{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T17:34:41Z","timestamp":1793295281340,"version":"4.5.0"},"reference-count":57,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2022,5,6]],"date-time":"2022-05-06T00:00:00Z","timestamp":1651795200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Research, Development and Innovation Fund of Hungary","award":["TKP2021-EGA-10"],"award-info":[{"award-number":["TKP2021-EGA-10"]}]},{"name":"National Research, Development and Innovation Fund of Hungary","award":["EFOP-3.6.1.-16-2016-00004"],"award-info":[{"award-number":["EFOP-3.6.1.-16-2016-00004"]}]},{"name":"Comprehensive Development for Implementing Smart Specialization Strategies at the University of P\u00e9cs","award":["TKP2021-EGA-10"],"award-info":[{"award-number":["TKP2021-EGA-10"]}],"id":[{"id":"https:\/\/ror.org\/037b5pv06","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Comprehensive Development for Implementing Smart Specialization Strategies at the University of P\u00e9cs","award":["EFOP-3.6.1.-16-2016-00004"],"award-info":[{"award-number":["EFOP-3.6.1.-16-2016-00004"]}],"id":[{"id":"https:\/\/ror.org\/037b5pv06","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Prader\u2013Willi syndrome (PWS) is a complex genetic disorder which involves the endocrine and neurologic systems, metabolism, and behavior. The aim of this paper is to summarize current knowledge on dietary management and treatment of PWS and, in particular, to prevent excessive weight gain. Growth hormone (GH) therapy is the recommended standard treatment for PWS children, because it improves body composition (by changing the proportion of body fat and lean body mass specifically by increasing muscle mass and energy expenditure), linear growth, and in infants, it promotes psychomotor and IQ development. In early childhood, the predominant symptom is hyperphagia which can lead to early onset, severe obesity with different obesity-related comorbidities. There are several studies on anti-obesity medications (metformin, topiramate, liraglutide, setmelanotide). However, these are still limited, and no widely accepted consensus guideline exists concerning these drugs in children with PWS. Until there is a specific treatment for hyperphagia and weight gain, weight must be controlled with the help of diet and exercise. Below the age of one year, children with PWS have no desire to eat and will often fail to thrive, despite adequate calories. After the age of two years, weight begins to increase without a change in calorie intake. Appetite increases later, gradually, and becomes insatiable. Managing the progression of different nutritional phases (0\u20134) is really important and can delay the early onset of severe obesity. Multidisciplinary approaches are crucial in the diagnosis and lifelong follow-up, which will determine the quality of life of these patients.<\/jats:p>","DOI":"10.3390\/nu14091950","type":"journal-article","created":{"date-parts":[[2022,5,8]],"date-time":"2022-05-08T21:31:20Z","timestamp":1652045480000},"page":"1950","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Prader\u2013Willi Syndrome: Possibilities of Weight Gain Prevention and Treatment"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6053-7833","authenticated-orcid":false,"given":"\u00c9va","family":"Erhardt","sequence":"first","affiliation":[{"name":"Department of Paediatrics, Medical School, University of P\u00e9cs, H-7623 P\u00e9cs, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3675-7019","authenticated-orcid":false,"given":"D\u00e9nes","family":"Moln\u00e1r","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Medical School, University of P\u00e9cs, H-7623 P\u00e9cs, Hungary"},{"name":"National Laboratory for Human Reproduction, Szent\u00e1gothai Research Centre, University of P\u00e9cs, H-7623 P\u00e9cs, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,5,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1038\/gim.0b013e31822bead0","article-title":"Prader\u2013Willi syndrome","volume":"14","author":"Cassidy","year":"2012","journal-title":"Genet. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1097\/MED.0000000000000517","article-title":"Prader Willi syndrome: Endocrine updates and new medical therapies","volume":"27","author":"Harris","year":"2020","journal-title":"Curr. Opin. Endocrinol. Diabetes Obes."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"227","DOI":"10.6065\/apem.2142164.082","article-title":"Prader\u2013Willi syndrome: An update an obesity and endocrine problems","volume":"26","author":"Kim","year":"2021","journal-title":"Ann. Pediatr. Endocrinol. Metab."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1159\/000313377","article-title":"French Reference Center for PWS. Endocrine disorders in children with Prader\u2013Willi syndrome-data from 142 children of the French database","volume":"74","author":"Diene","year":"2010","journal-title":"Horm. Res. Pediatr."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"e12992","DOI":"10.1111\/obr.12992","article-title":"Current and emerging therapies for managing hyperphagia and obesity in Prader\u2013Willi syndrome: A narrative review","volume":"21","author":"Tan","year":"2020","journal-title":"Obes. Rev."