{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T04:10:42Z","timestamp":1791951042215,"version":"4.3.4"},"reference-count":31,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T00:00:00Z","timestamp":1606780800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"},{"start":{"date-parts":[[2021,11,17]],"date-time":"2021-11-17T00:00:00Z","timestamp":1637107200000},"content-version":"vor","delay-in-days":351,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100005040","name":"Bontius Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005040","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000072","name":"National Institute of Dental and Craniofacial Research","doi-asserted-by":"publisher","award":["DE-000758-01"],"award-info":[{"award-number":["DE-000758-01"]}],"id":[{"id":"10.13039\/100000072","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005040","name":"Bontius Stichting","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005040","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000072","name":"National Institute of Dental and Craniofacial Research","doi-asserted-by":"publisher","award":["DE\u2010000758\u201001"],"award-info":[{"award-number":["DE\u2010000758\u201001"]}],"id":[{"id":"10.13039\/100000072","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["asbmr.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2020,12,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Fibrous dysplasia\/McCune-Albright syndrome (FD\/MAS) is a rare bone and endocrine disorder arising along a broad spectrum. Long-bone fractures are a common, painful, and potentially disabling complication. However, fracture prevalence and risk factors have not been well-established, making it difficult to predict which patients are at risk for a severe course. Clinical and imaging data were reviewed from two large, well-phenotyped cohorts (National Institutes of Health [NIH] in the United States and the Leiden University Medical Center [LUMC] in the Netherlands) to identify long-bone fractures at FD sites. Skeletal burden score was quantified using bone scintigraphy. Multiple linear regressions were performed to identify clinical associations with fractures. A total of 419 patients were included (186 NIH, 233 LUMC); 194 (46%) had MAS endocrinopathies. Median age at last follow-up was 30.2\u2009years (range 3.2\u201384.6, interquartile range [IQR] 25.5), and median skeletal burden score was 16.6 (range 0\u201375, IQR 33). A total of 48 (59%) patients suffered one or more lifetime fracture (median 1, range 0\u201370, IQR 4). Median age at first fracture was 8\u2009years (range 1\u201376, IQR 10). Fracture rates peaked between 6 and 10\u2009years of age and decreased thereafter. Lifetime fracture rate was associated with skeletal burden score (\u03b2\u00a0=\u00a00.40, p\u2009&amp;lt;\u20090.01) and MAS hyperthyroidism (\u03b2\u00a0=\u00a00.22, p\u00a0=\u00a00.01). Younger age at first fracture was associated with skeletal burden score (\u03b2\u00a0=\u00a0\u22120.26, p\u00a0=\u00a00.01) and male sex (\u03b2\u00a0=\u00a0\u22120.23, p\u00a0=\u00a00.01). Both skeletal burden score &amp;gt;25 and age at first fracture \u22647\u2009years were associated with a higher total number of lifetime fractures (median 4, range 1\u201370, IQR 5 versus median 1, range 1\u201313, IQR 1) (p\u2009&amp;lt;\u20090.01). In conclusion, higher skeletal burden score and MAS hyperthyroidism are associated with long-bone fractures in FD\/MAS. Both skeletal burden score \u226525 and age at first fracture \u22647\u2009years are associated with a higher lifetime long-bone fracture risk and may predict a more severe clinical course. These results may allow clinicians to identify FD\/MAS patients at risk for severe disease who may be candidates for early therapeutic interventions. \u00a9 2021 American Society for Bone and Mineral Research (ASBMR). This article has been contributed to by US Government employees and their work is in the public domain in the USA.<\/jats:p>","DOI":"10.1002\/jbmr.4463","type":"journal-article","created":{"date-parts":[[2021,10,20]],"date-time":"2021-10-20T18:16:47Z","timestamp":1634753807000},"page":"236-243","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Long Bone Fractures in Fibrous Dysplasia\/McCune-Albright Syndrome: Prevalence, Natural History, and Risk Factors"],"prefix":"10.1093","volume":"37","author":[{"given":"Raya E.S.","family":"Geels","sequence":"first","affiliation":[{"name":"Department of Medicine, Division of Endocrinology, Centre for Bone Quality Leiden University Medical Centre Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6258-2531","authenticated-orcid":false,"given":"Maartje E.","family":"Meier","sequence":"additional","affiliation":[{"name":"Department of Orthopedic Surgery Leiden University Medical Centre Leiden The Netherlands"},{"name":"Department of Medicine, Division of Endocrinology, Centre for Bone Quality Leiden University Medical Centre Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda","family":"Saikali","sequence":"additional","affiliation":[{"name":"Skeletal Disorders and Mineral Homeostasis Section, National Institute of Dental and Craniofacial Research National Institutes of Health Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roula","family":"Tsonaka","sequence":"additional","affiliation":[{"name":"Department of Medical Statistics and Bioinformatics, Einthovenweg Leiden University Medical Center Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5035-127X","authenticated-orcid":false,"given":"Natasha M.","family":"Appelman-Dijkstra","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Endocrinology, Centre for Bone Quality Leiden University Medical Centre Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2725-2908","authenticated-orcid":false,"given":"Alison M.","family":"Boyce","sequence":"additional","affiliation":[{"name":"Metabolic Bone Disorders Unit National Institute of Dental and Craniofacial Research, National Institutes of Health Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,10,20]]},"reference":[{"issue":"5","key":"2024012714013556100_jbmr4463-bib-0001","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1001\/archsurg.1938.01190230153012","article-title":"Polyostotic fibrous dysplasia","volume":"36","author":"Lichtenstein","year":"1938","journal-title":"Arch Surg"},{"issue":"17","key":"2024012714013556100_jbmr4463-bib-0002","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1056\/NEJM193704292161701","article-title":"Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females: report of five cases","volume":"216","author":"Albright","year":"1937","journal-title":"N Engl J Med"},{"issue":"4","key":"2024012714013556100_jbmr4463-bib-0003","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1359\/JBMR.0301262","article-title":"Fracture incidence in 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