Progressive familial intrahepatic cholestasis

Rashid Mirza, Zaigham Abbas, Nasir Hassan Luck, S. M.Zahid Azam, Sina Aziz, Syed Mujahid Hassan, Ghous Bakhsh Soomro

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)673-675
Number of pages3
JournalJournal of the College of Physicians and Surgeons--Pakistan : JCPSP
Issue number10
Publication statusPublished - Oct 2006
Externally publishedYes


  • Biliary cirrhosis
  • Cholestatic liver disease
  • Progressive familial intrahepatic cholestasis (PFIC)


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