title: Congenital hepatic fibrosis with fatal cholestatic liver damage.
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.
authors.affiliation: Department of Gastroenterology and Hepatology, St. Marianna University School of Medicine, Kawasaki.
authors.forename: M
authors.initials: M
authors.lastname: Takatori
authors.forename: S
authors.initials: S
authors.lastname: Iwabuchi
authors.forename: T
authors.initials: T
authors.lastname: Hayashi
authors.forename: Y
authors.initials: Y
authors.lastname: Takahashi
authors.forename: M
authors.initials: M
authors.lastname: Suzuki
authors.forename: S
authors.initials: S
authors.lastname: Iino
authors.forename: S
authors.initials: S
authors.lastname: Maeyama
authors.forename: T
authors.initials: T
authors.lastname: Uchikoshi
mesh_terms: D000293:Adolescent; D002780:Cholestasis, Intrahepatic / Q000209:etiology*; D017809:Fatal Outcome; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications* / Q000151:congenital* / Q000473:pathology; D008297:Male