Benign Recurrent Intrahepatic Cholestasis (Bric)

摘要

Benign recurrent intrahepatic cholestasis (BRIC) a rare form of hereditary cholestasis syndrome characterized by repeated self limited episodes of pruritus and jaundice. It recurs but occasionally leads to progressive liver disease. Treatment is symptomatic. We report a case of BRIC in a child. Case Report A 11-year-old boy who is the first child of his healthy non consanguineous parents, presented to us with complaints of pruritus, jaundice, dark coloured urine and intermittent pale stools. There was no history of febrile illness / ingestion of drugs or family history of liver disease. He had previous similar episode of jaundice one year back it lasted for one month for which he received symptomatic treatment . Liver function tests done during that previous episode showed conjugated hyperbilirubinemia and elevated alkaline phosphatase (900 IU). The child was asymptomatic in between the episodes. He was healthy with normal growth and development. On examination, the child had icterus, obvious itching, without pallor, pedal edema or signs of vitamin deficiencies. There were no signs of liver failure. Liver was just palpable in right hypochondrium with a span of 11 cm. Spleen was not palpable. Rest of the systemic examination was non contributory. Investigations revealed Hb of 12.1 g/dl with normal total and differential count. Total bilirubin was 19.53 mg% with a direct component of 14.06mg%. Alkaline phosphatase was elevated with a value of 769 IU. Liver enzymes including ALT, AST and gamma glutamyl transpeptidase (GGT: 8U/L) were within normal limits. Serum total proteins, albumin globulin ratio were also normal. Prothrombin time was prolonged but responded to vitamin K. Ultrasonography of abdomen showed normal echotexture of liver. MRCP (Magnetic Resonance Cholangio Pancreatogram) was normal. Viral markers for Hepatitis A, B and C were negative and serum ceruloplasmin (20 mg/dL) was within normal limits. In view of clinical and biochemical findings, a possibility of recurrent cholestasis was considered and a liver biopsy was performed. The biopsy showed preserved lobular architecture with marked cholestasis within hepatocytes with mild inflammatory cell infiltrates (Fig.1).