Department of Medicine (Austin Health), University of Melbourne, Melbourne, VIC, Australia.
Department of Endocrinology, Austin Health, Melbourne, VIC, Australia.
Endocrine. 2022 Jun;77(1):57-62. doi: 10.1007/s12020-022-03064-1. Epub 2022 May 7.
Prader-Willi syndrome (PWS) is characterised by childhood-onset hyperphagia and obesity however limited data are available to guide treatment of obesity in this population. We aimed to evaluate the safety, tolerability, and efficacy of intensive medical weight loss interventions (very-low-energy diets [VLED] and/or pharmacotherapy) in individuals with PWS attending a specialist obesity management service.
A retrospective audit was undertaken of individuals with PWS attending the Austin Health Weight Control Clinic between January 2010-April 2021. Main outcome measures were weight outcomes, duration of use, and adverse effects.
Data were available for 18 patients, of whom 15 were treated with intensive weight loss interventions. Median (interquartile range, IQR) age at baseline was 20 years (19-32) with median body weight 90 kg (75-118) and BMI 37 kg/m (30-51). Median weight loss during VLED (n = 7) was 14 kg (1-20 kg) over 60 weeks. Median weight loss with phentermine-topiramate (n = 7) was 17 kg (IQR 9-19 kg) over 56 weeks. Median weight loss with liraglutide 0.6-3 mg (n = 7), prescribed with topiramate in 3 individuals, was 9 kg (2-14 kg) over 96 weeks. Naltrexone-bupropion resulted in weight loss in 2 of 4 individuals. Thirteen individuals achieved ≥10% weight loss but only 5 individuals maintained ≥10% weight loss at last follow-up. Five individuals discontinued pharmacotherapy due to adverse effects.
VLED and pharmacotherapy can achieve substantial weight loss in some individuals with PWS though non-adherence results in substantial weight regain. Adverse effects were ascribed to phentermine and topiramate, whereas liraglutide was well-tolerated in this population.
普拉德-威利综合征（PWS）的特征是儿童期开始的暴食和肥胖，但目前用于指导该人群肥胖治疗的数据有限。我们旨在评估专门的肥胖管理服务中接受治疗的 PWS 个体强化医学减肥干预（极低能量饮食[VLED]和/或药物治疗）的安全性、耐受性和疗效。
对 2010 年 1 月至 2021 年 4 月期间在奥斯汀健康体重控制诊所就诊的 PWS 患者进行回顾性审计。主要结局指标为体重结局、使用时间和不良反应。
18 名患者的数据可用，其中 15 名接受了强化减肥干预。基线时的中位（四分位距，IQR）年龄为 20 岁（19-32），中位体重为 90kg（75-118），BMI 为 37kg/m（30-51）。7 名接受 VLED 治疗的患者的中位体重减轻量为 14kg（1-20kg），持续 60 周。7 名接受苯丁胺-托吡酯治疗的患者的中位体重减轻量为 17kg（9-19kg），持续 56 周。7 名接受利拉鲁肽 0.6-3mg 治疗的患者（3 名患者同时服用托吡酯）的中位体重减轻量为 9kg（2-14kg），持续 96 周。纳曲酮-安非他酮使 4 名患者中的 2 名患者减轻了体重。13 名患者体重减轻达到 10%以上，但只有 5 名患者在最后一次随访时体重减轻维持在 10%以上。5 名患者因不良反应而停止药物治疗。
VLED 和药物治疗可以使一些 PWS 患者显著减轻体重，但由于不依从导致体重大量反弹。将不良反应归因于苯丁胺和托吡酯，而利拉鲁肽在该人群中耐受性良好。
