Familial Congenital Hepatic Fibrosis: Report of a Family with Three Affected Children

Abstract

Congenital hepatic fibrosis (CHF) is a developmental disorder of the biliary system, characterized by defective remodeling of the ductal plate. Herein a family of three children, from consanguineous parents, with minor thalassemia is presented who suffered from congenital hepatic fibrosis (CHF). Prompt diagnosis and appropriate treatment are necessary to avoid further complications in the affected patients.

Keywords

• Autoimmune hepatitis
• Congenital hepatic fibrosis
• Ductal plate malformation
• Transjugular intrahepatic portosystemic shunt