Progressive familial intrahepatic cholestasis.
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.
Mirza
Rashid
R
Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi. rmirzamd@yahoo.com
Abbas
Zaigham
Z
Luck
Nasir Hassan
NH
Azam
S M Zahid
SM
Aziz
Sina
S
Hassan
Syed Mujahid
SM
Soomro
Ghous Bakhsh
GB
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