Congenital Hepatic Fibrosis with Fatal Cholestatic Liver Damage

Masao TAKATORI; Shogo IWABUCHI; Takeshi HAYASHI; Yasuto TAKAHASHI; Michihiro SUZUKI; Shiro IINO; Shiro MAEYAMA; Toshiyuki UCHIKOSHI

doi:10.2169/internalmedicine.39.930

Masao TAKATORI, Shogo IWABUCHI, Takeshi HAYASHI, Yasuto TAKAHASHI, Michihiro SUZUKI, Shiro IINO, Shiro MAEYAMA, Toshiyuki UCHIKOSHI

Author information

Masao TAKATORI
the Department of Gastroenterology and Hepatology, St. Marianna University School of Medicine
Shogo IWABUCHI
the Department of Gastroenterology and Hepatology, St. Marianna University School of Medicine
Takeshi HAYASHI
the Department of Gastroenterology and Hepatology, St. Marianna University School of Medicine
Yasuto TAKAHASHI
the Department of Gastroenterology and Hepatology, St. Marianna University School of Medicine
Michihiro SUZUKI
the Department of Gastroenterology and Hepatology, St. Marianna University School of Medicine
Shiro IINO
the Department of Gastroenterology and Hepatology, St. Marianna University School of Medicine
Shiro MAEYAMA
the Second Department of Pathology, St. Marianna University School of Medicine
Toshiyuki UCHIKOSHI
the Second Department of Pathology, St. Marianna University School of Medicine

Keywords: hepatic fibrosis, transplantation, portal hypertension, hepatic failure

JOURNAL FREE ACCESS

2000 Volume 39 Issue 11 Pages 930-935

DOI https://doi.org/10.2169/internalmedicine.39.930

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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.
(Internal Medicine 39: 930-935, 2000)

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