A cholestatic diagnostic dilemma.

[benign recurrent intrahepatic cholestasis]

A 28 -year -old man with hereditary spherocytosis presented with abdominal pain and jaundice . He had severe , mainly conjugated (642 Î¼mol /l ), hyperbilirubinaemia (1033 Î¼mol /l ), with elevated liver enzymes : alkaline phosphatase (ALP ) (163 IU /l ), Î³-glutamyltransferase (gGT ) (277 IU /l ) and aspartate transaminase (AST ) (358 IU /l ). Abdominal ultrasound and magnetic resonance cholangiopancreatography (MRCP ) showed gallstones in the gallbladder but an absence of biliary duct dilation . Liver biopsy was consistent with cholestasis but showed no large duct obstruction . The cause of the cholestasis was unclear , was it a primary intrahepatic pathology or secondary to a posthepatic cause ? He presented with similar symptoms days later with gallstones in the bile duct (choledocholithiasis ) and underwent endoscopic retrograde cholangiopancreatography (ERCP ) and cholecystectomy . This report guides one through the assessment of jaundice and serves as an example whereby the diagnosis of a common cause of illness is blurred by an atypical clinical presentation and relevant comorbidities . A diagnosis of benign recurrent intrahepatic cholestasis (BRIC ) is also considered .

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benign recurrent intrahepatic cholestasis

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