Physical Activity in Patients with Prader-Willi Syndrome-A Systematic Review of Observational and Interventional Studies.

Alice Bellicha,Alice Bellicha,Alice Bellicha,Jean-Michel Oppert,Jean-Michel Oppert,Christine Poitou,Christine Poitou,Christine Poitou,Christine Poitou,Muriel Coupaye,Muriel Coupaye,Héléna Mosbah,Héléna Mosbah,Maithé Tauber

doi:10.3390/jcm10112528

Abstract

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.

Highlights

Prader-Willi syndrome (PWS) is the most frequent cause of genetic obesity [1], with a prevalence between one in 20,000 and one in 30,000 births [2]
We have no explanation for this observation, the deficit in lean body mass in patients with PWS compared to people of similar corpulence may explain, at least in part, the lower level of physical activity (PA) observed in patients with PWS
The data included in this review suggest that reaching the recommended level of at least moderate-intensity PA may be challenging for patients with low physical fitness such as patients with PWS [3]

Summary

Introduction

Prader-Willi syndrome (PWS) is the most frequent cause of genetic obesity [1], with a prevalence between one in 20,000 and one in 30,000 births [2]. PWS is a complex genetic neurodevelopmental disorder caused by an absence of expression of imprinted alleles of paternal origin on chromosome 15 [1]. Patients with PWS display low to moderate intellectual disability, as well as decreased motor competencies and physical fitness [1,3]. Promoting physical activity (PA) is an important objective of the management of PWS in both children and adults [5]. Less than 10% of children with PWS reach the recommended level of PA [6,7], and this proportion does not exceed 20% in adults [8,9]

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Discussion

Conclusion