Progressive familial intrahepatic cholestasis (PFIC) is an important cause of cholestatic liver disease and biliary cirrhosis in pediatric population. Three cases of PFIC are described that were diagnosed on the basis of family history, pruritis, cirrhosis and/or paucity of interlobular bile ducts on liver biopsy and presence of extrahepatic biliary tree on imaging. These patients were initially labeled as suffering from extra-hepatic biliary atresia and neonatal hepatitis. PFIC-1 and 2 could not be differentiated on histological grounds, since these patients presented late and process of fibrosis was advanced.
|Number of pages||3|
|Journal||Journal of the College of Physicians and Surgeons--Pakistan : JCPSP|
|Publication status||Published - Oct 2006|
- Biliary cirrhosis
- Cholestatic liver disease
- Progressive familial intrahepatic cholestasis (PFIC)