Abstract
To reduce transmission of the coronavirus disease 2019 (COVID-19), many countries implemented lockdowns, causing the closure of childcare services. This study was designed to evaluate the impact of the COVID-19 lockdown in March–April 2020 on children, adolescents, and young adults with Prader–Willi syndrome (PWS) living in Germany. We recruited 180 participants with a genetically confirmed PWS. All families completed a questionnaire, and participants underwent a post-lockdown assessment; the last examination before the lockdown was determined as the pre-lockdown assessment. We used bivariate analyses to compare pre- and post-lockdown outcomes. Weight standard deviation scores (SDSPWS) and body mass index (BMI)-SDSPWS remained stable or even decreased in some age groups. A statistically significant gain in lean body mass (LBM) was found in all groups <18 years of age. We observed an increase in IGF-I and IGFBP-3 concentrations without a significant change in growth hormone (GH) dosage. Most families (95.4%) reported set mealtimes and implementation of structured activities (72.2%) during the lockdown period. We therefore suggest that the favorable development of weight/BMI and LBM was caused by an interplay of a suspected enhanced GH administration and continuous parental commitment. However, more intense behavioral problems were observed in 45.7%, which persisted post-lockdown in 33.7%.
Highlights
Prader–Willi syndrome (PWS) is a complex multisystem disorder caused by the absence of the expression of paternally inherited imprinting genes on chromosome 15q11q13 caused by a paternal de novo deletion, maternal uniparental disomy (UPD), or an imprinting center defect (ID) [1,2]
Weight-SDSPWS and body mass index (BMI)-SDSPWS decreased with no changes in fat mass in any age group
As weight gain is part of the natural history of PWS, we hypothesized that weight and BMI would increase in children, adolescents, and young adults with PWS
Summary
Prader–Willi syndrome (PWS) is a complex multisystem disorder caused by the absence of the expression of paternally inherited imprinting genes on chromosome 15q11q13 caused by a paternal de novo deletion, maternal uniparental disomy (UPD), or an imprinting center defect (ID) [1,2]. Cardinal features of the syndrome include neonatal hypotonia with feeding difficulties and failure to thrive, followed by hyperphagia and food-seeking behavior with onset in early childhood [1,3]. Individuals with PWS show an altered body composition, with increased adiposity and a decreased lean body mass (LBM). The reduction in muscle mass causes a reduced resting energy expenditure and avoidance of physical exercise. Together with increased energy intake due to hyperphagia, individuals with PWS show a high prevalence of severe obesity [8,9]. Apart from GH deficiency, common endocrinopathies in individuals with PWS include hypogonadism and hypothyroidism [6,7]. First-line management of PWS includes a low-calorie diet and physical exercise in conjunction with
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