},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Goldman, V.E., Naguib, M.N., and Vidmar, A.p. (2021). Anti-Obesity Medication Use in Children and Adolescents with Prader\u2013Willi Syndrome: Case Review and Literature Search. J. Clin. Med., 10.","DOI":"10.3390\/jcm10194540"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"e12728","DOI":"10.1111\/jne.12728","article-title":"Prader\u2013Willi syndrome: A model for understanding the ghrelin system","volume":"31","author":"Tauber","year":"2019","journal-title":"J. Neuroendocr."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1038\/s41573-021-00337-8","article-title":"Anti-obesity drug discovery: Advances and challenges","volume":"21","author":"DiMarchi","year":"2022","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1542\/peds.91.2.398","article-title":"Prader\u2013Willi syndrome: Consensus diagnostic criteria","volume":"91","author":"Holm","year":"1993","journal-title":"Pediatrics"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"207","DOI":"10.2174\/1573396315666190716120925","article-title":"Prader\u2013Willi Syndrome-Clinical Genetics, Diagnosis and Treatment Approaches: An Update","volume":"15","author":"Butler","year":"2019","journal-title":"Curr. Ped. Rev."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/j.tem.2003.11.003","article-title":"Prader\u2013Willi syndrome: Advances in genetics, pathophysiology and treatment","volume":"15","author":"Goldstone","year":"2004","journal-title":"Trends Endocrinol. Metab."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1040","DOI":"10.1002\/ajmg.a.33951","article-title":"Nutritional Phases in Prader\u2013Willi Syndrome","volume":"155","author":"Miller","year":"2011","journal-title":"Am. J. Med. Genet. A"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"4183","DOI":"10.1210\/jc.2008-0649","article-title":"On behalf of speakers and contributors at the Second Expert Meeting of the Comprehensive Care of Patients with PWS. Recommendations for the Diagnosis and Management of Prader\u2013Willi syndrome","volume":"93","author":"Goldstone","year":"2008","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"905","DOI":"10.3945\/an.117.016253","article-title":"Energy Metabolism Profile in Individuals with Prader\u2013Willi Syndrome and Implications for Clinical Management: A Systematic Review","volume":"8","author":"Alsaif","year":"2017","journal-title":"Adv. Nutr."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/j.neubiorev.2015.10.003","article-title":"Prader\u2013Willi syndrome: From genetics to behaviour, with special focus on appetite treatments","volume":"59","author":"Mathai","year":"2015","journal-title":"Neurosci. Biobehav. Rev."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.appet.2015.12.021","article-title":"A review of chemosensory perceptions, food preferences and food related behaviours in subjects with Prader\u2013Willi Syndrome","volume":"99","author":"Michel","year":"2016","journal-title":"Appetite"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"579","DOI":"10.2147\/DMSO.S141352","article-title":"Obesity management in Prader\u2013Willi syndrome: Current perspectives","volume":"11","author":"Crino","year":"2018","journal-title":"Diab. Metab. Syndr. Obes. Target. Ther."},{"key":"ref_18","first-page":"113","article-title":"Dietary management for adolescents with Prader\u2013Willi syndrome","volume":"11","author":"Miller","year":"2020","journal-title":"Adolesc. Health Med. Ther."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1111\/jhn.12882","article-title":"Dietary intake and growth in children with Prader\u2013Willi syndrome","volume":"34","author":"Meade","year":"2021","journal-title":"J. Hum. Nutr. Diet."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"2057","DOI":"10.1007\/s40618-021-01574-9","article-title":"On behalf of RESTARE. Obesity in Prader\u2013Willi syndrome: Physiopathological mechanisms, nutritional and pharmacological approaches","volume":"44","author":"Muscogiuri","year":"2021","journal-title":"J. Endocrinol. Investig."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1111\/cen.13933","article-title":"Hormonal and metabolic effects of carbohydrate restriction in children with Prader\u2013Willi syndrome","volume":"90","author":"Irizarry","year":"2019","journal-title":"Clin. Endocrinol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1111\/j.1365-277X.2012.01275.x","article-title":"A reduced-energy intake, well-balanced diet improves weight control in children with Prader\u2013Willi syndrome","volume":"26","author":"Miller","year":"2013","journal-title":"J. Hum. Nutr. Diet."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1007\/s11154-019-09502-2","article-title":"On behalf of RESTARE. Prader\u2013Willi syndrome: An uptodate on endocrine and metabolic complications","volume":"20","author":"Muscogiuri","year":"2019","journal-title":"Rev. End. Met. Dis."},{"key":"ref_24","first-page":"48","article-title":"KIGS highlights: Growth hormone treatment in Prader\u2013Willi syndrome","volume":"68","author":"Tauber","year":"2007","journal-title":"Horm. Res."