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

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 con...

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Detalles Bibliográficos
Autores principales: Barrea, Luigi, Vetrani, Claudia, Fintini, Danilo, de Alteriis, Giulia, Panfili, Filippo Maria, Bocchini, Sarah, Verde, Ludovica, Colao, Annamaria, Savastano, Silvia, Muscogiuri, Giovanna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9729321/
https://www.ncbi.nlm.nih.gov/pubmed/36063285
http://dx.doi.org/10.1007/s13679-022-00478-w
Descripción
Sumario: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. SUMMARY: 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