next 2 days on the ICU, the patient’s clinical condition improved (Glasgow Coma Scale score of 15) due to expected AED responsiveness. At day 4, after being extubated, brain magnetic resonance imaging (MRI) was performed and confirmed NCSE as a cause of her unexplained coma at the ED, showing multifocal bilateral cortical hyperintensities on diffusion weighted images (DWIs) with decreased apparent diffusion coefficient (ADC) and increased signal in T2 and FLAIR with no arterial distribution . These signal changes, that do not respect vascular territories and are usually reversible over days, typically result from seizure activity, and represent regional vasogenic and cytotoxic edema, reflecting peri-ictal hemodynamic and metabolic changes, respectively. At day 5, she left the ICU and 7 weeks later, she was discharged home from our COVID-19 neurorehabilitation unit, in excellent condition and without neurological deficit. Repeat brain MRI showed complete regression of previously described imaging changes and absence of any type of lesion. Table 1 Patient data at the time of presentation to the Emergency Department Value (normal range) Venous blood puncture Hemoglobin, g/dL (12.0–16.0) 13.4 White cell count, ×10 9 /L (3.5–11) 8.07 Neutrophil count, × 10 9 /L (1.50–6.70) 6.44 Lymphocyte count, × 10 9 /L (1.20–3.50) 0.95 Platelet count, ×10 9 /L (150–440) 202 C-Reactive Protein, mg/L (<5.0) 56.3 D-Dimer, ng/mL (0–500) 7393 PT, seconds (9.9–11.8) 11.8 APTT, seconds (21.6–28.7) 24 Anti-Factor Xa activity, U anti-Xa/mL 1.06 Sodium, mmol/L (136–145) 130 Potassium, mmol/L (3.4–4.4) 3.8 Chloride, mmol/L (98–107) 89 Bicarbonate, mmol/L (23–29) 27 Urea, mg/dL (17–48) 60 Creatinine, mg/dL (0.50–0.90) 1.73 Serum glucose, mg/dL (70–100) 142 Alanine transaminase, U/L (<33) 19 Creatine kinase, U/L (26–192) 2376 Myoglobin, mcg/L (<58) 6063 Lactate, mmol/L (0.7–2.0) 1.6 Ammonia, mcg/dL (18.7–86.9) 21 Bioactive monomeric prolactin, mcg/L (3.5–18.0) 29.4 TSH, mU/L (0.27–4.20) 3.78 Peripheral venous blood culture set (2 independent sets) Negative Arterial blood puncture at ED admission (FiO 2 100%) pH (7.35–7.45) 7.47 pCO 2 , mm Hg (32–45) 35 pO 2 , mm Hg (75–104) 234 P/F ratio (>300) 234 Saturation O 2 , % (95–98) 100 Carboxyhemoglobin, % (<1.0) 0.9 Methemoglobin, % (<0.5) 0.7 Arterial blood puncture at ICU admission (FiO 2 40%) pH (7.35–7.45) 7.48 pCO 2 , mm Hg (32–45) 35 pO 2 , mm Hg (75–104) 102 P/F ratio (>300) 255 Saturation O 2 , % (95–98) 98 CSF examination CSF macroscopic examination Crystal clear CSF opening pressure, cmH 2 0 - CSF red cell, × 10 9 /L (0–5) 0.1 CSF white cell, × 10 9 /L (0–5) 2.8 CSF glucose, mg/dL (45–80) 75.8 CSF protein, g/L (0.15–0.45) 0.44 CSF oligoclonal bands - CSF lactate, mmol/L (1.11–2.44) 3.76 CSF PCR for SARS-CoV2 Negative CSF FilmArray Meningitis/Encephalitis Panel* Negative CSF PCR for Herpes Simplex 1 Negative CSF bacterial and fungal culture Negative CSF antinuclear antibodies Negative Antibodies against NMDAR, LGi1, CASPR2, GABA-B1/B2, DPPX, AMPA1/2 in serum, and CSF Negative Paraneoplastic anti-neuronal antibodies in serum and CSF Negative Urine analysis Leucocytes/μL (<10) 26 Erythrocytes/μL (<12) 26 Epithelial cells Absent Hyalin casts +++ Crystals Absent Bacteria + Fungi Absent Urine culture set Negative PT prothrombin time, APTT activated partial prothrombin time, TSH thyroid stimulating hormone, CSF cerebrospinal fluid, NMDAR N -methyl- d -aspartate receptor, LGi1 leucine-rich glioma inactivated 1, CASPR2 contactin-associated protein 2, GABA-B γ-Aminobutyric acid-B receptor, DPPX dipeptidyl aminopeptidase-like protein 6, AMPA1/2 GluR1 and GluR2 subunits of the AMPA receptor; Paraneoplastic antibodies included anti-Hu, anti-Yo, anti-Ri, and anti-amphiphysin * Escherichia coli K1, Haemophilus influenzae , Listeria monocytogenes , Neisseria meningitidis , Streptococcus pneumoniae , Streptococcus agalactiae , cytomegalovirus, enterovirus, herpes simplex virus 1 and 2, human herpesvirus 6, human parechovirus, varicella-zoster virus, and Cryptococcus neoformans / Cryptococcus gattii Fig. 1 Patient’s chest CT showing ground glass opacities, predominantly peripheral, involving the five lung lobes, compatible with severe COVID-19 pneumonia. A – C Axial view. D Sagittal view Fig. 2 Initial brain MRI: arrows show the focal cortical signal hyperintensity changes on diffusion weighted ( A ) and T2 weighted images ( B ) with reduced apparent diffusion coefficient on ADC map ( C ). Lesions are located in the cortical gray matter and lack arterial distribution. They are topographically compatible with transient postictal MRI changes indicating the presence of cytotoxic and vasogenic edema. Follow-up MRI revealed complete resolution of signal changes and no new other lesions. D FLAIR signal hyperintensity on initial brain MRI (D1) and normalized FLAIR signal intensity on follow-up MRI illustrating the reversibility of postictal MRI abnormalities (D2)
| 3.929688
| 0.975586
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sec[0]/sec[0]/p[0]
|
en
| 0.999995
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35079294
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https://doi.org/10.1186/s12245-022-00412-w
|
[
"blood",
"arterial",
"anti",
"signal",
"changes",
"mmol",
"brain",
"saturation"
] |
[
{
"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 CLASSIFICATION ═══
【1. Diseases of the blood or blood-forming organs, unspecified (3C0Z)】
Synonyms: Blood, lymph or spleen disease | blood condition NOS | blood disorder NOS | bone marrow disease NOS | haematologic disease NOS
Hierarchy: Diseases of the blood or blood-forming organs (03) → Diseases of the blood or blood-forming organs, unspecified
【2. Haematuria, unspecified (MF50.4Z)】
Synonyms: Haematuria | blood in urine | urinary blood | haematuria NOS | urinary tract haemorrhage NOS
Hierarchy: Symptoms, signs or clinical findings involving the urinary system → Abnormal micturition (MF50) → Haematuria (MF50.4) → Haematuria, unspecified
【3. Finding of cocaine in blood (MA12.1)】
Synonyms: cocaine in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of cocaine in blood
【4. Finding of steroid agent in blood (MA12.4)】
Synonyms: steroid in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of steroid agent in blood
【5. Finding of hallucinogen in blood (MA12.2)】
Synonyms: hallucinogen in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of hallucinogen in blood
|
3C0Z
|
Diseases of the blood or blood-forming organs, unspecified
|
A 69-year-old male patient previously diagnosed with angina pectoris was referred to the department of cardiac surgery due to the increasing back pain. The patient's medical history included: type II diabetes mellitus, therapeutically neglected arterial hypertension, chronic obstructive pulmonary disease, obesity, chronic coronary artery disease, as well as carotid artery atherosclerotic disease with 70% stenosis in the left carotid artery (LCA). He had no history of stroke, transient ischemic attacks, syncope or thoracic trauma before the presentation. Coronary angiography (CAG) showed left dominant coronary system with triple vessel coronary disease: 75% stenosis of the distal segment of left anterior descending coronary artery (LAD), left circumflex coronary artery (LCx) in the mid and distal segments had stenosis of 80% and 70%, respectively, and 70% stenosis of distal segment of the right coronary artery (RCA) . Transthoracic echocardiography (TTE) performed at admission showed: severe decrease in left ventricle ejection fraction (EF = 30%), akinesia of the basal segment of the inferior wall and in 1/3 posterior wall, dilated (47 mm) right ventricle with global systolic dysfunction, mild mitral regurgitation, mild tricuspid regurgitation, pulmonary artery systolic pressure (sPAP) of 45 mmHg. Doppler echographic assessment of femoral and carotid arteries confirmed the presence of non-stenotic echo-lucent plaques in both femoral arteries and 70–75% stenosis on the LCA. The ECG showed signs of atrial fibrillation and left ventricular hypertrophy. Three-dimensional analysis of the preoperative computed tomography (3D CT-scan) was used to establish the dimension of the pseudoaneurysm, the distances between the pseudoaneurysm and the aortic arch branches, as well as to identify any signs of intramural hematoma at the level of the proximal seal zone. Apart from the complex lesions of the coronary arteries, 3D CT-scan revealed an AAP, with the neck present just caudally from the left subclavian artery (LSA) on the lesser aortic arch curvature which had a maximum diameter of 36 × 27 mm . Diameters of the ascending aorta, aortic arch, and descending aorta were 38 mm, 36 mm, and 28 mm, respectively. The distance between the pseudoaneurysm and the LCA origin from the aortic arch was 10 mm. CT scan images were sent and processed by an outside service for preoperative planning and proper sizing of stent-graft. The endovascular aortic arch repair was not recommended due to the lack of adequate landing areas for stent-graph placement. The patient's condition was critical due to the high probability of death due to rupture of the pseudoaneurysm of the aortic arch. Calculated Euroscor II revealed 31.25% risk of in hospital mortality. The multidisciplinary meeting, including cardiac surgeons, interventional cardiologists, and anesthesiologists, discussed the possible treatment strategies for the patient considering the 3D CT-scan, coronarography, Doppler ultrasound echography, and echocardiography image. We offered the patient open surgical repair of the aortic arch pseudoaneurysm and PCI for the lesions of the RCA and LCx, as the lesion on the LAD was considered unsuitable for PCI. The considerable risks of surgery regarding perioperative mortality and its possible complications, as well as the expected natural history without surgery, were communicated to the patient. The patient agreed to undergo the treatment established by the multidisciplinary team. The combined procedure was performed in a hybrid operating room by a multidisciplinary team of cardiac surgeons and interventional cardiologists. Intra-operative standard monitoring of the patient included: invasive blood pressure monitoring via the left radial artery, continuous electrocardiography (ECG), pulse oximetry, diuresis, capnography, and central venous pressure monitoring via a triple lumen central venous catheter. Intraoperative specific monitoring for aortic arch procedures included: two body temperature measurement sensors, rectal and esophageal, an additional arterial blood pressure monitoring via the left femoral artery and near-infrared spectroscopy (NIRS) for continuous monitoring of the cerebral oxygenation. A median sternotomy was performed, and CBP was initiated using a bifurcated arterial line with double arterial cannulation of the ascending aorta and the left femoral artery and right atrial cannulation with a dual-stage venous cannula. The left side of the heart was vented through the right superior pulmonary vein. Although the pseudoaneurysm was adherent to the vessels of the arch, with meticulous dissection of the surrounding tissues, we were able to mobilize all the branches of the aortic arch and their proximal segment before initiating the repair of the pseudoaneurysm. The aorta was first cross-clamped immediately after the origin of the LCA and a second clamp at the level of the aortic isthmus allowing us to perform the repair of the pseudoaneurysm on beating heart . The pseudoaneurysm was opened and removed until the neck. The aortic rim of the neck was only slightly calcified and was considered to be healthy, and thus we considered safe to perform the repair with a 3/4 cm oval Dacron patch. The time of CPB was 54 min, and the nasopharyngeal temperature was maintained at 35 °C during the entire procedure. After weaning of CPB the PCI with DES was performed for the RCA, and LCx lesions . The lesion on the LAD was considered unsuitable for PCI due to the extremely calcified aspect of the lesion, which had a very high risk of rupture during the procedure. The patient's postoperative course was unremarkable. He was extubated at 12 h after the procedure, without signs of myocardial ischemia and transient or permanent neurologic injury. In the first postprocedural day chest drainage was of 450 mL with significant haemoglobin level decrease for which the patient received two red cell units and four units of frozen fresh plasma, without the need of surgical revision, a grade II complication according to the Clavien-Dindo classification. Considering this complication, antiplatelet therapy with Clopidogrel was initiated on the second postprocedural day and maintained for 12 months postprocedural. Chest drains were removed on the third postprocedural day and the patient was discharged from the hospital on the 14th postoperative day. At three months after this procedure, a follow-up CT scan with 3D reconstruction was performed and revealed an excellent result, without residual arch pseudoaneurysm . Fig. 1 Preoperative coronarography: (A) Coronary angiogram (CAG) shows 70% stenosis in the distal segment of the RCA, (B) Coronary angiogram (CAG) shows 75% stenosis of the the distal segment of the LAD, 80% and 75% stenosis, respectively in the mid and distal segments of the LCx, (CAG - coronary angiogram, LAD - left anterior descending coronary artery, RCA - right coronary artery, LCx - left circumflex coronary artery). Fig. 1 Fig. 2 Preoperative contrast enhanced CT scan: (A) CT scan showing the AAP at the level of the aortic arch, (B) 3D CT-scan showing the AAP, with the neck present just caudally from LSA on the lesser aortic arch curvature. (AAP - aortic arch pseudoaneurysm, LSA - left subclavian artery). Fig. 2 Fig. 3 Intraoperator view of aortic arch with Dacron patch repair. Fig. 3 Fig. 4 (A) RCA CAG poststenting, (B) LCx CAG poststenting. (CAG - coronary angiogram, RCA - right coronary artery, LCx - left circumflex coronary artery). Fig. 4 Fig. 5 Multislice 3D reconstruction CT scan of the aortic arch after three months showing no evidence of filling or enlargement of the AAP. (AAP- aortic arch pseudoaneurysm). Fig. 5
| 4.121094
| 0.967773
|
sec[1]/p[0]
|
en
| 0.999998
|
32829016
|
https://doi.org/10.1016/j.ijscr.2020.07.085
|
[
"coronary",
"artery",
"aortic",
"arch",
"pseudoaneurysm",
"scan",
"stenosis",
"segment"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Diseases of coronary artery, unspecified (BA8Z)】
Synonyms: coronary artery insufficiency | coronary artery heart disease | CAD - [coronary artery disease] | coronary artery disorder | coronary arterial disease
Hierarchy: Diseases of the circulatory system (11) → Diseases of coronary artery → Diseases of coronary artery, unspecified
【2. Acute ischaemic heart disease, unspecified (BA4Z)】
Synonyms: acute coronary syndrome | ACS - [acute coronary syndrome] | Silent myocardial ischaemia | asymptomatic ischemia | subclinical myocardial ischaemia
Hierarchy: Diseases of the circulatory system (11) → Ischaemic heart diseases → Acute ischaemic heart disease → Acute ischaemic heart disease, unspecified
【3. Acute myocardial infarction, unspecified (BA41.Z)】
Synonyms: Acute myocardial infarction | cardiac attack | heart attack | acute cardiac infarction | acute MI - [myocardial infarction]
Hierarchy: Ischaemic heart diseases → Acute ischaemic heart disease → Acute myocardial infarction (BA41) → Acute myocardial infarction, unspecified
【4. Chronic ischaemic heart disease, unspecified (BA5Z)】
Synonyms: Ischaemic heart disease (chronic) NOS | coronary ischaemia | coronary damage NOS | atheroma of heart | coronary heart disease
Hierarchy: Diseases of the circulatory system (11) → Ischaemic heart diseases → Chronic ischaemic heart disease → Chronic ischaemic heart disease, unspecified
【5. Congenital coronary arterial fistula (LA8C.2)】
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 a...
Synonyms: coronary fistula | congenital arteriovenous coronary fistula | congenital coronary fistula to pulmonary artery | Congenital coronary arterial fistula to right ventricle | Congenital coronary arterial fistula to the left ventricle
Excludes: anomalous origin of coronary artery from pulmonary arterial tree
Hierarchy: Structural developmental anomalies of the circulatory system → Structural developmental anomaly of heart or great vessels → Congenital anomaly of coronary artery (LA8C) → Congenital coronary arterial fistula
|
BA8Z
|
Diseases of coronary artery, unspecified
|
Table 3 Case studies: pathways to diagnosis MLD subtype Diagnostic journey Late infantile: Case study 1 • First symptoms were observed at 2 years old. • The child did not progress from walking holding on to things to walking independently. • The child saw various doctors and hip dysplasia was suspected. During this time the child was finding it more difficult to walk and was referred to a community paediatrician who sent for blood tests and MRI. • Late infantile MLD was diagnosed at 2 years and 8 months old. Interviewer : “So, between the first symptoms and when you got the diagnosis, had your daughter deteriorated any further?” Parent : “Yes. She had deteriorated more by that point. Her speech had slurred quite significantly, and she was dribbling excessively. And her sleep was really disturbed as well.” Late infantile: Case study 2 • First symptoms were observed at 1 year old. • The child did not progress from walking holding on to things to walking independently. • The child saw various doctors and parents noticed a decline at 18 months old. • First referred to a community physiotherapist for walking issues, child was getting worse, after a long time was referred to a paediatrician . • The child was misdiagnosed with hyperkalaemia and neuropathy. • Genetic tests and initial MRI were inconclusive. • MLD was suspected after the second MRI and genetic tests confirmed diagnosis three months later. • Late infantile MLD was diagnosed at 2 years and 6 months old. Interviewer : “What led them to do the MRI?” Parent : “We knew that he was getting worse, and we had always kind of had to speak up for [name] and had some disagreements with the team and what they thought, and we demanded that he be seen again, that he was regressing. And so, they did the MRI the second day and they discovered that there was white matter accumulating in the brain and in that respect when they looked at the first MRI, they also discovered that they should have seen it back then too. 18 months lost.” Late infantile: Case study 3 • First symptoms were observed at 3 months old. • Parents were concerned that the child had a degenerative condition from an early age. • The child first went to the GP with feeding issues where they saw a breastfeeding specialist who noted the child had an asymmetric jaw. • Numerous visits to the GP for frequent chest infections, concerns over breathing at night and floppy baby were recorded. • This led to referral to paediatrician 1 , who felt that feeding issues were due to reflux. • Further visits to the GP were made, the child was very ill for 6 weeks, sick at every feed, and had a temperature. • The child did not pass urine for 24 h and was then hospitalised with pneumonia. The parents asked for help as they felt that the first paediatrician was not listening to them. • This led to referral to paediatrician 2 , who referred the child to physiotherapy. • The physiotherapist made some progress, but the child then started to regress. • The midwife noted delayed growth and motor skills at the “2-year check”. Referred the child to a community paediatrician. • The parents of the child talked through all their concerns with the community paediatrician , assessments were done, and the parents were told child was just delayed. • The mother persevered and asked for a test for muscular dystrophy, the child was referred to a neurologist. • The neurologist was concerned, tests were conducted, and a diagnosis was reached. • Late infantile MLD was diagnosed at 2 years and 6 months old. Parent : “We got rushed into the hospital and that’s where I met the second consultant, where I just broke down and said, I know he’s got a chest infection, but I think there’s more than this. I think there’s more to it than this and I feel like nobody’s listening to me. Like the doctor’s not listened to me, the other paediatrician didn’t listen to me. And I feel like we just need some help.” Early juvenile: Case study 1 • First symptoms were observed at 5 years old. • The child had previously been bright, but had lost interest in reading, was becoming clumsy and had wet themselves a few times. Behaviour issues were also reported at school. • The GP thought the child might be having petit mal seizures. • The child’s nursery teacher offered to assess them and could see there had been a significant change – she spoke to the doctor. • The doctor referred the child for a CT scan and a brain degenerative condition was confirmed. • The child had an MRI in July and by September was unable to walk. • Early juvenile MLD was diagnosed at 5 years and 6 months old. Parent : “I was quite often just shrugged off as a neurotic mother, I think. There was various things that just weren’t adding up to me. Just little things. And our initial thoughts were that she wasn’t settling in very well for school. She’d just started reception. And I had approached the school for help many times weekly. And probably on a weekly basis, I was in asking for her to be referred somewhere. And I was just constantly met with… Just made to be obviously neurotic, really. And she was just a naughty, difficult child.” Early juvenile: Case study 2 • First symptoms were observed at 3 years old. • The child started tripping up. • By age 4, the child would get frustrated trying to pull up a zip or put a lid on a pen. • The GP reassured the parents that children just develop at different rates and by age 5 all children have caught up. • The parents went back to the GP with more symptoms, which were getting worse, including constant frustration and behavioural issues. GP referred child to a psychologist and a child development unit . • A series of assessments were done, the school noted that the child’s hands would shake when they picked up a pen. Dyspraxia was diagnosed and occupational therapy given. • Parents were concerned about the hand tremor and had researched it and felt there could be a neurological issue. The GP referred them back to the child development unit. • The child development unit were resistant but agreed to do an MRI and blood tests. Some underdevelopment in myelination was found but they were told this was nothing to worry about. • The parents pushed for further investigation and MRI was sent to neurologist for review towards the end of the year. • Parents could not get in touch with the paediatrician to find out results, calls and emails were not answered. Diagnosis was finally given; paediatrician did not know about the disease and suggested the parents research it. • Parents found out that it was metabolic and approached Great Ormond Street Hospital where confirmatory diagnostic testing was done. • Early juvenile MLD was diagnosed at 6 years old. Adult onset: Case study 1 • First symptoms were observed at 20–21 years old. • The patient was at university and had become forgetful. However, in hindsight with an understanding of MLD, there were some early signs from 17 years old. The patient achieved lower grades than expected in A-levels and showed signs of aggression. • The patient was referred to a psychiatrist and investigated for schizophrenia and other possible causes. The trigger for diagnosis was when the patient could no longer tell the time. • The Parents pushed for further investigation and an MRI was done. • Adult onset MLD was diagnosed at 23 years old. Parent : “If we’d got the diagnosis a year earlier, he would probably have been living independent life still, albeit supported. Because it was that last year, was really when the symptoms started to manifest. And it was obvious we couldn’t leave him alone for any length of time. We had to monitor what was happening. He’d put a meal in the oven to cook and then go out.”
| 3.865234
| 0.832031
|
sec[2]/sec[2]/p[1]
|
en
| 0.999996
|
PMC9635117
|
https://doi.org/10.1186/s13023-022-02550-z
|
[
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"years",
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"paediatrician",
"they",
"symptoms",
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[
{
"code": "LD24.04",
"title": "Chondrodysplasia punctata"
},
{
"code": "QC2Y",
"title": "Other specified contact with health services associated with the health of others"
},
{
"code": "QE61.0",
"title": "Loss or death of child"
},
{
"code": "MG43.31",
"title": "Feeding problem of child"
},
{
"code": "QA00.1",
"title": "Routine child health examination"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Chondrodysplasia punctata (LD24.04)】
Synonyms: chondrodysplasia punctata (stippled epiphyses) group | chondrodysplasia punctata congenita | dysplasia punctata epiphysis | dysplasia punctata | dysplasia epiphysealis punctata
Hierarchy: Multiple developmental anomalies or syndromes → Syndromes with skeletal anomalies as a major feature (LD24) → Syndromes with micromelia (LD24.0) → Chondrodysplasia punctata
【2. Other specified contact with health services associated with the health of others (QC2Y)】
Synonyms: Boarder in health-care facility other than healthy person accompanying sick person | Health supervision or care of other healthy infant or child | child in care | healthy infant receiving care | well-baby care
Hierarchy: Factors influencing health status or contact with health services (24) → Reasons for contact with the health services → Contact with health services associated with the health of others → Other specified contact with health services associated with the health of others
【3. Loss or death of child (QE61.0)】
Synonyms: loss of child | death of child
Excludes: Prolonged grief disorder
Hierarchy: Factors influencing health status → Problems associated with absence, loss or death of others → Disappearance or death of family member (QE61) → Loss or death of child
【4. Feeding problem of child (MG43.31)】
Excludes: Feeding or eating disorders | Anorexia Nervosa | Avoidant-restrictive food intake disorder | Pica | Rumination-regurgitation disorder
Hierarchy: General symptoms → Symptoms or signs concerning food or fluid intake (MG43) → Feeding difficulties (MG43.3) → Feeding problem of child
【5. Routine child health examination (QA00.1)】
Definition: Routine health check for child over 28 days of age through 19 years of age.
Synonyms: routine health examination for child over 28 days of age | medical examination of infant or child over 28 days of age | health check-up of infant or child over 28 days of age | development testing of infant or child | examination of infant or child
Excludes: Health supervision or care of abandoned infant | Health supervision or care of other healthy infant or child
Hierarchy: Reasons for contact with the health services → Contact with health services for purposes of examination or investigation → General examination or investigation of persons without complaint or reported diagnosis (QA00) → Routine child health examination
|
LD24.04
|
Chondrodysplasia punctata
|
In this case report, we describe a probable drug-induced serotonergic overstimulation with paroxysmal exacerbations in a patient with PD. As other differential diagnoses could be ruled out, we suggest an interaction of several serotonergic medications, i.e., MAO-A inhibitor moclobemide and MAO-B inhibitor rasagiline as a causal factor. The hypothesis of a drug-induced symptomatology rather than genuine panic attacks is supported by subsiding of symptoms after discontinuation of both moclobemide and rasagiline, together with ranolazine. In addition, the paroxysmal episodes including jitteriness, perioral and acral formications, palpitations, thoracic tightness, elevated blood pressure, and darkish red discoloration (flush) of the scalp and neck were suggestive of drug-induced monoaminergic overstimulation. We are not aware of another case report describing serotonin toxicity with this combination in the context of Parkinson's disease. Several subtypes of panic attacks have been described in the literature postulating cardiac, cardiovascular, neurological, respiratory, and vestibular classifications of panic attacks . Patients presenting with chest pain frequently show symptoms/criteria for panic disorder which results in diagnostic uncertainty, and in the presented case, coronary vasospasms of unknown origin had been previously suspected and treated with ranolazine. However, as the patient did not experience a significant effect on her symptoms, she reported at admission that she had been taking ranolazine not regularly and the discontinuation of ranolazine at admission did not result in the occurrence of cardiovascular symptoms. On the contrary, the patient's physical activity, i.e., walking longer distances, was restored after motor symptoms were additionally stabilized by adjusting anti-Parkinson medication as described in detail above. The serotonin syndrome is commonly perceived as a potentially fatal entity following exposure to serotonergic substances. The risk is increased with combination of two or more drugs which directly enhance postsynaptic serotonin levels. However, serotonin toxicity could reflect a continuous, dose-related phenomenon with serotonin syndrome being the maximum clinical manifestation of serotonin toxicity . Frequently, the clinical symptoms of serotonin toxicity develop rapidly when a serotonergic drug is added to a preexisting medication with stimulating effects on serotonin neurotransmission . It is of note that the combined use of moclobemide and rasagiline is contraindicated in the product monographs of these compounds as well as that of levodopa/benserazide and to our knowledge clinical guidelines are not supporting this combination. Due to the high prevalence of psychiatric symptoms and psychiatric comorbidities in patients with PD, combined pharmacological treatment with anti-Parkinson drugs and drugs for treatment of depression and anxiety symptoms are often inevitable. In general, hypotheses and assumptions described in this case report have certain limitations. Quantitative measurements of plasma serotonin and other monoamines as well as their respective metabolites, i.e., urinary or CSF levels of 5-HIAA, have not been determined. Thus, our conclusions with respect to the aetiology of the patient's symptoms are mainly supported by the patient's medical history, clinical symptoms, considerations of drug-drug interactions, and rapid clinical improvement after discontinuation of suspected drugs, i.e., moclobemide and rasagiline. The pharmacological interactions between moclobemide (MAO-A inhibitor) and rasagiline (MAO-B inhibitor) likely resulted in intolerable side effects as described above. We speculate that the dual inhibition of MAO-A and MAO-B may have led to MAO-inhibition comparable to the effect of irreversible MAO-inhibitors such as tranylcypromine despite the reversible nature of MAO-A inhibition by moclobemide. This may have been intermittently potentiated by dietary amines since the patient was not on a low tyramine diet . It is of note that the combined use of moclobemide and rasagiline is contraindicated in the product monographs and to our knowledge clinical guidelines are not supporting this combination. We assume that moclobemide had been taken into consideration despite strong arguments against, including formal contraindication label, after a number of antidepressants were previously prescribed but eventually discontinued due to side effects and recurrence of depressive symptoms. Interestingly, when moclobemide had been given at a low dose of 150 mg daily in combination with rasagiline, the patient did indeed benefit for approximately 3 years describing improvement of depressive symptoms and good tolerability. Reported psychovegetative symptoms occurred for the first time 6 months after the dose increase of moclobemide to 450 mg daily. This long delay in onset of adverse effects related to increasing oscillations of serotonin toxicity may explain why drug-drug interactions were discarded as a potential cause by physicians. The aspect that MAO-B activity increases with age may have additionally contributed to this delayed onset of symptoms. The significant reduction of MAO-B as demonstrated by Bitsios et al. who found an approximately 29% reduction in human platelet MAO-B activity after a single dosage of 450 mg moclobemide and the supratherapeutic plasma concentration of moclobemide we detected in our patient likely constitute additional contributing factors. Hence, the following clinically relevant main aspects can be derived from the case report at hand to improve patient safety and care: The importance of assessing and considering drug-drug interactions: our patient has been prescribed with a contraindicated combination of two drugs. We believe that the association of reported symptoms with adverse effects due to a drug-drug interaction was not addressed by the patient's initial prescriber, GP and ER (emergency room) physicians. The fact that the treatment was well tolerated by the patient for a prolonged period of time has likely contributed to this outcome. The case presented here highlights the importance of comprehensive and thorough assessment of pharmacological treatment and medication history and that drug-drug interactions should always be considered a potential etiological factor of clinical symptoms. This is of particular relevance for drugs where interactions have been reported as well as drugs that are not commonly prescribed or usually prescribed by different disciplines. Drug-induced serotonergic overstimulation occurring on a continuous spectrum of changes in serotonergic neurotransmission: to the best of our knowledge, this is the first case report of a patient with PD with potentially drug-induced chronic serotonergic overstimulation with intermittent clinical exacerbations mimicking panic attacks. Most likely, this was caused by combined treatment with an antidepressant (moclobemide; MAO-A inhibitor) and anti-Parkinson medication (rasagiline; MAO-B inhibitor). Interestingly, signs of serotonin toxicity probably developed following an increase of moclobemide from 150 to 450 mg daily and “although polypharmacy is an important etiological factor in the development of serotonin syndrome per se, dose and speed of distribution may determine its severity" . We believe that this case report can support the clinically relevant assumption that drug-induced serotonergic overstimulation may occur on a continuous spectrum of changes in serotonergic neurotransmission which may present as mild clinical symptoms, clinical exacerbations, or the potentially life-threatening condition of serotonin syndrome.
| 4.296875
| 0.921387
|
sec[2]/p[0]
|
en
| 0.999997
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33728085
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https://doi.org/10.1155/2021/8868023
|
[
"drug",
"symptoms",
"moclobemide",
"serotonin",
"serotonergic",
"rasagiline",
"drugs",
"induced"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Hazardous drug use, unspecified (QE11.Z)】
Synonyms: Hazardous drug use | chronic drug use NOS | chronic IV substance use | drug use nos | intravenous drug use NOS
Hierarchy: Problems associated with health behaviours → Hazardous substance use → Hazardous drug use (QE11) → Hazardous drug use, unspecified
【2. Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified (6C4G.2Z)】
Synonyms: Unknown or unspecified psychoactive substance dependence | Drug dependence NOS
Hierarchy: Disorders due to substance use → Disorders due to use of unknown or unspecified psychoactive substances (6C4G) → Unknown or unspecified psychoactive substance dependence (6C4G.2) → Unknown or unspecified psychoactive substance dependence, substance and state of remission unspecified
【3. Harmful effects of drugs, medicaments or biological substances, not elsewhere classified (NE60)】
Synonyms: drugs, medicaments or biological substances, toxicity not elsewhere classified | adverse drug effects | drug reaction NOS | drug allergy NOS | drug toxicity NOS
Excludes: Alcohol intoxication | pathological drug intoxication | hypersensitivity reaction to correctly administered drug | Reactions or intoxications due to drugs administered to fetus or newborn | Opioid intoxication
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Harmful effects of substances → Harmful effects of drugs, medicaments or biological substances, not elsewhere classified
【4. Drug hypersensitivity of unspecified type (4A85.0Z)】
Synonyms: Drug or pharmacological agents hypersensitivity | medicinal hypersensitivity | drug sensitivity NOS
Hierarchy: Allergic or hypersensitivity conditions → Complex allergic or hypersensitivity conditions (4A85) → Drug or pharmacological agents hypersensitivity (4A85.0) → Drug hypersensitivity of unspecified type
【5. Intoxication due to unknown or unspecified psychoactive substance (6C4G.3)】
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 wh...
Synonyms: psychoactive substance abuse | trance and possession disorders in psychoactive substance intoxication | drug intoxication NOS
Hierarchy: Disorders due to substance use or addictive behaviours → Disorders due to substance use → Disorders due to use of unknown or unspecified psychoactive substances (6C4G) → Intoxication due to unknown or unspecified psychoactive substance
|
QE11.Z
|
Hazardous drug use, unspecified
|
A 79-year-old man was diagnosed with pancreatic IPMN, gallstones, and common bile duct stones before surgery for aortic regurgitation, mitral regurgitation, and tricuspid regurgitation. Pancreatic IPMN worsened after cardiac surgery and the patient was referred to our department. Pancreatic IPMN showed high-risk stigmata of enlarged cyst and dilated main pancreatic duct of 48 mm and 18 mm in diameter, respectively . Prior to surgery, endoscopic sphincterotomy was performed for common bile duct stones. We found that the common channel connecting the bile duct and the pancreatic duct was relatively long at 12 mm in length. The pancreatic duct was simultaneously visualized when the contrast agent was injected into the common bile duct . Laparoscopic distal pancreatectomy and splenectomy were performed using a stapler with biological reinforcement. The intraoperative appearance of the stump of the pancreas appeared to be well enclosed . The stump of the pancreas was covered with a polyglycolic acid mesh with fibrin glue. The operation time was 323 min, and the bleeding volume was 90 ml. Concomitant cholecystectomy was avoided to reduce operation time and physical burden. Although the drain amylase level at the stump of the pancreas on postoperative day (POD) 1 was elevated, it gradually decreased day by day after treatment with non-eating, antibiotic, and somatostatin analog (Table 1 ). Contrast-enhanced computed tomography (CT) on POD 4 showed no obvious PA formation (data not shown). Sudden bleeding was observed from the abdominal drain near the stump of the pancreas on POD 6 . Emergent stent graft placement. Emergent stent graft placement and coil embolization were performed for the ruptured PA from the stump of the splenic artery (SA) to the common hepatic artery (CHA). Initially, we placed a stent graft (φ 6 mm × 25 mm, GORE ® VIABAHN ® ) extending from the celiac artery (CA) to the CHA. However, extravasation from the SA persisted even after stent-graft placement, and we thought that this was because the stent graft was smaller than the CA. Next, we performed coil embolization at the proximal side of the stent graft. Then, we performed additional distal coil embolization from the superior mesenteric artery (SMA). The ruptured artery was completely isolated from the systemic circulation, and hepatic arterial flow was maintained from the SMA to the gastroduodenal artery (GDA) . The aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were not elevated after the placement of stent graft and coil embolization. We then replaced the abdominal drain and initiated continuous irrigation and suction of saline. On POD 9, the drainage fluid changed to yellowish in color with bile contamination (Table 1 ). Endoscopic retrograde pancreatography confirmed leakage from the main pancreatic duct. Endoscopic retrograde biliary drainage (ERBD) tube and endoscopic retrograde pancreatic drainage (ERPD) tube were placed as internal drainage of the digestive fluid . On POD 24, second emergent coil embolization was performed for bleeding from the periphery of the left gastric artery (data not shown). On POD 25, open abdominal drainage, omental filling around the pancreatic stump, cholecystectomy, and tube ileostomy were performed. The second operation time was 252 min, and the bleeding volume was 1090 ml. The second operation revealed that the arterial wall of the CHA had disappeared, and the stent graft was exposed . On POD 32, third emergent coil embolization was performed for the ruptured PA at the gastroduodenal artery . Intrahepatic arterial flow was maintained from the right inferior phrenic artery . A balloon catheter was placed at the root of the celiac artery. Subsequently, remnant pancreatic resection was performed. The stent graft placed at the CHA was removed under balloon occlusion of the celiac artery. During the operation, massive bleeding occurred from the right side of the portal vein and was controlled by continuous sutures. The third operation time was 506 min, and the bleeding volume was 5372 ml. After total pancreatectomy, the patient developed renal failure, liver failure, and sepsis. The AST and ALT levels after the third operation were 1662 U/L and 867 U/L, respectively. On POD 39, massive bleeding was again observed from the abdominal drain. Emergent arterial portography under occlusion of the intra-aortic balloon revealed stenosis of the portal vein sutured during the third operation. The bleeding point was the right wall of the superior mesenteric vein, where the remnant pancreas was removed . Although we attempted a percutaneous transhepatic approach, it was unable to treat due to poor blood flow in the intrahepatic portal vein. The patient died of hemorrhagic shock on the same day. Fig. 1 Preoperative imaging findings. a T2-weighted MRI showed a cystic tumor, 48 mm in diameter, in the pancreatic body. b Size of the main pancreatic duct was 18 mm in the pancreatic tail. c ERCP showed that the length of the common channel connecting the bile duct and pancreatic duct was 12 mm (arrowhead) Fig. 2 Intraoperative findings around the stump of the pancreas. The stump of the pancreas appeared to be well enclosed with a linear stapler. The stump of the splenic artery (arrow), splenic vein (arrowhead), and inferior mesenteric vein (dotted arrow) Table 1 Postoperative biological and culture data POD 1 POD 2 POD 3 POD 4 POD 7 POD 9 POD 14 POD 24 POD 26 POD 28 POD 30 B.T (℃) 37.0 37.8 37.5 37.0 37.3 37.2 37.6 37.8 38.4 37.2 37.1 CRP (mg/dl) 3.17 13.03 25.43 21.86 7.71 10.40 5.74 3.99 7.54 17.60 4.85 WBC (/μl) 11,980 17,530 15,380 9550 15,190 10,100 13,260 12,600 8470 9500 7710 Drain AMY (U/L) 17,623 10,447 4562 5243 115 124,999 168,010 378 116,760 191,584 248,090 Drain bil (mg/dl) N.A N.A N.A N.A N.A 4.6 0.1 N.A 0.4 0.1 0.1 Drain culture N.A N.A Negative N.A N.A Enterococcus Corynebacterium, Pseudomonas, Candida N.A N.A MRSA, Candida N.A N.A. not assessed Fig. 3 Imaging findings at postoperative day 6. a Enhanced CT showed a pseudoaneurysm at the stump of the splenic artery (arrowhead). b Enhanced CT showed extravasation of contrast medium (arrow). c Reconstruction image of the abdominal arteries and extravasation from the stump of the splenic artery (arrow). Stenosis and poststenotic dilatation of the common hepatic artery (arrowhead). d Superior mesenteric artery to the gastroduodenal artery angiography after placement of the stent graft (arrowhead), proximal coil (arrow), and distal coil (dotted arrow). e Schema after the placement of stent graft and coil embolization. GDA gastroduodenal artery, LGA left gastric artery, IPA infraphrenic artery, SA splenic artery Fig. 4 Endoscopic retrograde pancreatography at postoperative day 9. a , b Leakage from the main pancreatic duct (arrowhead), ERBD (arrow), and ERPD (dotted arrow) Fig. 5 Intraoperative findings during the second operation. The Arterial wall of the common hepatic artery disappeared, and the stent graft (arrow) was exposed Fig. 6 Imaging findings at postoperative day 32. a Enhanced CT showed pseudoaneurysm at the gastroduodenal artery (arrowhead). b Angiography showed the partially dislocated coil placed at the common hepatic artery (arrowhead), left gastric artery (arrow), and gastroduodenal artery (dotted arrow). c Angiography from the root of the celiac artery showed intrahepatic arterial flow (arrowhead) from the right inferior phrenic artery Fig. 7 Arterial portography at postoperative day 39. Extravasation of contrast medium from the right side of the superior mesenteric vein (arrow), faint visualization of the portal vein (arrowhead), and left gastric vein (dotted arrow)
| 4.011719
| 0.971191
|
sec[1]/p[0]
|
en
| 0.999998
|
34757521
|
https://doi.org/10.1186/s40792-021-01324-2
|
[
"artery",
"pancreatic",
"arrow",
"duct",
"stent",
"graft",
"stump",
"coil"
] |
[
{
"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 CLASSIFICATION ═══
【1. Diseases of arteries or arterioles, unspecified (BD5Z)】
Synonyms: artery disease NOS | arterial disease NOS | arteriolar disease NOS | disorder of artery NOS | arteriopathy
Hierarchy: Diseases of the circulatory system (11) → Diseases of arteries or arterioles → Diseases of arteries or arterioles, unspecified
【2. Certain specified disorders of arteries or arterioles (BD52)】
Synonyms: Aortic dilatation - joint hypermobility - arterial tortuosity | Generalised arterial calcification of infancy | Median arcuate ligament syndrome | Aortic root abscess | Periaortic abscess
Excludes: collagen (vascular) diseases | Hypersensitivity angiitis | Acute arterial occlusion | Chronic arterial occlusive disease
Hierarchy: Diseases of the circulatory system (11) → Diseases of arteries or arterioles → Certain specified disorders of arteries or arterioles
【3. Rupture of artery (BD52.3)】
Synonyms: ruptured artery | artery fistula | Aortic duodenal fistula | Aortic colon fistula | Aortic oesophageal fistula
Excludes: traumatic rupture of artery - see injury of blood vessel by body region
Hierarchy: Diseases of the circulatory system (11) → Diseases of arteries or arterioles → Certain specified disorders of arteries or arterioles (BD52) → Rupture of artery
【4. Stricture of artery (BD52.2)】
Synonyms: arterial stenosis | arterial stricture | artery stricture | stenosis of artery | Obliterative vascular disease
Hierarchy: Diseases of the circulatory system (11) → Diseases of arteries or arterioles → Certain specified disorders of arteries or arterioles (BD52) → Stricture of artery
【5. Atherosclerotic chronic arterial occlusive disease, unspecified (BD40.Z)】
Synonyms: Atherosclerotic chronic arterial occlusive disease | arteriosclerosis, NOS | generalised atherosclerosis | atherosclerosis NOS | peripheral arteriosclerosis obliterans
Hierarchy: Diseases of arteries or arterioles → Chronic arterial occlusive disease → Atherosclerotic chronic arterial occlusive disease (BD40) → Atherosclerotic chronic arterial occlusive disease, unspecified
|
BD5Z
|
Diseases of arteries or arterioles, unspecified
|
Although any kind of surgery is highly discouraged in FOP patients due to an increased risk of flare-ups and progression of the disease, this case demonstrates that in a life-threatening situation–an operative procedure can be considered and managed successfully even in severely affected patients. It requires the assembly of a multidisciplinary FOP-dedicated team with knowledge of the disease and preparations made in anticipation of complications that may occur. In the current case, the timely and detailed preparation on the multidisciplinary team and the innovative techniques employed throughout the perioperative period assured the benign outcome of the surgical procedure. Because there is no effective treatment available to stop the formation and progression of HO, surgical procedures are highly discouraged as standard care of FOP ( 5 , 29 ). Even small traumata–e.g., biopsies—can cause sufficient damage to the muscle and trigger a flare-up with subsequent HO formation ( 30 ). In the described case, surgery was the only life-saving option: it was judged that the patient was unlikely to survive the rapidly increasing, progressive infections of her leg due to antibiotic resistant organisms after many years of treatment. Surprisingly, a negligible amount of HO formed after the through-knee amputation, possibly due to a period of silent disease activity before and at the time of the operation. The reason for the quiescent disease in this patient is not known. One hypothesis is that, as it is known that the immune system plays a role in the pathogenesis of HO ( 31 ), the chronic inflammation and antibiotic use could have suppressed disease activity. Interestingly, 12 months after the surgical procedure disease activity was noted at various sites with HO. This could be the result of a normalized level of inflammation, or a systemic, late effect of the surgical procedure itself. Based on case reports in literature describing limb surgeries, where postoperatively HO formation was observed in almost 90% of the cases ( 12 – 21 , 23 – 28 ), it was expected that clinically relevant HO would form. It should be noted, that over 90% of the published limb surgeries were performed to remove HO. Only in two patients (7%) HO did not reoccur after the removal of HO. Both patients received bisphosphonate treatment ( 19 ). Due to the absence of the effect of bisphosphonate treatment in nine others, it is more likely that the good result in those two can be attributed either to an incomplete follow-up time or due to limited imaging modalities as both cases are reported in 1976 ( 19 ). Removal of HO might be complex when it has formed within a muscle or when it has fused with normal skeletal bone. Removal of HO can therefore be considered as a high impact procedure which triggers HO formation. In our case a through-knee amputation was performed which is a procedure with relatively limited trauma to muscles because the procedure does not affect normal skeletal bone and it mainly involves the origin and insertion of muscles and tendons. In addition, when possible, ankylosed bone parts were left in situ to minimize tissue damage. To limit the extent of HO formation after surgery, it has been suggested to administer corticosteroids as a prophylaxis for four consecutive days after surgery ( 5 ). Objective data on the effectiveness of glucocorticoids in flare-ups are lacking. But based on empirical data, it is believed that it reduces oedema and may cause symptom relief ( 4 ). Glucocorticoids are currently the only treatment available for FOP. Corticosteroids, however, also interfere with wound healing. Therefore, in the current case, they were only administered pre-operatively. Hopefully, an effective treatment will be available to halt the formation of HO in the near future. To date, four potential drugs are tested in a clinical trial: Palovarotene, Garatosmab, Rapamycin and Saracatinib ( 32 – 35 ). Once found effective in preventing HO formation, surgical treatment might be an option to unlock joints or to safely operate an FOP patient for any other condition under an umbrella of one (or a combination) of these drugs. Besides the impact of the surgical procedure and the attempt to suppress FOP activity with glucocorticoids, the anesthetic management is another major concern and challenge in FOP patients. Regional anesthesia techniques (peripheral nerve blocks) involve punctures causing tissue trauma with increased risk of flare-ups, and these are therefore considered contraindicated. Likewise, neuraxial (spinal or epidural) anesthesia is not recommended for the following reasons. First of all, the spine is often involved in the disease and thus inapproachable for puncture. Secondly, the puncture itself might trigger HO formation, which could compress the spinal cord ( 5 ). Therefore, general anesthesia is generally recommended for FOP patients. General anesthesia requires airway management and frequently mechanical ventilation, both of which can be extremely challenging in FOP patients ( 36 , 37 ). FOP patients often have jaw ankylosis, making conventional direct laryngoscopy or even video-laryngoscopy impossible for tracheal intubation. Moreover, even in the absence of a temporomandibular joint (TMJ) ankylosis, direct laryngoscopy is discouraged because hyperextension of the neck is limited–if not impossible–due to fused cervical vertebrae and in addition, overstretching of the TMJ joint or vertebral facet joints during tracheal intubation might induce a temporomandibular joint flare-up ( 5 ). Therefore, fiberoptic naso-tracheal intubation is preferred in all FOP scheduled for general anesthesia ( 5 ). This would have been possible in the current case, however, the risk of general anesthesia was deemed unacceptably high. The patient suffered from a severely restricted pulmonary function due to a completely immobile thoracic cage ( 7 , 9 ). It was anticipated that high inspiratory airway pressures would be needed during mechanical ventilation to maintain adequate gas exchange. This can lead to over-distention of alveoli causing pulmonary barotrauma ( 38 ). Other challenges that were anticipated were a ventilation-perfusion mismatch and difficulties in weaning form mechanical ventilation. In addition, FOP patients are known to have impaired thoracic flexibility and weakened respiratory muscles predisposing to ineffective coughing, with an increased risk of mucus retention and infection ( 5 ). Therefore, a regional anesthesia approach was chosen, with ultrasound guidance to identify structures and to limit tissue trauma. Glucocorticoids were locally injected via the placed nerve block catheters in an attempt to prevent a flare-up. Since regional anesthesia alone was insufficient to ensure complete analgesia and patient's comfort, systemic drugs were added. As these drugs might induce apnea, it is important to monitor the patient closely and keep high-flow nasal oxygen standby in case support of oxygenation is needed ( 39 , 40 ). To conclude, based on the literature it was almost certain that HO would form as a response to a surgical procedure of a limb. In the current case, HO was indeed formed, but even 12 months after surgery the volume of the formed HO minimal. It is hypothesized that the patient's silent disease activity and the continuous antibiotic treatments might have influenced this. If surgery needs to be performed, it is important that it is performed by a multidisciplinary team with knowledge about FOP and after carefully weighing the surgical benefits against the challenges and risks of both the anesthetic and surgical procedures for the FOP patient.
| 4.226563
| 0.728027
|
sec[3]/p[0]
|
en
| 0.999997
|
32973683
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https://doi.org/10.3389/fendo.2020.00570
|
[
"patients",
"procedure",
"disease",
"formation",
"anesthesia",
"surgery",
"flare",
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] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Patient received diagnostic test or treatment intended for another patient (PL14.C)】
Synonyms: wrong patient | incorrect patient
Excludes: Procedure undertaken at wrong site or wrong side, as mode of injury or harm
Hierarchy: External causes of morbidity or mortality (23) → Causes of healthcare related harm or injury → Mode of injury or harm associated with other health care related causes (PL14) → Patient received diagnostic test or treatment intended for another patient
【2. Unavailability or inaccessibility of health care facilities (QB14)】
Synonyms: unavailability of medical facilities | Unavailability of outpatient clinic | Unavailability or inaccessibility of residential aged care service
Excludes: bed unavailable
Hierarchy: Factors influencing health status or contact with health services (24) → Reasons for contact with the health services → Factors related to medical facilities or other health care → Unavailability or inaccessibility of health care facilities
【3. Problem associated with physical transfer of patient (PL14.2)】
Hierarchy: External causes of morbidity or mortality (23) → Causes of healthcare related harm or injury → Mode of injury or harm associated with other health care related causes (PL14) → Problem associated with physical transfer of patient
【4. Organ transplant candidate (QB12.0)】
Synonyms: 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
Hierarchy: Reasons for contact with the health services → Factors related to medical facilities or other health care → Waiting period for investigation or treatment other than awaiting admission to adequate facility elsewhere (QB12) → Organ transplant candidate
【5. Counselling related to sexual behaviour and orientation or sexual relationships of the person (QA15.1)】
Synonyms: advice on sexual behaviour or orientation | counselling on sexual behaviour or orientation | promiscuity counselling | patient concerned regarding sexual orientation | counselling related to patient's sexual behaviour and orientation
Hierarchy: Reasons for contact with the health services → Contact with health services for counselling → Counselling related to sexuality (QA15) → Counselling related to sexual behaviour and orientation or sexual relationships of the person
|
PL14.C
|
Patient received diagnostic test or treatment intended for another patient
|
A 67-year-old patient was admitted to this hospital due to gait alteration, cognitive disturbances and rapid clinical deterioration. The patient had a history of diabetes, asthma and a myocardial infarction 2 years earlier. Medications at the time of admission were acetylsalicylic acid, clopidogrel, glimepiride, pitavastatin and omeprazole. For the previous 2 years, he had intermittent syncope episodes. In one of them he suffered a fracture of the left tibia. Encephalogram, electrocardiogram (ECG), subcutaneos Holter, echocardiography and stress ECG were normal. A month before admission, hehad a new syncope episode while cultivating vegetables and he suffered a traumatic brain injury (TBI) with a left orbital and right occipital hematoma and he went to the Emergency Department of the Albacete General Hospital. A computed tomography (CT) showed a left subdural and retroorbital hematoma as well as a right occipital skull fracture. Neurologic examination was normal. A week after the injury, he suffered a respiratory infection with cough and yellow mucus and his primary care physician treated him with amoxicillin-clavulanic acid (875/125 mg every 8 h, 7 days, orally) with a consequent improvement of the respiratory symptoms. Then he was referred to the Internal Medicine Department for presenting continuous headache since the head injury and symptoms of progressive weakness in the lower limbs with instability while walking and confusion. The patient maintained normal vital signs and his body temperature was 36.5 °C. No skin lesions, lymph nodes or goiter were present. He carried a subcutaneous Holter device with no signs of local infection. Cardiopulmonary and abdominal explorations were normal. No edemas were present and the pulses were palpable. He showed disorientation to time and place, but not person. He had a paresis of the left seventh cranial nerve. He had a minimal decrease in strength in the lower limbs. Both the proprioceptive and the tactile sensitivity were diminished in the lower limbs, and showed no sensitivity to vibration. The tendinous reflexes were diminished in the examination. The finger-to-nose testing was impaired. The Romberg test was positive. The patient had gait disturbance with widened base and inability for tandem gait. He was admitted to the Internal Medicine Department. Laboratory tests were performed. Routine hematological testing showed a total hemoglobin 14.4 g/dl and a normal leucocyte and platelet count.Erytrocyte sedimentation raye was 51 mm/1 h (normal range values 1–20 mm/ 1 h). Prothrombin time and partial –thromboplastin time were normal. All blood chemistry values were normal except the values for glucose and C-reactive protein (CRP). The glucose concentration was 200 mg/dl (normal range values 74–109 mg/dl), the CRP level was 11.8 mg/l (normal range values 0–5 mg/l). Blood and urine cultures were negative. Chest x-ray was normal. Serology for Treponema pallidum, Coxiella burnetii, BrucellaBorrelia burgdorferi, HIV, Cytomegalovirus, Epstein Barr virus, Simple herpes virus, Toxoplasma, Mycoplasma pneumoniae was negative for acute infection with no seroconversion. A contrast magnetic resonance was carried out which revealed a right occipital fracture with a left frontobasal hematoma, a small left subdural occipital hematoma, a right occipital fracture with laminar subdural hematoma and a frontobasal contusion. A thoraco-abdomino-pelvic CT was carried out with no pathological findings. An electromyography (EMG) showed a sensory and motor mixed polyneuropathy with a severe demyelinization component. Moreover the patient showed a diabetic peripheral polyneuropathy. A lumbar puncture was carried out and CSF was obtained with a cell count of 105 cells/mcl with 100% mononuclear cells, glucose 100 mg/dl, 1.44 g/l proteins. As for the microbiological study, Gram staining of acid-fast bacilli, as well as aerobic,anaerobic,fungi, Mycobacteria and Brucella culture,were negative. Due to a positive tuberculin skin test (purified protein derivative, 5 TU) of 28 mm, a urine culture for Mycobacteria and a polymerase chain reaction (PCR) as well as a culture for Mycobacterium tuberculosis in CSF were requested and were negative. Faced with the possibility of a disimmune process and, after receiving the results of the electromyogram, it was decided on a treatment with 0.4 mg/kg/day intravenous immunoglobulin for 5 days with improvement of the clinical symptoms, recovery of the tendinous reflexes and the disappearance of the gait alteration. A second lumbar puncture was requested to check the characteristics of the fluid during treatment. The white blood cell count was 28 cells/mcl, predominantly mononuclear, no malignant cells, 0.88 g/l protein and adenosine deaminase (ADA) 5.9 u/l. The cytology of CSF showed lymphoplasmacytosis. In the microbiological analysis of CSF, Gram staining showed a few polymorphonuclear leukocytes and absence of microbial flora. The aerobic culture was performed on blood agar medium, chocolate agar medium and selective medium for Legionella (BCYE). After 7 days of incubation at 37 °C and 5% CO 2 , the culture showed growth of white, dry and rough colonies , which were presumably identified as Actinomyces spp. by microbiological routine testing such as the Gram stain (Panel B) and Kinyoun stain (Panel C), showing the presence of ramified, not acid-fast, Gram-positive rods..The bacterium was identified by molecular methods of rRNA 16S sequencing,as Pseudonocardia carboxydivorans in the National Center of Microbiology (ISCIII, Madrid, Spain). The isolate was identified by means of 16 s rRNA sequence analysis using a previously reported method The 1434 bp fragment obtained from the isolate showed a similarity of 99.7% with P. carboxydivorans . When using E -test, the strain was sensitive (MICs, μg/ml) to all antimicrobials tested: ciprofloxacin (0.12), amikacin (0.5), trimethoprim-sulfamethoxazole (2), linezolid (0.25), imipenem (0.12), amoxycilin-clavulanic (1), cefotaxime (0.5) and erythromycin (2) Treatment with trimethoprim-sulfamethoxazole was initiated (20 mg/Kg every 6 h) intravenously. He did not receive any other antibiotic treatment added.Once this germ was isolated and to complete the study, a third lumbar puncture was carried out (Table 1 ). The CSF flow cytometry was normal. Besides, a biopsy of the bone marrow and a karyotype were conducted, which were normal, and a PET-CT showed the absence of metabolically significant localization at any level. Fig. 1 Microbiologic outcomes in cerebral spinal fluid. Panel a shows the culture of the specimen obtained from SCF the patient on selective medium for Legionella (BCYE) showing growth of white, dry and rough colonies after 7 days of incubation at 37 °C and 5% CO2 atmosphere. Panels b and c show the microscopic examination of the colonies by Gram and Kinyoun modified strain, showing the presence of ramified, not acid-fast, Gram-positive bacilli. The bacterium was identified by molecular methods of RNAr 16S sequencing, being finally identified as Pseudonocardia carboxydivorans in the National Center of Microbiology (ISCIII, Madrid, Spain) Table 1 Profile of laboratory results among the 4 CFS samples collected from initial a Sample no Date of sample collection (day /mo/yr) Cell count cells/mcl Cell type ADA (u/l) Proteins g/l Glucose mg/dl CSF culture result 1 24/05/2013 105 100% MN ND 1.44 90 Neg ( Pseudonocardia ND) 2 05/06/2013 28 100% MN 5.9 0.88 84 P. carboxydivorans 3 20/06/2013 10 100% MN 5.8 0.78 96 P. carboxydivorans 4 27/08/2013 1 1 red blood cell ND 0.71 92 P. carboxydivorans a ND no data, Neg negative, MN mononuclear
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"culture",
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[
{
"code": "QE00",
"title": "Acculturation difficulty"
},
{
"code": "MD40.51",
"title": "Positive sputum culture"
},
{
"code": "MD40.52",
"title": "Positive throat culture"
},
{
"code": "QE0Z",
"title": "Problems associated with social or cultural environment, unspecified"
},
{
"code": "MG65",
"title": "Abnormal microbiological findings in specimens from other organs, systems and tissues"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Acculturation difficulty (QE00)】
Definition: Problems resulting from the inability to adjust to a different culture or environment.
Synonyms: acculturation problem | cultural shock | social migrant difficulty | migration | cultural deprivation
Excludes: Disorders specifically associated with stress
Hierarchy: Factors influencing health status or contact with health services (24) → Factors influencing health status → Problems associated with social or cultural environment → Acculturation difficulty
【2. Positive sputum culture (MD40.51)】
Hierarchy: Clinical findings in the respiratory system → Clinical findings in specimens from respiratory organs and thorax (MD40) → Abnormal microbiological findings in specimens from respiratory organs and thorax (MD40.5) → Positive sputum culture
【3. Positive throat culture (MD40.52)】
Hierarchy: Clinical findings in the respiratory system → Clinical findings in specimens from respiratory organs and thorax (MD40) → Abnormal microbiological findings in specimens from respiratory organs and thorax (MD40.5) → Positive throat culture
【4. Problems associated with social or cultural environment, unspecified (QE0Z)】
Synonyms: social environment problem
Hierarchy: Factors influencing health status or contact with health services (24) → Factors influencing health status → Problems associated with social or cultural environment → Problems associated with social or cultural environment, unspecified
【5. Abnormal microbiological findings in specimens from other organs, systems and tissues (MG65)】
Synonyms: positive wound culture | Positive culture findings on specimen from other organs, systems and tissue
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → General symptoms, signs or clinical findings → Clinical findings in specimens from other specified organs, systems and tissues → Abnormal microbiological findings in specimens from other organs, systems and tissues
|
QE00
|
Acculturation difficulty
|
At 41.8 months of age, she was first evaluated in a psychology clinic with ADI-R and ADOS, receiving scores in each domain of the ADI-R and ADOS much higher than that the cut-off values of ASD (Tables 1 , 2 and 3 ) and was consequently diagnosed with ASD (Tables 1 , 2 and 3 ). The assessment results of CPEP-3 and Gesell indicated that she had regression in behaviors, developmental skills, and neuropsychological development (Tables 4 and 5 ). Additionally, the patient had her first seizure at 2 years old without obvious predisposing factors. It was a series of repetitive absence seizure attacks with transient loss of consciousness for 3–5 s, accompanied by an atonic seizure with head drooping to one side and occasionally to the ground. Subsequently, similar seizures occurred more than 10 times per day. Her brain MRI was normal. Interictally EEG recordings showed 2.5–3.0 Hz generalized spike and slow waves , spike and slow waves in the bilateral prefrontal lobes, and slow waves (2.0–3.0 Hz) predominantly in the bilateral occipital area during both wakefulness and sleep . A diagnosis of generalized epilepsy was considered. The patient had not received AED therapy before 3.5 years old. She was initially treated with valproate (VPA) with a dose of 20 mg.kg -l d − 1 ; the seizure frequency significantly reduced but the odontoprisis was still observed. She was then treated with levetiracetam (LEV) with a dose of 28.57 mg.kg -l d − 1 . The seizures and odontoprisis disappeared, but she became irritable with frequent screaming. Finally, lamotrigine (LTG) was used as a substitute for LEV with a dose of 4.16 mg.kg -l d − 1 , and her condition was more stabilized than before. A recent EEG recording demonstrated the generalized epileptic discharge disappeared, but the focal EEG abnormalities did not show significant improvement . Table 1 Clinical features of the patient with P361T variants in SLC6A1 Patient ID Variant c.1081C > A Protein change p.P361T Origin De novo Sex Female Current age 6 years Age at seizure onset 2 years Seizure type at onset Absence and atonic Seizure frequency at onset 10 times per day Interictal EEG GSW, focal SW Seizure outcome Seizure free Duration before seizure free 3 years MRI findings Normal ADI-R RSI 24 ADI-R COM 14 ADI-R RRB 4 ADI-R abnormality of development at or before 36 months 5 ADOS SC 6 ADOS SA 14 Intellectual disability Severe ID Language Speech delay Behaviors and developmental skills Dysfunctional Neuropsychological development Severer or profound retardation Diagnosis Autism and generalized epilepsy SIFT (score) Damaging (0.0) Polyphen2 (score) Probably damaging (1.0) Mutation Taster (score) Disease causing (1) Frequency in gemomeAD_exome – Frequency in ExAC – Frequency in ExAC (East Asian) – Frequency in 1000 genomes – ADI-R Autism Diagnostic Interview-Revised, ADOS Autism Diagnostic Observation Schedule; COM qualitative abnormalities in communication, EEG electroencephalography, GSW : generalized spike and slow wave, ID intellectual disability, MRI magnetic resonance imaging, RSI qualitative abnormalities in reciprocal social interaction, RRB restricted and repetitive behavior, SA social affect, SC social communication, SW spike and wave complex Table 2 Autism Diagnostic Interview-Revised (ADI-R) of the patient at the age of 41.8 months Domain Score Qualitative Abnormalities in Reciprocal Social Interaction (RSD) Failure to use nonverbal communication to regulate social interaction (A1) 6 Failure to develop peer relationships (A2) 4 Lack of shared enjoyment (A3) 6 Lack of socioemotional reciprocity (A4) 8 Subtotal (Cut-off) 24 (10) Qualitative Impairments in Communication and Language (COM) Lack of, or delay in, spoken language and failure to compensate through gesture (B1) 8 Lack of varied spontaneous make-believe or social imitative play (B4) 6 Subtotal (Cut-off) 14 (7) Restricted, Repetitive, and Stereotyped Behaviors and interests (RRB) Encompassing preoccupations or circumscribed pattern of interest (C1) 0 Apparently Compulsive Adherence to Nonfunctional Routines or Rituals (C2) 0 Stereotyped and repetitive motor mannerisms (C3) 2 Preoccupation with part of objects or nonfunctional elements of material (C4) 2 Subtotal (Cut-off) 4 (3) Abnormality of development evident at or before 36 months (Cutoff) 5 (1) Total ( Cut-off ) 47 (21) COM qualitative abnormalities in communication, RRB restricted and repetitive Behavior; RSI qualitative abnormalities in reciprocal social interaction Table 3 Autism Diagnostic Observation Schedule (ADOS) of the patient at the age of 41.8 months Domain Score Language and Communication (SC) Frequency of spontaneous vocalization directed to others (A2) 2 Stereotyped/Idiosyncratic use of words or phrases (A5) 0 Use of another’s body (A6) 0 Pointing (A7) 2 Gestures (A8) 2 Subtotal (AUT, AS) 6 (4, 2) Reciprocal Social Interaction (SA) Unusual eye contact (B1) 2 Facial expression directed to others (B3) 2 Share enjoyment in interaction (B5) 2 Showing (B9) 2 Spontaneous initiation of joint attention (B10) 2 Response to joint attention (B11) 2 Quality of social overtures (B12) 2 Subtotal (AUT, AS) 14 (7, 4) Total of SA and SC (AUT, AS) 20 (12, 7) Play Functional play with objects (C1) 2 Imagination/Creativity (C2) 2 Subtotal 4 Stereotyped Behaviors and Restricted Interests (RRB) Unusual sensory interest in play material/person (D1) 1 Hand and finger and other complex mannerisms (D2) 2 Unusually repetitive interests or stereotyped behaviors (D4) 0 Subtotal 3 AS autism spectrum cut-off, AUT autism cut-off; SA Social Affect, SC Social Communication, RRB Restricted and Repetitive Behavior Table 4 Chinese Psychoeducational Profile-Third Edition (CPEP-3) of the patient at the age of 41.8 months CPEP-3 subsets Raw score Development ages (months) Percentiles Developmental /adaptive levels Performance Test CVP 0 < 12 < 2 Severe EL 0 < 12 2 Severe RL 0 < 12 2 Severe FM 1 < 12 < 2 Severe GM 3 < 12 < 2 Severe VMI 0 < 12 2 Severe AE 7 – 6 Severe SR 1 – < 2 Severe CMB 9 – 4 Severe CVB 0 – 2 Severe Caregiver Report PB 0 – < 2 Severe PSC 0 < 12 < 2 Severe AB 4 – < 2 Severe Composites Standard score Development ages (months) Percentiles Developmental /adaptive levels C (CVP + EL + RL) 14 6.0 6 Severe M (FM + GM + VMI) 4 6.0 1 Severe MB (AE + SR+ CMB + CVB) 14 – 2 Severe AB adaptive behavior, AE affective expression, CMB characteristic motor behaviors, CVB characteristic verbal behaviors, CVP cognitive verbal/preverbal, EL expressive language, FM fine motor, GM gross motor, PB problem behaviors, PSC personal self-care, RL receptive language, SR social reciprocity, VMI visual-motor imitation Table 5 Gesell developmental schedule of the patient at the age of 41.8 months Subfields DA (month) DQ Assessment Gross motor 14.23 34 Severe Fine motor 7.47 18 Profound Adaptive behavior 4.2 10 Profound Language 5.83 14 Profound Personal-social ability 5.37 13 Profound DA Development Age, DQ Development Quotient Fig. 3 Electroencephalogram (EEG) of a 6-year-old girl carrying GAT-1(P361T) mutation. Interictal video EEG recordings showed 2.5–3.0 Hz generalized spike and slow waves ( a ), 2.0–3.0 Hz spike and slow waves in the bilateral prefrontal lobes ( b ) and 2.0–3.0 Hz slow waves predominantly in the bilateral occipital area ( c ) during both wakefulness and sleep when the patient was 3.5 years old. Interictal video EEG recordings demonstrated 2.0–3.0 Hz spike and slow waves in the bilateral prefrontal lobes ( d ), and 2.0–3.0 Hz spike and slow waves predominantly in the bilateral occipital and posterior-temporal area ( e ) during both wakefulness and sleep when the patient was 6 years old
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sec[2]/sec[2]/p[1]
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32398021
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https://doi.org/10.1186/s13041-020-00612-6
|
[
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"slow",
"behaviors",
"development",
"spike",
"waves",
"years"
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[
{
"code": "MB23.Q",
"title": "Social withdrawal"
},
{
"code": "QE03",
"title": "Social exclusion or rejection"
},
{
"code": "6B04",
"title": "Social anxiety disorder"
},
{
"code": "6A01.22",
"title": "Developmental language disorder with impairment of mainly pragmatic language"
},
{
"code": "QE04",
"title": "Target of perceived adverse discrimination or persecution"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Social withdrawal (MB23.Q)】
Definition: Retreat from relationships and other social interactions
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Mental or behavioural symptoms, signs or clinical findings → Symptoms or signs involving appearance or behaviour (MB23) → Social withdrawal
【2. Social exclusion or rejection (QE03)】
Definition: Exclusion and rejection on the basis of personal characteristics such as physical appearance, sexual orientation, gender identity and expression, illness or behaviour.
Synonyms: Social deprivation | Social exclusion | Social isolation | Social rejection
Excludes: Target of perceived adverse discrimination or persecution
Hierarchy: Factors influencing health status or contact with health services (24) → Factors influencing health status → Problems associated with social or cultural environment → Social exclusion or rejection
【3. Social anxiety disorder (6B04)】
Definition: Social anxiety disorder is characterised by marked and excessive fear or anxiety that consistently occurs in one or more social situations such as social interactions (e.g. having a conversation), doing something while feeling observed (e.g. eating or drinking in the presence of others), or performing in front of others (e.g. giving a speech). The individual is concerned that he or she will act in...
Synonyms: social phobia | Social neurosis | Anthropophobia | fear of strangers | Gynaephobia
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Anxiety or fear-related disorders → Social anxiety disorder
【4. Developmental language disorder with impairment of mainly pragmatic language (6A01.22)】
Definition: Developmental language disorder with impairment of mainly pragmatic language is characterised by persistent and marked difficulties with the understanding and use of language in social contexts, for example making inferences, understanding verbal humour, and resolving ambiguous meaning. These difficulties arise during the developmental period, typically during early childhood, and cause significan...
Synonyms: Social pragmatic communication disorder | Social communication disorder
Excludes: Diseases of the nervous system | Selective mutism
Hierarchy: Neurodevelopmental disorders → Developmental speech or language disorders (6A01) → Developmental language disorder (6A01.2) → Developmental language disorder with impairment of mainly pragmatic language
【5. Target of perceived adverse discrimination or persecution (QE04)】
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
Synonyms: Exposure to discrimination | Ethnic discrimination | Political discrimination | Racial discrimination | Religious discrimination
Excludes: Social exclusion or rejection
Hierarchy: Factors influencing health status or contact with health services (24) → Factors influencing health status → Problems associated with social or cultural environment → Target of perceived adverse discrimination or persecution
|
MB23.Q
|
Social withdrawal
|
Our patient was a 27-year-old South Asian woman working as an intern medical officer. She is a teetotaller and nonsmoker, who was otherwise previously healthy, and has no significant family or social history of medical relevance. She presented with fever of 2 days’ duration associated with arthralgia and myalgia, for which she had taken only acetaminophen 1 g on an as-needed basis. On initial evaluation, she was febrile to touch, with a temperature of 100.6 °F. Her blood pressure on admission was 110 mmHg systole and 70 mmHg diastole, with a pulse rate of 96 beats per minute. A thorough general and systemic examination failed to elicit any other significant findings. DF was suspected and confirmed with a positive NS1 (nonstructural protein 1) antigen test on the second day. She was managed in accordance with national guidelines with precise fluid replacement, both orally and intravenously, with 0.9% normal saline. In addition to fluids, the only other medication administered was acetaminophen 1 g as needed based on her febrile state, which was stopped upon defervescence on day 4. Her vital signs and clinical indicators of perfusion (for example, pulse rate, blood pressure, capillary refill time, and urine output) were monitored and remained within acceptable reference ranges. Her initial complete blood count (CBC) on admission revealed hemoglobin (Hb) of 12.8 g/dl (normal range, 12–17.5) with hematocrit (HCT) of 34.4% (36–50%) and white blood cell count (WBC) of 4.59 × 10 9 /L (4–11 × 10 9 /L), a predominant neutrophilic differential of 83% (40–75%), and an initial platelet count of 186 × 10 9 /L (150–450 × 10 9 /L) (Table 1 ). Her CBC and HCT were monitored 6-hourly. On the second day, her baseline liver function tests revealed aspartate aminotransferase (AST) of 51 U/L (normal range, 10–35) and alanine aminotransferase (ALT) of only 34 U/L (10–40), but she complained of abdominal pain and significant nausea. Repeated assessment of liver function on day 5 revealed markedly elevated liver enzymes with AST of 1215 U/L (normal range, 10–35) and ALT of 630 U/L (10–40), which rose on day 6 to 1872 U/L and 1145 U/L, respectively (Table 1 ). Her total bilirubin remained normal at 17 mmol/L (normal range, 5–21). Her international normalized ratio remained within reference range, measuring 1.09, with a normal prothrombin time of 13.3 seconds (normal range, 10–14). Her activated partial thromboplastin time, however, was mildly prolonged at 45.4 seconds (normal range, 24–38) on day 6, rising to 57.6 seconds on day 7 (Table 1 ). An electrolyte profile was carried out and did not revealed gross abnormality. Her ionized calcium level was normal at 1.14 mmol/L (normal range, 1.12–1.32), serum potassium was 3.8 mmol/L (3.5–5.1), and sodium was mildly low at 133 mmol/L (135–148). Though her platelet counts declined, her packed cell volume and hemoglobin remained within acceptable stable parameters and together with her urine output did not indicate the onset of the leakage phase or suggest occult hemorrhage. On day 7, whole-blood analysis demonstrated Hb of 14.2 g/dl (normal range, 11–16) with HCT of 39% (37–54) and WBC of 3.35 × 10 9 /L (4–10 × 10 9 /L). The lowest documented platelet count was 27 × 10 9 /L (150–450 × 10 9 /L) (Table 1 ). Despite this, her clinical and other hematological parameters remained normal. The result of urinalysis was normal. On the eighth day of illness, upon waking up in the morning, the patient complained of blurred vision in both eyes. A bedside assessment revealed that the left eye detected only gross movements and the right eye had a visual acuity (VA) of 6/9. An urgent ophthalmology consult was obtained. Ocular examination with dilated funduscopy and fluorescein angiography showed that the patient had subretinal fluid collections at the macular region with retinal nerve fiber layer hemorrhages in the papulomacular bundle area in the left eye and areas of hemorrhage in the right eye as well. The right eye also showed mild subretinal fluid at the macula. Optical coherence tomography (OCT) and macular scans were used to assess the patient’s baseline status, which showed increased retinal thickness centering on the foveal region (more severe in the left eye) and elevation of the retinal pigment epithelial layer with collection of fluid with changes compatible with central serous choroid retinopathy in the left eye and foveolitis in the right eye . Repeat liver function tests on day 8 showed declining liver enzyme values (Table 1 ). Incidentally, her whole-blood analysis showed rising counts with WBC of 3.77 × 10 9 /L (normal range, 4–10 × 10 9 /L) and platelet value of 34 × 10 9 /L (150–450 × 10 9 /L) (Table 1 ), heralding recovery. A focused ultrasonographic study failed to reveal evidence of fluid leakage in the thoracic or abdominal cavities. However, her visual deficit remained. As treatment for the eye manifestations, she received a single 2-mg intravitreal triamcinolone injection into the right eye and 3 days of intravenous methylprednisolone 1 g following an ophthalmology consult. Table 1 Blood cell counts and liver enzyme levels during hospital stay Day 2 Day 5 Day 6 Day 7 Day 8 Day 9 Day 12 Whole-blood analysis White cell count (reference range 4–11 × 10 9 ) 4.59 3.35 3.77 18 Platelet count (reference range 150–450 × 10 9 ) 186 27 34 336 Liver function Aspartate aminotransferase (reference range 10–35 U/L) 51 1215 1872 1020 531 Alanine aminotransferase (reference range 10–40 U/L) 34 630 1145 805 617 Activated partial thromboplastin time (reference range 28–34 seconds) 45.4 57.6 Fig. 1 a and b are photo of both fundi. a is that of the right eye. The fovea (indicated by circle) appears relatively pale compared to that of what’s normal which is typical of dengue foveolitis. Additionally in the outer rim- temporally indicated by the arrow there are signs if resolving haemorrhages. b is that of the left eye. The faint circular light reflexion (indicated by the circle) centering around the fovea. This is the margin of retinal pigment epithelium (RPE) elevation. This demarcates the area of central serous chorioretinopathy (CSCR). Haemorrgahes are also observed. c and d are fourescein angiogras of both the right and left eye respectively. In d , the left eye the center of the macula and fovea is dark. There is a mild fluorecscnce of the fovea. The white halo clearly defines where the where the sub-retinal pigmental epithelium (RPE) fluid is present Fig. 2 a and b Optical coherence tomography (OCT) of both eyes demonstrates retinal region involved. a Right eye. Increased thickness in the foveal region is demonstrated by the orange to pink hue. b Left eye. More marked involvement with a greater area affected is demonstrated by the pink and reddish hue centered on the fovea. Normal retinal thickness is < 320 μm within the green color spectrum, and the foveal thickness is usually 250 μm. c – f Macular scans concentrating on the region described in the OCT scans. c and d Horizontal cuts. e and f Vertical cuts. c and e Right eye. d and f Left eye. In both the horizontal ( d ) and vertical ( f ) cuts of the left eye, the foveal depression is lost and instead is elevated. The retinal pigmental epithelial layer is lifted up, but not just at the fovea. Almost the whole macula is lifted up. The gap below the RPE is dark, suggesting the presence of fluid. This is a typical appearance of “central serous choroidoretinopathy.” In the right eye, depicted in c and e , elevation (second line from the bottom) is seen at the level of the fovea with a reflective material filling the space, suggestive of foveolitis
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sec[1]/p[0]
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| 0.999996
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|
[
"range",
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[
{
"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"
},
{
"code": "BD11.1",
"title": "Left ventricular failure with mid range ejection fraction"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Other specified examination of eyes or vision (QA00.6Y)】
Synonyms: No Impairment of Contrast vision | Normal colour vision | No Impairment of Dark adaptation | No diplopia | No visual spatial neglect
Hierarchy: Contact with health services for purposes of examination or investigation → General examination or investigation of persons without complaint or reported diagnosis (QA00) → Examination of eyes or vision (QA00.6) → Other specified examination of eyes or vision
【2. Neutropaenia, unspecified (4B00.0Z)】
Synonyms: Neutropenia | Disorders with decreased neutrophil counts | neutropaenic disorder | neutrophil count below reference range | absence of neutrophils
Hierarchy: Immune system disorders involving white cell lineages → Disorders of neutrophil number (4B00) → Neutropenia (4B00.0) → Neutropaenia, unspecified
【3. Acquired thrombocytosis, unspecified (3B63.1Z)】
Synonyms: Acquired thrombocytosis | Idiopathic haemorrhagic thrombocythaemia | Essential thrombocythaemia | primary haemorrhagic thrombocythaemia | idiopathic thrombocythaemia
Hierarchy: Coagulation defects, purpura or other haemorrhagic or related conditions → Thrombocytosis (3B63) → Acquired thrombocytosis (3B63.1) → Acquired thrombocytosis, unspecified
【4. Raised antibody titre (MA14.1C)】
Synonyms: antibody titre above reference range | high antibody titre | increased antibody titre
Excludes: isoimmunization, in pregnancy affecting fetus or newborn
Hierarchy: Clinical findings in blood, blood-forming organs, or the immune system → Immunological findings in blood, blood-forming organs, or the immune system (MA14) → Certain specified immunological findings (MA14.1) → Raised antibody titre
【5. Left ventricular failure with mid range ejection fraction (BD11.1)】
Synonyms: HFmEF - [heart failure with mid range ejection fraction] | Left ventricular failure with mid range ejection fraction due to cardiomyopathy | Left ventricular failure with mid range ejection fraction due to coronary artery disease | Left ventricular failure with mid range ejection fraction due to myocarditis | Left ventricular failure with mid range ejection fraction due to other disorders
Hierarchy: Diseases of the circulatory system (11) → Heart failure → Left ventricular failure (BD11) → Left ventricular failure with mid range ejection fraction
|
QA00.6Y
|
Other specified examination of eyes or vision
|
Various recent studies have investigated the diagnostic value of FDG-PET/CT in diagnosing cyst infections. For example, Sallée et al. described 8 patients in whom a cyst infection was diagnosed based on FDG-PET/CT. Five patients had a definite cyst infection based on cyst aspiration, and three patients a probable cyst infection based on the presence of all of the following five factors: fever, abdominal pain, increased CRP, the absence of cyst bleeding and the absence of any other possible cause of fever. In all 8 patients, FDG-PET/CT results were positive for infection . Fig. 1 A 61-year-old man with ADPKD and unilateral renal agenesis was admitted to the hospital because of a possible urinary tract infection. He presented with complaints of cold shivers, pollakiuria, stranguria, lower abdominal pain and cloudy urine. Blood tests showed a CRP level of 363 mg/L and a white blood cell count of 6.6 × 10 9 /L, while a urine culture tested positive for Escherichia coli . The patient’s clinical condition improved under ciprofloxacin treatment, but his CRP level remained high 5 days later at 154 mg/L. Because of the possibility of pyelonephritis or cyst infection, ultrasound was ordered. Ultrasonographic images of the liver ( a ) and kidney ( b ) showed multiple cysts, but no hydronephrosis and no potential site of infection. Subsequently, FDG-PET/CT was performed. Axial FDG-PET ( c ), low-dose CT ( d ), fused FDG-PET/CT ( e ), and coronal maximum intensity projection FDG-PET ( f ) showed pathologic FDG uptake of the wall of a cyst in the right kidney (arrows), consistent with cyst infection. No pathologic FDG-avid foci were detected elsewhere. The patient was discharged 1 day after the FDG-PET/CT scan in good clinical condition, where he continued his course of ciprofloxacin for 10 more days Fig. 2 A 53-year-old man with ADPKD presented at the emergency department because of abdominal pain, fever and a possible urinary tract infection. He had received a kidney transplant 1 month before because of end-stage renal disease. Blood tests showed a CRP level of 19 mg/L and a white blood cell count of 8.6 × 10 9 /L, while a urine culture revealed Gram-negative rods. The patient was admitted, and an FDG-PET/CT scan was ordered to assess the native kidneys for infection. Coronal maximum intensity projection FDG-PET ( a ), low-dose CT ( b ), and fused FDG-PET/CT ( c ) did not show any signs of (cyst) infection in the native kidneys, and no pathologic FDG-avid foci elsewhere. FDG-PET/CT findings provided confidence to the clinicians to attribute the clinical and laboratory findings to a simple cystitis. The patient was sent home in good clinical condition with a course of nitrofurantoin for 3 days Fig. 3 A 68-year-old woman with ADPKD presented with a feeling of general malaise that had been increasing over the course of 2 weeks. Nocturnal sweats were also present in the last week. There was no fever or abdominal pain. Blood tests showed a CRP level of 242 mg/L and a white blood cell count of 15.8 × 10 9 /L. Blood cultures were positive for Escherichia coli and urine cultures were positive for Klebsiella pneumoniae . The patient was admitted with a differential diagnosis of urosepsis or an infection of her abdominal aortic endograft. Despite intravenous treatment with cefuroxime for 4 days, infectious parameters remained high (CRP level of 228 mg/L). To find the source of infection, a contrast enhanced CT scan of the abdomen was ordered. Axial arterial phase ( a ) and portal-venous phase ( b ) full-dose CT scans showed multiple liver cysts that were otherwise unremarkable. Because CT failed to identify any site of infection, an FDG-PET/CT scan was ordered. Axial FDG-PET ( c ) fused FDG-PET/CT ( d ), and coronal maximum intensity projection FDG-PET ( e ) showed pathologic FDG uptake of the wall of a cyst in segment seven of the liver (arrows), in keeping with cyst infection. No pathologic FDG-avid foci were detected elsewhere. The antibiotic regimen was changed to ceftriaxone for better cyst penetration. CRP levels decreased to 49 mg/L in 4 days. The patient was sent home in good condition with a subsequent course of ciprofloxacin Fig. 4 A 71-year-old woman presented to the rheumatology and immunology department because of fatigue, general malaise and involuntary weight loss of 9 kg in 5 months. She did not have fever or abdominal pain. Blood tests showed a CRP level of 256 mg/L and a white blood cell count of 11.0 × 10 9 /L. An FDG-PET/CT scan was ordered to assess the possibility of vasculitis, infection, or malignancy. Axial FDG-PET ( a ), low-dose CT ( b ), and fused FDG-PET/CT ( c ) showed an abnormal spleen (arrows) that was markedly enlarged with inhomogeneous areas of pathologic FDG uptake and central photopenic areas that could not be clearly assessed on the low-dose CT ( b ). Coronal FDG-PET ( d ), low-dose CT ( e ), and fused FDG-PET/CT ( f ) demonstrated similar findings (arrows). No pathologic FDG-avid foci were detected elsewhere. Based on the laboratory and FDG-PET/CT findings, splenic cyst infection was strongly considered, although other conditions (in particular malignancy) could not be excluded. MRI was ordered for further assessment of the spleen. Coronal T2-weighted ( g ) and gadolinium-enhanced fat-suppressed T1-weighted ( h ) images showed a markedly enlarged and inhomogeneous spleen (arrows) with cyst-like changes, and centrally both T2 hypointense and hyperintense non-enhancing components. The differential diagnosis based on the MRI examination included hemangioma with hemorrhage, infected splenic cyst with abscess formation, and angiosarcoma. Splenectomy was performed 1 day later, which demonstrated angiosarcoma with central hemorrhagic areas Fig. 5 A 62-year-old woman was admitted to the department of internal medicine because of abdominal pain and fever. A couple of weeks before, she was diagnosed with a large obstructing right ureteral stone, for which a double-J catheter was placed. Blood and urine samples were taken, which both tested positive for Proteus mirabilis . The CRP level was 65 mg/L and the white blood cell count was 12.4 × 10 9 /L. The working diagnosis was urosepsis and the patient was started on ciprofloxacin and amoxicillin/clavulanic acid, which was later switched to meropenem. Despite antibiotic treatment, high infectious parameters (CRP level of 101 mg/L and white blood cell count of 13.8 × 10 9 /L) and fever remained. To assess for any underlying cause of infection, an FDG-PET/CT scan was ordered. Axial and coronal FDG-PET ( a , d ), low-dose CT ( b , e ) and fused FDG-PET/CT ( c , f ) show an enlarged contour of the right kidney that appeared cystic, but with only slightly increased FDG uptake (arrows). Because of the slightly increased FDG uptake, infection was deemed unlikely. No pathologic FDG-avid foci were detected elsewhere. Axial ( g ) and h portal-venous phase full-dose CT scans showed a multi-cystic lesion in the cortex of the right kidney (arrows), with a differential diagnosis that included cyst infection and cystic neoplasm. Also note normal pyelocaliceal FDG accumulation in the right kidney (arrowheads no. 1 in a , c , d , and f ) and the double-J catheter (arrowheads no. 2 in b , e , g , and h ). Because the patient had already been treated with high-dose intravenous antibiotics for a long time without clinical improvement, a nephrectomy was performed, which demonstrated a necrotizing and suppurative infection of multiple cysts, without any signs of malignancy. The patient was discharged from the hospital in good clinical condition shortly afterwards
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| 0.999997
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29568734
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https://doi.org/10.1007/s40336-017-0261-8
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[
"infection",
"cyst",
"blood",
"dose",
"level",
"pathologic",
"fever",
"abdominal"
] |
[
{
"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 CLASSIFICATION ═══
【1. Infection, unspecified (1H0Z)】
Synonyms: infection NOS | infectious disease NOS | infection unknown | infection process NOS | infection by unspecified organism and of unspecified site
Hierarchy: Certain infectious or parasitic diseases (01) → Infection, unspecified
【2. Sepsis without septic shock (1G40)】
Definition: Sepsis is defined as a life-threatening organ dysfunction caused by a dysregulated host response to infection.
