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Papers of the Week
Clinical outcomes of surgical management for rare types of progressive familial intrahepatic cholestasis: a case series.
Abstract
Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of genetic autosomal recessive diseases that cause severe cholestasis, which progresses to cirrhosis and liver failure, in infancy or early childhood. We herein report the clinical outcomes of surgical management in patients with four types of PFIC.