progressive familial intrahepatic cholestasis

progressive familial intrahepatic cholestasis
a genetically heterogeneous group of autosomal recessive disorders of bile acid transport, characterized by early onset of intrahepatic cholestasis that progresses to hepatic fibrosis, cirrhosis, and end-stage liver disease before adulthood. There are 3 types; type 1 is called also Byler disease.

Medical dictionary. 2011.

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