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婴儿期后出现心内膜弹力纤维增生症的家族性非梗阻性心肌病。

Familial nonobstructive cardiomyopathy with endocardial fibroelastosis beyond infancy.

作者信息

Singh A, Doyle E F, Danilowicz D A, Finegold M J

出版信息

Pediatrics. 1978 Mar;61(3):410-6. doi: 10.1542/peds.61.3.410.

Abstract

A 10-year-old boy with congestive heart failure died in five months in spite of comprehensive medical treatment. Autopsy showed patchy areas of endocardial fibroelastosis of the left ventricle. The sister of this patient had followed a similar course at 13 years of age with death within six months of the onset of congestive failure. Her postmortem examination also showed endocardial fibroelastosis. The clinical presentation of familial endocardial fibroelastosis in the preteen and teenage years is a rare event. Probably the endocardial fibroelastosis was secondary to a familial nonobstructive cardiomyopathy.

摘要

一名10岁的充血性心力衰竭男孩尽管接受了全面的药物治疗,但仍在5个月内死亡。尸检显示左心室有散在的内膜纤维弹性组织增生区域。该患者的姐姐在13岁时也经历了类似的病程,在充血性心力衰竭发作后6个月内死亡。她的尸检也显示有内膜纤维弹性组织增生。青少年期家族性内膜纤维弹性组织增生的临床表现是一种罕见的情况。内膜纤维弹性组织增生可能继发于家族性非梗阻性心肌病。

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Familial occurrence of the contracted form of endocardial fibroelastosis.
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