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贝克尔肌营养不良症中血管平滑肌的肾上腺素能受体反应。一项使用133Xe清除法的肌肉血流研究。

Adrenergic receptor responses of vascular smooth muscle in Becker dystrophy. A muscle blood flow study using the 133Xe clearance method.

作者信息

Mechler F, Mastaglia F L, Haggith J, Gardner-Medwin D

出版信息

J Neurol Sci. 1980 Jun;46(3):291-302. doi: 10.1016/0022-510x(80)90053-2.

Abstract

The effect of intravenous adrenaline and of alpha and beta blockade on muscle blood flow in the tibialis anterior muscle of 8 normal subjects and 8 patients with the Becker form of X-linked muscular dystrophy has been assessed using the 133Xe clearance method. Whereas beta receptor responses to stimulation by adrenaline and blocking by propranolol did not differ significantly in the Becker patients and normal controls, the degree of alpha receptor blockade produced by phentolamine was significantly less in the Becker patients, suggesting a quantitative or qualitative abnormality of vascular alpha receptors. This pattern of response was not found in patients with spinal muscular atrophy or polymyositis. The possible significance of these findings is considered.

摘要

采用¹³³Xe清除法评估了静脉注射肾上腺素以及α和β受体阻断对8名正常受试者和8名贝克型X连锁肌营养不良患者胫前肌肌肉血流的影响。尽管贝克型患者和正常对照组对肾上腺素刺激和普萘洛尔阻断的β受体反应没有显著差异,但酚妥拉明产生的α受体阻断程度在贝克型患者中明显较低,提示血管α受体存在数量或质量异常。脊髓性肌萎缩症或多发性肌炎患者未发现这种反应模式。文中考虑了这些发现的可能意义。

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