Sleep Disorders in Adults with Prader-Willi Syndrome: Review of the Literature and Clinical Recommendations Based on the Experience of the French Reference Centre

Prader–Willi syndrome (PWS) is a rare, genetic, multisymptomatic, neurodevelopmental disease commonly associated with sleep alterations, including sleep-disordered breathing and central disorders of hypersomnolence. Excessive daytime sleepiness represents the main manifestation that should be addressed by eliciting the detrimental effects on quality of life and neurocognitive function from the patients’ caregivers. Patients with PWS have impaired ventilatory control and altered pulmonary mechanics caused by hypotonia, respiratory muscle weakness, scoliosis and obesity. Consequently, respiratory abnormalities are frequent and, in most cases, severe, particularly during sleep. Adults with PWS frequently suffer from sleep apnoea syndrome, sleep hypoxemia and sleep hypoventilation. When excessive daytime sleepiness persists after adequate control of sleep-disordered breathing, a sleep study on ventilatory treatment, followed by an objective measurement of excessive daytime sleepiness, is recommended. These tests frequently identify central disorders of hypersomnolence, including narcolepsy, central hypersomnia or a borderline hypersomnolent phenotype. The use of wake-enhancing drugs (modafinil, pitolisant) is discussed in multidisciplinary expert centres for these kinds of cases to ensure the right balance between the benefits on quality of life and the risk of psychological and cardiovascular side effects

Exercise and bariatric surgery: A systematic review and meta-analysis of the feasibility and acceptability of exercise and controlled trial methods

This systematic review and meta-analysis assessed the feasibility and acceptability of exercise and controlled trial methods in adults awaiting or having undergone bariatric surgery (BS). Search methods used to identify relevant articles were inclusion of articles identified in a systematic review, new database search of articles published 2019–2021, and hand searching reference lists. Titles/abstracts and full-texts were screened by two reviewers independently against inclusion criteria: adults awaiting or having undergone BS, controlled trial, exercise group compared with a comparison group without exercise. Twenty-eight articles were reviewed; most interventions were supervised, performed after BS, and lasted ≤13 weeks. Pooled data for exercise intervention attendance and dropout rates were 84% (k = 10) and 5% (k = 19), respectively, though possibly misestimated due to poor/selective reporting. Median study and recruitment duration were 18 weeks and 24 months, respectively, with a pooled enrollment rate of 2.5 participants/month. Pooled data for refusal to participate, enrollment, and retention rates were 23% (k = 16), 43% (k = 18), and 87% (k = 26), respectively. Despite the lack of data available in studies included, exercise and controlled trial methods seem feasible and acceptable for adults awaiting or having undergone BS. To better identify methodological or practical challenges, and assess bias, better reporting of feasibility and acceptability indicators is needed in future studies

Analyse de l’activité physique et du mouvement dans les pathologies cardiométaboliques : applications à l’évaluation et à l’intervention chez les patients obèses

Physical activity is an essential component in the management of patients with cardiometabolic diseases such as obesity, including patients undergoing bariatric surgery or patients with rare forms of obesity. The design and assessment of interventions specifically tailored to these patients requires accurately measuring habitual physical activity and the different dimensions of physical capacity.Our first aim was to assess the effectiveness of structured physical activity programs in obese patients undergoing bariatric surgery and in patients with Prader-Willi syndrome. Our second aim was first, to identify methods for the measurement of physical activity and cardiorespiratory and muscular fitness that could be used in obese patients and, second, to describe in detail habitual physical activity and the different dimensions of cardiorespiratory and muscular fitness (muscle strength and power, postural and dynamic stability) using these methods.Our results showed the feasibility and effectiveness of structured physical activity programs performed after bariatric surgery or in patients with Prader-Willi syndrome. Objective physical activity data collected in these patients showed sporadic physical activity patterns that did not correspond to current physical activity guidelines for obese patients. In addition, we have identified several methods for the assessment of muscular fitness adapted to obese patients. The data collected with these methods in candidates to bariatric surgery showed a decrease in muscle power, dynamic and postural stability and 6-minute walking distance with increasing body mass. However, independently of age and BMI, cardiorespiratory fitness and muscle strength were positively associated with dynamic stability and 6-minute walking distance, suggesting the importance of these two dimensions in the maintenance of physical function in patients with severe obesity.Our data contribute to a better description of habitual physical activity and movement capacity in obese patients, as an example of cardiometabolic diseases, and to a better understanding of the benefits of physical activity and the maintenance of physical capacity in these patients.Keywords: physical activity, movement analysis, obesity, evaluation, intervention, accelerometersL'activité physique (AP) est un élément essentiel de la prise en charge des patients présentant des pathologies cardiométaboliques, comme les patients obèses, y compris dans des situations spécifiques comme celle de la chirurgie bariatrique ou des obésités rares. La conception et l'évaluation d'interventions d'AP spécifiquement adaptées à ces patients impliquent de mesurer avec précision l'AP habituelle et les différentes dimensions de la capacité physique.Notre premier objectif était d'évaluer l’efficacité de programmes d’AP dans le contexte de la chirurgie bariatrique et dans celui des obésités rares (syndrome de Prader-Willi). Notre deuxième objectif consistait, d'une part, à identifier des méthodes de mesure de l’AP et des capacités cardiorespiratoire et musculaire pouvant être utilisées chez des patients obèses et, d'autre part, à décrire avec ces méthodes l'AP habituelle et les différentes dimensions de la capacité cardiorespiratoire et de la fonction musculaire (force et puissance musculaires, stabilité posturale et dynamique).Nos résultats ont montré la faisabilité et l'efficacité de programmes d'entraînement structurés réalisés après une chirurgie bariatrique ou chez des patients présentant un syndrome de Prader-Willi. Les données objectives d'AP recueillies chez ces patients ont mis en évidence des profils d'AP sporadiques très éloignés des recommandations actuelles d'AP pour les patients obèses. Par ailleurs, nos travaux ont permis d'identifier plusieurs méthodes d'évaluation de la fonction musculaire adaptées aux patients obèses. Les données recueillies avec ces différentes méthodes chez des patients candidats à la chirurgie bariatrique ont montré une diminution de la puissance musculaire, de la stabilité dynamique et posturale et de la distance parcourue en 6 minutes avec l'augmentation de la corpulence. Cependant, indépendamment de l'âge et de l'IMC, la capacité cardiorespiratoire et la force musculaire étaient associées à une augmentation de la stabilité dynamique et de la distance parcourue en 6 minutes, suggérant l'importance de ces deux dimensions dans le maintien de la capacité physique fonctionnelle chez des patients sévèrement obèses.Nos travaux contribuent à une meilleure description de l'AP habituelle et de la capacité de mouvement chez les sujets obèses et à une meilleure compréhension des bénéfices de l'AP et du maintien de la capacité physique chez ces patients.Mots clés : activité physique, analyse du mouvement, obésité, évaluation, intervention, accéléromètre

