PDF(Pubmed)
Abstract：
BACKGROUND: Management of muscular dystrophies (MD) relies on conservative non-pharmacological treatments, but evidence of their effectiveness is limited and inconclusive.
OBJECTIVE: To investigate the effectiveness of conservative non-pharmacological interventions for MD physical management.
METHODS: This systematic review and meta-analysis followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and searched Medline, CINHAL, Embase, AMED and Cochrane Central Register of Controlled Trial (inception to August 2022). Effect size (ES) and 95% Confidence Interval (CI) quantified treatment effect.
RESULTS: Of 31,285 identified articles, 39 studies (957 participants), mostly at high risk of bias, were included. For children with Duchenne muscular dystrophy (DMD), trunk-oriented strength exercises and usual care were more effective than usual care alone in improving distal upper-limb function, sitting and dynamic reaching balance (ES range: 0.87 to 2.29). For adults with Facioscapulohumeral dystrophy (FSHD), vibratory proprioceptive assistance and neuromuscular electrical stimulation respectively improved maximum voluntary isometric contraction and reduced pain intensity (ES range: 1.58 to 2.33). For adults with FSHD, Limb-girdle muscular dystrophy (LGMD) and Becker muscular dystrophy (BMD), strength-training improved dynamic balance (sit-to-stand ability) and self-perceived physical condition (ES range: 0.83 to 1.00). A multicomponent programme improved perceived exertion rate and gait in adults with Myotonic dystrophy type 1 (DM1) (ES range: 0.92 to 3.83).
CONCLUSIONS: Low-quality evidence suggests that strength training, with or without other exercise interventions, may improve perceived exertion, distal upper limb function, static and dynamic balance, gait and well-being in MD. Although more robust and larger studies are needed, current evidence supports the inclusion of strength training in MD treatment, as it was found to be safe.
OBJECTIVE: To investigate the effectiveness of conservative non-pharmacological interventions for MD physical management.
METHODS: This systematic review and meta-analysis followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and searched Medline, CINHAL, Embase, AMED and Cochrane Central Register of Controlled Trial (inception to August 2022). Effect size (ES) and 95% Confidence Interval (CI) quantified treatment effect.
RESULTS: Of 31,285 identified articles, 39 studies (957 participants), mostly at high risk of bias, were included. For children with Duchenne muscular dystrophy (DMD), trunk-oriented strength exercises and usual care were more effective than usual care alone in improving distal upper-limb function, sitting and dynamic reaching balance (ES range: 0.87 to 2.29). For adults with Facioscapulohumeral dystrophy (FSHD), vibratory proprioceptive assistance and neuromuscular electrical stimulation respectively improved maximum voluntary isometric contraction and reduced pain intensity (ES range: 1.58 to 2.33). For adults with FSHD, Limb-girdle muscular dystrophy (LGMD) and Becker muscular dystrophy (BMD), strength-training improved dynamic balance (sit-to-stand ability) and self-perceived physical condition (ES range: 0.83 to 1.00). A multicomponent programme improved perceived exertion rate and gait in adults with Myotonic dystrophy type 1 (DM1) (ES range: 0.92 to 3.83).
CONCLUSIONS: Low-quality evidence suggests that strength training, with or without other exercise interventions, may improve perceived exertion, distal upper limb function, static and dynamic balance, gait and well-being in MD. Although more robust and larger studies are needed, current evidence supports the inclusion of strength training in MD treatment, as it was found to be safe.
摘要：
背景：肌营养不良（MD）的治疗依赖于保守的非药物治疗，但其有效性的证据有限且尚无定论。
目的：探讨非药物保守干预措施对MD身体管理的有效性。
方法：本系统评价和荟萃分析遵循系统评价和荟萃分析（PRISMA）指南的首选报告项目，并搜索Medline，CINHAL,Embase,AMED和Cochrane对照试验中央登记册(始于2022年8月)。效应大小(ES)和95%置信区间(CI)量化治疗效果。
结果：在31,285篇确定的文章中，39项研究（957名参与者），主要是偏见的高风险，包括在内。对于患有Duchenne肌营养不良症（DMD）的儿童，以躯干为导向的力量锻炼和常规护理在改善上肢远端功能方面比单纯常规护理更有效，坐姿和动态平衡（ES范围：0.87至2.29）。对于患有面肩肱骨营养不良（FSHD）的成年人，振动本体感觉辅助和神经肌肉电刺激分别改善了最大自愿性等距收缩并降低了疼痛强度（ES范围：1.58至2.33）。对于患有FSHD的成年人，四肢肌营养不良（LGMD）和贝克尔肌营养不良（BMD），力量训练改善了动态平衡（坐立能力）和自我感知的身体状况（ES范围：0.83至1.00）。多组分程序可改善1型肌强直性营养不良（DM1）成人的感知劳累率和步态（ES范围：0.92至3.83）。
结论：低质量证据表明力量训练，有或没有其他运动干预，可以改善感知的劳累，上肢远端功能，静态和动态平衡，步态和健康在MD。虽然需要更强大和更大的研究，目前的证据支持将力量训练纳入MD治疗，因为它被发现是安全的。
目的：探讨非药物保守干预措施对MD身体管理的有效性。
方法：本系统评价和荟萃分析遵循系统评价和荟萃分析（PRISMA）指南的首选报告项目，并搜索Medline，CINHAL,Embase,AMED和Cochrane对照试验中央登记册(始于2022年8月)。效应大小(ES)和95%置信区间(CI)量化治疗效果。
结果：在31,285篇确定的文章中，39项研究（957名参与者），主要是偏见的高风险，包括在内。对于患有Duchenne肌营养不良症（DMD）的儿童，以躯干为导向的力量锻炼和常规护理在改善上肢远端功能方面比单纯常规护理更有效，坐姿和动态平衡（ES范围：0.87至2.29）。对于患有面肩肱骨营养不良（FSHD）的成年人，振动本体感觉辅助和神经肌肉电刺激分别改善了最大自愿性等距收缩并降低了疼痛强度（ES范围：1.58至2.33）。对于患有FSHD的成年人，四肢肌营养不良（LGMD）和贝克尔肌营养不良（BMD），力量训练改善了动态平衡（坐立能力）和自我感知的身体状况（ES范围：0.83至1.00）。多组分程序可改善1型肌强直性营养不良（DM1）成人的感知劳累率和步态（ES范围：0.92至3.83）。
结论：低质量证据表明力量训练，有或没有其他运动干预，可以改善感知的劳累，上肢远端功能，静态和动态平衡，步态和健康在MD。虽然需要更强大和更大的研究，目前的证据支持将力量训练纳入MD治疗，因为它被发现是安全的。
