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杜氏进行性肌营养不良症中心脏传导系统的组织病理学研究。

Histopathological study of the conduction system of the heart in Duchenne progressive muscular dystrophy.

作者信息

Nomura H, Hizawa K

出版信息

Acta Pathol Jpn. 1982 Nov;32(6):1027-33. doi: 10.1111/j.1440-1827.1982.tb02082.x.

DOI:10.1111/j.1440-1827.1982.tb02082.x
PMID:7158338
Abstract

The cardiac conduction systems including sinoatrial (SA) node, atrioventricular (AV) node, atrioventricular(His) bundle, and peripheral conduction system (left and right bundle branch, and Purkinje fiber) of 23 patients with Duchenne progressive muscular dystrophy(DMD) were studied with light microscope. Infiltration of fat tissue and mild fibrosis were occasional findings in SA and AV nodes. Degeneration of the conduction muscle fiber was hardly noted in SA node, AV node, and His bundle. Only the peripheral conduction system (Purkinje fiber) showed significant degenerations such as eosinophilic, necrotic and vacuolar changes with fibrosis. These necrobiotic changes resembled hyaline and vacuolar skeletal and cardiac muscular degenerations in DMD and were assumed to have occurred on the basis of the structural and constitutional characteristics of the peripheral conduction fiber as a striated muscle fiber. The vascular changes and amyloid deposit suggesting precocious aging in the conduction systems were not observed.

摘要

对23例杜氏进行性肌营养不良(DMD)患者的心脏传导系统,包括窦房(SA)结、房室(AV)结、房室(希氏)束和外周传导系统(左右束支和浦肯野纤维)进行了光镜研究。脂肪组织浸润和轻度纤维化偶见于窦房结和房室结。在窦房结、房室结和希氏束中几乎未发现传导肌纤维变性。仅外周传导系统(浦肯野纤维)出现明显变性,如嗜酸性、坏死性和空泡性改变并伴有纤维化。这些坏死性改变类似于DMD中透明样和空泡性骨骼肌及心肌变性,被认为是基于外周传导纤维作为横纹肌纤维的结构和构成特征而发生的。未观察到提示传导系统早衰的血管改变和淀粉样沉积。

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