Synonyms: sepsis without septic shock with known organism | Sepsis-associated hypotension | Unspecified sepsis | general septic intoxication | septic intoxication
Excludes: Septicaemia | Sepsis of fetus or newborn
Hierarchy: Certain infectious or parasitic diseases (01) → Sepsis → Sepsis without septic shock
【3. Direct infections of joint, unspecified (FA10.Z)】
Synonyms: Direct infections of joint | septic arthritis | pyogenic arthritis | arthritis due to infection | joint infection
Hierarchy: Arthropathies → Infection related arthropathies → Direct infections of joint (FA10) → Direct infections of joint, unspecified
【4. Unspecified viral infection of unspecified site (1D9Z)】
Synonyms: viral infection NOS | viral disorder NOS | disease caused by virus | unspecified viremia | Viraemia NOS
Hierarchy: Certain infectious or parasitic diseases (01) → Certain other viral diseases → Viral infection of unspecified site → Unspecified viral infection of unspecified site
【5. Infectious gastroenteritis or colitis without specification of infectious agent (1A40.Z)】
Synonyms: Gastroenteritis or colitis without specification of infectious agent | diarrhoea and gastroenteritis of presumed infectious origin | diarrhoeal enteritis | GE - [gastroenteritis] | infectious diarrhoea NOS
Hierarchy: Certain infectious or parasitic diseases (01) → Gastroenteritis or colitis of infectious origin → Gastroenteritis or colitis without specification of infectious agent (1A40) → Infectious gastroenteritis or colitis without specification of infectious agent
|
1H0Z
|
Infection, unspecified
|
This patient was a 42-year-old man who had been admitted to several hospitals before being transferred to our hospital for further care. Acute epigastric pain was his initial complaint, and laboratory findings from nearby hospitals, which included routine blood tests, ALP, amylase, transaminase and creatinine, were normal (Table 1 ). Computed tomography revealed a small amount of retroperitoneal exudation around the body of the pancreas. A combination of a proton pump inhibitor, anisodamine and tramadol was administered, but his symptoms recurred. Six days after onset, he visited another hospital for further care. His alanine transaminase and γ-GGT levels were elevated (118 U/L and 146 U/L, respectively), but his amylase and lipase levels were normal. Because peripancreatic exudation was observed on the CT scan, he was admitted with the diagnosis of acute pancreatitis. After admission, his creatinine level gradually increased (6 days after onset: 89.9 μmol/l; 7 days after onset: 93.9 μmol/l), and the urinary sediment examination showed the following: urinary protein: 2+; and hematuria: 0–2/μL. Nine days after onset, he complained of abdominal colic pain and had diarrhea, and new ascites was found on the plain abdominal CT scan. Eleven days after onset, enhanced CT revealed that the amount of ascites had increased. Fourteen days after onset, he began to have progressive oliguria. Twenty-one days after onset, his illness was aggravated, with a urine output of approximately 200 ml/d, a CRP level of 137 mg/L, a creatinine level of 275 μmol/l, an albumin level of 23.2 g/L and an ALP level of 202 U/L. Therefore, he was transferred to a third hospital that specializes in kidney disease. He began treatment with intermittent bedside hemodiafiltration because of water overload and symptoms of heart failure 24 days after onset. Twenty-five days after onset, he began to develop a fever, with a maximum temperature of more than 39 degrees. His hemoglobin and platelet counts were normal on admission but gradually decreased as low as 67 g/l and 10 × 10 9 /l, respectively, after admission. Pathogen detection tests, including blood culture and the atypical pathogens and G/GM test, were negative. Hepatitis B virus (HBV) and hepatitis C virus (HCV) were negative. HIV antibody and HHV-8 PCR were negative. The polyclonal light chains in both the blood and urine were increased. The levels of serum kappa and lambda light chain were 170 mg/l (3.30–19.40) and 208.25 mg/l (5.71–26.3), respectively (kappa/lambda: 0.82). However, the levels of IgG and IgA were within the normal range, and the levels of IgM and complement 3/4 were below normal. Antibodies, including ANA, RF, ANCA, and platelet-associated IgG, were undetectable. His serum VEGF and IL-6 levels were > 800 pg/ml (0.0–142.0) and 11.5 ng/ml (0.0–7.0), respectively. An abdominal puncture was performed, and 1000 ml of ascites fluid was drained 40 days after onset. The total number of cells in ascites was 121 and included 40 nucleated cells (34 mononuclear cells and 6 multinuclear cells). Total protein, albumin and glucose levels in ascites were 28.0 g, 14.4 g/l and 14.96 mmol/l, respectively. The IL-6 level in ascites reached 1022 pg/ml (0.0–7.0). Bone marrow biopsy showed marked hyperplasia of granulocytes and megakaryocytes, and reticular fiber staining was negative. PET/CT showed that FDG activity was increased in the bilateral neck, bilateral axillary areas, mediastinum, bilateral hilar lymph nodes, bilateral parailiac artery, and bilateral inguinal lymph nodes and did not reveal any carcinoma. Pathology of the lymph node puncture showed plasmacytosis. After ineffective antibiotic treatment with meropenem, he was given intravenous methylprednisolone (60 mg qd) 36 days after onset combined with intravenous immunoglobulin (20 g qd for 5 days). At the early stage of glucocorticoid therapy, his pleural effusion and ascites decreased significantly, accompanied by a general improvement. However, 46 days after onset, his temperature increased again, with a mild increase in bilirubin and a significant decrease in blood cells. Therefore, lymphadenectomy was performed, and histopathology showed a massive hemorrhage in the lymph nodes with a normal lymph node structure, decreased lymphoid follicles, an atrophied germinal center, a concentric circle arrangement in the partial mantle zone, the proliferation of small vessels, enlargement of the paracortical zone, and flaky or focal infiltration of mature plasma cells. These findings are consistent with the mixed histopathological subtype of iMCD. After a multidisciplinary consultation, the patient was diagnosed with iMCD and was believed to have hemophagocytic syndrome according to the decreased NK cell activity (14.09%) and increased soluble CD25 level (10,615 pg/ml). For further care, he was referred to our hospital which was famous for being specialized in hematology. Based on his clinical manifestations (fever, ascites, thrombocytopenia, lymphadenopathy and progressive renal insufficiency) and lymph node biopsy (atrophied germinal center and the proliferation of small vessels), we diagnosed the patient with TAFRO syndrome. As the CMV DNA contents of the serum and lymphocytes were increased (4.76 × 10 4 cp/ml and 4.25 × 10 6 cp/ml, respectively) 53 days after onset, ganciclovir was added to his treatment plan. With an improvement in renal function, hemodiafiltration was stopped 2 months after onset. However, proteinuria was still serious, with 24-h urine protein reaching 31 g, so renal biopsy was performed 3 months after onset. Histopathology revealed 11 glomeruli in which a membranoproliferative glomerulonephritis (MPGN) pattern was observed, with diffuse endocapillary proliferation, mesangial proliferation, fuchsinophilic protein deposition in the subendothelial and mesangial areas by Masson stain, double contours and microthrombosis seen in some capillary loops, vacuole and granular degeneration of renal tubular epithelial cells, and arterial endothelial cell proliferation with thickened walls and a narrowed lumen. Immunofluorescence showed two glomeruli with immune complex deposition (IgA ++, IgG +++, IgM ++, C1q ++, C3 +++, FRA -, Kappa ++, Lambda ++), which were deposited with clumps and granules along the mesangial zero and capillary wall. The IgG subtype detection of immune complexes showed IgG1 ++, IgG2 ++, IgG3 and IgG4 -. Electron microscopy revealed intramembranous, subendothelial and partially subepithelial electron-dense deposits and diffuse podocyte foot processes . His renal histopathology were consistent with those of cases with TAFRO syndrome reported in literatures, which further confirmed his diagnosis of TAFRO syndrome. One hundred twenty days after onset, therapy with intravenous rituximab (CD20 B-cell monoclonal antibody, from Roche) was initiated (375 mg/m 2 once a week for the first month and then once evert 2 months). The patient has not been hospitalized since the fifth course of treatment. At the latest follow-up, his hemoglobin level was 133 g/L, his platelet level was 309 × 10 9 /L, his creatinine level was 103 μmol/L, and his body temperature and urine volume were normal . Fig. 4 Renal histopathological findings of case 2. a Light microgram × 400 periodic acid silver-methenamine (PASM) stain. b Light microgram × 200 Masson stain. c Electron micrograph × 5000 Fig. 5 Changes in clinical indicators over time in case 2. WBC: white blood cell. HGB: hemoglobin. Plt: platelet. T: temperature. CRP: C-reactive protein. APTT: activated partial thromboplastin time. ALB: albumin. CRE: creatinine
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|
sec[1]/sec[1]/p[0]
|
en
| 0.999998
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33225930
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https://doi.org/10.1186/s12882-020-02119-7
|
[
"onset",
"level",
"ascites",
"levels",
"increased",
"cells",
"respectively",
"lymph"
] |
[
{
"code": "MA81",
"title": "Speech dysfluency"
},
{
"code": "6A01.1",
"title": "Developmental speech fluency disorder"
},
{
"code": "4A42.0",
"title": "Paediatric onset systemic sclerosis"
},
{
"code": "8A68.Z",
"title": "Type of seizure, unspecified"
},
{
"code": "5C51.3",
"title": "Glycogen storage disease"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Speech dysfluency (MA81)】
Definition: 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 limits of normal variation and results in reduced intelligibility and significantly affects communication. It can involve repetitions of sounds, syllables or words, prolongations, word breaks, blockage of...
Synonyms: speech impediment NOS | Adult onset cluttering | Adult onset stammering | Adult onset stuttering
Excludes: Developmental language disorder | Developmental speech or language disorders | Developmental speech fluency disorder | Dysarthria | Selective mutism
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings of speech, language or voice → Symptoms or signs involving speech, language or voice → Speech dysfluency
【2. Developmental speech fluency disorder (6A01.1)】
Definition: Developmental speech fluency disorder is characterised by frequent or pervasive disruption of the normal rhythmic flow and rate of speech characterised by repetitions and prolongations in sounds, syllables, words, and phrases, as well as blocking and word avoidance or substitutions. The speech dysfluency is persistent over time. The onset of speech dysfluency occurs during the developmental period...
Synonyms: Speech fluency disorder | Developmental neurogenic stuttering | childhood onset stuttering | developmental onset stuttering | stuttering during developmental period
Excludes: Tic disorders
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Neurodevelopmental disorders → Developmental speech or language disorders (6A01) → Developmental speech fluency disorder
【3. Paediatric onset systemic sclerosis (4A42.0)】
Definition: Systemic sclerosis arising before the age of 16. Involvement of internal organs is less common but arthritis and myositis are more common than in adults.
Synonyms: Diffuse paediatric systemic sclerosis | Limited paediatric systemic sclerosis
Hierarchy: Diseases of the immune system (04) → Nonorgan specific systemic autoimmune disorders → Systemic sclerosis (4A42) → Paediatric onset systemic sclerosis
【4. Type of seizure, unspecified (8A68.Z)】
Synonyms: Types of seizures | uncontrolled seizures | Seizure NOS | fits NOS | onset seizure NOS
Hierarchy: Diseases of the nervous system (08) → Epilepsy or seizures → Types of seizures (8A68) → Type of seizure, unspecified
【5. Glycogen storage disease (5C51.3)】
Definition: The term Glycogen storage disease characterises a group of heterogeneous diseases resulting from defects in the process of glycogen synthesis or breakdown within muscles, liver, and other cell types.
Synonyms: Glycogenosis | GSD - [Glycogen storage disease] | glycogen thesaurismosis | diffuse glycogenosis | generalised glycogen storage disease
Hierarchy: Metabolic disorders → Inborn errors of metabolism → Inborn errors of carbohydrate metabolism (5C51) → Glycogen storage disease
|
MA81
|
Speech dysfluency
|
A female newborn, second child of healthy and nonconsanguineous parents, was born at term by elective caesarean delivery. Due to increased age of the mother (35 year-old), a non-invasive prenatal testing through cell free fetal DNA analysis on maternal blood was performed in the first trimester of gestation, showing no chromosomal abnormalities. Further invasive genetic analyses (chorionic villus sampling and amniocentesis) were refused by parents. Prenatal ultrasound (US) investigations disclosed no anomalies until the third trimester of pregnancy (32 weeks), when severe polyhydramnios along with narrow bitemporal diameter and frontal bossing were observed. Then, a fetal magnetic resonance imaging was carried out, confirming the alterations already documented by US, and revealing no further defects. Apgar scores were 7 and 9, at 1 and 5 min respectively. At birth, anthropometric measures were as follows: weight 3300 g (61 st centile, + 0.28 standard deviations, SD), length 45 cm (2 nd centile, -2.08 SD) and occipitofrontal circumference (OFC) 33 cm (23 rd centile, -0.74 SD). Postnatally, due to respiratory distress, she underwent non-invasive ventilation support through nasal continuous positive airway pressure (CPAP), suspended about 24 h later. On the fifth day of life, owing to feeding difficulties and biliary vomiting, along with characteristic facial features and cleft palate, the newborn was transferred to our Neonatal Intensive Care Unit from a first level birthing center. At admission, physical examination showed frontal and parietal bossing, narrow bitemporal diameter, low anterior hairline, facial asymmetry due to left hypoplasia, hypertelorism, epicanthal folds, palpebral ptosis of the left eye, horizontal palpebral fissures, prominent nose, long philtrum and thin lips . Dysplastic, low-set and posteriorly rotated ears and microretrognathia completed her craniofacial profile. Cleft of the secondary palate, rhizomelic shortening of lower limbs and bilateral sandal gap deformity between the first and second toe were also observed. Neurological examination documented normal findings. The clinical course was characterized by inspiratory tirage during crying with short hypoxemia episodes, for which a few days oxygen supplementation was needed. X-Ray investigation and then barium enema (with 50% dilution of sodium and meglumine amidotrizoate used as contrast medium) were also performed, due to feeding difficulties, persistent biliary gastric secretions and abdominal distension. Distension of rectum and sigma, poor opacification of the remaining colon sections, which appeared twisted and displaced to the left, together with gas distension of the remaining intestinal loops shifted to the right were detected. Therefore, a laparotomy was performed, which revealed dilated and left positioned transverse and ascending colon, and the cecum located in mesogastrium, according with intestinal malrotation. A possible duodenal stenosis was ruled out. Postoperative evolution was regular, with progressive increase and tolerance of enteral nutrition. Laboratory analyses including complete blood count, serum electrolytes, liver and kidney function tests showed normal results, as well as head, heart and abdominal ultrasound (US), with the exception of duplicated gallbladder. Ophthalmological evaluation documented no abnormalities. Conversely, hearing screening through transient evoked otoacoustic emissions (TEOAE) revealed abnormal results. In order to ascertain and characterize the hearing loss, an audiological assessment was started. It included serial auditory brainstem response (ABR) evaluations at 2 and 4 months of age, which detected right response threshold at 70 dB (decibel) HL (hearing level) and left one at 60 dB, according with moderate hypoacusis, which has not required any treatment to date. Limbs X-ray, carried out during the first month of life, identified femur hypoplasia (81 mm in length, the same as for tibia, with 1:1 ratio), according with the pediatric radiology criteria for lower-limb abnormalities (i.e., the normal difference between the femur and tibia length should be 12 mm at birth, with the tibia remaining at a constant length of 80% of femoral length throughout growth ), and no other abnormalities. Cranial computed tomography scan, later performed, showed no alterations, ruling out craniosynostosis (cephalic index 81, obtained as transverse diameter [TD]/anteroposterior diameter [APD] × 100, normal values 75–90; APD 137 mm, TD 111 mm). Array comparative genomic hybridization (a-CGH) was performed and detected no genomic rearrangements. Then, the NGS analysis of a panel of genes involved in skeletal malformations and neurodevelopmental disorders was carried out. A heterozygous nonsense mutation of the AMER1 gene was found, causing a premature stop codon at position 358 (p.Arg358Ter) of the encoded protein, out of a total of 1136 amino acids (condition A among the pathogenicity criteria of the American College of Medical Genetics, ACMG). This variant has been reported in literature as pathogenic in different affected female subjects and in a male fetus . It is extremely rare, and allelic frequency information in the dedicated population database is not available (condition G of ACMG); moreover, it is localized in a moderately conserved nucleotide position (phyloP-Vertebrate = 0.17/6.42; phyloP-Primate = 0.53/0.65; PhastCons = 0.95/1.00) (condition K). Gene sequencing was then extended to parents, who showed normal results, confirming the de novo origin of the genomic abnormality (condition C). Based on the clinical and genetic findings (the latter strengthened by the presence of the aforementioned pathogenicity criteria, necessary for the definition of a variant as pathogenic ), an OS-CS diagnosis was made. The infant was discharged at 2 months of age in good general conditions, despite poor weight and length growth, and included in a multidisciplinary (audiological/otolaryngological, ophthalmological, surgical, neurodevelopmental, orthopedic) follow-up. She is currently 9 months and 10 days old, she presents severe growth failure—according to World Health Organization growth chart for neonatal and infant close monitoring —and relative macrocephaly compared to the other anthropometric measures: weight 6490 g (2 nd centile, -2.06 SD), length 65 cm (1 st centile, -2.29 SD) and OFC 44.5 (66 th centile, + 0.40 SD). The patient has normal muscular tone and reflexes, and developmental delay due to language and cognitive impairment. She can stand upright with support, turn her head, follow and reach objects (red cube and suspended red ring put on the midline) with both her hands. However, no lallation or attribution of meaning to gestures are currently present. She is enrolled in a specific habilitation treatment including logopedic therapy, and the surgical correction of cleft palate is already planned. She presently shows no further abnormalities. Fig. 1 a Patient’s front view: frontal bossing, narrow bitemporal diameter, low anterior hairline, facial asymmetry due to left hypoplasia, hypertelorism, epicanthal folds, palpebral ptosis of the left eye, horizontal palpebral fissures, prominent nose, long philtrum and thin lips. b Lateral view: parietal bossing, dysplastic , low-set and posteriorly rotated ear, microretrognathia Fig. 2 a Cleft of the secondary palate; b Sandal gap deformity between the first and second toe Fig. 3 Barium enema: distension of rectum and sigma, and poor opacification of the remaining colon sections which appeared twisting and displaced to the left
| 4.046875
| 0.977539
|
sec[1]/p[0]
|
en
| 0.999998
|
36581928
|
https://doi.org/10.1186/s13052-022-01403-6
|
[
"length",
"centile",
"abnormalities",
"diameter",
"bossing",
"cleft",
"palate",
"palpebral"
] |
[
{
"code": "FA31.8",
"title": "Acquired unequal limb length"
},
{
"code": "DA0A.1",
"title": "Loss of teeth due to accident, extraction or local periodontal disease"
},
{
"code": "KA2Z",
"title": "Disorders of newborn related to length of gestation or fetal growth, unspecified"
},
{
"code": "KA03.2Z",
"title": "Fetus or newborn affected by abnormalities of umbilical cord length, unspecified"
},
{
"code": "KA2Y",
"title": "Other specified disorders of newborn related to length of gestation or fetal growth"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Acquired unequal limb length (FA31.8)】
Synonyms: unequal length of limbs | unequal limb length | Acquired unequal limb length, multiple sites | Acquired unequal limb length, shoulder region | Acquired unequal limb length, acromioclavicular joint
Hierarchy: Arthropathies → Certain specified joint disorders or deformities of limbs → Other acquired deformities of limbs (FA31) → Acquired unequal limb length
【2. Loss of teeth due to accident, extraction or local periodontal disease (DA0A.1)】
Synonyms: acquired anodontia | Acquired absence of teeth | Acquired absence of single tooth | Acquired absence of multiple teeth | Dental arch length loss due to loss of permanent teeth
Hierarchy: Diseases of the digestive system (13) → Diseases or disorders of orofacial complex → Certain specified disorders of teeth or supporting structures (DA0A) → Loss of teeth due to accident, extraction or local periodontal disease
【3. Disorders of newborn related to length of gestation or fetal growth, unspecified (KA2Z)】
Hierarchy: Certain conditions originating in the perinatal period (19) → Disorders of newborn related to length of gestation or fetal growth → Disorders of newborn related to length of gestation or fetal growth, unspecified
【4. Fetus or newborn affected by abnormalities of umbilical cord length, unspecified (KA03.2Z)】
Synonyms: Fetus or newborn affected by abnormalities of umbilical cord length
Hierarchy: Fetus or newborn affected by maternal factors or by complications of pregnancy, labour or delivery → Fetus or newborn affected by complications of umbilical cord (KA03) → Fetus or newborn affected by abnormalities of umbilical cord length (KA03.2) → Fetus or newborn affected by abnormalities of umbilical cord length, unspecified
【5. Other specified disorders of newborn related to length of gestation or fetal growth (KA2Y)】
Hierarchy: Certain conditions originating in the perinatal period (19) → Disorders of newborn related to length of gestation or fetal growth → Other specified disorders of newborn related to length of gestation or fetal growth
|
FA31.8
|
Acquired unequal limb length
|
A 48-day-old full-term male baby was admitted to our hospital with “growth retardation and persistent vomiting for more than one month”. The parents were nonconsanguineous, the pregnancy and delivery periods were unremarkable. After birth, the child gradually developed symptoms including dark skin, failure to thrive, poor appetite, vomiting gastric contents without bile and coffee grounds, occasional diarrhea without mucus and blood, and was previously treated with “reduced glutathione”, “creatine phosphate”, “calcium gluconate”, “hydrocortisone”, and “metformin” in another hospital for 9 days before transferred to our tertiary hospital. During the treatment, he had no seizures, no screaming, no abdominal distension, and his condition did not alleviate. For further evaluation, his family was readmitted to our hospital. The birth weight of the male infant was 3300 g, but at the time of admission, his weight was 3500 (< 3rd percentile) and length was 61 cm (90th percentile) . On physical examination, the infant was awake and dehydrated. The skin was hyperpigmented and less elastic without subcutaneous fat . His neck was supple. There was no facial dysmorphism noted. His lungs were clear, his heart rate was regular and no murmurs were found on auscultation. His abdomen was not tender, without organomegaly. Genital examination revealed penis scrotal hyperpigmentation and thickened penis, with normal testicles volume (1 ml on each side) . The neurologic examination was unremarkable. Routine laboratory tests revealed the following serum levels: potassium 5.9 mmol/L (normal range, 3.5 to 5.5), sodium 132 mmol/L (normal range, 135 to 145), alanine transaminases 141.1 U/L ( ALT, normal range: 0 to 40), aspartate transaminases 88.8 U/L (AST, normal range, 0 to 40), alkaline phosphatase 50.7 U/L (ALP, normal range, 147.7 to 309.3), creatine kinase 1586 U/L (CK, normal range, 16.5 to 211.5), creatine kinase isoenzyme 168 U/L (CK-MB, normal range, 16.5 to 211.5), α-hydroxybutyrate 314 U/L (α-HBDH, normal range, 75.5–211.5), lactate dehydrogenase isoenzyme 76U/L (LDH-MB, normal range, 30 to 120), lactate dehydrogenase 413U/L (LDH, normal range: 67 to 394.1). No clinically significant findings were noted on chest and abdomen radiography. An ultrasound cardiography revealed no other problems except for Patent Foramen Ovale. Abdominal and urinary ultrasound showed hepatomegaly and bilateral renal enlargement. Based on hyperkalemia, hyponatremia, penis scrotal hyperpigmentation, and his enlarged kidneys and liver, hydrocortisone (5 mg given intravenously every 8 h) and fludrocortisone (0.1 mg administered orally per day) were started or a presumed diagnosis of adrenal cortical hyperplasia (ACH). Glutathione and sodium fructose diphosphate were initiated orally for abnormal liver function and myocardial abnormalities especially. Over the next 3 weeks, the child’s appetite improved and the symptoms of dehydration were reduced. He increased by 0.5 kg in weight. Routine laboratories were re-ordered. The sodium level was 130 mmol/L (normal range, 135 to 145), potassium level 5.1 mmol/L (normal range, 3.5 to 5.5), ALT level 62.8 U/L (normal range, 0 to 40), AST level 53.3 U/L (normal range, 0 to 40), ALP level 147.2 U/L (normal range, 147.7 to 309.3), CK level 621 U/L (normal range, 16.5 to 211.5), CK-MB level 97 U/L (normal range, 15 to 80), LDH level 367.4 U/L (normal range, 67 to 394.1), LDH-MB level 64.9 U/L (normal range, 30 to 120), α-HBDH level 255.2 U/L (normal range, 75.5 to 211.5), 17-alpha hydroxyprogesterone 0.48 ng/L (17-α-OHP, normal range, 0 to 1.54). No lipid metabolism test was performed during the hospitalization. An adrenal function test showed no significant abnormalities (Table 1 ), and the results also contradicted the diagnosis of ACH. We revised the main diagnosis to probable adrenocortical insufficiency. The patient was discharged on day 23 after admission and continued oral treatment with reduced doses of cortisone acetate for relived symptoms, fludrocortisone, and sodium supplementation (Table 1 ). Fig. 1 a and b show the appearance of this boy when admitting to our hospital. His skin was hyperpigmented and hypotonic without subcutaneous fat. c reveals the boy gained weight and his skin color was relatively normal after treatment at 5 months of age and his mother held him on her leg (the photo was cropped) Table 1 Medical history timeline during the first 1.8 years of life and biochemical and hormonal values Chronologic Age(years) 40 + 3 weeks of GA (Birth) 46 + 6 weeks of GA (First hospitalization) 0.17(First discharge) 0.22 (Second hospitalization) 0.27 (Second discharge) 0.39 0.65 0.94 1.36 1.75 (Death) Length(cm) / 61 / 64 / 64.6 68 70 75 76 Weight(Kg) 3.3 3.5 / 4.3 4.8 6.2 8 8.3 9.6 10.4 Head Circumference(cm) / / / / / / / / / / ACTH(7.2–63.6 pg/ml) / / 15.04 8.76 72.83 4.48 3.42 3.86 2.36 / Cortisol(66–630 nmol/L) / / 647.9 874.4 12.72 29.88 11.57 6.41 7.64 / LH(1.5–12.4 IU/L) / / / 7.23 / / / 1.76 0.46 / FSH(0.1–5.4 IU/L) / / / 13.93 / / / 0.73 0.1 / E2(18-73 pmol/L) / / / 18.4 / / / 18.4 57.1 / P(0.7–4.3 nmol/L) / / / 0.1 / / / 0.1 0.65 / T(0.1–1.12 nmol/L) / / / 13.03 / / / 0.09 0.09 / PRL(106–713 mIU/L) / / / 518.3 / / / 131.6 243.6 / 17-α-OHP(0–1.54 ng/ml) / / 0.48 0.52 / / / / / / ALT(0–40 U/L) / 141.1 62.8 115 138 233 153 181 183 439 AST(0–40 U/L) / 88.8 53.3 121 173 253 163 206 212 1078 ALP(147.7–309.3 U/L) / 50.7 147.2 247 252 210 149 155 123 140.8 CK(16.5–211.5 U/L) / 1584 621 2620 6771 11,622 10,680 13,512 9822 26,055.3 CK-MB(15–80 U/L) / 203.1 97 254 343 540 520 538 549 2353 LDH(67–394.1 U/L) / 402.3 367.4 / 617 959 815 956 946 2440.4 LDH-MB(30–120 U/L) / 125.4 64.9 / 88 116 97 101 110 165.2 TCHOL(3.12–5.2 mmol/L) / / / 4.86 5.18 4.76 4.28 4.46 4.53 / α-HBDH(75.5–211.5 U/L) / 314 255.2 300 447 631 545 624 617 1560.9 TG(0.8–1.8 mmol/L) / / / 12.01 8.87 4.76 6.71 6.09 4.53 / Apo-A(1–1.6 g/L) / / / 1.67 1.45 1.68 1.43 1.65 1.74 / Apo-B(0.6–1.1 g/L) / / / 0.6 0.8 0.67 0.6 0.49 0.61 / Na(135–145 mmol/L) / 133 130.4 132 136 139 142 141 143 140.8 K (3.5–5.5 mmol/L) / 4.6 5.1 6.7 6.6 5 4.4 3.7 4.2 5.7 Ca(2.2–2.7 mmol/L) / 2.39 2.33 2.86 2.68 2.62 2.53 2.34 2.32 1.97 Mg(0.6–0.95 mmol/L) / 0.74 0.7 0.81 0.89 0.88 0.79 0.78 0.9 1.26 Cl(96–106 mmol/L) / 110.7 97.6 94 96 101 102 103 101 98 Phosphorus(1.45–2.1 mmol/L) / 1.08 1.54 2.11 2.45 2.16 1.84 1.76 1.78 3.48 Lac(0.5–2.2 mmol/L) / 1 2.5 4.2 / / / / / 10.1 Glu(3.9–5.8 mmol/L) / 3.27 4.5 4.1 5.4 / 3.6 / / 1.7 Medication given hydrocortisone acetate intravenously, 5 mg, q8h + oral fludrocortisone, 0.1 mg/d oral hydrocortisone acetate, 5 mg in the morning, 2.5 mg at noon and 2.5 mg at night + oral fludrocortisone, 0.1 mg/d + salt supplementation 2 g/day oral hydrocortisone acetate, 5 mg- 2.5 mg- 2.5 mg + oral fludrocortisone, 0.1 mg/d + salt supplementation 2 g/day same as the previous oral hydrocortisone acetate, 2 mg- 2 mg- 2 mg + oral fludrocortisone, 0.1 mg/d + salt supplementation 2 g/day same as the previous same as the previous same as the previous / GA Gestational age, ACTH Adrenocorticotropic hormone, LH Luteinizing hormone, FSH Follicle stimulating hormone, E2 Estradiol, P Progesterone, T Testosterone, PRL Prolactin, 17-α-OHP 17-alpha hydroxyprogesterone, ALT Alanine transaminases, AST Aspartate transaminases, ALP Alkaline phosphatase, CK Creatine kinase, CK-MB Creatine kinase isoenzyme, LDH Lactate dehydrogenase, LDH-MB Lactate dehydrogenase isoenzyme, TCHOL Total cholesterol, α-HBDH α-hydroxybutyrate, TG Triglycerides, Na Sodium, K Potassium, Ca Calcium, Mg Magnesium, Cl Chlorine, Lac Lactic acid, Glu Glucose
| 4.046875
| 0.970703
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sec[1]/p[0]
|
en
| 0.999998
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PMC9434940
|
https://doi.org/10.1186/s12887-022-03568-9
|
[
"range",
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[
{
"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"
},
{
"code": "BD11.1",
"title": "Left ventricular failure with mid range ejection fraction"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Other specified examination of eyes or vision (QA00.6Y)】
Synonyms: No Impairment of Contrast vision | Normal colour vision | No Impairment of Dark adaptation | No diplopia | No visual spatial neglect
Hierarchy: Contact with health services for purposes of examination or investigation → General examination or investigation of persons without complaint or reported diagnosis (QA00) → Examination of eyes or vision (QA00.6) → Other specified examination of eyes or vision
【2. Neutropaenia, unspecified (4B00.0Z)】
Synonyms: Neutropenia | Disorders with decreased neutrophil counts | neutropaenic disorder | neutrophil count below reference range | absence of neutrophils
Hierarchy: Immune system disorders involving white cell lineages → Disorders of neutrophil number (4B00) → Neutropenia (4B00.0) → Neutropaenia, unspecified
【3. Acquired thrombocytosis, unspecified (3B63.1Z)】
Synonyms: Acquired thrombocytosis | Idiopathic haemorrhagic thrombocythaemia | Essential thrombocythaemia | primary haemorrhagic thrombocythaemia | idiopathic thrombocythaemia
Hierarchy: Coagulation defects, purpura or other haemorrhagic or related conditions → Thrombocytosis (3B63) → Acquired thrombocytosis (3B63.1) → Acquired thrombocytosis, unspecified
【4. Raised antibody titre (MA14.1C)】
Synonyms: antibody titre above reference range | high antibody titre | increased antibody titre
Excludes: isoimmunization, in pregnancy affecting fetus or newborn
Hierarchy: Clinical findings in blood, blood-forming organs, or the immune system → Immunological findings in blood, blood-forming organs, or the immune system (MA14) → Certain specified immunological findings (MA14.1) → Raised antibody titre
【5. Left ventricular failure with mid range ejection fraction (BD11.1)】
Synonyms: HFmEF - [heart failure with mid range ejection fraction] | Left ventricular failure with mid range ejection fraction due to cardiomyopathy | Left ventricular failure with mid range ejection fraction due to coronary artery disease | Left ventricular failure with mid range ejection fraction due to myocarditis | Left ventricular failure with mid range ejection fraction due to other disorders
Hierarchy: Diseases of the circulatory system (11) → Heart failure → Left ventricular failure (BD11) → Left ventricular failure with mid range ejection fraction
|
QA00.6Y
|
Other specified examination of eyes or vision
|
The second endovascular step was scheduled ∼4 months after the first step and involved placement of a branched endoprosthesis (t-Branch; Cook Medical Inc) and bridging stenting of the visceral vessels with placement of a bifurcated abdominal graft scheduled. Bilateral percutaneous femoral access was obtained. On the right side, two ProGlide vascular closure devices (Abbott Vascular) were preimplanted with the insertion of an 11F introducer on a standard Terumo guidewire (Terumo Medical). From the left, after positioning a 5F introducer, a pigtail 5F diagnostic catheter was advanced, and preoperative angiography was performed to identify the visceral vessel origin and align the fusion computed tomography scan. From the right, after exchanging the guidewire with a Lunderquist 260 mm (Cook Medical Inc) and confirming correct alignment with the visceral vessel origin, the branched endoprosthetic module was inserted, with sequential release of the visceral branches. A complete branched module release and delivery system recovery were performed. On the right side, an 18F DrySeal introducer (W.L. Gore & Associates) was exchanged. After exchanging the Lunderquist guidewire with a Terumo standard guidewire (Terumo Medical), the Heli-Fx 22-mm catheter (Medtronic Vascular) and a Flexor 7F, 45-cm guiding sheath (Cook Medical Inc) were advanced and positioned at the prosthetic branch for the left renal artery. A Bern 5F, 65-cm catheter (Boston Scientific) was inserted, and an attempt at selective cannulation of the left renal artery was performed with a standard Terumo guidewire (Terumo Medical). After successful placement of the guidewire in the artery, attempts to stabilize the system by advancing the catheter were unsuccessful owing to resistance from the ostial stenosis and angulation of the renal artery. Furthermore, because of the pressure exerted on the ostium of the vessel, correct positioning of the guidewire was lost. A second engagement was attempted and was unsuccessful. A third attempt was made by exchanging the Bern catheter for the 5F, 65-cm vertebral hydrophilic catheter. Selective cannulation of the left renal artery was performed. However, during the exchange of the Terumo standard guide (Cook Medical Inc) with a Rosen 260-mm guidewire (Cook Medical Inc), owing to the lack of sufficient stability, the catheter was pushed to the proximal edge of the vessel. The Rosen guidewire was switched for a standard Terumo guidewire to reposition the catheter. Once the guidewire was advanced, the catheter was pushed out of the vessel along with the guidewire itself. The steerable catheter was repositioned at the prosthetic branch of the right renal artery. Two attempts at selective cannulation of the left renal artery with a standard Terumo guidewire and 5F Kumpe (Cook Medical Inc) and vertebral hydrophilic catheter, respectively, were performed without success. At this point, from the left femoral artery access, after removing the 5F pigtail catheter, the 5F introducer was exchanged for an 8F introducer and the Flexor 7F, 45-cm guiding sheath (Cook Medical Inc) was advanced up to the ostium of the left renal artery. After advancement of the V18 guidewire (Boston Scientific), selective cannulation of the vessel was performed. An ultrasoft, 5 × 20-mm balloon was then placed in the left renal artery, and, after crossing the ostial lesion, angioplasty was performed . The previously positioned inflated balloon was left in place. On the right, the Bern catheter (Boston Scientific) was advanced inside the guiding sheath and was positioned a few millimeters from the origin of the left renal artery up to the ostium of the vessel. The balloon positioned in the left renal artery was slightly deflated and, with a standard Terumo guidewire (Terumo Medical), the vessel was cannulated. After passage of the guidewire, the balloon was inflated again, and the guidewire was fixed. The Bern catheter was exchanged with a CXI 0.035-in. catheter (Cook Medical Inc). By modulating the inflation of the balloon, the stenosis was crossed, and the catheter was advanced into the distal renal artery. With the balloon inflated, the Terumo guidewire (Terumo Medical) was exchanged for the Rosen 260-mm guidewire (Cook Medical Inc), and the balloon-expandable BeGraft 6 × 58-mm stent (Bentley InnoMed GmbH) was advanced with proximal release inside the branch of the right renal artery of the branched endoprosthesis. The bridging stent of the left renal artery was completed with placement of two more Viabahn self-expandable stents (6 × 100 mm and 6 × 50 mm; W.L. Gore & Associates), with the balloon deflated to allow for correct placement of the stents . The procedure was completed with bridging stent placement of the remaining visceral vessels, implantation of the bifurcated Unibody 22-81 stent (Cook Medical) adequately imbricated, branching of the endoprosthetic component, and placement of the Zenith Alpha spiral Z 20-42 right iliac leg (Cook Medical Inc). The completion angiogram at the end of the procedure documented correct positioning of the branched endoprosthesis and bridging stents, normal patency of the aortic endoprosthesis, no signs of kinking and/or dissection in the stenting site of the visceral vessels, and normal patency of the femoral iliac axes with reperfusion of the aneurysmatic sac from the gate of the forked body. The fluoroscopy time was 178 minutes for the procedure. The dose ∙ area product was 800 Gy/cm 2 . The postoperative course was normal without any complications. The third step of endovascular aneurysm repair was performed 2 months after the second step using a Zenith Alpha 16-77 left iliac limb . Fig 2 A, DrySeal 18F introducer (W.L. Gore & Associates) insertion after branched module release and delivery system recovery. B, Positioning of Heli-Fx 22-mm catheter (Medtronic Vascular) and Flexor 7F, 45-cm guiding sheath (Cook Medical Inc) at the prosthetic branch for the left renal artery. Selective cannulation of the left renal artery using a Bern 5F, 65-cm catheter (Boston Scientific) and standard Terumo guidewire (Terumo Medical). C, Repositioning of the steerable catheter at the prosthetic branch of the right renal artery, and selective cannulation attempt with the diagnostic catheter and Terumo guidewire. Once the guidewire was advanced, the catheter was pushed out of the vessel, along with the guidewire itself. D, Flexor 7F, 45-cm guiding sheath (Cook Medical Inc) positioning and advancement up to the ostium of the left renal artery. After exchange of the 5F introducer for a 8F introducer from the left femoral access, cannulation with the V18 guidewire and predilatation with an ultrasoft 5 × 20-mm balloon was performed. Fig 3 A, Bern catheter (Boston Scientific) position corresponding to the ostium of the left renal artery with cannulation of the target vessel with a Terumo guidewire by modulating inflation of the previously positioned balloon and fixation of the guidewire inside the left renal artery by inflating the ultrasoft balloon. B, Bern catheter exchange with a CXI 0.035-in. catheter (Cook Medical), crossing of the stenosis by slightly deflating the balloon, and successful guidewire exchange (260-mm Rosen for a Terumo guidewire). C, Stenting of the target vessel with Viabahn self-expandable stents (W.L. Gore & Associates) on the Rosen guidewire (Cook Medical) facilitated by modulation of the inflation of the balloon. D, Final result of stenting of the hostile left renal artery. Fig 4 Postoperative computed tomography angiogram showing completion of the three-step treatment.
| 4.042969
| 0.881348
|
sec[0]/p[2]
|
en
| 0.999997
|
PMC10598395
|
https://doi.org/10.1016/j.jvscit.2023.101330
|
[
"guidewire",
"catheter",
"artery",
"renal",
"terumo",
"cook",
"balloon",
"vessel"
] |
[
{
"code": "QB62.Z",
"title": "Attention to artificial openings, unspecified"
},
{
"code": "QB30.5",
"title": "Fitting or adjustment of urinary device"
},
{
"code": "PK93.10",
"title": "Gastroenterology or urology devices associated with injury or harm, urinary catheter"
},
{
"code": "PK90.1",
"title": "Anaesthesiology devices associated with injury or harm, therapeutic, nonsurgical or rehabilitative devices"
},
{
"code": "PK91.2Y",
"title": "Other specified cardiovascular devices associated with injury or harm, prosthetic or other implants, materials or accessory devices"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Attention to artificial openings, unspecified (QB62.Z)】
Synonyms: Attention to artificial openings | toilet or cleansing of artificial opening | removal of catheter | passage of sounds or bougies | closure of artificial opening
Hierarchy: Reasons for contact with the health services → Surgical or postsurgical states → Attention to artificial openings (QB62) → Attention to artificial openings, unspecified
【2. Fitting or adjustment of urinary device (QB30.5)】
Synonyms: change of indwelling catheter | Removal of indwelling urinary catheter | removal of urinary catheter | removal of indwelling catheter
Hierarchy: Reasons for contact with the health services → Fitting, adjustment or management of devices → Adjustment or management of implanted devices (QB30) → Fitting or adjustment of urinary device
【3. Gastroenterology or urology devices associated with injury or harm, urinary catheter (PK93.10)】
Synonyms: Gastroenterology or urology devices associated with adverse incidents, Foley catheter | Gastroenterology or urology devices associated with adverse incidents, indwelling urinary catheter | Mechanical complication of urinary catheter | Mechanical complication of urinary indwelling catheter | malfunction of cystostomy catheter
Excludes: Circumstances associated with a surgical or other medical device influencing the episode of care without injury or harm
Hierarchy: Surgical or other medical devices, implants or grafts associated with injury or harm in therapeutic use → Gastroenterology or urology devices, implants or grafts associated with injury or harm (PK93) → Gastroenterology or urology devices associated with injury or harm, therapeutic, nonsurgical or rehabilitative devices (PK93.1) → Gastroenterology or urology devices associated with injury or harm, urinary catheter
【4. Anaesthesiology devices associated with injury or harm, therapeutic, nonsurgical or rehabilitative devices (PK90.1)】
Definition: An anaesthesiology device was involved in an adverse incident that occurred in a therapeutic (nonsurgical) and rehabilitative task
Synonyms: Anaesthesiology devices associated with injury or harm, spinal catheter | Mechanical complication of spinal catheter | Anaesthesiology devices associated with injury or harm, epidural catheter | Anaesthesiology devices associated with injury or harm, endotracheal tube | Mechanical complication of epidural or subdural infusion catheter
Excludes: Circumstances associated with a surgical or other medical device influencing the episode of care without injury or harm
Hierarchy: Causes of healthcare related harm or injury → Surgical or other medical devices, implants or grafts associated with injury or harm in therapeutic use → Anaesthesiology devices associated with injury or harm (PK90) → Anaesthesiology devices associated with injury or harm, therapeutic, nonsurgical or rehabilitative devices
【5. Other specified cardiovascular devices associated with injury or harm, prosthetic or other implants, materials or accessory devices (PK91.2Y)】
Synonyms: Cardiovascular devices associated with injury or harm, conduits | Mechanical complication of other cardiac and vascular devices and implants | Mechanical complication of artificial heart | Mechanical complication of vascular balloon implant or device | Mechanical complication of vascular shunt
Hierarchy: Surgical or other medical devices, implants or grafts associated with injury or harm in therapeutic use → Cardiovascular devices, implants or grafts associated with injury or harm (PK91) → Cardiovascular devices associated with injury or harm, prosthetic or other implants, materials or accessory devices (PK91.2) → Other specified cardiovascular devices associated with injury or harm, prosthetic or other implants, materials or accessory devices
|
QB62.Z
|
Attention to artificial openings, unspecified
|
A 54-year-old man with loss of appetite and fatigue 3 weeks prior was admitted to our hospital due to vomiting and anemia. His medical history included depression and arrhythmia. He had no family history of malignant neoplasia. During presentation, he had tachycardia with a heart rate of 95/min was present, but his other vital signs were within normal limit. Abdominal examination showed normal findings with no palpable mass or tenderness. The blood tests showed a low hemoglobin level (6.2 g/dL) with microlytic anemia, whereas the levels of carcinoembryonic antigen and carbohydrate antigen 19–9 were within the normal range. An esophagogastroduodenoscopy showed no obvious abnormalities. A double-balloon enteroscopy and enterography using meglumine sodium amidotrizoate showed a large circumferential tumor located in the jejunum just beyond the angle of Treitz . Contrast-enhanced computed tomography from the chest to the pelvis revealed a tumor that equally contrasted with jejunum, inducing intussusception and regional lymph node swelling at the angle of Treitz, although there were no obvious findings of distant metastasis . Fluorodeoxyglucose positron emission tomography demonstrated that this neoplasm had marked hypermetabolism, showing a standardized uptake value (SUV) max of 8.3, and the swollen regional lymph node showed mild hypermetabolism with a SUV max of 3.7 . Histopathological examination of a biopsy specimen revealed fascicles of spindle-shaped cells with elongated hyperchromatic nuclei showing variably pleomorphism and abundant eosinophilic cytoplasm in the stroma . Immunohistochemically, the tumor cells were positive for α-smooth muscle actin , h-caldesmon , calponin , and vimentin, and negative for cytokeratin (AE1/AE3) cytokeratin (CAM5.2), CD34 , c-kit , DOG-1, S-100 protein , and myogenin. The Ki-67 labeling index was approximately 30% . Therefore, we diagnosed the patient with a LMS of the jejunum located just beyond the angle of Treitz with intussusception. We considered that the intussusception gradually occurred with tumor growth; thus, elective laparoscopic tumor resection was performed without reducing the intussuscepted jejunum . The swollen lymph nodes around the tumor were also removed. After extracorporeal extraction of the tumor, an intracorporeal delta-shaped anastomosis was performed in the same manner as that for gastroduodenostomy, as previously described . In brief, the transection line of the proximal jejunum extended from the posterior wall to the anterior wall . In contrast, the transection line of the distal intestine extended from the mesenteric side to the antimesenteric side . The stump of the proximal side was mobilized by dissecting the ligament of Treitz from the retroperitoneum . Then, a small hole was created on the posterior side of the staple line of the proximal jejunum and on the mesenteric side of the staple line of the distal jejunum . A 45-mm linear stapler was inserted into both small holes. The posterior walls of the proximal and distal jejunums were put together, and the stapler was closed and fired . Then, maximum possible distance was kept between each staple line to secure sufficient blood perfusion in the area between the staple lines . The common entry hole was temporarily closed by three full-thickness stitches while widening the V-shaped anastomosis made by first stapling and then permanently closed with one application of a 60-mm linear stapler . These procedures produced a torsion-free anastomosis while maintaining the physiological axes of the intestinal tract, resulting in an appropriate gap in the staple line of the common entry hole closure between the staple lines of the proximal and distal jejunum stumps . The operative time and estimated blood loss were 263 min and 180 ml, respectively. The time required for delta-shaped anastomosis was 20 min. Macroscopy of the resected tumor revealed a white solid mass with a size of 10 × 8 × 5 cm located throughout the jejunal wall . Histologically, the tumor grew by engulfing the jejunal mucosa . The tumor cells showed a brisk mitotic activity (> 20 mitoses per 10 high power fields) , and there were scattered tumor necroses , indicating that the histological grade of the tumor was 3 according to the French Federation Nationale des Centers de Lutte Contre le Cancer system . All surgical margins were negative for the tumor, and there were no obvious findings of lymphovascular invasion. Furthermore, there were no metastatic findings in all dissected lymph nodes (0/13). The patient’s postoperative course was uneventful, and he was discharged at 10 days after surgery. Neither signs of recurrence nor stenosis has been observed within the 2 years after surgery . Fig. 1 Preoperative imaging findings. a Endoscopic findings: enteroscopy revealed an easily hemorrhagic tumor in the jejunum. b Findings of the small bowel series using meglumine sodium amidotrizoate: a tumor (white arrow) was found in the jejunum at the angle of Treitz. c Contrast-enhanced computed tomography findings: The tumor with intussusception appeared as a reniform shape (pseudokidney sign) longitudinally and demonstrated multilayered concentric rings of mass (target sign) transversely. A tumor with intussusception and regional lymph node swelling was located at the angle of Treitz. d Findings of the fluorodeoxyglucose positron emission tomography: this tumor had marked hypermetabolism, showing a standardized uptake value (SUV) max of 8.3, and the swollen regional lymph node showed mild hypermetabolism with a SUV max of 3.7 Fig. 2 Microscopic and immunohistochemical findings of a biopsy specimen. a Hematoxylin and eosin staining. b α-Smooth muscle actin. c h-Caldesmon. d calponin. e Vimentin and cytokeratin (AE1/AE3). f Cytokeratin (CAM5.2) and CD34. g c-Kit. h DOG-1 and S-100 protein. i Myogenin (the Ki-67 labeling index was approximately 30%) Fig. 3 Intraoperative findings. a Intestinal intussusception was seen at the angle of Treitz. b Transection line of the proximal jejunum extended from the posterior wall to the anterior wall. c After resection of the tumor, the residual duodenal stump was short. d Proximal jejunal stump was mobilized from the retroperitoneum Fig. 4 Procedure for creating a delta-shaped anastomosis. a Small hole was created on the posterior side of the staple line of the proximal jejunum and on the mesenteric side of the staple line of the distal jejunum. b Dorsal walls of the proximal and distal walls of the jejunum were stapled with sufficient distance (arrows) between each other’s staple lines to maintain blood flow. c Common entry hole was temporarily closed by three full-thickness sutures while widening the V-shaped anastomosis made by first stapling and then permanently closed with a stapler. d Jejunojejunostomy was performed using delta-shaped anastomosis (double arrow: an appropriate gap between the staple lines of the proximal and distal jejunum stumps maintained the physiological axes of the intestinal tract). PJ proximal jejunum, DJ distal jejunum, VSA V-shaped anastomosis Fig. 5 Macroscopic findings of the cross-section and histological findings of the resected specimen. a Tumor was a white solid mass (10 × 8 × 5 cm in size) located throughout the jejunal wall. b Tumor grew by engulfing the jejunal mucosa. c Tumor cells showed a brisk mitotic activity (arrow, > 20 mitoses per 10 high power fields). d There were scattered tumor necroses Fig. 6 Contrast-enhanced computed tomography findings 2 years after surgery. Neither signs of recurrence nor stenosis has been observed
| 4.132813
| 0.960938
|
sec[1]/p[0]
|
en
| 0.999997
|
36156747
|
https://doi.org/10.1186/s40792-022-01541-3
|
[
"tumor",
"jejunum",
"findings",
"staple",
"line",
"shaped",
"anastomosis",
"treitz"
] |
[
{
"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 CLASSIFICATION ═══
【1. Neoplasms of unknown behaviour of unspecified site (2F9Z)】
Synonyms: neoplasia | neoplasm growth NOS | tumour of unspecified site | tumourlet of unspecified site | tumour mass NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of unspecified site
【2. Subcutaneous swelling, mass or lump of uncertain or unspecified nature (ME61)】
Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature
Synonyms: localised swelling, mass or lump of skin and subcutaneous tissue | localised subcutaneous nodules | subcutaneous nodules | superficial subcutaneous nodules | localised swelling
Excludes: localized adiposity | mass and lump: breast | enlarged lymph nodes | mass and lump: intra-abdominal or pelvic | oedema
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings involving the skin → Symptoms or signs involving the skin → Subcutaneous swelling, mass or lump of uncertain or unspecified nature
【3. Carcinoma in situ of unspecified site (2E6Z)】
Synonyms: carcinoma in situ of unspecified site | carcinoma in situ NOS | carcinoma in situ | in situ neoplasm
Hierarchy: Neoplasms (02) → In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues → Carcinoma in situ of unspecified site
【4. Neoplasms of unknown behaviour of trachea, bronchus or lung (2F91.1)】
Synonyms: 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 | Lung hemangiopericytoma of unknown behaviour
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs (2F91) → Neoplasms of unknown behaviour of trachea, bronchus or lung
【5. Neoplasms of unknown behaviour of skin (2F92)】
Synonyms: skin tumour NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of skin
|
2F9Z
|
Neoplasms of unknown behaviour of unspecified site
|
A 48-year-old Chinese woman was admitted to our hospital on July 25, 2009, because of a left supraclavicular mass that had persisted for 2 months. The patient's left supraclavicular mass was painless. The patient had no clinical symptoms, such as cough, anhelation, hoarseness, chest pain, dysphagia, upper abdominal swelling, and no history of smoking or family history of tumors. Physical examination revealed that multiple enlarged lymph nodes were touched on left supraclavicular mass, their diameters in size were 0.8 to 1.4 cm. It has the characteristics of a hard texture, fusion, and unclear boundaries. The skin temperatures of the local masses were normal. The lymph node was not palpable in the right supraclavicular region. The whole body F18-fluorodeoxyglucose-positron emission tomography/computed tomography revealed that there was a pulmonary nodule on upper lobe of the left lung. The boundary of the nodule was unclear with spiculation sign, and the size was about 1.5 cm × 1.4 cm. The radioactive intake was moderate. There was a nodule with radioactive intake with mild concentration on left lung, and the size was about 2.7 cm × 2.4 cm . No placental lesions or radioactive intake abnormalities were found bilaterally in the breasts. Multiple enlarged lymph nodes with highly radioactive ingestion were found in the left supraclavicular area, with diameters in size were 0.4 to 1.4 cm . Nasopharyngeal morphology was normal, and there were no radioactive abnormalities. There were no masses in the abdomen or abdominal wall. Diagnosis by gastroscopy showed chronic inflammation of chronic erosive gastritis and chronic inflammation of the duodenum, and the esophagus was not found to be abnormal. Laboratory findings were within the normal range, except for the cancer antigen 125 (CA125) level of 104.4 U/mL (normal range is 0–35.00 U/mL) in the serum. We performed a B-type ultrasonography-guided core needle biopsy of the left supraclavicular lymph nodes, and the pathological diagnosis of the specimen was metastatic adenocarcinoma . The final diagnosis was left lung adenocarcinoma with left supraclavicular lymph node metastasis, cT1N3M0 IIIB (International association for the Study of lung cancer 6th version). The patient was treated with chemotherapy including gemcitabine (1.6 g/m 2 d1, d8) and cisplatin (40 mg/m 2 , d1–3) for 2 cycles: gemcitabine (1.6 g/m 2 d1, d8) and carboplatin (area under the curve [AUC] = 5, d1), for 2 cycles between August 5, 2009, and November 17, 2009. Since then, the patient rejected the above-mentioned chemotherapy regimen and switched to EGFR-TKI targeted therapy. However, the application of targeted therapy with EGFR-TKIs has not been advocated for patients with advanced NSCLC harboring wild-type EGFR. Although gene mutations in patients’ lesions have been detected, it was reported that the clinical features of adenocarcinoma in female non-smokers positively correlated with the effects of EGFR-TKI drugs. Starting on January 15, 2010, we prescribed gefitinib to this patient at a daily dose of 0.25 for maintenance treatment. The patient had no obvious adverse reactions during the course of the therapy. As of March 1, 2010, CT imaging of the patient's chest showed that the left supraventricular lymph nodes and left lung lesions had become smaller (CA125: 38.9 U/mL). The efficacy evaluation was partial response (PR) (response evaluation criteria in solid tumors). The patient recovered very well and continued to use targeted therapy with gefitinib alone at home during this period. On July 6, 2015, the patient returned to the hospital because of a headache. Magnetic resonance (MR) imaging of the brain showed thickening of the meningeal and arachnoid regions and suspected metastatic lesions. MR imaging of the chest showed that the clavicle and pulmonary lesion had disappeared, CA125 level was 15.6 U/mL. We performed local radiotherapy (40 GY/20 F) in patients with brain metastatic lesions from on July 20 to August 25, 2015. After radiotherapy, the patient's headache symptoms improved. Since September 2015, 80 mg of osimertinib has been administered daily as maintenance therapy at home, with no abnormal changes in her body and no obvious side effects. By on May 12, 2020, the patient experienced weakness in the lower limb myodynamia but had no obvious headaches, coughs, or other symptoms. The results of the driver gene detection for EGFR, anaplastic lymphoma kinase (ALK), and C-ros oncogene1 receptor-tyrosine kinase (ROS1) in cancer cells from peripheral blood samples were negative . Despite this, considering that patients had received osimertinib for >5 years, we continued to administer double-dose osimertinib (160 mg, quaque die Per os [PO]) alone, but the muscle strength of the lower limbs did not improve significantly. On August 15, 2020, neurological examination was performed, and the muscle strength of the lower limbs was grade 2. MR imaging showed thickening of lumbar arachnoid membrane and suspected metastatic lesion. Chest CT scan showed that there was a metastatic lesion in the lungs with a size of 1.7 cm × 3.3 cm (progressive lesion), and no lesions were seen on the left supraclavicular. CA125 levels were 60.68 U/mL. There were no inflammatory or cancer cells in the cerebro spinal fluid from the lumbar puncture. We considered that the lung lesions acquired resistance to osimertinib. From September 8, 2020, to November 21, 2020, the patient was administered combination chemotherapy pemetrexed (0.7 g, d1) with oxaliplatin (195 mg, d1). Meanwhile, the lumbar lesion was treated with hyperthermia, 40° for 1 hour, d1–4, for 4 cycles. By December 16, 2020, a chest CT scan showed progression of the lung lesion. The lumbar arachnoid and chorionic membranes were pinched. His muscle strength improved to grade 4. From December 20, 2020, we decided to combine toripalimab (240 mg, d1) with anlotinib (12 mg, quaque die, for 2 weeks, 1 week rest), q3w for 4 cycles. By April 22, 2021, physical examination revealed that the symptoms of muscle strength had recovered to Grade 4. MR imaging revealed that the lumbar arachnoid and chorionic membranes were not thickened . Chest computed tomography (CT) scan showed enlargement of the lung metastatic lesion (3.1 cm × 4.1 cm). Efficacy evaluation was based on the progressive disease (response evaluation criteria in solid tumors criteria). The results of the driver gene detection for EGFR, ALK, and ROS1 in cancer cells from peripheral blood samples were negative . Although the genetic test results were negative, the patient had always benefited from treatment with EGFR-TKi drugs since 2010 and did not benefit from combined immunity with antiangiogenic therapy. Double-dose icotinib treatment can benefit patients with previous resistance to EGFR-TKIs. Therefore, from May 20, 2021, the patient self-administered double-dose icotinib (125 mg × 2, Ter in die Per os [PO]) alone (the whole treatment process is shown in Table 1 ). However, on June 19, 2021, the patient suddenly appeared comatose. Chest computed tomography (CT) showed that the lung metastatic lesion had shrunk (1.7 cm × 1.8 cm, and original size: 3.1 cm × 4.1 cm). Efficacy evaluation was performed using the PR (response evaluation criteria in solid tumors criteria). Unfortunately, MRI of the brain showed brain infarction in multiple parts of the corona radiata, frontal lobe, and left cerebellar hemispheres . Finally, the patient died of brain infarction on July 23, 2021. The patient has been alive for nearly 12 years with comprehensive EGFR-TKI-based therapy.
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sec[1]/p[0]
|
en
| 0.999997
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35029237
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https://doi.org/10.1097/MD.0000000000028583
|
[
"lung",
"egfr",
"supraclavicular",
"lesion",
"chest",
"lesions",
"metastatic",
"therapy"
] |
[
{
"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 CLASSIFICATION ═══
【1. Other specified diseases of the respiratory system (CB40.Y)】
Synonyms: Secondary respiratory disorders | Respiratory disorders in diseases classified elsewhere | Diseases of bronchus, not elsewhere classified | Acquired bronchial diverticulum | acquired bronchus diverticulum
Hierarchy: Diseases of the respiratory system (12) → Certain diseases of the respiratory system (CB40) → Other specified diseases of the respiratory system
【2. Agenesis of lung (LA75.1)】
Definition: This refers to the absence or rudimentary residua of an undeveloped lung.
Synonyms: Pulmonary agenesis | absence of lung | aplasia of lung | apulmonism | congenital absence of lung
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the respiratory system → Structural developmental anomalies of lungs (LA75) → Agenesis of lung
【3. Pneumonia, organism unspecified (CA40.Z)】
Synonyms: Pneumonia | infectious pneumonia | PN - [pneumonia] | lobar pneumonia NOS | multifocal pneumonia
Hierarchy: Diseases of the respiratory system (12) → Lung infections → Pneumonia (CA40) → Pneumonia, organism unspecified
【4. Respiratory failure (CB41)】
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.
Synonyms: lung failure NOS | pulmonary failure
Excludes: Acute respiratory distress syndrome | Respiratory arrest | Respiratory distress of newborn
Hierarchy: Diseases of the respiratory system (12) → Respiratory failure
【5. Other injury of lung (NB32.3Y)】
Synonyms: Haematoma of lung | Traumatic hydropneumothorax | Acute traumatic lung congestion | Rupture of lung
Hierarchy: Injuries to the thorax → Injury of other or unspecified intrathoracic organs (NB32) → Certain injuries of lung (NB32.3) → Other injury of lung
|
CB40.Y
|
Other specified diseases of the respiratory system
|
A previously healthy 5-year-old girl was admitted to the first hospital on January 17, 2018, with symptoms of abdominal pain, vomiting, and new-onset refractory status epilepticus (NORSE) that had lasted for 4 h. The results of a cranial computed tomography (CT) performed by the emergency department were normal. She was transferred to our hospital 6 h after onset, demonstrating signs of coma, tachypnea, and tachycardia. Her past medical history was negative. Upon admission, her body temperature was 36.3 °C, her heart rate was 155 beats/min, her respiratory rate was 50 breaths/min, her blood pressure was 102/67 mmHg, and the Glasgow Coma Scale score (GCS) was E1V1M3. Hypermyotonia was observed in both limbs, and the Babinski sign was positive bilaterally. Other findings from the systemic physical examinations were unremarkable. Percutaneous oxygen saturation measured by pulse oximetry (SpO2) was 93–95% on 25% FiO 2 . A series of blood tests showed the white blood cell (WBC) count to be 15.98 × 10 9 /L, neutrophils 91.3%, lymphocytes 4.8%, red blood cells 4.99 × 10 12 /L, platelets 264 × 10 9 /L, CRP 2.5 mg/L, procalcitonin (PCT) 55.77 ng/mL, ferritin 120 ng/ml, coagulant function (fibrin 1.53 g/L, D-Dimer 0.27 mg/L, prothrombin time (PT) 12.2 s, activated partial thromboplastin time (APTT) 24.9 s, INR 1.04, ACT 85%), serum enzymes (CKMB 24 U/L, CK 298 U/L, LDH 797 U/L, aspartate transaminase (AST) 65 IU/L, alanine transaminase (ALT), 38 IU/L), creatinine 40.7 μmol/L, and BUN 4.34 mmol/L. Brain magnetic resonance imaging (MRI) was performed quickly when the patient was admitted to our hospital. The diffusion-weighted imaging (DWI) scan of the patient’s brain on admission showed symmetric areas with high signal intensity in the periventricular white matter involving the centrum semiovale and the corona radiate . The patient had rapid clinical deterioration that developed to hyperferritinemic sepsis with multiple organ dysfunction syndrome (MODS) 15 h after onset, including exhibiting a worsening mental status (GCS E1V1M1), fever (40.6 °C), hypotension (60/47 mmHg), tachycardia (heart rate: 178 beats/min), and tachypnea (respiratory rate: 70 breaths/min). She was intubated, and a vasopressor was quickly given to maintain blood pressure. A repeat blood test showed a WBC count of 9.09 × 10 9 /L, neutrophils 71.4%, lymphocytes 18.8%, red blood cells 4.49 × 10 12 /L, platelets 27 × 10 9 /L, ferritin 22,579.1 ng/ml, coagulant dysfunction (fibrin 1.5 g/L, D-dimer, 4.7 mg/L, PT 31.4 s, APTT 74.1 s, INR2.79, ACT 20.2%,), serum enzymes , creatinine 134.3 μmol/L, and BUN 10.82 mmol/L. Blood cultures did not identify any pathogens. Serum viral studies (influenza A and B viruses, respiratory syncytial virus, adenoviruses, cytomegalovirus, Epstein–Barr virus, hepatitis C virus, hepatitis B virus, and human immunodeficiency virus) and serology tests for syphilis, Mycoplasma pneumonia and Mycobacterium tuberculosis were all negative. Lumber punctures were performed 3 times, and showed increased intracranial pressure (the highest was over 300 mm H 2 O). Cerebrospinal fluid (CSF) tests revealed that protein levels and glucose levels were normal without pleocytosis. CSF PCRs for enterovirus, herpes simplex virus, cytomegalovirus, Epstein-Barr virus, and tuberculosis were all negative. CSF bacterial and fungal cultures were also negative. The echocardiographic results showed that the left ventricular ejection fraction was 52%. The electrocardiograph (ECG) showed tachycardia. The electroencephalography examination demonstrated diffuse, generalized and slow background activity. X-ray imaging showed the presence of bronchitis. Hyperferritinemic sepsis was diagnosed after ruling out haemophagocytic lymphohistiocytosis (HLH) since the diagnostic criteria for HLH were not fulfilled in this girl. SAE was also diagnosed by ruling out encephalitis, meningitis, acute necrotizing encephalopathy, acute disseminated encephalomyelitis, Guillain-Barre syndrome, cerebral vasculitis, and metabolic encephalopathy, according to laboratory tests and imaging features. Integrated treatment was initiated on admission, which included anti-infection treatments (meropenem, vancomycin, and voriconazole), anti-inflammation treatments (methylprednisolone, 15 mg/kg/day × 3 days), intravenous immunoglobulin (1 g/kg/day × 2 days), and frozen plasma as well as the administration of other medicines for hepatic and myocardial protection. The symptoms were not relieved, and the fulminant development of MODS indicated that hyperinflammation or an autoimmune response might be involved. Membrane-based therapeutic plasma exchange (TPE) was started on day 3 since it can eliminate pro-inflammatory cytokines rapidly and can modulate the sepsis cascade. The exchange volume was 1.5-fold the patient’s plasma volume. Plasma volume was estimated as follows: plasma volume (in liters) = 0.07 × weight (kg) × (1-hematocrit) . The removed plasma was substituted with fresh frozen plasma at a 1:1 ratio in our patient. The first three TPEs were performed daily. After the second TPE, the patient needed less vasopressor. By the third TPE treatment, liver enzymes had decreased. The patient was extubated and regained full consciousness on day 10, with mild remaining disseminated intravascular coagulation (DIC), acute kidney injury (AKI) and capillary leak syndrome (CLS). The last two TPEs were followed by continuous vena-venous hemofiltration (CVVH) 2 times to remove creatinine and BUN, to prevent liquid overload and to maintain coagulation function. After these integrated treatments, clinical and laboratory improvement were monitored in the patient (Table 1 ). Echocardiography showed that left ventricular systolic function was normalized, with a left ventricular ejection fraction (LVEF) of 62%. The ECG was normal. Her muscle strength gradually improved, showing increased movements, and she was able to walk short distances 17 days after onset. At this point, repeated brain MRI showed that the lesions were reduced, and T2/FLAIR scans indicated diffuse high signal intensity in the white matter of the occipital and parietal regions . The patient was transferred to a rehabilitation center 21 days after onset. She had regained full independence in activities of daily living at 55 days after onset. A repeat brain MRI showed that the lesions had completely disappeared in the white matter regions . Fig. 1 Brain magnetic resonance imaging (MRI) ( a-d ) DWI showed symmetric areas of high signal intensity in the periventricular white matter involving the centrum semiovale and corona radiate on day 1 (white arrow). ( e-h ) T2/FLAIR images showed diffuse high signal intensity in the white matter of occipital and parietal regions on day 17(white arrow). ( i-p ) DWI and T2/FLAIR images showed brain lesions had completely disappeared in the white matter on day 55 Table 1 Laboratory parameters for the patient Pre TPE status post one TPE status post two TPE status post three TPE status post last TPE Hospital day 1 3 4 10 13 WBC(×10 9 /L) 9.09 8.67 8.16 5.7 11.28 neutrophil(×10 9 /L) 6.49 7.68 7.15 5.22 10.1 Platelet(×10 9 /L) 27 37 73 30 151 CRP (mg/L) 2.5 2.5 2.5 – 2.5 PCT (ng/mL) 55.77 – 21.82 20.54 0.21 Ferritin (ng/mL) 22,579.1 5246.03 – 1238.96 – APTT(s) 74.1 43.3 31.5 34.8 31.7 PT(s) 31.4 23.5 18.1 20.5 12.8 Fib(g/L) 1.5 1.39 2.01 1.25 1.31 D-dimer (mg/L) 4.7 34.72 12.78 28.16 13.51 Creatine (μmol/L) 134.3 96.8 159.1 213.6 68.4 BUN (mmol/L) 10.82 9.03 12.99 23.29 10.31 ALT(U/L) 114 422 2580 1686 212 AST(U/L) 295 667 3415 1764 71 CKMB(U/L) 55 48 – 28 18
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sec[1]/p[0]
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en
| 0.999997
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32122316
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https://doi.org/10.1186/s12883-020-01661-z
|
[
"white",
"blood",
"virus",
"plasma",
"onset",
"status",
"brain",
"matter"
] |
[
{
"code": "EF5Y",
"title": "Other specified dermatoses attributable to hyperviscosity or microvascular occlusion"
},
{
"code": "JB41.1",
"title": "Deep phlebothrombosis in the puerperium"
},
{
"code": "EB60.Y",
"title": "Lichen sclerosus of other specified sites"
},
{
"code": "MC80.00",
"title": "White coat hypertension"
},
{
"code": "1F2D.2",
"title": "White piedra"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Other specified dermatoses attributable to hyperviscosity or microvascular occlusion (EF5Y)】
Synonyms: 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 | Cutaneous microvascular occlusion due to cardiac emboli
Hierarchy: Skin disorders involving specific cutaneous structures → Disorders of cutaneous blood and lymphatic vessels → Dermatoses attributable to hyperviscosity or microvascular occlusion → Other specified dermatoses attributable to hyperviscosity or microvascular occlusion
【2. Deep phlebothrombosis in the puerperium (JB41.1)】
Synonyms: postnatal deep vein thrombosis | postpartum deep phlebothrombosis | postpartum deep-vein thrombosis | DVT - [deep venous thrombosis] postnatal | puerperal deep thrombophlebitis
Hierarchy: Pregnancy, childbirth or the puerperium (18) → Complications predominantly related to the puerperium → Venous complications in the puerperium (JB41) → Deep phlebothrombosis in the puerperium
【3. Lichen sclerosus of other specified sites (EB60.Y)】
Synonyms: Extragenital lichen sclerosus | Guttate lichen sclerosus | White spot disease | Guttate scleroderma | Extragenital lichen sclerosus with morphoea
Hierarchy: Inflammatory dermatoses → Scarring or sclerosing inflammatory dermatoses → Lichen sclerosus (EB60) → Lichen sclerosus of other specified sites
【4. White coat hypertension (MC80.00)】
Definition: Persistently elevated office blood pressure readings with persistently normal out-of-the-office readings.
Synonyms: white coat syndrome
Hierarchy: Symptoms or signs involving the circulatory system → Abnormal blood-pressure reading, without diagnosis (MC80) → Elevated blood-pressure reading, without diagnosis of hypertension (MC80.0) → White coat hypertension
【5. White piedra (1F2D.2)】
Definition: A disease of the hair shaft, caused by an infection with the fungi Trichosporon beigelii. This disease is characterised by irregular, soft, white, or light brown nodules which adhere to the hair follicle. Transmission is by direct contact with contaminated soil or water, or by airborne transmission. Confirmation is by identification of Trichosporon beigelii in a hair follicle sample.
