先天性肝纤维化伴致命性胆汁淤积性肝损伤。

Congenital hepatic fibrosis with fatal cholestatic liver damage.

作者信息

Takatori M, Iwabuchi S, Hayashi T, Takahashi Y, Suzuki M, Iino S, Maeyama S, Uchikoshi T

机构信息

Department of Gastroenterology and Hepatology, St. Marianna University School of Medicine, Kawasaki.

出版信息

Intern Med. 2000 Nov;39(11):930-5. doi: 10.2169/internalmedicine.39.930.

DOI:10.2169/internalmedicine.39.930

PMID:11065245

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.

摘要

一名23岁先天性肝纤维化男性因进行性胆汁淤积性肝损伤死亡。病理上，除了肝门管区纤维化外，沿肝血窦的细纤维显著延伸、实质损伤导致的肝细胞小叶化和假腺样形成，以及结节性再生性增生被认为是肝损伤快速加重或门静脉高压的原因。