Article
Progressive familial intrahepatic cholestasis.
Clinics and research in hepatology and gastroenterology - 1 Sept 2012
Jacquemin Emmanuel
Abstract excerpt
Progressive familial intrahepatic cholestasis (PFIC) refers to a heterogeneous group of autosomal-recessive disorders of childhood that disrupt bile formation and present with cholestasis of hepatocellular origin. The exact prevalence remains unknown, but the estimated incidence varies between 1/50,000 and 1/100,000 births. Three types of PFIC have been identified and associated with mutations in hepatocellular...
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