Synonyms: Trichosporosis nodosa
Hierarchy: Certain infectious or parasitic diseases (01) → Mycoses → Non-dermatophyte superficial dermatomycoses (1F2D) → White piedra
|
EF5Y
|
Other specified dermatoses attributable to hyperviscosity or microvascular occlusion
|
A 42-year-hypertensive man having history of bronchial asthma presented with high-grade continuous fever, dry cough, and sore throat. He was brought to hospital for difficulty in breathing, experienced on day 5 of his febrile illness. On admission, he looked toxic, febrile (103 °F) and tachypnoeic (40 breaths/min) with low peripheral SpO2 (85%). He was hypotensive (90/40 mm Hg) having a rapid pulse (120/min). Wheeze and crackles were obvious on chest auscultation especially on mid and lower zones of both lung fields along with woody dull percussion note in the abovementioned areas. This suggested severe SARS-CoV2 pneumonia. He was immediately shifted to intensive care unit (ICU) addressing the severity of chest infection and need for respiratory assistance. High flow oxygen was given through nasal canula, intravenous fluid, and ceftriaxone 1g bd were administered and necessary investigations were sent including deep nasopharyngeal swab for PCR for SARS-CoV2. Inspite of receiving high flow oxygen, due to relentless desaturation of peripheral SpO2, he was orotracheally intubated for assisted mechanical ventilation. His peripheral blood picture showed high total leukocyte count with neutrophilic leukocytosis, elevated serum C-reactive protein, procalcitonin, ferritin, and serum CPK (Table 1 ). Chest X-ray showed bilateral bronchopneumonic patchy opacities but serum troponin, serum pro-BNP, and echocardiogram was within normal range. He was PCR positive for SARS-CoV2 and received intravenous remdesivir (100 mg daily for 10 days), tocilizumab (8 mg/kg; 2 doses), and dexamethasone (5 mg q6h for 5 days and 5 mg bd for 7 days). Otherwise, in favor, he had intact orientation and his hemoglobin level, coagulation profile (platelet count, PT, and APTT), serum electrolytes, serum anti-GM1 antibody titer and blood sugar level were within normal limits and he had preserved liver and renal functions (Table 1 ). However, his serum d -dimer was elevated. Subsequently, high resolution spiral CT scan of chest revealed bilateral diffuse ground glass densities and reticulation with features of consolidation and sub-segmental pulmonary embolism. Accordingly, he received subcutaneous low molecular weight heparin for 2 weeks from the second day of his ICU admission. In view of high CRP, increasing O 2 requirement and inability to sustain a stable blood pressure, tracheal aspirate, blood and urine samples were sent for culture and sensitivity (CS) on the 3rd day of admission to exclude secondary bacterial infection. Ceftriaxone was changed to intravenous teicoplanin (for 10 days) for a broader antibacterial coverage. Tracheal aspirate CS revealed growth of methicillin-resistant Staphylococcus aureus (MRSA), which was fortunately sensitive to teicoplanin. He required sedation (intravenous midazolam and fentanyl for 10 days) and muscle relaxation (IV vecuronium for 2 days) to maximize the tolerability of mechanical ventilation. On the 10th day of his mechanical ventilation, when sedation was gradually withdrawn, weaning from ventilator was unsuccessful and marked symmetrical upper and lower limb weakness was apparent. Both his upper and lower limbs were paralyzed (power 0/5), moderately wasted, flabby, and deep tendon reflexes could not be elicited with an intact sensation in all modalities. However, he was fully alert, and functions of all cranial nerves were preserved. At this time, serum CPK was higher than at admission (850 mcg/mL). Electrophysiology done on day 20 of admission revealed normal motor and sensory conduction but myopathic changes on needle electromyogram (EMG) examination, consisting of spontaneous muscle fiber activity, small motor unit potentials, and a full recruitment pattern . Thigh and calf muscle MRI done on day 20, showed marked hyperintensity in both the quadriceps muscles , mild hyperintensity in the hamstrings, and patchy hyperintensity in the leg muscles. Skeletal muscle histopathology done on day 23 and muscle sections sampled from quadriceps femoris showed variation in muscle fiber size with predominantly spherical shape myosites and multifocal and discrete myosite degeneration lacking infiltration of inflammatory cells . Other specific histopathologic or histochemical analyses could not be done. Eventually, he could be weaned from respirator and maintain SpO2 with ambient air at day 18 from the day of intubation. He was mechanically ventilated with control/assist control mode ventilation for the 1st 2 days and then put onto synchronized intermittent mandatory ventilation (SIMV) mode for 12 days and was on only pressure support ventilation (PSV) for the last 4 days using continuous positive airway pressure (CPAP) mode ventilation. Table 1 Laboratory investigation findings of the presented case with acute myopathy following SARS-CoV2 infection Investigations (normal value and unit of measurement) Patient value on admission and range during hospital stay Hemoglobin (13.5-17.5 g/dl) 14.5 (14.5-9.2) Total leukocyte count 21,100 (10,170-31,780) Polymorph (40-65%) 93% (62-94) Lymphocyte (30-50%) 4% (4-36) Total platelet count (150,000 to 400,000/μl) 295,000 (270,000 to 480,000/μl) C-reactive protein (< 5 mg/L) 300 (2.7-300) Serum procalcitonin (0.10-0.49 ng/mL) 5.69 (0.27-5.69) Serum ferritin (< 250 ng/mL) 811 Serum d -dimer (< 0.4 mcg/mL) 1.25 (1.25-5.55) Serum PT (11-13.5 s) 13 (13-15) Serum aPTT (25-35 s) 33 (30-33) Serum CPK (< 120 mcg/mL) 850 Serum electrolytes Na + (135-145) mmol/L 138 (134-152) K + (3.5-5.5) mmol/L 4.6 (3.5-5.1) Ca + (8.5-10.5) mg/dl 8.6 (7.6-8.6) Mg + (1.5-2.5) mmol/L 1.9 (1.9-3) Serum creatinine (60-110 μmol/L) 87 (71-155) Serum SGPT (7-56 U/L) 32 (32-45) Serum troponin-I (< 0.04 ng/ml) 0.02 (0.02-0.2) Serum Pro-BNP (< 125 pg/mL) 130 (109-130) Serum anti GM1 antibody (− ve) Cerebrospinal fluid (CSF) Total protein (up to 45 mg/dl) 10 Total WBC count (0-5/cmm) 05 PT prothrombin time, aPTT activated partial thromboplastin time, CPK creatinine phosphokinase, SGPT serum glutamic pyruvic transaminase, BNP brain natriuretic peptide, WBC white blood cells Fig. 1 Nerve conduction study, electromyogram, and disease trajectory. ( a ) Motor and sensory nerve conduction was normal despite severe muscle weakness. Compound muscle action potential (CMAP) amplitudes are measured in millivolts (mV); 2 mV per division for all motor study traces. DML, distal motor latency in ms. MCV1, motor conduction velocity in millisecond (ms). MCV2, motor conduction velocity in ms. Sensory nerve action potential (SNAP) amplitudes are measured in microvolts (μV); 20 μV per division for all sensory study traces. DSL, distal sensory latency. SCV, sensory conduction velocity. ( b ) Electromyogram (EMG) showing myopathic motor unit potentials, a full recruitment pattern and spontaneous muscle fiber activity in several sampled muscles. Motor unit potential (MUP) amplitudes are measured in microvolt (μV); 200 μV per division for all EMG traces Fig. 2 Muscle histopathology and MRI of upper thigh axial sections. ( a - d ) Muscle histopathology sections sampled from quadriceps femoris muscle stained with hematoxylin and eosin, showed variation in muscle fiber size with predominantly spherical shape myosites and multifocal and discrete myosite degeneration lacking infiltration of inflammatory cells. ( e ) T2 weighted MRI section of the upper thigh done on day 20, showed marked hyperintensity in both the quadriceps muscles. ( f ) Repeat T2-weighted MRI of the same section of the thigh muscles done after 48 days of the 1st MRI shows both the quadriceps muscles appear normal
| 3.912109
| 0.980469
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sec[1]/p[0]
|
en
| 0.999997
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33883014
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https://doi.org/10.1186/s13395-021-00266-5
|
[
"serum",
"muscle",
"motor",
"ventilation",
"high",
"admission",
"sensory",
"conduction"
] |
[
{
"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 CLASSIFICATION ═══
【1. Other serum reactions (NE80.3)】
Synonyms: Allergic reaction to serum | serum allergy | Complications of vaccination, protein sickness | Protein sickness | transfusion reaction due to serum protein reaction
Excludes: serum hepatitis
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injury or harm arising from surgical or medical care, not elsewhere classified → Injury or harm arising following infusion, transfusion or therapeutic injection, not elsewhere classified (NE80) → Other serum reactions
【2. Other specified metabolic disorders (5D0Y)】
Synonyms: Disorders of plasma-protein metabolism, not elsewhere classified | abnormal protein transport | dysproteinaemia | Absence of albumin in blood | Analbuminaemia
Hierarchy: Endocrine, nutritional or metabolic diseases (05) → Metabolic disorders → Other metabolic disorders → Other specified metabolic disorders
【3. Hypercalcaemia (5B91.0)】
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 concentrati...
Synonyms: Calcium excess | elevated serum calcium | hypercalcaemic crisis | hypercalcaemic syndrome | Burnett syndrome
Hierarchy: Overweight, obesity or specific nutrient excesses → Certain specified nutrient excesses → Mineral excesses (5B91) → Hypercalcaemia
【4. Other specified anaphylaxis (4A84.Y)】
Synonyms: Latex-induced anaphylaxis | Anaphylaxis due to latex | Latex anaphylaxis | Anaphylactic shock due to serum | anaphylactic shock due to allergy to serum
Hierarchy: Diseases of the immune system (04) → Allergic or hypersensitivity conditions → Anaphylaxis (4A84) → Other specified anaphylaxis
【5. Homocarnosinosis (5C50.F2)】
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...
Synonyms: Homocarnosinase deficiency | Serum carnosinase deficiency
Hierarchy: Inborn errors of metabolism → Inborn errors of amino acid or other organic acid metabolism (5C50) → Disorders of peptide metabolism (5C50.F) → Homocarnosinosis
|
NE80.3
|
Other serum reactions
|
This case report describes how the congenital muscular disorder can affect the growth of the craniofacial complex causing severe malocclusion. The prevalence rate in children is higher than the prevalence in adults because children with myopathy may not survive after particular age groups. The prevalence rate of congenital myopathy for children is 2.76 in 100000 in pediatric patients and for nemaline myopathy, the overall prevalence rate was 0.22 per 100,000 . Positive family history and changes in the gene pattern mutations may be the important risk factors for the congenital type of myopathy like nemaline myopathy with autosomal recessive pattern with the most common inheritance. A total number of 10 types of gene mutations have been identified for this condition. NEB-1 and ACTA 1 are the most common genes responsible for these conditions.The autosomal recessive form is more common in childhood and the autosomal dominant form is common in adulthood. For the acquired type of myopathy, the risk factor is autoimmune diseases (polymyositis, dermatomyositis, etc.), drug-induced myopathy (glucocorticoids, D-penicillamine or lipid-lowering agents, etc.), viral infections (mumps, Coxsackie, influenza virus, etc.), endocrine disorder (hypothyroidism or hyperthyroidism, etc.). Various causative factor has been noted in the literature for malocclusion including genetic disorder, hereditary conditions, environmental elements affecting the fetus's injuries, premature tooth loss, dysfunctions and parafunction of stomatognathic apparatus, and other diseases . Various researchers have noted the impact of muscular disorder involvement in the growth of the craniofacial complex causing facial dysmorphogenesis and functional malocclusion . There are few studies regarding this noted in the literature . Anterior open bites and long faces are the most common dental findings noted in most muscular disorders which is also seen in our case report . Similar to our case report, Deans et al. and Xue et al. describe the anterior open bite and long faces are characteristic features of nemaline myopathy. Currently, there is no definitive treatment for such congenital myopathy. Treatment planning needs to take into account the severity and progression of the disease. Biopsy and genetic molecular investigation are critical for the management of congenital myopathy. It comprises an interdisciplinary team of paediatric dentists, orthodontists, oral surgeons, craniofacial team neuromuscular surgeons, etc. The severe malocclusion requiring extensive orthodontic surgery should be deferred due to difficulty in intubation and tracheostomy for anaesthetics. Succinylcholine should be avoided in such cases. The anaesthetic risk and surgical stability of the procedure should be considered while planning any surgical procedure for such cases . The concomitant theory said that the reason for the anterior open bite and long faces is caused by the mandible growing downwards and forwards resulting in the increased lower facial height, marked large tongue size and low tongue position. Dystrophy of facial muscle causes the mandible clockwise rotation due to gravity or the absence of suprahyoid muscle pull. This affects the transverse position of the teeth, reducing palatal width and causing posterior crossbite in such cases. Mandibular rotation in a clockwise direction also increases the angle between the mandibular plane and the palatal plane also increases the gonial angle and contributes to the mouth-breathing habit in this case report. A triple furrow sign with the presence of tongue atrophy was also noted in the case report similar to another author . Quereshy FA et al. in their case report mentioned severe anterior open bite, high-arched palate, and mandibular retrognathia are characteristic oral features of nemaline myopathy and Yoo et al. also mentioned anterior open bite and mouth breathing habit and drooling in his case report. The current literature available on craniofacial management of nemaline myopathy is very limited. The first case report published on orthodontic surgery for nemaline myopathy in mixed dentition was by Quereshy FA et al. in the year 2020. Oral care and general care for nemaline myopathy should follow the guidelines reviewed in the consensus statement of the standard of care for congenital myopathy as there is a paucity of literature regarding standard treatment protocol for muscle disorder patients . Due to the multisystem involvement in nemaline myopathy patients require a multidisciplinary approach from various healthcare professionals to improve the quality of healthcare life. The anticipatory guidance for these patients should start from the first tooth erupts in the oral cavity. During the first year of life, feeding difficulty is the most commonly seen problem. It should be monitored with increased age otherwise alternative gastric feeding tubes should be considered. Feeding issues can impair the overall growth and development of patients. Monitoring of growth with respect to height and weight should be done at least every three months. Orofacial problems such as malocclusion, high arched palate and lower facial muscle contractures are seen as major problems in this disease. Various behaviour management techniques such as desensitizing techniques should be used for proper care of the oral cavity. Evaluation of high-arched palate and malocclusion should be at the age of six to eight years. Surgical planning for severe malocclusion should not be considered due to the high severe risk of complications such as difficulty in intubation and anaesthesia. The milder form of malocclusion should be treated after consultation with physicians to avoid respiratory difficulty for patients. Difficulty of speech problems is due to muscle hypotonia or abnormal anatomy. Referral speech pathologists should be considered at the earliest. Oral motor therapy should be considered useful to train patients with nemaline myopathy like lip exercise (oral screen) to prevent mouth breathing habitat. Excessive drooling of saliva is a common problem seen in muscle disorder patients and should be treated with pharmacologic agents such as anticholinergic drugs. Recommendations of this guideline include referral to a neurologist, pulmonologist, orthopedician, physiotherapist, occupational therapist and speech therapist should be done at the earliest to reduce morbidity. No specific pharmacologic treatment is available. Monitoring of respiratory function and spine should be done during every follow-up visit of patients. The follow-up care of patients should include the examination of cardiac status due to the risk of cardiac disease. The success of treatment for these patients requires coordinated oral and general care. The child has been recalled every three to four months for follow-up care. Only preventive type of treatment is given to the child including oral hygiene instructions and fluoride application. After consultation with the orthodontic team, extensive orthodontic surgery is delayed at an early age due to lower facial muscle weakness. Increase effort has been asked the child to produce a lip seal. The oral screen has not been given for this child due to respiratory difficulty. During the management of nemaline myopathy difficulty in intubation and complications from anaesthesia should be considered . According to Bagnall, there is no current specific intervention to overcome this structural facial progression . This case report describes the orofacial features of nemaline myopathy.
| 4.246094
| 0.923828
|
sec[2]/p[0]
|
en
| 0.999996
|
38125243
|
https://doi.org/10.7759/cureus.49091
|
[
"myopathy",
"patients",
"nemaline",
"oral",
"malocclusion",
"difficulty",
"muscle",
"care"
] |
[
{
"code": "8C7Y",
"title": "Other specified primary disorders of muscles"
},
{
"code": "8C72.Z",
"title": "Congenital myopathies, unspecified"
},
{
"code": "8C80",
"title": "Drug-induced myopathy"
},
{
"code": "8D43.3",
"title": "Myopathy due to toxicity"
},
{
"code": "8C75",
"title": "Distal myopathies"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Other specified primary disorders of muscles (8C7Y)】
Synonyms: Certain specified primary disorders of muscles | Myopathy due to calsequestrin or SERCA1 protein overload | Delayed muscle maturation | delayed muscle growth | delayed myogenesis
Hierarchy: Diseases of the nervous system (08) → Diseases of neuromuscular junction or muscle → Primary disorders of muscles → Other specified primary disorders of muscles
【2. Congenital myopathies, unspecified (8C72.Z)】
Synonyms: Congenital myopathies | benign congenital myopathy
Hierarchy: Diseases of neuromuscular junction or muscle → Primary disorders of muscles → Congenital myopathies (8C72) → Congenital myopathies, unspecified
【3. Drug-induced myopathy (8C80)】
Definition: Myopathy caused by drugs that ranges from mild myalgias with or without mild weakness to chronic myopathy with severe weakness, to massive rhabdomyolysis with acute renal failure. It could be due to several different mechanisms including direct myotoxicity, immune mediated and indirect muscle damage through drug-induced coma, drug-induced hypokalaemia, drug-induced hyperkinetic states or dystonic ...
Synonyms: drugs myopathy
Hierarchy: Diseases of the nervous system (08) → Diseases of neuromuscular junction or muscle → Secondary myopathies → Drug-induced myopathy
【4. Myopathy due to toxicity (8D43.3)】
Synonyms: Spastic paraplegia due to L-BOAA
Hierarchy: Diseases of the nervous system (08) → Nutritional or toxic disorders of the nervous system → Neurological disorders due to toxicity (8D43) → Myopathy due to toxicity
【5. Distal myopathies (8C75)】
Definition: Distal myopathies are heterogeneous group of myopathies characterised clinically by progressive weakness and atrophy starting in distal muscles and progressing to proximal ones, and histologically by nonspecific myopathic features on muscle biopsy.
Synonyms: Distal muscular dystrophy | Distal myopathy with anterior tibial onset | Markesbery-Griggs distal myopathy | Tibial muscular dystrophy | Distal myopathy with early respiratory muscle involvement
Hierarchy: Diseases of the nervous system (08) → Diseases of neuromuscular junction or muscle → Primary disorders of muscles → Distal myopathies
|
8C7Y
|
Other specified primary disorders of muscles
|
A 62-year-old male patient with no adverse medical history was admitted to our hospital with a high fever. Contrast-enhanced computed tomography (CT) revealed a liver tumor in the left lobe of the liver . Due to the presence of high fever, elevated inflammatory markers, and detection of a low-density area in the liver tumor on CT, our initial diagnosis was liver abscess. However, the detection of elevated tumor marker levels and a clear imaging of a solid tumor on abdominal ultrasonography, confirmed our diagnosis of intrahepatic malignant tumor . Laboratory findings on admission are listed in Table 1 . Further, a percutaneous ultrasound-guided needle biopsy of the liver tumor revealed it as SCC. The patient had no liver disease underlying the liver tumor. Endoscopic examinations showed that the upper and lower gastrointestinal tracts were free of primary malignancies. In addition, no head or neck malignancies were detected on CT. Fluorodeoxyglucose/positron emission tomography (FDG/PET) CT showed accumulation of FDG in the liver tumor, perihilar lymph nodes, and lymph nodes around the heart , These results confirmed our diagnosis of a primary tumor with multiple distant lymph node metastases. We suspected a diaphragmatic and pericardial invasion from the primary tumor as the tumor border was found to be obscured on CT. Therefore, we opted for systemic and TAI chemotherapies instead of surgery based on patient’s consent. The patient was administered with a TAI of 50 mg/body cisplatin (CDDP) (once every 6 weeks), for treating the primary tumor, and 120 mg/day tegafur/gimeracil/oteracil (S-1) systemically for both the primary tumor and distant metastases (repeating twice of 2-week administration and 1-week withdrawal). One course of chemotherapy was set at 6 weeks. A CT scan performed after five courses of chemotherapy, showed a reduction in the size of the primary tumor and lymph nodes. In addition, the tumor markers decreased after five courses of chemotherapy, which indicated partial remission (PR). Because no adverse events were reported from systemic and TAI chemotherapies after five courses, the treatment was continued for nine courses. A summary of treatments and changes in the tumor marker levels is shown in Fig. 3 . After 9 courses of treatment, further reduction in the size of the primary tumor and lymph nodes was seen on CT scan. Additionally, FDG/PET CT showed no accumulation of FDG in the distant lymph nodes with a reduction in their size . Due to the reduction in the size of the tumor, the patient underwent conversion surgery, in which a laparotomic left lateral segmentectomy, and resection of the diaphragm and pericardium were performed, as the tumor invasion was observed . The patient also underwent sampling of perihilar lymph nodes, which showed no cancer cells. Finally, postoperative pathology tests performed on the resected tumor revealed that the patient was affected by primary hepatic ASC. Although the patient became cancer-free by conversion surgery, he was treated with adjuvant chemotherapy with intravenous CDDP (40 mg every 6 weeks) and S-1 (120 mg/day, repeating twice of 2-week administration and 1-week withdrawal) as adjuvant chemotherapy. No tumor recurrence was observed for 6 months postoperatively. Fig. 1 Abdominal contrast-enhanced computed tomography and ultrasonography on admission. A , B Abdominal contrast-enhanced computed tomography shows a tumor with a low-density area in the left lobe of the liver ( A axial image, B sagittal image). C – E Axial images of the abdominal contrast-enhanced computed tomography shows swelling in multiple lymph nodes (white arrows). F Abdominal ultrasonography shows a hyperechoic solid tumor with 60.2 × 48.2 mm in diameter in the left lobe of the liver Table 1 Laboratory findings on the day of admission Laboratory findings Hematology Biochemistry Immunology WBC 12,590/µL AST 58 IU/L HBsAg (–) neut 81.9% ALT 51 IU/L HBsAb (–) lym 12.2% ALP 459 IU/L HBcAb (–) eosino 0.7% LDH 169 IU/L HCVAb (–) mono 4.1% γ-GTP 101 IU/L baso 0.2% TP 7.2 g/dL RBC 404 × 10 4 /µL Alb 2.9 g/dL Hb 11.9 g/dL T-bil 0.6 mg/dL Hct 37.6% D-bil 0.2 mg/dL Plt 57.5 × 10 4 /µL CRP 15.8 mg/dL Tumor marker BUN 11.7 mg/dL CEA 4 ng/mL Cre 0.81 mg/dL CA19-9 2.1 U/mL UA 4.1 mg/dL AFP 1.3 ng/mL Na 138 mEq/L PIVKA-II 12.9 mAU/mL K 4.3 mEq/L SCC 18.6 ng/mL Cl 102 mEq/L CYFRA 5.01 ng/mL PCT 0.06 ng/mL WBC, white blood cell; neut, neutrophil; lym, lymphocyte; eosino, eosinophil; mono, monocyte; baso, basophil; RBC, red blood cell; Hb, hemoglobin; Hct, hematocrit; Plt, platelet; CEA, carcinoembryonic antigen; CA19-9, carbohydrate antigen 19–9; AFP, alfa-fetoprotein; PIVKA-II, protein induced by vitamin K absence or antagonist-II; SCC, squamous cell carcinoma antigen; CYFRA, cytokeratin 19 fragment; AST, aspartate aminotransferase; ALT, alanine aminotransferase; ALP, alkaline phosphatase; LDH, lactate dehydrogenase; γ-GTP, gamma-glutamyl transpeptidase; TP, total protein; Alb, albumin; T-bil, total bilirubin; D-bil, direct bilirubin; CRP, C-reactive protein; BUN, blood urea nitrogen; Cre, creatinine; UA, uric acid; Na, sodium; K, potassium; Cl, chlorine; PCT, procalcitonin; HBsAg, hepatitis B virus surface antigenemia; HBsAb, hepatitis B virus surface antibody; HBcAb, hepatitis B virus core antibody; HCVAb, hepatitis C virus antibody Fig. 2 Abdominal fluorodeoxyglucose/positron emission tomography before systemic chemotherapy and transcatheter hepatic arterial injection. A Abdominal fluorodeoxyglucose/positron emission tomography shows a strong accumulation of fluorodeoxyglucose (FDG) in the tumor present in the left lobe of the liver. B , C Accumulation of FDG is observed in multiple lymph nodes around the heart (white arrows). D Accumulation of FDG is observed in the perihilar lymph node (white arrows) Fig. 3 Changes in tumor markers during chemotherapy, conversion surgery, and postoperative adjuvant chemotherapy. Tumor markers gradually decrease along with systemic and transcatheter hepatic arterial injection chemotherapies. After 9 courses of treatment, the patient undergoes conversion surgery. Postoperative adjuvant chemotherapy was administered with no recurrence of the tumor. CDDP, cisplatin; CT, computed tomography; PR, partial remission; S-1, Tegafur/Gimeracil/Oteracil; TAI, transcatheter hepatic arterial injection; SCC, squamous cell carcinoma antigen; CYFRA, cytokeratin 19 fragment Fig. 4 Abdominal fluorodeoxyglucose/positron emission tomography after chemotherapy and transcatheter hepatic arterial injection. A Accumulation of fluorodeoxyglucose (FDG) is observed in the primary tumor with reduced size after the treatment with systemic chemotherapy and transcatheter hepatic arterial injection. B – D Accumulation of FDG is not observed in the perihilar lymph node and multiple lymph nodes around the heart. The size of the lymph nodes is reduced after the treatment (white arrows) Fig. 5 Pathological findings of primary tumor of the liver. A Whole mount bright-field images of a solitary nodule with 21 × 20 × 18 mm in diameter. B Hematoxylin eosin (HE) staining shows adenocarcinoma (white arrows) and squamous cell carcinoma (black arrows) component. C HE staining shows adenocarcinoma. D HE staining shows squamous cell carcinoma component with a keratinization. E , F Immunostaining of carbohydrate antigen (CA) 19-9, and carcinoembryonic antigen (CEA), shows positive expression in the adenocarcinoma region. G , H Immunostaining of cytokeratin (CK) 5, and p40 shows a positive expression in the squamous cell carcinoma region
| 3.994141
| 0.971191
|
sec[1]/p[0]
|
en
| 0.999997
|
34930149
|
https://doi.org/10.1186/s12876-021-02070-3
|
[
"tumor",
"lymph",
"liver",
"chemotherapy",
"primary",
"nodes",
"tomography",
"shows"
] |
[
{
"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 CLASSIFICATION ═══
【1. Neoplasms of unknown behaviour of unspecified site (2F9Z)】
Synonyms: neoplasia | neoplasm growth NOS | tumour of unspecified site | tumourlet of unspecified site | tumour mass NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of unspecified site
【2. Subcutaneous swelling, mass or lump of uncertain or unspecified nature (ME61)】
Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature
Synonyms: localised swelling, mass or lump of skin and subcutaneous tissue | localised subcutaneous nodules | subcutaneous nodules | superficial subcutaneous nodules | localised swelling
Excludes: localized adiposity | mass and lump: breast | enlarged lymph nodes | mass and lump: intra-abdominal or pelvic | oedema
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings involving the skin → Symptoms or signs involving the skin → Subcutaneous swelling, mass or lump of uncertain or unspecified nature
【3. Carcinoma in situ of unspecified site (2E6Z)】
Synonyms: carcinoma in situ of unspecified site | carcinoma in situ NOS | carcinoma in situ | in situ neoplasm
Hierarchy: Neoplasms (02) → In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues → Carcinoma in situ of unspecified site
【4. Neoplasms of unknown behaviour of trachea, bronchus or lung (2F91.1)】
Synonyms: 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 | Lung hemangiopericytoma of unknown behaviour
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs (2F91) → Neoplasms of unknown behaviour of trachea, bronchus or lung
【5. Neoplasms of unknown behaviour of skin (2F92)】
Synonyms: skin tumour NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of skin
|
2F9Z
|
Neoplasms of unknown behaviour of unspecified site
|
Mrs X.Y. is a 70 years old, married woman who lives with her husband, without a family history of mental disorders. Since adolescence, the patient was characterized by a cyclothymic-irritable temperament, with swings in mood, energy and irritability. In addition, the patient showed since childhood behavioral pattern characterized by difficulties in socio-emotional reciprocity, empathy alterations, strong adherence to routine without flexibility, narrow and repetitive interests, described as abnormal for their intensity, ascribable to a subthreshold Autism Spectrum without an evident impairment in global functioning, showing instead hyper-functional features in specific fields. As a teenager she excelled in humanities, to the study of which she devoted many hours of the day until she reached very high school performances. She was the first of her class and this stubbornness allowed her to achieve and maintain managerial roles, with a successful academic and work career. She was also very selective in her relations with a reduced ability to share interests. Described as a ruminative person, she was incapable of coping with stressful or mildly traumatic social situations, often as a result of her difficulties in social interactions and in understanding the typical signs of verbal and non-verbal communication. The patient also reported an increased sensitivity to caffeine and intolerance to stress, increased workload or to sudden changes in daily routine. The onset of clinical symptoms was at the age of 20, when the patient developed mood oscillations of both polarities, with depressive episodes, characterized by low energies and clinophilia lasting a few weeks as well as with episodes of mood elevation, increase in energy levels and activities, reduced need for sleep in association with irritability, verbal aggressive behavior towards other people (particularly family members) and subsyndromal panic attacks with cardiorespiratory symptoms (tachycardia, dyspnea). However, the patient did not seek medical help for these symptoms and the clinical picture resolved spontaneously. Subsequently, for several decades, Mrs. X.Y. reported subjective well-being, with satisfying levels of adjustment and global functioning, despite the presence of subsyndromal mood oscillations, tendency to irritability and prodigality and anxiety fluctuation. During 2012 (when the patient was 60 years old), in association with a stressful life event, the patient experienced a progressive worsening of mood, with sadness and loneliness, social anhedonia, asthenia, irritability associated with emotional lability, recriminatory thoughts towards family members, prodigality. Furthermore, her tendency to ruminative thinking related on life events, particularly social ones, typical of ASD full-threshold and subthreshold spectrum, caused frequent mood swings and alterations in her circadian rhythms, determining subtotal insomnia. This condition progressively worsened, with the patient showing increased social withdrawal, dysphoria, verbal and physical aggressive behaviors, with a further detrimental effect on her socio-emotional functioning, anxiety elevation with cardiorespiratory and neurovegetative symptoms, leading to a reduced global functioning. However, also in this case Mrs. X.Y., firmly convinced of being affected by a cancer, did not listen to family members and did not refer to mental health professionals, undergoing instead several somatic examinations such as repeated blood tests, echocardiography, cardiovascular consultations, abdominal computed tomography, endoscopy, all of which reported negative results. In 2014 (62 years), after a period of reduced symptom severity and better adjustment, the patient progressively developed aphasia and as a consequence she was examined by a neurologist, undergoing a brain Magnetic Resonance Imaging (MRI) and Positron Emission Tomography (PET). According to neuroimaging examination, cortical metabolic changes were observable especially in the frontal and left temporal areas, presumably related to a neurodegenerative condition. Mrs. X.Y., was diagnosed with Primary Progressive Aphasia and a FTD (Semantic Variant). In April 2015 (63 years), the patient developed a new episode of the mood disorder, characterized by dysphoric mood, frequent crying, high anxiety levels, motor restlessness, aggressive behaviors and prodigality, with frequent pantoclastic episodes. Subsequently the patient was examined for the first time at a Psychiatric Clinic, where she was admitted and then discharged with a diagnosis of "BD, mixed episode, in patient with FTD, type II diabetes mellitus and essential hypertension". The pharmacological therapy featured mood stabilizers (Valproic Acid 500 mg/day), antipsychotics (Perphenazine 6 mg/day), antidepressants (Paroxetine 10 mg/day) and benzodiazepines (Lorazepam 2 mg/day), with reported clinical benefit. During the following months, the patient enjoyed a partial global functioning, despite the presence of anxiety and irritability fluctuation with episodes of verbal aggressiveness. However, from 2015 to 2018 the patient experienced other three episodes of mood alteration similar to the one reported at 63 years, in particular during spring (March–April), and a progressive worsening of the global conditions, with the development of chronic impairment in linguistic expression, increasing aggressive behaviors, apathy, abulia and hypochondriac thoughts, leading to a loss of autonomy in common daily activities and personal care, requiring continuous daily assistance. In August 2018, she was admitted to a Neurology Clinic. The diagnosis of Fronto- Temporal Cognitive Decay (semantic variant type) was confirmed. In April 2021 (69 years), the patient was admitted for a second time in a Psychiatric Clinic, where she was discharged with the diagnosis of "Bipolar Affective Syndrome, maniacal episode, moderate; aphasia" and a psychopharmacological therapy based on mood stabilizers (Lithium Carbonate 300 mg/day), antipsychotics (Clozapina 75 mg/day), antidepressants (Paroxetine 20 mg/day, Sertraline 25 mg/day) with partial clinical benefit. However, due to the remaining psychopathological symptoms, associated with severe aphasia and the progressive reduction of the overall functioning, the patient was no longer self-sufficient in her daily routine and needed constant support from her family members. In February 2022 (70 years) the caregivers seek the help of another psychiatrist who changed the psychopharmacological therapy based on mood stabilizers (Lithium Carbonate 300 mg/day, Valproic Acid 250 mg/day), antipsychotics (Clozapine 100 mg/day) and antidepressants (Paroxetine 5 mg/day). Subsequently, the patient was addressed to our clinic, where she was admitted on 4 March 2022 for the treatment and investigation of the case. At a first physical examination the patient presented catatonic symptoms, including waxy flexibility, grimacing, mutism, negativism, echolalia. The patient spoke only on few occasions, with a very poor language characterized by pass-par-tout words. Furthermore, she showed an oppositional behavior, refusing food and hydration and thus requiring enteral nutrition through nasogastric intubation, temporarily replaced with parenteral nutrition for a suspected aspiration pneumonia. She was not oriented in place, time and person, and scarcely responsive to verbal and physical stimuli. In agreement with the relatives, she was transferred to Intermediate Care and subsequently in a long-term structure.
| 4.015625
| 0.976074
|
sec[1]/p[0]
|
en
| 0.999996
|
36997907
|
https://doi.org/10.1186/s12888-023-04709-9
|
[
"mood",
"years",
"functioning",
"verbal",
"symptoms",
"family",
"characterized",
"irritability"
] |
[
{
"code": "6A8Z",
"title": "Mood disorders, unspecified"
},
{
"code": "6A8Y",
"title": "Other specified mood disorders"
},
{
"code": "MB24.8",
"title": "Elevated mood"
},
{
"code": "6D10.Z",
"title": "Personality disorder, severity unspecified"
},
{
"code": "6E62.1",
"title": "Secondary mood syndrome, with manic symptoms"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Mood disorders, unspecified (6A8Z)】
Synonyms: mood disorders, nonorganic or unspecified
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Mood disorders → Mood disorders, unspecified
【2. Other specified mood disorders (6A8Y)】
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Mood disorders → Other specified mood disorders
【3. Elevated mood (MB24.8)】
Definition: A positive mood state typically characterised by increased energy and self-esteem which may be out of proportion to the individual's life circumstances.
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Mental or behavioural symptoms, signs or clinical findings → Symptoms or signs involving mood or affect (MB24) → Elevated mood
【4. Personality disorder, severity unspecified (6D10.Z)】
Synonyms: Personality disorder | Specific personality disorders | Enduring personality change after psychiatric illness (deprecated) | Anankastic personality disorder | anancastic personality
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Personality disorders and related traits → Personality disorder (6D10) → Personality disorder, severity unspecified
【5. Secondary mood syndrome, with manic symptoms (6E62.1)】
Definition: A syndrome characterised by the presence of prominent manic symptoms such as elevated, euphoric, irritable, or expansive mood states, rapid changes among different mood states (i.e., mood lability), or increased energy or activity 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 histo...
Synonyms: Organic manic disorder | Manic symptoms | organic mood disorder of manic type | organic mood disorder | mood syndrome due to disorders or diseases not classified under Mental and behavioural disorders, with manic symptoms
Excludes: Adjustment disorder | Delirium
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Secondary mental or behavioural syndromes associated with disorders or diseases classified elsewhere → Secondary mood syndrome (6E62) → Secondary mood syndrome, with manic symptoms
|
6A8Z
|
Mood disorders, unspecified
|
Due to the sudden and unexpected onset of generalized rash, he was apprehensive and immediately set up a telehealth visit with his infectious diseases specialist who recommended the use of over-the-counter oral diphenhydramine. However, with the onset of fever and persistence of his rash, he was advised to discontinue the use of oxacillin and present to the emergency department. On arrival, he was alert, oriented to person, place, and time, and not in acute distress. He had unstable vital signs including fever at 101.2 °F, elevated blood pressure 152/87 mmHg, tachycardia with heart rate 136 beats per minute, respiratory rate 18 breaths per minute, and pulse oximetry 96% on room air. He had generalized blanchable maculopapular and morbilliform rash involving the face, trunk, upper and lower extremities, and sparing the palms, soles, and oral mucosa . He had palpable nontender lymph nodes in the cervical and inguinal regions bilaterally. His conjunctiva was not pale and sclera anicteric. He had clear vesicular breath sounds bilaterally. He had rapid and regular heart rate, normal rhythm, and normal heart sounds with no murmurs. His abdomen was obese, nontender, no palpable masses or hepatosplenomegaly, with the presence of normoactive bowel sounds. His neurological examination revealed normal cranial nerves II–XII with no neurologic deficits, 5/5 motor strength in his upper and lower extremities, normal deep tendon reflexes, strength, and sensation bilaterally. No swelling or tenderness on his spine. He had a normal gait and balance, with no evidence of cerebellar dysfunction. Hematologic lab values revealed white blood cell count 4.6 × 1000/µL , atypical lymphocytosis 0.5 × 1000/µL; 13% , eosinophilia 0.6 × 1000/µL , neutrophilic bandemia 18% [reference range 0–8%]. He had elevated transaminases including aspartate transaminase (AST) 101 U/L (reference range 0–55 U/L), alanine transaminase (ALT) 115 U/L (reference range 0–44 U/L), and alkaline phosphatase (ALP) 216 U/L (reference range 25–150 U/L). Inflammatory markers were elevated, including erythrocyte sedimentation rate (ESR) 31 20 mm/hour (reference < 20 mm/hour) and C-reactive protein (CRP) 34.5 mg/dL (reference < 8 mg/dL). He was further evaluated to rule out other potential infectious etiology, which were unrevealing as urine, blood, and PICC line catheter tip cultures showed no growth of organisms. Blood culture samples for aerobic and anerobic bacteria were obtained by venipuncture using two sets of sterile blood culture bottles and cultured in BacT-ALERT. No organism growth was seen in the blood cultures after 5 days of incubation. No fungal organisms were detected. Tests for herpes simplex virus (HSV) I and II immunoglobulin (IgM) antibody, infectious mononucleosis, rubeola IgM, urine legionella antigen, hepatitis A, B, and C panel, and CMV DNA polymerase chain reaction and IgM antibody were all negative, as well as human immunodeficiency virus (HIV) 1 and 2 antigen/antibody fourth generation test, which was nonreactive, and corona virus 2019 (COVID-19) SARS-CoV-2 was undetected (Table 1 ). Fig. 2 Thoracolumbar spine MRI during the patient’s previous hospitalization, which showed mildly enhancing central T11–T12 intervertebral disc with adjacent endplate enhancement suggestive of early discitis. No discrete epidural collection or abscess identified Table 1 Laboratory results of hematologic, biochemical, serological, and microbiological investigations during the patient’s current hospitalization and at follow-up Laboratory tests (reference range and units) Hospital Day 1 Hospital Day 2 Hospital Day 3 Follow-up Week 1 Follow-up Week 2 Complete blood count White blood cell count 4.6 4.0 4.2 6.9 7.6 Red blood cell count (4.30–5.80 million/µL) 4.39 4.61 4.14 4.22 4.31 Hemoglobin (12.5–17.0 g/dL) 13.3 13.9 12.3 12.5 13.1 Hematocrit (36.0–50.0%) 37.8 39.4 35.5 36.7 38.2 Mean corpuscular volume (80–100 fL) 86 86 86 87.0 88.6 Mean corpuscular hemoglobin (27–33 pg) 30 30 30 29.6 30.4 Mean corpuscular hemoglobin concentration (31–36 g/dL) 35 35 35 34.1 34.3 Red cell distribution width (11.4–14.4%) 13.7 13.9 14.0 14.0 13.9 Platelet count 201 193 195 296 463 Mean platelet volume (7.0–11.0 fL) 10.3 10.2 10.6 10.5 Reticulocyte count (1.6–2.6 %) 1.6 Absolute reticulocyte (35–101 billion/L) 74.7 Neutrophil count 3.4 4.0 4.8 Segmented neutrophils (40–75%) 73 24 58.2 63.4 Segmented neutrophils count 1.0 Band neutrophils (0.0–8.0%) 18 Band neutrophil count 0.7 Lymphocytes (%) 13 18 21.4 23.3 Lymphocyte count 0.6 0.7 1.5 1.8 Atypical lymphocyte count 0.5 Atypical lymphocytes (0.0–7.0%) 13 Monocytes (%) 7 11 Monocyte count 0.3 0.4 0.4 0.6 Eosinophils (%) 7 15 Eosinophil count 0.3 0.6 0.89 0.25 Basophils (%) 0 1.0 1.0 Basophil count 0.0 0.0 0.07 0.16 Metamyelocytes (%) 0 Myelocytes (%) 0 Promyelocytes (%) 0 Blast cells (%) 0 Inflammatory markers Erythrocyte sedimentation rate westergren (<20 mm/hour) 31.0 36.0 29.0 C-reactive protein (< 8.0 mg/dL) 34.5 17.3 7.7 Haptoglobin (29–370 mg/dL) 252 Basic metabolic panel Sodium (135–146 mmol/L) 135 137 139 140 Potassium (3.5–5.3 mmol/L) 3.9 4.4 4.0 4.5 Chloride (98–110 mmol/L) 101 102 100 104 Carbon dioxide (20–32 mmol/L) 22 25 24 24 Blood urea nitrogen (7–25 mg/dL) 14 12 12 8 Creatinine (0.70–1.33 mg/dL) 1.29 1.11 1.23 0.94 Estimated GFR (≥ 60 mL/minute/1.73 m 2 ) 62.0 74.4 65.7 91 Mean blood glucose (65–99 mg/dL) 140 130 118 Calcium (8.6–10.3 mg/dL) 9.2 9.5 8.9 9.5 Lactic acid (0.5–2.0 mmol/L) 1.0 1.4 Hepatic function panel Total bilirubin (0.1–1.2 mg/dL) 0.8 0.7 0.5 Direct bilirubin (0.0–0.4 mg/dL) 0.3 Aspartate transaminase (10–35 U/L) 101 82 46 29 12 Alanine transaminase (9–46 U/L) 115 128 91 37 6 Total alkaline phosphatase (25–150 U/L) 216 230 199 Total protein (6.0–8.5 g/dL) 6.8 7.3 6.4 Albumin (3.5–5.5 g/dL) 3.8 4.0 3.6 Coagulation profile PT (9.1–12.0 seconds) 10.6 INR (2.0–3.5) 1.0 APTT (25.0–35.0 seconds) 27.1 Enzyme levels Lactate dehydrogenase (< 226 U/L) 414 Creatine kinase (24–204 U/L) 20 Troponin T (< 0.030 ng/mL) <0.030 Amylase (31–124 U/L) 36 Lipase (0–59 U//L) 43 Serology Coronavirus-19 SARS COV2 (PCR) Not detected RSV nasal swab Negative CMV IgM Ab < 30.0 CMV DNA Quant PCR Negative Hepatitis A IgM Ab Negative Hepatitis B surface Ag Negative Hepatitis B surface Ab Nonreactive Hepatitis B core IgM Ab Negative Hepatitis C antibody < 0.1 HSV I and II IgM Ab < 0.91 Human herpes virus type 6, IgM < 1:10 [Negative] Human herpes virus type 6, IgG 0.96 (Equivocal) HIV 1 and 2 Ag/Ab, 4th Gen Nonreactive Infectious mononucleosis assay Negative Influenza type A Ag Negative Influenza type B Ag Negative Rubeola (measles) IgM < 0.91 Aerobic and anaerobic blood cultures No organism growth Fungal blood cultures No organism growth Peripherally inserted central catheter tip culture No organism growth Urinalysis Urine color Yellow Urine appearance Clear Urine pH 6.0 Urine specific gravity 1.015 Urine protein Negative Urine ketones Negative Urine blood Negative Urine nitrite Negative Urine bilirubin Negative Urine urobilinogen 1.0 Urine leukocyte esterase Negative Urine sediment examination Rare WBC Few bacteria Urine eosinophilic smear None seen Urine culture indicated? No Urine glucose Negative Ab antibody, Ag antigen, APTT activated partial thromboplastin time, CMV cytomegalovirus, GFR glomerular filtration rate, HIV human immunodeficiency virus, HHV-6 human herpes virus-6, HSV herpes simplex virus, INR international normalized ratio, PCR polymerase chain reaction, PT prothrombin time, RSV respiratory syncytial virus, WBC white blood cell
| 3.869141
| 0.976074
|
sec[1]/p[2]
|
en
| 0.999997
|
34955100
|
https://doi.org/10.1186/s13256-021-03202-9
|
[
"urine",
"blood",
"count",
"virus",
"reference",
"rate",
"hepatitis",
"cell"
] |
[
{
"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 CLASSIFICATION ═══
【1. Other specified clinical findings on examination of urine, without diagnosis (MF9Y)】
Synonyms: Methaemoglobinuria | Other and unspecified abnormal findings in urine | Calciuria | Cells and casts in urine | casts in urine
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings of the genitourinary system → Clinical findings on examination of urine, without diagnosis → Other specified clinical findings on examination of urine, without diagnosis
【2. Difficulties with micturition, unspecified (MF50.6Z)】
Synonyms: Other difficulties with micturition | difficulty passing urine NOS | difficulty urinating NOS | other difficulties with urination
Hierarchy: Symptoms, signs or clinical findings involving the urinary system → Abnormal micturition (MF50) → Other difficulties with micturition (MF50.6) → Difficulties with micturition, unspecified
【3. Frequent micturition (MF50.0)】
Definition: Needing to urinate more often than normal.
Synonyms: Urinary frequency | Frequency of micturition NOS | Frequent urination | Micturition frequency
Excludes: Pollakiuria
Hierarchy: Symptoms, signs or clinical findings of the genitourinary system → Symptoms, signs or clinical findings involving the urinary system → Abnormal micturition (MF50) → Frequent micturition
【4. Retention of urine (MF50.3)】
Definition: Incomplete emptying of the bladder
Synonyms: bladder retention of urine | not passing urine | unable to empty bladder | unable to pass urine | urinary retention
Hierarchy: Symptoms, signs or clinical findings of the genitourinary system → Symptoms, signs or clinical findings involving the urinary system → Abnormal micturition (MF50) → Retention of urine
【5. Haematuria, unspecified (MF50.4Z)】
Synonyms: Haematuria | blood in urine | urinary blood | haematuria NOS | urinary tract haemorrhage NOS
Hierarchy: Symptoms, signs or clinical findings involving the urinary system → Abnormal micturition (MF50) → Haematuria (MF50.4) → Haematuria, unspecified
|
MF9Y
|
Other specified clinical findings on examination of urine, without diagnosis
|
A preterm baby boy born at 31 weeks of gestational age was admitted to the neonatal intensive care unit (NICU) of Hangzhou First People's Hospital (Hangzhou, China) with dyspnoea and moaning for 19 min after birth. The birth weight was 1,610 g. The child's mother was first diagnosed with hypertrophic non-obstructive cardiomyopathy during an echocardiogram in late pregnancy. And then, she was treated with oral metoprolol. The child has an older sister, now 5 years old, with no history of similar disease. The child's maternal grandfather was diagnosed with the same disease at the age of 50 during a physical examination. The physical examination findings on admission were as follows: temperature 35.7°C, pulse 146 beats/min, respiration 65 breaths/min, blood pressure 52/26 (35) mmHg, transcutaneous oxygen saturation 80%, preterm appearance, weak cry, cyanosis of the mucous membranes of the lips and mouth, weak breath sounds on auscultation of both lungs and no rale heard. The heart rhythm was rhythmic, heart sounds were moderate, and no pathological murmur was heard in the precordial region. Routine blood test results and inflammatory marker levels [including C-reactive protein (CRP) level] were normal on the day of admission, and nucleic acid tests for Streptococcus agalactiae, cytomegalovirus and ureaplasma urealyticum in blood were negative, which together with negative stool, urine, sputum and gastric fluid cultures, ruled out the possibility of intrauterine infection. But the blood gas analysis suggested mild metabolic acidosis. Chest x-ray results showed that the translucency of both lungs was reduced, there was a ground-glass changes, and the size of cardiac shadows was in the normal range. The cardiac ultrasound results showed that the internal diameter of each atrioventricular cavity was normal in size, and a 1.8 mm diagonal fissure was seen in the middle of the interatrial septum. The left ventricular wall was asymmetrically hypertrophied, and the interventricular septum was markedly thickened (6 mm) with enhanced and coarsened echoes. The posterior left ventricular wall was 4 mm thick. No significant obstruction of the left ventricular outflow tract was seen at rest. ECG showed sinus tachycardia. The premature infant was put on nasal continuous positive airway pressure (nCPAP), but the dyspnoea progressively worsened 24 h after birth. When the inhalation oxygen concentration gradually increases to 40%, the symptoms of hypoxia still cannot be improved. Based on the clinical symptoms, signs, auxiliary examinations and treatment, the initial diagnosis of this child: 1. neonatal respiratory distress syndrome with respiratory failure (premature infant), 2. hypertrophic non-obstructive cardiomyopathy. Porcine alveolar surfactant 240 mg was administered by intratracheal drip to improve pulmonary ventilation, and soon the child's dyspnoea was reduced. When the inhalation oxygen concentration was gradually decreased, the transcutaneous oxygen saturation was maintained at more than 90%. At 30d of hospitalisation, the child weighed 2,170 g, already able to receive full intestinal nutrition and out of the ventilator, had no clinical signs of heart failure, arrhythmia, or pulmonary hypertension. Although the child's echocardiogram demonstrated localised myocardial hypertrophy, but he had no signs of obstructive cardiomyopathy at the present time, so the parents were informed of the need for regular follow-up after discharge from the hospital. The follow-up results and treatment process are shown in Table 1 . At 3 months of age, the child weighed 3,500 g, had no feeding difficulties and no cyanosis or sweating after crying. A grade 2 systolic murmur could be heard in the 3rd–4th intercostal space at the left edge of the sternum on physical examination. The second follow-up cardiac ultrasound showed asymmetric left ventricular wall hypertrophy (the anterior septum and interventricular septum were up to 8 mm at the thickest point and the posterior left ventricular wall was up to 4 mm in thickness), a rough echogenicity, a disturbed myocardial texture arrangement and hypokinesia. The left ventricular outflow tract did not show any obvious signs of obstruction at rest. The child was given the β -blocker metoprolol 3 mg/d(0.8 mg/kg.d) orally twice a day to slow down the process of myocardial hypertrophy. At 5 to 6months of age, the Chest x-ray findings indicated increased thickening of bilateral lung texture and full cardiac shadow. The electrocardiogram results showed sinus tachycardia, increased left ventricular integrated voltage, V5 lead deep q-wave, high wave of sharp T . The 3rd follow-up cardiac ultrasound results showed left atrial enlargement, systole could be seen in the anterior direction of the mitral valve motion (SAM sign means systolic anterior motion), and the posterior leaflet and the anterior leaflet showed motion reversal. The left ventricular wall was asymmetrically hypertrophied and the interventricular septum was markedly thickened. The thickest part was 11 mm and the posterior wall of the left ventricle was 6 mm thick. The dose of metoprolol tablets were increased to 6 mg/d(1.0 mg/kg.d) orally twice a day. Nine months after birth, the child showed pauses in breastfeeding, difficulty in feeding, sweating after activity or crying, no cyanosis and slow increase of body weight. The electrocardiogram was suggestive of sinus tachycardia with increased left ventricular integrated voltage. He weighed only 11 kg at 32 m. From the age of 2 after birth, the dose of metoprolol was increased to 12.5 mg/d(1.2 mg/kg.d). The patient's blood pressure was normal. A grade 3–4 systolic murmur was heard between the mitral valve area and the left sternal border since 4 months after birth. The results of the echocardiogram were reviewed regularly : the left atrium was enlarged (anteroposterior and posterior diameters of 15 mm–24 mm, LA/AO 1.33–1.80), the left ventricle and right heart were within the normal range, a bimodal anterior leaflet and a markedly diminished EPSS. The left ventricular wall was asymmetrically hypertrophied (6.0 mm–6.4 mm), the interventricular septum was markedly thickened (11.0 mm–15.4 mm), echogenic enhancement was thickened and protruded into the left ventricular outflow tract, and its motion was diminished. At the age of 4 years, echocardiography showed severe hypertrophic obstructive cardiomyopathy. Anzhen Hospital in Beijing, China, organized Multi-Disciplinary Treatment(MDT) and developed personalized treatment plans. At that time, considering the young age of the patient and the high risk of surgical surgery, after full communication with the patient's parents, a consensus was reached on percutaneous myocardial septal ablation guided by ultrasound. Postoperative cardiac ultrasound showed that the internal diameters of each atrium were normal, and the wall of the left ventricle was thick, with a thickness of 13 mm at the basal segment of the ventricular septum, 11.2 mm in the mid-segment, and 9 mm in the apical segment. The patient did not have any SAM symptoms, and the ventricular wall motion was normal. The anterior mitral valve flowed smoothly without an obvious regurgitation signal. The tricuspid valve was seen to have a trace regurgitation signal. The inner diameter of the left ventricular outflow tract is 13 mm, and the blood flow is smooth. The electrocardiogram result was sinus rhythm. Regular follow-up is still required after surgery. If necessary, this procedure can be repeated.
| 4.066406
| 0.976563
|
sec[1]/p[0]
|
en
| 0.999997
|
38094187
|
https://doi.org/10.3389/fped.2023.1295539
|
[
"ventricular",
"child",
"wall",
"results",
"septum",
"birth",
"blood",
"cardiac"
] |
[
{
"code": "LA89.Z",
"title": "Functionally univentricular heart, unspecified"
},
{
"code": "BC45",
"title": "Cardiomegaly"
},
{
"code": "BA41.Z",
"title": "Acute myocardial infarction, unspecified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Functionally univentricular heart, unspecified (LA89.Z)】
Synonyms: Functionally univentricular heart | Univentricular cardiopathy | Single ventricle | univentricular heart | Common ventricle
Hierarchy: Structural developmental anomalies of the circulatory system → Structural developmental anomaly of heart or great vessels → Functionally univentricular heart (LA89) → Functionally univentricular heart, unspecified
【2. Cardiomegaly (BC45)】
Synonyms: enlargement of heart | hypertrophic heart | heart hypertrophy | Cardiac hypertrophy | idiopathic cardiac hypertrophy
Hierarchy: Diseases of the circulatory system (11) → Diseases of the myocardium or cardiac chambers → Cardiomegaly
【3. Acute myocardial infarction, unspecified (BA41.Z)】
Synonyms: Acute myocardial infarction | cardiac attack | heart attack | acute cardiac infarction | acute MI - [myocardial infarction]
Hierarchy: Ischaemic heart diseases → Acute ischaemic heart disease → Acute myocardial infarction (BA41) → Acute myocardial infarction, unspecified
|
LA89.Z
|
Functionally univentricular heart, unspecified
|
The patient discussed in this case report is a frail 86-year-old female with a past medical history significant for dementia, multiple falls, recurrent urinary tract infections (UTIs), depression, cardiac arrhythmia, macular degeneration, chronic pain, depression, and cerebrovascular disease. CD lives independently at home along with her adult daughter who previously suffered a traumatic brain injury. CD was enrolled in a medication management program on 03/27/2020. At that time, the patient and her daughter had a caregiver assisting for 3 h every day. The caregiver ensured the appropriate medications were taken in the morning and organized the evening doses for self-administration. However, the patient was unable to safely self-administer medications and was therefore referred to the medication management program. Upon enrollment, an electronic medication box that rotates and alarms at set times was provided to help manage medications. CD had nine chronic medications at enrollment ( Table 1 ), including one anticholinergic (loratadine). Although loratadine is an anticholinergic medication, it is considered a safer alternative when an antihistaminic is necessary to prescribe. None of CD’s initially reviewed medications were potentially inappropriate for her based on the 2019 Beers List . The timeline of events relevant for this case is described below, and a summary is included in the supplementary table ( Supplementary Table S1 ). • April 20, 2020: CD’s initially reported medication list remained unchanged until this time. The caregiver alerted the pharmacist that the patient was having trouble sleeping. Melatonin was changed from a quick-dissolve tablet to a capsule due to the patient’s inability to appropriately use medications by sublingual route. • April 21, 2020: Upon scheduled delivery, the caregiver notified the pharmacist the patient had experienced a fall the weekend prior and injured her shoulder. The caregiver also mentioned a suspected UTI due to the patient having cloudy urine. The caregiver contacted the primary care provider (PCP) about the patient’s signs and symptoms. In order to limit exposure during the COVID-19 pandemic, the PCP requested a urine sample to be dropped off in place of a traditional appointment. The following day, the PCP confirmed the presence of a UTI and prescribed cephalexin 250 mg capsules by mouth three times daily for 7 days. The pharmacist simultaneously initiated a 30-days course of probiotics in an attempt to prevent a Clostridium difficile infection. In addition, the PCP placed CD’s donepezil on hold and expressed concern that the medication may be contributing to the patient’s falls and lack of appetite. At the time of the initial writing of this report, the cause of CD’s fall was unknown. Possible causes include drug-drug interactions due to polypharmacy (see Table 1 ), UTI, and decreased cognitive function. • April 24, 2020: The caregiver notified the pharmacist that the acetaminophen ER 650 mg tablets and lidocaine patches were not sufficiently relieving the patient’s pain that resulted from her fall. On April 28, 2020, the patient’s PCP prescribed tramadol 50 mg tablets by mouth three times daily as needed. At this time, the patient transitioned to 24-h, around-the-clock care. The original caregiver extended her daytime hours, and additional help was hired for evening assistance. • May 12, 2020: The caregiver notified the pharmacist about the patient’s unimproved state – she was still confused, lethargic, and had poor appetite. The pharmacist suggested UTI test strips to evaluate the potential of a refractory UTI from April 22, 2020. • May 13, 2020: The test strips were delivered to the patient’s residence by the pharmacist. The patient tested positive for nitrites and leukocytes. The pharmacist suggested a urinalysis, and culture and sensitivity after learning of the positive result. • May 14, 2020: The PCP prescribed sulfamethoxazole/trimethoprim 400/80 mg by mouth two times daily for 10 days and instructed the caregiver to re-check for infection three days after completing the course of antibiotics. • May 18, 2020: Upon scheduled delivery, the pharmacist incidentally noticed four bottles of liquid diphenhydramine that were not supplied by the pharmacy or recorded on the patient’s initial medication list. The caregiver informed the pharmacist she had seen it advertised for sleep. According to the caregiver, the patient had been taking 50 mg by mouth nightly for approximately one week. However, the exact initiation date remains unclear, as the diphenhydramine was not cleared by the pharmacist prior to use. The pharmacist discussed the potential adverse effects of anticholinergic medications in older adults with dementia (including increased risks for falls, urinary retention, and confusion) and suggested tapering the diphenhydramine to 25 mg nightly for a short period followed by discontinuation. Diphenhydramine is on the 2019 Beers List as a potentially inappropriate medication in due to its highly anticholinergic properties . • May 21, 2020: The patient was prescribed megestrol 400 mg/10 ml by mouth daily for appetite stimulation, along with a refill for tramadol. The prescriptions were sent in by a palliative care provider. Of note, a prior authorization was required for the megestrol, so the patient did not receive the medication prior to hospitalization of May 27, 20 (see below). In addition, the pharmacist had concerns that another medication was being added to CD’s already complex regimen without trying to find the root cause of her symptoms. In addition, megestrol is listed on the 2019 Beers List for increased risk of thrombotic events and possibly death in older adults. • May 22, 2020: The caregiver alerted the pharmacist of the patient’s consistently low blood pressure (∼90/60 mmHg). The nurse practitioner on the palliative care team instructed the patient’s metoprolol be held. Upon review of the patient’s medication list, the pharmacist also suggested holding lisinopril and contacting the provider to further evaluate. At this time, the caregiver confirmed diphenhydramine was tapered and discontinued. • May 27, 2020: CD’s conditioned continued to decline, and she was admitted to the hospital with a diagnosis of sepsis secondary to Gram-negative UTI. She also had hypokalemia. CD was treated with intravenous antibiotics while inpatient. • June 01, 2020: CD was discharged from the hospital at 10:00 pm with hospice referral. The patient was sent home with three prescriptions (a new order for potassium and decreased doses of metoprolol and lisinopril) from the hospital’s “Meds to Beds” service. • June 02, 2020: The pharmacist contacted the caregiver to check in and was informed of the patient’s discharge the night before. The pharmacist then attempted to contact the hospice nurse twice but did not receive a call back until after the home visit was already completed. Upon delivery, the pharmacist found handwritten notes from the hospice nurse on the medication list provided by the medication management program. The pharmacist reconciled the notes with the discharge paperwork from the hospital to ensure accuracy. • June 08, 2020: lisinopril, metoprolol, and pravastatin were all discontinued per hospice. Per the pharmacist’s recommendations, CD’s doses of Eliquis were spaced to 9:00 am and 9:00 pm (rather than 9:00 am and 6:00 pm) and the oral potassium was changed to 20 mEq twice daily rather than 40 mEq once daily to reduce GI upset ( Table 2 ).
| 3.941406
| 0.977051
|
sec[1]/p[0]
|
en
| 0.999997
|
33935693
|
https://doi.org/10.3389/fphar.2021.584667
|
[
"pharmacist",
"caregiver",
"medication",
"medications",
"list",
"daily",
"time",
"april"
] |
[
{
"code": "QE52.0",
"title": "Caregiver-child relationship problem"
},
{
"code": "QF27",
"title": "Difficulty or need for assistance at home and no other household member able to render care"
},
{
"code": "QC04.6",
"title": "Immunization not carried out because of caregiver refusal"
},
{
"code": "QA1Y",
"title": "Contact with health services for other specified counselling"
},
{
"code": "QA00.0",
"title": "General adult medical examination"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Caregiver-child relationship problem (QE52.0)】
Definition: Substantial and sustained dissatisfaction within a caregiver-child relationship, including a parental relationship, associated with significant disturbance in functioning.
Synonyms: parent-child relationship problem | Caregiver-child relationship problem with current caregiver | Caregiver-child relationship problem with former caregiver
Hierarchy: Factors influencing health status → Problems associated with relationships → Problem associated with interpersonal interactions in childhood (QE52) → Caregiver-child relationship problem
【2. Difficulty or need for assistance at home and no other household member able to render care (QF27)】
Synonyms: lack of care in home | lack of person able to render necessary care | dependent on care provider and no other household member able to render care | caregiver burn out
Hierarchy: Factors influencing health status or contact with health services (24) → Factors influencing health status → Difficulty or need for assistance with activities → Difficulty or need for assistance at home and no other household member able to render care
【3. Immunization not carried out because of caregiver refusal (QC04.6)】
Hierarchy: Reasons for contact with the health services → Contact with health services related to immunizations or certain other prophylactic measures → Immunization not carried out (QC04) → Immunization not carried out because of caregiver refusal
【4. Contact with health services for other specified counselling (QA1Y)】
Synonyms: Consanguinity counselling | Medical counselling | medical advice NOS | Health advice | health education
Hierarchy: Factors influencing health status or contact with health services (24) → Reasons for contact with the health services → Contact with health services for counselling → Contact with health services for other specified counselling
【5. General adult medical examination (QA00.0)】
Definition: Encounter for periodic examination (annual) (physical) and any associated laboratory and radiologic examinations on adult.
Synonyms: adult health checkup | check-up adult | general adult health examination | health check-up, adult NOS | adult medical examination
Excludes: Routine child health examination | Routine general health check-up of defined subpopulation | Routine newborn health examination | Symptoms, signs or clinical findings, not elsewhere classified
Hierarchy: Reasons for contact with the health services → Contact with health services for purposes of examination or investigation → General examination or investigation of persons without complaint or reported diagnosis (QA00) → General adult medical examination
|
QE52.0
|
Caregiver-child relationship problem
|
Mirror syndrome is uncommon and underreported, so the true incidence is difficult to ascertain. Despite recent advances in ultrasound and genetics, its pathology and etiology have yet to be determined. A variety of reasons leading to non-immune hydrops are described, such as chromosomal abnormalities, infection, fetal arrhythmias, and structural abnormalities (aneurysm of Galen’s vein, sacrococcygeal teratoma) . There are still many cases where the etiology of fetal hydrops/mirror syndrome has not been clarified. Although no conclusive cause of Mirror syndrome has been found, the edematous placenta is alleged to play a provocative role in the appearance of maternal edema mediated through circulating angiogenic factor . It is not easy to differentiate between mirror syndrome and preeclampsia. Clinical appearances of this illness are complex, including weight increase and edema, complemented by elevated blood pressure, with or without proteinuria, tachycardia, tachypnea, and oliguria. Laboratory values typically show signs of hemodilution with mild or moderate anemia, hypoalbuminemia, hyperuricemia, and higher human chorionic gonadotrophin (hCG) levels. Therefore, this disease can be simply mistaken for preeclampsia, making it difficult to diagnose and manage. This patient developed hydrops fetalis early in the pregnancy at 16 weeks of gestation. According to the literature reports, the gestational age for the onset of this syndrome ranges from 16 to 34 weeks . Due to poor compliance, the patient missed all of her further appointments until 26 weeks, when she presented in our unit. This is a classic situation of severe fetal hydrops that can cause maternal complications like “mirror syndrome”. With standard prenatal care, it is reasonable to believe that mirror syndrome would have been identified at an earlier gestational age, although probably with the same outcome, as the patient decided to continue the pregnancy until an emergency cesarean section was required. In several reported cases, treatment and improvement of fetal hydrops have led to the resolution of maternal symptoms . The decision to start MgSO 4 implies delivery of the fetus. This was a sensible decision as the mother’s condition worsened over the night despite amniodrainage. In mirror syndrome, liver function and platelets are usually unaffected . In our patients, liver enzymes have reached staggering values, making the differential diagnosis even more difficult. Mirror syndrome or preeclampsia is not a straightforward diagnosis. Many overlapping signs and symptoms might mislead clinicians. Hydrops fetalis was noticed at 16 weeks of gestation. At the time of admission, she presented various signs and symptoms, which could lead physicians toward one diagnosis or another. There was no proteinuria and the blood pressure was normal. Upon admission and amniodrainage, she did develop raised blood pressure and a clinical picture suggestive of preeclampsia. Therefore, MgSO 4 protocol was initiated. Looking at the chronology of events starting at 16 weeks of gestation, admission time, caesarean section, and postpartum events, we concluded that this case started initially as a mirror syndrome. Generally, maternal symptoms tend to disappear shortly after successful management of fetal hydrops or removal of the placenta. This was not our case . At the time of cesarean section, the patient was already in a life-threatening condition with liver and kidney insufficiency. Eisenmenger syndrome diagnosis came as an extra burden on an already critical patient. A low left ventricular ejection fraction (LVEF) is associated with a mortality of between 9 and 30% . Neither the doctors nor the patient were aware of the large VSD. Chest X-ray, ECG, and echocardiogram were not performed on admission. Bearing in mind the diagnosis mentioned above and the continuous deterioration of the patient’s condition, a decision was made to transfer the patient to ICU in a different hospital, where a coat was available. Although her RT PCR COVID-19 test was negative at admission, a second test was performed prior to her transfer, which turned out to be positive. During her admission in our unit, she did not present any apparent signs of COVID-19 infection. The negative PCR test and her clinical picture suggestive for mirror syndrome/preeclampsia might have misled clinicians. Pregnancy and obesity represent risk factors for severe illness from COVID-19. Pregnant women have a higher chance of death, pneumonia, and ICU admission, as shown by Poon et al. . When SARS-CoV-2 infection was diagnosed, our patient already met criteria for ICU transfer. As soon as she arrived in the ICU, she was started on heart failure treatment, CRRT, high flow canula, supportive treatment, and later, on mechanical ventilation. Her hepatic function worsened during hospitalization. COVID-19 predominantly affects the pulmonary tract causing mainly respiratory symptoms; nevertheless, involvement of other organs have been described, including the liver. In a study published by Yadav et al., liver biochemistry abnormalities were noted in as many as 76% of cases . The patient’s liver function test was already affected on admission. Most likely elevated liver enzymes were the result of multiple conditions affecting the patient. In patients with Eisenmenger syndrome, hepatic alterations are the consequence of cyanosis and hypoxemia due to the intra cardiac right-to-left shunt, low cardiac output with inadequate liver perfusion, and liver congestion in right heart failure or chronically elevated central venous pressure . While COVID-19 can affect liver function as well, we believe that, in our case, the patient’s progress towards hepatic insufficiency would have taken place even in the absence of SARS-CoV-2 infection, as her situation was already life-threatening. The highest risk lies in a patient with Eisenmenger syndrome during delivery and the early postpartum period due to large hemodynamic changes. Presumably, this is what lead to the sudden deterioration of her condition. The mortality rate for pregnant women with Eisenmenger syndrome is around 30–50%. For a patient diagnosed with additional conditions, these figures are even higher . Our patient was diagnosed with mirror syndrome on admission. Later during hospitalization, her condition evolved to preeclampsia, hepatic insufficiency, and Eisenmenger syndrome. SARS-CoV-2 infection was rather an incidental finding, and it is difficult to establish how much COVID-19 contributed to the patient’s collapse, if it contributed at all. Considering the diagnosis mentioned above, we can conclude that this critically ill patient had an extremely guarded prognosis even in the absence of COVID-19 infection. There are a lot of unknown answers in terms of organ pathology and system insufficiency in this patient. Unfortunately, an autopsy, which could have brought up more information, was not performed as this is the protocol for COVID-19 patients. Previous articles have described the association between mirror syndrome and heart failure. While in the case described by Xu et al., the patient had unspecified heart surgery in childhood, in the case published by Li, there was no obvious cause of heart failure . Though the etiology of mirror syndrome remains obscure, it is sensible to consider that mirror syndrome and its multiple effects such as pulmonary edema, anemia, hemodilution, and hypoalbuminemia can only worsen the outcome of patients with heart abnormalities.
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|
en
| 0.999998
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34684068
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https://doi.org/10.3390/medicina57101031
|
[
"syndrome",
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"liver",
"admission",
"covid",
"hydrops",
"infection",
"preeclampsia"
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[
{
"code": "DA90.0",
"title": "Syndromic diarrhoea"
},
{
"code": "1D9Z",
"title": "Unspecified viral infection of unspecified site"
},
{
"code": "LD27.0Y",
"title": "Other specified ectodermal dysplasia syndromes"
},
{
"code": "5A44",
"title": "Insulin-resistance syndromes"
},
{
"code": "LD24.04",
"title": "Chondrodysplasia punctata"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Syndromic diarrhoea (DA90.0)】
Definition: Syndromic diarrhoea (SD), also known as phenotypic diarrhoea (PD) or tricho-hepato-enteric syndrome (THE), is a congenital enteropathy presenting with early-onset of severe diarrhoea requiring parenteral nutrition (PN), associated with facial dysmorphism, woolly and poorly pigmented hair and liver disease, with extensive fibrosis or cirrhosis, in about half of the patients.
Synonyms: Phenotypic diarrhoea | THE - [tricho-hepato-enteric] syndrome
Hierarchy: Diseases of the digestive system (13) → Diseases of small intestine → Nonstructural developmental anomalies of small intestine (DA90) → Syndromic diarrhoea
【2. Unspecified viral infection of unspecified site (1D9Z)】
Synonyms: viral infection NOS | viral disorder NOS | disease caused by virus | unspecified viremia | Viraemia NOS
Hierarchy: Certain infectious or parasitic diseases (01) → Certain other viral diseases → Viral infection of unspecified site → Unspecified viral infection of unspecified site
【3. Other specified ectodermal dysplasia syndromes (LD27.0Y)】
Synonyms: Absence of fingerprints-congenital milia syndrome | Ackerman syndrome | ADULT syndrome | Acro-dermato-ungual-lacrimal-tooth syndrome | Pigment anomaly - ectrodactyly - hypodontia
Hierarchy: Multiple developmental anomalies or syndromes → Syndromes with skin or mucosal anomalies as a major feature (LD27) → Ectodermal dysplasia syndromes (LD27.0) → Other specified ectodermal dysplasia syndromes
【4. Insulin-resistance syndromes (5A44)】
Synonyms: Insulin-resistance syndrome type A | Insulin-resistance syndrome type B | Rabson-Mendenhall syndrome | Laminopathy type Decaudain-Vigouroux | Laminopathy with severe metabolic syndrome and myopathy
Hierarchy: Endocrine, nutritional or metabolic diseases (05) → Endocrine diseases → Other disorders of glucose regulation or pancreatic internal secretion → Insulin-resistance syndromes
【5. Chondrodysplasia punctata (LD24.04)】
Synonyms: chondrodysplasia punctata (stippled epiphyses) group | chondrodysplasia punctata congenita | dysplasia punctata epiphysis | dysplasia punctata | dysplasia epiphysealis punctata
Hierarchy: Multiple developmental anomalies or syndromes → Syndromes with skeletal anomalies as a major feature (LD24) → Syndromes with micromelia (LD24.0) → Chondrodysplasia punctata
|
DA90.0
|
Syndromic diarrhoea
|
The patient was a 31-year-old Chinese male logger. He was in good health and was from Xiangyang city, Hubei Province, China. The patient was admitted to the hospital on January 31, 2022 after experiencing fever for 1 week (body temperature up to 38.6 ℃) and severe headache (primarily in the forehead) for 3 days. He had also experienced nausea and projectile vomiting several times. These symptoms had not improved after oral medication (details not known), and the patient presented with fever on admission. He underwent lumbar puncture at a local hospital for cerebrospinal fluid (CSF) examination. The CSF pressure was 350 mmH 2 O , and the cell count was 118/µL. Biochemical analysis of the CSF revealed 2.15 mmol/L glucose, 119.4 mmol/L chloride, and 0.81 g/L albumin. The cranial MRI showed small ischemic lesions in the bilateral frontal lobes, and meningitis was suspected. The symptoms did not improve after injections of ganciclovir, 20% mannitol, and furosemide. The patient was transferred to the emergency department of our hospital on January 31, 2022. Since the cranial CT scan showed no significant abnormalities, he was eventually admitted to the neurology department. Physical examination on admission revealed a temperature of 36.3 °C, clear consciousness but weak spirit, neck resistance, two fingers of the chin to chest distance, equally rounded pupils about 3 mm in diameter, reactive to light muscle strength of level 5 in the four limbs, normal muscle tone, and negative Brudzinski’s sign. The results of blood test were as follows: 9.9 × 10 9 /L white blood cells (WBC, normal (4–10) × 10 9 /L), 143 g/L hemoglobin (Hb, normal 130-175 g/L), 363 × 10 9 /L platelets (PLT, normal (100–300) × 10 9 /L), 44.16 mg/L C-reactive protein (CRP, normal 0-5 mg/L), 0.62 mg/LFEU D-dimer, 14.9 s prothrombin time (PT), 0.031 ng/mL procalcitonin (PCT, normal < 0.05 ng/mL), 39.9 U/L alanine aminotransferase (ALT, normal 4–48 U/L), 13.5 U/L aspartate aminotransferase (AST, normal 4–42 U/L), 68.5U/L alkaline phosphatase (AKP, normal 34–121U/L), 102.4U/L glutamyl transpeptidase (GGT, normal 4–60U/L), 154.2U/L α-hydroxybutyrate dehydrogenase (HBDH, normal 74–199U/L), 37.2U/L creatine kinase (CK, normal 22–269U/L), 191.6U/L lactate dehydrogenase (LDH, normal 109–245U/L), 32.2 × 10 6 /L CD4 + T lymphocytes, negative anti-HIV antibody, and negative syphilis antibody. Based on these findings, intracranial infection was the first diagnosis, with cryptococcal meningitis (CM), tuberculous meningitis, or viral meningitis as the possibilities. The patient was given two intravenous injections of 0.25 g ganciclovir and one of 2 g ceftriaxone sodium on 31 January. Furthermore, 250 ml 20% mannitol was administered three times daily, and 250 ml glycerol fructose was administered twice daily to reduce intracranial pressure. Therapeutic lumbar puncture was performed several times to drain the CSF and relieve intracranial hypertension (Table 1 ). On February 2, the lumbar puncture revealed CSF pressure of more than 400 mmH 2 O and a positive ink stain. The patient was transferred to our department on February 3 and diagnosed with CM following consultation. Therefore, ganciclovir and ceftriaxone sodium were discontinued, and the treatment was switched to intravenous injection of 35 mg amphotericin B daily (progressively increased to 0.25 mg/kg twice daily) and 1.5 g oral fluorocytosine four times daily. Lumbar puncture was again performed on February 6, and the CSF pressure was 200 mmH 2 O, with positive ink stain, negative GeneXpert, and positive for Cryptococcus capsular antigen . The CSF was positive for Cryptococcus culture on February 8, and the drug sensitivity test was positive for amphotericin B, 5-fluorocytosine, fluconazole, itraconazole and voriconazole. The symptoms of fever, headache, orbital pain, or vomiting ceased after the treatment, and the CSF pressure steadily decreased and the Cryptococcus culture was negative multiple times. However, the patient developed headache and projectile vomiting on February 21, and lumbar puncture revealed CSF pressure of more than 400 mmH 2 O, positive ink stain, and negative for Cryptococcus capsular antigen (titer of 1:640). Contrast-enhanced cranial MRI showed patchy shadows in bilateral internal capsules and the right basal ganglia on February 25, and cryptococcal infection was considered . Due to the possibility of PIIRS, 5 mg dexamethasone sodium phosphate injection was added to the intensive anti-cryptococcal therapy. The patient no longer experienced headaches, orbital pain or vomiting. Based on these findings, a definitive diagnosis of CM was made. Contrast-enhanced cranial MRI on March 14 showed patchy shadows in bilateral internal capsules and right basal ganglia, which were smaller than previously observed. Therefore, dexamethasone sodium phosphate injection was discontinued and anticryptococcal treatment was continued. The patient started to experience headache and orbital discomfort on March 21, which were alleviated once 5 mg sodium phosphate dexamethasone was re-initiated. The treatment was switched to 28 mg oral methylprednisolone daily on March 23, and the patient did not experience any apparent discomfort. The patient was discharged on March 29 with instructions to take 800 mg oral fluconazole and 24 mg oral methylprednisolone. Table 1 Cerebrospinal fluid test Date Pressure (mmH 2 O) Ink stain Karyocyte (cell/uL) Percentage of neutrophils (%) Lymphocyte percentage (%) Protein content (0.20–0.40 g/L) Glucose (2.50–4.40 mmol/L) Chloride (120.0–130.0 mmol/L) Lactate dehydrogenase (3.0–40.0U/L) Adenosine deaminase (4.0–24.0U/L) Culture titers January 28 350 – 118 35.0 60.0 0.81 2.15 119.4 34.2 / – February 2 > 400 + 300 35.0 60.0 0.70 1.25 120.6 36.2 1.2 + February 6 90 + 55 36.0 59.0 0.80 1.01 120.3 31.8 1.1 + 1:1280 February 10 270 + 5 – – 0.60 1.59 122.5 21.2 1.6 – – February 16 175 + 80 2.0 93.0 0.70 2.26 124.1 20.7 1.2 – – February 21 > 400 + 100 2.0 98.0 0.50 2.46 121.4 17.3 0.3 – 1:640 February 24 290 + 55 6.0 82.0 0.80 1.48 122.2 16.3 0.5 – - March 1 160 + 95 2.0 96.0 0.70 2.83 125.1 15.3 0.5 – 1:320 March 7 130 + 20 – – 0.70 3.08 120.6 16.9 0.5 – – March 13 150 + 15 – – 0.50 3.29 124.6 15.3 0.3 – 1:160 March 20 350 + 70 2.0 96.0 0.40 2.18 121.3 15.4 0.1 – 1:160 March 27 200 + 30 – – 0.40 3.15 128.4 14.7 0.3 – - April 14 180 – 5 – – 0.50 3.00 125.7 14.9 0.3 – – May 17 230 – 5 – – 0.34 2.90 130.7 14.6 2.3 – 1:80 July 4 300 – 10 – – 0.46 2.47 125.4 13.3 - – – September 27 250 – 0 – – 0.36 3.29 121.8 13.0 1.7 – 1:20 Fig. 1 Cranial MRI images A – F . A January 28, 2022—no significant patchy shadows in the bilateral internal capsules and the right basal ganglia. B February 25, 2022—patchy abnormal shadows in bilateral internal capsules and right basal ganglia (arrows), which was diagnosed as Cryptococcal infection. C March 14, 2022—smaller patchy abnormal shadows in bilateral internal capsules and right basal ganglia (arrows). D May 17, 2022—patchy shadows in bilateral internal capsules and the right basal ganglia (arrows). The lesions were smaller than before. E July 6, 2022—patchy shadows in bilateral internal capsules and the right basal ganglia (arrows). The lesion in the right basal ganglia was slightly smaller than before, and the left lesion was roughly similar as before. F September 25, 2022—patchy shadows in bilateral internal capsules and the right basal ganglia (arrows). The lesions were significantly smaller than before. Note: Magnetic resonance imaging
| 3.912109
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|
sec[1]/p[0]
|
en
| 0.999998
|
37542340
|
https://doi.org/10.1186/s13256-023-04066-x
|
[
"february",
"march",
"basal",
"ganglia",
"patchy",
"shadows",
"internal",
"capsules"
] |
[
{
"code": "3A21.Y",
"title": "Other specified acquired haemolytic anaemia, non-immune"
},
{
"code": "FB80.A",
"title": "Stress fracture, not elsewhere classified"
},
{
"code": "EC30",
"title": "Epidermolysis bullosa simplex"
},
{
"code": "8A00.1Y",
"title": "Other specified atypical parkinsonism"
},
{
"code": "2F72.Y",
"title": "Other specified neoplasms of uncertain behaviour of skin"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Other specified acquired haemolytic anaemia, non-immune (3A21.Y)】
Synonyms: Infectious non-immune haemolytic anaemia | Drug-induced nonautoimmune haemolytic anaemia | nonautoimmune haemolytic anaemia due to drugs | acquired nonautoimmune haemolytic anaemia due to drugs | drug-induced enzyme deficiency anaemia
Hierarchy: Haemolytic anaemias → Acquired haemolytic anaemia → Acquired haemolytic anaemia, non-immune (3A21) → Other specified acquired haemolytic anaemia, non-immune
【2. Stress fracture, not elsewhere classified (FB80.A)】
Synonyms: Stress fracture NOS | fatigue fracture | Stress fracture, not elsewhere classified, multiple sites | Stress fracture, not elsewhere classified, shoulder region | Stress fracture, not elsewhere classified, clavicle
Excludes: stress fracture of vertebra
Hierarchy: Diseases of the musculoskeletal system or connective tissue (15) → Osteopathies or chondropathies → Certain specified disorders of bone density or structure (FB80) → Stress fracture, not elsewhere classified
【3. Epidermolysis bullosa simplex (EC30)】
Definition: Epidermolysis bullosa simplex is the name given to a heterogeneous group of genetically-determined defects in epidermal cell-cell adhesion. These give rise to blistering in response to frictional and shearing stresses.
