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遗传性出血性毛细血管扩张症中的“假性肝硬化”

'Pseudocirrhosis' in hereditary haemorrhagic telangiectasia.

作者信息

Cooney T, Sweeney E C, Coll R, Greally M

出版信息

J Clin Pathol. 1977 Dec;30(12):1134-41. doi: 10.1136/jcp.30.12.1134.

Abstract

Telangiectasia-associated hepatic fibrosis (TAHF) in a 68-year-old woman with hereditary haemorrhagic telangiectasia (HHT) is described. The patient died of oat-cell carcinoma of the lung. In addition to the structural alterations which have been described previously in HHT, the liver exhibited focal midlobular hepatocytic necrosis and tumour metastases. The possibility that treatment of HHT was causally related to some of the hepatic abnormalities found in our patient and the differentiation of TAHF from true cirrhosis are discussed.

摘要

本文描述了一名68岁患有遗传性出血性毛细血管扩张症(HHT)的女性患者的毛细血管扩张相关肝纤维化(TAHF)。该患者死于肺燕麦细胞癌。除了先前在HHT中所描述的结构改变外,肝脏还表现出局灶性小叶中部肝细胞坏死和肿瘤转移。文中讨论了HHT的治疗与我们患者所发现的一些肝脏异常之间存在因果关系的可能性,以及TAHF与真性肝硬化的鉴别。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/7deff5a00220/jclinpath00170-0046-a.jpg

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