Safety and efficacy of a 6-month home-based exercise program in patients with facioscapulohumeral muscular dystrophy A randomized controlled trial
Safety and efficacy of a 6-month home-based exercise program in patients with facioscapulohumeral muscular dystrophy A randomized controlled trial
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DOI：
10.1097/md.0000000000004497
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发表时间：
2016-08-01
期刊：
影响因子：
1.6
通讯作者：
Feasson, Leonard
中科院分区：
文献类型：
--
作者：
Bankole, Landry-Cyrille;Millet, Guillaume Y.;Feasson, Leonard
背景：以往研究面肩肩周型肌营养不良症(FSHD)患者运动训练方案的随机对照试验很少，而且持续时间很短。本研究评估了为期6个月的家庭运动训练方案对FSHD患者的体能、肌肉和运动功能的安全性和有效性。方法：将16例FSHD患者随机分为训练组和对照组，每组8例。训练包括每周骑自行车3次，每次35分钟(力量、高强度间歇和低强度有氧运动相结合)，持续24周。对照组的患者在24周后也进行了相同的训练计划(CTG)。主要结果是每6周测量一次最大摄氧量(VO2峰值)的变化。主要的次要结果是每12周最大股四头肌力量(MVC)和局部股四头肌耐力。其他观察指标包括最大有氧功率(MAP)和每6周一次的疲劳，每12周6分钟步行距离，以及干预前后股外侧肌的肌肉特征。结果：TG组的依从性为91%。随着训练的进行，VO2峰值(+19%，P=0.002)和MAP在第6周和第24周有显著改善。肌肉耐力、MVC和6分钟步行距离增加，疲劳感减少。肌纤维横截面积和柠檬酸合成酶活性分别提高34%(P=0.008)和46%(P=0.003)。营养不良的病理生理模式没有恶化。TG和CTG也有类似的改善。结论：与患者日常专业和社会活动相适应的力量和间歇性自行车运动训练计划可在不损害肌肉组织的情况下产生显著的功能益处。
Background: Previous randomized controlled trials investigating exercise training programs in facioscapulohumeral muscular dystrophy (FSHD) patients are scarce and of short duration only. This study assessed the safety and efficacy of a 6-month home-ased exercise training program on fitness, muscle, and motor function in FSHD patients.Methods: Sixteen FSHD patients were randomly assigned to training (TG) and control (CG) groups (both n=8) in a home-based exercise intervention. Training consisted of cycling 3 times weekly for 35minutes (combination of strength, high-intensity interval, and low-intensity aerobic) at home for 24 weeks. Patients in CG also performed an identical training program (CTG) after 24 weeks. The primary outcome was change in peak oxygen uptake (VO2 peak) measured every 6 weeks. The principal secondary outcomes were maximal quadriceps strength (MVC) and local quadriceps endurance every 12 weeks. Other outcome measures included maximal aerobic power (MAP) and experienced fatigue every 6 weeks, 6-minute walking distance every 12 weeks, and muscle characteristics from vastus lateralis biopsies taken pre- and postintervention.Results: The compliance rate was 91% in TG. Significant improvements with training were observed in the VO2 peak (+19%, P= 0.002) and MAP by week 6 and further to week 24. Muscle endurance, MVC, and 6-minute walking distance increased and experienced fatigue decreased. Muscle fiber cross-sectional area and citrate synthase activity increased by 34% (P=0.008) and 46% (P=0.003), respectively. Dystrophic pathophysiologic patterns were not exacerbated. Similar improvements were experienced by TG and CTG.Conclusions: A combined strength and interval cycling exercise-training program compatible with patients' daily professional and social activities leads to significant functional benefits without compromising muscle tissue.