含 anoctamin 5 型肌病和高肌酸激酶血症患者的有氧训练
Aerobic training in patients with anoctamin 5 myopathy and hyperckemia.
作者信息
Vissing Christoffer R, Preisler Nicolai, Husu Edith, Prahm Kira P, Vissing John
机构信息
Neuromuscular Research Unit, Department of Neurology, Section 3342, Rigshospitalet, University of Copenhagen, Blegdamsvej 9, DK-2100, Copenhagen, Denmark.
出版信息
Muscle Nerve. 2014 Jul;50(1):119-23. doi: 10.1002/mus.24112. Epub 2014 May 5.
INTRODUCTION
Anoctamin 5 deficiency has recently been defined to cause limb-girdle muscular dystrophy type 2L (LGMD2L) with pronounced hyperCKemia. No treatment interventions have been made so far in this condition.
METHODS
In 6 patients with LGMD2L, we studied the effect of home-based, pulse-watch monitored, moderate-intensity exercise on a cycle ergometer for 30 minutes, 3 times weekly, for 10 weeks. Plasma creatine kinase (CK) was assessed before, during, and after the program as a marker of muscle damage. Primary outcome measures were maximum oxygen uptake (VO(2max)) and time in the 5-repetitions-sit-to-stand test (FRSTST).
RESULTS
Training resulted in improvements in VO(2max) (27 ± 7%; P = 0.0001) and FRSTST time (35 ± 12%; P = 0.007). Improvements in physiologic and functional muscle testing were accompanied by stable CK levels and no reports of adverse effects.
CONCLUSIONS
These findings suggest that supervised aerobic exercise training is safe and effective in improving oxidative capacity and muscle function in patients with anoctamin 5 deficiency.
引言
最近已明确anoctamin 5缺乏会导致伴有明显高肌酸激酶血症的2L型肢带型肌营养不良（LGMD2L）。目前针对这种情况尚未进行治疗干预。
方法
在6例LGMD2L患者中，我们研究了在家中通过脉搏手表监测，在自行车测力计上进行30分钟中等强度运动的效果，每周3次，共10周。在该计划实施前、期间和之后评估血浆肌酸激酶（CK）作为肌肉损伤的标志物。主要结局指标是最大摄氧量（VO₂max）和5次坐立试验（FRSTST）的时间。
结果
训练使VO₂max提高（27±7%；P = 0.0001），FRSTST时间缩短（35±12%；P = 0.007）。生理和功能性肌肉测试的改善伴随着CK水平稳定，且无不良反应报告。
结论
这些发现表明，有监督的有氧运动训练对于改善anoctamin 5缺乏患者的氧化能力和肌肉功能是安全有效的。
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