{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T22:39:05Z","timestamp":1791326345974,"version":"4.3.1"},"reference-count":26,"publisher":"The Endocrine Society","issue":"9","license":[{"start":{"date-parts":[[2023,3,6]],"date-time":"2023-03-06T00:00:00Z","timestamp":1678060800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Foundation Growth Puberty Adolescence"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,8,18]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Context<\/jats:title>\n                  <jats:p>Prader-Labhart-Willi syndrome (PWS) is a rare genetic disorder characterized by intellectual disability, behavioural problems, and hypothalamic dysfunction combined with specific dysmorphisms. In PWS, growth hormone treatment is given primarily to improve body composition, yet lean body mass (LBM) does not normalize. Male hypogonadism is frequent in PWS and becomes evident during puberty. While LBM increases in normal boys during puberty, it is not known whether LBM and muscle mass concomitantly increase in PWS during spontaneous or induced puberty.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>To describe the peripubertal increment in muscle mass in boys with PWS undergoing growth hormone treatment.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Design<\/jats:title>\n                  <jats:p>Single-center, retrospective descriptive study, using data from 4 years before until 4 years after onset of puberty.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Setting<\/jats:title>\n                  <jats:p>Primary referral centre for PWS.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Patients<\/jats:title>\n                  <jats:p>Thirteen boys diagnosed with genetically proven PWS. The mean age at onset of puberty was 12.3 years; the mean observation period before (after) onset of puberty was 2.9 (3.1) years.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Intervention<\/jats:title>\n                  <jats:p>Puberty was induced upon pubertal arrest. All boys received internationally standardized growth hormone treatment.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Main Outcome Measure<\/jats:title>\n                  <jats:p>Lean mass index (LMI) determined by dual energy X-ray absorptiometry.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>LMI increased by 0.28 kg\/m2 per year before puberty and by 0.74 kg\/m2 per year after the onset of puberty. The time before puberty explained less than 10% of the variation in LMI, whereas the time after puberty onset explained about 25%.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Boys with PWS showed a recognizable increment in LMI during both spontaneous and induced puberty compared with the prepubertal phase, which was within the trajectories of normal boys. Therefore, timely testosterone substitution in the absence or at arrest of puberty during growth hormone treatment is important to optimize peak LBM in PWS.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1210\/clinem\/dgad101","type":"journal-article","created":{"date-parts":[[2023,3,4]],"date-time":"2023-03-04T12:50:34Z","timestamp":1677934234000},"page":"2299-2306","source":"Crossref","is-referenced-by-count":4,"title":["Lean Body Mass in Boys With Prader-Willi Syndrome Increases Normally During Spontaneous and Induced Puberty"],"prefix":"10.1210","volume":"108","author":[{"given":"Cees","family":"Noordam","sequence":"first","affiliation":[{"name":"Centre for Paediatric Endocrinology Zurich , Zurich 8006 , Switzerland"},{"name":"Department of Paediatrics, Radboud University Medical Centre , Nijmegen 6525 , Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anika","family":"Stephan","sequence":"additional","affiliation":[{"name":"Centre for Paediatric Endocrinology Zurich , Zurich 8006 , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5044-0715","authenticated-orcid":false,"given":"Urs","family":"Eiholzer","sequence":"additional","affiliation":[{"name":"Centre for Paediatric Endocrinology Zurich , Zurich 8006 , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2023,3,6]]},"reference":[{"key":"2023081819562760800_dgad101-B1","first-page":"1260","article-title":"Ein Syndrom von Adipositas, Kleinwuchs, Kryptorchismus und Oligophrenie nach myotonieartigem Zustand im Neugeborenenalter","volume":"86","author":"Prader","year":"1956","journal-title":"Schweiz Med