Synonyms: Epidermolytic epidermolysis bullosa | Suprabasal epidermolysis bullosa simplex | Suprabasal EBS | Acantholytic epidermolysis bullosa | LAEB - [lethal acantholytic epidermolysis bullosa] due to desmoplakin deficiency (MIM 609638)
Hierarchy: Diseases of the skin (14) → Genetic or developmental disorders affecting the skin → Genetically-determined epidermolysis bullosa → Epidermolysis bullosa simplex
【4. Other specified atypical parkinsonism (8A00.1Y)】
Synonyms: Corticobasal degeneration | CBD - [corticobasal degeneration] | Calcification of basal ganglia | Olivopontocerebellar atrophy | Déjerine-Thomas atrophy
Hierarchy: Movement disorders → Parkinsonism (8A00) → Atypical parkinsonism (8A00.1) → Other specified atypical parkinsonism
【5. Other specified neoplasms of uncertain behaviour of skin (2F72.Y)】
Synonyms: Atypical fibroxanthoma | Kaposiform haemangioendothelioma | Basal cell tumour of unspecified site | Composite haemangioendothelioma of unspecified site | Dabska tumour
Hierarchy: Neoplasms (02) → Neoplasms of uncertain behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of uncertain behaviour of skin (2F72) → Other specified neoplasms of uncertain behaviour of skin
|
3A21.Y
|
Other specified acquired haemolytic anaemia, non-immune
|
We describe a case of acute arthritis after a SARS-CoV2 infection in a 60-year-old Caucasian man without relevant comorbidities (Table 1 ). In April 2020, he was hospitalized for hyperpyrexia, headache, asthenia, and a worsening dyspnea. At the emergency room (ER), the thoracic ultrasound and chest X-ray revealed an interstitial pneumonia; a nasopharyngeal swab was positive for SARS-CoV2 and his blood test revealed a marked inflammatory state characterized by CRP (C-reactive protein) 240 mg/L, fibrinogen 9.83 g/L, interleukin-6 162 ng/L, ferritin 944 μg/L, and D-dimer 993 μg/L. He was admitted to the Internal Medicine department and treated with azithromycin, ceftriaxone, hydroxychloroquine (HCQ) (400 mg/die), anticoagulation for thromboembolism prophylaxis, and low-flow oxygen. For progressive respiratory failure, he was referred to the intensive care unit where he underwent nasotracheal intubation and received broad-spectrum antibiotics (meropenem, linezolid), antimycotic prophylaxis, continuous diuretic infusion, noradrenalin for hemodynamic support, and therapeutic dose of anticoagulants for elevation in D-dimer values. Due to a progressive improvement of respiratory gas exchange and chest X-ray, he was extubated after 10 days. He was discharged in good general conditions and low-grade inflammation on blood tests after overall 19 days of hospitalization. The weekly surveillance nasopharyngeal swabs for SARS-CoV2 persisted negative. Nevertheless, 13 days after discharge, he complained tenderness of the right ankle, knee, and hip in association with low-grade fever. He presented to the ER with oligoarthritis of the right lower limb and high CRP level (237 mg/L) on blood tests. Physical and ultrasound examination confirmed slight right ankle inflammation and clear right knee arthritis. Arthrocentesis led to the evacuation of 20 cc of a cloudy, yellow, and highly inflammatory synovial fluid (SF) whose analysis revealed 20.000/mm 3 white blood cells of which 90% polymorphonucleates and 10% monocytes; no crystals were detected. Synovial RT-PCR (real-time polymerase chain reaction) for SARS-CoV2, as well as SF culture for bacterial agents, was negative (Table 2 ). He denied any infectious symptom, recent history of physical trauma, dyspnea, previous episode of arthritis, dactylitis, conjunctivitis or uveitis nor inflammatory diarrhea, and personal or familial history of psoriasis. To proceed with further investigations, the patient was hospitalized again. A new nasopharyngeal swab and a negative research of SARS-CoV2 nucleic acid on sputum excluded a recurrence of the systemic infection while serology showed indisputable seroconversion (a-SARS-CoV2 IgG 37.120 KUA/L and a-SARS-CoV2 IgM 9.163 KUA/L). Urine and blood cultures were negative and procalcitonin within the normal range; urethral swab and stool culture did not show evidence of bacterial infection. The in-depth examination for systemic rheumatic causes of arthritis were negative including antinuclear antibodies (ANA), extractable antinuclear antibodies, rheumatoid factor (RF), anti-citrullinated peptide, and HLA-B27 typing (Table 3 ). The knees, ankles, and hip X-ray did not show erosions or intra-articular calcifications . Even though the patient’s SF was markedly inflammatory, which is an infrequent finding in infectious-related arthritis, the temporal relation with SARS-CoV2 infection made the hypothesis of post-viral acute arthritis the most probable. A nonsteroidal anti-inflammatory (NSAID) therapy with ibuprofen 600-mg; bid was started with clinical benefit and decrease of CRP. The patient was discharged after 9 days and continued the NSAIDs for other 3 weeks. Up to 6 months after therapy discontinuation, he presented no signs of arthritis recurrence. Table 1 Laboratory findings during clinical course in our patient Day of discharge for COVID-19 hospitalization Day of hospitalization for oligoarthritis arthritis Day of discharge WBCs 6490 9020 5510 Neutrophils (1.800–7.800/mmc) 2750 5750 2990 Lymphocytes 2080 990 1760 Monocytes (200–960/mmc) 910 630 560 Eosinophils (0–500/mmc) 690 220 190 Basophils (0–200 /mmc) 6 10 4 Hb (14–17.5 g/dL) 11.8 11.8 11.7 PLTs (150.000–410.000/mmc) 380.000 330.000 388.000 ESR (2–37 mm/h) - 111 72 CRP (0–6 mg/L) 12 237 14 D-dimer (0–300 μg/L) 1490 839 - Fibrinogen (1.5–4.5 g/L) 7.66 10.61 - LAD (135–225 U/L) 260 213 167 Ferritin (20–250 μg/L) 700 446 248 Procalcitonin (0–0.5 μg/L) < 0.04 < 0.04 - SARS-CoV2 swab Negative Negative Negative WBC white blood cell, Hb hemoglobin, PLT platelet, ESR erythrocyte sedimentation rate, CRP C-reactive protein, LAD lactate dehydrogenase, SARS-CoV2 severe acute respiratory syndrome coronavirus 2 Fig. 1 Patient’s synovial fluid Table 2 Synovial fluid analysis Aspect Yellow turbid Differential count of WBC WBC 20.000/mmc, PMN 90%, M 10% Polarized light microscopy No crystals SARS-Cov2 RT-PCR Negative Culture Negative WBC white blood cell, PMN polymorphonucleate, M monocyte, RT-PCR real-time polymerase chain reaction Table 3 Case reports of arthritis reactive to SARS-CoV2 infection, clinical characteristics and diagnostic workup Yokogawa et al. Liew et al. Ono et al. Saricaoglu et al. Danssaert et al. Parisi et al. Present case Age and sex 57-year-old man 47-year-old man Male in his 50s 73-year-old man 37-year-old female 58-year-old female 60-year-old man Time to arthritis 15 days after COVID19 diagnosis At diagnosis of COVID19 21 days after COVID19 diagnosis 15 days after COVID19 diagnosis 12 days after COVID19 diagnosis 25 days after prodrome infective symptoms 32 days after COVID19 diagnosis Affected joints Right knee Right knee Left and right ankle Left I MTP, PIP, DIP and right II PIP and DIP Tendonitis of the II, III and IV extensor of the right hand Ankle Right knee and ankle Therapy No therapy (self-recovery) Oral NSAID and intra-articular corticosteroid Oral NSAID and intra-articular corticosteroid Oral NSAID Topical NSAID, oral opioid, gabapentin Oral NSAID Oral NSAID SF polarised microscopic examination No crystals No crystals No crystals - - - No crystals SF culture - (negative Gram stain) Negative - - - Negative SF PCR for SARS-CoV2 Negative Negative - - - - Negative M. pneumoniae and C. pneumoniae serology - - Negative - - - Negative Gonococco PCR/serology Negative Negative Negative - - - - C. thracomatis PCR/serology - Negative Negative - - - - M. urealyticum PCR/serology - - - - - - - Urethral swab - - - - - - Negative Uric acid - - Within the normal range Within the normal range Within the normal range - Within the normal range RF - - Negative Negative Negative Negative Negative ACPA - - Negative Negative - Negative Negative ANA - - Negative - Negative Negative Negative Anti-ENA - - - - - Negative Negative HLA-B27 - - Negative - - Negative Negative HBV and HCV antibodies - - Negative - - - Negative CMV-DNA and EBV-DNA - - - - - - < 1000 copies/mL Urine culture - - - - - - Negative Blood culture - - - - - - Negative Stool culture - - - - - - Negative COVID19, Coronavirus disease 19; MTP, metatarsophalangeal, PIP, proximal interphalangeal; DIP, distal interphalangeal; NSAID, non-steroidal anti-inflammatory drug; SF, synovial fluid; PCR, polymerase chain reaction; SARS-CoV2, severe acute respiratory syndrome coronavirus 2; RE, rheumatoid factor; ACPA, anti-citrullinated peptide antibodies; ANA, anti-nuclear antibodies; ENA, Extractable nuclear antibodies; HLA, human leukocyte antigen, HBV, hepatitis B virus; HCV, hepatitis C virus Fig. 2 Knees X-ray
| 4.058594
| 0.976563
|
sec[1]/p[0]
|
en
| 0.999995
|
33587197
|
https://doi.org/10.1007/s10067-020-05550-1
|
[
"sars",
"arthritis",
"blood",
"nsaid",
"culture",
"inflammatory",
"crystals",
"antibodies"
] |
[
{
"code": "1D65",
"title": "Severe acute respiratory syndrome"
},
{
"code": "RA01.0",
"title": "COVID-19, virus identified"
},
{
"code": "FA2Z",
"title": "Inflammatory arthropathies, unspecified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Severe acute respiratory syndrome (1D65)】
Definition: 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 pneumonia. Transmission is by direct contact, inhalation of infected respiratory secretions, or airborne transmission. Confirmation is by identification of coronavirus in a blood, stool, respiratory ...
Synonyms: SARS - [severe acute respiratory syndrome]
Excludes: COVID-19, virus identified | COVID-19, virus not identified
Hierarchy: Certain infectious or parasitic diseases (01) → Certain zoonotic viral diseases → Severe acute respiratory syndrome
【2. COVID-19, virus identified (RA01.0)】
Synonyms: 2019-new Coronavirus acute respiratory disease (deprecated) | 2019-nCoV acute respiratory disease [temporary name] (deprecated) | Coronavirus disease 2019 | SARS-CoV-2 disease | confirmed COVID-19
Excludes: Coronavirus infection, unspecified site | Middle East respiratory syndrome | Severe acute respiratory syndrome
Hierarchy: Codes for special purposes (25) → International provisional assignment of new diseases of uncertain aetiology and emergency use → COVID-19 (RA01) → COVID-19, virus identified
【3. Inflammatory arthropathies, unspecified (FA2Z)】
Synonyms: polyarthritis NOS | inflammatory joint disease NOS | nonpyogenic arthritis NOS | arthritic nodosa | polyarthrosis NOS
Hierarchy: Diseases of the musculoskeletal system or connective tissue (15) → Arthropathies → Inflammatory arthropathies → Inflammatory arthropathies, unspecified
|
1D65
|
Severe acute respiratory syndrome
|
A 50 years-old woman referred at our hospital complaining dry cough, abdominal pain and vomit for the past 7 days. She was afebrile and referred history of asymptomatic left kidney lithiasis. Physical examination revealed an emaciated individual, during the previous year she had lost 35 pounds and had numerous episodes of back pain, nausea, and night sweats and and repeated urinary tract infections. Pulse rate was 70 per minute; blood pressure 90/65 mm Hg, and respiratory rate 12 per minute. Dullness to percussion and decrease respiratory sounds were noted posteriorly over the left lower chest and tenderness to percussion in the left costovertebral region. Laboratory data included hemoglobin 10 g/dl, hematocrit 30.6%, white blood cell count 15,7 103/mm3, neutrophils 86,8% (normal value 40-75), creatinine 1,02 mg/dl, C reactive protein 25,81 mg/dl, procalcitonin 0.17 (low risk >0.5) Urinalysis revealed urinary leukocyte esterase activity 500 (Leu/ul), trace of hemoglobin 0.10 mg/dl, proteins 70 mg/dl and many gram negative rods. A chest x-ray film showed a small area of infiltration on the posterobasal zone of the left hemidiaphragm . Abdominal ultrasound detected an enlarged left kidney with dilated calico-pelvic system fluid filled by inhomogeneous hypoechoic material and a staghorn calculous. The fluid filled calico-pelvic system appeared continuing into a loculated collection extending above the perirenal fascia with associated inhomogeneity of perirenal fat . A suspected ultrasound diagnosis of complicated pyonephrosis was formulated, and Computed Tomography (CT) of chest, abdomen and pelvis with intravenous contrast was performed in order to stage and define the extension of the pathology . CT with intravenous contrast confirmed the presence on and enlarged kidney with staghorn calculous and markedly dilated fluid filled calico-pelvic system . The calico-pelvic dilated system continued into a fluid collection in the perirenal fat at the upper pole of the kidney, with hyperemic wall referable to a perinephic abscess . The perinephric abscess was connected to bronchi through a fistulous tract that passed thorough the left hemidiaphragm . Bronchi involved into the fistulous tract appeared enlarged with thickened and irregular walls, they appeared air filled, and no endobronchial fluid stasis was detected . A diagnosis of complicated pyonephrosis with perinephric abscess and nephrobronchial fistula was formulated. The patient was admitted to the hospital and treated with antibiotic therapy (merren intravenous 1 gr x 3, bactrim 800/160 1 vial x 2). A left tube nephrostomy was placed. Six days after the placement of nephrostomy and intravenous antibiotic therapy, laboratory data (white blood cells 7,6 103/mm3, neutrophils 69,2 %, C reactive protein 2,99 mg/dl, procalcitonin 0.04 ng/dl-low risk <0.5) showed a significantly improvement of the inflammatory status. Follow up CT was performed after 15 days from the admission, CT findings demonstrating considerable reduction of the dilatation of calico pelvic system and of the perinephric abscess at the upper pole . Moreover the fistulous tract was not appreciable anymore, instead an inhomogeneous tissue with moderate contrastographic enhancement was detected, it was referred to the possibly presence of reparative tissue, and the healing of the fistulous connection was suspected at CT . Renal scintigraphy was performed, demonstrating a nonfunctioning left kidney . Left nephrectomy was performed under general anesthesia. At surgery, a high lumbar incision was made with partial sub- periostic extraction of the 12th rib. Extra-gerotal dissection was done all around the kidney, there were dense perinephric adhesion especially toward the upper pole, tracking toward the diaphragm. Careful dissection of all adhesions was done up. On separation of the upper renal pole there was an escape of pus and a fistulous communication was found between the upper renal pole and the left diaphragm, the fistulous wound was appreciable and appeared healed, confirming follow up CT findings. The fistulous tract was excised flus with the diaphragm, the diaphragmatic ren was closed with reinforcement by a pad of fat. Subdiaphragmatic drain was placed. The healing of the fistula avoided any pulmonary complications during surgery . Histological examination of the kidney showed xanthogranulomatous pyelonephritis. Postoperative course was unremarkable. Fig. 1 Chest X-Ray in posteroanterior (A) and lateral (B) projections. On the left basal lung, a small area of infiltrate is appreciable (black arrow). The diaphragm is well defined, no pleural effusion is detected. Fig 1 Fig. 2 Left kidney ultrasound. The kidney is enlarged (A, B) with fluid filled dilated calico-pelvic system, within inhomogeneous hypoechogenic material is appreciable (A, C). Hyperechogenic spot are visible, referable to calculi (A, B). The outer border of the lower pole is irregular, and dilated calyceal system appear corticalized and protruding into the perirenal fat close to the perirenal fascia that appears thickened (*) (C, D). Perirenal fat is inhomogeneous and slightly hypoechogenic (D). Fig 2 Fig. 3 CT with intravenous contrast, parenchymal phase. (A) multiplanar coronal plane recontruction . The left kidney is enlarged, calico-pelvic system in markedly dilated with thickening of parietal wall and calyceal corticalization. Calculi are appreciable in the pelvis. Perirenal fascia is thickened and there is stranding of the perirenal fat. On the lower pole, fluid dilated calyces are not well demarcated and close to perirenal fascia. Iliopsoas muscle appears enlarged and edematous as inflamed secondarily by the infectious renal process. Nodes are detectable. (B, C) axial images. On the upper pole of the left kidney a fluid collection with hyperemic wall is appreciable, the collection goes through the diaphragm that appears focally thickened. Fig 3 Fig. 4 CT with intravenous contrast, parenchymal phase. Multiplanar sagittal plane reconstruction . (A) Admission CT Left markedly dilation of calico- pelvic system with calyces corticalization. , A fistulous connection is clearly visible between the upper pole of the left kidney through the diaphragm. (B) CT after 15 days of antibiotics therapy and stent placement. The fistulous connection is no more appreciable, a soft tissue band with moderate enhancement is visible. The calico pelvic system appears significantly decompressed. Fig 4 Fig. 5 Axial (A, B) and multiplanar sagittal plane reconstruction (C) with lung window. On axial plane an air-filled cavity with irregular walls is detected on the left lower lung lobe, on sagittal (C) image the air filled lesion appears to be a focally dilated bronchi with thickened and irregular wall in direct communication through the fistulous connection with the upper left perinephric abscess. Fig 5 Fig. 6 CT with intravenous contrast, parenchymal phase. Follow up CT after left nephrostomy and 15 days of antibiotic therapy. (A) multiplanar coronal plane reconstruction,.. The left calico-pelvic system is significantly decompressed, nephrostomy tube is appreciable in the pelvis. Perirenal fascia and fat are still thickened (B,C) axial images. On the upper pole of the left kidney the fluid component of the perinephric abscess is almost completely reabsorbed, a soft tissue with moderate enhancement is visible over the previously reported fistulous connection. Fig 6 Fig. 7 Renal scintigraphy. Nonfunctional left kidney. Fig 7
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en
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34539942
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https://doi.org/10.1016/j.radcr.2021.08.025
|
[
"kidney",
"system",
"fistulous",
"calico",
"pelvic",
"perirenal",
"pole",
"fluid"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Kidney failure, unspecified (GB6Z)】
Synonyms: nontraumatic kidney injury | renal failure NOS | kidney block | renal impairment NOS | renal insufficiency NOS
Hierarchy: Diseases of the genitourinary system (16) → Diseases of the urinary system → Kidney failure → Kidney failure, unspecified
【2. Renal dysplasia (LB30.1)】
Definition: A condition characterised by abnormal development of one or both kidneys.
Synonyms: congenital renal dysplasia | dysplasia of kidney | dysplastic kidney | Primary renal dysplasia | Primary renal dysplasia, unilateral
Excludes: Autosomal dominant polycystic kidney disease
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the urinary system → Structural developmental anomalies of kidneys (LB30) → Renal dysplasia
【3. Other specified injury of kidney (NB92.0Y)】
Synonyms: Injury of kidney without open wound into cavity | Injury of kidney with open wound into cavity | Haematoma of kidney | traumatic perirenal haematoma | perirenal haematoma
Hierarchy: Injuries to the abdomen, lower back, lumbar spine or pelvis → Injury of urinary or pelvic organs (NB92) → Injury of kidney (NB92.0) → Other specified injury of kidney
【4. Ectopic or pelvic kidney (LB30.7)】
Definition: A birth defect characterised by an abnormally positioned kidney; may be asymptomatic or result in urine blockage, infection or kidney stones
Synonyms: Congenital displaced kidney | congenital misplaced kidney | congenital malposition of kidney | congenital prolapsed kidney | Malrotation of kidney
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the urinary system → Structural developmental anomalies of kidneys (LB30) → Ectopic or pelvic kidney
|
GB6Z
|
Kidney failure, unspecified
|
The clinical course of the patient is shown in Fig. 1 . Chest radiography findings showed diffuse ground-glass opacities in the bilateral lung fields, and chest computed tomography (CT) showed infiltrating shadows in the lower lung fields. Blood test results are presented in Table 1. . An echocardiogram was performed, and an ejection fraction of 65% and wall motion within normal limits helped rule out heart failure. The patient was diagnosed with acute respiratory distress syndrome (ARDS) due to sepsis because of significant inflammatory findings, including a white blood cell count of 1200/µL and a C-reactive protein level of 17.1 mg/dL. The patient was intubated, and ventilatory management was initiated due to poor oxygenation. The Murray score at this time was 4. The patient’s circulatory status was still under the shock state, with a BP of 66/50 mmHg even with catecholamine (noradrenaline 0.013 μg/kg/min) administration. As both respiratory and circulatory status could not be maintained despite ventilatory management, fluid administration, and catecholamine administration, VA-ECMO was started as an adjuvant circulatory therapy. As the patient had anuria, she was considered to have acute kidney injury and was therefore started on continuous hemodiafiltration. Peripheral circulation was poor from the time she was transported from her house to the hospital, and purpura was present in the peripheral extremities . After the insertion of an arterial line into the right femoral artery and initiation of VA-ECMO, the mottled purpura of the right lower limb worsened. Further deterioration was prevented by inserting a sheath into the right femoral artery toward the periphery to maintain blood flow and peripheral circulation in the right lower limb. The acute DIC score was 4 (platelet count, 92,000/µL; PT-INR prothrombin time-international normalized ratio, 1.10; D-dimer, 4.47); hence, the patient was considered to have complicated DIC . Because of her circulatory instability, we decided to administer meropenem (MEPM) and azithromycin as broad-spectrum antimicrobials. On day 4, penicillin G (PCG) was added due to the detection of Pasteurella multocida in blood cultures. On day 6, PCG was changed to ceftazidime because of proven susceptibility. On day 9, VA-ECMO was withdrawn because the patient’s circulatory status was stable (HR 70 beats/min, BP 120/60 mmHg), remaining so despite reduced support by VA-ECMO. On day 11, enteral feeding was initiated. On day 14, the patient was extubated as the respiratory condition had stabilized. On day 15, the patient presented with abdominal pain. However, there were no obvious findings on contrast-enhanced CT. On day 16, septic shock redeveloped; elevated lactate level led to a suspected diagnosis of intestinal ischemia and, thereafter, a confirmed diagnosis of NOMI. Therefore, an experimental laparotomy was performed. The small intestine was perforated due to ischemia. Partial small bowel resection and open abdominal management were performed for small bowel necrosis and perforation, respectively . After the operation, Polymyxin B-immobilized fiber column direct hemoperfusion was performed to improve circulatory dynamics. On day 17, no new abnormal findings were found in the second-look surgery; therefore, the separated small intestine was anastomosed, and the abdomen was closed. Subsequently, MEPM was changed to cefmetazole owing to suspected drug-induced leukocytopenia. However, micafungin was additionally administered due to lack of improvement in the inflammatory findings. On day 24, there was no evidence of circulatory disturbance, but a brown discharge from the wound was observed. Therefore, a third emergency laparotomy was performed, which revealed anastomotic leakage. A small-bowel anastomosis resection was subsequently performed. On day 30, the patient’s respiratory status deteriorated again, and she was considered to be having a flare-up. Intra-abdominal infection was suspected as the cause. On day 35, a fourth laparotomy was performed. Owing to anastomotic re-leakage, partial resection of the small intestine and double-barreled stoma were performed. On day 55, the patient underwent amputations of 2–5 fingers of both hands with ischemic necrosis and segmental skin grafting. On day 68, debridement of the right whole metatarsal bones was performed because of abscess formation in the right plantar region. On day 73, owing to delayed wound healing, we decided to amputate the right lower limb. Upon incision, however, the right lower leg revealed itself to be so extensively necrotic that a right femoral amputation was performed. On day 98, MEPM was initiated because of fever and elevated inflammatory findings, suspected to be due to bacteremia. On day 99, incisional abscess drainage was performed because of the presence of a right rectus femoris abscess. The patient continued to have liver dysfunction and gastrointestinal malabsorption, and although it took time for her to stabilize, she underwent stoma closure on day 382 and was discharged home on day 443. Fig. 1 The patient’s clinical course from transportation to third laparotomy. The time series is shown in hours only for day 1 and in days from day 2 onward. VA-ECMO: venoarterial extracorporeal membrane oxygenation; CHDF: continuous hemodiafiltration; PMX-DHP: polymyxin B immobilized fiber column direct hemoperfusion; HR: heart rate; RR: respiratory rate; MAP: mean arterial pressure; BT: body temperature; LPT: laparotomy; MEPM: meropenem; AZM: azithromycin; PCG: penicillin G; CAZ: ceftazidime; SBT/ABPC: sulbactam/ampicillin; DAP: daptomycin; CMZ: cefmetazole; MCFG: micafungin Fig. 2 Chest radiography findings show infiltrative shadows on both lung fields Fig. 3 Chest computed tomography findings show infiltrating shadows that predominate in the bilateral lower lung fields Table 1. Laboratory findings at the time of admission Complete blood count Reference White blood cells 1,200 4000-9000 /μL Red blood cells 438 × 10 4 380-540× 10 4 /μL Hemoglobin 13.6 11.5-15 g/dL Platelet 92 × 10 3 150-350× 10 3 /μL Coagulation status Activated partial thromboplastin time 41.4 26-36 seconds Prothrombin time-international normalized ratio 1.1 0.8-1.1 Fibrinogen 419 200-400 mg/dL D-dimer 4.47 0-0.49 μg/mL Arterial blood gas FiO 2 1.0 pH 7.394 7.35-7.45 PaCO 2 32.9 35-45 mmHg PaO 2 73.9 80-100mmHg(room air) HCO 3 − 21.1 22-26 mmol/L Base excess -4.4 -2 to +2 Lactate 7.4 <2mmol/L Biochemistry Total bilirubin 0.7 0.2-1.2 mg/dL Aspartate transaminase 139 12-35 U/L Alanine transaminase 85 5-30 U/L Alkaline phosphatase 235 109-344 U/L Lactate dehydrogenase 712 110-240 U/L Cholinesterase 300 217-491 U/L Creatine kinase 2007 13-187 U/L Blood urea nitrogen 13.7 8-20 mg/dL Creatinine 2.13 0.4-0.8 mg/dL Sodium 138 mEq/L Potassium 3.0 3.5-5 mmol/L Chloride 96 98-110 mmol/L Total protein 6.3 6.1-8.1 g/dL Albumin 2.9 3.2-5 g/dL C-reactive protein 17.1 0-0.3 mg/dL Blood sugar 121 <140 mg/dL Hemoglobin A1c 6.5 4.6-6.2 % Procalcitonin >100 0-0.49 ng/mL N-terminal pro-brain natriuretic peptide 2171 0-125 pg/mL Fig. 4 Image of the peripheral extremities shows degeneration due to ischemia, with black coloration. Purpura can be seen on the extremities, especially on the right lower extremity, with commensal extension to the thigh Fig. 5 Acute disseminated intravascular coagulation score. The score worsened day by day, peaking on day 3 Fig. 6 Intraoperative findings. The small intestines show ischemic changes and partial perforation
| 3.863281
| 0.974609
|
sec[1]/p[2]
|
en
| 0.999998
|
36894896
|
https://doi.org/10.1186/s12245-023-00493-1
|
[
"findings",
"blood",
"time",
"circulatory",
"small",
"respiratory",
"status",
"ecmo"
] |
[
{
"code": "MD40.Y",
"title": "Other specified clinical findings in specimens from respiratory organs and thorax"
},
{
"code": "ME20.Y",
"title": "Other specified clinical findings in specimens from digestive organs or abdominal cavity"
},
{
"code": "MF9Y",
"title": "Other specified clinical findings on examination of urine, without diagnosis"
},
{
"code": "MA12.1",
"title": "Finding of cocaine in blood"
},
{
"code": "MA12.2",
"title": "Finding of hallucinogen in blood"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Other specified clinical findings in specimens from respiratory organs and thorax (MD40.Y)】
Synonyms: Abnormal findings in bronchial washings | Abnormal findings in nasal secretions | Abnormal findings in pleural fluid | abnormal pleural fluid | abnormality in pleural fluid
Hierarchy: Symptoms, signs or clinical findings of the respiratory system → Clinical findings in the respiratory system → Clinical findings in specimens from respiratory organs and thorax (MD40) → Other specified clinical findings in specimens from respiratory organs and thorax
【2. Other specified clinical findings in specimens from digestive organs or abdominal cavity (ME20.Y)】
Synonyms: Abnormal findings in peritoneal fluid | abnormal peritoneal fluid | abnormality in peritoneal fluid | Abnormal findings in saliva | abnormal saliva
Hierarchy: Symptoms, signs or clinical findings of the digestive system or abdomen → Clinical findings in the digestive system → Clinical findings in specimens from digestive organs or abdominal cavity (ME20) → Other specified clinical findings in specimens from digestive organs or abdominal cavity
【3. Other specified clinical findings on examination of urine, without diagnosis (MF9Y)】
Synonyms: Methaemoglobinuria | Other and unspecified abnormal findings in urine | Calciuria | Cells and casts in urine | casts in urine
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings of the genitourinary system → Clinical findings on examination of urine, without diagnosis → Other specified clinical findings on examination of urine, without diagnosis
【4. Finding of cocaine in blood (MA12.1)】
Synonyms: cocaine in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of cocaine in blood
【5. Finding of hallucinogen in blood (MA12.2)】
Synonyms: hallucinogen in blood
Hierarchy: Symptoms, signs or clinical findings of blood, blood-forming organs, or the immune system → Clinical findings in blood, blood-forming organs, or the immune system → Clinical findings of drugs, medicaments and biological substances in blood, blood-forming organs, or the immune system (MA12) → Finding of hallucinogen in blood
|
MD40.Y
|
Other specified clinical findings in specimens from respiratory organs and thorax
|
A 52-year-old male was diagnosed with advanced colorectal cancer, with multiple metastases to the lungs, liver, and peritoneum. Histology revealed well-to-moderately differentiated adenocarcinoma, in which the KRAS and NRAS mutation tests were negative and microsatellite instability was stable, but the BRAF V600E mutation was positive. In his medical history, he had received an allogeneic BMT from his elder sister for severe aplastic anemia 33 years ago. As preparative therapy prior to BMT, the patient received total lymph node irradiation (7.5 Gy) and cyclosporine at 50 mg/kg for four days. Only the bilateral lung apices were involved in the irradiated field, but no pulmonary changes suggestive of radiation pneumonitis were noted thereafter. He experienced a grade 1 skin rash as an acute graft-versus-host disease (GVHD) symptom after BMT, which disappeared clinically without treatment; however, a skin biopsy performed several months later showed pathological findings suspicious of chronic GVHD. During follow-up, there was no worsening of GVHD at the skin or other sites, including interstitial pneumonia, and the patient received no immunosuppressive agents at the time of diagnosis of colon cancer. He had no history of smoking or known allergies. The patient had received two doses of COVID-19 vaccine manufactured by Moderna 9 and 10 months prior to the diagnosis of colorectal cancer and polymerase chain reaction (PCR) test for COVID-19 was negative multiple times during the DILD treatment. The vaccination history other than COVID-19 was unknown. He had type 2 diabetes and used long-acting insulin. Because the sigmoid colon tumor invaded the left ureter and lower gastrointestinal endoscope could not pass through the primary site, the patient underwent transverse colostomy for colon obstruction due to the tumor. Before receiving chemotherapy, the patient experienced a severe cough due to lung metastasis but had no respiratory distress, with a pulse oxygen saturation (SpO2) of 95% in room air. Chest computed tomography (CT) showed multiple pulmonary nodules, but no abnormal findings suggesting accumulative lung damage such as fibrosis . All screening tests including blood cultures for bacterial infections and serological examinations for viral infections were negative. One month after surgery, 1st-line chemotherapy with FOLFIRI + bevacizumab (5 mg/kg) with palonosetron hydrochloride and dexamethasone as prophylactic anti-emetic therapy was started. He developed a fever without myelosuppression on day 6; however, the bacterial culture was negative, and there was no new lung infiltration on chest radiography. Naproxen (600 mg/day) was administered for the noninfectious fever, which quickly resolved. He was discharged from the hospital in a good general condition and received a second course of chemotherapy at our outpatient clinic with no severe adverse events. On day 15 of the second course of chemotherapy, the patient had no dyspnea. However, because his SpO2 was 92% in room air, the patient was admitted for oxygen demand. A CT scan showed no apparent tumor growth, but there were slight bilateral ground-glass shadows . A bronchoscopy was performed on the same day. There were no abnormalities in the endotracheal lumen such as apparent redness, edema, or neoplastic lesions; bronchoalveolar lavage (BAL) was performed in the middle lobe, and a biopsy of the peripheral lung was performed in the lower lobe of the right lung. The pathology showed that there was only fibrosis around the tumor as a tumor environment, and there was no obvious fibrosis in the background lungs consistent with the CT findings . Bacterial and fungal cultures and PCR tests for pneumocystis jirovecii DNA and cytomegalovirus (CMV) DNA of the BAL fluid were negative. Pathology of the lung biopsy showed pulmonary metastasis of the colon cancer and no apparent findings of inflammation. Serum lactate dehydrogenase (LDH) and KL-6 levels were within the normal ranges, and the βD-glucan test was negative. The white blood cell count was 2,740/μL, neutrophil count 1,140/μL, and lymphocyte count 880/μL. The interferon-gamma release assay for tuberculosis was negative. Differential diagnoses were interstitial pneumonitis or atypical pneumonia. The clinical course after onset of DILD is summarized in the Supplementary Figure 2 . There were no apparent clinical manifestations of chronic GVHD around the time of DILD. As his respiratory status was stable, the antimicrobial agent levofloxacin was initiated. Four days after admission, the patient’s respiratory status was stable. However, at night on day 5, the cough and dyspnea worsened and the oxygen demand increased. CT examination revealed expanded bilateral interstitial infiltrations . The white blood cell count was 6,260/μL, neutrophil count 5,440/μL, and lymphocyte count 720/μL, and the KL-6 level was mildly elevated (531 U/ml). On day 6, methylprednisolone (1 g/day) and piperacillin-tazobactam (4.5 g q6hr) were administered. Thereafter, the patient’s respiratory condition improved rapidly, and the shadows in the bilateral lung fields disappeared on day 21 after starting steroid therapy . The steroid dose was gradually decreased and steroid administration was switched to oral prednisolone 30 mg/day after day 22. The prednisolone dose was reduced to 20 mg/day on day 33 after starting steroid treatment, and the patient was scheduled to be discharged. Immediately before discharge on day 39, the patient complained of abdominal pain. Abdominal CT revealed a retroperitoneal hematoma and pseudoaneurysm in the anterior superior pancreatic duodenal artery. Because bleeding from the metastasis near the pancreatic head was suspected, the patient was transferred to a tertiary hospital, where intra-arterial embolization was performed. During the intervention, oral prednisolone was maintained at the same dose, and the patient returned to our hospital immediately after the intervention. On day 43, the lung infiltration reappeared. On day 44, steroid pulse therapy with methylprednisolone (500 mg/day) and piperacillin-tazobactam (4.5 g q6hr) was resumed. The methylprednisolone dose was tapered to 125 mg/day on day 47. On day 49, nasal high-flow therapy was initiated because oxygen demand increased. On the same day, intravenous cyclosporine (150 mg/day) was started after consultation with a hematologist, and methylprednisolone (500 mg/day) was retried. On day 51, laboratory tests reported the levels of aspartate aminotransferase (32 U/L), alanine transaminase (53 U/L), alkaline phosphatase (351 U/L), gamma-glutamyl transpeptidase (236 U/L), and total bilirubin (3.0 mg/dL). Considering the possibility of exacerbation of sepsis, an antifungal agent, micafungin (100 mg/day), was administered to treat all infectious diseases. On day 53, because serum CMV antigenemia tested on day 51 was positive (Cytomegalovirus antigen positive cell count was 200-206 positive cells per two slides, CMV IgG was > 250 AU/mL, CMV IgM INDEX was 1.04, absolute lymphocyte count was 280/μL), ganciclovir (500 mg/day) was added in combination with other antibiotics. The measurements of the same blood sample showed that the white blood cell count was 22,740/μL, neutrophil count 22,060/μL, lymphocyte count 280/μL, LDH 654 U/L, creatinine kinase 22 U/L, CRP 3.89 mg/dL, and βD-glucan test was negative. Unfortunately, the patient eventually died of respiratory and hepatic failure 56 days after starting the steroid therapy.
| 3.998047
| 0.976563
|
sec[1]/p[0]
|
en
| 0.999998
|
PMC10313190
|
https://doi.org/10.3389/fonc.2023.1215789
|
[
"count",
"lung",
"therapy",
"steroid",
"tumor",
"respiratory",
"blood",
"cancer"
] |
[
{
"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 CLASSIFICATION ═══
【1. Acquired thrombocytosis, unspecified (3B63.1Z)】
Synonyms: Acquired thrombocytosis | Idiopathic haemorrhagic thrombocythaemia | Essential thrombocythaemia | primary haemorrhagic thrombocythaemia | idiopathic thrombocythaemia
Hierarchy: Coagulation defects, purpura or other haemorrhagic or related conditions → Thrombocytosis (3B63) → Acquired thrombocytosis (3B63.1) → Acquired thrombocytosis, unspecified
【2. Thrombocytopenia, unspecified (3B64.Z)】
Synonyms: Thrombocytopenia | low platelet count | low platelets | decreased platelets | platelet dysfunction NOS
Hierarchy: Diseases of the blood or blood-forming organs (03) → Coagulation defects, purpura or other haemorrhagic or related conditions → Thrombocytopenia (3B64) → Thrombocytopenia, unspecified
【3. Immune system disorders involving white cell lineages, unspecified (4B0Z)】
Hierarchy: Diseases of the immune system (04) → Immune system disorders involving white cell lineages → Immune system disorders involving white cell lineages, unspecified
【4. Eosinophilia, unspecified (4B03.Z)】
Synonyms: Eosinophilia | Disorders with increased eosinophil counts | Idiopathic hypereosinophilic syndrome
Hierarchy: Diseases of the immune system (04) → Immune system disorders involving white cell lineages → Eosinophilia (4B03) → Eosinophilia, unspecified
【5. Neutrophilia, unspecified (4B00.1Z)】
Synonyms: Neutrophilia | Disorders with increased neutrophil counts
Hierarchy: Immune system disorders involving white cell lineages → Disorders of neutrophil number (4B00) → Neutrophilia (4B00.1) → Neutrophilia, unspecified
|
3B63.1Z
|
Acquired thrombocytosis, unspecified
|
A 55-year-old male underwent pancreaticoduodenectomy as an initial treatment for biliary carcinoma in March, 2009 without preoperative chemoradiotherapy. Histopathological examination revealed papillary adenocarcinoma infiltrating to the fibromuscular layer with no metastasis to the regional lymph nodes, with the pathologic stage of the tumor defined as T1 N0 M0. The drainage tubes were placed at the anterior aspect of the pancreaticojejunostomy and the dorsal space of the hepaticojejunostomy. On the 11th day after surgery, the patient had a spiking temperature of up to 38.5°C with purulent and amylase-rich exudates from the bilateral drainage tubes. The initial clinical impression was a leakage of the pancreaticojejunostomy. Continuous lavage from the drainage tubes using 1.0 L saline /day was begun. Although the fever disappeared after three days, the drainage output of turbid fluids with necrotic tissues persisted. On the morning of the 16th day after surgery, the patient had an episode of hemorrhage from the drainage tubes with a duration of a few minutes, but it spontaneously disappeared with the patient remaining hemodynamically stable. A computed tomography (CT) was performed which demonstrated neither an abscess nor a hematoma around the pancreaticojejunostomy (Figures 1(a) and 1(b) ). The stump of the gastroduodenal artery (GDA) showed an outgrowth with a length of 6.0 mm without formation of pseudoaneurysm ( Figure 1(b) , arrow). The stump of the GDA was immediately adjacent to the tip of the drainage tube placed at the anterior aspect of the pancreaticojejunostomy ( Figure 1(b) , arrowhead). The sentinel bleeding disappeared just after transfer to the CT room. However, on the evening of the 16th day after surgery, the patient had an episode of back pain, followed by release of 500 mL of hemorrhagic fluid from the drainage tubes. There was no aneurysm or hematoma in the peritoneal cavity visualized with a second CT. The remnant stomach was markedly dilated, being filled with intralumanal fluids. The patient did not vomit, but had severe, burning epigastric pain. An aspiration of the gastric contents yielded 300 mL of hemorrhagic fluids. During the period between the 16th and 17th day postsurgery, the patient had been hemodynamically stable. On the evening of the 17th day postsurgery, the patient vomited large amounts of coffee-grounds liquid with fresh blood in the abdominal drains. Despite the patient being hemodynamically stable, a drop in hemoglobin necessitated transfusion of 2 units of packed RBC. Upper endoscopy revealed a stomach filled with blood but the bleeding point was unable to be identified around the gastrojejunostomy or pancreaticojejunostomy (Figures 1(c) and 1(d) ). The patient was kept hemodynamically stable during the 12 hours after the upper endoscopy without bleeding from abdominal drains or hematemesis. On the morning of the 18th day postsurgery, following the appearance of blood in the abdominal drains and hematemesis, copious bloody diarrhea was observed. The resistance of the abdominal wall had increased and a pulsatile mass was palpable at the left upper quadrant. In view of the patient being hemodynamically unstable, a visceral angiogram was performed immediately after fluid resuscitation and blood transfusion of 4 units of packed RBC. The patient underwent emergency angiography using the standard Seldinger technique and an angiography catheter. A selective celiac angiogram showed that the splenic artery and the left gastric artery were all patent with good organ perfusion. A 2.7 cm diameter pseudoaneurysm arising from the stump of the GDA was identified ( Figure 2(a) , arrow). It is generally accepted that gastroduodenal artery pseudoaneurhysm embolization with stent grafting of the common hepatic artery have significant clinical benefits to maintain liver blood flow [ 7 – 9 ]. However, stent-graft materials adapted to the common hepatic artery could not be prepared for the patient under the emergency conditions. Therefore, coil embolization, both proximal (common hepatic artery) and distal (right and left hepatic artery) of the pseudoaneurysm was performed with microcoils (MWCE-18S-TORNADO) (Figures 2(b) and 2(c) . The clinical course and schematic presentation are shown in Figures 3 and 4 . Bleeding from the abdominal drains and gastric tube disappeared about 48 hours after coil embolization. The hemoglobin value had decreased to 6.2 g/dL but gradually returned to 8.0 g/dL without blood transfusion. On the 3rd day postcoil embolization, the patient suffered a fever (up to 39°C). Coagulation tests on day 5 postcoil embolization revealed a marked elevation of fibrin degradation product (FDP). CT of the abdomen revealed that segments 2/3 of the liver were replaced by a low density area located near the edge of the liver ( Figure 5(a) ). The source of high fever was proved to be a hepatic infraction of segments 2/3. Because the right main branch of the portal vein was clearly visualized with a high value of portal vein peakvelocity (PVPV) on a doppler ultrasonography , we believed that severe hepatic failure would not occur. Symptomatic treatment with an initial dose of an antibiotic and vasodilator was started. High fever around 38°C to 39°C persisted for 10 days. Although the high fever resolved spontaneously over the following days, there was a high fever relapse of up to 40°C on the 16th day after coil embolization. CT demonstrated that segments 2/3 of the liver were replaced by fluid-level components with accumulation of gases ( Figure 5(b) ). We concluded that the hepatic infarction had led to an intrahepatic biloma and abscess formation. High output of purulent fluids from a decompression tube placed intraluminally at the hepaticojejunostomy was observed, suggesting that the formation of the hepatic abscess was associated with a delayed ischemia of the biliary ducts caused by the embolization of the hepatic artery. Conservative treatment with antibiotics was given based on cultures of fluids from the decompression tube and the patient became afebrile on the 30th day postcoil embolization. On the 32nd day after coil embolization, a CT scan revealed that segments 2/3 of the liver were replaced by a homogenous low-density area with thick capsule formation ( Figure 5(c) ). In addition, anastomotic leakage persisted with a high-output pancreatic fistula from the drain adjacent to the pancreaticojejunostomy ( Figure 7(a) ). Continuous irrigation from the drain with saline and enteral nutrition from the jejunostomy catheter yielded complete relief of symptoms associated with anastomotic leakage on the 34th day after coil embolization ( Figure 7(b) ). A solid diet was started on the 40th day after coil embolization. All drains and tubes were removed until the 50th day postcoil embolization. On the 68th day after coil embolization, the patient suffered high fever with epigastric pain. A hepatic abscess was localized by CT in the edge of segments 2/3 penetrating to the lesser sac, which was immediately treated by percutaneous drainage ( Figure 5(d) ). The patient recovered uneventfully and was discharged on the 101st day after coil embolization. Two months after discharge a CT scan showed disappearance of segments 2/3 of the liver with no abscess formation present ( Figure 5(e) ). The patient underwent no postoperative adjuvant therapy. The patient is in good condition without any recurrence in the 13 months since recovery.
| 3.927734
| 0.979004
|
sec[1]/p[0]
|
en
| 0.999998
|
20589213
|
https://doi.org/10.1155/2010/280430
|
[
"embolization",
"hepatic",
"artery",
"drainage",
"coil",
"high",
"fever",
"tubes"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Acute vascular disorders of intestine, unspecified (DD30.Z)】
Synonyms: Acute vascular disorders of intestine | acute intestinal ischemia NOS | acute intestinal ischemic syndrome | acute ischaemic bowel disease | acute intestinal vascular insufficiency
Hierarchy: Diseases of the digestive system (13) → Ischaemic vascular disorders of intestine → Acute vascular disorders of intestine (DD30) → Acute vascular disorders of intestine, unspecified
【2. Obstetric blood-clot embolism (JB42.2)】
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 ...
Synonyms: obstetrical blood-clot embolism | blood clot embolism in pregnancy, childbirth or puerperium | puerperal embolism | Puerperal embolism NOS | Obstetric blood-clot pulmonary embolism
Hierarchy: Pregnancy, childbirth or the puerperium (18) → Complications predominantly related to the puerperium → Obstetric embolism (JB42) → Obstetric blood-clot embolism
【3. Air embolism, traumatic, not elsewhere classified (NF0A.0)】
Synonyms: pulmonary air embolism | air embolism | arterial air embolism | arterial air embolus | arterial gas embolism
Excludes: air embolism complicating abortion or ectopic or molar pregnancy | air embolism complicating pregnancy, childbirth and the puerperium
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Other or unspecified effects of external causes → Certain early complications of trauma, not elsewhere classified (NF0A) → Air embolism, traumatic, not elsewhere classified
【4. Acute myocardial infarction, unspecified (BA41.Z)】
Synonyms: Acute myocardial infarction | cardiac attack | heart attack | acute cardiac infarction | acute MI - [myocardial infarction]
Hierarchy: Ischaemic heart diseases → Acute ischaemic heart disease → Acute myocardial infarction (BA41) → Acute myocardial infarction, unspecified
【5. Pulmonary thromboembolism, unspecified (BB00.Z)】
Synonyms: Pulmonary thromboembolism | pulmonary thromboembolus | pulmonary embolism NOS | pulmonary embolus | iatrogenic pulmonary embolism
Hierarchy: Diseases of the circulatory system (11) → Pulmonary heart disease or diseases of pulmonary circulation → Pulmonary thromboembolism (BB00) → Pulmonary thromboembolism, unspecified
|
DD30.Z
|
Acute vascular disorders of intestine, unspecified
|
A 73-year-old Caucasian man was referred to our hospital for evaluation of a right PHEO, diagnosed two months before at another hospital, after the identification of a large retroperitoneal mass on abdominal computed tomography (CT). At that time, the patient experienced abdominal discomfort, unintentional weight loss of approximately 5 Kg within the previous 3 months, associated to sporadic episodes of watery diarrhea. At admission to our hospital, the patient was moderately dehydrated and tachypnoic. He denied any history of headache, palpitations, sweating, or hypertension. He reported episodes of watery diarrhea, up to 5-6 times a day and 2-3 times a week, without blood or mucus. He also had no relevant familial history of endocrine nor cancer diseases but only a paternal history of arterial hypertension. Physical examination showed blood pressure (BP) of 100/67 mmHg and heart rate of 88 beats/min; no significant orthostatic pressure gradient was measured. BP values, evaluated on several occasions, were 94/58 and 91/62 mm Hg. Laboratory tests showed a hypokalemia (3.3 mmol/L) with metabolic acidosis (pH 7.29, HCO3 - 19 mmol/L), a serum magnesium level of 1.5 mg/dl and fasting blood glucose of 149 mg/dl. A 24-h urinary sample showed only a slight increase in normetanephrine excretion, 638 μg (normal values: 162-528/day), while metanephrine and methoxytyramine resulted within normal range. Serum chromogranin A was elevated , as well as neuron-specific enolase level (NSE 35.7 nl/ml, normal values 1.0-13.5). Plasma cortisol, adrenocorticotropic hormone, thyroid-stimulating hormone, thyroxine, parathyroid hormone, and calcitonin were within the normal ranges. Contrast enhanced abdominal CT scan confirmed the presence of inhomogeneous right adrenal mass measuring 8.1 x 7.7 x 7.9 cm . A 18 F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) coupled with CT showed an area of high uptake (maximum standardized uptake value, SUV max 8.6) in the right adrenal gland, with a prevailing peripheral signal and central hypoactivity, and another area of high uptake (SUV max 9.6) in the lumbar region suspicious of lymph node localization . In addition, 68 GaDOTA-octreotate (DOTATATE) PET confirmed the peripheral high uptake (SUV max 6) in the right adrenal gland and the high uptake area (SUV max 3.7) in the lumbar region; a high uptake (SUV max 4.5) was also detected at the base of the left lung . Based on these results, patient diagnosis was metastatic adrenal PHEO. Intravenous fluid infusion, sodium bicarbonate, potassium aspartate, magnesium sulphate supplementations were started allowing an improvement of clinical condition and blood pressure levels. Then, after 10-days pre-operative treatment with low dose alpha1-adrenergic antagonist doxazosin (given just before bed), he underwent surgical resection of the tumor. The patient had an uneventful postoperative course, except for sporadic watery diarrhea. Gross examination revealed a 10x8x6 cm brownish-yellow, friable adrenal mass. Histology showed a highly cellular tumor made up of monotonous medium-sized cells with discrete nuclear pleomorphism and mild hyperchromasia. Mitotic figures were above 3/10 high power fields, with some atypical mitoses. The cells were arranged in nests with areas of diffuse growth in more than 10% of the tumor. Confluent areas of necrosis were present. Foci of capsular and vascular invasion were noted as well as extension into periadrenal adipose tissue. The histological features were consistent with a malignant PHEO, with a PASS score (Pheochromocytoma of the Adrenal Gland Scaled Score) of 20 , indicating a high risk of aggressive cellular behavior (PASS≥4). DNA genetic analysis of the patient with a next generation sequencing (NGS) approach using Trusight One Sequencing Panel by Illumina, revealed a synonymous single nucleotide variant of gene SDHA [rs6555055, NM_004168.2:c.619A>C, (p.Arg207=)] indicated by ClinVar database as associated to “probably benign” catecholamine-secreting PHEO ( 12 ). The patient was discharged in satisfactory clinical condition. Therapy with lanreotide, a somatostatin analogue, at a dose of 60 mg once a month was initiated. At 2 months, multiple metastatic pulmonary and hepatic nodules were identified on CT scan . The patient once again experienced abdominal discomfort, 4 kg weight loss, yet only sporadic watery diarrhea. Peptide receptor radionuclide therapy and sunitinib, a multi-targeted receptor tyrosine kinase inhibitor, were scheduled. In the meantime, lanreotide therapy was increased to 120 mg once a month. However, after about one month, the patient was re-admitted with a 10-day history of severe watery diarrhea, up to 20 times in 24 hrs, accompanied by nausea, vomiting and occasionally quick flushing. At presentation, he was suffering and markedly dehydrated. Physical examination showed BP of 90/67 mmHg, heart rate of 120 beats/min, the pulse was fast and weak, the breath was fast and short, the skin cold and clammy, and the urination was decreased. Laboratory tests were as follows: blood urea nitrogen 96 mg/dl; serum creatinine 3.5 mg/dl; Na + 136 mmol/l; K + 2.5 mmol/l; Cl - 115 mmol/l; pH 7.08; HCO 3 - 5.5 mmol/l; Pa CO2 30 mm Hg; Pa O2 67 mm Hg; lactate 5 mmol/l (normal values 0.5-2.2); serum anion gap 15 mmol (corrected for serum albumin levels 16 mmol); urine anion gap was negative. Serum prealbumin was 29 mg/dl (normal values 15-35) and albumin 3.9 g/dl (normal values 3.5-5.0). At that time, serum chromogranin A was 2896 ng/ml and neuron-specific enolase 49.6 ng/ml. Twenty-four hours urinary normetanephrine excretion was 920.4 μg, while metanephrine resulted at 432.6 μg (normal values 64-302 μg/day). VIP plasma levels were measured, and circulating values were more than 10 times the upper normal limit . Cardiac ultrasound showed a reduced left ventricular ejection fraction (35%). Due to the emerging clinical picture, histological sections were re-evaluated with additional immunostainings. Sections were stained with the following primary antibodies: anti-Chromogranin A (clone LK2H10 ready to use; Ventana-Roche), Anti-Vasoactive intestinal polypeptide -VIP (rabbit 1:500; Biogenex) and anti-Somatostatin Receptor 2A -SSTR2A (rabbit 1:100; Bio-Trend). The sections were immunostained with HRP Polymer (Optiview DAB IHC Detection kit; Roche) in accordance with the manufacturer’s specifications. Negative controls consisted of substituting normal mouse serum for the primary antibodies. A set of sections adjacent to these used for single labelling with VIP, was used for double labelling with Chromogranin A. The second antibody was immunostained with AP Polymer (Ultraview Universal Alcaline Phosphatase Red Detection Kit; Roche). Permanent red chromogen was used for staining development. Immunostaining revealed strong positivity for neuroendocrine marker chromogranin A and VIP ; a large number of cells co-expressed chromogranin A and VIP . Weak was the positivity for SSTR2A . A diagnosis of VIP-secreting PHEO was rendered. The patient was then transferred to the intensive care unit. He was managed with intensive intravenous fluid hydration, potassium salts and bicarbonates, as well as with octreotide (0.1 mg/8 h s.c.), sunitinib 50 mg/day and loperamide. However, his diarrhea worsened with further exacerbation of metabolic acidosis (pH 6.99, HCO 3 - 4.3 mmol/l), leading to hemodynamic instability and shock. He died five days later.
| 4.179688
| 0.960938
|
sec[1]/p[0]
|
en
| 0.999996
|
33815297
|
https://doi.org/10.3389/fendo.2021.652045
|
[
"mmol",
"values",
"serum",
"high",
"diarrhea",
"chromogranin",
"adrenal",
"uptake"
] |
[
{
"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": "MB26.6",
"title": "Overvalued ideas"
},
{
"code": "NE80.3",
"title": "Other serum reactions"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Albuminuria, Grade A3 (GB42.1)】
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 ...
Synonyms: albuminuria >30 mg/mmol creatinine | macroalbuminuria | overt albuminuria | overt nephropathy | overt proteinuria
Hierarchy: Diseases of the urinary system → Glomerular diseases → Persistent proteinuria or albuminuria (GB42) → Albuminuria, Grade A3
【2. Albuminuria, Grade A2 (GB42.0)】
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 ...
Synonyms: microalbuminuria | incipient nephropathy | mild to moderate albuminuria | albuminuria 3-30 mg/mmol creatinine
Hierarchy: Diseases of the urinary system → Glomerular diseases → Persistent proteinuria or albuminuria (GB42) → Albuminuria, Grade A2
【3. Other specified elevated blood glucose level (MA18.0Y)】
Synonyms: 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 | Blood glucose greater than or equal to 14.0 mmol/L post-meal or not otherwise specified
Hierarchy: Clinical findings in blood, blood-forming organs, or the immune system → Certain clinical findings of blood chemistry (MA18) → Elevated blood glucose level (MA18.0) → Other specified elevated blood glucose level
【4. Overvalued ideas (MB26.6)】
Definition: Unreasonable and sustained beliefs that are maintained with less than delusional intensity (i.e., the person is able to acknowledge the possibility that the belief may not be true). An alternative use of this term is to refer to conventional or plausible thoughts (e.g., religious concepts, political ideas, or excessively idealistic beliefs) that are held with such a level of intensity so that the ...
Excludes: Delusion | Grandiosity | Paranoid ideation | Referential thinking
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Mental or behavioural symptoms, signs or clinical findings → Symptoms or signs involving content of thought (MB26) → Overvalued ideas
【5. Other serum reactions (NE80.3)】
Synonyms: Allergic reaction to serum | serum allergy | Complications of vaccination, protein sickness | Protein sickness | transfusion reaction due to serum protein reaction
Excludes: serum hepatitis
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injury or harm arising from surgical or medical care, not elsewhere classified → Injury or harm arising following infusion, transfusion or therapeutic injection, not elsewhere classified (NE80) → Other serum reactions
|
GB42.1
|
Albuminuria, Grade A3
|
The patient was the second child born to non-consanguineous healthy parents of Caucasian origin. Past medical history revealed recurrent mild upper respiratory infections and one episode of pneumonia at the age of 3. The patient presented at 8 years of age with a one-month history of recurrent headaches and behavioral changes. Subsequent cranial magnetic resonance imaging (cMRI) revealed multiple bilateral necrotizing lesions in the basal ganglia as well as in the right temporal lobe. Protein levels in cerebrospinal fluid (CSF) were elevated at 941mg/dL with increased cell count (100 cells/µL) and lymphocytic pleocytosis. Human herpesvirus 7 (HHV-7) PCR was tested positive in the CSF as well as in peripheral blood, while PCR for Epstein–Barr virus (EBV), Cytomegalovirus , Herpes simplex virus , Human herpesvirus 6 and enterovirus were negative. Bacterial cultures of the CSF and peripheral blood, including Borrelia and Mycoplasma , were negative. Additionally, an extensive autoimmunologic work-up, including antineuronal antibody testing (e.g. NMDAR, AMPAR, GABA(B)R, LGI1 and CASPR2 antibodies) was unsuspicious. Open cerebral biopsy showed extensive lymphomonocytic infiltration of the meninges and parenchyma as well as most perivascular regions with fibrinoid necrosis of the vessel walls and brain parenchyma . EBV in situ hybridization and HHV-7 PCR of the tissue was negative. Overall, these findings indicated a florid meningoencephalitis with thrombotic occlusive vasculopathy of unknown cause, although HHV-7 infection as a possible trigger could not be excluded. The patient was treated with broad-spectrum antibiotics, acyclovir, as well as an aggressive immunosuppressive therapy with mycophenolate mofetil (MMF) and high-dose glucocorticoids which led to clinical improvement. Long-term immunosuppressive therapy was continued after the discharge. After 3 months the patient was readmitted with acute left-side hemiparesis due to intraparenchymal and ventricular hemorrhage. After trepanation and decompression, the hemiparesis clinically improved, while the patient developed organic brain disorder and a central diabetes insipidus, which was treated with vasopressin analogs and antipsychotics. At the age of 10, neurological functions rapidly declined with changes in movement patterns and increased myotonus of the lower extremities. A cMRI revealed a hypertrophic pachymeningitis with progression of the lesions bilaterally within the hemispheres of the brain with mild hemorrhage . Additionally, fusiform aneurysms were found within the M2 and M3 segments of the right middle cerebral artery and the right posterior cerebral artery without signs of vessel narrowing. Protein levels in CSF were elevated, with increased cell count at 25 cells/µL. We could detect immunoglobulins within the CSF, without oligoclonal bands. HHV-7 PCR was again tested positive in CSF and peripheral blood. Upon escalation of immunosuppressive therapy with glucocorticoids and MMF as well as short-term intravenous immunoglobulin therapy, the patient clinically improved. Two months later, the patient was readmitted for the third time with acute sepsis due to lobar pneumonia and transferred to the intensive care unit (ICU), where he survived resuscitation following sudden cardiac arrest. A subsequent cranial computer tomography scan showed massive intraventricular hemorrhage, which was treated with an external ventricular drain (EVD), however extensive neurological dysfunction developed. The patient received antibiotic therapy and supportive care as well as high dosage glucocorticoids and MMF. During recovery at the ICU the patient developed the reoccurrence of a massive intracerebral hemorrhage after removal of EVD which resulted in progression of extensive neurological dysfunctions. The patient entered a minimally conscious state with flaccid tetraparesis, lost protective reflexes and developed vegetative instability. He required surgical placement of a tracheostoma and the insertion of a PEG (percutaneous endoscopic gastrostomy) tube. In the following year, the patient developed recurring respiratory infections as well as septic pyelonephritis, requiring repeated intravenous antibiotic therapy. Necrotizing CNS inflammatory disease in combination with susceptibility to infections lead to evaluation for PID. Extensive immunological work-up revealed severe IgG1-IgG3-IgG4 subclass deficiency ( Table 1 ). Antibody response to previous vaccination against tetanus and diphtheria, T-cell dependent protein antigens, was absent. T-cell independent polysaccharide vaccine against Pneumococcus resulted in IgM and IgA antibody response but defective IgG- and IgG2-response ( Table 1 ). Repeated serum EBV serology was negative. We could detect reduced switched memory B-cells (IgD - CD27 + cells) and complete absence of iNKT-cells (CD3 + Vα24 + Vβ11 + ) previously described in XLP1 ( Table 1 ). Long-term immunoglobulin replacement therapy was initiated. Family history revealed a half-brother from the same mother with a previous partner who died in early childhood at 3,5 years of age from fulminant leukemia many years before the patient was born. The definite genotype as well as EBV status of the half-brother is unknown. The family pedigree of the patient is shown in Figure 3A . A T to G transposition within the start codon of the SH2D1A gene was revealed independently by targeted resequencing of genes associated with PID’s and whole exome sequencing. Sequencing of the patient’s mother confirmed maternal segregation of the variant . We could not identify any other pathogenic or variants of unknown significance in PID genes nor those associated with vasculopathies or autoimmunity. We hypothesized that this novel variant resulted in an aberrant protein expression of SH2D1A. SAP protein expression in the patient’s peripheral blood mononuclear cells (PBMCs) was evaluated using flow cytometry. We could demonstrate a significant reduction of intracellular SAP-intensities within NK-cells, CD4 + T-cells and CD8 + T-cells of the patient as compared to those of six healthy controls (3 anonymous blood donors and 3 sex-matched controls) . The findings indicate that the start codon mutation led to a drastic reduction but possibly not a complete absence of SH2D1A expression. Additionally, we stimulated PBMCs, isolated from the patient and five healthy controls (2 anonymous blood donors and 3 sex-matched controls), with phytohemagglutinin (PHA) for 3 days. We could demonstrate, that SH2D1A expression in the CD4 + T-cells and CD8 + T-cells of the patient seemed to be upregulated following PHA stimulation but was still drastically decreased compared to detected SAP levels in cells of healthy controls . Therefore, the results indicate the reduced translational activity for SAP protein expression in the patient. The final diagnosis of XLP1 with EBV-negative CNS inflammation was established. Nevertheless, the patient’s clinical condition, despite neurological rehabilitation and extensive therapeutic efforts, did not improve considerably, with indication of neurological irreversibility. Therefore, bone marrow transplantation was not considered as a therapeutic option. The patient is under continuous immunosuppressive as well as immunoglobulin replacement therapy and receives supportive care. Two years later the patient developed pulmonal vasculitis and is up to this point hospitalized under continuous supportive care.
| 4.128906
| 0.976074
|
sec[1]/p[0]
|
en
| 0.999996
|
34987501
|
https://doi.org/10.3389/fimmu.2021.747738
|
[
"cells",
"well",
"therapy",
"protein",
"blood",
"extensive",
"neurological",
"expression"
] |
[
{
"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 CLASSIFICATION ═══
【1. Other specified clinical findings on examination of urine, without diagnosis (MF9Y)】
Synonyms: Methaemoglobinuria | Other and unspecified abnormal findings in urine | Calciuria | Cells and casts in urine | casts in urine
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings of the genitourinary system → Clinical findings on examination of urine, without diagnosis → Other specified clinical findings on examination of urine, without diagnosis
【2. Mucolipidosis (5C56.20)】
Synonyms: Mucolipidosis type 3 | Pseudo-Hurler polydystrophy | Pseudo-Hurler disease | Mucolipidosis type 2 | N-acetyl-glucosamine 1-phosphotransferase deficiency
Excludes: Sialidosis (mucolipidosis type 1)
Hierarchy: Inborn errors of metabolism → Lysosomal diseases (5C56) → Glycoproteinosis (5C56.2) → Mucolipidosis
【3. Sickle cell disease without crisis (3A51.1)】
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.
Synonyms: Hb-SS disease without crisis | HbSS without crisis | Sickle-cell anaemia without crisis | SCD - [sickle cell disease] | SCA - [sickle cell anaemia]
Hierarchy: Diseases of the blood or blood-forming organs (03) → Anaemias or other erythrocyte disorders → Sickle cell disorders or other haemoglobinopathies (3A51) → Sickle cell disease without crisis
【4. Primary anterior uveitis (9A96.3)】
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.
Synonyms: anterior chamber cell
Hierarchy: Disorders of the eyeball - anterior segment → Disorders of the anterior uvea → Anterior uveitis (9A96) → Primary anterior uveitis
【5. Acquired pure red cell aplasia, unspecified (3A61.Z)】
Synonyms: Acquired pure red cell aplasia | acquired red cell aplasia | red cell aplasia NOS | pure red cell aplastic anaemia | red cell aplastic anaemia
Hierarchy: Anaemias or other erythrocyte disorders → Pure red cell aplasia → Acquired pure red cell aplasia (3A61) → Acquired pure red cell aplasia, unspecified
|
MF9Y
|
Other specified clinical findings on examination of urine, without diagnosis
|
Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease (CHD) that presents with an absence of dysplastic pulmonary valve leaflets, aneurysmal pulmonary arteries (PA), and respiratory symptoms . It occurs in 0.2–0.4 % of all live births with CHD , and in approximately 2.4 % to 6.3 % of cases with tetralogy of Fallot (TOF) . APVS is commonly classified into two types. The first type, more common, is TOF-type APVS, which includes a ventricular septal defect (VSD), an overriding aorta, and the absence of ductus arteriosus (DA). The second type, less common, is non-TOF-type APVS which includes an intact ventricular septum, less severe PA dilation, and a patent DA, with or without tricuspid atresia. Herein, we discuss the patient's case with the first type of APVS (TOF-type APVS). The reason for the absence of DA remained uncertain . During fetal development, the absence of DA blocks the flow of blood from the PA to the descending aorta. This leads to higher PA pressure and significant pulmonary regurgitation, impacting the normal development of the pulmonary valve . According to the clinical courses and ages of patients, APVS is also categorized into two groups. Newborns in the first group suffer from dyspnea, recurrent lung infections, pulmonary emphysema, and atelectasis. The second group consists of older patients with mild symptoms who survived infancy. Later on, these patients may undergo minimally risky elective procedures to close their VSD and relieve their pulmonary stenosis . Moreover, the cyanosis observed stems from both lung-related and heart-related diseases. Desaturation of blood in the pulmonary veins may be attributable to a mismatch in ventilation-perfusion and shunting within the lungs. Additionally, a right-to-left cardiac shunt at the ventricular level can also induce cyanosis, although it is uncommon for an obstruction in the right ventricular outflow tract (RVOT) to be significant enough to provoke a substantial right-to-left shunt . Numerous newborns exhibit signs of respiratory distress immediately after birth. This condition is characterized by trapped air, causing the lungs to become overly inflated. As a result, ventilation becomes inadequate and the respiratory effort significantly increases . In our case a membranous VSD of 9.5 mm (large) with malposition of the great vessels. Bidirectional shunt with left-to-right predominance. Despite the rarity of cyanosis, the patient exhibited central cyanosis along with respiratory distress. The primary pulmonary artery typically undergoes dilation, expanding to at least double or triple its normal width. Likewise, the right and left pulmonary artery branches, which usually measure between 4 and 5 mm in diameter, frequently exhibit an expansion to two or three times their standard size . In our case, the diameter of the left pulmonary artery was 10 mm and the right was 11 mm.In cases of APVS, the area at the valve annulus is marked by the presence of rudimentary, myxomatous tissue remnants, rather than the actual development of proper valve leaflets . In our case, the pulmonary Valve is absent at the level of the constricted fibrous ring with a diameter of 9 mm. An interesting note about APVS is that the DA is almost invariably absent in cases where the pulmonary arteries are connected In continuity , and This matches our case where there is also no patent ductus arteriosus present. The pulmonary artery and its branches may undergo aneurysmal dilation, which commonly exerts pressure on the bronchial tree and the esophagus, resulting in conditions such as bronchomalacia and polyhydramnios . In our case, the internal organs were normal following the ultrasound examination. APVS is usually associated with a poor prognosis. According to one study, there is a strong correlation between respiratory complications and prognosis. Furthermore, the results of a different study indicated that the following complications were poor prognostic indicators of APVS: balloon-type pulmonary arterial morphology, hydrops fetalis, polyhydramnios, and bronchomalacia . TOF-type APVS fetuses are typically diagnosed post-20 weeks of gestation through echocardiography, where color Doppler flow imaging (CDFI) reveals a distinct color mosaic pattern reflecting the “to-and-from” blood flow between the right ventricle and PA, except positive grayscale images. Fast blood flow, both antegrade and retrograde, is visible on a spectrum Doppler in PA . Furthermore, in cases where other techniques, such as angiography, are impractical, it can serve as a preoperative assessment of APVS . Chest X-ray findings may demonstrate lung hyperexpansion, with varying involvement of individual lobes or the entire lung based on the site of bronchial obstruction. Unilateral obstruction often results in a significant mediastinal shift towards the contralateral side. Computed tomography (CT) and magnetic resonance imaging (MRI) scans are useful to identify the exact relationship between central PA dilatation and airway compression locations. Bronchoscopy is a useful tool for assessing the extent of airway compression. Additionally, in cases of mild central PA dilation, it can help exclude tracheal and bronchial compression as potential causes of respiratory symptoms. The utilization of cardiac catheterization in APVS patients is rare . In our case, chest X-rays revealed an increase in cardiothoracic ratio (CTR). In addition, echocardiography showed dilatation of left PA and right PA, absence of the pulmonary valve with a severely stenotic fibrous ring in its place, and retrograde blood flow from the PA trunk to RV. For children suffering from absent pulmonary valve syndrome, there is a lack of satisfactory palliative treatments . When a newborn experiences significant respiratory distress immediately following birth, it necessitates either an emergency or a prompt surgical remedy. The optimal surgical approach is generally as outlined below. This involves a complete substitution of the main pulmonary arteries ron patch is applied using 5/0 Tevdek sutures with interrupted pledgeted horizontal mattress stitches, following standard Procedure . It has been proposed that early surgical closure of the ductus arteriosus may enhance hemodynamic stability, thereby postponing the need for The monetary valve surgical procedure . When an infant exhibits symptoms such as wheezing and recurrent respiratory infections, it's crucial to proceed with surgical intervention soon after confirming the diagnosis . The outcomes of surgical treatment for absent pulmonary valve syndrome, a notably uncommon condition that appears across a broad range of severity, are challenging to predict due to the variability in risks associated with the surgical treatment of a particular child. The existence of various surgical methods developed during the early years of heart surgery indicates that the results were not optimal until the recent decade . In our case, the VSD patch was closed and the fibrous valve ring was biopsied, while the pulmonary regurgitation was left untreated. After 10 years, if there Is severe right ventricular failure, a procedure to create a pulmonary valve from a living patch may be conducted. The child is currently two years old and in good condition post-surgery with the VSD securely closed without any leakage, no gradient across the pulmonary valve, and pulmonary regurgitation still present.
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https://doi.org/10.1016/j.ijscr.2024.110076
|
[
"pulmonary",
"apvs",
"valve",
"respiratory",
"type",
"absent",
"absence",
"cases"
] |
[
{
"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 CLASSIFICATION ═══
【1. Other specified diseases of the respiratory system (CB40.Y)】
Synonyms: Secondary respiratory disorders | Respiratory disorders in diseases classified elsewhere | Diseases of bronchus, not elsewhere classified | Acquired bronchial diverticulum | acquired bronchus diverticulum
Hierarchy: Diseases of the respiratory system (12) → Certain diseases of the respiratory system (CB40) → Other specified diseases of the respiratory system
【2. Agenesis of lung (LA75.1)】
Definition: This refers to the absence or rudimentary residua of an undeveloped lung.
Synonyms: Pulmonary agenesis | absence of lung | aplasia of lung | apulmonism | congenital absence of lung
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the respiratory system → Structural developmental anomalies of lungs (LA75) → Agenesis of lung
【3. Pneumonia, organism unspecified (CA40.Z)】
Synonyms: Pneumonia | infectious pneumonia | PN - [pneumonia] | lobar pneumonia NOS | multifocal pneumonia
Hierarchy: Diseases of the respiratory system (12) → Lung infections → Pneumonia (CA40) → Pneumonia, organism unspecified
【4. Respiratory failure (CB41)】
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.
Synonyms: lung failure NOS | pulmonary failure
Excludes: Acute respiratory distress syndrome | Respiratory arrest | Respiratory distress of newborn
Hierarchy: Diseases of the respiratory system (12) → Respiratory failure
【5. Other injury of lung (NB32.3Y)】
Synonyms: Haematoma of lung | Traumatic hydropneumothorax | Acute traumatic lung congestion | Rupture of lung
Hierarchy: Injuries to the thorax → Injury of other or unspecified intrathoracic organs (NB32) → Certain injuries of lung (NB32.3) → Other injury of lung
|
CB40.Y
|
Other specified diseases of the respiratory system
|
A 57-year-old Thai man from Sa Kaeo, a province in the Eastern region of Thailand referred to a University Teaching Hospital in Bangkok due to swelling and pain at the left side of the neck for one month. One week prior he was admitted to the local hospital due to low-grade fever, difficulty swallowing and hoarseness. He received intravenous ceftriaxone and clindamycin for presumptive diagnosis of deep neck infection. He had history of hypertension treated with amlodipine 10 mg and enalapril 10 mg daily. He had habits of heavy alcohol drinking for 40 years, and smoking. He works at the department of fisheries. He swam, cleaned fish pond and mowed the lawn. Upon admission ( day 0 ), the patient's body weight was 52 kg, body mass index was 19.7 kg/m 2 . His vital signs were as follows: body temperature, 38.7 °C; blood pressure, 170/100 mmHg, pulse rate, 100 beats/min; respiratory rate, 24 breaths/min. On physical examination, mild pale conjuctivae, anicteric sclerae. The neck exam revealed pulsatile left neck mass size 5 × 5 cm in diameters, mild tender on palpation, no sign of inflammation. No limitation of neck movement. Oropharyngeal exam revealed bulging of left posterior pharyngeal wall and tonsil enlargement causing the narrowing of upper airway. Thyroid gland was not enlarged. Other exams included neurological exam were normal. Skin exam revealed multiple ill-defined scaly mild erythematous patches on both legs and dystrophic nails. Initial laboratory results showed anemia with hemoglobin concentration of 9.6 g/L and Hematocrit of 28%, MCV of 75 fl, white blood cell count of 6800 cells/mm 3 with 80% neutrophil 7.7% lymphocytes, platelet count of 574,000 cells/mm 3 . Hemoglobin typing was normal (HbF 0.2% HbA2 2.9% HbA 85.9%; HbA2A). Liver function test showed AST 87 U/L, ALT 97 U/L, ALP 127 U/L, GGT 936 U/L, TB 0.3 mg/dl, DB 0.1 mg/dl, TP 81.2 g/L, Alb 28.7 g/L. Fasting glucose of 95 mg/dL, HbA1C of 4.74%, BUN 14 mg/dL, Cr 1.05 mg/dL, Anti-HIV test was negative. Viral hepatitis profile were negative. His chest X-ray was normal. He was diagnosed with anemia of chronic disease, alcoholic hepatitis, and xerotic eczema. Computer tomography of the neck showed a concealed ruptured of left external carotid artery 0.9 × 1.9 cm in size with surrounding hematoma (3.6 × 3.6 ×5.8 cm) at medial aspect of an aneurysm resulted in narrowing of the upper airway . Urgent surgical exploration on day 0 revealed severe adhesion around pseudoaneurysm (size 5 × 6 cm) confined around common carotid artery, carotid bifurcation, extended to the angle of mandible. The diameter of pseudoaneurysm neck was one cm, located at medial wall of common carotid artery just distal to carotid bifurcation. External carotid artery was obliterated. Angiogram and balloon occlusion was performed at the left common carotid artery. External carotid artery and internal carotid artery were ligated at the arterial stump just beneath the angle of mandible. Pseudoaneurysm was resected and internal content show pus and clot. Surgical margins were not free in gross section. The pus was sent for bacterial culture. Blood agar plate revealed rare growth of whitish colony, direct exam from the colony revealed broad rare septate fungal hyphae. Infectious disease was consulted on day 5 of admission. Serum antibodies to pythium antigen using an in-house rapid immunochromatographic test were positive on day 5 . Sabouraud's glucose agar (SGA) grew fungal colony which identified as P. insidiosum by the induction of motile zoospore and confirmed by fungal broad-range 18S rDNA gene polymerase chain reaction. Pathology of carotid artery revealed acute suppurative inflammation with branching broad rare septate hyphae demonstrated by Gomori Methanamine Silver stain and Periodic acid-Shiff stain Immunohistochemistry stain for P. insidiosum was positive . Medical therapy with oral itraconazole 200 mg oral twice daily combined with terbinafine 250 mg oral twice daily were started on day 5 . Adjunctive immunotherapy with subcutaneous injection of PIA vaccine 500 microliter (4 mg/ml) was given on day 6 and 18 of admission. Day 5, Computer tomography of the aorta shows atherosclerotic change without aneurysm or dissection. Postoperatively, physical exams revealed narrowing of upper airway, hypoglossal nerve palsy on the left side without motor deficit. Neurology was consulted on day 6 . He was diagnosed with hypoglossal nerve palsy secondary to compression of carotid artery aneurysm. On Day 6 , MRI and MRA of the brain revealed pseudoaneurysm of carotid artery at left carotid-parapharyngeal spaces (2.8 × 2.0 × 3.1 cm) associated with extensive inflammation of the surrounding soft tissue resulting in mild narrowing of upper airway. Left common carotid artery was occluded along the origin to the cavernous part of left internal carotid artery with the evidence of wall enhancement. Multifocal cerebritis consistent with cerebral septic emboli and leptomeningeal enhancement at the left cerebral hemisphere . The patient underwent second exploration of the left neck on day 9 aiming to remove the residual infected necrotic tissue. Operative findings revealed pus with necrotic soft tissue extended to parapharyngeal space, however artery cannot be defined. The radical neck dissection could not be performed due to the morbidity outweigh the possibility of the cure. Tissue specimen revealed identical findings with the first operation. On day 6 , the dosage of terbinafine was increased to 250 mg three times daily, itraconazole was continued. The patient and family decided for palliative care, no aggressive treatment. He was discharged on day 19 of hospitalization. On day 29 after discharge, upon an outpatient visit, his family mentioned that he developed progressive right hemiparesis over the two days after discharge. Physical exams revealed healed surgical wound of the left neck, narrowing of upper airway, neurological exams revealed global aphasia, right facial palsy (upper motor neuron), motor power grade I on the right side. His family denied further investigation. He continued to take combination of oral terbinafine, itraconazole and PIA vaccine. On day 49 , upon an outpatient visit, he had flaccid hemiparesis on the right side without other deficit. He received fourth dose of PIA vaccine and continued oral itraconazole and terbinafine. On day 82 , he expired at a local hospital due to complication of diseases. Fig. 1 A. Computer tomography of the neck (coronal plain) showed a concealed ruptured of left external carotid artery aneurysm with surrounding hematoma at the medial aspect resulted in the narrowing of upper airway. B. Computer tomography of the neck (cross sectional plain). Fig. 1 Fig. 2 Histopathology of carotid vessel. Power photomicrograph: [400 × Hematoxylin and eosin stain] revealed acute suppurative inflammation. A. Histopathology of carotid vessel. Power photomicrograph: [400 × Gomori Methanamine Silver stain] revealed branching broad rare septate hyphae. B. Histopathology of carotid vessel. Power photomicrograph: [400 × Immunohistochemistry stain for P. insidiosum stain] revealed branching broad rare septate hyphae with positive stain. Fig. 2 Fig. 3 Magnetic resonance of the brain (T2FS) showed multifocal foci of restricted diffusion with internal microhemorrhage consistent with cerebral septic emboli and leptomeningeal enhancement at the left cerebral hemisphere. Fig. 3
| 3.947266
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|
sec[1]/p[0]
|
en
| 0.999997
|
30013898
|
https://doi.org/10.1016/j.mmcr.2018.05.003
|
[
"carotid",
"artery",
"neck",
"stain",
"narrowing",
"airway",
"exam",
"rare"
] |
[
{
"code": "8D88.Y",
"title": "Other specified autonomic neuropathies"
},
{
"code": "8B10.Y",
"title": "Other specified transient ischaemic attack"
},
{
"code": "2F9A",
"title": "Neoplasms of unknown behaviour of endocrine glands"
},
{
"code": "BD55",
"title": "Asymptomatic stenosis of intracranial or extracranial artery"
},
{
"code": "NA60.00",
"title": "Laceration of carotid artery, minor"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Other specified autonomic neuropathies (8D88.Y)】
Synonyms: Autonomic neuropathy due to Fabry’s Disease | Autonomic neuropathy due to Refsum’s Disease | Autonomic neuropathy due to Allgrove syndrome | Autonomic neuropathy due to Tangier’s disease | Navajo Indian autonomic neuropathy
Hierarchy: Diseases of the nervous system (08) → Disorders of autonomic nervous system → Autonomic neuropathies (8D88) → Other specified autonomic neuropathies
【2. Other specified transient ischaemic attack (8B10.Y)】
Synonyms: Vertebrobasilar artery syndrome | vertebrobasilar arterial insufficiency | vertebrobasilar insufficiency | vertebro-basilar artery syndrome, course of resolution unspecified | Vertebrobasilar artery syndrome, complete resolution within 1 to 24 hours
Hierarchy: Cerebrovascular diseases → Cerebral ischaemia → Transient ischaemic attack (8B10) → Other specified transient ischaemic attack
【3. Neoplasms of unknown behaviour of endocrine glands (2F9A)】
Synonyms: endocrine gland tumour NOS | Neoplasms of unknown behaviour of thyroid gland | thyroid gland tumour NOS | Neoplasms of unknown behaviour of adrenal gland | adrenal gland tumour NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of endocrine glands
【4. Asymptomatic stenosis of intracranial or extracranial artery (BD55)】
Definition: Stenosis of intracranial or extracranial artery that has not caused TIA or cerebral ischemic stroke.
