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伴有多神经病的家族性淀粉样变性中高折射性心肌回声。一项超声心动图与组织病理学相关性研究。

Highly refractile myocardial echoes in familial amyloidosis with polyneuropathy. A correlative echocardiographic and histopathological study.

作者信息

Eriksson P, Eriksson A, Backman C, Hofer P A, Olofsson B O

出版信息

Acta Med Scand. 1985;217(1):27-32. doi: 10.1111/j.0954-6820.1985.tb01630.x.

DOI:10.1111/j.0954-6820.1985.tb01630.x
PMID:3976431
Abstract

Two-dimensional echocardiography in systemic amyloidosis has in most cases shown highly refractile myocardial echoes. The morphological basis for this finding has so far been little examined. In the present study, echocardiographic examinations were performed in vitro on autopsied hearts from ten patients with familial amyloidosis with polyneuropathy (FAP). An ultrasonically visible needle was used to localize the abnormal echoes, and samples were taken for histological analysis of the region of the echocardiographic abnormality. Our results indicate that the highly refractile myocardial echoes in FAP are due to nodules composed of collagen and amyloid.

摘要

二维超声心动图检查发现,大多数系统性淀粉样变性患者的心肌回声具有高度折射性。迄今为止,对这一发现的形态学基础研究甚少。在本研究中,我们对10例家族性淀粉样多神经病(FAP)患者的尸检心脏进行了体外超声心动图检查。使用超声可见的针来定位异常回声,并采集样本对超声心动图异常区域进行组织学分析。我们的结果表明,FAP患者心肌回声高度折射是由胶原蛋白和淀粉样蛋白组成的结节所致。

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Highly refractile myocardial echoes in familial amyloidosis with polyneuropathy. A correlative echocardiographic and histopathological study.伴有多神经病的家族性淀粉样变性中高折射性心肌回声。一项超声心动图与组织病理学相关性研究。
Acta Med Scand. 1985;217(1):27-32. doi: 10.1111/j.0954-6820.1985.tb01630.x.
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Transthyretin V122I (pV142I)* cardiac amyloidosis: an age-dependent autosomal dominant cardiomyopathy too common to be overlooked as a cause of significant heart disease in elderly African Americans.转甲状腺素蛋白 V122I(pV142I)*心脏淀粉样变性:一种与年龄相关的常染色体显性遗传性心肌病,在老年非裔美国人中因显著心脏病而被忽视的常见病因。
Genet Med. 2017 Jul;19(7):733-742. doi: 10.1038/gim.2016.200. Epub 2017 Jan 19.