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Updated: May 14, 2026

Partial Bile Duct Ligation in the Mouse: A Controlled Model of Localized Obstructive Cholestasis
Published on: March 28, 2018
Progressive familial intrahepatic cholestasis and benign recurrent intrahepatic cholestasis: a review
B Strubbe1, A Geerts, H Van Vlierberghe
1Department of Hepatology and Gastroenterology, Ghent University Hospital, Ghent, Belgium. beatrijs_strubbe@yahoo.com
Abstract:
Progressive familial intrahepatic cholestasis (PFIC) and benign recurrent intrahepatic cholestasis (BRIC) are two rare autosomal recessive disorders, characterized by cholestasis. They are related to mutations in hepatocellular transport system genes involved in bile formation. The differentiation between PFIC and BRIC is based on phenotypic presentation: PFIC is a progressive disease, with evolution to end-stage liver disease. BRIC is characterized by intermittent recurrent cholestatic episodes, with irresistible pruritus, mostly without evident liver damage. Between symptomatic periods, patients are completely asymptomatic. In this article, a short overview of the aetiology, the clinical and diagnostic characteristics and the therapy of both PFIC and BRIC are given.
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