A Rare Case Report on Benign Recurrent Intrahepatic Cholestasis

Abstract

Benign recurrent intrahepatic cholestasis is an inherited and occasionally sporadic disease presents as recurrent episodes of obstructive jaundice without any obstruction in billiary channel with intervening symptom free periods. Here we are presenting a case of 20-year-old male with a recurrent jaundice and pruritus who later diagnose as BRIC.Faridpur Med. Coll. J. 2014;9(2): 108-110

Role of biopsy in Benign recurrent intrahepatic cholestasis

“…There are 3 types of BRIC -1, 2 and 3 7,8 . BRIC 1 and 2 are autosomal recessive disorders whereas BRIC 3 is an autosomal dominant disorder 7 .…”

“…BRIC 1 and 2 are autosomal recessive disorders whereas BRIC 3 is an autosomal dominant disorder 7 . Progressive Familial Intrahepatic Cholestasis (PFIC) and BRIC represent two extreme ends of the spectrum of following mutational disorders 7,8 . Missense mutations leading to partial functional deficiency of FIC1 and BSEP occur in 50% cases of BRIC 7 .…”

“…They translocate phospholipids along the membrane and protect from high bile salt concentration by neutralizing their detergent effect 7 . BSEP encoded by ABCB11 is the main exporter of hepatocyte bile acids to canaliculi 7,8 .…”

“…Very long symptom free interval of more than 15 years has been reported only thrice in the literature with the longest duration being 23 years 10 . There is no progression to cirrhosis in most of the cases and explains its benign nature 8 . C.Putterman et al in a followup of a case of BRIC over 25 years with repeated biopsies observed no evidence of chronic liver disease 10 .…”

“…Patients are managed symptomatically with Ursodeooxycholic acid, Cholestyramine, Rifampin, Sertraline or Naltrexone. Other treatment modalities include endoscopic nasobiliary drainage, partial external ileal diversion, phototherapy and liver transplantation 8,13 .…”