Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:2.6.1.1 (aspartate aminotransferase)
 21,665 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Actinomycin-D (Act-D) is a rare cause of veno-occlusive disease (VOD). Between 1993 and 1998, we managed 6 patients, all male, median age 19 months (range 6-48 months) who received Act-D for Wilms' tumour (n=4), clear cell sarcoma (n=1) or rhabdomyosarcoma (n=1). VOD presented with a median platelet count of 12 x 10(9)/l, INR 3.8, fibrinogen 16 mg/l, fibrinogen degradation products (FDPs) > or =80 microg/l, aspartate aminotransferase (AST) 6922 IU/l, bilirubin 47 micromol/l. In 3 cases, transient liver dysfunction and thrombocytopenia without neutropenia had been observed after a previous course of Act-D. All six children developed encephalopathy, hepatomegaly, ascites, reversed portal flow and renal impairment. All received mechanical ventilation and two required haemofiltration. The treatment was supportive. Severe Adult Respiratory Distress Syndrome developed in 3 patients, all of whom died. 3 patients recovered. The outcome of VOD with multi-organ failure is poor. Intravascular coagulopathy precedes and characterises severe VOD during Act-D treatment.
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PMID:Veno-occlusive disease with multi-organ involvement following actinomycin-D. 1137 45

A 3-year old male presented with complaints of pruritus, abdominal pain for 3 weeks and jaundice. Stools were acholic. There was jaundice, liver palpable 3 cm below right costal margin, no ascites or palpable masses. Serology revealed albumin 2.9 g/dl; ammonia of 31 mmol/l; elevated conjugated bilirubin, GGT, ALT, AST and alkaline phosphatase; alpha fetoprotein 1.3 ngm/ml; BhCG 9.1 IU/; PT 12.3 secs, INR 0.9; negative hepatitis A,B,C serology. CT scan showed a non-calcified heterogeneously enhancing mass centered at the liver hilum. MRCP showed a large heterogeneously enhancing, partially solid mass in the region of the porta hepatic. Liver biopsy revealed patternless proliferation of polymorphic oval to spindled shaped neoplastic cells. There was bile ducts distortion. Immunohistochemistry revealed positivity for vimentin, desmin.These findings were diagnostic for biliary rhabdomyosarcoma.There was no evidence of metastasis. Chemotherapy was initiated. Repeat imaging 6 months after initiation of treatment showed improvement in the degree of intrahepatic ductal dilatation and decrease in tumor bulk size. Rhabdomyosarcoma is the most common malignant tumor of the biliary tree in childhood. It is difficult to diagnose and delayed diagnosis influences the prognosis.
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PMID:Biliary rhabdomyoscarcoma mimicking choledochal cyst. 1933 43