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长期血液透析患者的致命性心脏β2微球蛋白淀粉样变性

Fatal cardiac beta2-microglobulin amyloidosis in patients on long-term hemodialysis.

作者信息

Kawano M, Muramoto H, Yamada M, Minamoto M, Araki H, Koni I, Mabuchi H, Nonomura A

机构信息

Pathology Section, Kanazawa University Hospital, Kanazawa, and Naruwa Hospital, Kanazawa, Japan.

出版信息

Am J Kidney Dis. 1998 Mar;31(3):E4. doi: 10.1053/ajkd.1998.v31.pm10074578.

Abstract

We report two long-term hemodialysis patients who developed severe congestive heart failure attributable to cardiac heavy amyloid deposition. Both patients became hypotensive during dialysis sessions, gradually making it difficult to continue hemodialysis, and they died of congestive heart failure. At autopsy, left ventricle walls in each case contained diffuse extensive deposits of amyloid. The distribution of amyloid was not localized to vessel walls but was widely disseminated throughout the left ventricle walls and replaced myocardial muscle fibers. Immunohistochemical examination showed positive staining for anti-human beta2-microglobulin antibody. We conclude that cardiac dialysis-related amyloidosis should also be considered in long-term hemodialysis patients with congestive heart failure as a life-threatening complication.

摘要

我们报告了两名长期血液透析患者,他们因心脏大量淀粉样蛋白沉积而发展为严重的充血性心力衰竭。两名患者在透析过程中均出现低血压,逐渐难以继续进行血液透析,并死于充血性心力衰竭。尸检时,每例患者的左心室壁均有弥漫性广泛的淀粉样蛋白沉积。淀粉样蛋白的分布并非局限于血管壁,而是广泛散布于整个左心室壁,并取代了心肌纤维。免疫组织化学检查显示抗人β2-微球蛋白抗体染色呈阳性。我们得出结论,对于患有充血性心力衰竭的长期血液透析患者,也应考虑心脏透析相关淀粉样变性这一危及生命的并发症。

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