Synonyms: stenosis of carotid artery NOS | stenosis of internal carotid artery NOS | stenosis of precerebral arteries, not resulting in cerebral infarction | narrowing of basilar, carotid or vertebral arteries, not resulting in cerebral infarction | partial obstruction of basilar, carotid or vertebral arteries, not resulting in cerebral infarction
Excludes: Transient ischaemic attack | Cerebral ischaemic stroke
Hierarchy: Diseases of the circulatory system (11) → Diseases of arteries or arterioles → Asymptomatic stenosis of intracranial or extracranial artery
【5. Laceration of carotid artery, minor (NA60.00)】
Synonyms: incomplete transection of carotid artery | laceration of carotid artery NOS | superficial laceration of carotid artery
Hierarchy: Injuries to the neck → Injury of blood vessels at neck level (NA60) → Injury of carotid artery (NA60.0) → Laceration of carotid artery, minor
|
8D88.Y
|
Other specified autonomic neuropathies
|
A 61-year-old Japanese man who had no significant medical, family, or psycho–social history was admitted with a 2-week history of fever, whole-body lymph node swelling, and abdominal swelling caused by hepatosplenomegaly. He was diagnosed with peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), by histopathological analysis of the lymph nodes. On admission, his laboratory data showed pancytopenia, hyperbilirubinemia, acute kidney injury (AKI), and disseminated intravascular coagulation (DIC) (Table 1 ). Bone marrow aspiration revealed proliferating hemophagocytosis and lymphocytes, and we diagnosed the patient with lymphoma associated with hemophagocytic lymphoma (LAHS). The day after he was admitted to our hospital, he was treated with etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin (EPOCH) chemotherapy. He responded to the chemotherapy; on day 9 after admission, DIC, hyperbilirubinemia, AKI, and hepatosplenomegaly improved. However, the period of bone marrow suppression from day 9 to day 19, lasted for 11 days, and he developed febrile neutropenia (FN). From day 9 after admission, he was treated for FN with meropenem 6 g/day and micafungin 100 mg/day, and was administered granulocyte colony-stimulating factor (G-CSF). His blood cultures were negative. On day 10, computed tomography of the chest revealed bilateral diffuse infiltration shadow of the lungs, and due to progressive respiratory failure, he required noninvasive positive pressure ventilation . However, on day 15, type 2 respiratory failure progressed. The findings from a blood gas test were as follows; pH 7.18, PaO 2 77.6 mmHg, PaCO 2 72.3 mmHg, \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$${\text{HCO}}_{3}^{ - }$$\end{document} HCO 3 - 26.8 mmol/L, and PaO 2 to FiO 2 (P/F) ratio 110. He underwent tracheal intubation for mechanical ventilation. Bronchoscopic findings revealed a widespread pseudomembranous necrotizing tissue formation covering the trachea and the bronchi, and numerous filamentous fungal hyphae infiltration were revealed in the pathologic examination . In addition, the β- d -glucan level was significantly increased to 1160 pg/mL, and the Aspergillus galactomannan antigen level [on enzyme-linked immunosorbent assay(ELISA)] was > 5.0. Aspergillus fumigatus was isolated from a pseudomembrane culture. We started L-AmB at 3 mg/kg/day. After intubation, he developed AKI with worsening of his general condition; therefore, we started continuous renal replacement therapy (CRRT). We performed daily bronchoscopy for pseudomembrane and necrotic tissue removal to release atelectasis. However, he was placed under deep sedation and fully controlled ventilation, and type 2 respiratory failure was worsened for atelectasis and obstructive pneumoniae. The findings from a blood gas test were as follows; pH 7.141, PaO 2 67.9 mmHg, PaCO 2 67.9 mmHg, \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$${\text{HCO}}_{3}^{ - }$$\end{document} HCO 3 - 22.9 mmol/L, (FiO 2 0.8, PEEP 15 mmHg), and P/F ratio, 86. The patient showed severe hypoxemia (P/F = 86) under PEEP 15 mmHg, and severe respiratory acidosis with a Murray score of 4, so we initiated VV-ECMO as a reversible condition. Since severe respiratory failure occurred due to Aspergillus pseudomembrane that fills the tracheobronchial airway, systemic administration of only L-AmB was thought to be inadequate. After VV-ECMO, we continued to perform bronchoscopy three times a week for pseudomembrane removal and intratracheal instillation of aerosolized L-AmB. With the informed consent of his family, off-label use of L-AmB was administered via intratracheal instillation as a life saver. The L-AmB dose was referenced as an AmB attachment; 1 vial (50 mg) was dissolved in 10 mL of distilled water and 0.2–4 mL (1–20 mg) of the vial was added to it and further diluted (0.1–2 mg/mL of amphotericin B) in about 10 mL of distilled water. The L-AmB instillation allowed targeted high local drug concentration, which led to an improvement. In addition, we increased the systemic dose of the L-AmB to 6 mg/kg/day (the dose was increased to twice the normal dosage). Ten days after initiating VV-ECMO, a bleeding event from the gastric vestibular area was reported; however, VV-ECMO was successfully weaned after 14 days, with the improvement of lung function. Bronchoscopy revealed disappeared pseudomembrane necrotizing tissue . However, although ITBA improved, the patient developed an impairment of consciousness caused by central nervous system involvement of the lymphoma, at the same time, splenomegaly and enlarged inguinal lymph nodes were revealed again, soluble IL2 receptor was elevated , and uncontrollable metabolic acidosis had developed by lymphoma; the patient developed recurrent lymphoma and he died 50 days after admission. Table 1 Laboratory data on admission Hematology Biochemistry Arterial blood gas (room air) WBC 1800 /μL T-Bil 7.6 U/L pH 7.39 Neut 94.0 % D-Bil 5.5 mg/dL PaCO 2 30.3 mmHg Lym 5.5 % TP 5.3 mg/dL PaO 2 105.5 mmHg Mono 0.5 % Alb 3.1 mg/dL \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$${\text{HCO}}_{3}^{ - }$$\end{document} HCO 3 - 17.9 mmol/L Eosino 0 % AST 678 U/L BE − 6.2 mmol/L Baso 0 % ALT 312 U/L Lactate 7.24 mmol/L RBC 272 × 10 4 /μL LDH 3828 U/L Hb 9.0 g/dL γGTP 155 U/L MCV 92.3 fl ALP 297 U/L Plt 3.1 × 10 4 /μL BUN 47.4 mg/dL Coagulation Cre 1.96 mg/dL PT–INR 1.23 eGFR 27.9 APTT 67.4 s UA 9.9 mg/dL Fib 86.0 mg/dL Na 131 mEq/L FDP 27.3 mcg/mL K 4.6 mEq/L Ddimer 11.9 mcg/mL Cl 99 mEq/L Ca 7.8 mg/dL IP 5.4 mg/dL GLU 85 mg/dL CRP 5.33 mg/dL HbA1c 5.7 % sIL2R 26,824 U/mL WBC white blood cell, Neu neutrophil, Lym lymphocyte, Mono monocyte, Eosino eosinophil, Baso basophil, RBC red blood cell, Hb hemoglobin, Plt platelet, PT prothrombin time activity, PT–INR prothrombin time activity–international normalized ratio, APTT activated partial thromboplastin time, Fib fibrinogen, FDP fibrin/fibrinogen degradation products, T-Bil total bilirubin, D-Bill direct bilirubin, TP total protein, Alb albumin, AST aspartate aminotransferase, ALT alanine aminotransferase, LDH lactate dehydrogenase, BUN blood urea nitrogen, Cre creatinine, eGFR estimate glomerular filtration rate, UA uric acid, Glu glucose, CRP C-reactive protein, HbA1c hemoglobin A1c, sIL2R soluble IL2 receptor, BE base excess Fig. 1 Chest computed tomography showed infiltration and bronchial thickening of right upper lobe Fig. 2 Bronchoscopy revealed diffuse wide raised pseudomembrane necrotizing tissue at right upper lobe ( a ). Pseudomembrane obstruction from the trachea to the left main bronchus resulted in atelectasis ( b ). Pathological finding of pseudomembrane showed numerous filamentous fungal hyphae (Grocott–Gomori’s methenamine silver stain, ×100) ( c ). After systemic and intratracheal instillation of liposomal amphotericin B, pseudomembrane necrotizing tissue disappeared ( d )
| 4.097656
| 0.969727
|
sec[1]/p[0]
|
en
| 0.999998
|
36536458
|
https://doi.org/10.1186/s13256-022-03692-1
|
[
"usepackage",
"pseudomembrane",
"mmhg",
"blood",
"lymphoma",
"document",
"admission",
"respiratory"
] |
[
{
"code": "1A04",
"title": "Intestinal infections due to Clostridioides difficile"
},
{
"code": "9A60.Y",
"title": "Other specified conjunctivitis"
},
{
"code": "DA24.Y",
"title": "Other specified oesophagitis"
},
{
"code": "1C1H.0",
"title": "Other Vincent infections"
},
{
"code": "GC00.0",
"title": "Trigonitis"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Intestinal infections due to Clostridioides difficile (1A04)】
Definition: A disease of the colon, caused by an infection with toxigenic strains of the gram-positive bacteria Clostridioides difficile (formerly known as Clostridium difficile). This disease is characterised by colitis, diarrhoea, abdominal pain, and fever. Transmission is commonly by direct or indirect contact. A disturbance of the normal bacterial flora of the colon, e.g. due to antibiotic treatment, faci...
Synonyms: Clostridium difficile diarrhoea | colitis due to Clostridium difficile | Clostridium difficile gastroenteritis | C diff colitis | necrotizing enterocolitis due to clostridium difficile infection
Excludes: Necrotising enterocolitis of newborn
Hierarchy: Certain infectious or parasitic diseases (01) → Gastroenteritis or colitis of infectious origin → Bacterial intestinal infections → Intestinal infections due to Clostridioides difficile
【2. Other specified conjunctivitis (9A60.Y)】
Synonyms: Infectious conjunctivitis | Bacterial Conjunctivitis | Protozoal conjunctivitis | Pseudomembranous conjunctivitis | Parinaud's oculoglandular syndrome
Hierarchy: Disorders of the eyeball - anterior segment → Disorders of conjunctiva → Conjunctivitis (9A60) → Other specified conjunctivitis
【3. Other specified oesophagitis (DA24.Y)】
Synonyms: Oesophagitis due to Crohn disease | Oesophagitis associated with human immunodeficiency virus disease | Oesophagitis due to systemic autoimmune diseases | Oesophagitis due to sarcoidosis | Oesophagitis due to dermatological diseases
Hierarchy: Diseases of the digestive system (13) → Diseases of oesophagus → Oesophagitis (DA24) → Other specified oesophagitis
【4. Other Vincent infections (1C1H.0)】
Synonyms: Vincent angina | Fusospirochaetal pharyngitis | pseudomembranous angina | Vincent laryngotracheitis | Vincent laryngitis
Hierarchy: Certain infectious or parasitic diseases (01) → Other bacterial diseases → Necrotising ulcerative gingivitis (1C1H) → Other Vincent infections
【5. Trigonitis (GC00.0)】
Definition: A condition of the bladder that is frequently idiopathic. This condition is characterised by inflammation of the nonkeratinizing squamous metaplasia in the trigone region of the bladder.
Synonyms: bladder trigonitis | pseudomembranous trigonitis | Urethrotrigonitis
Hierarchy: Diseases of the urinary system → Certain specified diseases of urinary system → Cystitis (GC00) → Trigonitis
|
1A04
|
Intestinal infections due to Clostridioides difficile
|
A 35-year-old Caucasian male presented with discomfort and pain in the popliteal area of the right knee that had persisted for 11 years, without a history of trauma. He complained of pain progression from sporadic to continuous, intermittent swelling, and decreased ROM for the last 3 years. The medical history of the patient was unknown and not reported during the first medical examination. He reported he had undergone a surgical procedure to his knee because of the pain, 4 years before, without any significant relief of the symptoms. The surgical report documented an arthroscopic synovectomy combined with medial meniscus partial meniscectomy for a peripherical lesion through the standard anteromedial and anterolateral portals. After the surgery he continued to use painkillers, but no improvement of ROM has been registered. Our first physical examination revealed swollen knee, tenderness to palpation, decreased ROM (15–80°), and valgus limb alignment. Anterior–posterior and varus/valgus stress stability were preserved. No meniscal signs and focal neurologic deficits were reported. The symptoms were exacerbated upon deep flexion. On occasions, he could not walk or go up and down stairs owing to pain and articular locking. Weight-bearing radiographs showed advanced stage of osteoarthritis (Kellgren–Lawrence stage II–III), mild valgus limb alignment, and multiple loose bodies in the posterior compartment . In order to accurately localize the loose bodies in the posterior compartments of the knee, a MRI was performed. MRI scan is able to characterize synovial lesions, owing to their high resolution of soft tissue and non‐calcified cartilage nodules. It revealed diffuse chondropathy, minimal synovial hypertrophy areas, multiple osteophytes in the intercondylar notch, and more than 30 low density loose radio dense bodies in the posteromedial compartment. Owing to this, we classified the SC as stage III according to Milgram . Considering the high grade of osteoarthrosis, we felt confident in performing a CT scan to better define the location of the osteophytes, the signs of osteoarthritis such as narrowing of the joint space and bone spurs, and the presence of calcifications and calcified loose bodies. In detail, the CT scan documented the bony structures, the morphology of the intercondylar notch, and the presence of osteophytes localized to the anterior and posterior side of the medial and lateral femoral condyles, allowing for a meticulous planning on how to reach the posterior compartment of the knee, passing anterior to posterior through a trans-notch passage . Even though the radiological exams showed an advanced grade of arthritis, the patient’s age and the desire for a full functional recovery drove us to perform a less invasive procedure than a total knee replacement, with the aim to preserve his native joint. For this reason we chose an arthroscopic procedure, considering that the large amount of loose bodies in the posterior knee might be the reason for the limited ROM and recurrent synovitis. The patient underwent an arthroscopic surgical approach 2 months after our first examination. Under spinal anesthesia, 2 g of intravenous cephalosporin was administered before inflation of a thigh tourniquet. The patient was placed supine on the operating table with the operative leg hanging in a 90° flexion position. A lateral post was placed just proximal to the knee at the level of the padded tourniquet, and a foot roll to prevent the hip from externally rotating and to maintain 90° of knee flexion. An arthroscopic examination of the anterior knee compartment was performed using conventional anteromedial (AM) and anterolateral (AL) portals, close to the patellar tendon border to facilitate passage of the arthroscope or instruments through the intercondylar notch. Multiple osteophytes were localized on the lateral side of medial femoral condyle, interfering with the trans-notch arthroscope passage. Meticulous debridement, removal of anterior osteophytes and a few anterior loose bodies that probably escaped from the posterior compartment, and tunneling, using a burr through the intercondylar osteophytes, were performed to allow the arthroscope to pass trans-notch . We performed the modified Gillquist maneuver to reach the posteromedial knee compartment. The arthroscope from AL portal was introduced into the posteromedial compartment through the intercondylar notch, passing between the lateral border of the medial femoral condyle and the medial border of the posterior cruciate ligament, with the knee in 90° of flexion. The posteromedial (PM) portal was than created under the guidance of trans-illumination by the light source introduced into the AL portal, with the intent to prevent injury to the neurovascular structures. With the arthroscope in the AL portal, the forceps were introduced through the PM portal and all the loose bodies were removed. To avoid additional accessory posterior portals, we used a 70° arthroscope to better explore the posteromedial knee compartment . To complete the procedure, synovectomy of the inflamed areas was performed to remove the active synovial proliferative tissue. A total of 33 loose bodies were removed from the posteromedial compartment . A final radiographic control documented the result of the procedure . Rehabilitation to recover ROM and full weight-bearing were permitted on postoperative day 1. Anti-thromboembolic prophylaxis, antibiotic prophylaxis, and pain killers were recommended. The patient was discharged on postoperative day 1. Histological examination showed that the loose bodies were composed mainly of hyaline cartilage embedded in the connective tissue, which confirmed the diagnosis of SC . After 1 month, pain and swelling were limited. ROM was 5–90° and the anteroposterior and varus/valgus stability was maintained. After 3 months, the wound was completely healed without swelling of the knee. The patient reported pain only during high demanding activities and ROM was 0–110°. At 12 months of FU, the patient was pain free with a complete recovery of ROM, and was able to participate in some light sport activity. The Tegner Lysholm Knee scoring scale was excellent (95/100 points). No recurrence of swelling or locking symptoms were reported. Fig. 1 a Anterior–posterior and b lateral radiographs of the right knee. Multiple loose bodies in the posterior compartment Fig. 2 a MRI axial view and b MRI sagittal view. Chondropathy, minimal synovial hypertrophy areas, multiple osteophytes in the intercondylar notch. c Computed tomography axial view: posterior loose bodies and multiple intercondylar notch osteophytes Fig. 3 a Arthroscopic tunneling, using a shaver, through the intercondylar osteophytes, b intercondylar notch tunnel performed to allow the arthroscope to pass trans-notch Fig. 4 a Arthroscopic approach to the posteromedial compartment of the knee using a posteromedial (PM) portal. A 70° arthroscope from the anterolateral (AL) portal through the intercondylar tunnel, with the knee in 90° of flexion and forceps introduced through the PM portal. b Arthroscopic view of loose bodies removal using a 70° arthroscope Fig. 5 Loose bodies removed from the posteromedial compartment of the knee Fig. 6 a Anterior–posterior and b lateral postoperative radiograph Fig. 7 Histological examination of loose bodies, composed mainly of hyaline cartilage embedded in the connective tissue
| 4.097656
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|
sec[1]/p[0]
|
en
| 0.999999
|
36494697
|
https://doi.org/10.1186/s13256-022-03667-2
|
[
"knee",
"loose",
"bodies",
"compartment",
"notch",
"intercondylar",
"osteophytes",
"posteromedial"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Inflammatory arthropathies, unspecified (FA2Z)】
Synonyms: polyarthritis NOS | inflammatory joint disease NOS | nonpyogenic arthritis NOS | arthritic nodosa | polyarthrosis NOS
Hierarchy: Diseases of the musculoskeletal system or connective tissue (15) → Arthropathies → Inflammatory arthropathies → Inflammatory arthropathies, unspecified
【2. Other specified superficial injury of knee or lower leg (NC90.Y)】
Synonyms: 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 | Knee haematoma
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injuries to the knee or lower leg → Superficial injury of knee or lower leg (NC90) → Other specified superficial injury of knee or lower leg
【3. Ankylosis of joint (FA34.4)】
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.
Synonyms: ankylosis | ankylosis of joint, site unspecified | frozen joint | fusion of joint | joint ankylosis
Excludes: stiffness of joint without ankylosis | Ankylosis of spinal joint
Hierarchy: Arthropathies → Certain specified joint disorders or deformities of limbs → Certain specified joint derangements (FA34) → Ankylosis of joint
【4. Chronic instability of knee, unspecified (FA33.4Z)】
Synonyms: Chronic instability of knee | instability of knee | old disruption of ligament of knee
Hierarchy: Certain specified joint disorders or deformities of limbs → Internal derangement of knee (FA33) → Chronic instability of knee (FA33.4) → Chronic instability of knee, unspecified
【5. Abrasion of knee (NC90.0)】
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injuries to the knee or lower leg → Superficial injury of knee or lower leg (NC90) → Abrasion of knee
|
FA2Z
|
Inflammatory arthropathies, unspecified
|
The most common etiologies of hypoglycemia were ruled out as follows: the absence of digestive disorders was not in favor of dumping syndrome; blood measurements did not show plasma cortisol deficiency (8 a.m. cortisolemia at 446 nmol/L [reference values-RV: 145–535], above the cut-off previously described by our team to exclude adrenal insufficiency , with ACTH at 53 ng/L [RV: 7–63]) or serum IGF1 (147 μg/L [RV: 37–165]) deficiency nor renal deficiency (creatininemia at 62 μmol/L [RV: 49–90]); liver function tests (plasma alanine aminotransferase, aspartate aminotransferase, total bilirubin, alkaline phosphatase, gamma-glutamyl transferase) were normal. The patient then underwent a 72-h fasting test with glycemia, insulinemia, and blood C-peptide measurements, controlled by positive capillary and urinary ketone bodies. The results were evocative of a hyperinsulinemic etiology of the hypoglycemic episodes ( Table 1 ). During this test, insulinemia was initially assessed by an assay recognizing insulin analogs [ , , ]. Therefore, the presence of a very high fasting insulinemia (190 mIU/L, RV: 5–25) in contrast to a normal blood C-peptide level raised the question of hidden injections of insulin analogs not admitted by the patient (exogenous hyperinsulinemia). This hypothesis however, was ruled out for four reasons: i) because the glycemia was not as low as could be expected (4.5 mmol/L for a reference range of 4.6–6.1) given the hyperinsulinemia at 190 mIU/L at day (D) 1 , ii) because the C-peptide concentration should be decreased in such a situation, iii) because the hyperinsulinemia was secondarily confirmed using an assay with low sensitivity to insulin analogs (Elecsys insulin assay on Cobas e411, Roche Diagnostics, Basel, Switzerland; Table 1 , Table 2 ) , and iv) because the insulinemia was not as high as expected using a third assay recognizing insulin analogs (iso-insulin ELISA kit from Mercodia, Uppsala, Sweden; Table 1 , Table 2 ) . The exclusion of exogenous hyperinsulinemia, with the addition of a normal C-peptide concentration, guided the diagnosis toward an endogenous hyperinsulinemia. The absence of hypoglycemia <2.5 mmol/L during the fasting test and the absence of pancreatic lesions on the three abdominal CT scans performed prior to her hospitalization were not in favor of an insulinoma. Furthermore, the intake of insulin secretagogues (repaglinide and sulphonylurea [glibenclamide, gliclazide, glimepiride, glipizide]) was investigated on a D5 sample using in-house liquid chromatography coupled with tandem mass spectrometry and was negative. These findings, associated with an elevated insulin/C-peptide molar ratio (sometimes >1 according to the sample and assay considered), finally guided the diagnosis toward an IAS. Therefore, free AIA measurement was performed using a radioimmunoassay detecting all immunoglobulin classes and subclasses (DiaSource Diagnostics, Ottignies-Louvain-la-Neuve, Belgium). The AIA level was extremely high (about 90 %, RV <8.2 %) on several samples ( Table 1 ). The presence of insulin-antibody complexes was then confirmed retrospectively using the two strategies below. Table 1 Laboratory investigations during the two hospitalizations in the endocrinology department. Table 1 Date (since first entry in the endocrinology department) and sample characteristics Plasmatic glycemia (4.6–6.1 mmol/L) Insulinemia on EDTA plasma (5–25 mIU/L) Insulinemia on serum (Elecsys insulin assay, Cobas e411) (2.6–24.9 mIU/L) Insulinemia on serum (Mercodia iso-insulin ELISA kit) (2–25 mIU/L) C-peptide on EDTA plasma (0.8–5.2 μg/L) Free AIA on serum (<8.2 %) D1 - 7:30 a.m. Fasting test Non-hemolyzed sample 4.5 ↘ 190 ↗↗ (0.91) a >1000 ↗↗↗ (>4.78) a 1813 ↗↗↗ (dilution 1/10) NA 4.5 89.35 % ↗↗↗ D2 - 8:00 a.m. Fasting test Non-hemolyzed sample 4.0 ↘ 129 ↗↗ (1.21) a >1000 ↗↗↗ (>9.36) a 1426 ↗↗↗ (dilution 1/10) NA 2.3 NA D3 - 8:10 a.m. Fasting test Non-hemolyzed sample 3.4 ↘ 92 ↗↗ (0.99) a 720.7 ↗↗↗ (7.76) a 934 ↗↗↗ (dilution 1/10) NA 2.0 NA D4 - 8:00 a.m. Fasting test Non-hemolyzed sample 2.9 ↘ 86 ↗↗ (1.32) a 738.7 ↗↗↗ (11.36) a 811 ↗↗↗ (dilution 1/10) NA 1.4 NA D5 - 9:00 a.m. Non-hemolyzed sample NA 193 ↗↗ >1000 ↗↗↗ 2782 ↗↗↗ (dilution 1/10) 27.5 ↗ NA 89.98 % ↗↗↗ D6 - 8:40 a.m. NA NA >1000 ↗↗↗ 28.0 ↗ NA 90.08 % ↗↗↗ D76–8:15 a.m. Non-hemolyzed sample 4.7 273 ↗↗↗ (1.01) a >1000 ↗↗↗ (>3.71) a 27.0 ↗ (0.10) a 5.8 ↗ 87.52 % ↗↗↗ D76–11:20 a.m. Non-hemolyzed sample NA 269 ↗↗↗ >1000 ↗↗↗ 54.6 ↗ NA NA The assays and their reference range are detailed in brackets in the column headings. C-peptide assay shows <0.01 % of cross-reactivity with insulin (supplier data). Please note that the AIA assays were performed on D1 and D5 samples having undergone a −20°c freeze-thaw cycle. AIA: anti-insulin antibodies; D: day; N: normal; NA: not available; PEG: polyethylene glycol; ↘ or ↗: decreased or increased, respectively, when compared to reference ranges. a (.) = insulin (pmol/L)/C-peptide (pmol/L) ratio. Conversion factor used : insulin mIU/L x 7.175 = insulin pmol/L; C-peptide μg/L x 333.33 = C-peptide pmol/L. Table 2 Insulin assay characteristics (supplier data unless otherwise specified). Table 2 Architect i2000 insulin assay (Abbott) Elecsys insulin assay on Cobas e411 (Roche) Iso-insulin ELISA assay (Mercodia) Cross-reactivity with recombinant human insulins and insulin analogs Assay sensitive to recombinant human insulins and insulin analogs [ , , ]. According to Moriyama et al. : Insulin lispro (10 mIU/L) 110 %, Insulin lispro (100 mIU/L) 100 %, Insulin aspart (10 mIU/L) 76 %, Insulin aspart (100 mIU/L) 75 %, Insulin glargine (10 mIU/L) 105 %, Insulin glargine (100 mIU/L) 83 %, C-peptide (1.10 7 ng/L) < 0.00001 %, Proinsulin (1.10 6 ng/L) < 0.005 %. According to Violin et al. : Insulin lispro 101 %, Insulin aspart (NovoRapid®) 82 %, Insulin aspart (NovoMix® 50) 30 %, Insulin detemir 139 %, Insulin glargine 110 %, Insulin glulisine 8.7 %, Insulin degludec 33 %. Assay sensitive to recombinant human insulins but absent or very low sensitivity to insulin analogs , except for insulin glargine metabolite M1 (cross-reactivity around 22 % ). From supplier and : C-peptide (100 ng/ml) not detectable, Human proinsulin 0.05 %, Proinsulin des (31–32) 20 %, Proinsulin des (64–65) 0.1 %. Assay sensitive to recombinant human insulins and insulin analogs . From supplier: Insulin lispro 112 %, Insulin aspart 100 %, Insulin detemir 28 %, Insulin glargine 58 %, Insulin glargine M1 47 %, Insulin glargine M2 32 %, Insulin glulisine 123 %, C-peptide <0.1 %, Proinsulin 54 %, Proinsulin des (31–32) 58 %, Proinsulin split (32–33) 56 %, Proinsulin des (64–65) 66 %, Proinsulin split (65–66) 78 %. Assay principle Chemiluminescent microparticle immunoassay Sandwich one step Electrochemiluminescence immunoassay Sandwich one step ELISA Sandwich one step Dilution factor of plasma/serum sample induced by addition of reagents during assay 5.2 8.5 3 Incubation time in the presence of capture and detection antibodies 25 minutes 9 minutes 1 hour Incubation temperature in the presence of capture and detection antibodies 37°c 37°c Room temperature (18–25°c) Hook effect Absent at least until 30000 mIU/L Absent at least until 20000 mIU/L Absent at least until 2000 mIU/L Upper limit of linearity 300 mIU/L 1000 mIU/L 100 mIU/L Lower limit of measurement range 1 mIU/L (limit of detection) 0.2 mIU/L (limit of detection) 3 mIU/L (first calibration point)
| 4.273438
| 0.599121
|
sec[1]/sec[1]/p[1]
|
en
| 0.999998
|
PMC11280251
|
https://doi.org/10.1016/j.heliyon.2024.e34009
|
[
"insulin",
"assay",
"peptide",
"sample",
"proinsulin",
"analogs",
"fasting",
"test"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Insulin-resistance syndromes (5A44)】
Synonyms: Insulin-resistance syndrome type A | Insulin-resistance syndrome type B | Rabson-Mendenhall syndrome | Laminopathy type Decaudain-Vigouroux | Laminopathy with severe metabolic syndrome and myopathy
Hierarchy: Endocrine, nutritional or metabolic diseases (05) → Endocrine diseases → Other disorders of glucose regulation or pancreatic internal secretion → Insulin-resistance syndromes
【2. Other specified disorders of glucose regulation or pancreatic internal secretion (5A4Y)】
Synonyms: Other hypoglycaemia | Hyperinsulinaemia | hyperinsulinism | functional hyperinsulinaemia | Hyperinsulinemia due to ectopic tumour source
Hierarchy: Endocrine, nutritional or metabolic diseases (05) → Endocrine diseases → Other disorders of glucose regulation or pancreatic internal secretion → Other specified disorders of glucose regulation or pancreatic internal secretion
【3. Presence of endocrine implants (QB51.5)】
Synonyms: presence of insulin pump
Hierarchy: Reasons for contact with the health services → Presence of device, implants or grafts → Presence of devices other than cardiac or vascular implants (QB51) → Presence of endocrine implants
【4. Fat hypertrophy (EF02.0)】
Definition: Focal hypertrophy of subcutaneous adipose tissue. It is a common sequela of long-term insulin injection into the skin.
Synonyms: Insulin-induced localised fat hypertrophy | Insulin-induced lipohypertrophy
Hierarchy: Disorders of the dermis or subcutis → Disorders of subcutaneous fat → Certain noninflammatory disorders of subcutaneous fat (EF02) → Fat hypertrophy
【5. Other or unspecified medical devices associated with injury or harm, prosthetic or other implants, materials or accessory devices (PK9C.2)】
Synonyms: 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
Hierarchy: Causes of healthcare related harm or injury → Surgical or other medical devices, implants or grafts associated with injury or harm in therapeutic use → Other or unspecified medical devices, implants or grafts associated with injury or harm (PK9C) → Other or unspecified medical devices associated with injury or harm, prosthetic or other implants, materials or accessory devices
|
5A44
|
Insulin-resistance syndromes
|
An 81-year-old male, a non-smoker, presented to our hospital in January 2023 during the Omicron wave of the COVID-19 pandemic in China. He had been experiencing fever, cough, and expectoration for 14 days, despite self-symptomatic treatment, and presented progressive weakness. Notably, he had no history of exertional dyspnea. His medical history included rheumatoid arthritis without lung involvement, and he had received two doses of Sinovac COVID-19 Vaccine. On physical examination, he had a temperature of 38.8 °C, pulse rate of 105 beats/min, respiratory rate of 18 breaths/min, blood pressure of 150/90 mmHg, and percutaneous oxygen saturation of 95% on room air at rest. Chest auscultation revealed clear lung sounds. A reverse transcription-polymerase chain reaction (RT-PCR) test confirmed severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2) infection. Blood tests showed elevated C-reactive protein (CRP) level of 121.83 mg/L, white blood cell (WBC) count within the normal range of 6.9 × 10 9 /L, low lymphocyte count of 0.95 × 10 9 /L, elevated alanine transaminase (ALT) of 103 U/L and aspartate transaminase (AST) of 142 U/L, and lactate dehydrogenase (LDH) of 437 U/L. A chest CT scan on day 14 from symptom onset revealed bilateral patchy consolidation and ground-glass opacities , consistent with moderate COVID-19. Due to his age, underlying conditions, weakened state, and history of receiving methotrexate before the onset of fever, moxifloxacin was given besides prone breathing and optimal supportive care, maintaining oxygen saturation above 93% without supplemental oxygen. However, on day 19, he developed progressive chest tightness and dyspnea, along with mental symptoms. Blood gas analysis (with 3 L/min oxygen administration) showed a pH value of 7.47, partial pressure of carbon dioxide (PCO 2 ) of 32 mmHg, and partial pressure of oxygen (PO 2 ) of 74 mmHg [PaO 2 /FIO 2 (P/F): 224 mmHg]. Blood tests revealed a CRP of 77.93 mg/L, WBC of 6.3 × 10 9 /L with a lymphocyte count of 0.96 × 10 9 /L, ALT of 45 U/L, AST of 36 U/L, LDH of 440 U/L, and elevated D-dimer of 38.5 mg/L. Subsequent chest CT scanning performed on day 20 showed the progression of lung lesions, and a subpleural air bronchogram in the anterior segment of the right upper lobe . Treatment escalated to include dexamethasone therapy (5 mg/day for 6 days) as severe COVID-19 was diagnosed, and moxifloxacin was halted based on its potential mental side effects. Although no signs of pulmonary embolism were found based on chest CT and echocardiography, anticoagulant therapy with low molecular weight heparin was administrated, as the B ultrasound of bilateral lower limb veins demonstrated highly probably thrombosis in the left peroneal vein and intramuscular veins. On day 26, the CT scan was repeated due to unimproved dyspnea and oxygenation status, indicating significantly aggravated lung consolidation as well as subpleural cystic lesions (4.5 cm × 3.5 cm and 2.5 cm × 1.7 cm, respectively). The proximal bronchi also had a thickened wall and constricted lumen in the anterior segment of the right upper lobe . Post-infection organizing pneumonia was suspected, and treatment was modified to methylprednisolone (40 mg/day) for enhanced effectiveness. As the patient was too weak to expectorate sputum, and glucocorticoid therapy was continued, the empirical ceftriaxone was administered, which gradually improved the patient’s condition. On day 38, his dyspnea and lung consolidation were significantly alleviated following anti-inflammatory treatment with methylprednisolone (40 mg/day for 7 days, and gradually tapered), in addition to optimal supportive care, prone breathing, and ceftriaxone treatment. Unexpectedly, subpleural cystic lesions in the anterior segment of the right upper lobe remained mostly unaltered, presenting as a cavity-like lesion with an air-fluid level in the dorsal segment of the right lower lobe , and another cystic lesion in the posterior basal segment of left lower lobe . No pathogen was found after repeated sputum smear staining and cultures, as well as the detection of 1,3-β-d-glucan (G test) and galactomannan (GM test) in serum, tuberculous interferon release assay, and X-pert MTB/RIF assay. Bronchoscopy was not performed as the patient and his family refused it. This culminated on day 42, when the patient presented with suddenly worsened dyspnea and severe cough, accompanied by reduced breath sounds in the right lung upon auscultation. Chest CT scanning demonstrated the evolution of the cavity-like lesion into an enlarged cystic lesion (5.7 cm × 4.0 cm) within the dorsal right lung, alongside right pneumothorax and subcutaneous emphysema . The rapid use of chest tube drainage and oxygen therapy produced an amelioration of the right pneumothorax and gradual resolution of the subcutaneous emphysema on day 50 , after which the patient was discharged. On day 80, a follow-up CT scan showed a gradual resolution of lesions in both lungs, but the subpleural cystic lesions were still present but constricted, with emphysema or air trapping, and bronchiectasis . On day 116, mild exertional dyspnea was observed, and chest CT demonstrated a recurrent right pneumothorax due to cyst rupture in the right upper lobe , alongside the improvement of cystic lesions in bilateral lower lobes . By day 156, the patient reported satisfactory recovery, with mild discomfort after exercise. Informed consent was obtained for the publication of this case. Fig. 1 Evolution of lung lesions and secondary pneumothorax in a convalescent COVID-19 case. A Day 14: peripheral distributed ground-glass opacities (GGO) and patchy consolidation in both lungs. B Day 20: progression of lung lesions, more significant lung consolidation, along with subpleural air bronchogram in the right upper lobe (red arrow). C Day 26: in addition to aggravated lung consolidation, subpleural cystic lesions and the proximal bronchi with thickened walls and constricted lumen in the anterior segment of the right upper lobe. D Day 38: a cavity-like lesion with air-fluid level in the dorsal segment of the right lower lobe, and improved consolidation throughout both lungs. E Day 38: a cystic lesion in the posterior basal segment of the left lower lobe. F Day 42: evolution of the cavity-like lesion into an enlarged cystic lesion in the dorsal segment of the right lung, along with right pneumothorax and subcutaneous emphysema, while the two cystic lesions in the right upper lobe remained unaltered (transverse section). G Day 42: right pneumothorax and subcutaneous emphysema, with two cystic lesions in the right upper lobe (Coronal section). H Day 50: implementation of chest tube drainage produced amelioration of right pneumothorax and resolution of subcutaneous emphysema. I Day 80: consolidation of both lungs further improved, with subpleural cystic lesions constricted but still present, along with emphysema or air trapping (red arrow), and bronchiectasis (yellow arrow). J Day 116: recurrent right pneumothorax due to cyst rupture in the right upper lobe, amelioration of PHC in the right lower lobe, and significant absorption of lung consolidation (transverse section). K Day 116: recurrent right pneumothorax due to cyst rupture in the right upper lobe (coronal section). L Day 116: recurrent right pneumothorax along with amelioration of the cystic lesion in the left lower lobe
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| 0.97168
|
sec[1]/p[0]
|
en
| 0.999999
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39030558
|
https://doi.org/10.1186/s12890-024-03169-5
|
[
"lobe",
"lung",
"cystic",
"lesions",
"pneumothorax",
"chest",
"consolidation",
"segment"
] |
[
{
"code": "CB40.2",
"title": "Pulmonary collapse"
},
{
"code": "LA75.0",
"title": "Accessory lobe of lung"
},
{
"code": "MD41",
"title": "Clinical findings on diagnostic imaging of lung"
},
{
"code": "LA75.1",
"title": "Agenesis of lung"
},
{
"code": "JA8A.1",
"title": "Malformation of placenta"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Pulmonary collapse (CB40.2)】
Synonyms: Atelectasis | lung collapse | pulmonary atelectasis | pulmonary collapse with atelectasis | collapse of lung NOS
Excludes: Primary atelectasis of newborn | tuberculous atelectasis, not confirmed | tuberculous atelectasis, confirmed | Respiratory tuberculosis, not confirmed | Respiratory tuberculosis, confirmed
Hierarchy: Diseases of the respiratory system (12) → Certain diseases of the respiratory system (CB40) → Pulmonary collapse
【2. Accessory lobe of lung (LA75.0)】
Definition: An extra lobe of lung beyond the 3 on the right and the 2 on the left
Synonyms: supernumerary lung lobe | azygos lobe of lung | azygos lobe fissure of lung | azygos lobe | accessory lung NOS
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the respiratory system → Structural developmental anomalies of lungs (LA75) → Accessory lobe of lung
【3. Clinical findings on diagnostic imaging of lung (MD41)】
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.
Synonyms: abnormal diagnostic imaging of lung | Hyperinflation of lung | Lung mass | Pulmonary lobe mass | Lung shadow
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings of the respiratory system → Clinical findings in the respiratory system → Clinical findings on diagnostic imaging of lung
【4. Agenesis of lung (LA75.1)】
Definition: This refers to the absence or rudimentary residua of an undeveloped lung.
Synonyms: Pulmonary agenesis | absence of lung | aplasia of lung | apulmonism | congenital absence of lung
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the respiratory system → Structural developmental anomalies of lungs (LA75) → Agenesis of lung
【5. Malformation of placenta (JA8A.1)】
Synonyms: variation of placenta form | deformity of placenta | placental deformity | Abnormal placenta NOS | pregnancy management affected by abnormal placenta
Hierarchy: Pregnancy, childbirth or the puerperium (18) → Maternal care related to the fetus, amniotic cavity or possible delivery problems → Maternal care related to placental disorders (JA8A) → Malformation of placenta
|
CB40.2
|
Pulmonary collapse
|
Diverticular disease of the colon is a multifactorial disease influenced by ethnicity, diet, and possibly genetics . It is also an age-related disease affecting less than 10% of people younger than the age of 40 to two-thirds (67%) of the population older than 80 [ 2 – 4 ]. In one study, diverticulum of the descending colon and sigmoid was recorded in more than 5% of postmortem examinations performed in persons aged 40 and older at the Mayo Clinic . The disease has a high prevalence in Western countries. It affects mostly the distal colon, with 90% of cases having a sigmoid involvement and 15% having a right-sided diverticulum . Between 10% and 20% of patients affected with diverticular disease present with diverticulitis [ 2 – 4 ]. Perforation can be localized and cause an abscess, which may be drained under CT guidance or it may perforate into the free peritoneal cavity and cause extensive peritonitis . To our knowledge, there have been only three descriptions of a sigmoid diverticulitis perforation into an inguinal hernia [ 6 – 8 ] and only one previous case of a subcutaneous emphysema with necrotizing fasciitis caused by a sigmoid diverticulitis perforation reported in the literature . Furthermore, we believe that this present case report is the first description of subcutaneous emphysema caused by a diverticulum perforating into an incisional hernia following an appendectomy. In both of our cases, perforation of the diverticulum into the extraperitoneal space occurred via a point of weakness in the abdominal wall. In the first case, it was through an incisional hernia. By definition, these hernias develop at the site at which an incision has been made for a previous surgery, complicate 5% to 11% of wound closures , and carry a relatively high mortality rate (5.3%) with complications such as an enterocutaneous fistula, adhesions, and strangulation . In the second case, the sigmoid diverticulum perforated into an inguinal hernia. Salemis et al. described a case of an incarceration of Meckel's diverticulum through a ventral incisional defect , while Alvarez-Zepeda et al. reported a case of perforated sigmoid diverticulum in a Spigelian hernia causing a necrotizing fasciitis . Both of these cases are already very rare presentations of diverticulum perforation. Diverticula most commonly form due to a rise in intraabdominal pressure, such as that caused by muscle contractions during straining for defecation. They usually occur on the antimesenteric border, at a point of weakness on the musculature wall of the bowel, mostly between the taeniae coli around the point of entry of the vasa recta . Patients in Western nations mostly present with left-sided involvement of the colon (90%), whereas, in contrast, those in Asian nations show right-sided involvement . Painter and Burkitt suggested that a poor-fiber diet predisposes individuals to diverticular disease by increasing transit time and decreasing stool volume and therefore, diverticular disease is a preventable condition. One feared complication of diverticular disease is perforation with abscess formation. In very rare instances, gas cracklings under the skin can be felt following perforation. Subcutaneous emphysema can be a nonspecific sign of rupture of a hollow organ . Air formation can occur by way of two different mechanisms, either (1) due to the air gradient between the perforated organ with the persistent peristalsis and the subcutaneous tissues or (2) secondary to an infection by gas-forming bacteria, mainly Escherichia coli , Clostridium sporogenes , Enterobacter aerogenes , Klebsiella , and Proteus . In some cases, perforation of the gastrointestinal tract may drain to the buttock, hip, thigh, or lower extremities or into the retroperitoneal space of the abdomen by dissecting along the anatomical planes . Morton reported a case of a sigmoid diverticulitis perforating into the right buttock, the left buttock, and the hips . In gas gangrene, due to the above-cited microorganisms, the air lies within the muscles, as opposed to that in the case of subcutaneous emphysema, where it lies in between the muscles and interstitial tissues . In our first case, the gas had spread via a complicated diverticulitis, which had caused a colocutaneous fistula and the perforation of a diverticulum in the abdominal wall, possibly secondary to a weakened abdominal wall and previous surgery. During her physical examination, the crackling was extending diffusely on her abdomen, but there were no signs of peritonitis or widespread cellulitis, suggesting a direct spread of gas into the subcutaneous tissues. Furthermore, the bacteria found with the culture swab were organisms that were part of the endogenous flora. The perforation was localized and occurred on the opposite side due to a redundant sigmoid loop. In our second case, which had a fatal outcome, the patient first presented with a cellulitis that evolved into a necrotizing fasciitis of the anterior lateral wall, requiring another surgery. There was also no gas gangrene, but, with the infection being more advanced, the case required a sigmoid resection, extensive necrosectomy, and a Hartmann terminal colectomy. Neither of the two patients were diabetics, but the second patient was obese and had hypertension, chronic renal insufficiency, and chronic venous lower extremity insufficiency, whereas the first patient was in good general heath otherwise. Fecal peritonitis, which has a high mortality rate (46%) , was not present in both of the cases we have described, as the feces had accumulated in the extraperitoneal space. CT scanning, which was necessary in both cases to determine the nature of the disease and the extent of the perforation, is the best imaging modality available to clinicians at this time and permits the elucidation of characteristic features for detecting the site of perforation, free gas, or fluid accumulation, allowing for an easier differentiation between air and fatty tissues. The findings can be wall thickening, pericolonic stranding, or a massive pneumoperitoneum . An emergency surgery with extensive debridement and necrosectomy is the usual outcome of such findings, as simple percutaneous drainage is rarely enough and a second operation is often needed. Agaba et al. reported a case of subcutaneous emphysema with muscular necrosis and necrotizing fasciitis from a perforated sigmoid diverticulitis that required two consecutive surgeries . During the operation, it is important for the surgeon to differentiate between a subcutaneous tissue infection with emphysema caused by free air drained in the subcutaneous tissue and necrotizing fasciitis and gas gangrene, as the latter two carry much higher rates of mortality and morbidity. This diagnosis should be considered in patient experiencing exquisite pain with a systemic toxicity, extensive cellulitis, crepitus, and/or hyponatremia . In both of our current cases, the wounds were then closed with a vacuum-assisted closure device very early on, which helps with wound healing by stimulating blood flow, decreasing local tissue edema, and removing excess fluid. Its use also increases cell division, tissue granulation, and bacterial clearance . Notably, despite the administration of prompt and appropriate treatment, the outcome of our second case was fatal. This outlines the difficulty that persists in handling such infections.
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en
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30155336
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https://doi.org/10.1155/2018/3030869
|
[
"perforation",
"sigmoid",
"disease",
"diverticulum",
"subcutaneous",
"cases",
"diverticulitis",
"emphysema"
] |
[
{
"code": "CA0A.Z",
"title": "Chronic rhinosinusitis, unspecified"
},
{
"code": "NB32.31",
"title": "Laceration of lung"
},
{
"code": "ME24.31",
"title": "Perforation of large intestine"
},
{
"code": "9A76",
"title": "Corneal ulcer"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Chronic rhinosinusitis, unspecified (CA0A.Z)】
Synonyms: Chronic rhinosinusitis | Chronic sinusitis | chronic sinusitis NOS | unspecified sinusitis | sinus disease
Hierarchy: Diseases of the respiratory system (12) → Upper respiratory tract disorders → Chronic rhinosinusitis (CA0A) → Chronic rhinosinusitis, unspecified
【2. Laceration of lung (NB32.31)】
Synonyms: perforated lung NOS | pulmonary perforation NOS
Hierarchy: Injuries to the thorax → Injury of other or unspecified intrathoracic organs (NB32) → Certain injuries of lung (NB32.3) → Laceration of lung
【3. Perforation of large intestine (ME24.31)】
Definition: Perforation of large intestine is a complete penetration of the colonic wall, often resulting in the leakage of luminal contents into the abdominal cavity. Perforation of large intestine results in the potential for bacterial contamination of the abdominal cavity and peritonitis.
Synonyms: bowel perforation NOS | Non-traumatic perforation of large intestine
Excludes: Diverticular disease of large intestine | Ulcerative colitis | Crohn disease | Neoplasms of the large intestine
Hierarchy: Clinical findings in the digestive system → Clinical manifestations of the digestive system (ME24) → Digestive system perforation (ME24.3) → Perforation of large intestine
【4. Corneal ulcer (9A76)】
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.
Synonyms: cornea ulcer | ulcerative keratitis | corneal ulcer NOS | Central corneal ulcer | Ring corneal ulcer
Hierarchy: Diseases of the visual system (09) → Disorders of the eyeball - anterior segment → Disorders of the cornea → Corneal ulcer
|
CA0A.Z
|
Chronic rhinosinusitis, unspecified
|
A 38-week gestational age baby boy, a product of a non-consanguineous marriage, arrived at our hospital’s emergency department (ED) on his first day of life, exhibiting signs of tachypnea and cyanosis. Upon arrival, a nasal cannula was connected to the patient with an oxygen flow 2L/m. Examination revealed no apparent dysmorphic features. The patient had respiratory distress (respiratory rate 80/min) with intercostal and subcostal recessions and tachycardia (heart rate 170–190/min). Peripheral pulses were intact. The cardiovascular examination revealed differential cyanosis (oxygen saturation in the left hand was 90%, and in the right hand and foot was 85% and 75%, respectively), intact peripheral pulsations, sternal heave with normal first heart sound (S1), accentuated second heart sound (S2), and a continuous machinery murmur heard over the infraclavicular area. Additionally, the liver was palpable 2 cm below the right costal margin. No significant blood pressure gradient was observed between the four limbs. Prostaglandin E1(PGE1) infusion started (0.05 mcg/kg/min) and was connected to continuous positive airway pressure (CPAP). Echocardiography revealed patent foramen ovale (PFO) with bidirectional shunting, moderate tricuspid regurgitation (TR) with estimated right ventricle systolic pressure (RVSP) of 70 mmHg, dilated right ventricle (RV), right atrium, large PDA with a right-to-left shunt, and diastolic reversal flow in the aortic arch, with no coarctation of the aorta. The RPA anomalously arose from the ascending aorta . Due to the significant flow reversal in the aortic arch, a bedside cranial ultrasound was performed, which excluded brain arteriovenous malformation. Multidetector computed tomography (MDCT) revealed an anomalous origin of RPA (5.5 mm) from the proximal ascending aorta, large PDA (7.5 mm) with a focal narrowing at the pulmonary arterial connection to a diameter of 5 × 3.5 mm, and left-sided aortic arch with an aberrant right subclavian artery with no focal aortic coarctation . After MDCT, we thought the PGE1 was unnecessary after excluding aortic coarctation or aortic arch interruption. We thought that the PGE1 may increase the steal from the aorta to the AORPA by decreasing the pulmonary vascular resistance, so prostaglandin E1 (PGE1) was discontinued. Following the cessation of PGE1, the patient’s condition progressively worsened over the following 8 h. The patient had more tachypnea, low cardiac output manifested by hypotension (BP 45/30 mmHg), oliguria, and hepatomegaly with liver 4 cm below the right costal margin, and arterial blood gas done at that time revealed severe metabolic acidosis with PH 6.9, HCO3 5 mmol/l, lactate 18 mmol/l, and BE −20. The patient was intubated, and an echocardiogram was performed immediately after deterioration, which revealed a closing ductus arteriosus along with a significantly reduced blood flow in the descending aorta and a dilated right ventricle (RV) with poor systolic function , and small PFO with right-to-left shunt. After this deterioration, a prostaglandin bolus was administered (0.2 mcg/kg), followed by continuous intravenous infusion, which was resumed at 0.2 mcg/kg/min. Additionally, epinephrine infusion was initiated at 0.05 mcg/kg/min. Ten minutes later, echocardiography revealed a large PDA with good flow and pulsatility in the descending aorta. A few hours later, the patient’s hemodynamics markedly improved, the patient’s blood pressure gradually increased to 65–70/40 mmHg (mean 50 mmHg), and the preductal oxygen saturation (in the left hand) was 85%, and then increased to 95%. Additionally, the patient’s urine output increased, and metabolic acidosis and hyperlactatemia gradually improved until they returned to normal. After this notable improvement, PGE1 was gradually tapered to 0.05 mcg/kg/min. Following this incident, the patient experienced renal and hepatic injury, and their liver and kidney function tests (LFTs and KFTs) were impaired. It took a few days for LFTs and KFTs to return to baseline after stabilizing the patient’s condition. The patient then underwent surgical repair in which deep hypothermic circulatory arrest was achieved with the body temperature lowered to 18 degrees Celsius. The PDA was divided. The arch appeared unremarkable from its exterior. The ascending aorta was surgically divided, and the right pulmonary artery (RPA) was separated from it. A surgical incision was made on the right side of the MPA. The RPA was anastomosed to MPA. A portion of the equine pericardium was used to augment the proximal RPA. CPB was gradually discontinued following successful hemostasis. Two intercostal drainage tubes were placed. The chest was covered for delayed closure. The patient was transferred to the pediatric cardiac intensive care unit (PCICU) with an open sternum. The patient’s hemodynamic status remained stable, and the sternum was closed two days later. The patient underwent extubation and transitioned smoothly to noninvasive ventilation (NIV), followed by a successful transition to room air. The initial postoperative echocardiogram revealed a peak systolic pressure gradient of 24 mmHg on the RPA with normal cardiac function. The patient was discharged home and returned in good condition for a follow-up appointment at the outpatient department after two months. Echocardiography was done in the clinic that revealed severe RPA stenosis, and the patient was booked for cardiac catheterization for balloon angioplasty . Fig. 1 A 2D image of parasternal long axis view showing the RPA arising from the ascending aorta (white asterisk). B Color Doppler of parasternal long axis view showing the RPA (white asterisk). C: Suprasternal long axis view showing RPA (white asterisk) and the retrograde flow in the aortic arch (yellow arrow). D The parasternal short-axis view shows the right-to-left PDA flow (yellow arrow). E An apical four-chamber view shows moderate tricuspid regurgitation (TR) with dilated RA and RV F Continuous wave Doppler at TR showing a TR gradient of 70 mmHg (indicating severe pulmonary hypertension). G Suprasternal long axis view showing the aortic arch and the proximal descending aorta with no coarctation. Ao: aorta: LA: left atrium, LPA: left pulmonary artery, LV: left ventricle, MPA: mean pulmonary artery, PDA: patent ductus arteriosus, RA: right atrium, RPA: right pulmonary artery, RV: right ventricle, TR: tricuspid regurgitation Fig. 2 A MDCT angiography with 3D reconstruction and right lateral projection showing the anomalous RPA from the ascending aorta (white arrow). B MDCT angiography with 3D reconstruction and posterior projection showing the aberrant right subclavian artery (white arrow) with no coarctation of the aorta. C MDCT angiography with 3D reconstruction and left lateral projection showing the large PDA (white arrow) connected to the descending aorta. D The parasternal short-axis view shows constriction of the PDA with right-to-left shunting after the discontinuation of prostaglandins (yellow arrow). E Preoperative chest x-ray showing plethoric right lung. F The parasternal short-axis view shows postoperative RPA stenosis (yellow arrow). G continuous wave Doppler showing postoperative RPA stenosis with diastolic runoff (yellow arrows). MDCT: multidetector computed tomography, MPA: mean pulmonary artery, PDA: patent ductus arteriosus, RPA: right pulmonary artery
| 4.035156
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|
sec[1]/p[0]
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en
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39903373
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https://doi.org/10.1186/s43044-025-00614-6
|
[
"aorta",
"showing",
"pulmonary",
"aortic",
"artery",
"view",
"flow",
"arch"
] |
[
{
"code": "BD5Z",
"title": "Diseases of arteries or arterioles, unspecified"
},
{
"code": "LA8A.3",
"title": "Congenital supravalvar aortic stenosis"
},
{
"code": "BD40.1",
"title": "Atherosclerosis of aorta"
},
{
"code": "BB71.Z",
"title": "Aortic valve insufficiency, unspecified"
},
{
"code": "LA8B.2Y",
"title": "Other specified congenital anomaly of aorta or its branches"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Diseases of arteries or arterioles, unspecified (BD5Z)】
Synonyms: artery disease NOS | arterial disease NOS | arteriolar disease NOS | disorder of artery NOS | arteriopathy
Hierarchy: Diseases of the circulatory system (11) → Diseases of arteries or arterioles → Diseases of arteries or arterioles, unspecified
【2. Congenital supravalvar aortic stenosis (LA8A.3)】
Definition: A congenital cardiovascular malformation with narrowing of the aorta at the level of the sinotubular junction which may extend into the ascending aorta.
Additional information: 'Congenital supravalvar aortic stenosis' is described as three forms: an hourglass deformity, a fibrous membrane, and a diffuse narrowing of the ascending aorta. Supravalvar aortic stenosis may involve the coronary artery ...
Synonyms: stenosis of aorta | supravalvular aortic stenosis | stricture of aorta | congenital narrowed aorta | ascending aortic stenosis
Excludes: Congenital aortic valvar stenosis
Hierarchy: Structural developmental anomalies of the circulatory system → Structural developmental anomaly of heart or great vessels → Congenital anomaly of a ventriculo-arterial valve or adjacent regions (LA8A) → Congenital supravalvar aortic stenosis
【3. Atherosclerosis of aorta (BD40.1)】
Synonyms: aorta atheroma | aorta calcification | aorta arteriosclerosis | aortic degeneration | aortic calcification
Hierarchy: Diseases of arteries or arterioles → Chronic arterial occlusive disease → Atherosclerotic chronic arterial occlusive disease (BD40) → Atherosclerosis of aorta
【4. Aortic valve insufficiency, unspecified (BB71.Z)】
Synonyms: Aortic valve insufficiency | aortic insufficiency | aortic valve incompetency | AI - [aortic incompetence] | incompetent aortic valve
Hierarchy: Heart valve diseases → Aortic valve disease → Aortic valve insufficiency (BB71) → Aortic valve insufficiency, unspecified
【5. Other specified congenital anomaly of aorta or its branches (LA8B.2Y)】
Synonyms: Congenital anomaly of ascending aorta | Hypoplasia of ascending aorta | Congenital ascending aorta aneurysm or dilation | congenital ascending aortic aneurysm or dilation | Congenital anomaly of aortic arch
Hierarchy: Structural developmental anomaly of heart or great vessels → Congenital anomaly of great arteries including arterial duct (LA8B) → Congenital anomaly of aorta or its branches (LA8B.2) → Other specified congenital anomaly of aorta or its branches
|
BD5Z
|
Diseases of arteries or arterioles, unspecified
|
A 30-year-old Japanese man was referred to our hospital because of fatigue, palpitation, body weight loss, finger tremor, and heat intolerance for 3 months. He was 166.2 cm in height and 60.2 kg in weight. His blood pressure was 110/50 mmHg, and his heart rate was 70 beats/minute and regular. His body temperature was 36.3 °C. The thyroid gland was slightly enlarged, non-tender, and softly elastic on palpation. Exophthalmos was not observed. Thyroid function test demonstrated elevated levels of FT3 and FT4, and complete suppression of TSH level, which was below the limit of detection. Serum anti-TSH receptor antibody (TRAb), anti-thyroid peroxidase antibody (TPOAb), and anti-thyroglobulin antibody (TgAb) were positive. Ultrasonography of the thyroid gland revealed diffuse enlargement without tumor lesions. The right and left lobes of the thyroid gland measured 49 × 24 × 20 mm and 54 × 22 × 20 mm, respectively. These results indicated that Graves’ disease was responsible for primary hyperthyroidism in this case. A diagnosis of Graves’ disease was made based on these findings. Methimazole (MMI) therapy was started at 15 mg/day. His symptoms improved gradually, and the dosage of methimazole was decreased as thyroid function normalized. After 6 months, methimazole therapy was continued at 10 mg/day, and the levels of TSH, FT3, and FT4 were maintained within the respective normal ranges. He was transferred to a local hospital. His thyroid function was followed up periodically, and the levels of FT3 and FT4 were elevated, but TSH levels were within normal range or slightly elevated on some occasions. At the age of 48 years, he was referred to our hospital for further endocrinological examination. On physical examination, his height was 165.4 cm, body weight was 58.6 kg, body mass index was 22.1 kg/m 2 , blood pressure was 122/70 mmHg, and his pulse was regular at 80 beats/minute. His body temperature was 36.8 °C. The thyroid was diffusely enlarged, softly elastic, and non-tender on palpation. Exophthalmos was not observed. Laboratory findings are presented in Table 1 . The results of thyroid function tests with methimazole treatment at 10 mg/day revealed serum levels of FT3, FT4, and TSH of 5.4 pg/mL (normal range 2.4–4.3 pg/mL), 1.7 ng/dL (normal range 0.9–1.8 ng/dL), and 4.77 μIU/mL (normal range 0.35–4.94 μIU/mL), respectively, indicating SITSH. Chest X-ray and electrocardiographic findings were normal. His bone mineral density was 0.712 g/cm 2 (88% of the young adult mean) in the femur. He showed no stigmata of thyrotoxicosis. Ultrasonography of the thyroid showed diffuse enlargement of both lobes with no tumor lesions. Increased blood flow was not detected on Color-Doppler imaging . Thyroid radioactive 123 I uptake was 11.8% in 3 hours (reference range, 5–15%) . The titer of TRAb was weakly positive, and the dosage of methimazole was decreased gradually to maintain normal TSH level . Six years after he visited our hospital, methimazole treatment was ceased, and the titer of TRAb became negative. These findings indicated that Graves’ disease was in remission. The symptoms of hyperthyroidism, such as finger tremors, palpitations, and weight loss, were not observed. After cessation of methimazole treatment, his thyroid hormone levels fluctuated and showed occasional elevation of FT3 or FT4 with normal TSH level. His state of SITSH persisted. Magnetic resonance imaging (MRI) was performed for the differential diagnosis of TSH producing pituitary tumor, and revealed no tumor lesions. The results of TSH-releasing hormone (TRH) stimulation test are presented in Table 2 . His TSH level showed a normal response to TRH stimulation. He was suspected to have RTH. A genetic investigation was conducted with the patient’s written informed consent. Blood samples were obtained, and genomic DNA was isolated from leukocytes. Direct sequencing analysis of the TRβ gene identified a heterozygous, missense point mutation in exon 8, AGA to AGC, resulting in substitution of arginine by serine at codon 282 (R282S) . He reported no thyroid disease in his family. Thyroid function tests and DNA analysis were not performed for other family members, because they declined to provide consent. Table 1 Laboratory data on arrival Urinalysis Biochemistry Endocrinological examination Color Yellow LDH 181 IU/L TSH 4.77 μIU/mL (0.35–4.94) pH 6.5 ALP 191 IU/L FT3 5.4 pg/mL (2.4–4.3) Glucose (−) γ-GTP 33 IU/L FT4 1.7 ng/dL (0.9–1.8) Protein (−) CK 151 IU/L Tg 3.0 ng/mL (0–33.7) Occult blood (−) LDL-C 121 mg/dL TRAb 3.9 IU/L (0–1.0) Ketone (−) TG 146 mg/dL TSAb 269% (0–120) HDL-C 48 mg/dL TPO Ab 536 IU/mL (0–16.0) Hematology Na 140 mEq/L Tg Ab 398 IU/mL (0–28) WBC 5600/μL K 4.2 mEq/L ACTH 18.4 pg/mL (7.2–63.3) RBC 485 × 10 4 /μL Cl 102 mEq/L cortisol 9.9 μg/dL (4.5–21.1) Hb 13.5 g/dL Ca 9.4 mEq/L GH 0.48 ng/mL (0.13–9.88) Ht 45.60% P 4.9 mEq/L IGF-1 127 ng/mL (89–248) Plts 26.4 × 10 4 /μL BUN 16 mg/dL PRL 7.72 ng/mL (3.6–12.8) CRP 0.1 mg/dL Cr 0.7 mg/dL FSH 6.74 mIU/mL (2.0–8.3) TP 7.5 g/dL UA 6.7 mg/dL LH 5.29 mIU/mL (0.8–5.7) Alb 4.5 g/dL FPG 81 mg/dL T-Bil 0.47 mg/dL HbA1c 5.50% AST 22 IU/L ALT 19 IU/L (Normal range) γ-GTP: γ-glutamyl transpeptidase; ACTH: adrenocorticotropic hormone; Alb: albumin; ALP: alkaline phosphatase; ALT: alanine aminotransferase; AST: aspartate aminotransferase; BUN: blood urea nitrogen; CK: creatine kinase; Cr: creatine; CRP: C-reactive protein; FPG: fasting plasma glucose; FSH: follicle stimulating hormone; FT3: free triiodothyronine; FT4: free thyroxine; GH: growth hormone; Hb: hemoglobin; HbA1c: glycated hemoglobin; HDL-C: high-density lipoprotein-cholesterol; Ht: hematocrit; IGF-1: insulin-like growth factor-1; LDH: lactate dehydrogenase; LDL-C: low-density lipoprotein-cholesterol; LH: luteinizing hormone; Plts: platelets; PRL: prolactin; RBC: red blood cells; T-Bil: total bilirubin; TG: triglyceride; Tg: thyroglobulin; Tg Ab: anti-thyroglobulin antibody; TP: total protein; TPO Ab: anti-thyroid peroxidase antibody; TRAb: anti-TSH receptor antibody; TSAb: thyroid-stimulating antibody; TSH: thyroid-stimulating hormone; UA: uric acid; WBC: white blood cells Fig. 1 Thyroid ultrasonography showed diffuse enlargement of both lobes with heterogeneous echogenicity. No tumor lesions were detected. Neither lobe showed increased blood flow on Color-Doppler imaging Fig. 2 Thyroid radioactive 123 I uptake showed no increase. The uptake was 11.8% in 3 hours (reference range, 5–15%) Fig. 3 Clinical course after anti-hyperthyroidism treatment for Graves’ disease. Thyroid hormone levels showed occasional SITSH in the periods of anti-hyperthyroidism treatment. Although this treatment led to remission of Graves’ disease, it failed to normalize the FT3, FT4, and TSH levels. SITSH persisted even after cessation of methimazole treatment Table 2 Thyrotropin-releasing hormone stimulation test Time (minutes) Basal 30 60 90 120 TSH (μIU/mL) 7.24 37.5 33.3 24.0 17.4 FT3 (pg/mL) 4.19 4.40 PRL (ng/mL) 7.16 88.03 51.12 29.90 20.89 FT3: free triiodothyronine; PRL: prolactin; TSH: thyroid-stimulating hormone Fig. 4 Direct sequencing analysis of TRβ gene in genomic DNA extracted from peripheral blood leukocytes. Sequence analysis revealed a heterozygous point mutation in exon 8, AGA to AGC, resulting in substitution of arginine by serine at codon 282 (R282S). The arrow indicates the site of the point mutation
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|
sec[1]/p[0]
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en
| 0.999996
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34560890
|
https://doi.org/10.1186/s13256-021-03061-4
|
[
"thyroid",
"blood",
"hormone",
"levels",
"anti",
"methimazole",
"antibody",
"range"
] |
[
{
"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 CLASSIFICATION ═══
【1. Thyroiditis, unspecified (5A03.Z)】
Synonyms: Thyroiditis | inflammation of thyroid | thyroiditis NOS
Hierarchy: Endocrine diseases → Disorders of the thyroid gland or thyroid hormones system → Thyroiditis (5A03) → Thyroiditis, unspecified
【2. Disorders of the thyroid gland or thyroid hormones system, unspecified (5A0Z)】
Hierarchy: Endocrine, nutritional or metabolic diseases (05) → Endocrine diseases → Disorders of the thyroid gland or thyroid hormones system → Disorders of the thyroid gland or thyroid hormones system, unspecified
【3. Other specified thyroiditis (5A03.Y)】
Synonyms: Riedel thyroiditis | Chronic invasive fibrous thyroiditis | Ligneous thyroiditis | Riedel struma | Drug-induced thyroiditis
Hierarchy: Endocrine diseases → Disorders of the thyroid gland or thyroid hormones system → Thyroiditis (5A03) → Other specified thyroiditis
【4. Acquired hypothyroidism, unspecified (5A00.2Z)】
Synonyms: Acquired hypothyroidism | hypothyrea | thyroid insufficiency | hypothyroidea | subthyroidism
Hierarchy: Disorders of the thyroid gland or thyroid hormones system → Hypothyroidism (5A00) → Acquired hypothyroidism (5A00.2) → Acquired hypothyroidism, unspecified
【5. Acute thyroiditis (5A03.0)】
Definition: Acute thyroiditis is a rare form of thyroiditis directly caused by an infection, frequently bacterial.
Synonyms: infectious thyroiditis | Acute thyroiditis due to bacterial infection | Acute thyroiditis due to fungal infection | Abscess of thyroid | thyroid gland abscess
Hierarchy: Endocrine diseases → Disorders of the thyroid gland or thyroid hormones system → Thyroiditis (5A03) → Acute thyroiditis
|
5A03.Z
|
Thyroiditis, unspecified
|
A 57-year-old Japanese man with a chief complaint of hoarseness visited the Otolaryngology Department at our hospital. A contrast-enhanced computed tomography (CT) scan showed a tumor, with its major axis measuring approximately 40 mm in the left upper lobe of the lung. The hilar and mediastinal lymph nodes were also swollen . Primary lung cancer was suspected, and the patient was directed to the Department of Respiratory Medicine on the same day that he was admitted to the Otolaryngology Department. He was diagnosed with lung adenocarcinoma, which was confirmed by bronchoscopy. We performed transbronchial lung biopsy (TBLB) for the primary lesion in the left upper lobe and endobronchial ultrasonography-guided transbronchial needle aspiration in the mediastinal lymph nodes 1 week after the first visit. Genetic mutation testing was not performed because the sample was insufficient. Subsequently, 18 F-fluorodeoxyglucose positron emission tomography/CT ( 18 F-FDG PET/CT) was performed, and abnormal accumulation was observed in the longitudinal hilar lymph nodes (#4L, #5, #10L), vertebral body, and left pleura. The results of the 18 F-FDG PET/CT and other findings led to the diagnosis of lung adenocarcinoma [cT2bN2M1c (PUL, OSS); stage IVB]. We recommended rebiopsy considering the possibility of a genetic mutation, but the patient refused the examination because of strong coughing during the first bronchoscopy and progressive symptoms of hoarseness. The patient strongly desired an early treatment, so we initiated first-line therapy with cisplatin (75 mg/m 2 ) and pemetrexed (500 mg/m 2 ) 3 weeks after the first visit. However, the patient developed grade 2 renal dysfunction [creatinine, 1.83 mg/dL; estimated glomerular filtration rate (eGFR), 31.23 mL/min/1.73 m 2 ], making it difficult to continue the therapy . We informed the patient about the need for a second bronchoscopy for consideration of further treatment and obtained his consent. Bronchoscopy was performed again, and the patient was diagnosed with ALK-positive NSCLC (immunohistochemistry, 3+; fluorescence in situ hybridization, 56%). In addition, progressive disease was noted . Development of progressive disease required approximately 9 months, and cisplatin and pemetrexed were administered over four cycles, after which pemetrexed alone was administered over five cycles. Thereafter, second-line therapy with alectinib (300 mg/day) was initiated. Three months after starting alectinib, chest CT showed that the primary lesion had shrunk, and we judged it to be a partial response, but the ground-glass opacities and infiltration continued to spread on both sides . At this time, the patient complained of dyspnea on exertion, and his saturation of percutaneous oxygen was approximately 82% in room air. Blood gas analysis showed that the partial pressure of arterial oxygen was 58.2 torr, indicating respiratory failure. A pulmonary function test revealed restrictive ventilatory impairment, with a vital capacity (VC) of 2.11 L and a %VC of 57.1%. A diffuse lung capacity for carbon monoxide (DLCO) of 4.84 mL/min/mmHg and a %DLCO of 22.8% indicated diffusion disorder. Bronchoalveolar lavage fluid (BALF) and TBLB samples were obtained at this time. The BALF showed an increased lymphocyte ratio (34.4%), and pathological analysis of the TBLB specimen revealed an organizing pneumonia (OP)/nonspecific interstitial pneumonia (NSIP) overlapping pattern. On the basis of the chest CT results, we suspected alectinib-induced DILD. However, we also considered respiratory infection or cancerous lymphangiopathy with cancer progression in the differential diagnoses. Laboratory testing revealed a white blood cell count of 9300/µL and a C-reactive protein level of 1.41 mg/dL. The BALF culture results were negative. The BALF was lymphocyte dominant, and no findings suggestive of cancer infiltration were observed in the TBLB specimen. On the basis of these results, we excluded the other conditions evaluated in the differential diagnosis. Our final diagnosis was alectinib-induced DILD. On the basis of the results of chest CT and bronchoscopic pathology, methylprednisolone was prescribed (1 g/day for 3 days). The patient then received oral prednisolone (0.8 mg/kg/day), which was gradually reduced, and he was administered steroids for about 3 months. Third-line therapy with nanoparticle albumin bound paclitaxel (nab-paclitaxel) (100 mg/m 2 ) was initiated after treatment with steroids. However, 3 months later, obstructive pneumonia associated with an increase in the size of the primary lesion was observed . Moreover, mild renal dysfunction persisted (creatinine, 1.63 mg/dL; eGFR, 35.45 mL/min/1.73 m 2 ). Because of this complication and upon the patient’s request for oral medication, lorlatinib (100 mg/day) was administered as fourth-line therapy. Treatment with antibiotics and lorlatinib reduced the lesion’s shadow, and no recurrence of DILD was observed. Thirty-three months after the start of treatment, stable disease has been maintained, and lorlatinib has been continued . Fig. 1 Treatment course. First-line treatment with cisplatin/pemetrexed shrunk the primary lesion and mediastinal lymph nodes ( a , b ), but PD was noticed 9 months after the beginning of treatment ( c ). Re-biopsy performed during treatment with cisplatin/pemetrexed indicated that the tumor was ALK-positive. Therefore, second-line treatment with alectinib was subsequently initiated. Although alectinib treatment was successful, the patient developed DILD 4 months after the start of treatment ( d ). After DILD was relieved upon treatment with steroids, third-line treatment with nab-paclitaxel was initiated, but PD was observed 4 months later ( e ). Thereafter, fourth-line treatment with lorlatinib was started and has been continued for more than 33 months while maintaining SD ( f ). PD progressive disease, ALK anaplastic lymphoma kinase, DILD drug-induced interstitial lung disease, SD stable disease, nab-paclitaxel nanoparticle albumin bound paclitaxel Fig. 2. Thin-slice CT before and after the onset of DILD. Prior to the onset of DILD ( a ), a shadow of the primary lesion was observed in the left upper lobe of the lung. No abnormal shadow was seen in the lung field. After the patient developed DILD ( b ), the primary lesion was observed to be shrinking. However, ground-glass opacities and infiltrations were seen spreading on both sides. CT computed tomography, DILD drug-induced interstitial lung disease Fig. 3 BALF and TBLB at the onset of DILD. Giemsa staining of the BALF at a magnification of ×200 ( a ) showing an increase in the proportion of lymphocytes (34.4%). Hematoxylin and eosin staining of the TBLB specimen at a magnification of ×200 ( b ) showing exudate and inflammatory cell infiltration in the alveolar space with alveolar wall thickness. No findings suggestive of cancer infiltration were observed. BALF bronchoalveolar lavage fluid, TBLB transbronchial lung biopsy, DILD drug-induced interstitial lung disease Fig. 4 Thin-slice CT before and after the administration of lorlatinib. Compared with the patient’s condition before the administration of lorlatinib ( a ), SD has been maintained 33 months after the start of lorlatinib ( b ), and no recurrence of DILD has been observed. CT computed tomography, SD stable disease, DILD drug-induced interstitial lung disease
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sec[1]/p[0]
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en
| 0.999997
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PMC9400211
|
https://doi.org/10.1186/s13256-022-03556-8
|
[
"dild",
"lung",
"disease",
"line",
"primary",
"tblb",
"lesion",
"balf"
] |
[
{
"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 CLASSIFICATION ═══
【1. Other specified diseases of the respiratory system (CB40.Y)】
Synonyms: Secondary respiratory disorders | Respiratory disorders in diseases classified elsewhere | Diseases of bronchus, not elsewhere classified | Acquired bronchial diverticulum | acquired bronchus diverticulum
Hierarchy: Diseases of the respiratory system (12) → Certain diseases of the respiratory system (CB40) → Other specified diseases of the respiratory system
【2. Agenesis of lung (LA75.1)】
Definition: This refers to the absence or rudimentary residua of an undeveloped lung.
Synonyms: Pulmonary agenesis | absence of lung | aplasia of lung | apulmonism | congenital absence of lung
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the respiratory system → Structural developmental anomalies of lungs (LA75) → Agenesis of lung
【3. Pneumonia, organism unspecified (CA40.Z)】
Synonyms: Pneumonia | infectious pneumonia | PN - [pneumonia] | lobar pneumonia NOS | multifocal pneumonia
Hierarchy: Diseases of the respiratory system (12) → Lung infections → Pneumonia (CA40) → Pneumonia, organism unspecified
【4. Respiratory failure (CB41)】
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.