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"744","DOI":"10.1016\/j.jpeds.2004.08.002","article-title":"Growth hormone improves mobility and body composition in infants and toddlers with Prader\u2013Willi syndrome","volume":"145","author":"Carrel","year":"2004","journal-title":"J. Pediatr."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1203\/PDR.0b013e31809871dd","article-title":"Psychomotor development in infants with Prader\u2013Willi syndrome and associations with sleep-related breathing disorders","volume":"62","author":"Festen","year":"2007","journal-title":"Pediatr. Res."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1274","DOI":"10.1210\/jc.2008-1844","article-title":"Randomized controlled trial to investigate the effects of growth hormone treatment on scoliosis in children with Prader\u2013Willi syndrome","volume":"94","author":"Festen","year":"2009","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1111\/j.1651-2227.1999.tb14416.x","article-title":"Five years of growth hormone treatment in children with Prader\u2013Willi syndrome. Swedish National Growth Hormone Advisory Group","volume":"433","author":"Lindgren","year":"1999","journal-title":"Acta Paediatr. Suppl."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"4205","DOI":"10.1210\/jc.2009-0454","article-title":"Efficacy and safety of long-term continuous growth hormone treatment in children with Prader\u2013Willi syndrome","volume":"94","author":"Siemensma","year":"2009","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1020084","DOI":"10.1016\/j.npep.2020.102084","article-title":"Growth hormone treatment for Prader\u2013Willi syndrome: A review","volume":"83","author":"Yang","year":"2020","journal-title":"Neuropeptides"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1590\/1984-0462\/;2018;36;3;00003","article-title":"Prader\u2013Willi syndrome: What is the general pediatrician supposed to do? A review","volume":"36","author":"Passone","year":"2018","journal-title":"Rev. Paul. Pediatr."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1089\/cap.2015.0240","article-title":"Prader\u2013Willi Syndrome, Management of Impulsivity, and Hyperphagia in an Adolescent","volume":"26","author":"Puri","year":"2016","journal-title":"J. Child Adolesc. Psychopharmacol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"e76","DOI":"10.2337\/dc13-2575","article-title":"Use of GLP-1 receptor agonists in Prader\u2013Willi syndrome: Report of six cases","volume":"37","author":"Fintini","year":"2014","journal-title":"Diab. Care"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1515\/jpem-2013-0116","article-title":"Effects of metformin in children and adolescents with Prader\u2013Willi syndrome and early onset morbid obesity: A pilot study","volume":"27","author":"Miller","year":"2014","journal-title":"J. Pediatr. Endocrinol. Metab."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1111\/cen.14583","article-title":"The effects of glucagon-like peptide (GLP)-1 receptor agonists on weight and glycaemic control in Prader\u2013Willi syndrome: A systematic review","volume":"96","author":"Ng","year":"2022","journal-title":"Clin. Endocrinol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1111\/ijpo.12131","article-title":"Efects of exenatide on weight and appetite in overweight adolescents and young adults with Prader\u2013Willi syndrome","volume":"12","author":"Salehi","year":"2017","journal-title":"Pediatr. Obes."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1038\/s41398-019-0597-0","article-title":"Effect of topiramate on eating behaviours in Prader\u2013Willi syndrome: TOPRADER double-blind randomised placebo-controlled study","volume":"9","author":"Consoli","year":"2019","journal-title":"Transl. Psych."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1016\/S0887-8994(02)00490-3","article-title":"Topiramate effectiveness in Prader\u2013Willi syndrome","volume":"28","author":"Smathers","year":"2003","journal-title":"Pediatr. Neurol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"7","DOI":"10.9740\/mhc.2017.01.007","article-title":"Topiramate in the treatment of Prader\u2013Willi syndrome: A case report","volume":"7","author":"East","year":"2017","journal-title":"Ment. Health Clin."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"2042018820918789","DOI":"10.1177\/2042018820918789","article-title":"Medications for the treatment of obesity in adolescents","volume":"11","author":"Woodard","year":"2020","journal-title":"Ther. Adv. Endocrinol. Metab."