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杜兴氏肌营养不良症:功能性缺血会重现其特征性病变。

Duchenne muscular dystrophy: functional ischemia reproduces its characteristic lesions.

作者信息

Mendell J R, Engel W K, Derrer E C

出版信息

Science. 1971 Jun 11;172(3988):1143-5. doi: 10.1126/science.172.3988.1143.

Abstract

The highly characteristic early and midstage histological lesions of Duchenne dystrophy were reproduced experimentally in the rat by the combination of a vascular abnormality, aortic ligation, which does not affect the structure of the intramuscular blood vessels, and the humoral vasoactive substance 5-hydroxytryptamine. Neither ligation nor injection of 5-hydroxytryptamine alone causes changes in the muscle fibers. This result establishes the possibility of a similar combined mechanism for a nonstructural ischemia pathogenesis in Duchenne dystrophy. The proposed pathogenesis is contrary to the generally held idea that the cause is an intrinsic abnormality of muscle fiber metabolism.

摘要

通过血管异常(不影响肌内血管结构的主动脉结扎)与体液血管活性物质5-羟色胺的联合作用,在大鼠身上实验性地再现了杜兴氏肌营养不良高度特征性的早期和中期组织学病变。单独的结扎或5-羟色胺注射均不会引起肌纤维变化。这一结果证实了杜兴氏肌营养不良中非结构性缺血发病机制存在类似联合机制的可能性。所提出的发病机制与普遍认为病因是肌纤维代谢内在异常的观点相反。

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