Two Siblings Cases of Congenital Hepatic Fibrosis.

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  • 同胞に発症した先天性肝線維症の2例  腹腔鏡所見の文献的考察を加えて

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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.

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