Progressive familial intrahepatic cholestasis

Tomohide Hori, Justin H. Nguyen, Shinji Uemoto

Research output: Contribution to journalReview articlepeer-review

21 Scopus citations


BACKGROUND: Three types of progressive familial intrahepatic cholestasis (PFIC) have been identified, but their etiologies include unknown mechanisms. DATA SOURCES: A PubMed search on "progressive familial intrahepatic cholestasis" and "PFIC" was performed on the topic, and the relevant articles were reviewed. RESULTS: The etiologies of the three PFIC types still include unknown mechanisms. Especially in PFIC type 1, enterohepatic circulation of bile acid should be considered. Ursodeoxycholic acid, partial external biliary diversion and liver transplantation have been used for the treatment of PFIC patients according to disease course. CONCLUSIONS: Since the etiologies and disease mechanisms of PFIC are still unclear, detailed studies are urgently required. Strategies for more advanced therapies are also needed. These developments in the future are indispensable, especially for PFIC type 1 patients.

Original languageEnglish (US)
Pages (from-to)570-578
Number of pages9
JournalHepatobiliary and Pancreatic Diseases International
Issue number6
StatePublished - Dec 15 2010


  • Byler's disease
  • Liver transplantation
  • Progressive familial intrahepatic cholestasis
  • Steatosis

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology


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