Physical activity phenotyping and movement analysis in cardiometabolic diseases

L'activité physique (AP) est un élément essentiel de la prise en charge des patients présentant des pathologies cardiométaboliques, comme les patients obèses, y compris dans des situations spécifiques comme celle de la chirurgie bariatrique ou des obésités rares. La conception et l'évaluation d'interventions d'AP spécifiquement adaptées à ces patients impliquent de mesurer avec précision l'AP habituelle et les différentes dimensions de la capacité physique.Notre premier objectif était d'évaluer l’efficacité de programmes d’AP dans le contexte de la chirurgie bariatrique et dans celui des obésités rares (syndrome de Prader-Willi). Notre deuxième objectif consistait, d'une part, à identifier des méthodes de mesure de l’AP et des capacités cardiorespiratoire et musculaire pouvant être utilisées chez des patients obèses et, d'autre part, à décrire avec ces méthodes l'AP habituelle et les différentes dimensions de la capacité cardiorespiratoire et de la fonction musculaire (force et puissance musculaires, stabilité posturale et dynamique).Nos résultats ont montré la faisabilité et l'efficacité de programmes d'entraînement structurés réalisés après une chirurgie bariatrique ou chez des patients présentant un syndrome de Prader-Willi. Les données objectives d'AP recueillies chez ces patients ont mis en évidence des profils d'AP sporadiques très éloignés des recommandations actuelles d'AP pour les patients obèses. Par ailleurs, nos travaux ont permis d'identifier plusieurs méthodes d'évaluation de la fonction musculaire adaptées aux patients obèses. Les données recueillies avec ces différentes méthodes chez des patients candidats à la chirurgie bariatrique ont montré une diminution de la puissance musculaire, de la stabilité dynamique et posturale et de la distance parcourue en 6 minutes avec l'augmentation de la corpulence. Cependant, indépendamment de l'âge et de l'IMC, la capacité cardiorespiratoire et la force musculaire étaient associées à une augmentation de la stabilité dynamique et de la distance parcourue en 6 minutes, suggérant l'importance de ces deux dimensions dans le maintien de la capacité physique fonctionnelle chez des patients sévèrement obèses.Nos travaux contribuent à une meilleure description de l'AP habituelle et de la capacité de mouvement chez les sujets obèses et à une meilleure compréhension des bénéfices de l'AP et du maintien de la capacité physique chez ces patients.Mots clés : activité physique, analyse du mouvement, obésité, évaluation, intervention, accéléromètresPhysical activity is an essential component in the management of patients with cardiometabolic diseases such as obesity, including patients undergoing bariatric surgery or patients with rare forms of obesity. The design and assessment of interventions specifically tailored to these patients requires accurately measuring habitual physical activity and the different dimensions of physical capacity.Our first aim was to assess the effectiveness of structured physical activity programs in obese patients undergoing bariatric surgery and in patients with Prader-Willi syndrome. Our second aim was first, to identify methods for the measurement of physical activity and cardiorespiratory and muscular fitness that could be used in obese patients and, second, to describe in detail habitual physical activity and the different dimensions of cardiorespiratory and muscular fitness (muscle strength and power, postural and dynamic stability) using these methods.Our results showed the feasibility and effectiveness of structured physical activity programs performed after bariatric surgery or in patients with Prader-Willi syndrome. Objective physical activity data collected in these patients showed sporadic physical activity patterns that did not correspond to current physical activity guidelines for obese patients. In addition, we have identified several methods for the assessment of muscular fitness adapted to obese patients. The data collected with these methods in candidates to bariatric surgery showed a decrease in muscle power, dynamic and postural stability and 6-minute walking distance with increasing body mass. However, independently of age and BMI, cardiorespiratory fitness and muscle strength were positively associated with dynamic stability and 6-minute walking distance, suggesting the importance of these two dimensions in the maintenance of physical function in patients with severe obesity.Our data contribute to a better description of habitual physical activity and movement capacity in obese patients, as an example of cardiometabolic diseases, and to a better understanding of the benefits of physical activity and the maintenance of physical capacity in these patients.Keywords: physical activity, movement analysis, obesity, evaluation, intervention, accelerometer

