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Congenital hepatic fibrosis with fatal cholestatic liver damage
M Takatori1, S Iwabuchi, T Hayashi
1Department of Gastroenterology and Hepatology, St. Marianna University School of Medicine, Kawasaki.
Internal Medicine (Tokyo, Japan)
|November 7, 2000
Abstract:
A 23-year-old male with congenital hepatic fibrosis died because of progressive cholestatic liver damage. Pathologically, marked extension of fine fibers along the sinusoids in addition to fibrosis in Glisson's sheath, miniaturization and pseudo-glandular formation of hepatocytes as parenchymal damage, and nodular regenerative hyperplasia were considered as the cause of rapid aggravation of liver damage or portal hypertension.