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

Claudia Vetrani, Ludovica Verde, Silvia Savastano, Annamaria Colao, Luigi Barrea, Danilo Fintini, Giulia de Alteriis, Filippo Maria Panfili, Sarah Bocchini, Giovanna Muscogiuri

Journal: Current obesity reports 2022;11(4):263-276

PMID: 36063285

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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.

Address: 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.; Dipartimento Di Medicina Clinica E Chirurgia, Unit of Endocrinology, Federico II University Medical School of Naples, Via Sergio Pansini 5, 80131, Naples, Italy.; Endocrinology Unit, Bambino Gesù Children Hospital, Reference Center for Prader-Willi Syndrome, Rome, Italy.; School of Medicine, University of Rome Tor Vergata, Rome, Italy.; Cattedra Unesco "Educazione Alla Salute E Allo Sviluppo Sostenibile", University Federico II, 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. [email protected].; Dipartimento Di Medicina Clinica E Chirurgia, Unit of Endocrinology, Federico II University Medical School of Naples, Via Sergio Pansini 5, 80131, Naples, Italy. [email protected].; Cattedra Unesco "Educazione Alla Salute E Allo Sviluppo Sostenibile", University Federico II, Naples, Italy. [email protected].

DOI: 10.1007/s13679-022-00478-w