{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T05:23:14Z","timestamp":1783488194168,"version":"3.55.0"},"reference-count":22,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2018,4,18]],"date-time":"2018-04-18T00:00:00Z","timestamp":1524009600000},"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":[[2018,5,22]],"date-time":"2018-05-22T00:00:00Z","timestamp":1526947200000},"content-version":"vor","delay-in-days":34,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000862","name":"Doris Duke Charitable Foundation","doi-asserted-by":"publisher","award":["#2014194"],"award-info":[{"award-number":["#2014194"]}],"id":[{"id":"10.13039\/100000862","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005345","name":"American Association for Dental Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005345","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004368","name":"Colgate-Palmolive Company","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004368","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004328","name":"Genentech","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004328","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["asbmr.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2018,9,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:sec>\n                  <jats:title>\u2003<\/jats:title>\n                  <jats:p>Scoliosis is a complication of fibrous dysplasia\/McCune-Albright syndrome (FD\/MAS); however, risk factors and long-term outcomes are unknown. Bisphosphonates are commonly used; however, it is unknown whether their use decrease the risk of progressive scoliosis. Clinical data from the National Institutes of Health (NIH) cohort study was reviewed. Cobb angles were measured, and variables associated with scoliosis progression were identified. Of 138 subjects with available radiographs, 84 (61%) had scoliosis, including 55 (65%) classified as mild (Cobb angle &amp;gt;10 to \u226430 degrees), 11 (13%) as moderate (&amp;gt;30 to \u226445 degrees), and 18 (22%) as severe (&amp;gt;45 degrees). Total skeletal disease burden was highly associated with scoliosis severity (p\u2009&amp;lt;\u20090.0001). Endocrinopathies associated with scoliosis included fibroblast growth factor 23 (FGF23)-mediated hypophosphatemia (p\u2009&amp;lt;\u20090.001) and hyperthyroidism (p\u2009&amp;lt;\u20090.001). Bone turnover markers, including osteocalcin and NTX-telopeptides, were associated with severe scoliosis (p\u2009&amp;lt;\u20090.01). Associations were identified between Cobb angle and functional metrics, including leg length discrepancy (p\u2009&amp;lt;\u20090.01), hip range of motion (p\u2009&amp;lt;\u20090.05), and strength of the gluteus medius and maximus (p\u2009&amp;lt;\u20090.01). Longitudinal analyses were conducted in 69 subjects who had serial radiographs over a median 4.9-year period (range, 0.9 to 14.7 years). Twenty-two subjects were treated with bisphosphonates; there was no difference in Cobb angle progression compared to untreated subjects (0.10 versus 0.53 degrees\/year, p\u2009=\u20090.36). Longitudinal data was available for 10 of 12 subjects treated with spinal fusion; one had instrumentation failure, but in nine subjects Cobb angles were stable with 6.1 years of follow-up (range, 0.9 to 14.7 years). Two fatalities from scoliosis-associated restrictive lung disease occurred in subjects managed non-operatively. Scoliosis occurs frequently in patients with polyostotic FD, and may be potentially fatal. The primary risk factor for progressive scoliosis is total skeletal disease burden. Treatable features that contribute to scoliosis progression include leg length discrepancy, FGF23-mediated hypophosphatemia, and hyperthyroidism. Current data do not support routine use of bisphosphonates to prevent progression of spinal curvature. Spinal fusion is frequently effective in providing long-term stability, and may be lifesaving. Published 2018. This article is a U.S. Government work and is in the public domain in the USA.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1002\/jbmr.3446","type":"journal-article","created":{"date-parts":[[2018,4,18]],"date-time":"2018-04-18T19:26:06Z","timestamp":1524079566000},"page":"1641-1648","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":37,"title":["Scoliosis in Fibrous Dysplasia\/McCune-Albright Syndrome: Factors Associated With Curve Progression and Effects of Bisphosphonates"],"prefix":"10.1093","volume":"33","author":[{"given":"Jason A","family":"Berglund","sequence":"first","affiliation":[{"name":"Tufts University School of Dental MedicineBostonMAUSA"},{"name":"Skeletal Disorders and Mineral Homeostasis SectionNational Institute of Dental and Craniofacial Research (NIDCR)National Institutes of Health (NIH)BethesdaMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sri Harsha","family":"Tella","sequence":"additional","affiliation":[{"name":"Skeletal Disorders and Mineral Homeostasis SectionNational Institute of Dental and Craniofacial Research (NIDCR)National Institutes of Health (NIH)BethesdaMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaitlyn F","family":"Tuthill","sequence":"additional","affiliation":[{"name":"Boston CollegeLynch School of EducationDepartment of Measurement, Evaluation, Statistics, and AssessmentChestnut HillMAUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren","family":"Kim","sequence":"additional","affiliation":[{"name":"Radiology and Imaging SciencesMark O. Hatfield Clinical Research CenterNIHBethesdaMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lori C","family":"Guthrie","sequence":"additional","affiliation":[{"name":"Skeletal Disorders and Mineral Homeostasis SectionNational Institute of Dental and Craniofacial Research (NIDCR)National Institutes of Health (NIH)BethesdaMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott M","family":"Paul","sequence":"additional","affiliation":[{"name":"Rehabilitation Medicine DepartmentMark O. Hatfield Clinical Research CenterNIHBethesdaMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Stanton","sequence":"additional","affiliation":[{"name":"Department of OrthopedicsNemours Children's Health SystemOrlandoFLUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael T","family":"Collins","sequence":"additional","affiliation":[{"name":"Skeletal Disorders and Mineral Homeostasis SectionNational Institute of Dental and Craniofacial Research (NIDCR)National Institutes of Health (NIH)BethesdaMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alison M","family":"Boyce","sequence":"additional","affiliation":[{"name":"Skeletal Disorders and Mineral Homeostasis SectionNational Institute of Dental and Craniofacial Research (NIDCR)National Institutes of Health (NIH)BethesdaMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2018,4,18]]},"reference":[{"issue":"12","key":"2024021014365720300_jbmr3446-bib-0001","doi-asserted-by":"crossref","first-page":"5459","DOI":"10.1210\/en.2004-0865","article-title":"Minireview: GNAS: normal and abnormal functions","volume":"145","author":"Weinstein","year":"2004","journal-title":"Endocrinology"},{"issue":"11","key":"2024021014365720300_jbmr3446-bib-0002","doi-asserted-by":"crossref","first-page":"5152","DOI":"10.1073\/pnas.89.11.5152","article-title":"Identification of a mutation in the gene encoding the alpha subunit of the stimulatory G protein of adenylyl cyclase in McCune-Albright syndrome","volume":"89","author":"Schwindinger","year":"1992","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2024021014365720300_jbmr3446-bib-0003","author":"Boyce","year":"2015"},{"issue":"Suppl 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