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Article

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

Acta gastro-enterologica Belgica - 1 Dec 2012

Strubbe B, Geerts A, Van Vlierberghe H, Colle I

Abstract excerpt

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...

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PMID: 23402083

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