Imaging findings in congenital hepatic fibrosis


Akhan O., KARAOSMANOĞLU A. D. , Ergen B.

EUROPEAN JOURNAL OF RADIOLOGY, vol.61, no.1, pp.18-24, 2007 (Peer-Reviewed Journal) identifier identifier identifier

  • Publication Type: Article / Review
  • Volume: 61 Issue: 1
  • Publication Date: 2007
  • Doi Number: 10.1016/j.ejrad.2006.11.007
  • Journal Name: EUROPEAN JOURNAL OF RADIOLOGY
  • Journal Indexes: Science Citation Index Expanded, Scopus
  • Page Numbers: pp.18-24

Abstract

Congenital hepatic fibrosis (CHF) is a rare congenital multisystemic disorder, mostly inherited in autosomal recessive fashion, primarily affecting renal and hepatobiliary systems. Main underlying process of the disease is the malformation of the ductal plate, the embryological precursor of the biliary system, and secondary biliary strictures and periportal fibrosis ultimately leading to portal hypertension.