Synonyms: lung failure NOS | pulmonary failure
Excludes: Acute respiratory distress syndrome | Respiratory arrest | Respiratory distress of newborn
Hierarchy: Diseases of the respiratory system (12) → Respiratory failure
【5. Other injury of lung (NB32.3Y)】
Synonyms: Haematoma of lung | Traumatic hydropneumothorax | Acute traumatic lung congestion | Rupture of lung
Hierarchy: Injuries to the thorax → Injury of other or unspecified intrathoracic organs (NB32) → Certain injuries of lung (NB32.3) → Other injury of lung
|
CB40.Y
|
Other specified diseases of the respiratory system
|
A 71-year-old female patient presented to the emergency ward in our psychiatric clinic. For about half a year, she had been suffering from a depressed mood, pronounced restlessness, a concentration disorder, diffuse anxiety, and sleep disturbances . The depressive symptoms had been very obvious for 3 weeks. She had no appetite and cried more often during conversations. Her sense of joy was reduced, and there was general listlessness. She suffers from early waking, feeling depressed in the morning, and feeling a deep sense of shame. She also described passive death wishes, but no concrete suicidal thoughts. She reports no relevant psychosocial stress factors and even when asked, describes no stress factors. After careful questioning of her daughter, we learned that her retention and memory disorders had already existed for a year . The daughter also spoke of her considerably impaired daily living skills, stating that she could probably no longer manage alone at home and depended on her daughter for help with self-care. Nursing help would also be needed at home to ensure that her medications are taken. The patient is a Kazakhstan native who has lived in Germany for 30 years. She is a widow with two children. She is a trained hotel manager, a pensioner, and lives alone. Her daughter has the power of attorney for her healthcare. There are no known psychiatric diseases in her family history. She had no known somatic diseases. In her psychopathological examination, we observed indications of concentration and comprehension disturbances as well as impaired retentiveness and memory. Furthermore, she worries about the future. Her emotional state was depressed, and there was a disturbance of vital feelings. She also reported being less able to feel joy, a loss of interest, and reduced drive. She had also become socially withdrawn. Her neurological examination, however, was inconspicuous apart from the cognitive dysfunction. Her physical examination revealed no pathologies. Due to her serious memory problems that started before the depressive symptoms, we conducted extensive neuropsychological testing to make a differential diagnosis. The detailed neuropsychological examination revealed significant impairments in several cognitive domains such as orientation, semantic word fluency, confrontation naming, cognitive processing speed, visuomotor coordination, the clock test, action planning, working and figural memory, encoding and consolidation of verbal non-associated information, encoding and delayed recall of complex verbal content, visuoconstructive skills, and visual-spatial perception objectified . However, her phonematic word fluency was not age appropriate. From a neuropsychological point of view, the aforementioned dysfunctions go beyond depression-related cognitive impairments and are compatible with dementia syndrome. Her major memory impairment, reduced semantic word fluency with naming disorders, and limited visuoconstructive skills indicate AD dementia. Atypical for AD dementia is the pronounced psychomotor slowing and visual-spatial perceptual disturbances. To enable additional differential diagnosis of neurodegenerative dementia, she underwent magnetic resonance imaging (MRI). Cranial MRI showed isolated cerebral lesions in the periventricular white matter . Cerebrospinal fluid was collected to ensure a differential diagnosis of inflammatory and neurodegenerative causes of dementia syndrome. Cerebrospinal fluid (CSF) revealed a constellation of biomarkers compatible with AD, e.g., a reduced Aß42 /40 ratio (0.05, reference level: >0.06) and elevated ptau 181 protein (70 pg/ml, reference level: <50 pg/ml). We detected VGlut2 autoantibodies (1:100) in serum samples, observed again in serum samples 1 month later. In addition, after incubating the patient's serum with rat and monkey brain tissues, immunohistochemistry revealed relevant spotty fluorescence of the granular layer in the hippocampus and streaky fluorescence in the thalamus and molecular layer in the cerebellum caused by the antibody against VGlut2. 18 F-fluorodesoxyglucose positron emission tomography ( 18 F-FDG-PET) of the brain showed left parietal and left temporal hypometabolism . In addition, a reduced tracer uptake was also detected in the right cerebellum consistent with diaschisis. Furthermore, the lower 18 F-FDG uptake was detected in the left primary visual cortex . Overall, her PET provided evidence compatible with a DLB pattern or a pattern associated with a logopenic variant of primary progressive aphasia. To summarize her status, we suspect mixed dementia with Alzheimer's pathology and Lewy body pathology. The presence of AD is suggested by the CSF findings and her neuropsychological exam indicating impaired memory, reduced semantic word fluency and naming disorders, and impaired visuoconstructive skills. However, neuropsychological findings include marked psychomotor slowing and visuospatial perceptual disturbances, which are more indicative of the presence of DLB. However, note that biomarkers to diagnose DLB such as (123)-I-2-ß-carbomethoxy-3ß-(4-iodophenyl)- N -(3-fluoropropyl) nortropane single photon emission computed tomography (123I-FP-CIT SPECT) or [ 123 I] metaiodobenzylguanidine (MIBG) cardiac scintigraphy were not explored. However, as she presented no other DLB symptoms other than her cognitive symptoms, i.e., evidence of Parkinson's, a REM sleep behavior disorder, or visual hallucinations, we did not investigate this potential component of Lewy pathology further. Overall, therefore, hers is probably a mixed pathology. As we consistently detected VGlut2 autoantibodies in her serum, that finding is relevant. We, therefore, diagnosed mixed dementia associated with VGlut2 autoantibodies. We ruled out an acute inflammatory CNS event because of her CSF results. To exclude a tumor, she also underwent whole-body FDG PET, which revealed a suspicious nodule near the anterior sternum. An additional dermatologic workup revealed nothing conclusive. Due to mixed dementia with components of Alzheimer's pathology, we started anti-dementia therapy with the acetylcholinesterase inhibitor donepezil at a 10 mg/d dosage. In the absence of evidence of inflammation in the CSF, we have refrained from immunotherapy with methylprednisolone off-label for the time being. We also had this patient undergo antidepressant effective therapy with sertraline 100 mg/day and quetiapine 150 mg/day. Quetiapine was discontinued during the course in favor of mirtazapine 45 mg/day after observing the lack of efficacy under mirtazapine. We find it quite tempting to postulate that her depressive symptoms are an expression of the underlying neurodegenerative mixed dementia associated with VGlut2 autoantibodies. Overall, her diagnosis is probably consistent with the prognosis of mild dementia syndrome due to AD, but it remains unclear whether the additional association with VGlut2 tends to favor the prognosis. After inpatient treatment, the patient experienced two more outpatient follow-up visits. Under antidepressant and anti-dementia therapy, her cognitive symptoms remained stable, and the depressive symptoms largely disappeared. She now presents a euthymic mood with no significant loss of drive and no dangerous behavior. Her medical therapy has been well tolerated, and no relevant side effects have occurred.
| 4.199219
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|
sec[1]/p[0]
|
en
| 0.999997
|
PMC10368954
|
https://doi.org/10.3389/fpsyt.2023.1227824
|
[
"dementia",
"symptoms",
"reduced",
"memory",
"cognitive",
"neuropsychological",
"mixed",
"pathology"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Dementia, unknown or unspecified cause (6D8Z)】
Synonyms: Dementia NOS | Dementia, unspecified | senile melancholia | senile depression | senile paranoid reaction
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Neurocognitive disorders → Dementia → Dementia, unknown or unspecified cause
【2. Dementia due to cerebrovascular disease (6D81)】
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 consid...
Synonyms: arteriosclerotic dementia | Strategic-infarct dementia | Post stroke dementia | vascular cognitive impairment | vascular dementia
Excludes: Alzheimer disease dementia, mixed type, with cerebrovascular disease
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Neurocognitive disorders → Dementia → Dementia due to cerebrovascular disease
【3. Dementia, other specified cause (6D8Y)】
Synonyms: Degenerative dementia | primary degenerative dementia NOS
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Neurocognitive disorders → Dementia → Dementia, other specified cause
【4. Dementia due to use of volatile inhalants (6D84.2)】
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 sy...
Synonyms: inhalant dementia | volatile solvents dementia
Hierarchy: Neurocognitive disorders → Dementia → Dementia due to psychoactive substances including medications (6D84) → Dementia due to use of volatile inhalants
【5. Dementia due to diseases classified elsewhere (6D85)】
Hierarchy: Mental, behavioural or neurodevelopmental disorders (06) → Neurocognitive disorders → Dementia → Dementia due to diseases classified elsewhere
|
6D8Z
|
Dementia, unknown or unspecified cause
|
The proband (IV6) is a 36-year-old woman. The proband had paroxysmal dizziness and memory decline at the age of 20, manifested by frequent forgetfulness, accompanied by rapid temper, but her life and work had not been significantly affected. Brain MRI in the local hospital showed white matter degeneration and cerebellar atrophy. Urine routine indicates microalbuminuria. No headache, limb numbness, fever, nausea, vomiting, speech confusion, and no dyskinesia. There was a history of delayed eruption of deciduous teeth and infertility. She has no history of drinking, taking drugs, and taking phenothiazines, no history of psychosis, encephalitis, brain trauma, and stroke, and no history of adverse reactions to vaccines. The proband had menarche at the age of 17 and had irregular menstruation, requiring long-term oral estradiol tablets/estradioland dydrogesterone tablets. Married at the age of 34, currently infertile. Her parents (III1 and III 2) were consanguineous marriage and had no similar medical history, but her three older brothers (IV1, IV2, and IV4) all had a history of cognitive dysfunction. Physical examination: height 167 cm, weight 60 kg, BMI 21.26 kg/m 2 , good nutritional status, normal blood pressure in both prone and standing positions, no K-F ring in the cornea, dentin dysplasia , no significant abnormalities in heart, lung, and abdominal examination. Breast and pubes development stagnated at Tanner Stage II, vulvar development was immature, and there were no deformities in spine and limb development. Nervous system physical examination: clear consciousness, clear speech, answering questions correctly, cooperative examination; memory slightly decreased, MMSE 27 points, MoCA 15 points (bachelor degree). The sense of smell was normal, and the cranial nerve examination was normal. The muscle strength, muscle tension, and tendon reflex of the limbs were normal. Bilateral superficial and deep sensation existed symmetrically. The movement was flexible, and the gait was normal. The bilateral finger-nose test was accurate, the alternating movement test was coordinated, the heel-knee-tibia test was normal, and Romberg’s sign was negative. Bilateral pathological signs were negative. Auxiliary examination: Thyroid function examination in other hospitals showed that TSH 0.92 (0.27 ~ 4.20) uIU/ml, FT3 5.87 (3.1 ~ 6.8) pmol/L, and FT4 16.9 (12 ~ 22) pmol/L; anti-mullerian hormone 5.24 (0.777 ~ 5.24) ng/ml; repeated monitoring of sex hormone levels showed E2 13.7 ~ 26 (49 ~ 291) pg/ml, FSH 0.05 ~ 0.17 (1.79 ~ 5.12) mIU/ml, LH 0.01 (1.20 ~ 12.86) mIU/ml, PRL 2.06 ~ 3.7 (3.34 ~ 26.72) ng/ml, P4 0.29 ~ 0.55 (5.16 ~ 18.56) ng/ml, and T 0.35 ~ 0.46 (0.1 ~ 0.75) ng/ml. At that time, the gonadotropin-releasing hormone (GnRH) stimulation test showed that LH and FSH had no significant response. Breast color ultrasound showed that the structure of the glandular layer was disordered and the arrangement was not clear. Vaginal ultrasound shows the posterior position of the uterus, with a clear contour and regular shape, approximately 42 mm × 39 mm × 27 mm in size; its muscular layer has uniform echoes and no obvious abnormal mass echoes was observed. After admission, a craniocerebral MRI showed multiple tiny nodular foci in the pituitary gland, considering microadenoma; hypomyelination of the white matter of the brain; brain atrophy; and atrophy and thinning of the corpus callosum . EEG, EMG, and blink reflex tests showed no significant abnormalities (the detailed results of EMG can be found in Supplementary Table S1 ). SEP: both upper limbs were normal, the left lower limb was abnormal, and the right lower limb was normal (see Supplementary Table S2 ). P300 detection: P300 waveform differentiation and repeatability were acceptable, the latency was slightly prolonged and the reaction time was prolonged (Table 2 ). No obvious abnormality was found in biochemistry and serum ceruloplasmin. Figure 2 Craniocerebral magnetic resonance imaging (MRI) results of the proband. ( a – i ) Craniocerebral MRI showed that the pituitary gland was approximately 3.8 mm in height, with a flat upper edge and a slightly sunken saddle base, and multiple tiny nodules with equal and slightly long T1 signals and slightly long T2 signals were seen inside, and the dynamic enhancement showed relatively slightly low signals, the larger one being approximately 2.2 mm in diameter; bilateral periventricular white matter atrophy and degeneration, with short linear and patchy equal and slightly long T1 and slightly long T2 signals, and slightly high T2-Flair signal; cerebral sulcus, cistern, and ventricle were generally widened; corpus callosum atrophy and thinning, particularly at the knee and body of the corpus callosum. Table 2 Clinical characteristics of the patients in the family with hypomyelinating leukodystrophy-7. Subject IV6 IV1 IV2 IV4 Gender Female Male Male Male Age (years) 36 Deceased 39 38 Height (cm) 167 180 184 180 POLR3A p.Cys767Phe Hom Unknown Hom Hom Age of onset of neurological symptoms (years) 20 16 20 23 Cognitive dysfunction Mild Moderate to severe Moderate to severe Severe Puberty development Delayed, stagnant Delayed Delayed Delayed Hypogonadotropic hypogonadism Yes Unknown No No Abnormal tooth development Delayed eruption of deciduous teeth and dentin dysplasia Delayed eruption of permanent teeth and lack of the right maxillary median incisor Delayed eruption of permanent teeth Delayed eruption of deciduous teeth and lack of the left maxillary canine Hair and nails Pubes development stagnated at Tanner Stage II with normal nails Unknown Normal Normal hair development, but nails partially missing Ataxia No Yes Yes Yes Dysarthria No Yes No Yes Dystonia No Yes No Yes Muscle atrophy of extremities No Unknown Mild Moderate to severe Extremity muscle strength Normal Unknown Muscle strength of both lower limbs is grade 4 Extremity muscle strength grade 3 Tendon reflexes of extremities Normal Unknown Bilateral upper limbs ( +), bilateral lower limbs (+ + +) Bilateral upper limbs ( +), bilateral lower limbs (+ + +) Bilateral superficial and deep sensation Normal Unknown ND ND Bilateral pathological signs Negative Unknown Positive ( +) Positive ( +) EMG Normal Unknown ND ND Latency of blink reflex R1/R2/R2’ (ms) Left stimulus: 11.3/34.2/34.5; Right stimulus: 12.2/34.5/35.0 Unknown ND ND SEP Bilateral upper limbs normal; left lower limb abnormal, right lower limb normal Unknown ND ND P300 latency Fz/Cz/Pz (ms) 398/416/436 Unknown 454/453/401 No definite waveform P300 amplitude Fz/Cz/Pz (mV) 487/536/533 Unknown 243/243/260 No definite waveform Wheel-chair use No Yes No Yes Age at death (years) N/A 38 N/A N/A The craniocerebral magnetic resonance imaging of patients in this family with hypomyelinating leukodystrophy-7 (HLD-7) all showed symmetrical white matter abnormalities around the ventricles, corpus callosum thinning, and cerebellar atrophy. Patients with HLD-7 all showed psychiatric symptoms such as euphoria, anxiety disorders, and emotional instability at an early stage of the disease. This family has not presented with symptoms of optic atrophy, epilepsy, or postural tremor. The detailed results of IV6’s EMG and SEP can be found as Supplementary Tables S1 and S2 online. Hom, homozygote; EMG, electromyography; SEP, somatosensory evoked potential; ND, not done; N/A, not applicable.
| 3.921875
| 0.959961
|
sec[2]/sec[0]/p[0]
|
en
| 0.999997
|
38561452
|
https://doi.org/10.1038/s41598-024-58452-6
|
[
"unknown",
"delayed",
"slightly",
"atrophy",
"limbs",
"development",
"limb",
"muscle"
] |
[
{
"code": "MG48",
"title": "Unknown or unspecified causes of morbidity"
},
{
"code": "1H0Z",
"title": "Infection, unspecified"
},
{
"code": "EC90.6",
"title": "Pruritus of unknown cause"
},
{
"code": "MH14",
"title": "Other ill-defined or unspecified causes of mortality"
},
{
"code": "2F9C",
"title": "Neoplasms of unknown behaviour of connective or other soft tissue"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Unknown or unspecified causes of morbidity (MG48)】
Synonyms: undetermined cause | unknown cause of disease | illness | Illness NOS | sick
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → General symptoms, signs or clinical findings → General symptoms → Unknown or unspecified causes of morbidity
【2. Infection, unspecified (1H0Z)】
Synonyms: infection NOS | infectious disease NOS | infection unknown | infection process NOS | infection by unspecified organism and of unspecified site
Hierarchy: Certain infectious or parasitic diseases (01) → Infection, unspecified
【3. Pruritus of unknown cause (EC90.6)】
Definition: Pruritus without identifiable cause despite thorough investigation.
Synonyms: Pruritus sine materia | Itch of unknown cause
Hierarchy: Sensory or psychological disorders affecting the skin → Disturbances of cutaneous sensation → Pruritus (EC90) → Pruritus of unknown cause
【4. Other ill-defined or unspecified causes of mortality (MH14)】
Synonyms: death NOS | cause of mortality not stated | death of unknown cause | unknown cause of mortality
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Ill-defined and unknown causes of mortality → Other ill-defined or unspecified causes of mortality
【5. Neoplasms of unknown behaviour of connective or other soft tissue (2F9C)】
Synonyms: connective or other soft tissue tumour NOS | Abdominal desmoid of unknown behaviour of unspecified site | Mixed mesenchymal tumour of unspecified site | Muscular neoplasm of unknown behaviour | Myofibromatosis of unknown behaviour of unspecified site
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of connective or other soft tissue
|
MG48
|
Unknown or unspecified causes of morbidity
|
Patient 1 . The pregnancy was complicated by maternal gestational diabetes, polyhydramnios, fetal overgrowth and caesarean section (37 weeks). Birth weight was above the 97th centile, length was on the 75th centile, and head circumference was greater than the 98th centile (Table 1 ). Limb shortening, varus posture of wrist joints, adducted thumbs, elbow contractures, rocker bottom feet, extended knees, hip dysplasia and macrocephaly were noted . At 5 weeks old the boy showed recurrent severe obstructive apneic episodes with cyanosis, loss of responsiveness, seizures and choking episodes during feeding. Pharyngeal and laryngeal obstruction required supraglottoplasty and recurrent apneic episodes and aspiration required permanent tracheostomy. Dysfunctional swallowing and feeding difficulties were noted. Weight gains improved with small frequent percutaneous endoscopic gastrostomy feeds. Details on tonic clonic seizures and treatment are given in the Supporting Results . Abdominal ultrasound detected no malignancy. The patient was following visually, smiling and breathing normally. Tracheostomy was removed at 9 months old. He had generalized developmental delay, was unable to roll or sit with marked head lag. Deep palmar and plantar creases were noted. At 2 2/3 years, weight and length were below the 3rd centile and head circumference was greater than the 95th centile (Table 1 ). Cardiac arrhythmia was documented, seizures persisted. Trio exome sequencing identified two likely pathogenic de novo variants, c.466 T > C p.(Phe156Leu) in HRAS and c.4907 G > C p. in SCN1A . The latter most probably underlies the seizures in this patient . Table 1 Comparison of the clinical presentation of patients with different HRAS variants. HRAS variant p.Phe156Leu p.Ala146Pro/Val/Thr p.Thr58Ile p.Gly12Ser Patient/number of probands References Patient 1 This report Patient 2 This report N = 3 Gripp , Chiu , Zampino , Gripp N = 4 Gripp , Gripp , Gripp , Gripp , Hippala N = 30 Gripp , Gripp Prenatal findings GDM, polyhydramnios, large fetal size, Ces. S. 37 wks. gestation Polyhydramnios, NVD Term Uneventful, N/R Borderline polyhydramnios (1/2) Polyhydramnios (almost universal), large fetal size (frequent) [ age ] Weight Length OFC [Birth] 4.51 kg (>97 th ctl) 50 cm (75 th ctl) 41 cm (>98 th ctl) [2 yrs 8 mths] 10 kg (<3 rd ctl) 79.1 cm (<3 rd ctl) 52 cm (>95 th ctl) [Birth] 3.5 kg (50 th ctl) N/D 36 cm (50 th ctl) [6 yrs 8 mths] 22.5 kg (50 th ctl) 113 cm (10 th ctl) 55.3 cm (>97 th ctl) [Birth] Normal (3/3) Normal (1) Normal (2/2) [6 mths–6 yrs] < 3rd ctl (2/2) Normal (2/2) <3rd ctl (2/3) Height < −2 SD below mean of age (0/4) Height < −2 SD below mean of age (26/30) Craniofacial features Macrocephaly, short nasal tip, long philtrum, subtle dysmorphic features Prominent forehead, long philtrum, full cheeks, macroglossia, subtle dysmorphic features Microcephaly (1/3), flat occiput (1/3), low set ears (1), short nasal tip (2/2), long philtrum (2/2), mildly prominent earlobes (1), subtle dysmorphic features (2/2) Macrocephaly (3/4), full lips (1/3), prominent ear lobes (2/4), subtle/mild dysmorphic features (3/4) Macrocephaly (9/30), coarse facial features (universal), full lips (almost universal), large mouth (almost universal) Musculoskeletal manifestations Limb shortening, varus posture of wrist joints, adducted thumbs, elbow contractures, congenital vertical talus, extended knees, hip dysplasia Tight Achilles tendons, gait abnormalities Osteopenia (1), osteoporosis (1), hypotonia (1), coxa valga (1), minor involvement of joints (1) Ligamentous laxity (2/3), hypotonia (1/2), mild pectus excavatum (1), narrow shoulders (1), limited elbow extension (1), cubitus valgus (1), limited supination (1), joint pain (1) Ulnar deviation (24/30), hypotonia (22/30), tight achilles tendons (almost universal) Cardiovascular abnormalities Cardiac arrhythmias (ectopic beats) HCM, VH, mild PS HCM (2/2), tachycardia (1), VSD (1), PDA (1), MR (1), mild AR (1) HCM (2/4), DCRV (1), MVP (1), moderate LVH (1) HCM (13/30), PS (15–20%), ASD (5–7%), atrial arrhythmias (>50%) Dermatologic findings Mildly deep palmar and plantar creases Thickened skin on elbows, excessive sweating, sensitive skin Frontal bossing (1), sparse, short hair (1), long eyelashes (1), deep set eyes (1), dry skin (1), mildly deep palmar creases (1), thin, sparse, not curly hair (1), minor involvement of skin (1) (Moderately) deep palmar/plantar creases (2/3), prominent digital pads (1), redundant soft tissue (2), fine hair (2/3), long eyelashes (1) Deep palmar or plantar creases (universal), curly or sparse, fine hair (almost universal) Neurologic and behavior issues LOR, tonic clonic seizures Mild ventriculomegaly, (no seizures) Arachnoid cyst (1), mild CTH (1), anxiety (1) Possible ADHD (1), tremors (1), behavior problems (1), very active (1), poor fine motor skills (1), anxiety (1), tonic-clonic seizures (1) Sleep disturbance (frequent), anxiety (sometimes), behavior problems (50%) Neurocognitive findings DD, little active movement, no communication Moderate DD (Moderate) DD (2/2), moderate ID (1) DD (1/3) DD (universal) Gastroenterologic, endocrinologic and metabolic findings dysfunctional swallowing, FTT, FTP, choking episodes FTT, FTP, severe GORD, severe hypoglycemia, hyperinsulinism, cow’s milk intolerance (Severe) FTT (3/3), FTP (2/3), metabolic acidosis (1), GORD (1), GH deficiency (1), swallowing difficulty (1) FTT (3/4), FTP (2/3), pyloric stenosis (1/3) FTT (universal), FTP (almost universal), GORD (frequent), hypoglycemia (at risk) Hematologic and oncologic findings No No No (2/2) Papillomata (0/4), malignant tumor (0/4) Papillomata (14/30), malignant tumor (2/30) Respiratory and otolaryngologic findings Obstructive apnea, aspiration, pharyngeal and laryngeal obstruction Laryngomalacia N/R N/R sleep apnea (at risk) Other findings No Esotropia to right eye, (no hernia) Inguinal hernia (1), Strabismus (1) Strabismus (3/4), Nystagmus (1/4), severe myopia (1/4) Strabismus (>50%), Nystagmus (13/30) GDM maternal gestational diabetes, Ces. S. Cesarean section, NVD Term natural vaginal delivery, OFC occipitofrontal circumference, ctl centile, HCM hypertrophic cardiomyopathy, (L)VH (left) ventricular hypertrophy, PS pulmonic stenosis, VSD ventricular septal defect, PDA patent ductus arteriosus, MR mitral regurgitation, AR aortic regurgitation, DCRV double-chambered right ventricle, MVP mitral valve prolapse, ASD atrial septal defect, LOR loss of responsiveness, CTH cerebellar tonsillar herniation, ADHD attention deficit hyperactivity disorder, DD developmental delay, ID intellectual disability, FTT failure-to-thrive, FTP feeding tube placement, GORD gastroesophageal reflux disorder, GH growth hormone, wks weeks, yrs years, N/D not documented, N/R not reported. Summaries are given for the variants p.Ala146Pro/Val/Thr, p.Thr58Ile and p.Gly12Ser: findings present/patients with information on the finding; ( n ), feature was reported in n individuals. Other findings include dental, oral, ophthalmologic and genitourinary manifestations. Fig. 1 Photographs of patients 1 and 2. A Patient 1. Note macrocephaly, the adducted thumbs and wrist varus posture and congenital vertical talus. B . Patient 2. Note the prominent forehead, long philtrum and full cheeks. C Schematic representation of HRAS and position of the p.Phe156Leu variant.
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sec[2]/sec[0]/p[0]
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en
| 0.999995
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35764878
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https://doi.org/10.1038/s41431-022-01139-1
|
[
"universal",
"findings",
"gripp",
"seizures",
"centile",
"deep",
"almost",
"features"
] |
[
{
"code": "LB18",
"title": "Congenital anomalies of intestinal fixation"
},
{
"code": "EC23.Y",
"title": "Other specified genetic disorders of skin pigmentation"
},
{
"code": "ED63.0",
"title": "Vitiligo"
},
{
"code": "ED70.2Y",
"title": "Other specified forms of alopecia areata"
},
{
"code": "MD40.Y",
"title": "Other specified clinical findings in specimens from respiratory organs and thorax"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Congenital anomalies of intestinal fixation (LB18)】
Definition: A condition caused by failure of the intestines to correctly develop during the antenatal period. This condition may present with intermittent abdominal pain, vomiting, or diarrhoea. Confirmation is through observation of intestinal rotation by imaging.
Synonyms: Congenital malrotation of large intestine | Congenital intestinal malrotation | Volvulus of midgut | Volvulus neonatorum | Congenital malrotation of caecum
Hierarchy: Developmental anomalies (20) → Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the digestive tract → Congenital anomalies of intestinal fixation
【2. Other specified genetic disorders of skin pigmentation (EC23.Y)】
Synonyms: Genetically-determined mixed hyper- and hypomelanotic disorders of skin pigmentation | Hereditary universal dyschromatosis | Dyschromatosis universalis hereditaria | DUH1 - [Dyschromatosis universalis hereditaria] (MIM 127500) | Hereditary symmetrical dyschromatosis
Hierarchy: Diseases of the skin (14) → Genetic or developmental disorders affecting the skin → Genetic disorders of skin pigmentation (EC23) → Other specified genetic disorders of skin pigmentation
【3. Vitiligo (ED63.0)】
Definition: Vitiligo is an acquired pigmentary disorder of the skin and mucous membranes where progressive destruction of melanocytes results in loss of skin pigmentation. Half of all cases first appear before the age of 20. The clinical course is unpredictable but gradual extension of the areas involved is the norm. The disease may have a devastating psychological impact, particularly in people with dark ski...
Synonyms: Localised vitiligo | Generalised vitiligo | Universal vitiligo | Segmental vitiligo | Mucosal vitiligo
Hierarchy: Disorders of the epidermis and epidermal appendages → Disorders of skin colour → Acquired hypomelanotic disorders (ED63) → Vitiligo
【4. Other specified forms of alopecia areata (ED70.2Y)】
Synonyms: Alopecia universalis | Universal alopecia areata | Ophiasis | Ophiasic alopecia areata | Alopecia areata of eyelashes
Hierarchy: Disorders of hair → Alopecia or hair loss (ED70) → Alopecia areata (ED70.2) → Other specified forms of alopecia areata
【5. Other specified clinical findings in specimens from respiratory organs and thorax (MD40.Y)】
Synonyms: Abnormal findings in bronchial washings | Abnormal findings in nasal secretions | Abnormal findings in pleural fluid | abnormal pleural fluid | abnormality in pleural fluid
Hierarchy: Symptoms, signs or clinical findings of the respiratory system → Clinical findings in the respiratory system → Clinical findings in specimens from respiratory organs and thorax (MD40) → Other specified clinical findings in specimens from respiratory organs and thorax
|
LB18
|
Congenital anomalies of intestinal fixation
|
A 54-year-old man with type C cirrhosis was admitted to another hospital complaining of hematemesis due to rupture of the esophageal varices and underwent hemostasis with endoscopic variceal ligation (EVL). Abdominal ultrasonography revealed ascites, and color Doppler ultrasonography showed IAPF between the branch of the left hepatic artery and umbilical part of the left branch of the portal vein. The right portal venous flow was hepatopetal, and the left portal venous flow was hepatofugal . Contrast-enhanced computed tomography (CT) demonstrated IAPF in the left lobe, and the umbilical part of the left branch of the portal vein was enhanced simultaneously in the arterial phase . Digital subtraction angiography (DSA) revealed diffuse IAPF and an early filling of the left branch of the portal vein . The cause of portal hypertension was IAPF supplied by A2, A3, and A4, and transcatheter arterial embolization (TAE) using microcoils was performed to close the fistula. A2, A3, and A4 were embolized; however, the fistula was not completely occluded . Thereafter, there were a total of four hematemeses due to esophageal variceal rupture, and a total of six EVLs were performed. The second TAE also failed to reach complete occlusion because of diffuse collateralization. As hematemesis was repeated after treatment, the patient was transferred to our hospital for further treatment. Laboratory results were as follows: white blood cell count of 4500/μL ; red blood cell count of 328 × 10 4 /μL (normal, 427–570 × 10 4 /μL); serum hemoglobin concentration of 10.2 g/dL (normal, 14–18 g/dL); serum platelet count of 12.8 × 10 4 /μL (normal, 15–35 × 10 4 /μL); aspartate transaminase concentration of 69 IU/L (normal, 8–38 IU/L); alanine transaminase concentration of 45 IU/L (normal, 4–44 IU/L); serum albumin concentration of 4.1 g/dL (normal, 3.9–4.9 g/dL); total bilirubin concentration of 0.6 mg/dL (normal, 0.2–1.2 mg/dL); prothrombin time of 67.0%; and ICGR15 level of 12.4%. The clinical Child-Pugh classification status was B. As with previous hospital examinations, abdominal CT demonstrated ascites and remaining IAPF in the left lobe of the liver. Although left hepatectomy including IAPF was thought to be needed, we concluded that major hepatectomy at this point had a high risk because of poor general condition due to frequent hematemesis and deterioration of liver function. Although an apparent decline in liver function due to frequent massive bleeding was possible, the general condition was extremely poor and was not suitable for left hepatectomy. Therefore, we performed ligation of the draining left portal vein and dissection of the left gastric vein that supplied varicose veins . A catheter was inserted from the paraumbilical vein to measure the portal venous pressure. Portal venous pressure decreased from 330 to 210 mmH 2 O after ligation of the left portal vein. The operating time was 251 min, and the intraoperative bleeding was 340 mL. However, melena appeared on the 5th postoperative day, and the progression of anemia was observed. An emergency upper gastrointestinal endoscopy was performed on suspecting bleeding from the esophageal varices. Although there was no active bleeding, EVL was performed for the esophageal varices with red color signs. The laboratory results on the 14th postoperative day were as follows: aspartate transaminase concentration, 48 IU/L; alanine transaminase concentration, 34 IU/L; serum albumin concentration, 3.6 g/dL; total bilirubin concentration, 0.5 mg/dL; prothrombin time, 67.9%; and ICGR15 level, 13.8%. Ascites disappeared at the CT findings in the postoperative course, and the clinical Child-Pugh classification status improved from grade B to grade A. After the first surgery, the general condition and liver function were improved on the 14th postoperative day. Therefore, left hepatectomy was performed to remove the IAPF completely on the 21st postoperative day. Adhesion was observed around the hepatic hilum because of the first operation. Furthermore, the division of the hepatic hilum was hemorrhagic owing to portal hypertension. As the left portal vein was ligated at the time of the first operation, the demarcation line was found on the liver surface by dissection of the left hepatic artery. After mobilization of the left liver, parenchymal dissection was performed under intermittent inflow occlusion, that is, 15 min of occlusion followed by 5 min perfusion. The operating time was 318 min, and the intraoperative bleeding amount was 1800 mL. In the macroscopic findings of the resected specimen, arterioportal fistula could not be identified . In the microscopic findings, the background liver tissue showed the presence of many pseudo-nodules, indicating liver cirrhosis. Many dilatated vessels in Glisson’s sheath and arterioportal fistula were observed . Contrast-enhanced CT after left hepatectomy revealed that earlier enhancement of the branch of the portal vein disappeared in the hepatic arterial phase . Although anorexia and wound infection were noted, there were no other major complications, and he was discharged on the 32nd postoperative day. There was no recurrence of portal hypertension for 1 year and 3 months after hepatectomy. Fig. 1 Color Doppler ultrasonography findings. a Hepatopetal flow was shown in the right branch of the portal vein. b Hepatofugal flow was shown in the left branch of the portal vein, and disturbed flow was shown around the left branch of the vein Fig. 2 Contrast-enhanced computed tomography of the abdomen revealed ascites. Earlier enhancement of the umbilical part of the left branch of the portal vein in the hepatic arterial phase (arrow). a Plain. b Arterial phase. c Delayed phase Fig. 3 Digital subtraction angiography findings. a Superior mesenteric arteriography reveals intrahepatic arterioportal fistula (arrow) and early filling of the left branch of the portal vein. b Proper hepatic angiography after coil embolization of the A2, A3, and A4 (arrowhead) also shows incomplete occlusion of the fistula Fig. 4 Intraoperative findings of the primary surgery. a A catheter was inserted from the paraumbilical vein, and the portal venous pressure was measured. b Identification of the left hepatic artery and left branch of the portal vein. c Identification of the left gastric vein (arrow), which supplies the varicose veins Fig. 5 Intraoperative findings of the second surgery. a Identification of the left hepatic artery (arrow). Adhesion was observed because of the first surgery. b The left branch of the portal vein (arrowhead), firmly adhered. The left branch of the portal vein and left branch of the bile duct are taped together. c The main trunk of the middle hepatic vein is exposed in the transection plane Fig. 6 Macroscopic and microscopic findings. a Arterioportal fistula could not be identified macroscopically. b Many dilatated vessels can be observed in Glisson’s sheath. Arterioportal fistula (arrow) can also be observed (hematoxylin-eosin stain). c The portal vein and artery are distinguished with special staining. The same specimen shows an arterioportal fistula (Elastica van Gieson staining) Fig. 7 Contrast-enhanced computed tomography image of the abdomen after left hepatectomy. The earlier enhancement of the portal vein disappeared in the hepatic arterial phase. a Arterial phase. b Delayed phase
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| 0.970703
|
sec[1]/p[0]
|
en
| 0.999995
|
31016545
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https://doi.org/10.1186/s40792-019-0623-8
|
[
"portal",
"vein",
"branch",
"hepatic",
"fistula",
"concentration",
"phase",
"liver"
] |
[
{
"code": "DB98.3",
"title": "Portal vein thrombosis"
},
{
"code": "DB98.7Z",
"title": "Portal hypertension, unspecified"
},
{
"code": "LA90.21",
"title": "Congenital portosystemic shunt"
},
{
"code": "DB98.7Y",
"title": "Other specified portal hypertension"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Portal vein thrombosis (DB98.3)】
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.
Synonyms: Phlebitis of portal vein | deep vein thrombosis of portal vein | portal thrombosis | PVT - [portal vein thrombosis] | portal venous thrombosis
Hierarchy: Diseases of the digestive system (13) → Diseases of liver → Vascular disorders of the liver (DB98) → Portal vein thrombosis
【2. Portal hypertension, unspecified (DB98.7Z)】
Synonyms: Portal hypertension | PHT - [portal hypertension] | Portal HTN
Hierarchy: Diseases of liver → Vascular disorders of the liver (DB98) → Portal hypertension (DB98.7) → Portal hypertension, unspecified
【3. Congenital portosystemic shunt (LA90.21)】
Synonyms: anomalous pulmonary venous drainage to hepatic veins | anomaly of portal vein connection | portal vein deformity | portal vein anomaly | anomalous portal venous connection
Hierarchy: Structural developmental anomalies of the circulatory system → Structural developmental anomalies of the peripheral vascular system (LA90) → Peripheral venous malformations (LA90.2) → Congenital portosystemic shunt
【4. Other specified portal hypertension (DB98.7Y)】
Synonyms: Banti syndrome | Banti spleen | fibrocongestive splenomegaly | congestive splenomegaly | hepatolienal fibrosis
Hierarchy: Diseases of liver → Vascular disorders of the liver (DB98) → Portal hypertension (DB98.7) → Other specified portal hypertension
|
DB98.3
|
Portal vein thrombosis
|
In 1990, a 40-year-old male underwent anterior resection of rectal adenocarcinoma (Dukes B2; preoperative carcinoembryonic antigen (CEA) serum level: 35 ng/mL), that was diagnosed synchronously with a 9-cm metastasis in liver segments V-VIII. After postoperative 5-fluorouracil based chemotherapy, the CEA serum level was slightly decreased (22 ng/mL); liver metastasis was markedly downsized and was radically resected. Follow-up revealed no evidence of residual disease until 2002; follow-up was then intentionally interrupted because the patient was considered to be free of disease. On March 2007, the patient developed dyspnea and cough, and a chest computed tomography (CT) scan revealed a 5-cm right hilar mass bulging into the main bronchus and infiltrating the upper lobe . Bronchoscopy demonstrated a neoplastic growth that was obstructing the right main bronchus 2 cm distal to the carina; biopsy revealed moderately differentiated adenocarcinoma, compatible with rectal cancer recurrence. Colonoscopy and CT of the abdomen and pelvis showed no other evidence of neoplastic disease, and the CEA serum level was normal (1 ng/mL). On April 2007, the patient was aged 57 years and was fit for major thoracic surgery. He was treated by means of right pneumonectomy and regional lymphadenectomy to obtain complete resection . Histology of the excised lung metastasis revealed an adenocarcinomatous proliferation with morphological features overlapping those of primary rectal cancer and of previously resected hepatic metastasis. The primary cancer and all of the metastatic sites were characterized by extensive complex tubular gland formation with sparse centroglandular necrotic foci. Nuclei were moderately or highly atypical, elongated and stratified. The mitotic index ranged from 17 (in rectal cancer) to 24 × 10 high power fields (in metastatic sites). A mild peritumoral lymphocytic infiltrate (cluster of differentiation 3 positive) was present. Budding was absent and there was no lymphovascular invasion. The immunophenotype included caudal-type homeobox protein 2 and cytokeratin (CK) 20 (CK 20) positivity , whereas thyroid transcription factor-1 and CK 7 were negative. The main bronchus resection margin was tumor-free. Of the 18 lymph nodes excised, 4 (all peribronchial) were metastatic. The early postoperative course was complicated by empyema that was successfully treated with antibiotics and drainage. Follow-up with CT scans of the chest and abdomen at 6 months after pneumonectomy revealed enlarged left supraclavicular lymph nodes . Biopsy of the latter revealed a metastatic adenocarcinomatous growth with a high mitotic index (14 × 10 high-power fields) and a morphological and immunohistochemical profile identical to that previously described . There were no other signs of relapse. Systemic chemotherapy (leucovorin, 5-fluorouracil and oxaliplatin regimen) was then initiated and continued until July 2008, resulting in complete clinical remission. Subsequent follow-up with semi-annual assessments of CEA level and total body CT and/or a positron emission tomography scans revealed no evidence of recurrence. At the last examination, in February 2015, the patient was relapse-free and enjoyed a good quality of life, at 7 years after pneumonectomy for lung metastasis and at 24 years after rectal cancer resection. Genetic and epigenetic profiles of the primary tumor and the three matched metastases (pulmonary, hepatic and lymph nodal) were assessed to verify the clonal origin of the four tumor samples and to characterize key biological traits during the tumor evolution. Tables 1 and 2 summarize the molecular results. Methylation specific-multiplex ligation dependent probe amplification (MS-MLPA) was performed by using the ME001 MS-MLPA tumor suppressor-1 kit and the ME002 MS-MLPA tumor suppressor-2 Kit (MRC-Holland, Amsterdam, The Netherlands) to simultaneously analyze the methylation status of 33 tumor suppressor genes and copy number alterations (CNA) of 53 genes . The primary rectal carcinoma showed a high frequency of CNAs at multiple chromosome regions suggesting an unstable karyotype in this tumor. Clonal CNAs including chromosome gains at 7q, 9pq, 11pq, 13q, 19p and 20q were recurrent in the primary tumor and in the matched metastases, demonstrating a common origin of the four tumor samples (Table 1 ). Similarly, clonal gene specific hypermethylation was observed at adenomatous polyposis coli (APC) and cadherin 13 (CDH13) loci, while additional hypermethylated genes, namely WT1, DAPK1, CHFR, PAX5 and GATA5 , were found in the lung and in supraclavicular lymph node metastases (Table 2 ). Microsatellite instability (MSI) analysis, carried out using a pentaplex panel of monomorphic mononucleotide repeats (BAT25, BAT26, NR-21, NR-22 and NR-24) as previously reported , demonstrated the absence of MSI in all tumor samples. The Maldi-TOF mass spectrometry platform and the myriapod colon status kit (Diatech Pharmacogenetics, Jesi, Italy) were used to profile the patient’s four tumor samples, analyzing the 216 common hot-spot cancer mutations in the four major oncogenes involved in CRC pathogenesis ( KRAS, BRAF, PIK3CA and NRAS ). This analysis demonstrated no gene mutation in the primary rectal adenocarcinoma, and interestingly this genetic profile remained relatively well preserved in the three metastatic sites. Fig. 1 Chest Computed Tomography (CT). Chest CT scan coronal view showing 5-cm right hilar mass bulging into the main bronchus Fig. 2 Chest CT details, histological and immunohystochemical studies. a Chest CT-scan axial view (lung window) showing a right hilar mass and post-obstructive collapse in the upper lobe anterior segment. b Close view of right pneumonectomy bronchial section margin (arrows); the main bronchus is obstructed by a polypoid tumor. c Lung metastatic adenocarcinomatous proliferation (hematoxylin & eosin staining, original magnification × 400); inset shows CDX-2 immunohistochemical staining (left), and CK20 positivity (right). d Chest CT-scan six months after right pneumonectomy, demonstrating enlarged left supraclavicular lymph nodes (arrow). e Lymph node metastasis with morphological features similar to Fig. 2c (hematoxylin & eosin staining, original magnification × 400); inset shows CDX-2 positivity Table 1 Copy number alterations (CNAs) observed in the primary tumor and in the matched metastases Chromosomal position Primary rectal carcinoma Hepatic metastasis Lung metastasis Lymph nodal metastasis 1p + + - + 2p + + - + 3p + + - - 7q + + + + 9p + + + + 11p + + + + 11q + + + + 12p + + - - 12q + + - - 13q + + + + 19p + + + + 20q + + + + +: presence of CNA; -: absence of CNA Table 2 Genetic and epigenetic alterations in the primary tumor and matched metastases Tumor sample Gene hypermethylation a MSI b status BRAF KRAS NRAS PIK3CA Primary rectal cancer APC; CDH13; WT1 MSS - - - - Hepatic metastasis APC; CDH13 MSS - - - - Lung metastasis APC; CDH13; WT1; DAPK1; CHFR; GATA5 MSS - - - - Lymph nodal metastasis APC; CDH13; PAX5; DAPK1; CHFR; GATA5 MSS - - - - a List of hypermethylated genes; clonal gene hypermethylations are shown in bold font. APC adenomatous polyposis coli, CDH13 cadherin 13 b MSI microsatellite instability. MSS: microsatellite stable. -: absence of BRAF, KRAS, NRAS, PIK3CA mutations assayed using the myriapod colon status panel
| 4.132813
| 0.971191
|
sec[1]/p[0]
|
en
| 0.999996
|
26231173
|
https://doi.org/10.1186/s12885-015-1585-2
|
[
"tumor",
"metastasis",
"primary",
"rectal",
"lymph",
"chest",
"cancer",
"lung"
] |
[
{
"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 CLASSIFICATION ═══
【1. Neoplasms of unknown behaviour of unspecified site (2F9Z)】
Synonyms: neoplasia | neoplasm growth NOS | tumour of unspecified site | tumourlet of unspecified site | tumour mass NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of unspecified site
【2. Subcutaneous swelling, mass or lump of uncertain or unspecified nature (ME61)】
Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature
Synonyms: localised swelling, mass or lump of skin and subcutaneous tissue | localised subcutaneous nodules | subcutaneous nodules | superficial subcutaneous nodules | localised swelling
Excludes: localized adiposity | mass and lump: breast | enlarged lymph nodes | mass and lump: intra-abdominal or pelvic | oedema
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings involving the skin → Symptoms or signs involving the skin → Subcutaneous swelling, mass or lump of uncertain or unspecified nature
【3. Carcinoma in situ of unspecified site (2E6Z)】
Synonyms: carcinoma in situ of unspecified site | carcinoma in situ NOS | carcinoma in situ | in situ neoplasm
Hierarchy: Neoplasms (02) → In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues → Carcinoma in situ of unspecified site
【4. Neoplasms of unknown behaviour of trachea, bronchus or lung (2F91.1)】
Synonyms: 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 | Lung hemangiopericytoma of unknown behaviour
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs (2F91) → Neoplasms of unknown behaviour of trachea, bronchus or lung
【5. Neoplasms of unknown behaviour of skin (2F92)】
Synonyms: skin tumour NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of skin
|
2F9Z
|
Neoplasms of unknown behaviour of unspecified site
|
OSFT is an extremely rare intraorbital tumor. This tumor mostly originates from mesenchymal cells in the orbit and often involves the bone in the orbit. Even more, it breaks through the bony fissure of the cranial orbit and invades the intracranial structure. Most of these tumors require surgical treatment, and radiotherapy and chemotherapy are not sensitive. Clinically, the postoperative recurrence of tumors is very common. Most of these tumors are first diagnosed in ophthalmology, and most of the first operations are performed by ophthalmologists. However, some OSFTs are not suitable for ophthalmic surgery based on their location and scope of involvement. For example, the tumor is located in the retrobulbar muscle cone . It is difficult to ensure the protection of the optic nerve through the ophthalmic surgical approach. Neurosurgery can better expose the tumor, give the incision of the periosteum of orbit, explore the medial side of the superior rectus muscle, and then completely remove the tumor. Sometimes OSFT grows into the skull along the supraorbital fissure and involves the dura mater. The lateral orbital approach is difficult to completely remove the tumor and at the same time, expand the bone and dura mater involved by the tumor. The remaining tumor will cause the tumor to recur. The craniotomy can fully expose the supraorbital tumor. After the transfronto-orbital approach was given to the craniotomy, the MRI re-examinations immediately after the operation showed that the tumor and involved dura were removed completely . When the tumor further involves the intracranial structure, it will invade the cavernous sinus and even the intracranial brain tissue . In this situation, craniotomy can still completely remove the tumor. Generally, it is difficult for the tumor to break through the bilateral dural structure of the cavernous sinus. Adequate exposure, remove the tumor in pieces, and protect the internal carotid artery, oculomotor nerve, trochlear nerve, abductor nerve, and trigeminal nerve (V1, V2). The enlarged surgical field can not only prevent the traction of the orbital contents but also ensure that all the tumor-involved areas are removed. In our clinical cases, we found that most of the OSFT involved periorbital bone to varying degrees, and all craniotomy operations involved extensive removal of the involved bone . The tumors located on the lateral or superior side of the orbit are often accompanied by bone destruction, absorption, and hyperplasia. Wrong surgical procedures and missed imaging studies often accompany tumor recurrence. The forcible removal of the skull base through the transorbital approach may result in cerebrospinal fluid leakage contusion and laceration of the brain. It is difficult to adequately deal with the involved periorbital or skull base bone with the ophthalmic surgical approach, which left a hidden danger to the recurrence of the tumor. In the case of tumors involving the periorbital and skull base bones, we have adopted craniotomy. The tumor is completely resected through the transfronto-orbital approach. It is worth noting that during the operation, the affected bone must be extensively removed, and the involved dura mater and brain tissue must be removed completely. In this study, most of the patients we treated were patients who relapsed after ophthalmic surgery or patients who were difficult to remove completely with ophthalmic surgery alone. For this type of patient, we believe that the can give a total resection of the tumor and reduce the recurrence of the tumor. Fig. 1 Case 1. The patient was a 23-year-old with a chronic disease process and left exophthalmos for more than 10 months. a , b : CT and MRI showed that the tumor was located in the muscle cone behind the eyeball and compressed the optic nerve. c , d : After the Transfronto-orbital approach was given to the craniotomy, the MRI re-examinations immediately after the operation and 1 year after the operation showed that the tumor was removed completely without recurrence, and the optic nerve was protected. There was no recurrence after 5 years of MRI follow-up Fig. 2 Case 2. The patient was a 45-year-old with a chronic disease process, left exophthalmos, and vision loss for more than 2 years. a : MRI showed that the main body of the tumor was located lateral orbit and invaded the intracranial dura through the superior orbital fissure (arrow). b : After the Transfronto-orbital approach was given to the craniotomy, the MRI re-examinations immediately after the operation showed that the tumor and involved dura were removed completely (arrow). There was no recurrence after 8 years of MRI follow-up Fig. 3 Case 3. The patient was a 78-year-old with a chronic disease process, left exophthalmos, and vision loss for more than 19 years. a : MRI showed that the main body of the tumor was located lateral orbit and invaded the intracranial dura, and cavernous sinus through the superior orbital fissure (arrow). b : The tumor broke through the dura mater of the skull base and invaded the intracranial structure (arrow). c , d : After the Transfronto-orbital approach was given to the craniotomy, the MRI re-examinations immediately after the operation showed that the tumors involving the cavernous sinus and intracranial were removed completely. There was no recurrence after 3 years of MRI follow-up Fig. 4 Case 4. The patient was a 25-year-old with chronic disease process and right exophthalmos for more than 6 months. a, b : CT showed that the tumor involved the bone of the lateral orbital wall with bone destruction (arrow). c : MRI showed that the main body of the tumor was located lateral orbit and invaded the intracranial dura through the superior orbital fissure (arrow). d : After the Transfronto-orbital approach was given to the craniotomy, the MRI re-examinations immediately after the operation showed that the tumors were removed completely. There was no recurrence after 7 years of MRI follow-up Fig. 5 Case 5. The patient was a 46-year-old with a chronic disease process and right exophthalmos for more than 3 months. The patients underwent 2 operations (Transorbital approach) 12 years ago and 6 years ago. a, b : CT showed that the tumor involved the bone of supraorbital bone with bone destruction (arrow). C, D: MRI showed that the main body of the tumor invaded the intracranial dura through the destruction of the skull base bone (arrow). e, f : After the Transfronto-orbital approach was given to the craniotomy, the MRI re-examinations immediately after the operation showed that the tumors were removed completely. There was no recurrence after 5 years of MRI follow-up Fig. 6 Case 6. The patient was a 48-year-old with a chronic disease process and left exophthalmos for more than 10 months. The patient underwent an operation (Transfronto-orbital approach) 6 years ago. a, b : CT showed that the tumor involved the bone of supraorbital bone with bone destruction (arrow). c, d : MRI showed that the main body of the tumor invaded the intracranial dura and cavernous sinus through the destruction of the skull base bone (arrow). e, f : After the Transfronto-orbital approach was given to the craniotomy, the MRI re-examinations immediately after the operation showed that the tumors were removed completely. There was no recurrence after 4 years of MRI follow-up
| 4.292969
| 0.513184
|
sec[3]/sec[4]/p[0]
|
en
| 0.999998
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33499815
|
https://doi.org/10.1186/s12886-021-01825-6
|
[
"tumor",
"bone",
"orbital",
"approach",
"completely",
"involved",
"recurrence",
"dura"
] |
[
{
"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 CLASSIFICATION ═══
【1. Neoplasms of unknown behaviour of unspecified site (2F9Z)】
Synonyms: neoplasia | neoplasm growth NOS | tumour of unspecified site | tumourlet of unspecified site | tumour mass NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of unspecified site
【2. Subcutaneous swelling, mass or lump of uncertain or unspecified nature (ME61)】
Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature
Synonyms: localised swelling, mass or lump of skin and subcutaneous tissue | localised subcutaneous nodules | subcutaneous nodules | superficial subcutaneous nodules | localised swelling
Excludes: localized adiposity | mass and lump: breast | enlarged lymph nodes | mass and lump: intra-abdominal or pelvic | oedema
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings involving the skin → Symptoms or signs involving the skin → Subcutaneous swelling, mass or lump of uncertain or unspecified nature
【3. Carcinoma in situ of unspecified site (2E6Z)】
Synonyms: carcinoma in situ of unspecified site | carcinoma in situ NOS | carcinoma in situ | in situ neoplasm
Hierarchy: Neoplasms (02) → In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues → Carcinoma in situ of unspecified site
【4. Neoplasms of unknown behaviour of trachea, bronchus or lung (2F91.1)】
Synonyms: 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 | Lung hemangiopericytoma of unknown behaviour
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs (2F91) → Neoplasms of unknown behaviour of trachea, bronchus or lung
【5. Neoplasms of unknown behaviour of skin (2F92)】
Synonyms: skin tumour NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of skin
|
2F9Z
|
Neoplasms of unknown behaviour of unspecified site
|
This study was approved by ethic committee of Meizhou People's Hospital . In this study, informed consent was obtained from the patient. The consent was obtained on October 7, 2022, through a written form signed by the patient. Before signing, the patient received a detailed explanation of the study's purpose, procedures, potential risks, and benefits, as well as assurances regarding the protection of their personal information and privacy. It was emphasized that consent was voluntary, with the right to withdraw at any time without adverse consequences. Additionally, all personally identifiable information has been de‐identified to safeguard the patient's privacy. A 27‐year‐old unmarried woman with a history of sexual activity visited our hospital due to prolonged menstrual periods (extending from the usual 4–5 days to 10–12 days) and increased menstrual flow (accompanied by large blood clots) over the past 4 months. She experienced menarche at 14 and currently has a menstrual cycle of 24–26 days, with no significant changes in the cycle pattern. Occasionally, she reported dizziness and fatigue but no post‐coital bleeding, abdominal pain, nausea, or vomiting. Gastrointestinal and urinary system examinations showed no abnormalities, and her mental state, sleep, appetite, bowel movements, and weight remained stable. She had no history of drug allergies and no significant medical, surgical, or family history. A gynecologic examination revealed an enlarged uterus, comparable in size to an 8‐week pregnancy. Auxiliary examinations included complete blood count, liver and kidney function tests, electrolytes, ECG, chest X‐ray, cardiac ultrasound, and ultrasounds of the liver and urinary system. The complete blood count indicated moderate anemia with a hemoglobin (Hgb) level of only 74 g/L. Liver and kidney function tests and electrolyte levels were normal. ECG, chest X‐ray, and ultrasounds of the heart and urinary system showed no significant abnormalities. Magnetic resonance imaging (MRI) revealed an enlarged, anteriorly positioned uterus with multiple round‐like masses between the muscle walls/submucosa, the largest being approximately 2.7 × 2.2 × 2.2 cm. These masses appeared isointense on T1‐weighted images, iso to slightly hyperintense on T2‐weighted images, slightly hyperintense on diffusion‐weighted imaging (DWI) sequences, with reduced ADC values. The enhanced scan showed uneven, marked enhancement, with patchy weak/non‐enhanced areas within some lesions. The uterine cavity was enlarged, showing patchy high and low signals on T2WI, with no clear enhancement on the enhanced scan . Considering these findings, we diagnosed DUL with moderate anemia. In the preoperative assessment, we used the size, topography, extension of the base, penetration and lateral wall position (STEPW) scoring system, assigning eight points (Table 1 ). We recommended gonadotropin‐releasing hormone agonist (GnRHa) treatment to correct anemia and reduce tumor size, followed by surgical treatment, and informed her of the potential need for multiple hysteroscopic surgeries. However, the patient was unable to accept a long, multistage surgical treatment plan. Considering her unique situation, we organized a multidisciplinary discussion. As a young woman with predominantly submucosal fibroids, the use of laparotomy or laparoscopy might damage her endometrium, leading to pelvic adhesions, increased infertility risk, or ectopic pregnancy. If traditional hysteroscopic electrosurgery is employed, it may lead to extensive damage to the endometrium, with a high risk of postoperative intrauterine adhesions. Furthermore, due to the numerous fibroids, there is a risk of hyponatremia during traditional hysteroscopic surgery, which may require multiple procedures to complete, increasing the economic burden on the patient and causing additional damage to the endometrium. Therefore, we devised a personalized treatment plan for the patient, aimed at treating multiple submucosal fibroids in a single session while maximally preserving her fertility. This approach primarily involves the use of hysteroscopy combined with ultrasound‐guided clamping and excision of submucosal uterine fibroids. For the first time, we used thoracic tissue forceps as cold instruments to excise submucosal uterine fibroids. The forceps' rounded, blunt head minimizes damage to surrounding tissues. Additionally, its gripping arm is equipped with sharp cutting blades, which effectively excise lymphoid tissue while preserving the integrity of the surrounding tissues. With these forceps, assisted by hysteroscopy and ultrasound, we could precisely excise submucosal fibroids. The surgical procedure is detailed in Video S1 . A urinary catheter was inserted, and the uterus was probed to a depth of about 9 cm, revealing a patent cervical canal and a pear‐shaped uterine cavity with slightly thickened endometrium. Multiple uterine fibroids were observed within the cavity, some with rich surface vasculature, ranging in size from ~ 0.5 to 2–3 cm . Under ultrasound guidance, biopsy forceps were used to grasp and fix the part of the submucosal fibroid protruding into the uterine cavity, penetrating its capsule to reach the tumor body. By rotating and pulling, we disrupted the capsule and separated part of the tumor, which then protruded into the cavity. The remaining tumor was gradually clamped and excised under ultrasound monitoring. The final hysteroscopic review showed no tumors in the cervical canal or uterine cavity, and the endometrium was not thickened. After these procedures, an Interceed absorbable adhesion barrier was wrapped around the uterine balloon stent (model ZJ‐5, Jiangxi Xinnuo Bioengineering Co., Ltd., China) and inserted into the uterine cavity under ultrasound guidance, followed by the injection of 5 mL of saline to reduce bleeding and prevent adhesion . The entire surgery lasted 50 min (15 min for hysteroscopic examination, 30 min for fibroid surgery in a no‐distension state, and 5 min for placing the uterine balloon stent), with 2900 mL of irrigation used and 2800 mL returned, and an estimated blood loss of about 20 mL. A total of 38 uterine fibroids were successfully excised . According to the FIGO classification system: three (7.89%) were type III, nine (23.68%) were type II, 15 (39.47%) were type I, and 11 (28.96%) were type 0. Postoperative pathology suggested typical uterine leiomyomas . No complications such as uterine perforation or hyponatremia occurred during or after the surgery, and the patient was discharged smoothly on the second day post‐operation. Ten days post‐surgery, the uterine balloon was removed. A one‐month follow‐up revealed a good recovery, with a complete blood count showing Hgb 109 g/L and MRI showing no recurrence of uterine fibroids . A second office hysteroscopic examination also found no significant tumors. The uterine cavity was normal in shape, with no endometrial thickening. At the uterine fundus, we discovered a yellowish mass‐like substance, presumed to be residual anti‐adhesion film, which was scraped out . The patient resumed her menstrual cycle 40 days after surgery, with a cycle of 25–26 days, a period of 5–6 days, and normal menstrual flow without dysmenorrhea. A one‐year postoperative review showed Hgb 116 g/L and MRI revealed no recurrence of uterine fibroids .
| 4.019531
| 0.908691
|
sec[1]/p[0]
|
en
| 0.999996
|
39072716
|
https://doi.org/10.1002/ijgo.15800
|
[
"uterine",
"fibroids",
"cavity",
"ultrasound",
"hysteroscopic",
"submucosal",
"surgery",
"menstrual"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Inflammatory disorders of the uterus, except cervix, unspecified (GA01.Z)】
Synonyms: Inflammatory disorders of the uterus, except cervix | inflammatory disease of the uterus | uterine inflammatory disease | uterus inflammation | syncytioma
Hierarchy: Diseases of the female genital system → Inflammatory disorders of the female genital tract → Inflammatory disorders of the uterus, except cervix (GA01) → Inflammatory disorders of the uterus, except cervix, unspecified
【2. Other specified acquired abnormalities of uterus, except cervix (GA16.Y)】
Synonyms: Polyp of corpus uteri | intrauterine polyp | polyp of body of uterus | polyp of uterus | uterine polyp
Hierarchy: Diseases of the female genital system → Noninflammatory disorders of female genital tract → Acquired abnormalities of uterus, except cervix (GA16) → Other specified acquired abnormalities of uterus, except cervix
【3. Injury of uterus (NB92.6)】
Synonyms: uterine injury | intrauterine injury NOS | Injury of uterus without open wound into cavity | Injury of uterus with open wound into cavity | Laceration of uterus
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injuries to the abdomen, lower back, lumbar spine or pelvis → Injury of urinary or pelvic organs (NB92) → Injury of uterus
【4. Other specified fistulae involving female genital tract (GC04.1Y)】
Synonyms: Other female intestinal-genital tract fistulae | Intestinouterine fistula | enterouterine fistula | Cervicosigmoidal fistula | Rectouterine fistula
Hierarchy: Certain specified diseases of urinary system → Fistula of the genitourinary tract (GC04) → Fistulae involving female genital tract (GC04.1) → Other specified fistulae involving female genital tract
|
GA01.Z
|
Inflammatory disorders of the uterus, except cervix, unspecified
|
A 64-year-old man was admitted to our hospital for acute osteomyelitis of the toes, presenting with left toes ulcer due to infection developed after a fall injury 3 days prior to admission. The patient had a past medical history of hypertension, chronic kidney disease (CKD), and diabetes mellitus controlled using insulin. The patient's initial vital signs were as follows: blood pressure, 189/104 mmHg; heart rate, 109 beats/min; and blood temperature, 39.8°C. Physical examination of his left great and second toes revealed ulcers with pus, swelling, and surrounding erythema. Laboratory test results revealed white blood cell count, 10,000 cells/µl (differential count, 88.9% neutrophils); elevated C-reactive protein levels, 3.09 mg/dl (normal: <0.14 mg/dl); fasting blood glucose, 316 mg/dl (normal: <110 mg/dl); and glycated hemoglobin, 12.4% (normal: <6.0%). Moderate renal dysfunction was observed. SARS-CoV-2 was undetectable using reverse-transcriptase polymerase chain reaction (RT-PCR) on a nasopharyngeal swab specimen. Magnetic resonance imaging (MRI) indicated acute osteomyelitis of the toes . Subsequently, the patient underwent surgical debridement of the ulcers to enhance the healing process. Following the collection of blood and pus for cultures, the patient was intravenously administered with ampicillin-sulbactam (1.5 g every 8 h). Simultaneously, a continuous insulin infusion was initiated to strictly control hyperglycemia. On the second day after admission, nosocomial transmission of SARS-CoV-2 infection occurred. A repeated nasopharyngeal swab for RT-PCR showed negative results; however, on day 3, the patient complained of a sore throat. His vital signs were stable except for low-grade fever of 36.9°C. A follow-up RT-PCR showed positive results for SARS-CoV-2. His physical examination and chest radiograph showed unremarkable findings . Serum cardiac enzyme levels were within normal ranges. ECG showed sinus rhythm and concave ST-segment elevation in precordial leads, suggestive of early repolarization . Echocardiography showed mild concentric left ventricular (LV) hypertrophy with normal contraction and enlarged left atrium suggestive of diastolic dysfunction; however, pericardial effusion was not observed . After the patient was isolated, intravenous infusions of antiviral agent (remdesivir, a loading dose of 200 mg followed by 100 mg for 2 days) and SARS-CoV-2 neutralizing antibody (sotrovimab, a single dose of 500 mg) were administered for mild COVID-19 treatment. On day 7, all the cultures collected on admission yielded Staphylococcus aureus sensitive to cefazolin and de-escalation of intravenous cephazolin (2 g every 8 h) was performed for 4 weeks. The patient achieved improvement in glycemic control and was then switched to conventional regular subcutaneous insulin. His wound healing process was uneventful. On day 13, the patient experienced dyspnea. His vital signs were blood pressure, 134/77 mmHg; heart rate, 68 beats/min; body temperature, 36.7°C; respiratory rate, 18 breaths/min; and oxygen saturation, 85% on ambient air. A follow-up chest radiograph revealed cardiomegaly with left pleural effusion. The patient's body weight increased by 8 kg. Jugular vein distention and bilateral leg edema were noted; serum brain natriuretic peptide level was elevated (332 pg/ml, normal: <18 pg/ml). Therefore, a presumptive diagnosis of acute heart failure was made, and the patient was administered oxygen at 2 L/min and treated with intravenous loop diuretics (furosemide, 20 mg twice daily) to control volume overload. On day 15, a repeated RT-PCR confirmed SARS-CoV-2 negativity, ending isolation. On day 17, the patient's condition exacerbated . Chest computed tomography (CT) revealed moderate pericardial and bilateral pleural effusions . The follow-up ECG showed ST-segment normalization . The follow-up echocardiography revealed a moderate pericardial effusion with tamponade physiology, suggestive of PT . Cardiac MRI suggested active pericarditis . Right heart catheterization confirmed PT with equalization of diastolic pressures across all chambers and marked hemodynamic pulsus paradoxus ( Supplementary Table S1 ). Subsequently, the patient underwent an urgent pericardiocentesis with a placement of pericardial drainage, showing 750 ml of hemorrhagic exudate fluid . Pericardial fluid (PF) analysis showed hypercytokinemia consistent with an inflammatory process ( Supplementary Table S2 ). SARS-CoV-2 in the PF was undetectable using RT-PCR. Gram and Ziehl-Neelsen staining and bacterial and fungal cultures yielded negative results. Serologic testing for autoimmune diseases and cardiotropic viruses workup indicated negative results. Notably, PF cytology suggested adenocarcinoma cells suspecting carcinomatous pericarditis , for which ibuprofen (600 mg three times daily) and colchicine (0.5 mg twice daily) were initiated. On day 18, the patient underwent bilateral thoracocentesis with drainage of serous transudate pleural fluids, which also showed hypercytokinemia ( Supplementary Table S3 ). SARS-CoV-2 in the pleural fluids was also undetectable using RT-PCR. After confirmation of no pericardial effusion recurrence, the drain was removed. Upper endoscopy and colonoscopy for cancer screening showed unremarkable findings. CT screening revealed right paraesophageal and hilar lymph nodes swelling . On day 26, positron emission tomography (PET)/CT with the glucose analog 2-[18F]-fluoro-2-deoxy-d-glucose (FDG) disclosed the slight hypermetabolic activities in the same lesions . Owing to the concern for malignancy, the patient underwent video-assisted thoracoscopic biopsy (VATS-biopsy) of the FDG-avid hilar lymph node, revealing multiple non-caseating granulomas suggestive of sarcoidosis . However, considering normal serum angiotensin converting enzyme (ACE) levels and the absence of pulmonary, skin, and eye involvement, the patient was diagnosed with a sarcoid-like reaction. Furthermore, re-examination of the PF cytological materials using immunohistochemistry revealed that the atypical cells were reactive mesothelial cells because they were positive for D2-40, a specific cell-marker for mesothelial cell , which was unlikely due to malignancy. Therefore, based on the temporal association between preceding SARS-CoV-2 infection and the development of subacute PT without other identifiable causes, a final diagnosis of COVID-19-associated acute pericarditis was made. On day 36, the patient developed leukopenia during treatment, which was suspected to be drug-induced, that resolved with colchicine discontinuation. Alternatively, oral prednisolone (20 mg/day) was added because of residual pericardial thickening with pericardial effusion on the follow-up echocardiography. Thereafter, the patient's clinical condition improved steadily, and he was discharged on day 55. A significant improvement in size of the affected lymphadenopathies was also observed on day 90 . Furthermore, a complete resolution of pericardial structural and functional abnormalities with concurrent pleural effusions was observed at the 6-month follow-up . Thereafter, ibuprofen and prednisolone were tapered and discontinued over 3 months. The patient remains clinically stable during the first year of follow-up. We present a summarized illustration of the case presentation in Figure 5 .
| 3.982422
| 0.977051
|
sec[1]/p[0]
|
en
| 0.999998
|
38264260
|
https://doi.org/10.3389/fcvm.2023.1329952
|
[
"pericardial",
"sars",
"follow",
"blood",
"using",
"results",
"effusion",
"pleural"
] |
[
{
"code": "BB2Z",
"title": "Pericarditis, unspecified"
},
{
"code": "LA8D",
"title": "Congenital pericardial anomaly"
},
{
"code": "BB2Y",
"title": "Other specified pericarditis"
},
{
"code": "BB22",
"title": "Constrictive pericarditis"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Pericarditis, unspecified (BB2Z)】
Synonyms: pericarditis NOS | pericardial inflammation | pericardium inflammation
Hierarchy: Diseases of the circulatory system (11) → Pericarditis → Pericarditis, unspecified
【2. Congenital pericardial anomaly (LA8D)】
Definition: A congenital cardiovascular malformation in which there is a structural and/or functional abnormality of the pericardium.
Synonyms: malformations of pericardium | structural developmental anomalies of the pericardium | congenital anomaly of pericardium | structural developmental anomaly of the pericardium | Complete agenesis of pericardium
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the circulatory system → Structural developmental anomaly of heart or great vessels → Congenital pericardial anomaly
【3. Other specified pericarditis (BB2Y)】
Synonyms: Certain diseases of pericardium | Chronic adhesive pericarditis | adherent pericarditis | adherent pericardium | adhesive pericarditis
Hierarchy: Diseases of the circulatory system (11) → Pericarditis → Other specified pericarditis
【4. Constrictive pericarditis (BB22)】
Definition: Chronic fibrous pericarditis due to the presence of dense fibrous tissue between the parietal and visceral layers of pericardium and neighbouring structures.
Synonyms: pericarditis calculosa | Hutinel-Pick syndrome | chronic tamponade | chronic pericardial constriction | Pick syndrome of heart
Hierarchy: Diseases of the circulatory system (11) → Pericarditis → Constrictive pericarditis
|
BB2Z
|
Pericarditis, unspecified
|
One traditional CoT prompt and four clinical reasoning prompts were developed (differential diagnosis, analytical, Bayesian and intuitive reasoning). Each prompt included two example questions (Table 1 ) with rationales employing the target reasoning strategy. This is a technique known as few-shot learning 14 . The full prompts used for the MedQA dataset are provided in Table 2 ; the full prompts used for the NEJM challenge set are provided in Supplementary Note 1 . Table 1 Example MedQA questions. Example Question 1 Shortly after undergoing a bipolar prosthesis for a displaced femoral neck fracture of the left hip acquired after a fall the day before, an 80-year-old woman suddenly develops dyspnea. The surgery under general anesthesia with sevoflurane was uneventful, lasting 98 min, during which the patient maintained oxygen saturation readings of 100% on 8 l of oxygen. She has a history of hypertension, osteoporosis, and osteoarthritis of her right knee. Her medications include ramipril, naproxen, ranitidine, and a multivitamin. She appears cyanotic, drowsy, and is oriented only to person. Her temperature is 38.6 °C (101.5 °F), pulse is 135/min, respirations are 36/min, and blood pressure is 155/95 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 81%. There are several scattered petechiae on the anterior chest wall. Laboratory studies show a hemoglobin concentration of 10.5 g/dl, a leukocyte count of 9000/mm 3 , a platelet count of 145,000/mm 3 , and a creatine kinase of 190 U/l. An ECG shows sinus tachycardia. What is the most likely diagnosis? Example Question 2 A 55-year-old man comes to the emergency department because of a dry cough and severe chest pain beginning that morning. Two months ago, he was diagnosed with inferior wall myocardial infarction and was treated with stent implantation of the right coronary artery. He has a history of hypertension and hypercholesterolemia. His medications include aspirin, clopidogrel, atorvastatin, and enalapril. His temperature is 38.5°C (101.3 °F), pulse is 92/min, respirations are 22/min, and blood pressure is 130/80 mm Hg. Cardiac examination shows a high-pitched scratching sound best heard while sitting upright and during expiration. The remainder of the examination shows no abnormalities. An ECG shows diffuse ST elevations. Serum studies show a troponin I of 0.005 ng/ml ( N < 0.01). What is the most likely cause of this patient’s symptoms? Example questions used in all MEDQA prompts provided in Table 2 . Table 2 CoT and diagnostic reasoning prompts. Traditional CoT reasoning prompt Traditional chain-of-thought (CoT) Prompt: Provide a step-by-step deduction that identifies the correct response {Example Question 1} Example Rationale 1: The patient had a surgical repair of a displaced femoral neck fracture. The patient has petechiae. The patient has a new oxygen requirement, meaning they are having difficulty with their breathing. This patient most likely has a fat embolism. {Example Question 2} Example Rationale 2: This patient is having chest pain. They recently had a heart attack and has new chest pain, suggesting he may have a problem with his heart. The EKG has diffuse ST elevations and he has a scratching murmur. This patient likely has Dressler Syndrome. Clinical reasoning prompts Differential diagnosis CoT Prompt: Use step by step deduction to create a differential diagnosis and then use step by step deduction to determine the correct response . {Example Question 1} Example Rationale 1: This patient has shortness of breath after a long bone surgery. The differential for this patient is pulmonary embolism, fat embolism, myocardial infarction, blood loss, anaphylaxis, or a drug reaction. The patient has petechiae which makes fat embolism more likely. This patient most likely has a fat embolism. {Example Question 2} Example Rationale 2: This patient has chest pain with diffuse ST elevations after a recent myocardial infarction. The differential for this patient includes: myocardial infarction, pulmonary embolism, pericarditis, Dressler syndrome, aortic dissection, and costochondritis. This patient likely has a high-pitched scratching sound on auscultation associated with pericarditis and Dressler Syndrome. This patient has diffuse ST elevations associated with Dressler Syndrome. This patient most likely has Dressler Syndrome. Intuitive reasoning CoT Prompt: Use symptom, signs, and laboratory disease associations to step by step deduce the correct response . {Example Question 1} Example Rationale 1: This patient has findings of petechiae, altered mental status, shortness of breath, and recent surgery suggesting a diagnosis of fat emboli. The patient most likely has a fat embolism. {Example Question 2} Example Rationale 2: This patient had a recent myocardial infarction with new development of diffuse ST elevations, chest pain, and a high pitched scratching murmur which are found in Dressler’s syndrome. This patient likely has Dressler’s Syndrome. Analytic reasoning CoT Prompt: Use analytic reasoning to deduce the physiologic or biochemical pathophysiology of the patient and step by step identify the correct response . {Example Question 1} Example Rationale 1:The patient recently had large bone surgery making fat emboli a potential cause because the bone marrow was manipulated. Petechiae can form in response to capillary inflammation caused by fat emboli. Fat micro globules cause CNS microcirculation occlusion causing confusion and altered mental status. Fat obstruction in the pulmonary arteries can cause tachycardia and shortness of breath as seen in this patient. This patient most likely has a fat embolism. {Example Question 2} Example Rationale 2: This patient had a recent myocardial infarction which can cause myocardial inflammation that causes pericarditis and Dressler Syndrome. The diffuse ST elevations and high pitched scratching murmur can be signs of pericardial inflammation as the inflamed pericardium rubs against the pleura as seen with Dressler Syndrome. This patient likely has Dressler Syndrome. Bayesian reasoning CoT Prompt: Use step-by-step Bayesian Inference to create a prior probability that is updated with new information in the history to produce a posterior probability and determine the final diagnosis . {Example Question 1} Example Rationale 1: The prior probability of fat embolism is 0.05% however the patient has petechiae on exam which is seen with fat emboli, which increases the posterior probability of fat embolism to 5%. Altered mental status increases the probability further to 10%. Recent orthopedic surgery increases the probability of fat emboli syndrome to 60%. This patient most likely has a fat embolism. {Example Question 2} Example Rationale 2: The prior probability of Dressler Syndrome is 0.01%. The patient has diffuse ST elevations, increasing the probability of Dressler Syndrome to 5%. The patient has a scratching murmur which increases the probability to 10%. In the setting of a recent MI the posterior probability of myocardial infarction is 55%. This patient likely has Dressler Syndrome. The traditional and diagnostic CoT prompts evaluated in this study. The provided rationales are in response to the example questions provided in Table 1 . Note that the full text of the example questions has been omitted for readability.
| 4.078125
| 0.80127
|
sec[0]/p[6]
|
en
| 0.999995
|
38267608
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https://doi.org/10.1038/s41746-024-01010-1
|
[
"example",
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"dressler",
"question",
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[
{
"code": "1C62.Z",
"title": "Human immunodeficiency virus disease without mention of tuberculosis or malaria, clinical stage unspecified"
},
{
"code": "1E32",
"title": "Influenza, virus not identified"
},
{
"code": "5C56.0Y",
"title": "Other specified sphingolipidosis"
},
{
"code": "8A44.1",
"title": "Adrenoleukodystrophy"
},
{
"code": "4A01.31",
"title": "DNA repair defects other than combined T-cell or B-cell immunodeficiencies"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Human immunodeficiency virus disease without mention of tuberculosis or malaria, clinical stage unspecified (1C62.Z)】
Synonyms: Human immunodeficiency virus disease without mention of tuberculosis or malaria | human immunodeficiency virus infection | HIV - [human immunodeficiency virus infection] | HIV positive NOS | HIV disease
Hierarchy: Certain infectious or parasitic diseases (01) → Human immunodeficiency virus disease → Human immunodeficiency virus disease without mention of tuberculosis or malaria (1C62) → Human immunodeficiency virus disease without mention of tuberculosis or malaria, clinical stage unspecified
【2. Influenza, virus not identified (1E32)】
Definition: Any disease of the respiratory system, caused by an unidentified strain of influenza virus. These diseases are characterised by fever, cough, headache, myalgia, arthralgia, or malaise. Transmission is by inhalation of infected respiratory secretions.
Synonyms: flu | Influenza NOS | Viral influenza specific virus not stated to have been identified | Influenza specific virus not stated to have been identified | Influenza with pneumonia, virus not identified
Excludes: Haemophilus influenzae [H. influenzae] meningitis | Haemophilus influenzae [H. influenzae] pneumonia
Hierarchy: Certain infectious or parasitic diseases (01) → Influenza → Influenza, virus not identified
【3. Other specified sphingolipidosis (5C56.0Y)】
Synonyms: Mucolipidosis type 4 | Sialolipidosis | Gaucher disease | Acid beta-glucosidase deficiency | Glucocerebrosidase deficiency
Hierarchy: Inborn errors of metabolism → Lysosomal diseases (5C56) → Sphingolipidosis (5C56.0) → Other specified sphingolipidosis
【4. Adrenoleukodystrophy (8A44.1)】
Definition: X-linked genetic disorder associated with accumulation of very-long-chain fatty acids in the brain and adrenal cortex due to a mutation in the ABCD1 gene causing defects in peroxisomal oxidation. Neurological symptoms can present in childhood or adulthood with almost all patients having concurrent adrenal insufficiency.
