Faculty of Sport Sciences, Universidad Europea de Madrid, Madrid, Spain.
Physiology Unit, Systems Biology Department, University of Alcalá, Madrid, Spain.
Clin Endocrinol (Oxf). 2019 May;90(5):649-661. doi: 10.1111/cen.13953. Epub 2019 Mar 18.
The aim of this systematic review was to summarize evidence on the acute responses of individuals with Prader-Willi syndrome (PWS) to physical exercise, and on the effectiveness of long-term exercise interventions to improve the clinical manifestations of this syndrome.
DESIGN/METHODS: Relevant articles were identified in the electronic databases PubMed, Medline, CINAHL and SPORTDiscus (from inception to December 2018). Twenty-two studies including a total of 356 patients with PWS met all inclusion criteria and were included in the review.
Patients with PWS present with a decreased physical performance and impaired cardiorespiratory (maximal oxygen consumption, heart rate recovery after exercise) and hormonal (growth hormone release) responses to exercise. Most long-term exercise interventions have proven to decrease body mass while improving physical performance. Some benefits have also been reported in biochemical (glucose homeostasis, lipid profile) and biomechanical (gait pattern) variables, although there is controversy regarding the effects on body composition. No exercise-related adverse events have been reported in patients with PWS.
Physical exercise seems to be safe and effective for improving several phenotypes in PWS, notably physical fitness. However, further research is needed to confirm these results and especially to corroborate whether exercise per se or combined with dietary intervention is an effective coadjuvant treatment for reducing body mass in these patients.
本系统综述旨在总结个体普拉德-威利综合征（PWS）对运动的急性反应的证据，以及长期运动干预对改善该综合征临床症状的有效性。
设计/方法：在电子数据库 PubMed、Medline、CINAHL 和 SPORTDiscus 中（从创建到 2018 年 12 月）确定相关文章。22 项研究共纳入 356 例 PWS 患者，均符合所有纳入标准，并纳入本综述。
PWS 患者的身体表现下降，心肺（最大摄氧量、运动后心率恢复）和激素（生长激素释放）对运动的反应受损。大多数长期运动干预已被证明可降低体重，同时提高身体表现。在生化（血糖稳态、血脂谱）和生物力学（步态模式）变量方面也有一些益处的报道，尽管关于对身体成分的影响存在争议。在 PWS 患者中，没有报告与运动相关的不良事件。
运动似乎安全且有效，可以改善 PWS 的多种表型，尤其是身体适应性。然而，需要进一步的研究来证实这些结果，特别是要证实运动本身或与饮食干预相结合是否是减轻这些患者体重的有效辅助治疗方法。
