Bellicha Alice, Coupaye Muriel, Mosbah Héléna, Tauber Maithé, Oppert Jean-Michel, Poitou Christine
INSERM, Nutrition and Obesities: Systemic Approaches (NutriOmics), Research Unit, Sorbonne University, F-75013 Paris, France.
Assistance Publique-Hôpitaux de Paris (AP-HP), Pitié-Salpêrière Hospital, Department of Nutrition, Rare Diseases Center of Reference 'Prader-Willi Syndrome and Obesity with Eating Disorders' (PRADORT), Sorbonne University, F-75013 Paris, France.
J Clin Med. 2021 Jun 7;10(11):2528. doi: 10.3390/jcm10112528.
Physical activity (PA) is an important aspect of the management of patients with Prader-Willi syndrome (PWS). However, the day-to-day implementation of PA programs is particularly challenging in these patients. This systematic review aimed (1) to describe habitual PA and sedentary behavior and (2) to assess the effects of PA interventions and to describe their implementation process, in children and adults with PWS. A systematic search of controlled trials, single-group interventions, observational, and qualitative studies published up to December 2020 was performed. Twenty-five studies were included. Habitual PA was found to be lower in patients with PWS compared to controls without obesity or with non-syndromic obesity. Habitual PA was positively associated with lean body mass and bone parameters in children with PWS, and these finding were strengthened by intervention studies reporting an increase in both outcomes after a PA program. PA programs also improved physical function (muscle strength, walking distance, and coordination), without significant effect on weight and fat mass. Attendance to exercise sessions was usually high and no serious adverse effect was reported. In conclusion, supervised PA programs are beneficial for children and adults with PWS. Support should be provided to families to facilitate their implementation in real-life settings.
体育活动（PA）是普拉德-威利综合征（PWS）患者管理的一个重要方面。然而，对这些患者而言，日常实施体育活动计划极具挑战性。本系统评价旨在（1）描述习惯性体育活动和久坐行为，以及（2）评估体育活动干预的效果并描述其实施过程，涉及患有PWS的儿童和成人。对截至2020年12月发表的对照试验、单组干预、观察性和定性研究进行了系统检索。纳入了25项研究。结果发现，与无肥胖症或非综合征性肥胖的对照组相比，PWS患者的习惯性体育活动水平较低。在患有PWS的儿童中，习惯性体育活动与瘦体重和骨骼参数呈正相关，而干预研究报告称体育活动计划后这两个结果均有所增加，进一步强化了这些发现。体育活动计划还改善了身体功能（肌肉力量、步行距离和协调性），对体重和脂肪量无显著影响。锻炼课程的出勤率通常较高，且未报告严重不良反应。总之，有监督的体育活动计划对患有PWS的儿童和成人有益。应向家庭提供支持，以促进其在现实生活环境中的实施。
