Format

Send to

Choose Destination
See comment in PubMed Commons below
Acta Gastroenterol Belg. 2012 Dec;75(4):405-10.

Progressive familial intrahepatic cholestasis and benign recurrent intrahepatic cholestasis: a review.

Author information

1
Department 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.

PMID:
23402083
[Indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Loading ...
    Support Center