Telethon Kids Institute, The Centre for Child Health Research, The University of Western Australia, PO Box 855, West Perth, WA, 6872, Australia.
School of Medicine, The University of Western Australia, Crawley, WA, Australia.
J Autism Dev Disord. 2022 Sep;52(9):3877-3889. doi: 10.1007/s10803-021-05265-5. Epub 2021 Sep 8.
Prader-Willi syndrome (PWS) is a rare genetic disorder characterised by neurodevelopmental delays, hyperphagia, difficulties with social communication and challenging behaviours. Individuals require intensive supervision from caregivers which may negatively affect caregiver quality of life. This study used data collected in the Australasian PWS Registry (n = 50, mean age 11.2 years) to evaluate associations between child behaviours and caregiver mental well-being. Symptoms of sleep-related breathing disorder, child depression and social difficulties were associated with poorer caregiver mental and physical well-being. Growth hormone therapy use was associated with better caregiver mental and physical well-being. Optimising management of problematic behaviours and sleep disturbances have the potential to support caregivers who are the most vital network of support for individuals affected by PWS.
普拉德-威利综合征（PWS）是一种罕见的遗传疾病，其特征是神经发育迟缓、食欲过盛、社交沟通困难和行为挑战。患者需要照顾者的密集监督，这可能会对照顾者的生活质量产生负面影响。本研究使用在澳大拉西亚 PWS 注册中心（n=50，平均年龄 11.2 岁）收集的数据，评估儿童行为与照顾者心理健康之间的关联。睡眠相关呼吸障碍、儿童抑郁和社交困难的症状与照顾者的身心健康较差相关。生长激素治疗的使用与照顾者的身心健康较好相关。优化行为问题和睡眠障碍的管理有可能为照顾者提供支持，他们是受 PWS 影响的个体最重要的支持网络。
