{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T01:14:41Z","timestamp":1793754881203,"version":"4.6.1"},"reference-count":23,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,2,7]],"date-time":"2024-02-07T00:00:00Z","timestamp":1707264000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,2,7]],"date-time":"2024-02-07T00:00:00Z","timestamp":1707264000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Orphanet J Rare Dis"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Prader-Willi syndrome (PWS) is a complex genetic neurodevelopmental condition characterised by a range of debilitating and lifelong symptoms. The many physical and behavioural challenges that arise with adults with PWS often necessitate full-time (i.e., 24-hour) professional care support. However, despite the fact that many clinicians regard full-time PWS-specific care to represent best practice, relatively few studies have directly examined the benefits of such services. The purpose of this paper is to use archival data to investigate the impact of full-time care services on people with PWS, and to assemble a large statistical dataset on which robust analyses of improvements in weight, BMI, and behavioural outcomes can be based.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Information collated by the International PWS Organisation (IPWSO), an international non-profit membership organisation supporting national PWS associations around the world, was combined into a single anonymised dataset for statistical analysis. Data were supplied by service-providers from several countries who provide full-time support to people with PWS. The dataset included details on the specific services provided, basic demographic information on service recipients, including weight, body mass index (BMI), and observational records relating to behaviours of concern (BOC; consisting of temper outbursts, skin-picking, egocentrism, inflexibility, and striving for dominance).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>A total of 193 people with PWS (ranging in age from &lt;\u200910 yrs to &gt;\u200950 yrs; 93% of whom were &gt;\u200918 yrs), residing in 11 services across 6 countries, were represented in the dataset. On average, people with PWS showed significant reductions in weight and BMI after joining a full-time care service, with improvements within one year of entering, which were cumulative over time and independent of age or initial weight at entry. Similar cumulative improvements over time were seen for BOC within one year and were unrelated to age or severity of BOC at entry. The degree to which services are specialised for residents with PWS appeared to confer particular benefits, with people living in PWS-exclusive services showing the greatest improvements in weight, BMI, and BOC. Reductions in BOC were associated with greater, rather than less, social contact, suggesting that these improvements were not achieved at the expense of broader freedoms, such as the opportunity to meet with families and friends.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>We conclude that full-time care services have a high likelihood of enhancing the lives of people with PWS within one year with long-lasting benefits, especially if those services are exclusive and specialised around the particular needs of PWS.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13023-024-03035-x","type":"journal-article","created":{"date-parts":[[2024,2,7]],"date-time":"2024-02-07T12:02:27Z","timestamp":1707307347000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Body weight, behaviours of concern, and social contact in adults and adolescents with Prader-Willi syndrome in full-time care services: Findings from pooled international archival data"],"prefix":"10.1186","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6545-9248","authenticated-orcid":false,"given":"Brian M.","family":"Hughes","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony","family":"Holland","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norbert","family":"H\u00f6debeck-Stuntebeck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynn","family":"Garrick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony P.","family":"Goldstone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Lister","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Craig","family":"Moore","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marguerite","family":"Hughes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,2,7]]},"reference":[{"issue":"12","key":"3035_CR1","doi-asserted-by":"publisher","first-page":"1249","DOI":"10.1007\/s40618-015-0312-9","volume":"38","author":"MA Angulo","year":"2015","unstructured":"Angulo MA, Butler MG, Cataletto ME. Prader-Willi syndrome: a review of clinical, genetic, and endocrine findings. J Endocrinol Invest. 2015;38(12):1249\u201363.","journal-title":"J Endocrinol Invest"},{"issue":"11","key":"3035_CR2","doi-asserted-by":"publisher","first-page":"792","DOI":"10.1136\/jmg.38.11.792","volume":"38","author":"JE Whittington","year":"2001","unstructured":"Whittington JE, Holland AJ, Webb T, Butler J, Clarke D, Boer H. Population prevalence and estimated birth incidence and mortality rate for people with prader-Willi syndrome in one UK Health Region. J Med Genet. 2001;38(11):792\u20138.","journal-title":"J Med Genet"},{"key":"3035_CR3","volume-title":"GeneReviews\u00ae","author":"DJ Driscoll","year":"2017","unstructured":"Driscoll DJ, Miller JL, Schwartz S, Cassidy SB. Prader-Willi Syndrome. In: Adam MP, Everman DB, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, et al. editors. GeneReviews\u00ae. Seattle (WA): University of Washington, Seattle; 2017."},{"key":"3035_CR4","unstructured":"H\u00f6debeck-Stuntebeck N. Perspektivwechsel Bei Prader-Willi-Syndrom: Ein Schl\u00fcssel Zum Sozialverhalten\u2014Entwicklung Einer Zielgruppenspezifischen Diagnostik und evaluation eines Trainingsprogramms Zur F\u00f6rderung Der Perspektiv\u00fcbernahme. Logos Verlag; 2012."