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运动与贝克型肌营养不良症:喜忧参半。

Motor activity and Becker's muscular dystrophy: lights and shadows.

机构信息

Department of Surgery and Medical-Surgical Specialties, University of Catania, Catania, Italy.

Department of Neurology IC, Oasi Research Institute - IRCCS, Troina, Italy.

出版信息

Phys Sportsmed. 2020 May;48(2):151-160. doi: 10.1080/00913847.2019.1684810. Epub 2019 Oct 30.

Abstract

Becker's disease is an inherited muscular dystrophy caused by mutations in the gene coding for the dystrophin protein that leads to quantitative and/or qualitative protein dysfunction and consequent muscle degeneration. Studies in animal models demonstrate that, while eccentric or high-intensity training are deleterious for dystrophic muscles, low-intensity aerobic training may slowdown the disease process and progression. Based on these preclinical data, the available studies in patients with Becker's muscular dystrophy undergoing workout on a cycle ergometer or on a treadmill, at a heart rate ≤65% of their maximal oxygen uptake, showed that aerobic exercise counteracts physical deterioration and loss of functional abilities. These findings suggest an improvement of physical performance through an increase of muscle strength, fatigue resistance, and dexterity capacities, without substantial evidence of acceleration of muscular damage progression. Therefore, individually tailored mild-to-moderate intensity aerobic exercise should be considered as part of the management of these patients. However, further research is necessary to define specific and standardized guidelines for the prescription of type, intensity, frequency, and duration of motor activities. In this review, we provided a summary of the impact of physical activity both in animal models and in patients with Becker's muscular dystrophy, with the intent to identify trends and gaps in knowledge. The potential therapeutic implications and future research directions have been also highlighted.

摘要

贝克氏病是一种遗传性肌肉萎缩症,由编码肌营养不良蛋白的基因突变引起,导致蛋白质功能定量和/或定性异常,进而导致肌肉退化。动物模型研究表明,虽然离心或高强度训练对萎缩肌肉有害,但低强度有氧运动可能会减缓疾病进程和进展。基于这些临床前数据,对接受自行车测力计或跑步机运动的贝克氏肌肉萎缩症患者进行的现有研究表明,有氧运动可以抵抗身体恶化和功能丧失。这些发现表明,通过增加肌肉力量、疲劳抵抗力和灵巧能力,可改善身体表现,而肌肉损伤进展加速的证据并不充分。因此,应将个体化的轻度至中度强度有氧运动视为这些患者管理的一部分。然而,需要进一步的研究来确定运动活动的类型、强度、频率和持续时间的具体和标准化指南。在这篇综述中,我们总结了运动活动对动物模型和贝克氏肌肉萎缩症患者的影响,旨在确定知识的趋势和差距。还强调了潜在的治疗意义和未来的研究方向。

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