Silvia Savastano1,2, Giovanna Muscogiuri3,4,5, Luigi Barrea6,1, Claudia Vetrani1,2, Danilo Fintini7, Giulia de Alteriis1,2, Filippo Maria Panfili8, Sarah Bocchini7, Ludovica Verde1,2, Annamaria Colao1,2,9. 1. 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. 2. Dipartimento Di Medicina Clinica E Chirurgia, Unit of Endocrinology, Federico II University Medical School of Naples, Via Sergio Pansini 5, 80131, Naples, Italy. 3. 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. giovanna.muscogiuri@unina.it. 4. Dipartimento Di Medicina Clinica E Chirurgia, Unit of Endocrinology, Federico II University Medical School of Naples, Via Sergio Pansini 5, 80131, Naples, Italy. giovanna.muscogiuri@unina.it. 5. Cattedra Unesco "Educazione Alla Salute E Allo Sviluppo Sostenibile", University Federico II, Naples, Italy. giovanna.muscogiuri@unina.it. 6. Dipartimento Di Scienze Umanistiche, Centro Direzionale, Università Telematica Pegaso, Via Porzioisola F2, 80143, Naples, Italy. 7. Endocrinology Unit, Bambino Gesù Children Hospital, Reference Center for Prader-Willi Syndrome, Rome, Italy. 8. School of Medicine, University of Rome Tor Vergata, Rome, Italy. 9. Cattedra Unesco "Educazione Alla Salute E Allo Sviluppo Sostenibile", University Federico II, Naples, Italy.
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.
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.
Authors: Michelle L Mackenzie; Lucila Triador; Jasmeena K Gill; Mohammadreza Pakseresht; Diana Mager; Catherine J Field; Andrea M Haqq Journal: Am J Med Genet A Date: 2018-09-14 Impact factor: 2.802
Authors: Jennifer Proffitt; Kathryn Osann; Barbara McManus; Virginia E Kimonis; Janalee Heinemann; Merlin G Butler; David A Stevenson; June-Anne Gold Journal: Am J Med Genet A Date: 2018-12-19 Impact factor: 2.802
Authors: Maha Alsaif; Sarah A Elliot; Michelle L MacKenzie; Carla M Prado; Catherine J Field; Andrea M Haqq Journal: Adv Nutr Date: 2017-11-15 Impact factor: 8.701
Authors: G Muscogiuri; L Barrea; F Faggiano; M I Maiorino; M Parrillo; G Pugliese; R M Ruggeri; E Scarano; S Savastano; A Colao Journal: J Endocrinol Invest Date: 2021-04-23 Impact factor: 4.256