},{"key":"3035_CR5","first-page":"3","volume":"17","author":"S Cassidy","year":"2009","unstructured":"Cassidy S, Driscoll D. Prader-Willi syndrome. Pract Genet. 2009;17:3\u201313.","journal-title":"Pract Genet"},{"issue":"2","key":"3035_CR6","doi-asserted-by":"publisher","first-page":"188","DOI":"10.1038\/ijo.2010.139","volume":"35","author":"CJ McAllister","year":"2011","unstructured":"McAllister CJ, Whittington JE, Holland AJ. Development of the eating behaviour in Prader-Willi syndrome: advances in our understanding. Int J Obes (Lond). 2011;35(2):188\u201397.","journal-title":"Int J Obes (Lond)"},{"issue":"1","key":"3035_CR7","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1186\/s11689-021-09373-2","volume":"13","author":"L Schwartz","year":"2021","unstructured":"Schwartz L, Caix\u00e0s A, Dimitropoulos A, Dykens E, Duis J, Einfeld S, et al. Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium. J Neurodev Disord. 2021;13(1):25.","journal-title":"J Neurodev Disord"},{"issue":"1","key":"3035_CR8","doi-asserted-by":"publisher","first-page":"104379","DOI":"10.1016\/j.ejmg.2021.104379","volume":"65","author":"SA Bellis","year":"2022","unstructured":"Bellis SA, Kuhn I, Adams S, Mullarkey L, Holland A. The consequences of hyperphagia in people with prader-Willi syndrome: a systematic review of studies of morbidity and mortality. 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J Appl Res Intellect Disabil. 2022;35(2):460\u201370.","journal-title":"J Appl Res Intellect Disabil"},{"issue":"3","key":"3035_CR11","doi-asserted-by":"publisher","first-page":"e0194655","DOI":"10.1371\/journal.pone.0194655","volume":"13","author":"N Kayadjanian","year":"2018","unstructured":"Kayadjanian N, Schwartz L, Farrar E, Comtois KA, Strong TV. High levels of caregiver burden in Prader-Willi syndrome. PLoS ONE. 2018;13(3):e0194655.","journal-title":"PLoS ONE"},{"issue":"5","key":"3035_CR12","doi-asserted-by":"publisher","first-page":"1665","DOI":"10.1111\/apa.15738","volume":"110","author":"C Meade","year":"2021","unstructured":"Meade C, Martin R, McCrann A, Lyons J, Meehan J, Hoey H, et al. Prader-Willi syndrome in children: quality of life and caregiver burden. Acta Paediatr. 2021;110(5):1665\u201370.","journal-title":"Acta Paediatr"},{"key":"3035_CR13","unstructured":"Gallagher L, Roche E, Feighan S-M, Kang H-J, Hughes M. A population-based profile of Prader-Willi syndrome in Ireland. Prader Willi Syndrome Association Ireland; 2017."},{"key":"3035_CR14","unstructured":"Bohonowych J. Living situations for people with PWS change as they get older. Foundation for Prader-Willi Research Blog. December 2, 2022. Accessed June 1, 2023. https:\/\/www.fpwr.org\/blog\/living-situations-for-people-with-pws-change-as-they-get-older."},{"key":"3035_CR15","unstructured":"Forster J, ed. Proceedings of the 2008 and 2009 International PWS Caregivers\u2019 Conferences: Best Practice Guidelines for Standard of Care in PWS. Absberg H, SoyerNobert H\u00f6debeck-Stuntebeck\/IPWSO.; 2010. Accessed December 11, 2023. https:\/\/ipwso.org\/pws-information\/information-for-professional-caregivers\/best-practice-guidelines-for-residential-care\/."},{"issue":"3","key":"3035_CR16","doi-asserted-by":"publisher","first-page":"419","DOI":"10.1002\/1098-108X(198705)6:3<419::AID-EAT2260060312>3.0.CO;2-I","volume":"6","author":"JB Mullins","year":"1987","unstructured":"Mullins JB, Vogl-Maier B. Weight management of youth with prader-Willi syndrome. Int J Eat Disord. 1987;6(3):419\u201325.","journal-title":"Int J Eat Disord"},{"issue":"4","key":"3035_CR17","doi-asserted-by":"publisher","first-page":"1175","DOI":"10.1002\/ajmg.a.62101","volume":"185","author":"HJ Hirsch","year":"2021","unstructured":"Hirsch HJ, Benarroch F, Genstil L, Pollak Y, Derei D, Forer D, et al. Long-term weight control in adults with prader-Willi syndrome living in residential hostels. 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New York: Routldege; 1988."}],"container-title":["Orphanet Journal of Rare Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-024-03035-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13023-024-03035-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-024-03035-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,2,7]],"date-time":"2024-02-07T12:10:04Z","timestamp":1707307804000},"score":1,"resource":{"primary":{"URL":"https:\/\/ojrd.biomedcentral.com\/articles\/10.1186\/s13023-024-03035-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,2,7]]},"references-count":23,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["3035"],"URL":"https:\/\/doi.org\/10.1186\/s13023-024-03035-x","relation":{},"ISSN":["1750-1172"],"issn-type":[{"value":"1750-1172","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,2,7]]},"assertion":[{"value":"3 July 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 January 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 February 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This retrospective review of archival data did not involve primary data collection.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The research was carried out under the auspices of IPWSO, which is a parent- and clinician-led organisation. BMH, LG, and MH are parents of individuals who have PWS. NHS, LG, ML, and CM have worked for providers of services to people with PWS. APG has been on the Data Safety Monitoring Committee and received a speaker honorarium from Novo Nordisk, on the Medical Advisory Board for Millendo Therapeutics and Radius Health, and is a consultant for Soleno Therapeutics, Helsinn Healthcare S.A., Evidera and Idera Pharmaceuticals Inc. The other authors have no individual conflicts of interest to declare.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing Interests"}}],"article-number":"48"}}