Wochenschr"},{"issue":"4","key":"2023081819562760800_dgad101-B2","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/S2213-8587(21)00002-4","article-title":"Endocrine disorders in Prader-Willi syndrome: a model to understand and treat hypothalamic dysfunction","volume":"9","author":"Tauber","year":"2021","journal-title":"Lancet Diabetes Endocrinol"},{"issue":"5","key":"2023081819562760800_dgad101-B3","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1007\/s004310050832","article-title":"Treatment with human growth hormone in patients with Prader-Labhart-Willi syndrome reduces body fat and increases muscle mass and physical performance","volume":"157","author":"Eiholzer","year":"1998","journal-title":"Eur J Pediatr"},{"issue":"3","key":"2023081819562760800_dgad101-B4","doi-asserted-by":"crossref","first-page":"1131","DOI":"10.1210\/jc.2009-1389","article-title":"Long-term growth hormone therapy changes the natural history of body composition and motor function in children with Prader-Willi syndrome","volume":"95","author":"Carrel","year":"2010","journal-title":"J Clin Endocrinol Metab"},{"issue":"10","key":"2023081819562760800_dgad101-B5","doi-asserted-by":"crossref","first-page":"4013","DOI":"10.1210\/jc.2013-2012","article-title":"Eight years of growth hormone treatment in children with Prader-Willi syndrome: maintaining the positive effects","volume":"98","author":"Bakker","year":"2013","journal-title":"J Clin Endocrinol Metab"},{"issue":"5","key":"2023081819562760800_dgad101-B6","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1067\/mpd.2001.118399","article-title":"Body composition in Prader-Willi syndrome compared with nonsyndromal obesity: relationship to physical activity and growth hormone function","volume":"139","author":"van Mil","year":"2001","journal-title":"J Pediatr"},{"issue":"1","key":"2023081819562760800_dgad101-B7","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1067\/mpd.2003.mpd0334","article-title":"Improving body composition and physical activity in Prader-Willi syndrome","volume":"142","author":"Eiholzer","year":"2003","journal-title":"J Pediatr"},{"key":"2023081819562760800_dgad101-B8","doi-asserted-by":"crossref","first-page":"89","DOI":"10.2147\/VHRM.S168946","article-title":"Obesity paradox in cardiovascular disease: where do we stand?","volume":"15","author":"Carbone","year":"2019","journal-title":"Vasc Health Risk Manag"},{"issue":"9","key":"2023081819562760800_dgad101-B9","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1136\/bjsm.2004.014431","article-title":"Muscular development and physical activity as major determinants of femoral bone mass acquisition during growth","volume":"39","author":"Vicente-Rodriguez","year":"2005","journal-title":"Br J Sports Med"},{"issue":"5","key":"2023081819562760800_dgad101-B10","doi-asserted-by":"crossref","first-page":"1583","DOI":"10.1093\/ajcn\/nqab260","article-title":"Physical activity and fat-free mass during growth and in later life","volume":"114","author":"Westerterp","year":"2021","journal-title":"Am J Clin Nutr"},{"issue":"4","key":"2023081819562760800_dgad101-B11","first-page":"178","article-title":"Schlumpf M, l'Allemand D. Clinical effects of treatment for hypogonadism in male adolescents with Prader-Labhart-Willi syndrome","volume":"68","author":"Eiholzer","year":"2007","journal-title":"Horm Res"},{"issue":"3","key":"2023081819562760800_dgad101-B12","doi-asserted-by":"crossref","first-page":"892","DOI":"10.1210\/jc.2005-0902","article-title":"Hypothalamic and gonadal components of hypogonadism in boys with Prader-Labhart-Willi syndrome","volume":"91","author":"Eiholzer","year":"2006","journal-title":"J Clin Endocrinol Metab"},{"issue":"5-6","key":"2023081819562760800_dgad101-B13","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1159\/000518342","article-title":"Gonadal hormone substitution in people with Prader-Labhart-Willi syndrome: an international Prader-Willi Syndrome Organisation survey","volume":"94","author":"Eiholzer","year":"2021","journal-title":"Horm Res Paediatr"},{"issue":"9","key":"2023081819562760800_dgad101-B14","doi-asserted-by":"crossref","first-page":"e108838","DOI":"10.1371\/journal.pone.0108838","article-title":"Testosterone trajectories and reference ranges in a large longitudinal sample of male adolescents","volume":"9","author":"Khairullah","year":"2014","journal-title":"PLoS One"},{"key":"2023081819562760800_dgad101-B15","first-page":"1","article-title":"Physical