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[原发性心内膜弹力纤维增生症]

[Primary endocardial fibroelastosis].

作者信息

Nishikawa T, Ishiyama S, Hiroe M

机构信息

Tokyo Women's Medical Univ. Dept. Pathology.

出版信息

Nihon Rinsho. 2000 Jan;58(1):171-5.

Abstract

Endocardial fibroelastosis is a disease characterized by a prominent proliferation of dense collagenous and elastic tissue in the endocardium with the occurrence of congestive heart failure and death in early life. There are, however, many recorded instances of infants surviving the initial illness and living up to or beyond puberty. The clinicopathological factors for good prognosis include the increase in myocardial mass of ventricle evaluated by ventriculography and the histopathologic evidence for compensatory myocardial cell hypertrophy in the biopsied myocardium.

摘要

心内膜弹力纤维增生症是一种以内膜致密胶原和弹性组织显著增生为特征的疾病,常伴有充血性心力衰竭,并在早年死亡。然而,有许多记录表明,婴儿在最初患病后存活下来,并活到青春期或更长时间。预后良好的临床病理因素包括通过心室造影评估的心室心肌质量增加,以及活检心肌中代偿性心肌细胞肥大的组织病理学证据。

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