Synonyms: ALD - [adrenoleukodystrophy] | Addison-Schilder | Adult-onset autosomal dominant leukodystrophy | Autosomal dominant Pelizaeus-Merzbacher disease | Multiple sclerosis-like disorder
Hierarchy: Diseases of the nervous system (08) → Multiple sclerosis or other white matter disorders → Leukodystrophies (8A44) → Adrenoleukodystrophy
【5. DNA repair defects other than combined T-cell or B-cell immunodeficiencies (4A01.31)】
Synonyms: Nijmegen breakage syndrome | Autosomal recessive nonsyndromal microcephaly with normal intelligence | Immunodeficiency - microcephaly - chromosomal instability | Microcephaly - immunodeficiency - lymphoreticuloma | Ataxia-telangiectasia, variant 1
Hierarchy: Primary immunodeficiencies → Primary immunodeficiencies due to disorders of adaptive immunity (4A01) → Other well-defined immunodeficiency syndromes due to defects in adaptive immunity (4A01.3) → DNA repair defects other than combined T-cell or B-cell immunodeficiencies
|
1C62.Z
|
Human immunodeficiency virus disease without mention of tuberculosis or malaria, clinical stage unspecified
|
The patient was a woman in her seventies and had a history of appendectomy, hypothyroidism, and cataracts. She had undergone endoscopic papillary balloon dilatation for choledocholithiasis about 2 years ago. Thereafter, the choledocholithiasis recurred and endoscopic sphincterotomy was performed. Subsequently, the patient visited Saiseikai Toyama Hospital for surgery due to symptoms of recurrence. No findings of jaundice or anemia were noted; the abdomen was flat and soft without tenderness and peristaltic sounds of the bowel were regular. Laboratory findings revealed no inflammation or increases in the levels of the hepatobiliary enzymes. The levels of the tumor markers carcinoembryonic antigen (CEA) and carbohydrate antigen (CA) 19-9 were within the normal ranges. Abdominal computed tomography (CT) showed a low absorption zone in the gallbladder wall suggestive of a cyst. The low absorption areas displayed lower CT values than the gallbladder lumen . In addition, the presence of stones (about 15 mm in diameter) in the gallbladder was detected by drip infusion cholecystocholangiography-CT (DIC-CT) imaging. No contrast medium was observed in the suspected cyst area . Abdominal ultrasonography revealed the presence of bile sludge, but no abnormalities in the gallbladder wall other than the cysts and the stone were noted. Laparoscopic cholecystectomy was performed and the patient was diagnosed with cholecystocholedocholithiasis. No adhesions in the surrounding tissues of the gallbladder were observed during intraoperative examination. A whitish region different from the original color of the wall was observed at the boundary between the liver bed and the bottom of the gallbladder when the gallbladder was stripped from the liver. The gallbladder was carefully resected to include the whitish region. Macroscopically, apart from the biliary sludge, no stones were observed in the gallbladder. The mucosal surface of the gallbladder maintained a normal reticular pattern, and no ulcerated or depressed lesions were observed. However, a slightly elevated lesion was observed on the liver bed side at the bottom of the gallbladder, which appeared like a white-colored submucosal tumor . Although no thickening of the gallbladder wall was observed on the cut surface, several cysts were found within the wall corresponding to the elevated lesion on the liver bed side . The lumen of the cyst contained a transparent serous liquid with no bile juice, biliary sludge or stones. Histopathologically, several cysts with diameters ranging from 3 to 5 mm were found in the subserosal layer on the hepatic side of the liver bed . The lining epithelium on the inner surface of the cyst, although partially flattened, consisted of a monolayer of cuboidal or low columnar cells, which did not demonstrate atypia. The nuclei of the cells were small and round to oval in shape, and the cytoplasm was clear or weakly eosinophilic. No cilia were found in the lining epithelium. Only exfoliated epithelial pieces were observed floating in the cyst lumen. The cysts were encircled by dense fibrous tissue intermingled with the fibroblastic cells . However, no muscle fibers like those existing in the fibromuscular layer of the gallbladder were observed around the cyst. Furthermore, a muscle fiber layer reminiscent of the muscularis propria of the intestinal tract was absent. The small tubules were surrounded by concentric dense fibrous tissue and comprised of narrow lumens lined by low columnar epithelia; these were thought to be the ducts of Luschka . Lymphocytic infiltration was observed in the existing lamina propria, but no hyperplastic changes, invagination of the mucosal epithelium or RAS were detected. The immunohistochemical results are shown in Table 1 . The cyst lining epithelium and mucosal epithelium were positive for cytokeratin 7 and negative for cytokeratin 20 (CK20) and CDX-2. A positive reaction for CD10 was observed on the luminal surface side. In the mucous phenotype, MUC5AC was strongly positive in the cytoplasm of the mucosal epithelium but negative in the cystic epithelium. Although positive immunoreactions for MUC6 were observed in both types of epithelia, only low columnar cells on the crypt bottom of the mucosal epithelium demonstrated partial positive reactions , whereas in the cystic epithelium, scanty positive reactions were observed in the cytoplasm of the cells . Positive reactions for MUC2 and CEA were observed only in the cystic epithelium. However, podoplanin and CD34 were negative in both the epithelia. In addition, the coexisting Luschka’s duct epithelium showed the same expression pattern as that in the cystic epithelium . Furthermore, the fibroblastic cells and fibrous components around the cyst and the Luschka’s duct, as well as the smooth muscle cells and fibers that constituted the fibromuscular layer in the gallbladder wall, were all positive for α-smooth muscle actin . Nonetheless, immunoreactions for desmin were positive only in the fibromuscular layer of the gallbladder and negative in the cyst wall . Fig. 1 Radiological findings. (A) A cystic lesion (red arrow) was noted in the wall of the gallbladder via enhanced abdominal computed tomography (CT). (B) The cyst was not enhanced by drip infusion cholecystocholangiography-CT (red arrowhead). Fig. 1 Fig. 2 Macroscopic and histopathological findings. (A) On the inner surface of the gallbladder, a slightly elevated lesion was observed on the liver bed side at the bottom (red arrow). (B) On the cut surface, several cysts were seen within the wall of the elevated lesion (red arrowhead). (C) Several well-defined bordered cysts were seen in the subserosal layer only. (D) The cyst lining consisted of a monolayer of cuboidal cells. Dense and cellular fibrous tissue encircled the cyst. (E) Small structures suggestive of Luschka’s duct were scattered around the cyst. Fig. 2 Table 1 Results of immunohistochemical studies. Table 1 Epithelium Antibody (clone, source) Cyst Luschka's duct Mucosa Cytokeratin 7 (SP53, Roche) +++ +++ +++ Cytokeratin 19 (A53B/A2.26, Roche) +++ +++ +++ Cytokeratin 20 (SP33, Roche) − − − CD10 (SP67, Roche) +* +* +* CDX-2 (AMT28, Leica) − − − MUC1 (H23, Roche) + + + MUC2 (MRQ-18, Roche) + − − MUC5AC (AMT28, Novocastra) − − ++ MUC6 (CLH5, Novocastra) + + ++ CEA (TF3H8-1, Roche) ++ − − CA19-9 (121SLE, Roche) +++ +++ +++ Podoplanin (D2-40, Roche) − − − CD34 (QBEnd/10, Roche) − − − −: negative, +: week cytoplasmic positive, ++: strong cytoplasmic positive. +++: strong membranous and cytoplasmic positive, +*: luminal surface positive. Surrounding cell and fiber Antibody (clone, source) Cyst Luschka's duct Fibromuscular layer Actin, Smooth Muscle (1A4, Roche) + + + Desmin (DE-R-11, Roche) − − + −: negative, +: positive. Fig. 3 Immunohistochemical findings. (A and G) CK7, (B) MUC5AC, (C, D and H) MUC6, (E) MUC2, (F) CEA, (I and J) α-smooth muscle actin (αSMA), and (K, L) desmin. Cytoplasmic expression was observed in the cystic epithelium (A, D, E, F) and Luschka’s duct epithelium (G, H). Cytoplasmic expression was observed in the mucosal epithelium (B, C). Dense cellular fibrous tissue around the cyst and fibromuscular layer showed a positive reaction for αSMA (I and J), but desmin was expressed only in the fibromuscular layer (K) and not in the cyst wall. Fig. 3
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| 0.967773
|
sec[1]/p[0]
|
en
| 0.999995
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33887865
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https://doi.org/10.1016/j.ijscr.2021.105794
|
[
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"roche",
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[
{
"code": "DC12.Z",
"title": "Cholecystitis, unspecified"
},
{
"code": "DC10.01",
"title": "Obstruction of gall bladder"
},
{
"code": "QF01.Y",
"title": "Other specified acquired absence of organs"
},
{
"code": "DC10.2",
"title": "Fistula of gallbladder or bile duct"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Cholecystitis, unspecified (DC12.Z)】
Synonyms: Cholecystitis | gallbladder inflammation
Hierarchy: Diseases of the digestive system (13) → Diseases of gallbladder or biliary tract → Cholecystitis (DC12) → Cholecystitis, unspecified
【2. Obstruction of gall bladder (DC10.01)】
Synonyms: obstruction of gallbladder | gallbladder obstruction | Constriction of gallbladder | gallbladder constriction | Occlusion of gallbladder
Hierarchy: Diseases of gallbladder or biliary tract → Acquired anatomical alterations of gallbladder or bile ducts (DC10) → Obstruction of gallbladder or bile ducts (DC10.0) → Obstruction of gall bladder
【3. Other specified acquired absence of organs (QF01.Y)】
Synonyms: Acquired absence of part of head or neck | Acquired absence of eye | absence of eye | absence of eyeball | acquired anophthalmos
Hierarchy: Factors influencing health status → Acquired absence of body structure → Acquired absence of organs (QF01) → Other specified acquired absence of organs
【4. Fistula of gallbladder or bile duct (DC10.2)】
Definition: This is an abnormal connection or passageway between gallbladder or bile duct and other organs.
Synonyms: fistula of gallbladder | gallbladder fistula | Cholecystocolic fistula | Cholecystoduodenal fistula | Choledochoduodenal fistula
Hierarchy: Diseases of the digestive system (13) → Diseases of gallbladder or biliary tract → Acquired anatomical alterations of gallbladder or bile ducts (DC10) → Fistula of gallbladder or bile duct
|
DC12.Z
|
Cholecystitis, unspecified
|
If neutrophil counts cannot be sustained at adequate levels, the use of lithium or Granulocyte Colony Stimulating Factor (G-CSF) can be considered if the risk of a dyscrasia and managing a rebound psychosis is considered too great. Tables 2 and 3 outline the use of G-CSF and Lithium. Our approach has evolved. Our first case involved a patient who had had multiple episodes of non-clozapine drug included neutropenia with sepsis, CIN with severe clozapine withdrawal psychosis and prolonged hospitalization, extensive violence, self-harm and extremely long seclusion; prior to clozapine withdrawal there had been an excellent clinical response. The risks of rechallenge were high; the haematology advice favoured G-CSF over lithium to be used from the outset. In less extreme cases lithium can be a useful first line as set out by Manu . BEN followed by treatment emergent CIN : Lithium or G-CSF can be used to support neutrophil counts depending on the severity of the neutropenia and the patient’s history when psychotic. Lithium or G-CSF may be used to continue clozapine treatment . Lithium or G-CSF can be given without interruption of clozapine treatment. Decision making will balance the risks of clozapine withdrawal against those of a dyscrasia and the likely benefit of the strategy used. The initial approach we used for one patient, with the assistance of a haematologist, was to give G-CSF as required (plus-rescue). After each dose of G-CSF there was an excellent response in neutrophil count; however, after eight episodes of neutropenia with a nadir of 0.4 × 10 9 /L the patient was established on regular G-CSF. Although clozapine naïve, this patient had had several episodes of life threatening self-harm and of serious assaults on others in the context of active psychosis. CIN with gradual deterioration on clozapine withdrawal, disturbed behaviour (not the most extreme) : Lithium appears to have a good record of success when used to support clozapine rechallenge and has fewer difficulties in terms of side effects, administration, cost and downstream issues. There is little evidence regarding dose but in our experience a serum level of greater than 0.4 mmol/L has been sufficient to have a meaningful effect on neutrophil counts. This is easily assessed for each individual patient. If required, lithium can be replaced with G-CSF. These approaches were all successful in our patient group. CIN with rapid deterioration on clozapine withdrawal, disturbed behavior (extreme) : These patients present by far the most extreme problems in terms of the severity of neutropenia and psychosis. We have used prophylactic G-CSF in this group, with haematology assessment and support regarding dose, interval and monitoring. 3. For all cases Avoid other implicated drugs. For all patients we avoid the use of any other drugs implicated in neutropenia. This is most relevant if there is a history of clozapine induced seizures or if affective symptoms are prominent in which case topiramate may be preferable to valproate, lamotrigine or carbamazepine. Planning and Liaison. Careful teamwork will be required. A variety of simple practical steps were taken to ensure that G-CSF was available on the wards where it might be needed, with simple plans for its use should this be required by on-call junior medical staff. Whilst we increased the frequency of FBC testing (arbitrarily to initially twice weekly) plans were made to ensure that this was optimized in the afternoons, but not so late that results only became available out of hours. Liaison with other medical professionals can sometimes be confused with attempts to diffuse responsibility. It will be the treating psychiatrist who is responsible for the decision-making and not the haematologist. On occasion haematologists report requests from psychiatrists asking whether or not clozapine should or should not be restarted, rather than to advise on the hematological risks and how these might be managed. For cases when capacity is impaired or when patients do not consent, the difficulties and uncertainties must be shared with the legal power authorising treatment. The use of medication to support rechallenge may need to be specified depending on the approach taken by the approving body. In all cases, off-license agreements will be required with the clozapine provider. Family liaison will be important. In several of our cases both patients and their families, recognizing the benefits when clozapine had been used previously and the severity of the deterioration when it was withdrawn, were eager to rechallenge. Retitration rates. Given the probable contribution of an immune mechanism, a slower clozapine titration may be useful; as yet there is no evidence to support this . How long to continue Lithium or G-CSF. This is not known. Timing. Clozapine rechallenge is anxiety provoking. Given the types of judgments required, we planned our blood sampling regimes to avoid results out of hours or when key decision makers were away from the hospital. 4. Downstream Table 2 G-CSF Physiological role cytokine glycoprotein stimulating differentiation, release and survival of neutrophils and other granulocytes Drug Filgrastim (recombinant G-CSF) has been available since the early 1990’s; originally to treat patients with chemotherapy-induced neutropenia Current usage Severe chronic neutropenia, mobilisation of haematopoietic progenitors for stem cell transplantation [ 49 – 51 ], drug induced neutropenia . G-CSF and clozapine rechallenge after CIN / CIA Multiple case reports [ 49 , 53 – 65 ] reviewed by Lally et al ; G-CSF successfully supported over 70% of initial CIN cases. Success was markedly reduced following CIA. Common adverse effects and management Flu like symptoms, bone pain, headache, pyrexia and fatigue. Rare adverse effects Splenic rupture, glomerulonephritis, alveolar haemorrhage, thrombocytopenia and capillary leak syndrome. Side effect management and monitoring Analgesia, monitoring for splenomegaly and bone mineral density assessment . Long term safety Available from the 20,000 annual healthy volunteer peripheral blood donors. Initial concerns of increased leukaemia risk alleviated by long term follow up . Dose, We took advice from haematology colleagues. G-CSF dosages ranged between single as required injections of 15million units to 30 million units twice weekly. Administration Pre-loaded syringe for subcutaneous injection, volume < 1 ml Storage Refrigerated Cost UK approx. £50 ($65) per dose Institutional / systemic factors High cost novel drug in psychiatric practice. Treating teams likely to be unfamiliar with usage and this may not be supported by all mental health organisations. Table 3 Lithium and Neutrophils Physiological effects Enhances production of endogenous G-CSF, directly stimulating differentiation of stem cells and protecting neutrophils from the toxicity of some drugs, although to a far smaller extent than G-CSF. Haematological usage Reports of use to treat idiopathic neutropenia Lithium and clozapine rechallenge Reviews by Manu and Boazak show high rates of success. Adverse effects in clozapine rechallenge Isolated case reports of failure / fatality and of neurotoxicity Use, dose, side effects Familiar to psychiatrists Institutional / systemic factors Systemic difficulties not likley
| 4.121094
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sec[2]/sec[0]/p[2]
|
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| 0.999997
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https://doi.org/10.1186/s12888-020-02592-2
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[
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[
{
"code": "NE60",
"title": "Harmful effects of drugs, medicaments or biological substances, not elsewhere classified"
},
{
"code": "PB26",
"title": "Unintentional exposure to or harmful effects of antipsychotics"
},
{
"code": "PC96",
"title": "Intentional self-harm by exposure to or harmful effects of antipsychotics"
},
{
"code": "PH46",
"title": "Exposure to or harmful effects of undetermined intent of antipsychotics"
},
{
"code": "PE86",
"title": "Assault by exposure to or harmful effects of antipsychotics"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Harmful effects of drugs, medicaments or biological substances, not elsewhere classified (NE60)】
Synonyms: drugs, medicaments or biological substances, toxicity not elsewhere classified | adverse drug effects | drug reaction NOS | drug allergy NOS | drug toxicity NOS
Excludes: Alcohol intoxication | pathological drug intoxication | hypersensitivity reaction to correctly administered drug | Reactions or intoxications due to drugs administered to fetus or newborn | Opioid intoxication
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Harmful effects of substances → Harmful effects of drugs, medicaments or biological substances, not elsewhere classified
【2. Unintentional exposure to or harmful effects of antipsychotics (PB26)】
Synonyms: accidental overdose of antipsychotics | accidental poisoning by antipsychotics | antipsychotics taken in error | Unintentional exposure to or harmful effects of phenothiazine antipsychotics or neuroleptics | Unintentional exposure to or harmful effects of butyrophenone or thiothixene neuroleptics
Excludes: Substances associated with injury or harm in therapeutic use
Hierarchy: Unintentional causes → Unintentional exposure to or harmful effects of substances → Unintentional exposure to or harmful effects of drugs, medicaments or biological substances → Unintentional exposure to or harmful effects of antipsychotics
【3. Intentional self-harm by exposure to or harmful effects of antipsychotics (PC96)】
Synonyms: intentional self-poisoning by antipsychotics | Intentional overdose of antipsychotics | Toxic effect of neuroleptics | Intentional self-harm by exposure to or harmful effects of phenothiazine antipsychotics or neuroleptics | Intentional overdose of phenothiazine antipsychotics or neuroleptics
Hierarchy: Intentional self-harm → Intentional self-harm by exposure to or harmful effects of substances → Intentional self-harm by exposure to or harmful effects of drugs, medicaments or biological substances → Intentional self-harm by exposure to or harmful effects of antipsychotics
【4. Exposure to or harmful effects of undetermined intent of antipsychotics (PH46)】
Synonyms: harmful effects of neuroleptics, undetermined intent | Harmful effects of or exposure to phenothiazine antipsychotics or neuroleptics, undetermined intent | Harmful effects of or exposure to butyrophenone or thiothixene neuroleptics, undetermined intent | Harmful effects of or exposure to typical, second-generation antipsychotics, undetermined intent | Harmful effects of or exposure to lithium carbonate, undetermined intent
Hierarchy: Undetermined intent → Exposure to or harmful effects of substances, undetermined intent → Exposure to or harmful effects of undetermined intent of drugs, medicaments or biological substances → Exposure to or harmful effects of undetermined intent of antipsychotics
【5. Assault by exposure to or harmful effects of antipsychotics (PE86)】
Synonyms: Assault by exposure to or harmful effects of neuroleptics | homicidal poisoning by exposure to or harmful effects of antipsychotics | Assault by exposure to or harmful effects of phenothiazine antipsychotics or neuroleptics | Assault by exposure to or harmful effects of butyrophenone or thiothixene neuroleptics | Assault by exposure to or harmful effects of typical, second-generation antipsychotics
Hierarchy: Assault → Assault by exposure to or harmful effects of substances → Assault by exposure to or harmful effects of drugs, medicaments or biological substances → Assault by exposure to or harmful effects of antipsychotics
|
NE60
|
Harmful effects of drugs, medicaments or biological substances, not elsewhere classified
|
Our working group will aim to provide standardized best practices for multimodal neuroimaging analysis in AMS-TBI. This need is critical partly because analyzing MRI scans from AMS-TBI patients poses unique challenges from the standpoint of lesion mapping, pathology characterization, and clinical interpretation . The heterogeneity of lesion profiles (e.g, biomechanical cause, type of pathology, location, or volume) frequently makes automatic MRI analysis pipelines break down or fail due to causes that frequently include (but are not exclusive to) inaccurate co-registration of scans across modalities and time points, faulty voxel-wise morphometric analysis, and incorrect automatic parcellations of brain structures . Fig. 4 The complexity of lesion characterization and behavioral phenotyping after AMS-TBI. From a structural neuroimaging perspective trauma-induced abnormalities differ by time post-injury as well as the imaging modality being used. a are all CT based showing that the size and location of the hemorrhage, parenchymal displacement and edema dynamically change over time. b demonstrates that each MRI sequence has its own unique sensitivity in assessing different aspects of neuroanatomy and neuropathology. c which presents the FLAIR, SWI and T1 signal abnormalities, demonstrates the widespread pathology differently presented by these imaging methods. By 5 months’ post-injury, widespread volume loss, cortical atrophy, ventriculomegaly and encephalomalacia have occurred. d show summary findings from a neuropsychological assessment at ~8 months post injury. This case example depicts the neuropathological heterogeneity associated with TBI along with the dynamic changes over time and their influence on neuropsychological test results. This patient sustained a severe TBI from a motorcycle collision with a vehicle. The patient was not helmeted at the time of injury and, by witness accounts, was immediately rendered unconscious. Upon emergent care at the scene of the accident, the patient was assessed to have a Glasgow Coma Scale (GCS) of 3, was life-flighted to a Level I emergency department (ED) with GCS remaining 3 throughout transport and during ED assessment and treatment. In addition to the head injuries he sustained multiple systemic injuries including leg and rib fractures, pulmonary contusion and liver laceration. An intracranial pressure monitor was inserted, the patient underwent tracheostomy for airway management and shunted. The patient remained in a coma and received neurocritical care for almost 2 months, followed by 3 months of inpatient neurorehabilitation. a Initial day-of-injury computed tomography was performed about 90 min’ post-injury. What is important to note in the initial scan is the original size of the frontal intraparenchymal hemorrhage along with the size, symmetry and configuration of the ventricular system. Within 24 h, enlargement of the intraparenchymal hemorrhage is observed along with distinct effacement of the anterior horn of the lateral ventricle and surrounding edema associated with the hemorrhage. Subsequent to this scan he was shunted, with the shunt catheter clearly visible in the 2-week follow-up scan which depicts more edema and midline shift. By 5 months’ post-injury, there is prominence of the ventricular system and cortical sulci in association with cortical atrophy and frontal encephalomalacia associated with the location of the prior hemorrhage. b At 2 weeks post-injury, MRI studies were obtained. Each sequence demonstrates a different aspect of the “Lesion.” The T1 sequence, which is the one commonly used for automated methods of image segmentation and classification for quantitative analyses, depicts coarse anatomical features of the brain, but the focal intraparenchymal hemorrhage and surrounding edema is not fully appreciated, being better distinguished by the T2 and FLAIR sequences. The SWI sequence depicts multiple, bilaterally scattered foci of hemosiderin deposition reflective of shear injury, with particularly exquisite demarcation differentiating hemorrhage, parenchymal degradation along with the surrounding edema. c Using a thresholding method for detecting white matter signal abnormality in FLAIR scans, the regions of white matter hyperintensity are depicted three dimensionally in the images on the left. Each signal abnormality likely reflects localized white matter pathology. In the middle are the regions of hemosiderin deposition detected on SWI, likewise reflecting specific foci of shear-lesion pathology constituting diffuse axonal injury. On the right are the abnormalities found on T1. d Findings from neuropsychological assessment at almost 8 months post injury are presented as z-score deviations from test manual normative data. The following tests were administered: Repeatable Battery for the Assessment of Neuropsychological Status (RBANS, https://www.pearsonassessments.com/ ), Rey Complex Figure Test (RCFT, https://www.parinc.com ), California Verbal Learning Test-II (CVLT-II, https://www.pearsonassessments.com/ ), Delis-Kaplan Executive Function System (D-KFES, https://www.pearsonassessments.com/ ); Symptom Checklist-90 (SCL-90, https://www.pearsonassessments.com/ ) and the Behavioral Rating Inventory of Executive Function (BRIEF, https://www.parinc.com ). Clinically, the 25-year-old presented with left side hemiparesis, emotional lability and major cognitive impairments, most notable in terms of memory and executive functioning. Family and caregivers were most concerned about the patient’s irritability and inappropriate outbursts along with impaired insight and judgment. Neuropsychological tests (lower z-scores = poorer function) demonstrated the expected left side reductions in motor control (reduced finger tapping and grip strength) consistent with the location of the large intraparenchymal right frontal hemorrhagic injury . He was anosmic and unable to identify basic odors on the Smell Identification Test ( https://sensonics.com/ ) along with diminished tactile discrimination on the left side, but no visual field defect. Constructional praxis was diminished as evident in the copy of the Rey Complex Figure Test (RCFT), with the more profound deficits most notable with impaired immediate as well as delayed memory. Memory and executive impairments were evident on the RBANS, CVLT-II and DKFES tasks. Caregiver observation, based on the BRIEF (higher z-scores = more problems) also confirmed real-world deficits in day-to-day impairments in planning, organization, decision making and problem solving. Emotionally, as also reflected in the BRIEF results, the family caregiver reported marked dysfunction in emotional regulation with poor self-monitoring and impaired insight. In contrast, on the SCL-90 (higher z-scores = more symptoms), which is a self-report measure, while somatic issues that related to mobility and pain were prominently endorsed, the Global Severity Index (GSI) was only minimally elevated, with no significant endorsement of symptoms related to depression or anxiety. This would be consistent with caregiver observations that the patient lacked insight into changes in personality and emotional control, impairments often reported to be present in TBI patients with extensive frontotemporal pathology , as evident in this patient
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32797398
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https://doi.org/10.1007/s11682-020-00313-7
|
[
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"https",
"pathology",
"test",
"lesion",
"time"
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[
{
"code": "ND56.Z",
"title": "Unspecified injury to unspecified part of trunk, limb or body region"
},
{
"code": "ND37",
"title": "Unspecified multiple injuries"
},
{
"code": "ND56.4",
"title": "Injury of nerve of unspecified body region"
},
{
"code": "NF0A.Z",
"title": "Early complications of trauma, not elsewhere classified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Unspecified injury to unspecified part of trunk, limb or body region (ND56.Z)】
Synonyms: Injury of unspecified body region | injury NOS | trauma NOS | traumatic injury NOS | body trauma
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injuries to unspecified part of trunk, limb or body region → Injury of unspecified body region (ND56) → Unspecified injury to unspecified part of trunk, limb or body region
【2. Unspecified multiple injuries (ND37)】
Synonyms: multiple trauma NOS | multiple traumatic injuries | multiple sites of injury | multiple system injury | multiple system trauma
Excludes: injury NOS
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injuries involving multiple body regions → Unspecified multiple injuries
【3. Injury of nerve of unspecified body region (ND56.4)】
Synonyms: injuries to nerves, nerve plexuses and roots | injury to nerves, unspecified site | nerve damage NOS | Injury of nerve NOS | Traumatic injury of nerves
Excludes: multiple injuries of nerves NOS
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injuries to unspecified part of trunk, limb or body region → Injury of unspecified body region (ND56) → Injury of nerve of unspecified body region
【4. Early complications of trauma, not elsewhere classified (NF0A.Z)】
Synonyms: Certain early complications of trauma, not elsewhere classified | early trauma complications | early complication of trauma | trauma complications
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Other or unspecified effects of external causes → Certain early complications of trauma, not elsewhere classified (NF0A) → Early complications of trauma, not elsewhere classified
|
ND56.Z
|
Unspecified injury to unspecified part of trunk, limb or body region
|
A 43-year-old woman who presented with a 2-month painful blurred vision of her left eye was referred to our uveitis clinic with hypopyon panuveitis in the left eye. Ocular examination revealed a visual acuity (VA) of 6/6 in the right eye and counting fingers at 1-ft in the left eye, intraocular pressure was 12 and 6 mmHg. Her left eye showed 4+ anterior chamber cells with 1.7 mm of hypopyon and a 2+ to 3+ flare with only mild conjunctival injection. There were multiple iris nodules, as shown in Fig. 1 . Fundus was obscured. The examination of the right eye was unremarkable. Ultrasonography of the left globe showed a heterogenous vitreous echogenicity, from which endogenous endophthalmitis was initially suspected. She was treated for bacterial endophthalmitis with intravitreal (IVT) vancomycin and ceftazidime injection, and fortified vancomycin and fortified ceftazidime eye drops. Systemic treatment included vancomycin 1-g intravenous infusion every 12 h and ceftazidime 1-g intravenous infusion every 8 h. Relevant investigations to identify the pathogen included vitreous gram stain, potassium hydroxide test, acid-fast bacilli stain, aerobic/anaerobic/mycobacterium culture, and polymerase chain reaction for bacteria/fungus/mycobacteria, which all returned negative results. Also, a hemoculture was performed and displayed no microbial growth. Whole abdominal ultrasound was performed and revealed only a few gallstones, a prominent size of the spleen, and possibly myoma. An echocardiogram showed no evidence of infective endocarditis. A Mantoux test, pathergy test, and anergy test were performed and all returned negative results. The chest radiograph was unremarkable. A plain-film radiograph of the paranasal sinuses showed maxillary sinusitis. Four days later, her clinical presentation worsened, and her VA was recorded as light projection (LP). Atypical mycobacterial infection was considered, for which an injection of IVT amikacin was given. Due to the patient’s worsening clinical status, a blood sample was taken and sent for analysis to identify the cause of the inflammation, as shown in Table 1 . Prednisolone at 1 mg/kg/day was given without clear supportive evidence of the inflammatory cause. However, the clinical outcome became even worse. Despite an improving VA from LP to hand motion (HM), her ocular examinations showed increasing hypopyon to 2.0 mm. Newly developed scleral thinning and melting were also found over the vitreous tapping and intravitreal injection sites (superonasally and superotemporally) with a prolapsed uveal tissue, as shown in Fig. 2 . During admission, an oral ulcer at the palatoglossal fold and tonsillar bed was found together with enlargement of the cervical lymph nodes. A cervical lymph node and oral ulcer biopsy was done by an otolaryngologist, with the pathological report revealing ENKTL, as shown in Fig. 3 . A computerized tomography scan of the chest, neck, and whole abdomen was done presuming ocular masquerade syndrome with NKTL. Topical and systemic antibiotics were then withdrawn, and a hematologist consultation was done. The patient underwent bone marrow biopsy and aspiration, which showed minimal marrow involvement by ENKTL with scattered Epstein–Barr virus positive cells. NKTL stage IV was diagnosed. Then, preparations were made for the patient to receive chemotherapy via a SMILE regimen [steroid (dexamethasone), methotrexate (MTX), ifosfamide, L-asparaginase, and etoposide]. After her first cycle of chemotherapy (CMT), it was found that her VA in the left eye was LP, there was no progression in the scleral melting, the hypopyon contracted, and the size and amount of iris nodules were decreased, as shown in Fig. 4 . Unfortunately, her left eye became phthisis with a complicated mature cataract, 9 months following the diagnosis. Regrettably, after her 5th cycle of CMT, or 2 years after the initial diagnosis, she developed septic shock and died. Fig. 1 Anterior segment photo of the left eye. The photo shows a subtle conjunctival injection, hypopyon, and multiple hypopigmented iris nodules Table 1 Patient blood investigations Value Reference range Unit Complete blood count Hemoglobin 11.6 12.0–14.9 g * /dl † Hematocrit 36.5 37.0–45.7 % White blood cell 5.17 × 10 3 4.4–10.3 × 10 3 /μl ‡ Neutrophil 59.2 40.4–73.1 % Lymphocyte 26.5 20.3–47.9 % Platelet count 262 × 10 3 179–435 × 10 3 /μl Erythrocyte sedimentation rate 27 0–20 mm/h § C-reactive protein 11.83 < 5.0 mg II /l ¶ Blood urea nitrogen 9.4 6–20 mg/dl Creatinine 0.53 0.51–0.95 mg/dl Aspartate aminotransferase 48 0–32 u ** /l Alanine aminotransferase 79 0–33 u/l Fasting blood sugar 88 74–99 mg/dl Venereal disease research laboratory non-reactive non-reactive Treponema pallidum hemagglutination assay non-reactive non-reactive Antinuclear antibody negative negative Rheumatoid factor 4.51 < 4.5: negative, 4.5 - ≤6: borderline, > 6: positive u/ml †† Interferon-gamma for tuberculosis negative negative Hepatitis profile Hepatitis B surface antigen negative negative Hepatitis C antibody negative negative HIV antibody test negative negative Toxoplasma IgG ‡‡ , IgM §§ negative negative Hemoculture 2 samples no growth no growth Mid-stream urine culture no growth no growth Stool exam no RBC/ WBC/ parasite Antibody to proteinase 3 negative negative Antibody to lactoferrin negative negative Antibody to myeloperoxidase negative negative Antibody to elastase negative negative Antibody to cathepsin G negative negative Antibody to bactericidal/permeability-increasing protein negative negative CD ¶¶ 4+ T cell CD 4+ T cell (%) 30.20 24.10–50.70 % CD 4+ T cell (Absolute count) 540 470–1404 cells/μl CD 8+ T cell CD 8+ T cell (%) 35.88 17.10–44.60 % CD 8+ T cell (Absolute count) 642 360–1250 cells/μl CD4/CD8 ratio 0.84 0.65–2.49 – * g gram, † dl deciliter, ‡ μl microliter, § mm/h millimeter/hour, II mg milligram, ¶ l liter, ** u = unit, †† ml = milliliter, ‡‡ IgG immunoglobulin G, §§ IgM immunoglobulin M, ¶¶ CD cluster of differentiation Fig. 2 Scleral thinning and prolapsed uveal tissue. Scleral thinning can be seen over the area of the vitreous tapping and intravitreal injection site. A poor-healing needle puncture wound of the sclera is shown adjacent to the slit beam Fig. 3 Histopathological reports for the lymph nodes and oral ulcer. Extranodal NK/T-cell lymphoma, nasal type. A ) Angioinvasion by pleomorphic lymphoid cells. Note one blood vessel in the center with infiltration by lymphoma cells. B ) Cluster of differentiation (CD) 3 positive lymphoma cells with typical cytoplasmic staining pattern. C ) Occasional CD30 positive lymphoma cells (membrane staining). D ) CD56 positive lymphoma cells (membrane staining). E ) T-cell intracellular antigen-1 positive lymphoma cells, indicative of cytotoxic granules in the cytoplasm of the lymphoma cells. F ) High proliferation index by Ki-67 in lymphoma cells (nuclear staining). G ) Epstein-Barr virus-encoded small ribonucleic acid positive lymphoma cells by in situ hybridization (nuclear staining) Fig. 4 Subsided inflammation. Left eye demonstrating improving inflammation. There was residual fibrin and dispersed pigment over the anterior lens capsule without hypopyon. Posterior synechiae and a complicated cataract were found. Prominent scleral thinning could be obviously seen superonasally
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sec[1]/p[0]
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PMC8803407
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https://doi.org/10.1186/s12886-022-02277-2
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[
"cells",
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"antibody",
"lymphoma",
"blood",
"hypopyon",
"injection",
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] |
[
{
"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 CLASSIFICATION ═══
【1. Other specified clinical findings on examination of urine, without diagnosis (MF9Y)】
Synonyms: Methaemoglobinuria | Other and unspecified abnormal findings in urine | Calciuria | Cells and casts in urine | casts in urine
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings of the genitourinary system → Clinical findings on examination of urine, without diagnosis → Other specified clinical findings on examination of urine, without diagnosis
【2. Mucolipidosis (5C56.20)】
Synonyms: Mucolipidosis type 3 | Pseudo-Hurler polydystrophy | Pseudo-Hurler disease | Mucolipidosis type 2 | N-acetyl-glucosamine 1-phosphotransferase deficiency
Excludes: Sialidosis (mucolipidosis type 1)
Hierarchy: Inborn errors of metabolism → Lysosomal diseases (5C56) → Glycoproteinosis (5C56.2) → Mucolipidosis
【3. Sickle cell disease without crisis (3A51.1)】
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.
Synonyms: Hb-SS disease without crisis | HbSS without crisis | Sickle-cell anaemia without crisis | SCD - [sickle cell disease] | SCA - [sickle cell anaemia]
Hierarchy: Diseases of the blood or blood-forming organs (03) → Anaemias or other erythrocyte disorders → Sickle cell disorders or other haemoglobinopathies (3A51) → Sickle cell disease without crisis
【4. Primary anterior uveitis (9A96.3)】
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.
Synonyms: anterior chamber cell
Hierarchy: Disorders of the eyeball - anterior segment → Disorders of the anterior uvea → Anterior uveitis (9A96) → Primary anterior uveitis
【5. Acquired pure red cell aplasia, unspecified (3A61.Z)】
Synonyms: Acquired pure red cell aplasia | acquired red cell aplasia | red cell aplasia NOS | pure red cell aplastic anaemia | red cell aplastic anaemia
Hierarchy: Anaemias or other erythrocyte disorders → Pure red cell aplasia → Acquired pure red cell aplasia (3A61) → Acquired pure red cell aplasia, unspecified
|
MF9Y
|
Other specified clinical findings on examination of urine, without diagnosis
|
A 4½-year old girl presented with an abdominal mass and a 6-week-history of weight loss, decreased appetite and subsequent development of fever and abdominal pain. Physical examination and vital signs revealed no further pathological findings except of pallor, a reduced nutritional status and a slightly elevated temperature. Abnormal laboratory parameters indicative of a tumorous or inflammatory process are presented in Table 1 . Urine catecholamines (homovanillic acid, vanillylmandelic acid) were not elevated. Abdominal magnetic resonance imaging (MRI) demonstrated an 8.8 × 7.9 × 9.4 cm retroperitoneal mass located at the superior pole of the left kidney with the tumor capsule passing into the renal capsule despite a non-infiltrative growth-pattern as shown in Fig. 1 a (coronal image; marked by triangle). No vascular encasement or calcification was seen, but the tumor extended into the left renal vein and the inferior vena cava . Except of enlargement of ipsilateral lymph nodes up to 9 mm no other tumor manifestations within the abdomen were seen. Chest X-ray and echocardiogram were normal, but chest computed tomography (CT) identified several bilateral pulmonary lesions strongly suggestive of metastases, the largest measuring 6 × 4 mm in the medial segment of the middle lobe . Further lesions were seen in the posterior segment of the right superior lobe and in the dorsobasal segment of the left inferior lobe. Both inferior lobe arteries showed evidence for an occlusion by either a thrombus or particles of the intracaval tumor cone . Meta-iodobenzylguanidine scan was negative as were bone scan and cranial MRI. Based on the imaging and laboratory findings the tumor was suspected to be a nephroblastoma and a biopsy omitted due to the presumed risk of tumor rupture. Preoperative chemotherapy over 6 weeks according to the SIOP2001/GPOH-protocol with weekly intravenous administration of vincristine (1.5 mg/m 2 ), actinomycine D (45 µg/m 2 ) and adriamycine (50 mg/m 2 ) was initiated, accompanied by continuous intravenous anticoagulation with unfractionated heparin (100 IU/kg/24 h) with respect to intravasal tumor manifestation. As abdominal MRI after 2 weeks of treatment displayed a constant renal tumor size but an expansion of the IVC thrombus the anticoagulation regime was changed to subcutaneous low-molecular-weight heparin twice daily (1 mg/kg/24 h, then escalated to 1.5 mg/kg/24 h aiming at factor anti-Xa-levels of 0.4–0.8 μmol/l). After 4 weeks of chemotherapy, MRI scans showed a slight regression of both the primary tumor mass and the IVC thrombus. After completion of 6 weeks of preoperative chemotherapy, the pulmonary metastases and the emboli in the inferior lobe arteries had regressed on chest CT. Radical tumor-nephrectomy, extraction of the caval thrombus and regional staging-lymphadenectomy were performed without major complications. Histopathology revealed clustering small tumor cells, partially arranged in pseudorosettes with focal calcifications, invading the left kidney. Immunohistochemistry was positive for synaptophysin, a specific marker of neuronal and neuroendocrine tumors and VMAT2, expressed in the CNS and sympathetic postganglionic neurons [Fig. 2 a: expression of synaptophysin (400×); Fig. 2 b: expression of VMAT (400×)]. PAN-cytokeratin and WT1, two major markers for nephroblastoma were negative as was desmin, a mesenchymal marker which can be positive in blastema-predominant nephroblastoma. These results further supported the diagnosis of undifferentiated neuroblastoma. The MYCN gene which is the most relevant genetic biomarker in neuroblastoma predicting poor prognosis was not amplified in this specimen. In order to substantiate the diagnosis gene-expression profiling and multiplex RT-PCR were done. Comparison of the gene expression profile using Affymetrix genechip human genome U133 plus 2.0 and artificial neural network (ANN) analysis of the patient’s sample with 36 tumor samples (12 rhabdomyosarcoma/RMS, 6 Ewing’s sarcoma/EWS, 14 neuroblastoma/NB and 4 Wilms’-Tumor/WT specimen) revealed that the patient sample (test as grey) was closely clustering with neuroblastoma . Hierarchical clustering of 10 samples (1 patient test sample, 3 RMSs, 3 NBs and 3 WTs) using a subset of 23 genes out of a previously established panel of 39 genes to distinguish small round blue cell tumors confirmed by multiplex RT-PCR showed that the patients’ sample was clustering with neuroblastoma tumors . A pseudocolored representation of z-scored log 2 ratio is shown . At that time the NSE had decreased to 20.2 μg/l (normal <17 μg/l), urine and serum catecholamines were still undetectable. Bone marrow aspiration and biopsy did not show any malignant cells. The following antineoplastic treatment comprised 6 cycles with alternating i.v. administration of either cisplatin/etoposide/vindesine or dacarbacine/ifosfamide/vincristine/adriamycin according to the GPOH NB2004 High Risk Trial Protocol. Intensification by high-dose, myeloablative chemotherapy with melphalan/carboplatin/etoposide and autologous stem cell rescue and a maintenance therapy with 13-cis retinoic acid p.o. over 10 months followed. Neither laboratory nor imaging investigations have been indicative of a tumor recurrence since and the child is well more than 4 years after completing treatment. Timeline summarizing patient management according to CARE Guidelines is provided in Additional file 2 . Table 1 Abnormal laboratory values at initial admission of the patient Test Result Reference range Units LDH 670 104–311 U/l Ferritin 146 12–60 ng/ml NSE 260.2 <17 µg/l C-reactive proteine 65 ≤5 mg/l D-dimers 422 ≤130 µg/l Hemoglobin 10.4 11.0–15.0 g/dl MCV 71 75–87 fl Fig. 1 MRI abdomen at initial diagnosis (T2-weighted images). a (Coronal). Left-sided abdominal mass ( triangle ) arising from the upper pole of the left kidney and tumor thrombus in the vena cava inferior ( arrow ). b (Transversal). Main tumor formation ( triangle ) and tumor cone in the left vena renalis ( arrow ) extending to the vena cava inferior ( asterisk ). Computed tomography scan of the lungs. c The arrows indicate the presence of lung metastases in the middle lobe ( reverse triangle ) and right inferior lobe close to the pulmonary fissure ( arrow ). d Embolic occlusion ( arrow ) of the left inferior lobe artery by either a thrombus or tumor Fig. 2 Immunohistochemistry of the primary tumor. a Expression of synaptophysin (×400). b Expression of VMAT (×400) Fig. 3 Gene expression profiling of the test patient tumor. a Loading plot of top three principal components of the 37 tumor samples (1 test patient sample, 12 RMS, 6 EWS, 14 NB and 4 WT) using 84 genes shows that the patient sample (test as grey ) is closely clustering with NB. Affymetrix genechip human genome U133 plus 2.0 genechip is used to generate the data. RMS is depicted as red circles , EWS as green , NB as blue and WT as yellow . b Hierarchical clustering of 10 samples (1 test patient sample, 3 RMSs, 3 NBs and 3 WTs) and a subset of 23 genes out of a panel of 39 genes known to be differentially expressed in SRBCT using multiplex RT-PCR assay data shows that the test sample is clustering with NB. Each row represents a sample, and each column a gene. A pseudocolored representation of z-scored log 2 ratio is shown
| 4.152344
| 0.958008
|
sec[1]/p[0]
|
en
| 0.999995
|
28818093
|
https://doi.org/10.1186/s13104-017-2724-4
|
[
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"lobe",
"test",
"clustering",
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"thrombus",
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[
{
"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 CLASSIFICATION ═══
【1. Neoplasms of unknown behaviour of unspecified site (2F9Z)】
Synonyms: neoplasia | neoplasm growth NOS | tumour of unspecified site | tumourlet of unspecified site | tumour mass NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of unspecified site
【2. Subcutaneous swelling, mass or lump of uncertain or unspecified nature (ME61)】
Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature
Synonyms: localised swelling, mass or lump of skin and subcutaneous tissue | localised subcutaneous nodules | subcutaneous nodules | superficial subcutaneous nodules | localised swelling
Excludes: localized adiposity | mass and lump: breast | enlarged lymph nodes | mass and lump: intra-abdominal or pelvic | oedema
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings involving the skin → Symptoms or signs involving the skin → Subcutaneous swelling, mass or lump of uncertain or unspecified nature
【3. Carcinoma in situ of unspecified site (2E6Z)】
Synonyms: carcinoma in situ of unspecified site | carcinoma in situ NOS | carcinoma in situ | in situ neoplasm
Hierarchy: Neoplasms (02) → In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues → Carcinoma in situ of unspecified site
【4. Neoplasms of unknown behaviour of trachea, bronchus or lung (2F91.1)】
Synonyms: 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 | Lung hemangiopericytoma of unknown behaviour
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of middle ear, respiratory or intrathoracic organs (2F91) → Neoplasms of unknown behaviour of trachea, bronchus or lung
【5. Neoplasms of unknown behaviour of skin (2F92)】
Synonyms: skin tumour NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of skin
|
2F9Z
|
Neoplasms of unknown behaviour of unspecified site
|
Managing soft tissue defects in the lower extremities, especially around the calcaneus and external malleolus, presents a substantial challenge to reconstructive surgeons. The case of a 20-year-old male, who sustained injuries from a motor vehicle accident leading to a 7 × 7 cm 2 soft tissue defect and Achilles tendon rupture, highlights the complexity of treating such injuries. Immediate debridement and soft-tissue coverage with a skin graft, followed by NPWT, initially seemed promising. However, the subsequent necrosis and infection necessitated further surgical intervention, employing the reverse sural flap technique for soft tissue reconstruction. The onset of lupus-like syndrome, diagnosed through various autoimmune markers, introduces an additional layer of complexity to this case. The autoimmune condition likely contributed to thrombosis and the flap’s necrosis, illustrating the critical role of systemic factors in flap viability and wound healing. This aspect emphasizes the need for comprehensive preoperative assessment and consideration of systemic conditions that may affect surgical outcomes. The prothrombotic pathophysiology of SLE significantly elevates the risk of thrombotic events, which can compromise the delicate blood supply to surgical flaps, leading to ischemia and tissue necrosis . Therefore, venous thromboembolism (VTE) prophylaxis is critical in optimizing surgical duration and non-pharmacological interventions like compression stockings . Effective post-operative management of thromboses, including low-molecular-weight heparin and nitroglycerin, is equally crucial . Robertson et al. found that topical nitroglycerin can reduce the incidence of mastectomy flap necrosis postoperatively . Systematic reviews by Boissiere et al. and Herlin et al. have highlighted the effectiveness of leech therapy as a viable strategy to reduce venous congestion in flaps . Odorico et al. and Shen et al. emphasize the importance of meticulous flap monitoring to prevent failure, underlining its significance in reconstructive surgery outcomes . Furthermore, Dang et al. have identified infrared thermography as a potentially valuable tool . However, they note that the scarcity of available evidence limits the reliability of their findings, suggesting a need for further research in this area. This underscores the multifaceted approach needed for patient safety and flap viability in plastic surgery. The distally based sural flap is the workhorse flap for the reconstruction of the lower leg, the ankle, and the foot. Another alternative reconstructive option includes a free flap. There is ongoing debate among reconstructive surgeons about whether the free flap is better than a sural flap. When considering the reconstructive ladder, reverse sural flap, as a locoregional flap, is preferable as a reconstructive option. The greatest advantage of a sural flap is its simplicity, allowing even non-plastic surgeons to perform it easily and rendering it advantageous in resource-poor centres . Tripathee et al. conducted a systematic review encompassing 2575 patients across 89 articles over 19 years, reporting an overall complication rate of 25.20%, with partial flap loss being the most common complication at 7.85% . This extensive pooled analysis underscores the relative reliability and highlights significant room for improvement in outcomes related to reverse sural flaps . An observational study by Clivatti GM et al. on nine reverse-flow fasciocutaneous sural flaps reported four cases with partial necrosis, indicating variability in success rates potentially due to patient-specific factors or surgical technique nuances . Cho ÁB et al.’s prospective cohort study on modified sural flaps with covered pedicles reported satisfactory outcomes without flap loss among their twenty cases, suggesting modifications can reduce complication rates compared to conventional techniques . Yammine K et al.’s systematic review specifically evaluated the effectiveness of reverse sural flaps in diabetic foot ulcers among 187 patients across 33 studies . They found a high healing rate but acknowledged that RSF might be less successful in diabetic wounds than trauma-induced ones due to inherent patient-related risk factors. Ciofu RN et al.’s work, Schmidt K et al.’s literature analysis, and Özkan HS’s study on RSA flaps in head and neck reconstruction, Chauhan VS’s examination into plaster burns treated by reverse sural flap, Grandjean A’s pediatric-focused study on distally based sural flaps for ankle and foot coverage each contribute unique insights into specific applications or modifications that could potentially influence outcomes . These depend on context-specific variables, such as the patient’s age, or underlying conditions like diabetes mellitus or peripheral vascular disease affecting wound healing capabilities . In 2003, Baumeister et al. clarified that the RSA flap yielded a higher rate of necrosis in high-risk, critically multimorbid, and older patients . Karacalar et al.’s standard bi-pedicled delay procedure enhanced flap perfusion by dilating the arterial network but did not demonstrate increased vascular neogenesis . We hypothesized that delaying the flap (the delay time ranged from 48 h to 2 weeks) and using a 4 cm wide pedicle would decrease the amount of partial flap necrosis that commonly occurs with this flap using the previously described techniques . Significant factors contributing to tissue necrosis, such as severe ischemia, are more prevalent in the lower extremity. This prevalence underscores an urgent need for effective treatments for chronic wounds in the leg, ankle, and heel regions. The collective body of research emphasizes several key points: the adaptability and utility of reverse sural flaps in various clinical contexts, a recurring theme that suggests technique modifications can increase flap viability and decrease the risk of complications, and the critical importance of thorough preoperative planning, especially in assessing patient comorbidities that greatly influence the success of the outcomes . Challenges persist despite its benefits, such as esthetic considerations at the donor site and the preservation of major vessels. Venous congestion often leads to partial or complete flap losses, necessitating further interventions . Through guided secondary healing processes, these interventions may be surgical, such as skin grafting, flap revisions, or conservative. These challenges highlight the necessity for technical expertise and comprehensive postoperative management strategies to ensure optimal recovery paths. The existing literature on reverse sural flaps establishes this method as a generally reliable approach for soft tissue reconstruction within certain anatomical areas . However, it also comes with notable risks of complications, primarily partial or complete flap losses, primarily due to venous congestion. Other issues include infection rates and unsightly scarring at the donor site, which may require further corrective actions. This underscores the ongoing need for surgical innovation, the adoption of modifications, and improved perioperative care protocols. Such efforts aim to reduce the incidence of adverse events and elevate the overall effectiveness of reconstructive surgery practices.
| 4.371094
| 0.738281
|
sec[2]/p[0]
|
en
| 0.999999
|
PMC11678630
|
https://doi.org/10.3390/medicina60122053
|
[
"flap",
"sural",
"flaps",
"necrosis",
"reverse",
"tissue",
"reconstructive",
"outcomes"
] |
[
{
"code": "EM0Y",
"title": "Other specified diseases of the skin"
},
{
"code": "EL51",
"title": "Cutaneous flap necrosis"
},
{
"code": "EL52",
"title": "Myocutaneous flap necrosis"
},
{
"code": "8C11.4",
"title": "Lesion of tibial nerve"
},
{
"code": "MC85",
"title": "Gangrene"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Other specified diseases of the skin (EM0Y)】
Synonyms: 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 | Postprocedural cutaneous complications of surgical procedures
Hierarchy: Diseases of the skin (14) → Other specified diseases of the skin
【2. Cutaneous flap necrosis (EL51)】
Definition: Necrosis of surgical skin flap
Synonyms: Cutaneous flap necrosis, partial | Cutaneous flap necrosis, total
Hierarchy: Diseases of the skin (14) → Postprocedural disorders of the skin → Cutaneous flap necrosis
【3. Myocutaneous flap necrosis (EL52)】
Definition: Necrosis of a surgical flap containing both skin and muscle
Synonyms: Myocutaneous flap necrosis, partial | Myocutaneous flap necrosis, total
Hierarchy: Diseases of the skin (14) → Postprocedural disorders of the skin → Myocutaneous flap necrosis
【4. Lesion of tibial nerve (8C11.4)】
Synonyms: Lesion of medial popliteal nerve | Lesion of sural nerve
Excludes: Injury of tibial nerve at lower leg level
Hierarchy: Disorders of nerve root, plexus or peripheral nerves → Mononeuropathy → Mononeuropathies of lower limb (8C11) → Lesion of tibial nerve
【5. Gangrene (MC85)】
Definition: Gangrene, not elsewhere classified is the death of tissues in the body which happens when a part of the body loses its blood supply.
Synonyms: gangrene NOS | dry gangrene | wet gangrene | ulcerative gangrene | tissue devitalization
Excludes: Pyoderma gangrenosum | Gas gangrene | Polymicrobial necrotising fasciitis
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings of the circulatory system → Symptoms or signs involving the circulatory system → Gangrene
|
EM0Y
|
Other specified diseases of the skin
|
A nonsmoker 60-year-old woman with a history of hiatal hernia with Barrett's esophagus and depression was admitted to the Emergency Department due to persistent fever during the last month. Blood tests, ultrasounds (US), and computed tomography (CT) imaging showed an 8 cm right mesorenal cancer with infiltration of the perirenal fat, urinary collecting system, and renal sinus. Moreover, the presence of floating thrombus in the right renal vein up to the inferior caval vein was detected, while the absence of a cleavage plane with the lower face of the liver was reported. Radical right nephrectomy and homolateral renal vein thrombectomy were performed after 15 days from diagnosis. Histological examination revealed a pT3a G4 N1 clear cell renal cancer with extensive pleomorphic and rhabdoid aspects. A week after surgery, the patient suffered from left pulmonary thromboembolism, which required anticoagulant therapy. An acute pain at the left arm and right forearm occurred two months after surgery with no history of relevant trauma. Pathologic fractures were found in both the diaphysis of the left humerus and the proximal metaphysis of the right radius. The patient showed pain (NRS score 8/10 ), swelling, and both upper limbs' complete limitation of movement plus systemic fatigue and poor appetite. No fever was detected. The peripheral blood erythrocyte count was below 3 × 10 12 /L, and hemoglobin was around 95 g/L. The Karnofsky score was 30. After hospital admission, the patient received parenteral nutrition, analgesics, and other symptomatic treatments. Oral anticoagulant therapy was suspended and replaced with subcutaneous low-molecular-weight heparin (LMWH) therapy. After a multidisciplinary team discussion, the patient was addressed for surgery. While undergoing the routine preoperative workups, a spontaneous diaphyseal fracture of the right humerus occurred. Preoperative radiographs demonstrated the fractures and wide lytic lesions involving the left humerus and the proximal right radius and confirmed the pathologic fracture of the right humerus. A CT scan of the thorax and abdomen was performed, and no secondary lesions were detected apart from the bone localizations. An additional CT scan of the upper limbs was performed to refine the surgical indication . Large osteolytic lesions with soft tissue invasion, involving the proximal left humerus and the proximal right radius were noticed. Our approach consisted of delivering a local adjuvant therapy on the lesions of the left humerus and right radius with electrochemotherapy using Cliniporator VITAE® technology (IGEA Spa; Carpi, MO, Italy). Thus, closed reduction and intramedullary fixation using a photodynamic polymeric stabilization system (IlluminOss® -IlluminOss Medical GmbH; Hilden, Germany) of both humeri was performed. The patient underwent general anesthesia and the entire procedure in a supine position. To treat both intraosseous and extraskeletal components of the lesions, electrochemotherapy was applied as follows: 4 and 7 single long (16 cm) needle VGD-1830T16 electrodes (variable geometry) with 1.8 mm in diameter and 3 cm active part were positioned around the right radial lesion and the left humeral lesion, respectively, and then the patient received a bolus of bleomycin (Bleomycin Nippon-Kayaku, Sanofi-Aventis, vials 15 mg, 15,000 UI/m 2 of body surface area). Eight minutes after the infusion, to allow the drug diffusion into the tumour tissues, electric pulses were applied (electroporation) according to the standard operating procedures. A single train of eight electric pulses of 100 µ s of duration at 1000 V/cm was generated by the Cliniporator Vitae® (IGEA spa Carpi). Afterwards, a small incision was performed anterolaterally to the acromion process of the left scapula, the deltoid muscle was split longitudinally to expose the subdeltoid bursa, and then the supraspinatus tendon was incised in line with its fibers. The fracture was reduced with the aid of an intramedullary guidewire under fluoroscopic guidance and the canal prepared with flexible cannulated reamers. Thus, the Dacron® balloon catheter was inserted, and the monomer was infused. The light source was finally used to activate the monomer, the tissues were closed in layers, and the correct position of the implant was checked by fluoroscopy. When the stability of the left humerus was reached, the contralateral fracture was internally stabilized with the same photodynamic bone stabilization system. After the operation, the right arm was immobilized with a long splint for 20 days, while arm slings were positioned bilaterally. Passive mobilization was prescribed from the first day following the operation, active elevation of the arms was restricted for the first 20 days, while lifting and strength-training were prohibited for a period of six weeks. The postoperative period was complicated by anemia, and five blood units were given to the patient. The pain was controlled with the administration of oral oxycodone/naloxone 5 mg/2.5 mg combination every 12 hours. After oncological examination, 24 days after surgery, a systemic treatment with pazopanib 400 mg once a day was prescribed, and the patient was discharged to an outpatient rehabilitative long-term care ward. Two months following the operation, a clinical follow-up reported that all surgical scars were well healed, and the range of motion was acceptable for low-impact daily activities. Anterior elevation and abduction of both shoulders were around 90°, internal rotation was limited to the buttock, while the elbows demonstrated full range of motion bilaterally. While slight pain (NRS score 3/10) was reported during the mobilization of the left shoulder, no pain was evocable by palpation of the treated bone lesions or at rest (NRS score 0/10). Nonetheless, the patient continued the same opioid dosage as at hospital discharge. MSTS score was 12/30 (40%) bilaterally, while DASH scores were 46.7 and 48.3 for the right and left side, respectively. Along with the clinical examination, radiographs were performed and showed no secondary mobilization of the implants, an initial callus formation, and new ossification processes. A radiographic partial response with a reduction of approximately 30% of the lesions' diameters was reported (RECIST criteria ). In conjunction with the oncologic team, the administration of pazopanib was confirmed, and a further clinical and radiological follow-up was scheduled at 6 months from surgery. At 4 months after surgery, the patient was contacted by telephone and reported substantial stability of the clinical scenario with well-controlled pain by mild opioid analgesics. At 6 months after surgery, a follow-up chest and abdomen CT exam detected lymph node infiltration of the caudate lobe of the liver and bone progression of disease. Furthermore, neck and supraclavicular lymph node metastases were reported. The oncologist suspended the treatment with pazopanib, and palliative care was initiated. The patient died within a month. This case report was approved by the United Ethical Committee of “AOU Città della Salute e della Scienza,” Turin, Italy, in accordance with the Declaration of Helsinki. The patient signed an informed consent form and authorized the use of data for research purposes.
| 4.019531
| 0.973633
|
sec[1]/p[0]
|
en
| 0.999999
|
33110432
|
https://doi.org/10.1155/2020/8408943
|
[
"surgery",
"humerus",
"lesions",
"pain",
"score",
"renal",
"therapy",
"radius"
] |
[
{
"code": "MD81.3",
"title": "Acute abdomen"
},
{
"code": "PL11.5",
"title": "Procedure undertaken at wrong site or wrong side, as mode of injury or harm"
},
{
"code": "QB8Z",
"title": "Contact with health services for specific surgical interventions, unspecified"
},
{
"code": "QB83",
"title": "Follow-up care involving plastic surgery"
},
{
"code": "NE8Z",
"title": "Injury or harm arising from surgical or medical care, not elsewhere classified, unspecified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Acute abdomen (MD81.3)】
Definition: A clinical syndrome with acute abdominal pain that is severe, and rapid onset. Acute abdomen may be caused by a variety of disorders, injuries, or diseases
Synonyms: acute abdominal pain syndrome | surgical abdomen | abdominal acute syndrome | severe abdomen pain | severe abdominal pain
Hierarchy: Symptoms, signs or clinical findings of the digestive system or abdomen → Symptoms or signs involving the digestive system or abdomen → Abdominal or pelvic pain (MD81) → Acute abdomen
【2. Procedure undertaken at wrong site or wrong side, as mode of injury or harm (PL11.5)】
Synonyms: 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
Hierarchy: External causes of morbidity or mortality (23) → Causes of healthcare related harm or injury → Mode of injury or harm associated with a surgical or other medical procedure (PL11) → Procedure undertaken at wrong site or wrong side, as mode of injury or harm
【3. Contact with health services for specific surgical interventions, unspecified (QB8Z)】
Synonyms: persons encountering health services for specific procedures and health care | elective surgical procedure | elective procedure | elective surgery
Hierarchy: Factors influencing health status or contact with health services (24) → Reasons for contact with the health services → Contact with health services for specific surgical interventions → Contact with health services for specific surgical interventions, unspecified
【4. Follow-up care involving plastic surgery (QB83)】
Synonyms: repair of scarred tissue | plastic and reconstructive surgery following healed injury or operation | reconstructive surgery | reconstruction procedure | surgical reconstruction
Hierarchy: Factors influencing health status or contact with health services (24) → Reasons for contact with the health services → Contact with health services for specific surgical interventions → Follow-up care involving plastic surgery
【5. Injury or harm arising from surgical or medical care, not elsewhere classified, unspecified (NE8Z)】
Synonyms: complication of medical procedure not elsewhere classified | Complication of surgery not elsewhere classified
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injury or harm arising from surgical or medical care, not elsewhere classified → Injury or harm arising from surgical or medical care, not elsewhere classified, unspecified
|
MD81.3
|
Acute abdomen
|
A 33-year-old woman with no significant medical history successfully underwent TICL (V4c TICL, STAAR Surgical, Switzerland) implantation in both eyes on separate days to correct her bilateral myopia with astigmatism. Preoperative best-corrected visual acuity (BCVA) was 20/20 in the right eye (OD) with −4.75 −1.25 × 180° and 16/20 in the left eye (OS) with −5.50 −2.25 × 5°. The TICL rotates 17° clockwise after horizontal implantation in the left eye. The left eye surgery was performed on the first day. When the patient left the hospital, the cornea was transparent, the anterior chamber reaction was mild, the aqueous humor was clear, and the IOP was 15 mmHg. On the morning of the second day, she felt slightly dizzy and bloated. However, it did not attract her attention owing to her good vision. Surgery on the second eye was scheduled for the following afternoon, and the first eye was reviewed at 1 p.m. According to the results of the reexamination of the left eye, the visual acuity was 20/20, and the IOP was 40 mmHg. Slit lamp examination showed that the cornea was transparent. Many pigments floated in the anterior chamber. The pupil was dilated to approximately 4–5 mm, and the pupillary light reaction was absent . The patient received IOP-lowering and anti-inflammatory treatment. The right eye was implanted with a TICL on the second day because the patient insisted on continuing the surgery. The sizes of the TICLs in both eyes were the same. She left the hospital after a 4-h postoperative observation. Her IOP was 20 mmHg in the right eye and 26 mmHg in the left eye when she left. Postoperative examinations were performed on the third day. The vision acuity of both eyes was 20/20. The IOP of the right eye was 15 mmHg, and the IOP of the left eye was 18 mmHg. The IOP of both eyes remained normal since then. The cornea of the left eye was transparent. The anterior chamber pigments were significantly reduced. The size of the pupil in the left eye was still 4.5 mm, and no pupillary light reflex was observed. The patient was instructed to take routine medication and was re-examined around a week after the operation. Six days later, the patient returned to the hospital because the pupil of her left eye had further enlarged. The slit-lamp examination showed that the pupil of her left eye had dilated to approximately 6 mm, appearing slightly elliptical in shape, with no light reflex . The vault volumes of both eyes were normal, approximately 1 cornea thickness (CT) (vault: the distance from the anterior surface of the lens to the posterior surface of the ICL). According to anterior segment optical coherence tomography (AS-OCT), the vault was 690 µm in the right eye and 650 µm in the left eye . The left eye’s measurement was taken after pupil contraction. The pupil of the left eye could contract to approximately 4 mm after applying pilocarpine eye drops. The vault of the left eye was approximately 1.2 CT after the contraction of the pupil. The patient was subsequently administered pilocarpine eye drops 3 times per day. However, on the ninth day post-operation, the examination indicated that the pupil of the left eye appeared more dilated. Slit-lamp examination revealed that the pupil of the left eye was fixed at approximately 7 mm. After four drops of pilocarpine, the pupil could constrict to a certain extent but was not smaller than 4.5 mm. On the 12th day after the operation, the vault in the left eye was measured by AS-OCT before the pupil constriction and was found to be 390 µm. During the examination, the oval-shaped dilation of the pupil was somewhat consistent with the placement of the TICL lens. In addition, the vault of the left eye increased after pupil contraction instead (contrary to common practice). Thus, it is suspected that the pupil dilation was linked to the oversized TICL, despite the vault being considered “normal.” The TICL needed to be replaced with a smaller one. The size of the original TICL was 12.6 mm horizontally and rotated 17° clockwise. The alternative smaller TICL was 12.1 mm or 12.6 mm vertically. The toric ICL should not be too small in case the vault becomes too low and the astigmatic position may be unstable after implantation. Therefore, a 12.6-mm vertical TICL was considered the most appropriate size. However, the 12.6 mm vertical /12.1 mm TICL needed to be prepared for 3–4 months owing to the COVID-19 epidemic. Moreover, the effect of 12.6 vertical/12.1 TICL replacement or even ICL removal was unclear. After fully communicating with the patient and obtaining the patient’s consent, the TICL lens in the patient’s left eye was realigned to the vertical position from the original position. There was no additional mydriasis used before the realignment surgery. In this way, it was possible to know in advance whether the size of the alternative TICL was appropriate. While the postoperative visual acuity of the left eye was 20/50, it did not bother the patient much as the left eye is non-dominant and the patient was longing for the recovery of the pupil. The pupil of the left eye did not dilate further after the realignment operation and gradually decreased and became round. A slight light reflex was observed in the left eye 2 weeks after the realignment operation. The pupillary light reflex of the left eye was normal around 1 month after the realignment operation. This indicates that the treatment was effective, so the replacement of TICL would be implemented. One month after the realignment surgery, an ultrasound biomicroscopy (UBM) examination was performed, and several small cysts were found at 3–5 o’clock in the left eye, which was not shown by the UBM before implantation. However, no further examination was conducted as it was unclear whether it was related to this case. The alternative TICL became available more than 2 months after the first operation. On the 75th day after the first implantation, the left eye underwent TICL replacement surgery, which changed the TICL from 12.6 mm horizontally to 12.6 mm vertically. The degree of rotation of the intraocular lens was 4° clockwise in the vertical orientation. The pupil dilated only once before the operation. Carbachol injection was used to constrict the pupil immediately after replacement surgery. After that operation, the left eye pupil gradually returned to normal. One month after the replacement surgery, the pupil measured approximately 5–6 mm in diameter, and the light reflex was normal. The vaults of both eyes were normal . Three months after the operation, the pupil completely recovered, and the light reflex was normal . The visual acuity of both eyes was 20/20. The IOP and vault were both normal. Fig. 1 One day after the left eye surgery, the pupil dilated and fixed, with more pigments in the anterior chamber Fig. 2 Six days after the left eye surgery, the pupil further dilated and fixed Fig. 3 Postoperative vault in both eyes 1 week after surgery (measured by anterior segment optical coherence tomography, Zeiss); pilocarpine eye drops were applied in the left eye Fig. 4 Postoperative vault in both eyes 1 month after the replacement surgery (measured by anterior segment optical coherence tomography, TowardPi, Beijing) Fig. 5 Slit-lamp examination of left eye 3 months after replacement surgery
| 3.859375
| 0.980957
|
sec[1]/p[0]
|
en
| 0.999997
|
40355934
|
https://doi.org/10.1186/s13256-025-05255-6
|
[
"pupil",
"ticl",
"surgery",
"vault",
"operation",
"eyes",
"approximately",
"light"
] |
[
{
"code": "LA11.62",
"title": "Anomalies of pupillary function"
},
{
"code": "LA11.Y",
"title": "Other specified structural developmental anomalies of the anterior segment of eye"
},
{
"code": "9A9Y",
"title": "Other specified disorders of the anterior uvea"
},
{
"code": "9A92",
"title": "Persistent pupillary membranes"
},
{
"code": "9B0Z",
"title": "Functional disorders of the pupil, unspecified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Anomalies of pupillary function (LA11.62)】
Definition: This is a group of conditions associated with pupillary function which is to regulate the amount of light that enters the eye controlled by the muscular structures of the iris.
Synonyms: abnormal pupillary function | anomaly of pupil function | anomaly of pupil reaction | disorder of pupil | pupillary disorder
Hierarchy: Structural developmental anomalies of the eye, eyelid or lacrimal apparatus → Structural developmental anomalies of the anterior segment of eye (LA11) → Structural disorders of the pupil (LA11.6) → Anomalies of pupillary function
【2. Other specified structural developmental anomalies of the anterior segment of eye (LA11.Y)】
Synonyms: Structural developmental anomalies of the pupil | Corectopia | Polycoria | multiple pupil | Microcoria
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the eye, eyelid or lacrimal apparatus → Structural developmental anomalies of the anterior segment of eye (LA11) → Other specified structural developmental anomalies of the anterior segment of eye
【3. Other specified disorders of the anterior uvea (9A9Y)】
Synonyms: Bilateral diffuse uveal melanocytic proliferation, paraneoplastic | Congenital anisocoria | Ectopia pupillae | ectopia of pupil | ectopic pupil
Hierarchy: Diseases of the visual system (09) → Disorders of the eyeball - anterior segment → Disorders of the anterior uvea → Other specified disorders of the anterior uvea
【4. Persistent pupillary membranes (9A92)】
Synonyms: PPM - [persistent pupillary membranes] | Cyclitic membrane | Iris bombé | Iris bombe | Pupillary occlusion
Hierarchy: Diseases of the visual system (09) → Disorders of the eyeball - anterior segment → Disorders of the anterior uvea → Persistent pupillary membranes
【5. Functional disorders of the pupil, unspecified (9B0Z)】
Hierarchy: Diseases of the visual system (09) → Disorders of the eyeball - anterior segment → Functional disorders of the pupil → Functional disorders of the pupil, unspecified
|
LA11.62
|
Anomalies of pupillary function
|
A ten-year-old girl with a history of cystic fibrosis (CF) with gastrointestinal and pulmonary involvement, presented to her pediatrician with newly diagnosed growth retardation, fatigue and frequent headache. An arginine growth hormone-releasing hormone test confirmed growth hormone (GH) deficiency, and after further laboratory analysis, a central hypothyroidism and hypercortisolism was diagnosed. Substitutional therapy with levothyroxine and hydrocortisone was initiated, which improved the patient’s headache and fatigue. Ophthalmologic examination revealed bitemporal hemianopia. Further work-up with magnetic resonance imaging (MRI) revealed a 3 × 3 × 2.5 cm cystic space-occupying lesion in the sellar and suprasellar compartment with compression of the optic chiasm, with partial calcifications in computed tomography (CT), highly suspicious of a craniopharyngioma (CP) . Hydrocephalus was not present at the time of presentation. Due to the compression of the chiasm causing clinical hemianopia, the indication for surgical decompression was given. We weighed the possibilities of a neuroendoscopic transventricular (NET) cyst fenestration and partial tumor resection versus an endonasal endoscopic approach (EEA) or an open transcranial approach. The advantage for the transventricular neuroendoscopic approach is its minimal-invasive nature and straight-forward decompression of the cyst, which is causing compression of the optic chiasm. However, the patient presented with very small ventricles, making neuroendoscopy cumbersome, and the neuroendoscopic approach would only allow a partial tumor resection. EEA shares the advantage of being minimal-invasive. However, due to her age and the concomitant diagnosis of CF, known to be associated with hypoplasia and markedly reduced pneumatization of the paranasal sinuses, a non-pneumatized (conchal type) sphenoid was present , making the AAE more challenging . In addition, gross total resection (GTR) was not considered achievable through an EEA, due to a supra-chiasmatic tumor extension. Still, both an NET or EEA seemed superior to an open approach (e.g. subfrontal or interhemispheric) given the invasiveness and associated morbidity of such approaches, while similar to the EEA and NET approach, GTR would most probably not be achieved either [ 22 – 26 ]. We ultimately decided to perform an EEA together with our colleagues from ENT. The conchal configuration of the sphenoid sinus required meticulous drilling of squamous intrasphenoidal bone, exposure of the harder sellar bone, and a superior trans-chiasmatic sulcus extension to achieve satisfactory exposure of the suprasellar tumor cyst . Intraoperatively, crystals and cystic fluid, suspicious of CP, were drained from the cyst, and the cyst was dissected from the cavernous sinus walls, the sellar diaphragm, and the dorsum sellae without risking injury of adjacent structures. At the end of the operation, a symmetrical diaphragmal descent was achieved as indirect sign for the decompression of the optic chiasm . Postoperative MRI showed the expected near total tumor removal. While the cyst was completely drained, tumor remnants extending posteriorly to the superiorly displaced chiasm remained as expected . Postoperatively, the patient developed diabetes insipidus (DI) for which she received desmopressin under the supervision of the pediatric endocrinologists. During her inpatient stay, she recovered from her DI with stable sodium levels but required vasopressin substitution. Overall recovery was good, while the hemianopia persisted. No signs of rhinorrhea resulting from cerebrospinal fluid (CSF) fistula were noted. We were able to discharge the patient to her home 11 days after surgery. Unexpectedly, the histopathologic analysis found conspicuous areas of skin with formation of hairs and squamous epithelia, compatible with a mature teratoma . The cytokeratin staining was positive for epithelial cells consistent with the finding in a mature teratoma . These findings led to the diagnosis of a rare case of infantile mature teratoma originating from the sellar region After discussion in our interdisciplinary pediatric neuro-tumor-board, no further treatments (e.g. chemotherapy, radiation therapy) were indicated and a clinical and radiological follow up was initiated. Fig. 1 A Coronal T1-weighted magnetic resonance imaging (MRI). B Sagittal soft tissue-weighted computed tomography (CT). C Sagittal T1-weighted MRI demonstrating a 3 × 3 × 2.5 cm cystic space-occupying partially calcified lesion in the sellar and suprasellar compartment with compression of the optic chiasm, highly susceptive of a craniopharyngioma (CP). D Axial bone-weighted CT demonstrating a non-pneumatized sphenoid bone with a hypo intense bone-marrow ( asterisk ) Fig. 2 Endonasal endoscopic approach in a conchal type sphenoid sinus for a sellar and suprasellar cystic lesion. A Binostril approach and exposure of the rostrum sphenoidale [RS]. B Removal of the spongious and fatty sphenoidal bone marrow [M]. C Change of the color and consistency indicates exposition of the harder sellar bone [S]. D Trans-sellar exposure of the sellar dura [D]. E Trans-chiasmatic sulcus superior extension. F Complete osteodural trans-sellar trans-chiasmatic sulcus exposure prior dural opening Fig. 3 Endonasal endoscopic approach in a conchal type sphenoid sinus for a sellar and suprasellar cystic lesion. A Extrusion of crystals [*] and brownish “oily” cystic fluid. B Close-up inspection of the cystic content reveals solid component consisting of crystals [*] and debris. C Dissection of the suprasellar tumor cyst from the partially descending sellar diaphragm [D]. D Dissection of the tumor cyst from the left medial cavernous sinus wall [CS]. E Incomplete diaphragmal [D] descent indicating remaining suprasellar tumor components. Dissection from the left medial cavernous sinus wall [lCS] and the dorsum sellae [DS] was possible, while the cyst was very adherent to the right medial cavernous sinus wall [rCS]. F Symmetrical diaphragmal [D] descent at the end of the surgery prior skull base defect closure Fig. 4 Radiological course. A Preoperative sagittal and B coronal T2-weighted MRI of the cystic sellar and suprasellar infantile mature teratoma. C Immediate postoperative sagittal and D coronal T2-weighted MRI demonstrating skull base defect reconstruction material within the sphenoid cavity and the sella turcica [*], along with a decompressed and anatomically located optic chiasm and remnant solid tumor supero-posteriorly [**]. E 1-year postoperative sagittal and F coronal T2-weighted MRI demonstrating resorption of the intrasellar reconstruction material along with volumetric regression and descent of the solid tumor remnant along the pituitary stalk into the dorsal aspect of the sella turcica. The optic nerves and chiasm descent into the sellar region and remains decompressed and anatomically preserved Fig. 5 Histopathology of the sellar tumor. A Hematoxylin-Eosin (HE) stain 10× magnified showing areas of skin formation, circle indicates the area which is B zoomed into with a magnification of 20x. C Pan-Cytokeratin (CK22) stain, 5× magnified, proving epithelial cell presence and circle indicates the area which is D zoomed into with a magnification of 20×
| 4.242188
| 0.939453
|
sec[1]/p[0]
|
en
| 0.999997
|
38276973
|
https://doi.org/10.1007/s00381-024-06296-w
|
[
"sellar",
"tumor",
"cystic",
"cyst",
"approach",
"suprasellar",
"chiasm",
"sinus"
] |
[
{
"code": "5A61.Y",
"title": "Other specified hypofunction or disorders of pituitary gland"
},
{
"code": "5A61.0",
"title": "Hypopituitarism"
},
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Other specified hypofunction or disorders of pituitary gland (5A61.Y)】
Synonyms: Prolactin deficiency | Isolated prolactin deficiency | Hypothalamic dysfunction, not elsewhere classified | dyspituitarism | hypophysis dysfunction
Hierarchy: Endocrine diseases → Disorders of the pituitary hormone system → Hypofunction or certain other specified disorders of pituitary gland (5A61) → Other specified hypofunction or disorders of pituitary gland
【2. Hypopituitarism (5A61.0)】
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.