growth of Swiss children from birth to 20 years of age. First Zurich longitudinal study of growth and development","volume":"52","author":"Prader","year":"1989","journal-title":"Helv Paediatr Acta Suppl"},{"key":"2023081819562760800_dgad101-B16","first-page":"256","volume-title":"Radiographic Atlas of Skeletal Development of the Hand and Wrist [by] William Walter Greulich [and] S. Idell Pyle","author":"Greulich","year":"1959","edition":"2d ed"},{"issue":"1","key":"2023081819562760800_dgad101-B17","doi-asserted-by":"crossref","first-page":"e12695","DOI":"10.1111\/ijpo.12695","article-title":"Reference charts for body composition parameters by dual-energy X-ray absorptiometry in European children and adolescents aged 6 to 18 years-results from the Austrian LEAD (Lung, hEart, sociAl, boDy) cohort","volume":"16","author":"Ofenheimer","year":"2021","journal-title":"Pediatr Obes"},{"key":"2023081819562760800_dgad101-B18","volume-title":"R: A Language and Environment for Statistical Computing","author":"R Core Team","year":"2021"},{"issue":"1","key":"2023081819562760800_dgad101-B19","doi-asserted-by":"crossref","first-page":"1","DOI":"10.18637\/jss.v067.i01","article-title":"Fitting linear mixed-effects models using lme4","volume":"67","author":"Bates","year":"2015","journal-title":"J Stat Softw"},{"issue":"13","key":"2023081819562760800_dgad101-B20","doi-asserted-by":"crossref","first-page":"1","DOI":"10.18637\/jss.v082.i13","article-title":"Lmertest package: tests in linear mixed effects models","volume":"82","author":"Kuznetsova","year":"2017","journal-title":"J Stat Softw"},{"key":"2023081819562760800_dgad101-B21","author":"Noordam","year":"2022"},{"issue":"9","key":"2023081819562760800_dgad101-B22","doi-asserted-by":"crossref","first-page":"2167","DOI":"10.1002\/ajmg.a.36048","article-title":"Testosterone replacement therapy to improve secondary sexual characteristics and body composition without adverse behavioral problems in adult male patients with Prader-Willi syndrome: an observational study","volume":"161A","author":"Kido","year":"2013","journal-title":"Am J Med Genet A"},{"issue":"5","key":"2023081819562760800_dgad101-B23","doi-asserted-by":"crossref","first-page":"2705","DOI":"10.3390\/ijms22052705","article-title":"Prader-Willi syndrome and hypogonadism: a review article","volume":"22","author":"Noordam","year":"2021","journal-title":"Int J Mol Sci"},{"issue":"11","key":"2023081819562760800_dgad101-B24","doi-asserted-by":"crossref","first-page":"2587","DOI":"10.1093\/humrep\/dev213","article-title":"Sexual dichotomy of gonadal function in Prader-Willi syndrome from early infancy through the fourth decade","volume":"30","author":"Hirsch","year":"2015","journal-title":"Hum Reprod"},{"issue":"3","key":"2023081819562760800_dgad101-B25","doi-asserted-by":"crossref","first-page":"260","DOI":"10.3390\/genes11030260","article-title":"Clinical observations and treatment approaches for scoliosis in Prader-Willi syndrome","volume":"11","author":"van Bosse","year":"2020","journal-title":"Genes (Basel)."},{"issue":"4","key":"2023081819562760800_dgad101-B26","first-page":"200","article-title":"Body composition abnormalities in children with Prader-Willi syndrome and long-term effects of growth hormone therapy","volume":"53","author":"Eiholzer","year":"2000","journal-title":"Horm Res"}],"container-title":["The Journal of Clinical Endocrinology &amp; Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jcem\/advance-article-pdf\/doi\/10.1210\/clinem\/dgad101\/49446735\/dgad101.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jcem\/article-pdf\/108\/9\/2299\/51172056\/dgad101.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jcem\/article-pdf\/108\/9\/2299\/51172056\/dgad101.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,18]],"date-time":"2023-08-18T20:04:59Z","timestamp":1692389099000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jcem\/article\/108\/9\/2299\/7069619"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,6]]},"references-count":26,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2023,3,6]]},"published-print":{"date-parts":[[2023,8,18]]}},"URL":"https:\/\/doi.org\/10.1210\/clinem\/dgad101","relation":{},"ISSN":["0021-972X","1945-7197"],"issn-type":[{"value":"0021-972X","type":"print"},{"value":"1945-7197","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,9]]},"published":{"date-parts":[[2023,3,6]]}}}