ENBD (endoscopic nasobiliary drainage) was effective for prolonged jaundice due to benign recurrent intrahepatic cholestasis; a case report

Abstract

Benign recurrent intrahepatic cholestasis (BRIC) is an autosomal recessive disorder characterized by episodic cholestasis without elevation of serum gamma-glutamyltransferase (GGT) levels, and usually lacks progressive liver damage. Recent studies have demonstrated that BRIC, or BRIC type 1, is caused by mutations in ATP8B1 gene encoding FIC1 (familial intrahepatic cholestasis) protein, P-type ATPase. We herein report a female case with BRIC, who developed prolonged jaundice at 22 years old. While medical treatments including ursodeoxycholic acid administration were not effective, we performed ENBD (endoscopic nasobiliary drainage) to interrupt the enterohepatic circulation of bile salts. Her subjective symptoms rapidly subsided, and serum levels of total bile acids and bilirubin was reduced to the normal levels in a week. She has remained asymptomatic for two years after the removal of ENBD. It is assumed that the reduction of bile acids pool in the enterohepatic circulation contributed to resolving her symptoms and prolonged cholestasis. We propose that temporary ENBD is a successful therapeutic option for prolonged jaundice due to BRIC.