Synonyms: subpituitarism | hypophyseal dystrophy | hypohypophysism | anterior pituitary insufficiency (in part) | deficient secretion of one or more pituitary hormones
Hierarchy: Endocrine diseases → Disorders of the pituitary hormone system → Hypofunction or certain other specified disorders of pituitary gland (5A61) → Hypopituitarism
【3. Neoplasms of unknown behaviour of unspecified site (2F9Z)】
Synonyms: neoplasia | neoplasm growth NOS | tumour of unspecified site | tumourlet of unspecified site | tumour mass NOS
Hierarchy: Neoplasms (02) → Neoplasms of unknown behaviour, except of lymphoid, haematopoietic, central nervous system or related tissues → Neoplasms of unknown behaviour of unspecified site
【4. Subcutaneous swelling, mass or lump of uncertain or unspecified nature (ME61)】
Definition: One or more localised subcutaneous soft tissue masses of undetermined or unspecified nature
Synonyms: localised swelling, mass or lump of skin and subcutaneous tissue | localised subcutaneous nodules | subcutaneous nodules | superficial subcutaneous nodules | localised swelling
Excludes: localized adiposity | mass and lump: breast | enlarged lymph nodes | mass and lump: intra-abdominal or pelvic | oedema
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings involving the skin → Symptoms or signs involving the skin → Subcutaneous swelling, mass or lump of uncertain or unspecified nature
【5. Carcinoma in situ of unspecified site (2E6Z)】
Synonyms: carcinoma in situ of unspecified site | carcinoma in situ NOS | carcinoma in situ | in situ neoplasm
Hierarchy: Neoplasms (02) → In situ neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues → Carcinoma in situ of unspecified site
|
5A61.Y
|
Other specified hypofunction or disorders of pituitary gland
|
On the day of admission, his blood pressure was 171/75 mmHg, heart rate was 67 bpm, SpO 2 97% at ambient room air, and body temperature was 36.6 °C. He denied abdominal pain, and pain or numbness in the lower extremities. General appearance was not in acute distress. There was no conjunctiva pallor or icterus. Respiratory sounds were clear to auscultation bilaterally and there were no wheezes or crackles. Cardiovascular examination revealed normal S1 and S2. There was no S3, S4, or murmurs. Abdominal examination revealed a flat and soft abdomen with audible bowel sounds. There was no bruit. There was no abdominal tenderness or hepatosplenomegaly. There was no spinal tenderness or costovertebral angle tenderness on percussion. There was no edema of his lower extremities. There was no joint swelling bilaterally at the wrists, ankles, and knees. General physical examinations revealed no abnormalities. His neurologic examination 2 to 12 were intact. There were no abnormalities with sensation and strength throughout with normal reflexes. Although laboratory analysis revealed normal results for complete blood count, electrolyte level, creatinine level, liver function, and coagulation test, levels of beta- d -glucan were slightly elevated at 24 pg/mL (reference value, < 20 pg/mL) (Table 1 ). Urinalysis was negative for proteinuria, pyuria, and hematuria (Table 1 ). Blood culture of aerobic and anaerobic bacteria including fungi and urine culture were all negative (Table 1 ). Transthoracic echocardiography revealed no valve vegetation, no valve regurgitation, no stenosis, and a normal ejection fraction. Computed tomography (CT) of the chest and abdomen revealed an irregularly shaped aortic aneurysm measuring 45 × 33 mm at the origin of the celiac artery and a partially expanded common hepatic artery with disproportionate fat stranding; no extravasation was observed using contrast enhancement . There was a high possibility that the aortic aneurysm was infected because it was at the site of the catheter that was inserted for the femoral artery via the common hepatic artery. The patient was diagnosed with impending rupture of acute thoracoabdominal aortic aneurysm and was admitted to the intensive care unit of our hospital. Graft replacement was performed for the thoracoabdominal aortic aneurysm, and the implanted catheter was removed during surgery and tested for culture. Pus was discharged from the aortic aneurysm wall incision and collected with swab for culture. The cultures of both the removed catheter and the pus of the aneurysm revealed Escherichia coli , Serratia marcescens , Eikenella corrodens , Streptococcus anginosus , α-Streptococcus , and Candida glabrata . The reported antimicrobial sensitivities of these organisms are shown in Table 2 . Antimicrobial susceptibilities were determined by the disk diffusion method, and the results were interpreted according to the Clinical and Laboratory Standards Institute (CLSI) guidelines. Results of pathology examination of the wall tissue of the aneurysm were compatible with those of the infected aneurysm cultures because the former showed infiltration of neutrophils mainly in the small blood vessels around the adventitia and infiltration of neutrophils, lymphocytes, and plasma cells in the media of the blood vessels . On the basis of these findings, a diagnosis of catheter-related thoracoabdominal infected aortic aneurysm was made. Table 1 Results of laboratory findings Complete blood count Biochemistry test Urinalysis White blood cell 7.6 10 3 /μL Total protein 7.8 g/dL Dipstick Neutrophils 69.1 % Albumin 3.6 g/dL Color Yellow Lymphocytes 22.0 % Aspartate aminotransferase 16.0 IU/L Specific gravity 1.024 Eosinophils 0.8 % Alanine aminotransaminase 12.0 IU/L pH 5.5 Basophils 0.8 % Total bilirubin 0.4 mg/dL Glucose Negative Monocytes 7.3 % Gamma-glutamyl transferase 20.0 IU/L Protein Negative Hemoglobin 12.3 g/dL Alkaline phosphatase 341.0 IU/L Bilirubin Negative Hematocrit 39.1 % Lactate dehydrogenase 216.0 IU/L Ketones + Platelets 40.5 10 4 /μL Urea nitrogen 12.4 mg/dL Hemoglobin Negative Creatinine 0.6 mg/dL Nitrate Negative Coagulation test Sodium 134.0 mEq/L Leukocyte esterase + Prothrombin time 85.7 % Potassium 4.3 mEq/L Microscopy exam International normalized ratio 1.1 Chloride 93.0 mEq/L Red blood cells 1–4 /HPF d -Dimer 1.5 μg/mL Calcium 9.3 mg/dL White blood cells 1–4 /HPF Fibrinogen 452.0 mg/dL Phosphate 3.1 mg/dL Epithelial cells 1–4 /HPF Fibrin degradation products 3.9 μg/mL Creatine kinase 92.0 IU/L Casts 1–4 /HPF C-reactive protein 1.6 mg/dL Crystals Negative Procalcitonin 0.1 ng/mL Beta- d -glucan 24.0 pg/mL Interferon-gamma release assays Negative Cultures Triglyceride 91.0 mg/dL Blood of aerobic Negative Total cholesterol 108.0 mg/dL Blood of anaerobic Negative LDL-cholesterol 54.0 mg/dL Urine Negative HDL-cholesterol 41.0 mg/dL Removed implanted catheter * HbA1c 5.9 % Aneurysm pus * HBs antigen Negative HCV antibody Negative HIV antigen/antibody Negative *Refer to Table 2 Fig. 1 Computed tomography of the chest and abdomen reveals the thoracoabdominal aortic aneurysm along with the implanted arterial catheter inserted from the left femoral artery to the hepatic artery ( a , b arrow). An irregularly shaped aortic aneurysm was identified at the origin of the celiac artery, with partially expanded common hepatic artery with disproportionate fat stranding ( c , d arrowhead) along the catheter ( c , d arrow); no extravasation was observed using contrast enhancement ( c , d ) Table 2 Result of antimicrobial susceptibility for causative pathogens from implanted catheter and aneurysm pus Antimicrobial agent E. coli S. marcescens S. anginosus α-Streptococcus C. glabrata MIC (μg/mL) Categorization MIC (μg/mL) Categorization MIC (μg/mL) Categorization MIC (μg/mL) Categorization MIC (μg/mL) Categorization Benzylpenicillin ≤ 0.06 S ≤ 0.06 S Ampicillin ≤ 2 S 8 R ≤ 0.25 S ≤ 0.25 S Piperacillin ≤ 4 S ≤ 4 S Amoxicillin/clavulanate ≤ 2 S 4 R Ampicillin/sulbactam S R Piperacillin/tazobactam S S Cefazolin ≤ 4 ≤ 4 R Cefaclor S R Cefmetazole ≤ 1 S 2 S Cefotiam ≤ 8 S ≤ 8 S Cefotaxime ≤ 1 S ≤ 1 S ≤ 0.12 S ≤ 0.12 S Ceftriaxone 0.5 S ≤ 0.06 S Ceftazidime ≤ 1 S ≤ 1 S Cefepime ≤ 1 S ≤ 1 S Cefditoren pivoxil S Cefpodoxime proxetil ≤ 0.25 S 1 S Imipenem/cilastatin ≤ 0.25 S ≤ 0.25 S Meropenem ≤ 0.25 S ≤ 0.25 S Doripenem S S Gentamicin ≤ 1 S ≤ 1 S Amikacin ≤ 2 S ≤ 2 S Minocycline ≤ 1 S 2 S Tetracycline 0.5 S 0.5 S Erythromycin ≤ 0.12 S ≤ 0.12 S Fosfomycin ≤16 S ≤ 16 S Sulfamethoxazole/trimethoprim ≤ 20 S ≤ 20 S Clindamycin ≤ 0.25 S ≤ 0.25 S Levofloxacin ≤ 0.12 S 1 S Ciprofloxacin ≤ 0.25 S ≤ 0.25 S Vancomycin 0.5 S 0.5 S Linezolid ≤ 2 S ≤ 2 S Fluconazole ≤ 0.12 S ≤ 0.12 S 0.5 S 8 S Amphotericin B ≤ 0.25 S Flucytosine ≤ 1 S Voriconazole 0.25 S Micafungin ≤ 0.06 S Caspofungin ≤ 0.25 S Eikenella corrodes were not used for this antimicrobial susceptibility test MIC minimum inhibitory concentration, S susceptible, R resistant Fig. 2 Histopathology examination of the aortic aneurysm wall confirmed an infected aortic aneurysm based on infiltration of neutrophils mainly in the small blood vessels around the adventitia and infiltration of neutrophils, lymphocytes, and plasma cells in the media of the blood vessels (hematoxylin and eosin staining)
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sec[1]/p[2]
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en
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33610163
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https://doi.org/10.1186/s13256-021-02661-4
|
[
"aneurysm",
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"aortic",
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[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Aneurysm and dissection of unspecified artery (BD51.Z)】
Synonyms: Arterial aneurysm or dissection, excluding aorta | cirsoid aneurysm NOS | false aneurysm NOS | ruptured aneurysm NOS | aneurysm of unspecified site
Hierarchy: Diseases of the circulatory system (11) → Diseases of arteries or arterioles → Arterial aneurysm or dissection, excluding aorta (BD51) → Aneurysm and dissection of unspecified artery
【2. Venous varicosities of other specified sites (BD75.Y)】
Synonyms: Caput medusae | Jugular venous aneurysm | jugular vein aneurysm | Orbital varices | orbit varix
Hierarchy: Diseases of the circulatory system (11) → Diseases of veins → Venous varicosities of sites other than lower extremity (BD75) → Venous varicosities of other specified sites
【3. Coronary artery aneurysm (BA81)】
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.
Synonyms: aneurysm of coronary vessels | aneurysmal lesion of coronary artery | arteriovenous aneurysm of coronary vessels | coronary aneurysm | atherosclerotic coronary artery aneurysm
Excludes: Congenital coronary arterial aneurysm | Mucocutaneous lymph node syndrome
Hierarchy: Diseases of the circulatory system (11) → Diseases of coronary artery → Coronary artery aneurysm
【4. Aneurysm of pulmonary artery, unspecified (BB02.1Z)】
Synonyms: Aneurysm of pulmonary artery | pulmonary artery aneurysm | PA - [pulmonary artery aneurysm] | pulmonary aneurysm
Hierarchy: Pulmonary heart disease or diseases of pulmonary circulation → Certain specified diseases of pulmonary vessels (BB02) → Aneurysm of pulmonary artery (BB02.1) → Aneurysm of pulmonary artery, unspecified
【5. Aneurysm or dissection of renal artery (BD51.4)】
Synonyms: aneurysm of renal artery | renal artery aneurysm | renal aneurysm
Hierarchy: Diseases of the circulatory system (11) → Diseases of arteries or arterioles → Arterial aneurysm or dissection, excluding aorta (BD51) → Aneurysm or dissection of renal artery
|
BD51.Z
|
Aneurysm and dissection of unspecified artery
|
A now 38-year-old Caucasian female was born as the second child from a non-consanguineous marriage. Her older sister was diagnosed with hypogammaglobulinemia and died of a severe respiratory infection at the age of 10 years. She has two younger sisters who were healthy and had normal serum Ig levels at the time when our patient’s diagnosis was established. Neither parent showed signs and symptoms of agammaglobulinemia so in the absence of genetic analysis at diagnosis, our patient was considered as an autosomal recessive agammaglobulinemia. From the age of four, she presented with recurrent bronchopneumonias requiring frequent hospital admissions, and recurrent middle ear infections which eventually resulted in hearing impairment. The patient was fully vaccinated including live Bacille Calmette-Guerin vaccine and live-attenuated oral polio vaccine without infectious complications. At the age of fifteen years, on the basis of severely decreased serum Ig levels, absence of circulating CD19 + B-cells, and lymphoid hypoplasia, the diagnosis of agammaglobulinemia was established and the patient started with Ig replacement therapy (IRT). One year later, an allogeneic hematopoietic stem cell transplant (HSCT) was performed from a phenotypically healthy sibling donor. Unfortunately, engraftment failure occurred, no donor DNA material was detected with chimerism testing, and extremely low Ig levels were recorded six months after HSCT [IgG 2.5 g/L, normal range (NR) by age 8.0-18.0 g/L), IgA 0.21 g/L (NR by age 0.9-4.5 g/L), IgM 0,14 g/L (NR by age 0.7-2.8 g/L)]. During the following seventeen years, the patient continued intravenous IRT with only occasional respiratory and intestinal infections which were successfully treated conservatively without sequelae. Due to abdominal pain, frequent diarrhea, and weight loss (body mass index 16,75 kg/m 2 ), an esophagogastroduodenoscopy and computed tomography (CT) enterography was performed at the age of thirty-three years . Histopathology reports of duodenum mucosa biopsy revealed intraepithelial lymphocytosis with complete villous atrophy (Marsh-Oberhuber type 3c/Corazza-Villanacci grade B2) suggesting celiac disease. Human leukocyte antigen (HLA) analysis revealed the DQ5(1), 9(3) haplotype. The patient was diagnosed with a celiac-like disease and was started on a gluten-free diet. The gluten-free diet was initiated not only as a therapeutical intervention but also for diagnostic reappraisal purposes which resulted in a partial regression of symptoms. Through the whole process of chronic care management, patient presented with persistent leukocytosis (leukocytes 20.26x10 9 /µl (NR 4-10x10 9 /µL, 76.6% neutrophils (NR 40-60%). Immunophenotype of peripheral lymphocytes showed no circulating CD19 + B lymphocytes (NR 80-490/µL), CD3 + CD4 + T lymphocytes 824 , CD3 + CD8 + T lymphocytes 1171 (NR 170-880/µl) and CD16 + CD56 + 82 (NR 80-690/µL). In August 2020, at the age of thirty-five years, the patient was diagnosed with coronavirus disease 2019 (COVID-19) pneumonia and treated with ABO-compatible donor convalescent plasma, favipiravir, hydroxychloroquine, and antibiotics according to the current national protocol at the time. Despite the clinical and laboratory recovery, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) detected by reverse transcription polymerase chain reaction (RT-PCR) analysis from nasopharyngeal swabs remained positive for the next four months. During the period of persistent SARS-CoV-2 positivity, significantly low serum IgG levels were repeatedly measured [with the lowest value of 1.8 g/L (NR 5.5-16.3 g/L)], despite regular IRT, pointing to possible reactivation of enteropathy [(total protein 43 g/L (NR 62-81 g/L), albumins 28 g/L (NR 35-53 g/L)]. Subcutaneous IRT was considered, but due to the patient’s preferences intravenous IRT was continued maintaining low serum IgG values. Soon after the patient developed bacterial meningitis with cerebrospinal fluid (CSF) findings: clear appearance, high cells, >90% polymorphonuclear cells, low glucose level 0.1 mmol/L (NR 2.77-4.44 mmol/L), and high protein level 1.97 g/L (NR 0.2-0.45 g/L). A blood and CSF culture were positive for E.coli and the patient was successfully treated according to the antibiogram without any neurological consequences. Following that, the patient was administered only a single dose of the Pfizer-BioNTech COVID-19 vaccine due to personal preferences. Eight months after the first COVID-19 episode, she was admitted to the hospital with fever, cough, anorexia, elevated inflammatory parameters, and bilateral pneumonia . At that moment, the RT-PCR for SARS-CoV-2 from the nasopharyngeal swab was negative, but a repeated sample from the induced sputum was positive so she was diagnosed with second episode of COVID-19. The treatment with remdesivir and antibiotics was initiated, and the patient recovered within two weeks. Six months later, she re-presented with a high fever and severe diarrheal syndrome. Blood cultures were positive for gram-negative anaerobic cocci and she was treated with moxifloxacin and metronidazole according to the antibiogram. Clinical symptoms improved, but laboratory findings indicating impaired liver function persisted [total protein 40 g/L (NR 62-81 g/L), albumins 21 g/L (NR 35-53 g/L), AST 158 U/L (NR 0-37 U/L), ALT 79 U/L (NR 0-41 U/L), ALP 504 U/L (NR 40-120 U/L), GGT 91 U/L (NR 0-38 U/L)]. Abdominal magnetic resonance imaging (MRI) and MRI cholangiopancreatography showed chronic inflammation of the gut, liver parenchyma and a hypoplastic spleen. Due to the recurring episodes of diarrhea, liver abnormalities, significantly high calprotectin levels [787 μg/g, (NR <50 μg/g)], and normal colonoscopy finding, small intestine bacterial overgrowth with subsequent bacterial translocation was suspected. Therapy consisted of rifaximin and norfloxacin along with regular IRT in a three-week intervals. The patient’s symptoms and laboratory findings improved rapidly, and she remained in good condition until the next clinical evaluation when she presented with severe diarrhoea. An extended microbiologic examination of the stool revealed positive RNA Norovirus GI/GII for the first time during the patient’s follow-up. The patient was treated with supportive therapy and continuous IRT according to current recommendations. A gastrointestinal multiplex PCR panel (Campylobacter ( C. jejuni/C. coli/C. upsaliensis ), Clostridioides (Clostridium) difficile (toxin A/B), Plesiomonas shigelloides, Salmonella, Yersinia enterocolitica, Vibrio (V. parahaemolyticus/V. vulnificus/V. cholerae), Vibrio cholerae, Enteroaggregative E. coli, Enteropathogenic E. coli, Enterotoxigenic E. coli lt/st, Shiga-like toxin-producing E. coli stx1/stx2, E. coli O157, Shigella/Enteroinvasive E. coli ; Cryptosporidium, Cyclospora cayetanensis, Entamoeba histolytica, Giardia lamblia; Adenovirus F40/41, Astrovirus, Norovirus GI/GII, Rotavirus A, Sapovirus (I, II, IV, and V) ( 8 ) also revealed Clostridium difficile (toxin A/B), and the patient was treated with oral vancomycin at a daily dose of 500 mg for 14 days. The patient responded well to therapy and her clinical condition was stable for a year until symptoms of neurocognitive deterioration appeared.
| 4.148438
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|
sec[1]/p[0]
|
en
| 0.999998
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38500873
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https://doi.org/10.3389/fimmu.2024.1324679
|
[
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[
{
"code": "2E92.40",
"title": "Polyposis syndrome"
},
{
"code": "DB3Y",
"title": "Other specified diseases of large intestine"
},
{
"code": "2B90.Y",
"title": "Other specified malignant neoplasms of colon"
},
{
"code": "GC08.0",
"title": "Urinary tract infection, site not specified, due to Escherichia coli"
},
{
"code": "GC00.1",
"title": "Infectious cystitis"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Polyposis syndrome (2E92.40)】
Definition: Intestinal polyposis syndromes can be divided, based on histology, into the broad categories of familial adenomatous polyposis (FAP), hamartomatous polyposis syndromes, and other rare polyposis syndromes, such as hereditary-mixed polyposis syndrome (HMPS).
Synonyms: adenomatous intestinal polyposis | polyposis coli | adenomatous polyposis coli | colon polyposis | APC - [adenomatous polyposis coli]
Hierarchy: Benign neoplasms except of mesenchymal origin → Benign neoplasm of digestive organs (2E92) → Benign neoplasm of the large intestine (2E92.4) → Polyposis syndrome
【2. Other specified diseases of large intestine (DB3Y)】
Synonyms: Melanosis coli | melanosis colon
Hierarchy: Diseases of the digestive system (13) → Diseases of large intestine → Other specified diseases of large intestine
【3. Other specified malignant neoplasms of colon (2B90.Y)】
Synonyms: Neuroendocrine neoplasm of colon | Colon endocrine neoplasm | Neuroendocrine carcinoma of colon | NEC - [neuroendocrine carcinoma] of colon | Large cell neuroendocrine carcinoma of colon
Hierarchy: Malignant neoplasms of intestine → Malignant neoplasms of large intestine → Malignant neoplasms of colon (2B90) → Other specified malignant neoplasms of colon
【4. Urinary tract infection, site not specified, due to Escherichia coli (GC08.0)】
Synonyms: E coli UTI | E coli urinary tract infection | Escherichia coli UTI
Hierarchy: Diseases of the urinary system → Certain specified diseases of urinary system → Urinary tract infection, site not specified (GC08) → Urinary tract infection, site not specified, due to Escherichia coli
【5. Infectious cystitis (GC00.1)】
Definition: Inflammation of the urinary bladder caused by microbes
Synonyms: inflammation of bladder | suppuration bladder | infective cystitis | infection of bladder | perivesical inflammation
Excludes: tuberculous cystitis (NHEA) | bladder disorder in schistosomiasis [bilharziasis] (NHEB)
Hierarchy: Diseases of the urinary system → Certain specified diseases of urinary system → Cystitis (GC00) → Infectious cystitis
|
2E92.40
|
Polyposis syndrome
|
A 21-year-old female patient without any known adverse medical background presented with a 1 month history of headache, nausea, fatigue and blurred vision. Physical examination and computer tomographic (CT) scan showed pericardial inflammation and splenomegaly (2 cm). Ophthalmoscopy of the right eye revealed papillary edema, retinal hemorrhages (Roth’s spots) and arteriovenous nickings . Initial laboratory evaluation of peripheral blood (PB) revealed a white blood cells (WBC) of 113.2 × 10 9 /l (72% were blasts), red blood cells (RBC) count was 2.53 × 10 6 /mm 3 , with a hemoglobin level of 9 g/dl and a platelet count (Plt) of 61 × 10 9 /l. Prothrombine time was 15.1 s (normal value 10.0–13.0 s) while partial thromboplastin time (PTT) was 25.8 s (normal value 29 ± 3.5 s). Creatinine value showed 38.7 μmol/l (normal 45–120) and uric acid value 498.2 μmol/l (normal 150–450). Bone marrow (BM) aspiration revealed 70% of blasts . Fig. 1 Summarizing scheme of disease progress Table 1 Clinical history of the patient together with diagnostic results and treatment Date Symptoms Analyses on BM sample Treatment and Outcomes 26 June 2016 - Headache, nausea, fatigue and blurred vision for 1 month ago. - Abdomen CT scan showed pericardial inflammation and splenomegaly (2 cm). - Ophthalmoscopy of the right eye revealed papillary edema, retinal hemorrhages (Roth’s spots) and arteriovenous nickings. -CT scan for brain was normal. -Peripheral blood (PB) showed: Leukocytosis (113.2 × 10 9 /l), anemia (9 g/dL), and thrombocytopenia (61 × 10 9 /L). -Bone marrow (BM) smear showed almost 70% of blats. - Prior to chemotherapy treatment GTG-banding cytogenetics revealed a karyotype 48–50,X,-X, der(1)t(1;2)(p35;p22),der(1)t(1;3)(p36.21;p26.2),der(2)(:1p36.21- > 1p35::2p22- > 2qter),+ 4,+ 4,+ 4,+ 6,der(8)t(8;11)(q24.3;q13.4),der(10)t(10;12)(p15.3;q24.11),del(10)(q21q21),dic(12;17)(p11.2;p11.2), del(15)(q14q14), del(15)(q21.1q21.1), del(15)(q22.32q24)del(17) (q12q12) - Molecular cytogenetic studies showed: confirmed the complex aberrations and a monoallelic loss of tumor suppressor gene TP53. - Flow cytometric (FCM) analysis of BM specimen prior to chemotherapy treatment characterized this case as AML-M1 according to WHO classifications. The abnormal cell population (60% in BM) was positive for CD45dim, CD34, HLADr, CD33, CD117, CD13 and CD11c. This cell population was negative for cyCD3, cyCD79a, CD14, CD64, CD32, CD7, CD19, CD10, and CD5. - The aCGH analysis revealed different genomic imbalances: deletion on 17q21.3; duplication of 3q26.1q29; and trisomy # 6). 26 June −02 July 2016 - (3 + 7) protocol: (Daunorubicin 60 mg/m 2 for 3 days and Cytarabine 200 mg/m 2 for 7 days) 10 Jul 2016 Peripheral blood (PB) showed cytopenia (WBC 0.4 × 10 9 /l), anemia (Hgb 9.5 g/dl); thrombocytopenia (Plt 12 × 10 9 /l). Serum creatinine value was 19.8 umol/l (normal 45–120) and serum total bilirubin value was 22.2 (normal value 2–21 umol/l), serum Ca + 2 value 2 (2.15–2.55 mmol/l), serum Na + value 132.3 (135–148 mmol/l). -BM smear showed almost 7% of blats. – – 26 Jul 2016 -Complete remission (CR) PB showed: WBC (6.1 × 10 9 /l), Hgb (11.7 g/dl); Plt (303 × 10 9 /l). -BM smear showed almost 5% of blats 46,XX /HeH 11 Aug-17 Aug 2017 Re-Induction: ICE cytrabin 200 mg/d Day1 ➔Day7 etobside 100 mg/d Day1➔Day5 idarubicin 20 mg/d Day1 ➔Day3 25 Sep 2016 -Blurred vision in the right eye (retinal detachment sensory serous). -CR -PB showed: WBC (7.4 × 10 9 /l), Hgb (11.6 g/dl); Plt (183 × 10 9 /l). -BM smear showed almost 4% of blats 46,XX 26 Sep-28 Sep 2017 Consolidation1 - HAM Cytrabin 3G/m 2 /d Day1➔Day3 Methoxantron 20 mg/d D1,D2 15 Nov 2016 Relapse. -Secondary treatment event: mass under vascular arch with splint edema of optical nerve of the right eye which causes to sever decrease of vision in the right eye. -BM smear showed almost 20–30% of blats. -Cerebrospinal fluid (CSF) was negative. PB showed: WBC (5.6 × 10 9 /l, with 98.5 of neutrophils), Hgb (11.6 g/dl); thrombocytopenia [Plt (70 × 10 9 /l)]. -Serum creatinine value was 39 umol/l (normal 45–120) and serum Ca + 2 value was 1.94 (2.15–2.55 mmol/l). -CT scan of brain was normal. – 17 Nov-19 Nov 2017 Consolidation2 - HAM Cytrabin 3G/d Day1➔Day3 Methoxantron 20 mg/d D1,D2 30 Nov 2016 PB showed: Cytopenia [WBC (0.1 × 10 9 /l)], anemia [Hgb (8.4 g/dl)]; thrombocytopenia [Plt (20 × 10 9 /l)]. Serum creatinine value was 33 umol/l (normal 45–120), serum serum K + value 2.89 (3.5–5.2 mmol/l), and serum Na + value 134.6 (135–148 mmol/l). – The mass behind the retina of the right eye was still present 03 Jan 2017 -Disappeared the previous Mass behind retina. -Relapse. PB showed: WBC (7.5 × 10 9 /l, with 77.7 of neutrophils), Hgb (12 g/dl); Plt (178 × 10 9 /l). -BM smear showed almost 15% of blats - Post to chemotherapy treatment GTG-banding cytogenetics revealed a karyotype 92,XXXX /62,XX,+ 1,+ 4,+ 5,+ 5,+ 6, + 6, + 11, + 15, + 16, + 17, + 19, + 19, + 20, + 20,+ 21, + 22 /46,XX . FCM analysis of BM specimen post to chemotherapy treatment characterized this case as AML-M6 according to WHO classifications. The abnormal cell population (15%) was positive for CD45dim, CD36, HLADr, CD33, CD34, CD117, CD13, CD235a and MPO. Those blasts were negative for: CD10, CD19, CD20, CD22, CD5, CD7, CD2, CD3, CD16, CD56, CD1a, CD14, CD64, CD32, TdT, cyCD3 and cyCD79a. 05 Jan 2017 PB showed: WBC (3.5 × 10 9 /l, with 84.4 of neutrophils), anemia [Hgb (9.3 g/dl)]; thrombocytopenia [Plt (33 × 10 9 /l)]. 10 Jan 2017 The MD’s suggested Flag-Ida protocol. No Flag-Ida treatment available because the political situation in his country. She was given Cytrabin 100 mg per day 25 Jan 2017 -Blurred vision in the right eye (central retinal detachment serous). -PB showed: WBC 60 × 10 9 /l (70% of them were blats), Hgb 13.3 g/dl; thrombocytopenia Plt 13 × 10 9 /l. -Brain MRI was normal. – She was treated with: Cytrabin 1 g/d Day1➔day3 Etoposide 100 mg/d day1 ➔day3 Methoxantron 20 mg/d Day1 ➔ Day2 13 Feb 2017 PB showed: Cytopenia [WBC (0.5 × 10 9 /l)], anemia [Hgb (9.7 g/dl)]; thrombocytopenia [Plt (13 × 10 9 /l)]. Serum creatinine value was 34 umol/l (normal 45–120),serum serum K + value 2.92 (3.5–5.2 mmol/l), serum Na + value 134.9 (135–148 mmol/l), and serum total bilirubin value was 24.09 (normal value 2–21 umol/l). – 16 Feb 2017 PB showed: Cytopenia [WBC (1.5 × 10 9 /l, with 44% of them were basts)], anemia [Hgb (9.6 g/dl)]; thrombocytopenia [Plt (17 × 10 9 /l)]. Serum creatinine value was 34 umol/l (normal 45–120),serum serum K + value 2.57 (3.5–5.2 mmol/l), serum and Na + value 134.1 (135–148 mmol/l). 17 Mar 2017 -Her MD’s stooped her treatment depended on her request from 1 month. -Relapse. -BM smear showed almost 44% of blats. -PB showed: WBC (7.5 × 10 9 /l, with 77.7 of neutrophils), Hgb (12 g/dl); Plt (178 × 10 9 /l). - She suffered from fever more than 40 C° for more than 3 days, menorrhagia and blurred vision in the right eye. -Approximately 8.5 months after initial diagnosis she died due to unknown causes. -No autopsy was performed because she died in her house. Fig. 2 Bone marrow smears of an acute myeloid leukemia without maturation case showing numerous blasts with round nuclei, fine nuclear chromatin, and dark blue cytoplasm (Leishman stain, oil immersion × 100)
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|
sec[1]/p[0]
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en
| 0.999998
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30186609
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https://doi.org/10.1186/s12878-018-0114-3
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[
"value",
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"mmol",
"thrombocytopenia",
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[
{
"code": "MB26.6",
"title": "Overvalued ideas"
},
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Overvalued ideas (MB26.6)】
Definition: Unreasonable and sustained beliefs that are maintained with less than delusional intensity (i.e., the person is able to acknowledge the possibility that the belief may not be true). An alternative use of this term is to refer to conventional or plausible thoughts (e.g., religious concepts, political ideas, or excessively idealistic beliefs) that are held with such a level of intensity so that the ...
Excludes: Delusion | Grandiosity | Paranoid ideation | Referential thinking
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Mental or behavioural symptoms, signs or clinical findings → Symptoms or signs involving content of thought (MB26) → Overvalued ideas
【2. Other serum reactions (NE80.3)】
Synonyms: Allergic reaction to serum | serum allergy | Complications of vaccination, protein sickness | Protein sickness | transfusion reaction due to serum protein reaction
Excludes: serum hepatitis
Hierarchy: Injury, poisoning or certain other consequences of external causes (22) → Injury or harm arising from surgical or medical care, not elsewhere classified → Injury or harm arising following infusion, transfusion or therapeutic injection, not elsewhere classified (NE80) → Other serum reactions
【3. Other specified metabolic disorders (5D0Y)】
Synonyms: Disorders of plasma-protein metabolism, not elsewhere classified | abnormal protein transport | dysproteinaemia | Absence of albumin in blood | Analbuminaemia
Hierarchy: Endocrine, nutritional or metabolic diseases (05) → Metabolic disorders → Other metabolic disorders → Other specified metabolic disorders
【4. Hypercalcaemia (5B91.0)】
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 concentrati...
Synonyms: Calcium excess | elevated serum calcium | hypercalcaemic crisis | hypercalcaemic syndrome | Burnett syndrome
Hierarchy: Overweight, obesity or specific nutrient excesses → Certain specified nutrient excesses → Mineral excesses (5B91) → Hypercalcaemia
【5. Other specified anaphylaxis (4A84.Y)】
Synonyms: Latex-induced anaphylaxis | Anaphylaxis due to latex | Latex anaphylaxis | Anaphylactic shock due to serum | anaphylactic shock due to allergy to serum
Hierarchy: Diseases of the immune system (04) → Allergic or hypersensitivity conditions → Anaphylaxis (4A84) → Other specified anaphylaxis
|
MB26.6
|
Overvalued ideas
|
Vision loss in our patient was most probably due to infiltration of optic nerve by Cryptococcus causing optic neuritis and acute vision loss. This was supported by fact that her MRI showed enhancement of both optic nerves and presence of papillitis on ophthalmoscopy with cryptococcal infiltration in both ganglio-capsular regions. This also explains her poor recovery of vision despite of receiving steroids as noted in previous studies (Table 1 ). The general belief that pregnancy is a state of immune suppression may not hold true in all patients. Pregnancy, indeed, is a state of an altered immune status which may respond to different stimuli variably. Thus, in the background of pregnancy and a HIV positive state, this rare manifestation of Cryptococcus appears to have evolved . Table 1 A review of published cases of vision loss in Cryptococcal meningitis Reference HIV status Summary of case MRI Suggested cause of vision loss Outcome Ng et al. Positive A 39-year-old Chinese man presented with bitemporal headache, giddiness and vomiting over a period of 2 days. There was no Cryptococcus detected by Indian ink examination of the CSF but CSF culture grew Cryptococcus neoformans. The patient was treated with amphotericin, and then to fluconazole. Normal increased intracranial pressure irreversible blindness in both eyes Positive A 36-year-old Chinese man presented with subacute onset of severe headache and confusional state. CSF microscopy with Indian ink examination showed Cryptococcus. Eight days after starting IV amphotericin, he complained of bilateral blurring of vision and had a seizure. A repeat lumbar puncture showed raised opening CSF pressure of more than 40 cm and 20 mL of CSF was withdrawn. During follow up, concentric diminution of visual field in both eyes was recorded. A lumbo-peritoneal shunt was done. CT was normal Increased intracranial pressure Normal vision Mohan et al. Positive A 41-year-old male with a history of cryptococcal meningitis admitted with severe headache. On the 6th day, He had complete loss of hearing, vision loss as well as bilateral facial palsy and bilateral sixth nerve palsy. Bilateral papilledema was seen. CSF opening pressure was 460 mm H2O. Amphotericin and Flucytosine were given. Repeated lumbar punctures were done but pressure remained over 500 mm H2O. Ommaya reservoir placed. There was dramatic clinical improvement of the patient. Hydrocephalus Increased intracranial pressure Regained his vision partially Hong et al. Positive A 58-year-old man presented with acute vision loss. Patient had normal CSF opening pressure and fundus. He received antiretroviral and antifungal agents. Normal Possibly, direct fungal infiltration of the optic nerve, optic chiasm, or optic tracts Vision improved Milman et al. Positive 25-year-old patient developed headaches, seizures, altered mental status, and visual loss. Lumbar puncture showed markedly increased opening CSF pressure; cryptococcal organisms were identified by India ink preparation and in culture. Despite treatment with amphotericin and fluconazole, visual loss progressed. leptomeningeal and optic nerve enhancement without hydrocephalus increased intracranial pressure Improved with bilateral optic nerve sheath fenestration. Muslikhan et al. Negative A 17-year-old boy presented with blurring of vision in both eyes and diplopia for 3 weeks. Extraocular muscles movement showed bilateral sixth nerve palsies. Discs were hyperaemic and slightly elevated. Lumbar puncture revealed high opening pressure >300 mmH(2)O. CSF showed Cryptococcus neoformans . IV amphotericin and fluconazole were given. CT scan of the brain was normal high intracranial pressure His vision was improved to 6/6 in both eyes with recovery of peripheral visual field. De Socio et al. Positive A 32-year-old man with disseminated Cryptococcosis was being treated with antiretroviral therapy. On day 7 he had a unilateral vision loss. retrobulbar neuritis IRIS leading to optic nerve neuritis following anti-retroviral therapy At 3 months, vision was normal After starting ART and IV methyl-prednisolone Duggan and Walls Positive A 39-year-old man with AIDS presented with recent onset of headache, dizziness, and syncope. CSF showed C neoformans . Amphotericin and flucytosine were started. On day 2, the patient had several episodes of complete loss of vision bilaterally. Drainage of CSF to decrease ICP resulted in the immediate return of vision. External ventricular drain was placed and later optic nerve sheath fenestration. optic nerve edema or neuritis high intracranial pressure permanent vision loss Positive A 39-year-old African female with AIDS presented with headache, neck pain, and altered mental status of 3 days. CSF showed C neoformans . On hospital day 2, the patient complained of sudden complete loss of vision bilaterally. Retinal microvasculopathy was noted. Normal Retinal microvasculopathy Patient died Espino Barros Palau et al. Out of three cases one was HIV positive A 46-year-old male had bilateral optic atrophy. He had been well until 3 months prior when he experienced vision loss, headache, nausea, and fever. Normal Intracranial hypertension serial lumbar drain A 43-year-old female presented with 3 weeks of headache, horizontal diplopia, and bilateral vision loss. Patient had renal transplantation requiring immunosuppression in 2005. CSF opening pressure was markedly elevated. Amphotericin B and flucytosine were initiated. Diffuse leptomeningeal enhancement and punctate areas of enhancement in the pons and basal ganglia. A 35-year-old male presented with 1 month of bilateral progressive vision loss. Ophthalmoscopy showed peripapillary subretinal fluid extending into the macula bilaterally. Both optic discs had edema. CSF opening pressure was elevated. Amphotericin B and flucytosine were started. Multiple T2-hyperintense lesions throughout the cerebral hemispheres, brainstem, and cerebellum as well as with leptomeningeal enhancement. Portelinha et al. Negative A 52-year-old woman with headaches, vomiting and fatigue for 3 weeks. She was diagnosed with cryptococcal meningitis and treated with antifungal therapy. She also had decreased vision in the left eye, bilateral sixth nerve palsy and papilloedema. A ventriculo-peritoneal shunt was placed and methylprednisolone was started. Signs of intracranial hypertension as well as multiple parenchymal lesions and optic nerve sheath enhancement. high intracranial pressure and optic nerve fungal infiltration In spite of the control of intracranial pressure there was a decrease in vision in the right eye and deterioration of visual fields. Ghatalia et al. Positive A 34 year old man of vision loss in a patient with Cryptococcal meningitis and normal ICP. Abnormal circumferential enhancement within the bilateral optic nerve sheaths. IRIS leading to optic nerve neuritis Vision improved with corticosteroids Merkler et al. Negative A 38-year-old woman presented with bilateral vision loss. Empiric steroids resulted in improvement in visual acuity, while tapering steroids, she had visual loss again. Multiple areas of ill-defined enhancement in the optic chiasm and tracts. Invasion of the optic apparatus by Cryptococcus neoformans One year later, symptom free.
| 4.210938
| 0.905273
|
sec[2]/p[4]
|
en
| 0.999997
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27756232
|
https://doi.org/10.1186/s12879-016-1925-0
|
[
"vision",
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"nerve",
"intracranial",
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] |
[
{
"code": "9E1Z",
"title": "Diseases of the visual system, unspecified"
},
{
"code": "MC1Y",
"title": "Other specified symptoms or signs involving the visual system"
},
{
"code": "9D9Z",
"title": "Vision impairment, unspecified"
},
{
"code": "9D90.2",
"title": "Moderate vision impairment"
},
{
"code": "QA00.6Z",
"title": "Examination of eyes or vision, unspecified"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Diseases of the visual system, unspecified (9E1Z)】
Synonyms: eye diseases NOS | disorder of vision | visual disorder
Hierarchy: Diseases of the visual system (09) → Diseases of the visual system, unspecified
【2. Other specified symptoms or signs involving the visual system (MC1Y)】
Synonyms: Erythema of eyelid | Visual disturbances | disturbances of vision | difficulty seeing | defective vision
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Symptoms, signs or clinical findings of the visual system → Symptoms or signs involving the visual system → Other specified symptoms or signs involving the visual system
【3. Vision impairment, unspecified (9D9Z)】
Synonyms: sight impaired | blindness and low vision | impaired vision
Hierarchy: Diseases of the visual system (09) → Vision impairment → Vision impairment, unspecified
【4. Moderate vision impairment (9D90.2)】
Synonyms: low vision, both eyes | visual impairment category 2, in both eyes | Low vision | LW - [low vision]
Hierarchy: Diseases of the visual system (09) → Vision impairment → Vision impairment including blindness (9D90) → Moderate vision impairment
【5. Examination of eyes or vision, unspecified (QA00.6Z)】
Synonyms: Examination of eyes or vision | general eye examination | routine eye examination | vision examination | vision test
Hierarchy: Contact with health services for purposes of examination or investigation → General examination or investigation of persons without complaint or reported diagnosis (QA00) → Examination of eyes or vision (QA00.6) → Examination of eyes or vision, unspecified
|
9E1Z
|
Diseases of the visual system, unspecified
|
In September 2021, a 23-year-old female patient requested an orthodontic evaluation for possible therapy with clear aligners. The patient was not available to receive aligner treatments that included attachments. She considered them unaesthetic and uncomforTable on the basis of friends’ experiences. The patient residing many kilometers away from the dental clinic and very busy for work despite the pandemic did not want to give up an orthodontic treatment which, however, was limited as much as possible in discomfort, check-ups and unsightliness. Before satisfying her needs and expectations, the clinical situation was assessed and orthopantomography and teleradiography in lateral-lateral projection were requested. The patient, who had never previously undergone orthodontic therapy, was in permanent dentition, first molar class both right and left, first canine class on the right and second on the left side . There was contraction of the upper arch in both posterior lateral sectors (from first premolar to second premolar) and moderate crowding and contraction of the lower arch with lingual orientation and rotation of the second premolars on both sides. The right lower lateral incisor was lingually displaced. The left lower first molar presented a large restoration with underlying endodontic therapy visible on the orthopanoramic radiograph . Also, the second upper left molar showed the presence of a composite resin restoration. The right upper first molar showed mild enamel discoloration of no pathological significance. There was no caries or other injuries, except the need for professional scaling . From the cephalometric analysis performed using the ViewBox© Software Version 3.1.1.14 on the initial lateral teleradiography , the patient was mainly a hyperdivergent (SN to GoGn35,5°; SN to GoMe 34,4°) Class II malocclusion (ANB 4,6°), with upper and lower incisors pro-inclined (Interincisal Angle 107,8°; Upper Incisor to NA 28,5°; Lower Incisor to NB 39,2°). The double impression technique in silicone material (Elite HD+ Putty Soft Normal and Elite HD+ Super Light Body, Zhermack SpA, Badia, Italy) allowed to obtain virtual models and setup based on these therapeutic goals: lower crowding resolution, expansion of the posterior lateral sectors in both the dental arches, reduction of rotation of the rotated dental elements, reduction of the pro-inclination. The setup obtained indicated the need to subject the patient to the treatment with a succession of 20 upper and 20 lower aligners for a total of 10 movements per arch (for a total period of 5 months of treatment). The Sorridi® system uses two aligners in PET-G with different thicknesses defined as “soft” and “hard” for each programmed movement. The soft aligner has a thickness of 0.06 mm and is used first for a week. Then the hard thickness of 0.08 mm is used for another week until the next soft type aligner. In this way the two thicknesses are alternated weekly. For each movement there are two pairs of soft and hard aligners, respectively. The gingival margin design is not scalloped but straight beyond the gingival zenith above 2 mm . Before applying the first and second soft aligner (respectively in the first and third week of treatment), the programmed interproximal reduction (stripping) or IPR was carried out. In the upper dental arch, a stripping of 0.20 mm was performed mesially and distally to the first and second premolars. In the lower dental arch, a stripping of 0.10 mm was performed mesially and distally to the central incisors, of 0.20 mm mesially and distally to the canines, the first and second premolars and the first and second molars. The procedure was performed using a reciprocating contra-angle handpiece with its interproximal reduction set (Intensiv Swingle Professional Kit, WG-69 LT Ortho PROF, W&H Synea, Intensiv©, Montagnola, Switzerland). Starting from the fifth hard aligner, a pair of divots were pre-inserted to torque mesially the upper right canine in the V1-L1 conFiguration, i.e., on the facial and lingual distal surface of the tooth on the gingival. At the end of the treatment no further refinements were judged particularly urgent . Orthodontic retainers were applied. Post treatment radiographs were requested and cephalometric analysis was performed again too . The Interincisal Angle increased its value (110,7°) and the overjet and overbite values improved as well (3,5 and 3 mm respectively). Incisors pro-inclination decreased (Upper Incisor to NA 26,1°; Lower Incisor to NB 38,6°). On the Allineatori Sorridi® viewer, which is a free of charge module of the Maestro 3D Ortho Studio software (Maestro 3D©, AGE Solutions S.r.l., Pontedera- Pisa, Italy) released by the company to every dentist or orthodontist who carries out a treatment with their aligners, the superimposition of the virtual models between the initial and final impressions made it possible to evaluate the extent of the movements actually obtained . Furthermore, the following distances were measured before and after the orthodontic treatment: between the cusps mesio-buccal of the first upper molars; between the disto-palatal cusps of the same; between the mesio-lingual cusps of the lower first molars and between the disto-buccal cusps of the same (the distal cusp of the right first lower molar was scarcely detailed due to the conservative restoration); the distance between the cusp top of the upper canines . The intermolar and intercanine distances of the upper dental arch (respectively 46.92 mm, 39.46 mm and 33.87 mm) remained unchanged as expected from the virtual setup and reported in the detailed movements sheet . In the lower arch, on the other hand, the intermolar and intercanine distances have changed as expected by the virtual setup. The lower intercanine distance increase of 0.65 mm, the intermolar distances increase of 2.25 mm between the disto-buccal cusps and increased of 2.44 mm between the mesio-lingual cusps . The distances between the buccal cusps and between the palatal cusps of the first and second upper premolars also increased, with a millimeter difference of 0.58 and 2.76 for the distances between the buccal cusps of the first and second premolars, respectively, and a difference of 1.27 and 2.97 mm for the distances between the palatal cusps of first and second premolars . The important variation of these values must be attributed to the expected coronal-root torque movements and derotation. The lower premolars also underwent significant displacements with an important variation in the distances between the buccal and lingual cusps related to the correction of rotations and coronal-root torque of these elements. The distances between the buccal cusps of the lower first and second premolars increased by 7.49 and 1.31 mm, respectively, while the distances between the lingual cusps increased by 5.93 and 1.24 mm . The only pair of divots to guide the mesial torque pre-inserted on the hard aligners (from the fifth) in correspondgnce with the right upper canine did not affect the intercanine distance. On the lower anterior group, not only the vestibularization of the right lateral incisor is remarkable, but also the degree of intrusion between -0.26 mm and -0.11 mm .
| 4.046875
| 0.967773
|
sec[1]/p[0]
|
en
| 0.999996
|
PMC9233912
|
https://doi.org/10.4317/jced.59618
|
[
"cusps",
"distances",
"premolars",
"arch",
"buccal",
"aligners",
"dental",
"lingual"
] |
[
{
"code": "LA30.4",
"title": "Abnormalities of size or form of teeth"
},
{
"code": "LA87.11",
"title": "Congenital mitral valvar stenosis"
},
{
"code": "LA87.01",
"title": "Congenital tricuspid valvar stenosis"
},
{
"code": "LA8A.0Y",
"title": "Other specified congenital anomaly of pulmonary valve"
},
{
"code": "LA8A.2Y",
"title": "Other specified congenital anomaly of aortic valve"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Abnormalities of size or form of teeth (LA30.4)】
Definition: A group of conditions characterised by abnormal size and form of teeth.
Synonyms: Microdontia | Macrodontia | Fused mandibular incisors | synodontia | Fusion of teeth
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the face, mouth or teeth → Structural developmental anomalies of teeth and periodontal tissues (LA30) → Abnormalities of size or form of teeth
【2. Congenital mitral valvar stenosis (LA87.11)】
Definition: A congenital cardiovascular malformation of the mitral valve in which there is narrowing or stricture of the valvar orifice (obstruction to flow).
Synonyms: Duroziez disease | congenital mitral stenosis | congenital stenosis of mitral valve | congenital mitral valve stricture | congenital mitral valve obstruction
Hierarchy: Structural developmental anomaly of heart or great vessels → Congenital anomaly of an atrioventricular valve or atrioventricular septum (LA87) → Congenital anomaly of mitral valve (LA87.1) → Congenital mitral valvar stenosis
【3. Congenital tricuspid valvar stenosis (LA87.01)】
Definition: A congenital cardiovascular malformation of the tricuspid valve in which there is narrowing or stricture (obstruction to flow).
Synonyms: congenital stenosis of tricuspid valve | congenital tricuspid stenosis | congenital tricuspid valve stricture | tricuspid cusps fusion
Hierarchy: Structural developmental anomaly of heart or great vessels → Congenital anomaly of an atrioventricular valve or atrioventricular septum (LA87) → Congenital anomaly of tricuspid valve (LA87.0) → Congenital tricuspid valvar stenosis
【4. Other specified congenital anomaly of pulmonary valve (LA8A.0Y)】
Synonyms: Pulmonary annular hypoplasia | Dysplasia of pulmonary valve | dysplastic pulmonary valve | pulmonary valve dysplasia | Pulmonary valve cusp dysplasia
Hierarchy: Structural developmental anomaly of heart or great vessels → Congenital anomaly of a ventriculo-arterial valve or adjacent regions (LA8A) → Congenital anomaly of pulmonary valve (LA8A.0) → Other specified congenital anomaly of pulmonary valve
【5. Other specified congenital anomaly of aortic valve (LA8A.2Y)】
Synonyms: Aortic annular hypoplasia | hypoplasia of the aortic annulus | Dysplasia of aortic valve | aortic valvar dysplasia | aortic valve cusp dysplasia
Hierarchy: Structural developmental anomaly of heart or great vessels → Congenital anomaly of a ventriculo-arterial valve or adjacent regions (LA8A) → Congenital anomaly of aortic valve (LA8A.2) → Other specified congenital anomaly of aortic valve
|
LA30.4
|
Abnormalities of size or form of teeth
|
We describe a 76-year-old woman presenting with a focal SE, primarily manifesting as aphasia, likely secondary to a left-sided dAVF. Notably, several of her electroencephalograms (EEGs) also demonstrated LPDs, defined as unilateral waveforms with relatively uniform morphology and duration, recurring at nearly regular intervals throughout the entire EEG . We searched online databases including MEDLINE and Google Scholar using the search terms “dural arteriovenous fistula” (or AVF) AND “status epilepticus”, “dural arteriovenous fistula” (or AVF) AND “lateralized periodic discharges” (or LPD) and “dural arteriovenous fistula” (or AVF) AND “periodic lateralized epileptiform discharges” (or PLED). Previously reported cases of dAVF with SE or LPDs or both are presented in Table 1 : there have been only a handful of cases reported of focal SE associated with dAVF [ 5 – 11 ], and of these, even fewer demonstrated LPDs on EEG . Table 1 Previously reported patients with dural arteriovenous fistulae and status epilepticus or lateralised periodic discharges Report, year Age, sex Seizure type Other neurological features EEG Imaging Angiographic findings Outcome Comment Current, 2023 76, F Focal SE: recurrent aphasia evolving to bilateral seizures Receptive aphasia, anomia, alexia, hemianopia, Gerstmann syndrome Focal SE with paroxysmal fast activity and LPDs recorded on multiple occasion CVST involving left TS/Sigmoid sinus; multiple parenchymal microbleeds, left temporal microbleed, venous infarction Left posterior fossa dAVF, underwent embolisation of left middle meningeal artery and left occipital feeders, very minimal residual shunting SE partially controlled after dAVF embolisation, needed ongoing treatment with 3 × ASMs Resolution of focal cortical deficits after dAVF embolisation and seizure control Dericioglu 2022 58, M Right-sided focal seizures of face and hand, evolving to generalised seizures Dysarthric, aphasic. Prior left hemiparesis and left focal seizures due to right parietal CVST 5 years prior Moderate background slowing, left fronto-central continuous periodic 1-Hz spike–wave discharges Restricted diffusion left frontal operculum, insula and perirolandic cortex; sequelae of right parietal venous infarct Cognard type 4 dAVF Restricted diffusion improved with AEDs to treat SE. Later EEG demonstrated loss of periodicity and only infrequent left frontal sharp transients Initial left focal seizures secondary to CVST, but SE from right focal seizures attributed to dAVF. Clinical improvement with ASMs alone, followed by embolisation of dAVF Quaranta 2021 82, M Jerking of right-sided limbs and face, confusion, aphasia Initial presentation with motor paraparesis and hyperreflexia, after SE developed progressive cognitive deterioration Slowing, lateralised sharp waves in left parieto-temporo-occipital regions T2/FLAIR hyperintensities left parietoinsular-occipital regions and left cerebellum, with mass effect from oedema dAVF left TS. Left IJV occluded Sudden haemodynamic worsening during procedure led to abandonment. Left ICH 7d later caused death Spinal and cerebral involvement suggests a tentorial dAVF. Venous congestion and thrombosis led to SE, and venous hypertension implicated in ICH Tamura 2020 68, M Recurrent intractable seizures – – Left basifrontal and basitemporal signal change on FLAIR and ASL dAVF left anterior cranial fossa draining towards the left mesial temporal lobe dAVF obliterated after cauterisation of draining vein, SE controlled with medication, resolution of FLAIR and ASL changes ASMs needed for seizure control after dAVF obliteration, resolution of imaging changes after successful treatment Derasse 2018 56, M Tonic–clonic, “complex-partial” seizures, evolving to refractory SE Known left carotid-cavernous fistula, after partial embolisation developed dysphagia, hiccup, diplopia, alternating hypoesthesia, right hemiparesis, progressive impairment of cognition “Diffuse epileptic activity of the left hemisphere” Following SE: chronic, extensive ischaemic changes in left parieto-occipital white matter Arterial feeders from left occipital artery, right TS/sigmoid sinus partially occluded. Later, thrombosis of right TS and posterior SSS, venous outflow via left TS and cortical collaterals Attempted dAVF embolisation produced venous congestion and refractory SE. Progressive occlusion of left TS, right IJV and left innominate veins. Extensive left-sided parenchymal signal change Clinical worsening attributed to venous ischaemia due to late occlusion of left TS after previous thrombosis of right TS and SSS Lee 2015 49, M Generalised convulsive SE Bilateral pseudoperiodic lateralised epileptiform discharges Cognard type 4 dAVF around SSS with narrowing of SSS lumen Balloon angioplasty of SSS followed by subtotal trans-arterial dAVF embolisation; seizures controlled with ASMs. Left focal SE two months later led to detection of restenosis of SSS and residual dAVF, treated with stenting of SSS and re-embolisation Seizures recurred after incomplete dAVF treatment, control achieved on two occasions after embolisation, with infrequent focal seizures thereafter Lee 2015 71, M Right hemiparesis, right focal seizures with generalisation LPDs in the left cerebral hemisphere Venous infarction, multiple intracerebral hematomas Cognard type 4 dAVF around left transverse-sigmoid sinus SE controlled with ASMs. dAVF occluded by trans-venous embolisation; no further seizures Complete cessation of seizures after treatment of dAVF Lee 2015 80, F Left hemiparesis, altered consciousness LPDs and episodic lateralised fast activity in the right hemisphere followed by background attenuation dAVF at right TS, with thrombosis of both transverse and sigmoid sinuses SE controlled with ASMs. No further seizures after trans-arterial embolisation of the dAVF and stenting of transverse-sigmoid sinuses Complete cessation of seizures after treatment of dAVF Moll 2015 45, F Focal seizures with generalisation, evolving to SE Known right carotid-cavernous fistula for 7 years, cognitive deterioration – Multiple parenchymal calcifications, dilated TS, CVST left parietal and right temporal. Increased vascular signal on contrast CT dAVF involving SSS, vertebral artery, cerebellar cortical veins and occipital artery. Right TS occluded distally with collateral flow producing a dilated right superior ophthalmic vein ASMs, refused embolisation or resection Yanagihara 2006 62, M SE, semiology not described further Previous stroke and presumed post-stroke epilepsy, new right hemiparesis – Widespread hyperintense signal in the left occipital, parietal temporal cortices Multiple dAVF associated with superior sagittal sinus; left transverse sinus thrombosis Cortical signal changes resolved after control of SE and dAVF embolisation Cytotoxic and vasogenic oedema due to venous hypertension from dAVF and SE may lead to reversible cortical signal changes ASL arterial spin labelling; ASMs anti-seizure medications; CVST cerebral venous sinus thrombosis; EEG electroencephalogram; FLAIR fluid-attenuated inversion recovery; IJV internal jugular vein; LPDs lateralised periodic discharges; SE status epilepticus; SSS superior sagittal sinus; TS transverse sinus
| 4.296875
| 0.882324
|
sec[2]/p[0]
|
en
| 0.999994
|
38240868
|
https://doi.org/10.1007/s10072-024-07325-x
|
[
"davf",
"seizures",
"focal",
"embolisation",
"venous",
"sinus",
"asms",
"lpds"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Type of seizure, unspecified (8A68.Z)】
Synonyms: Types of seizures | uncontrolled seizures | Seizure NOS | fits NOS | onset seizure NOS
Hierarchy: Diseases of the nervous system (08) → Epilepsy or seizures → Types of seizures (8A68) → Type of seizure, unspecified
【2. Epilepsy or seizures, unspecified (8A6Z)】
Synonyms: Cerebral seizures | Seizure disorder | seizure disorder, so described | epilepsy NOS | epileptiform attack
Hierarchy: Diseases of the nervous system (08) → Epilepsy or seizures → Epilepsy or seizures, unspecified
【3. Seizure due to other acute cause (8A63.Y)】
Synonyms: 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 | Seizures due to cerebrovascular diseases
Hierarchy: Diseases of the nervous system (08) → Epilepsy or seizures → Seizure due to acute causes (8A63) → Seizure due to other acute cause
【4. Acute repetitive seizures (8A67)】
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.
Synonyms: complex partial status epilepticus | Cluster seizures | Serial seizures | Recurrent seizures | Crescendo seizures
Hierarchy: Diseases of the nervous system (08) → Epilepsy or seizures → Acute repetitive seizures
【5. Other specified type of seizure (8A68.Y)】
Synonyms: Absence episode | Absence seizure episode | Pseudotetanus | Clonic seizure disorder
Hierarchy: Diseases of the nervous system (08) → Epilepsy or seizures → Types of seizures (8A68) → Other specified type of seizure
|
8A68.Z
|
Type of seizure, unspecified
|
ITP first manifested in the Caucasian male patient at the age of 11 years. He was admitted due to signs of bleeding and laboratory tests revealed severe isolated thrombocytopenia (platelet count 3 × 10 9 /L). Pertinent diagnostic data are provided in Table 1 . Except for elevated anti-nuclear antibodies (ANA 1:640, normal negative), no abnormalities were found, and a diagnosis of ITP was made. While repetitive treatments with IVIG (0.8 g/kg) and a short course of corticosteroids resulted in transiently increased platelet counts (> 100 × 10 9 /L), he failed, however, in achieving a stable remission. During each relapse, platelet count was < 10 × 10 9 /L with the presence of cutaneous and/or mucous bleedings. 17 months after diagnosis, treatment with eltrombopag was started at a dosage of 50 mg/day. An initial increase of the platelet count to 144 × 10 9 /L within 3 weeks led to subsequent dose reductions to a final dose of 25 mg/every second day, which resulted in stable platelet counts ranging between 77 × 10 9 /L and 128 × 10 9 /L (median 99 × 10 9 /L). Table 1 Diagnostic work-up and results for chronic immune thrombocytopenia (cITP) and autoimmune pancreatitis (AIP) Diagnostics Results (normal values) Chronic immune thrombocytopenia Humoral (auto-)immunity Anti-platelet antibodies Negative Nuclear antibodies : ANA, DNA, SM, RNP, RO, LA, SCL, JO, HIST, NUC, ACENA ANA 1:640 (negative), all others negative Antiphospholipid antibodies : CARG, CARA, B2GPG, B2GPA, B2GPM Negative Mannan binding lectin, complement analyses (C3, C4) Normal Immunoglobulins: IgG, IgM, IgA, IgG1, IgG2, IgG3, IgG4 Normal Cellular immunity (flow cytometry) T-cells : CD3, CD4/3, CD8/3, CD4/8, HLA-D3, CD25/3, Ta/b, Tg/d, 45RA/45R0, 45R0/45RA, 4/45RA, 4/45R0, 8/45RA, 8/45R0, 45RA/62L/8, 3/127, 3/132, 3/154, DNT T cells and subtypes: normal B-cells : CD19, IgD + /CD27 + , IgD-/CD27 + , CD21 B cells and subtypes: normal NK-cells : CD56/3 NK-cells: normal Lymphocyte function testing: CD3, SEA and TT induced proliferation Normal Bone marrow failure or myelodysplasia/leukemia Cytogenetics, diepoxybutan testing 46, XY, normal FLOW No leukemic cells detectable FISH-MDS (monosomy 7, trisomy 8, monosomy 5, deletion 5q, deletion 7q) No MDS typical chromosomal anomalies detectable Bone marrow analysis (Giemsa staining) Normal cellularity except for increased number of megakaryocytes, compatible with ITP Infection diseases Virus nucleic acid testing via PCR in bone marrow/plasma: CMV, PVB19, AdV A/B/C/D/E/F/G, EBV Negative Helicobacter pylori (PCR stool) Negative HIV serology Negative Hepatitis serology: anti-HAV IgG, anti-HAV IgM, HBsAg, anti-HBc IgG, anti-HBc IgM All negative except for anti-HAV IgG 4 IU/l (negative) and anti-HbSAg > 1000 IU/l Other autoimmune diseases Thyroid hormones (TSH, fT4) Normal Celiac disease serology Normal Autoimmune pancreatitis Pancreas function testing: serum or stool analyses Amylase 395 U/L (28–100 U/L), lipase 1262 U/L (7–39 U/L), HbA1c 5,4% (4–6%), OGTT normal, elastase < 50 µg/g stool Liver function testing: serum analyses GGT 773 U/L (< 52 U/L), ALT 453 U/L (0–31 U/L), AST 247 U/L (0–34 U/L), ALP 947 U/L (< 390 U/l), TBIL 3.1 mg/dl (0–1 mg/dl), BC 2,76 mg/dl (0–0.25 mg/dl), TP 58,7 g/L (60–80 g/L), PRALB 19 mg/dl (12–42 mg/dl) Tumor marker: Serum analyses CA 19–9 30.1 kU/L (0–27 kU/L), CEA 0.8 µg/L (0–3.8 µg/L), NSE 19.6 µg/L (0–16.3 µg/L) Immunology testing: immunoglobulins: IgG, IgM, IgA, IgG1, IgG2, IgG3, IgG4 IgG 548 mg/dl , all others normal Autoantibodies: ANA, DNA, NUC, ENA subsets (RO, LA, SCL-70, SM, RNP, Jo-1, centromer B, c-ANCA, p-ANCA, X-ANCA, smooth muscle, mitochondria, parietal cells, LKM, CARG, CARA, B2GPG, B2GPA, B2GPM, AMA-M2, SP-100, GP210, LC1, SLA ANA 1:160 (negative), all others negative Imaging: abdominal ultrasonography Slightly enlarged liver with normal tissue echogenicity, dilatation of the intra- and extra-hepatobiliary ducts, and a hypoechoic and enlarged pancreatic head Magnetic resonance cholangiopancreatography Abrupt termination of the dilated common bile and pancreatic ducts caused by a pancreatic ‘head mass’ Endoscopic ultrasound (EUS)-guided core biopsy-histopathology: HE and immunological staining Marked fibrosis, lymphoplasmacytic infiltration, and destruction of pancreatic ducts without an increased number of IgG4-positive plasma cells ACENA anti-centromere antibodies, AdV adenovirus, ALP alkaline phosphatase, ALT alanin-aminotransferase, AMA-M2 : anti-mitochondrial antibodies M2, ANA anti-nuclear antibodies, ANCA anti-neutrophil cytoplasmic antibodies, anti - HAV anti-hepatitis A virus antibodies, anti-HBc anti-hepatitis B core antigen antibodies, AST aspartate transaminase, BC conjugated bilirubin, B2GPA beta-2-glycoprotein-1 IgA antibodies, B2GPG beta-2-glycoprotein-1 IgG antibodies, B2GPM beta-2-glycoprotein-1 IgM antibodies, c-ANCA cytoplasmic ANCA, CARA : anti-cardiolipin IgA antibodies, CARG anti-cardiolipin IgG antibodies, CA19-9 cancer antigen 19–9, CD3 CD3 positive T-lymphocytes, CD4/3 CD4 positive T-cell subsets, CD4/8 CD4/CD8 ratio, CD8/3 CD8 positive T-cell subsets, CD19 B-lymphocytes, CD21 CD21 positive B-cell subsets, CD25/3 activated CD25 positive T-cell subsets, CD56/3 CD56 positive CD3 negative NK-cell subsets, CEA carcinoembryonic antigen, CMV cytomegalovirus, DNT TCR alpha/beta positive CD4 negative CD8 negative T-lymphocytes, DNA native/double-stranded deoxyribonucleic acid antibodies, EBV Epstein–Barr virus, ENA extractable nuclear antigens, FISH-MDS fluorescence in situ hybridization-myelodysplastic syndrome, FLOW flow cytometry, fT4 free thyroxine, GGT gamma-glutamyl transpeptidase, GP210 anti-glycoprotein-210 antibodies, HbA1c hemoglobin A1c, HBsAg hepatitis B surface antigen, HE hematoxylin and eosin, HLA-D3 activated HLA-D positive T-cell subsets, HIST anti-histone antibodies, HIV human immunodeficiency virus, IgD+/CD27+ IgD positive CD27 positive memory B-cell subsets, IgD-/CD27+ IgD negative CD27 positive memory B-cell subsets, JO anti Jo-1 antibodies, LA anti-La antibodies, LC1 anti-liver cytosol antibodies type 1, LKM anti–liver-kidney microsomal antibodies, NUC anti-nucleosome antibodies, NSE neuron specific enolase, OGTT oral glucose tolerance test, p-ANCA perinuclear ANCA, PCR polymerase chain reaction, PRALB prealbumin, PVB19 parvovirus B19, RNP anti-nuclear ribonucleoprotein antibodies, RO anti-Ro-antibodies, SCL anti-Scl-70 antibodies, SEA staphylococcus enterotoxin a, SLA anti-soluble liver antigen antibodies, SM anti-Smith antibodies, SP-100 anti-sp100 antibodies, Ta/b TCR alpha/beta positive T-lymphocytes, TBIL total bilirubin, Tg/d TCR gamma/delta positive T-lymphocytes, TP total protein, TSH thyroid stimulating hormone, TT tetanus toxoid, X-ANCA atypical ANCA, 3/127 IL-7R alpha-chain positive T-lymphocytes, 3/132 common gamma-chain positive T-lymphocytes, 3/154 CD40L positive T-lymphocytes, 4/45RA CD4 positive naive T-cell subsets, 4/45R0 CD4 positive memory T-cell subsets, 8/45RA CD8 positive naive T-cell subsets, 8/45R0 CD8 positive memory T-cell subsets, 45RA/45R 0 CD45RA positive naive T-lymphocytes, 45RA/62L/8 CD45RA positive CD62L positive CD8 positive naive T-lymphocytes, 45R0/45RA CD45R0 positive memory T-lymphocytes
| 4.25
| 0.84375
|
sec[1]/p[0]
|
en
| 0.999997
|
33743140
|
https://doi.org/10.1007/s12328-021-01383-w
|
[
"anti",
"antibodies",
"subsets",
"cell",
"lymphocytes",
"anca",
"cells",
"platelet"
] |
[
{
"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"
}
] |
═══ ICD-11 CLASSIFICATION ═══
【1. Maternal care for other specified fetal problems (JA86.Y)】
Synonyms: Maternal care for other isoimmunization | Isoimmunization NOS | maternal antibodies NOS | pregnancy management affected by incompatibility of blood groups NOS | Maternal care for ABO isoimmunisation
Hierarchy: Pregnancy, childbirth or the puerperium (18) → Maternal care related to the fetus, amniotic cavity or possible delivery problems → Maternal care for other fetal problems (JA86) → Maternal care for other specified fetal problems
【2. Antisocial behaviour (MB23.1)】
Definition: Behaviour in which the basic rights of others or major age-appropriate societal norms, rules, or laws, are violated.
Synonyms: Child or adolescent antisocial behaviour
Hierarchy: Symptoms, signs or clinical findings, not elsewhere classified (21) → Mental or behavioural symptoms, signs or clinical findings → Symptoms or signs involving appearance or behaviour (MB23) → Antisocial behaviour
【3. Coagulation defects, unspecified (3B4Z)】
Synonyms: blood clotting disturbance | blood clotting defect | blood clotting factor deficiency | clotting abnormality | clotting disorder
Hierarchy: Diseases of the blood or blood-forming organs (03) → Coagulation defects, purpura or other haemorrhagic or related conditions → Coagulation defects → Coagulation defects, unspecified
【4. Antiphospholipid syndrome, unspecified (4A45.Z)】
Synonyms: Antiphospholipid syndrome | Hughes syndrome | Anticardiolipin syndrome
Hierarchy: Diseases of the immune system (04) → Nonorgan specific systemic autoimmune disorders → Antiphospholipid syndrome (4A45) → Antiphospholipid syndrome, unspecified
【5. Other specified overlap non-organ specific systemic autoimmune disease (4A43.Y)】
Synonyms: Antisynthetase syndrome | Reynolds syndrome | Syndromic multisystem autoimmune disease due to ITCH deficiency | Eosinophilia myalgia syndrome | EMS - [eosinophilia myalgia syndrome]
Hierarchy: Diseases of the immune system (04) → Nonorgan specific systemic autoimmune disorders → Overlap or undifferentiated nonorgan specific systemic autoimmune disease (4A43) → Other specified overlap non-organ specific systemic autoimmune disease
|
JA86.Y
|
Maternal care for other specified fetal problems
|
Patient was a 31-year-old woman with a 10-year history of the binge eating/purging type of AN. S She had no past history of neurological or cardiovascular diseases, and well-known risk factors such as dyslipidemia, hypertension, and diabetes mellitus. Also, she had no family history of stroke. She was a current smoker, with a smoking history of one pack-year. She was admitted to our hospital with a history of easy fatigability and immobility. She was unable to walk without assistance; however, her consciousness was clear, and full neural examinations revealed no signs of neurological deficits. We diagnosed her as having severe malnutrition, as her body mass index (BMI) was 8.8 (height: 1.64 m, weight: 23.4 kg). Refeeding therapy was initiated at 2000 kcal/day with adequate electrolyte supplementation, during which she showed no complications; the electrolyte levels remained under good control, along with normal blood glucose levels. Water intake was restricted to 1 ml/1 kcal, and there were no complications such as congestive heart failure. On the 10th hospital day, she complained of dysesthesia on her left shoulder. The patient was alert, attentive, and oriented. The cranial nerve was intact. The Barre and Mingazzini test revealed that both flexors and extensors of the left upper and lower extremities were weaker than the right upper and lower ones. The results of manual muscle testing were as follows (R/L); deltoid 5/4+, biceps 5/4+, triceps 5/4+, wrist flexor 5/4, wrist extensor 5/4, hip flexor 5/4+, hip extensor 5/4+, knee flexor 5/4+, knee extensor 5/4+, ankle flexor 5/4+, ankle extensor 5/4+. The tendon reflexes of the extremities were symmetrical, and no abnormal reflexes were found. There was no dysmetria on a finger-to-nose test. Sensory examination reveals hypesthesia to touch and pain on her left shoulder to fingers. But her neurological symptoms resolved spontaneously within 10 min. Her vital signs were as follows: HR 74/min, BP 86/53 mmHg, BT 36.7 °C. Brain magnetic resonance imaging (MRI) revealed disseminated infarctions in the cortex bilaterally; however, her motor symptoms were not explained by the imaging findings . We diagnosed her as having had a transient ischemic attack, with incidental detection of multiple infarctions. Extensive clinical workup, including blood test (biochemistry, blood count, coagulation profile, and autoimmune profile.), ultrasound (carotid artery and lower extremities vein), whole-body computation tomography, Holter electrocardiogram, echocardiography, ankle-brachial index (ABI), and cardio-ankle vascular index (CAVI), to determine the etiology of ischemia revealed that she had no systemic atherosclerosis or any source of embolism. However, her ABI and CAVI values were elevated (Table 1 ), suggesting that she had arteriosclerotic damage without atherosclerosis. Laboratory tests performed on the day that she was diagnosed as having a stroke excluded common causes of early-onset ischemic stroke (vasculitis, dissection, moyamoya disease, antiphospholipid antibody syndrome). Elevated BUN/Cre levels suggested that she had dehydration, and the elevated serum levels of thrombin-antithrombin complex (TAT-III), platelet factor 4 (PF4), and β-thromboglobulin (βTG) indicated that she was in a hypercoagulable state (Table 1 ). She was started on aspirin 100 mg per day for secondary prevention. After the refeeding therapy, she was discharged, and developed no recurrence of the ischemic attack during the subsequent two-year follow-up period. We checked up her head MRI scan one year after her discharge with no findings. Fig. 1 Brain MRI (Patient 1, 2). Patient 1 (Upper row) Brain diffusion-weighted MRI revealed disseminated cortical infarctions bilaterally. MR angiography showed no arterial stenosis. (Lower low) Brain diffusion-weighted MRI and MR angiography (one year after the discharge) revealed no findings. Patient 2 (Upper row) Brain diffusion-weighted MRI revealed focal brain infarction in the white matter underlying the left temporal transverse temporal gyrus, the left supramarginal gyrus, and the left parietal cortex. MR angiography showed no arterial stenosis. (Lower low) Brain diffusion-weighted MRI and MR angiography (one year after the discharge) revealed no findings Table 1 Laboratory data (Patient 1, 2) Pt 1 Pt 2 Reference value Admission Onset Nadir During First two weeks Discharge Admission a Onset Nadir During First two weeks Discharge Biochemistry BUN (mg/dL) 40.9 13.3 12.2 19.8 23.3 23.3 17.7 9.6 8–20 Cre (mg/dL) 0.73 0.32 0.30 0.42 0.55 0.55 0.55 1.29 0.46–0.79 BUN/Cre 56.03 41.56 NA 47.14 42.36 42.36 NA 7.44 Glucose (mg/dL) 177 86 80 103 99 99 89 90 73–109 Na (mmol/L) 135 142 135 139 137 137 137 141 138–145 Cl (mmol/L) 88 112 88 109 96 96 96 105 101–108 K (mmol/L) 2.8 4.5 2.8 4.3 3.4 3.4 3.4 3.4 3.6–4.8 Ca (mg/dL) 7.7 7.7 7.7 8.8 8.0 8.0 7.9 8.3 8.8–10.2 Mg (mg/dL) 2.8 1.5 1.4 1.8 1.8 1.8 1.8 3.0 1.8–2.4 P (mg/dL) 3.6 2.4 2.2 4 4.5 4.5 4.5 5.1 2.4–4.6 AST 244 52 26 23 46 46 37 15 ALT 135 87 51 40 88 88 19 12 CRP < 0.01 < 0.01 < 0.01 < 0.01 < 0.10 < 0.10 < 0.10 < 0.10 TC (mg/dL) 195 155 155 191 212 212 212 295 142–248 TG (mg/dL) 24 58 24 47 71 71 71 151 30–117 HDL-C (mg/dL) 119 65 65 89 82 82 82 90 48–103 LDL-C (mg/dL) 77 81 77 91 122 122 122 181 65–163 FT3 (pg/mL) < 1.5 2.24 < 1.5 NA 2.05 2.05 2.05 1.79 1.88–3.18 FT4 (ng/dL) 1.31 0.74 0.74 NA 0.72 0.72 0.72 0.82 0.7–1.4 TSH (μIU/mL) 2.04 1.18 1.18 NA 1.24 1.24 1.24 0.28 0.35–4.94 Hb-A1c (%) 5.8 5.8 5.8 NA 6.2 6.2 6.2 4.9 4.9–6.0 BNP (pg/mL) 41.9 50.4 41.9 NA NA NA NA NA 0–18.41 Blood count WBC (/μL) 4200 2500 2300 4600 10,500 10,500 4200 8700 3300–8600 RBC (X10^4/μL) 392 260 233 333 286 286 262 372 386–492 Hb (g/dL) 13.3 9.1 8.1 11.8 7.5 7.5 6.9 7.8 11.6–14.8 Hct (%) 38.3 27.5 25.4 37.4 24.3 24.3 23.0 27.5 35.1–44.4 Plt (X10^4/μL) 15.5 14.6 9.5 21.3 54.3 54.3 49.1 54.5 Coagulation profile PT-INR 1.49 1.14 1.14 NA 0.99 0.99 0.93 NA 0.8–1.2 APTT (sec) 29.3 24.6 24.6 NA 23.3 23.3 18 37.7 23.5–31.5 D-dimer (μg/mL) < 0.25 1.2 < 0.25 NA 1.7 1.7 1.2 0.6 0–1 TAT III (ng/L) NA 26.9 26.9 3.6 3.6 3.6 3.6 NA 0–3 Protein C activity (%) NA 57 57 NA 97 97 97 NA 64–146 Protein S (%) NA 84 84 NA 120 120 120 NA 60–150 PF4 (ng/mL) NA 33 33 70 24 24 24 NA 0–20 βTG (ng/mL) NA 104 104 129 105 105 105 NA 0–50 Autoimmune antibodies b ANA 20 < 20 < 20 PR3-ANCA (U/mL) < 1.0 < 1.0 0–3.5 MPO-ANCA (U/mL) < 1.0 < 1.0 0–3.5 Anti-cardiolipin antibody (U/mL) < 10 8 0–10 Imaging findings b Ultrasound (carotid artery) no atherosclerosis no atherosclerosis Ultrasound (lower extremity veins) no embolism no embolism Whole-body CT no evidence of malignancy no evidence of malignancy Holter ECG no evidence of Af no evidence of Af Echocardiography EF 70% Wall motion normal No embolism EF 70% Wall motion normal No embolism CAVI/ABI b CAVI (R/L) 7.8/7.7 7.1/6.9 ABI (R/L) 0.77/0.83 1.04/1.05 CSF b CSF cell count (/μL) NA 1 0–5 CSF protein (mg/dL) NA 24 15–45 CSF IgG-index NA 0.19 0–0.7 CSF MBP NA negative negative CSF OCB NA negative negative a In patient2, the values on ischemic stroke onset are same as on admission b For autoimmune antibodies, imaging findings, CAVI/ABI, and CSF, only the values on ischemic stroke onset are shown in the table
| 3.890625
| 0.98291
|
sec[1]/sec[0]/p[0]
|
en
| 0.999996
|
33743808
|
https://doi.org/10.1186/s40337-021-00393-w
|
[
"brain",
"findings",
"stroke",
"ischemic",
"cavi",
"embolism",
"onset",
"discharge"
] |
[
{
"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 CLASSIFICATION ═══
【1. Other specified diseases of the nervous system (8E7Y)】
Synonyms: Circumscribed brain atrophy | circumscribed cerebral atrophy | atrophic lobar sclerosis | atrophic lobar brain sclerosis | Progressive isolated aphasia
Hierarchy: Diseases of the nervous system (08) → Other specified diseases of the nervous system
【2. Cerebral structural developmental anomalies, unspecified (LA05.Z)】
Synonyms: Cerebral structural developmental anomalies | Malformations of brain | brain abnormality NOS | brain deformity NOS | brain maldevelopment
Hierarchy: Structural developmental anomalies primarily affecting one body system → Structural developmental anomalies of the nervous system → Cerebral structural developmental anomalies (LA05) → Cerebral structural developmental anomalies, unspecified
【3. Infectious encephalitis, unspecified (1D00.Z)】
Synonyms: Infectious encephalitis, not elsewhere classified | encephalitis NOS | acute encephalitis NOS | acute brain inflammation | acute encephalomyelitis NOS
Hierarchy: Certain infectious or parasitic diseases (01) → Non-viral and unspecified infections of the central nervous system → Infectious encephalitis, not elsewhere classified (1D00) → Infectious encephalitis, unspecified
【4. Anencephaly, unspecified (LA00.0Z)】
Synonyms: Anencephaly | anencephalic monster | anencephalus | brain absence | brain agenesis
Hierarchy: Structural developmental anomalies of the nervous system → Anencephaly or similar anomalies (LA00) → Anencephaly (LA00.0) → Anencephaly, unspecified
【5. Other specified diffuse brain injury (NA07.3Y)】
Synonyms: Brain contusion | Cerebral contusion NOS | Diffuse cortex contusion | diffuse cortical contusion | Laceration of brain
Hierarchy: Injuries to the head → Intracranial injury (NA07) → Diffuse brain injury (NA07.3) → Other specified diffuse brain injury
|
8E7Y
|
Other specified diseases of the nervous system
|
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