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Review
. 2022 Dec;11(4):263-276.
doi: 10.1007/s13679-022-00478-w. Epub 2022 Sep 5.

Prader-Willi Syndrome in Adults: An Update On Nutritional Treatment and Pharmacological Approach

Luigi Barrea  1   2 , Claudia Vetrani  2   3 , Danilo Fintini  4 , Giulia de Alteriis  2   3 , Filippo Maria Panfili  5 , Sarah Bocchini  4 , Ludovica Verde  2   3 , Annamaria Colao  2   3   6 , Silvia Savastano #  2   3 , Giovanna Muscogiuri #  7   8   9
Affiliations
Review

Prader-Willi Syndrome in Adults: An Update On Nutritional Treatment and Pharmacological Approach

Luigi Barrea et al. Curr Obes Rep. 2022 Dec.

Abstract

Purpose of review: 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.

Recent findings: 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.

Keywords: Diet; Drugs; Ketogenic diet; Nutrition; Nutritionist; Obesity; Prader–Willi syndrome.

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Conflict of interest statement

The authors have no competing interests to declare that are relevant to the content of this article.

Figures

Fig. 1
Fig. 1
Metabolic and hormonal disorders in patients with PWS. T2DM, type 2 diabetes mellitus; GHD, growth hormone deficiency
Fig. 2
Fig. 2
Clinical assessment and potential therapeutic strategies for the management of obesity and its complications in adults with PWS. Abbreviations: PWS, Prader–Willi syndrome; GLP-1, glucagon-like peptide-1
See this image and copyright information in PMC

References

    1. Angulo MA, Butler MG, Cataletto ME. Prader-Willi syndrome: a review of clinical, genetic, and endocrine findings. J Endocrinol Invest. 2015;38(12):1249–1263. doi: 10.1007/s40618-015-0312-9. - DOI - PMC - PubMed
    1. Cassidy SB, Schwartz S, Miller JL, Driscoll DJ. Prader-Willi syndrome. Genet Med. 2012;14(1):10–26. doi: 10.1038/gim.0b013e31822bead0. - DOI - PubMed
    1. Mackenzie ML, Triador L, Gill JK, et al. Dietary intake in youth with prader-willi syndrome. Am J Med Genet A. 2018;176(11):2309–2317. doi: 10.1002/ajmg.a.40491. - DOI - PubMed
    1. Guinovart M, Coronas R, Caixas A. Psychopathological disorders in Prader-Willi syndrome. Endocrinol Diabetes Nutr (Engl Ed) 2019;66(9):579–587. doi: 10.1016/j.endinu.2019.03.004. - DOI - PubMed
    1. Muscogiuri G, Barrea L, Faggiano F, et al. Obesity in Prader-Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches. J Endocrinol Invest. 2021;44(10):2057–2070. doi: 10.1007/s40618-021-01574-9. - DOI - PMC - PubMed