title: Progressive familial intrahepatic cholestasis.
abstract: 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, pruritus, 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.
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi. rmirzamd@yahoo.com
authors.forename: Rashid
authors.initials: R
authors.lastname: Mirza
authors.forename: Zaigham
authors.initials: Z
authors.lastname: Abbas
authors.forename: Nasir Hassan
authors.initials: NH
authors.lastname: Luck
authors.forename: S M Zahid
authors.initials: SM
authors.lastname: Azam
authors.forename: Sina
authors.initials: S
authors.lastname: Aziz
authors.forename: Syed Mujahid
authors.initials: SM
authors.lastname: Hassan
authors.forename: Ghous Bakhsh
authors.initials: GB
authors.lastname: Soomro
mesh_terms: D001656:Biliary Atresia / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D018450:Disease Progression; D006505:Hepatitis / Q000175:diagnosis; D006801:Humans; D008103:Liver Cirrhosis / Q000453:epidemiology; D008297:Male; D011537:Pruritus / Q000209:etiology