TWO SIBLINGS CASES OF CONGENITAL HEPATICFIBROSIS

Abstract

Siblings (21-year-old female and 26-year-old male) were hospitalized for examination ofsuspected portal hypertension. Both had esophageal and gastric varicosis, and CT demonstrat-ed liver fibrosis. Laparoscopy revealed diffuse whitish marking on thelIiver surface and was auseful procedure for making the diagnosis of congenital hepatic fibrosis. Although congenitalhepatic fibrosis is a genetic disease, siblings cases are rare. we reviewed some literatures andreported two cases.