Imaging findings in congenital hepatic fibrosis

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

EUROPEAN JOURNAL OF RADIOLOGY, cilt.61, sa.1, ss.18-24, 2007 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Derleme
  • Cilt numarası: 61 Sayı: 1
  • Basım Tarihi: 2007
  • Doi Numarası: 10.1016/j.ejrad.2006.11.007
  • Dergi Adı: EUROPEAN JOURNAL OF RADIOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.18-24
  • Hacettepe Üniversitesi Adresli: Evet

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.