Dipartimento Di Scienze Umanistiche, Centro Direzionale, Università Telematica Pegaso, Via Porzioisola F2, 80143, Naples, Italy.
Department of Clinical Medicine and Surgery, Endocrinology Unit, Centro Italiano Per La Cura E Il Benessere del Paziente Con Obesità (C.I.B.O), University Medical School of Naples, Via Sergio Pansini 5, 80131, Naples, Italy.
Curr Obes Rep. 2022 Dec;11(4):263-276. doi: 10.1007/s13679-022-00478-w. Epub 2022 Sep 5.
Prader-Willi syndrome (PWS) is a rare and complex genetic disorder with multiple effects on the metabolic, endocrine, and neurological systems, as well as behavioral and intellectual difficulties. Despite advances in understanding the genetic basis of obesity in PWS, there are conflicting data on its management. Therefore, the present manuscript aims to provide an update on the nutritional treatment and pharmacological approach in adult patients with PWS.
The management of obesity in patients with PWS is challenging and requires the cooperation of an experienced multidisciplinary team, including the nutritionist. An adequate clinical evaluation including nutritional and biochemical parameters should be performed to tailor the best therapeutic strategy. Both lifestyle and pharmacological interventions may represent useful strategies to prevent the high rate of morbidity and mortality related to PWS. The use of bariatric surgery is still controversial. Although it is imperative to adopt an obesity prevention strategy in childhood, there is promising evidence for the treatment of obesity in adulthood with current obesity medications in conjunction with lifestyle interventions.
普拉德-威利综合征(PWS)是一种罕见且复杂的遗传性疾病,对代谢、内分泌和神经系统以及行为和智力都有多种影响。尽管人们对 PWS 肥胖的遗传基础有了更多的了解,但在其管理方面仍存在相互矛盾的数据。因此,本文旨在提供成人 PWS 患者营养治疗和药物治疗方法的最新进展。
PWS 患者的肥胖管理具有挑战性,需要有经验的多学科团队(包括营养师)的合作。应进行充分的临床评估,包括营养和生化参数,以制定最佳的治疗策略。生活方式和药物干预都可能是预防与 PWS 相关的高发病率和死亡率的有用策略。减重手术的应用仍存在争议。尽管在儿童期采取肥胖预防策略至关重要,但目前的肥胖药物结合生活方式干预治疗成人肥胖有一定的前景。