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肝血窦的毒性损伤:肝窦阻塞综合征(静脉闭塞性疾病)。

Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease).

作者信息

DeLeve Laurie D, Shulman Howard M, McDonald George B

机构信息

USC Research Center for Liver Diseases, Division of Gastrointestinal and Liver Diseases, University of Southern California Keck School of Medicine, Los Angeles, California, USA.

出版信息

Semin Liver Dis. 2002 Feb;22(1):27-42. doi: 10.1055/s-2002-23204.

Abstract

The term veno-occlusive disease of the liver refers to a form of toxic liver injury characterized clinically by the development of hepatomegaly, ascites, and jaundice, and histologically by diffuse damage in the centrilobular zone of the liver. The cardinal histologic features of this injury are marked sinusoidal fibrosis, necrosis of pericentral hepatocytes, and narrowing and eventual fibrosis of central veins. Recent studies suggest that the primary site of the toxic injury is sinusoidal endothelial cells, followed by a series of biologic processes that lead to circulatory compromise of centrilobular hepatocytes, fibrosis, and obstruction of liver blood flow. Thus we propose a more appropriate name for this form of liver injury--sinusoidal obstruction syndrome. This review encompasses historical perspectives, clinical manifestations of sinusoidal obstruction syndrome in the setting of hematopoietic cell transplantation, histologic features of centrilobular injury, and a discussion of the pathophysiology of sinusoidal injury, based on both animal and clinical investigations.

摘要

肝静脉闭塞病是指一种中毒性肝损伤形式,临床特征为肝肿大、腹水和黄疸,组织学特征为肝脏小叶中心区弥漫性损伤。这种损伤的主要组织学特征是显著的窦状隙纤维化、中央周围肝细胞坏死以及中央静脉狭窄并最终纤维化。最近的研究表明,中毒性损伤的主要部位是窦状隙内皮细胞,随后是一系列导致小叶中心肝细胞循环受损、纤维化和肝血流阻塞的生物学过程。因此,我们为这种肝损伤形式提出一个更合适的名称——窦状隙阻塞综合征。这篇综述涵盖了历史观点、造血细胞移植背景下窦状隙阻塞综合征的临床表现、小叶中心损伤的组织学特征,以及基于动物和临床研究对窦状隙损伤病理生理学的讨论。

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