Department of Animal Science, Iowa State University, Ames, IA.
Med Sci Sports Exerc. 2018 Sep;50(9):1723-1732. doi: 10.1249/MSS.0000000000001639.
Duchenne muscular dystrophy (DMD) is a neuromuscular disease caused by a dystrophin protein deficiency. Dystrophin functions to stabilize and protect the muscle fiber during muscle contraction; thus, the absence of functional dystrophin protein leads to muscle injury. DMD patients experience progressive muscle necrosis, loss of function, and ultimately succumb to respiratory failure or cardiomyopathy. Exercise is known to improve muscle health and strength in healthy individuals as well as positively affect other systems. Because of this, exercise has been investigated as a potential therapeutic approach for DMD.
This review aims to provide a concise presentation of the exercise literature with a focus on dystrophin-deficient muscle. Our intent was to identify trends and gaps in knowledge with an appreciation of exercise modality.
After compiling data from mouse and human studies, it became apparent that endurance exercises such as a swimming and voluntary wheel running have therapeutic potential in limb muscles of mice and respiratory training was beneficial in humans. However, in the comparatively few long-term investigations, the effect of low-intensity training on cardiac and respiratory muscles was contradictory. In addition, the effect of exercise on other systems is largely unknown.
To safely prescribe exercise as a therapy to DMD patients, multisystemic investigations are needed including the evaluation of respiratory and cardiac muscle.
杜氏肌营养不良症(DMD)是一种神经肌肉疾病,由肌营养不良蛋白缺乏引起。肌营养不良蛋白的功能是在肌肉收缩过程中稳定和保护肌肉纤维;因此,功能性肌营养不良蛋白的缺失会导致肌肉损伤。DMD 患者会经历进行性肌肉坏死、功能丧失,最终因呼吸衰竭或心肌病而死亡。运动已知可改善健康个体的肌肉健康和力量,并对其他系统产生积极影响。因此,运动已被研究作为 DMD 的一种潜在治疗方法。
本综述旨在简明介绍与肌营养不良蛋白缺乏肌肉相关的运动文献。我们的目的是确定运动方式方面的知识趋势和差距。
在编译了来自小鼠和人类研究的数据后,很明显,游泳和自愿轮跑等耐力运动对小鼠的肢体肌肉具有治疗潜力,呼吸训练对人类有益。然而,在为数不多的长期研究中,低强度训练对心脏和呼吸肌肉的影响是相互矛盾的。此外,运动对其他系统的影响在很大程度上尚不清楚。
为了安全地将运动作为 DMD 患者的治疗方法,需要进行多系统研究,包括对呼吸和心肌的评估。