A Rare case of Cholestatic Liver Disease Progressive Familial Intrahepatic Cholestasis: Case Report
Abstract
Progressive familial intrahepatic cholestasis (PFIC) is a class of chronic cholestasis disorders that begin in infancy and usually progress to cirrhosis within the first decade of life. The average age at onset is 3 months, although some patients do not develop jaundice until later, even as late as adolescence. PFIC can progress rapidly and cause cirrhosis during infancy or may progress relatively slowly with minimal scarring well into adolescence. Few patients have survived into the third decade of life without treatment. Here we present a rare case of Progressive familial intrahepatic cholestasis masquerading as Wilson’s disease.
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