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Controls were selected from the National Health and Nutrition Examination Survey (<jats:styled-content style=\"fixed-case\">NHANES<\/jats:styled-content>2005\u20102006).<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients<\/jats:title><jats:p>A total of 22 individuals with<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>, each matched by age, sex and race to four<jats:styled-content style=\"fixed-case\">NHANES<\/jats:styled-content>controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Measurements<\/jats:title><jats:p>Blood sampling, oral glucose tolerance test, dual\u2010energy X\u2010ray absorptiometry scan.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>and control groups were 59% female and 29\u00a0\u00b1\u00a08\u00a0years old. Compared to controls, individuals with<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>were shorter but had similar body weight, with more fat and less lean mass. Per cent body fat was higher in<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>even after adjusting for<jats:styled-content style=\"fixed-case\">BMI<\/jats:styled-content>(+2.1% [95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>0.4, 3.9%]). Four<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>patients had abnormal lower extremity fat accumulation resembling lipedema. HbA1c (+0.5% [0.2, 0.7]) and 2\u2010hour glucose (+68\u00a0mg\/dL [44, 93]) were higher in<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>vs controls, differences which persisted after adjusting for<jats:styled-content style=\"fixed-case\">BMI<\/jats:styled-content>. Fasting glucose was comparable between groups.<jats:styled-content style=\"fixed-case\">LDL<\/jats:styled-content>(\u221218\u00a0mg\/<jats:styled-content style=\"fixed-case\">dL<\/jats:styled-content>[\u221235, \u22122]) and triglycerides (\u221245\u00a0mg\/<jats:styled-content style=\"fixed-case\">dL<\/jats:styled-content>[\u221287, \u22122]) were significantly lower in<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>. Whole\u2010body<jats:styled-content style=\"fixed-case\">BMD<\/jats:styled-content>was significantly lower (\u22120.15\u00a0g\/cm<jats:sup>2<\/jats:sup>[\u22120.20, \u22120.11]) in<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>, and this remained true controlling for height (\u22120.06\u00a0g\/cm<jats:sup>2<\/jats:sup>[\u22120.11, \u22120.02]). Vitamin D was &lt;30\u00a0ng\/<jats:styled-content style=\"fixed-case\">mL<\/jats:styled-content>in 81% of those with WS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>On average, adults with<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>have increased fat, decreased lean mass, impaired glucose homeostasis and reduced<jats:styled-content style=\"fixed-case\">BMD<\/jats:styled-content>. Clinical efforts to build muscle and bone mass, and to ensure vitamin D sufficiency, are warranted. Genotype\u2013phenotype research efforts are also warranted.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cen.13829","type":"journal-article","created":{"date-parts":[[2018,8,12]],"date-time":"2018-08-12T13:09:08Z","timestamp":1534079348000},"page":"596-604","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Glucose and lipid metabolism, bone density, and body composition in individuals with Williams syndrome"],"prefix":"10.1111","volume":"89","author":[{"given":"Sofia","family":"Shaikh","sequence":"first","affiliation":[{"name":"Program in Nutritional Metabolism Massachusetts General Hospital and Harvard Medical School Boston Massachusetts"},{"name":"Drexel University College of Medicine Philadelphia Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jessica L.","family":"Waxler","sequence":"additional","affiliation":[{"name":"Division of Genetics Department of Pediatrics Massachusetts General Hospital and Harvard Medical School Boston Massachusetts"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hang","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Biostatistics Massachusetts General Hospital and Harvard Medical School Boston Massachusetts"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kathy","family":"Grinke","sequence":"additional","affiliation":[{"name":"Clinical Research Center Massachusetts General Hospital Boston Massachusetts"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jamie","family":"Garry","sequence":"additional","affiliation":[{"name":"Clinical Research Center Massachusetts General Hospital Boston Massachusetts"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4210-7634","authenticated-orcid":false,"given":"Barbara R.","family":"Pober","sequence":"additional","affiliation":[{"name":"Division of Genetics Department of Pediatrics Massachusetts General Hospital and Harvard Medical School Boston Massachusetts"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takara L.","family":"Stanley","sequence":"additional","affiliation":[{"name":"Program in Nutritional Metabolism Massachusetts General Hospital and Harvard Medical School Boston Massachusetts"},{"name":"Pediatric Endocrine Unit Department of Pediatrics Massachusetts General Hospital and Harvard Medical School Boston Massachusetts"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,9,18]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.30261"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejmg.2017.02.007"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0401516101"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.2337\/diabetes.54.9.2744"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bone.2012.09.024"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.4.12.370"},{"key":"e_1_2_7_8_1","volume-title":"Paper 061\u20102010: Using SAS to Perform Individual Matching in Design of Case\u2010Control Studies","author":"Grandits G","year":"2010"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0185371"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.30400"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1159\/000360476"},{"key":"e_1_2_7_12_1","unstructured":"MasseriniB BedeschiMF BianchiV et\u00a0al.High prevalence of impaired glucose metabolism in young adult patients with Williams syndrome. 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