Assistance Publique-Hôpitaux de Paris, Centre de Référence Maladies Rares (PRADORT, Syndrome de Prader-Willi Et Autres Formes Rares D'Obésité Avec Troubles du Comportement Alimentaire), Service de Nutrition, Groupe Hospitalier Pitié-Salpêtrière, Hôpital Pitié-Salpêtrière, Sorbonne Université, 91 bd de l'Hôpital, Bâtiment E3M, 75013, Paris, France.
INSERM, Nutrition et Obésité: Approches Systémiques «NutriOmics», Sorbonne Université, Paris, France.
Orphanet J Rare Dis. 2021 May 5;16(1):202. doi: 10.1186/s13023-021-01833-1.
Prader-Willi syndrome (PWS) is a neurodevelopmental disorder with hypothalamic dysfunction leading to obesity and behavioral disabilities, including eating disorders (EDs). We evaluated the effects of the COVID-19 infection and lockdown on mental and physical health in PWS. At the end of April, 85 adults with PWS completed a self-administered questionnaire, including lockdown conditions, physical activity (PA), ED, and medical and behavioral outcomes. Body weight was measured at home and self-reported.
Patients (52.9% women, 44.8% disomic) were assessed, with a mean age of 28.05 ± 8.73 years and body mass index (BMI) of 36.76 ± 10.74 kg/m. Seventy percent lived in the Paris region (France) and were confined with their parents. The mean weight change was 0.96 ± 3.28 kg. We compared patients showing weight loss (n = 39, - 3.30 ± 2.93 kg) to patients showing weight gain (n = 22, + 2.35 ± 1.54 kg): the BMI was lower (34.60 ± 9.18 versus 40.45 ± 9.45 kg/m, p = 0.02), PA increased (25.6% versus 4.5%, p = 0.04), and EDs improved (51.3% versus 13.6%, p = 0.005). Behavioral disorders increased for 12.9% of the cohort. Three individuals (3.5%) were diagnosed with non-severe COVID-19.
Lockdown during the COVID-19 pandemic was associated with positive effects for most French adults with PWS, with weight loss probably associated with a more favourable environment during this period. We observed no severe forms of COVID-19.
普拉德-威利综合征(PWS)是一种神经发育障碍,下丘脑功能障碍导致肥胖和行为障碍,包括饮食失调(EDs)。我们评估了 COVID-19 感染和封锁对 PWS 患者心理健康和身体健康的影响。四月底,85 名成年 PWS 患者完成了一份自我管理问卷,包括封锁条件、身体活动(PA)、ED 和医疗及行为结果。在家中测量体重并自我报告。
患者(52.9%女性,44.8%二倍体)平均年龄 28.05±8.73 岁,体重指数(BMI)为 36.76±10.74kg/m。70%患者居住在法国巴黎地区,与父母一起被隔离。体重平均变化为 0.96±3.28kg。我们比较了体重减轻(n=39,-3.30±2.93kg)和体重增加(n=22,+2.35±1.54kg)的患者:BMI 更低(34.60±9.18 与 40.45±9.45kg/m,p=0.02),PA 增加(25.6%与 4.5%,p=0.04),ED 改善(51.3%与 13.6%,p=0.005)。该队列中有 12.9%的患者出现行为障碍增加。有 3 人(3.5%)被诊断为非重症 COVID-19。
COVID-19 大流行期间的封锁对法国大多数成年 PWS 患者产生了积极影响,体重减轻可能与这一时期更有利的环境有关。我们没有观察到严重的 COVID-19 病例。