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"1213","DOI":"10.1001\/jama.2015.1617","article-title":"Naltrexone Extended-Release Plus Bupropion Extended-Release for Treatment of Obesity","volume":"313","author":"Yanovski","year":"2015","journal-title":"JAMA"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1243","DOI":"10.1002\/ajmg.a.38160","article-title":"Oxytocin treatment in children with Prader\u2013Willi syndrome: A double-blind, placebo-controlled, crossover study","volume":"173","author":"Miller","year":"2017","journal-title":"Am. J. Med. Genet. Part A"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"2232","DOI":"10.1002\/ajmg.a.36653","article-title":"A double-blind randomized controlled trial of oxytocin nasal spray in Prader Willi syndrome","volume":"164","author":"Einfeld","year":"2014","journal-title":"Am. J. Med. Genet. Part A"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1111\/cen.13169","article-title":"Promising effects of oxytocin on social and food-related behaviour in young children with Prader\u2013Willi syndrome: A randomized, double-blind, controlled crossover trial","volume":"85","author":"Kuppens","year":"2016","journal-title":"Clin. Endocrinol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1111\/cen.14387","article-title":"Oxytocin in young children with Prader\u2013Willi syndrome: Results of a randomized, double-blind, placebo-controlled, crossover trial investigating 3 months of oxytocin","volume":"94","author":"Damen","year":"2020","journal-title":"Clin. Endocrinol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1016\/j.jpsychires.2020.11.006","article-title":"Intranasal oxytocin versus placebo for hyperphagia and repetitive behaviors in children with Prader\u2013Willi Syndrome: A randomized controlled pilot trial","volume":"137","author":"Hollander","year":"2021","journal-title":"J. Psychiatr. Res."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1906","DOI":"10.1016\/S0140-6736(08)61525-1","article-title":"Effect of tesofensine on bodyweight loss, body composition, and quality of life in obese patients: A randomised, double-blind, placebo-controlled trial","volume":"372","author":"Astrup","year":"2008","journal-title":"Lancet"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1016\/j.ymgme.2018.02.018","article-title":"Chronic diazoxide treatment decreases fat mass and improves endurance capacity in an obese mouse model of Prader\u2013Willi syndrome","volume":"123","author":"Bischof","year":"2018","journal-title":"Mol. Genet. Metab."},{"key":"ref_49","first-page":"211","article-title":"Efficacy and safety of diazoxide choline controlled-release tablet in patients with Prader\u2013Willi syndrome: An updated analysis","volume":"88","author":"Kimonis","year":"2017","journal-title":"Horm. Res. Paediatr."},{"key":"ref_50","doi-asserted-by":"crossref","unstructured":"Allas, S. (2018). AZP-531, an unacylated ghrelin analog, improves food-related behavior in patients with Prader\u2013Willi syndrome: A randomized placebo-controlled trial. PLoS ONE, 13.","DOI":"10.1371\/journal.pone.0190849"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"e514","DOI":"10.1002\/mgg3.514","article-title":"A multidisciplinary approach to the clinical management of Prader\u2013Willi syndrome","volume":"7","author":"Duis","year":"2019","journal-title":"Mol. Genet. Genom. Med."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"3081","DOI":"10.1016\/j.ridd.2014.07.035","article-title":"Patterns of habitual physical activity in youth with and without Prader\u2013Willi syndrome","volume":"35","author":"Castner","year":"2014","journal-title":"Res. Dev. Disabil."},{"key":"ref_53","doi-asserted-by":"crossref","unstructured":"Bellicha, A., Coupaye, M., Mosbah, H., Tauber, M., Oppert, J.M., and Poitou, C. (2021). Physical Activity in Patients with Prader\u2013Willi Syndrome- Systematic Review of Observational and Interventional Studies. J. Clin. Med., 10.","DOI":"10.3390\/jcm10112528"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1515\/JPEM.2006.19.1.65","article-title":"Comprehensive Muscle Training Programme Increases Lean Mass and Spontaneous Activity in Children with Prader\u2013Willi Syndrome after 6 Months","volume":"19","author":"Schlumpf","year":"2006","journal-title":"J. Pediatr. Endocrinol. Metab."},{"key":"ref_55","doi-asserted-by":"crossref","unstructured":"Rubin, D.A., Wilson, K.S., Orsso, C.E., Gertz, E.R., Haqq, A.M., Castner, D.M., and Dumont-Driscoll, M. (2020). A 24-Week Physical Activity Intervention Increases Bone Mineral Content without Changes in Bone Markers in Youth with PWS. Genes, 11.","DOI":"10.3390\/genes11090984"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1515\/jpem-2015-0233","article-title":"Association between Physical Activity and Bone in Children with Prader\u2013Willi Syndrome","volume":"29","author":"Duran","year":"2016","journal-title":"J. Pediatr. Endocrinol. Metab."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"864","DOI":"10.3389\/fendo.2019.00864","article-title":"Genotype-Phenotype Relationships and Endocrine Findings in Prader\u2013Willi Syndrome","volume":"10","author":"Costa","year":"2019","journal-title":"Front. Endocrinol."}],"container-title":["Nutrients"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6643\/14\/9\/1950\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T23:07:05Z","timestamp":1760137625000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6643\/14\/9\/1950"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,5,6]]},"references-count":57,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2022,5]]}},"alternative-id":["nu14091950"],"URL":"https:\/\/doi.org\/10.3390\/nu14091950","relation":{},"ISSN":["2072-6643"],"issn-type":[{"value":"2072-6643","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,5,6]]}}}