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肌营养不良蛋白缺陷的mdx肌肉不易发生恶性高热易感性:一项体外研究。

Dystrophin deficient mdx muscle is not prone to MH susceptibility: an in vitro study.

作者信息

Mader N, Gilly H, Bittner R E

机构信息

Neuromuscular Research Department, University of Vienna, Austria.

出版信息

Br J Anaesth. 1997 Jul;79(1):125-7. doi: 10.1093/bja/79.1.125.

Abstract

The association between malignant hyperthermia (MH) and neuromuscular disorders is controversial. An association between MH and Duchenne muscular dystrophy, a common and lethal disorder caused by deficiency of dystrophin, has been reported sporadically but is still not proved. To examine this problem, we performed halothane and caffeine in vitro contracture tests on skeletal muscles from dystrophin deficient mdx mice, an animal model for human Duchenne muscular dystrophy. As neither halothane nor caffeine triggered abnormal responses in mdx muscles, we conclude that dystrophin deficiency per se is not the primary cause of MH-like crises, as reported for patients with Duchenne muscular dystrophy.

摘要

恶性高热(MH)与神经肌肉疾病之间的关联存在争议。MH与杜氏肌营养不良症(一种由肌营养不良蛋白缺乏引起的常见致命疾病)之间的关联虽有零星报道,但仍未得到证实。为研究此问题,我们对肌营养不良蛋白缺陷的mdx小鼠(人类杜氏肌营养不良症的动物模型)的骨骼肌进行了氟烷和咖啡因体外挛缩试验。由于氟烷和咖啡因均未在mdx小鼠肌肉中引发异常反应,我们得出结论,如杜氏肌营养不良症患者报道的那样,肌营养不良蛋白缺乏本身并非MH样危象的主要原因。

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