Abstract
Background: The diagnosis of intrahepatic cholestasis in a young may be challenging. We present an unusual case of intrahepatic cholestasis in a young male. Case Summary: A 15 yr male came with complaints of yellowish discoloration of eyes and urine, severe pruritus all over body, decreased apetite, mild epigastric discomfort and nausea since 1 month. There was no history of ethanol intake, fever, diarrhea, outside food, travelling, high risk behavior, blood transfusion, colicky abdominal pain, weight loss, malaise or bodyaches. There was a history of jaundice at the age of 3 years and 14 years of age that resolved on its own with self-medications in few days. There was a history of death of elder sister at age of 15 yrs due to jaundice; details of which were not available. No similar complaints in father or mother were reported. There was history of alternative medicine (CAM) intake for 15 days. On examination, he was conscious, oriented, vitals were normal. Severe scratch marks were seen all over body. Investigations showed hemoglobin 14.5 g/dl, leucocyte count 14,580 mm3, platelet 2.97 lac/mm3, IN 3.14, BSL-144, creatnine 0.4, total bilirubin- 39.5 mg/dl (direct-30), AST-93U/l, ALT-83U/l, alkaline phosphatase- 419 U/l, albumin 4 g/dl. HBV/HCV/HIV/HAV/HEV were negative. Serum ceruloplasmin, iron, transferrin saturation, GGT were normal. Serum bile acids were elevated. IgG 4 were normal. ANA, p-ANCA were negative (full autoimmune workup was not done due to affordibility issues). USG abdomen showed GB calculi with no choledocholithiasis and no ihbd/ehbd dilatation and no ascites. Liver, spleen, pancreas were normal on ultrasound. MRCP was normal. Liver biopsy (after correcting INR) showed centrilobular hepatocellular and canalicular cholestasis without portal inflammation. Based on above findings and history diagnosis of BRIC- Benign Recurrent Intrahepatic Cholestasis was considered. Genetic workup adviced and sent. (report awaited) patient was started on steroids and phenobarbitone, UDCA, supportive care, symptomatically improved. T BIL dropped to 28 from 39.5 within 6 days. (as genetic test report for ABCB11/ATP8B1/OR BRIC 3 will take 4 weeks minimum. The authors have none to declare.
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