Prescribing of Electronic Activity Monitors in Cardiometabolic Diseases: Qualitative Interview-Based Study

International audienceBackground: The prevalence of noncommunicable diseases, including those such as type 2 diabetes, obesity, dyslipidemia, and hypertension, so-called cardiometabolic diseases, is high and is increasing worldwide. Strong evidence supports the role of physical activity in management of these diseases. There is general consensus that mHealth technology, including electronic activity monitors, can potentially increase physical activity in patients, but their use in clinical settings remains limited. Practitioners’ requirements when prescribing electronic activity monitors have been poorly described.Objective: The aims of this qualitative study were (1) to explore how specialist physicians prescribe electronic activity monitors to patients presenting with cardiometabolic conditions, and (2) to better understand their motivation for and barriers to prescribing such monitors.Methods: We conducted qualitative semistructured interviews in March to May 2016 with 11 senior physicians from a public university hospital in France with expertise in management of cardiometabolic diseases (type 1 and type 2 diabetes, obesity, hypertension, and dyslipidemia). Interviews lasted 45 to 60 minutes and were audiotaped, transcribed verbatim, and analyzed using directed content analysis. We report our findings following the Consolidated Criteria for Reporting Qualitative Research (COREQ) checklist.Results: Most physicians we interviewed had never prescribed electronic activity monitors, whereas they frequently prescribed blood glucose or blood pressure self-monitoring devices. Reasons for nonprescription included lack of interest in the data collected, lack of evidence for data accuracy, concern about work overload possibly resulting from automatic data transfer, and risk of patients becoming addicted to data. Physicians expected future marketing of easy-to-use monitors that will accurately measure physical activity duration and intensity and provide understandable motivating feedback.Conclusions: Features of electronic activity monitors, although popular among the general public, do not meet the needs of physicians. In-depth understanding of physicians’ expectations is a first step toward designing technologies that can be widely used in clinical settings and facilitate physical activity prescription. Physicians should have a role, along with key health care stakeholders—patients, researchers, information technology firms, the public, and private payers—in developing the most effective methods for integrating activity monitors into patient care

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

International audiencePhysical 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 setting

Changes in Cardiorespiratory Fitness After Gastric Bypass: Relations with Accelerometry-Assessed Physical Activity

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Vertical Jump on a Force Plate for Assessing Muscle Strength and Power in Women With Severe Obesity: Reliability, Validity, and Relations With Body Composition

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Stair-use interventions in worksites and public settings - a systematic review of effectiveness and external validity

International audienceOBJECTIVE: We performed a literature review with the main aims to propose an updated overview of the effectiveness of stair-use interventions and to determine the most effective type of intervention. METHODS: We systematically searched stair-use interventions performed in worksites or public settings, published up to mid 2013. We used a harvest plot approach to visualize the findings in addition to a quantitative synthesis. We also assessed external validity using the Reach, Efficacy, Adoption, Implementation, Maintenance (RE-AIM) framework. RESULTS: Of 8571 articles identified, 50 were included. In worksites (25 studies) and public settings (35 studies), an increase in stair climbing was found during the intervention period in 64% and 76% of studies, respectively. Combining motivational and directional signs in worksites or conducting a second intervention phase in public settings increased stair climbing in 83% and 86% of studies, respectively. Elements of external validity were overall largely under-reported. CONCLUSION: There is evidence that stair-use interventions are effective to increase stair climbing in public settings, but evidence of such effect is limited in worksites. Issues regarding the best sequencing of interventions or the potential importance of environmental interventions should be addressed in future studies. Process evaluation should be an integral part of interventions

Effect of exercise training after bariatric surgery: A 5-year follow-up study of a randomized controlled trial

International audienceBackground and objectives We previously showed in a 6-month randomized controlled trial that resistance training and protein supplementation after bariatric surgery (Roux-en-Y gastric bypass, RYGB) improved muscle strength without significant effect on weight loss and body composition changes. We performed a 5-year follow-up study in these subjects with the aim 1) to assess the longterm effect of this exercise training intervention and 2) to analyze associations between habitual physical activity (PA) and weight regain at 5 years