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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.

DOI:10.1136/jcp.30.12.1134
PMID:203609
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC476696/
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/f4d102fa724e/jclinpath00170-0049-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/7deff5a00220/jclinpath00170-0046-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/dc94325c1e9d/jclinpath00170-0046-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/e07ee97de7e9/jclinpath00170-0047-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/ceac0dc57f73/jclinpath00170-0047-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/16c8bd18a5bd/jclinpath00170-0048-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/23225985e4b1/jclinpath00170-0048-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/f4d102fa724e/jclinpath00170-0049-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/7deff5a00220/jclinpath00170-0046-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/dc94325c1e9d/jclinpath00170-0046-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/e07ee97de7e9/jclinpath00170-0047-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/ceac0dc57f73/jclinpath00170-0047-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/16c8bd18a5bd/jclinpath00170-0048-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/23225985e4b1/jclinpath00170-0048-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28f5/476696/f4d102fa724e/jclinpath00170-0049-a.jpg

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本文引用的文献

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The rarity of intrahepatic metastasis in cirrhosis of the liver. A statistical explanation with some comments on the interpretation of necropsy data.肝内转移在肝硬化中的罕见性。基于尸检数据解读的统计学解释及一些评论
Am J Pathol. 1961 Dec;39(6):739-46.
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HEPATIC ARTERY ANEURYSM WITH PORTAL VEIN FISTULA IN A PATIENT WITH FAMILIAL HEREDITARY TELANGIECTASIA.一名患有家族遗传性毛细血管扩张症的患者出现肝动脉瘤伴门静脉瘘
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FAMILIAL HAEMORRHAGIC TELANGIECTASIA. 20 CASES TREATED WITH SYSTEMIC OESTROGEN.
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Hepatic and pancreatic involvement in hereditary hemorrhagic telangiectasia: quantitative and qualitative evaluation with 64-section CT in asymptomatic adult patients.遗传性出血性毛细血管扩张症的肝脏和胰腺累及:64 层 CT 对无症状成年患者的定量和定性评估。
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Clinical outcome of transfemoral embolisation in patients with arteriovenous malformations of the liver in hereditary haemorrhagic telangiectasia (Weber-Rendu-Osler disease).遗传性出血性毛细血管扩张症(韦-伦-奥氏病)患者肝动静脉畸形经股动脉栓塞术的临床结果
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