Imaging findings in congenital hepatic fibrosis


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

EUROPEAN JOURNAL OF RADIOLOGY, cilt.61, ss.18-24, 2007 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 61 Konu: 1
  • Basım Tarihi: 2007
  • Doi Numarası: 10.1016/j.ejrad.2006.11.007
  • Dergi Adı: EUROPEAN JOURNAL OF RADIOLOGY
  • Sayfa Sayıları: ss.18-24

Özet

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.