{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,2]],"date-time":"2026-04-02T15:03:31Z","timestamp":1775142211253,"version":"3.50.1"},"reference-count":37,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2018,5,17]],"date-time":"2018-05-17T00:00:00Z","timestamp":1526515200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Endocrinology"],"published-print":{"date-parts":[[2018,7]]},"abstract":"Summary<\/jats:title>Context<\/jats:title>In fibrous dysplasia (BFD<\/jats:styled-content>), normal bone and bone marrow are replaced by fibro\u2010osseous tissue, leading to fracture, deformity and pain. BFD<\/jats:styled-content> may be isolated, or in association with cutaneous hyperpigmentation and\/or hyperfunctioning endocrinopathies, termed McCune\u2010Albright syndrome (MAS<\/jats:styled-content>). GH<\/jats:styled-content> hypersecretion has been described in 10%\u201020% of MAS<\/jats:styled-content>\u2010BFD<\/jats:styled-content> patients. Aim of the study was to determine the impact of GH<\/jats:styled-content>\u2010insulin like growth factor 1 (IGF<\/jats:styled-content>1) axis hyperactivity on MAS<\/jats:styled-content>\u2010BFD<\/jats:styled-content> morbidities and the efficacy of GH<\/jats:styled-content> excess therapy.<\/jats:p><\/jats:sec>Design and patients<\/jats:title>A multicentric cross\u2010sectional analysis was conducted on three different MAS<\/jats:styled-content> cohorts. From 195 MAS<\/jats:styled-content> patients, 37 subjects (19%) with GH<\/jats:styled-content> excess were identified and compared with 34 MAS<\/jats:styled-content> controls without GH<\/jats:styled-content> hypersecretion.<\/jats:p><\/jats:sec>Results<\/jats:title>Mean head circumference SDS<\/jats:styled-content> was significantly higher in GH<\/jats:styled-content> excess: 4.025 SDS<\/jats:styled-content> vs 0.683 SDS<\/jats:styled-content> (P<\/jats:italic>\u00a0<\u00a0.0001). The risk of optic neuropathy (Odds ratio 4.231; P<\/jats:italic>\u00a0=\u00a0.039), hearing deficit (Odds ratio 2.961; P<\/jats:italic>\u00a0=\u00a0.0481), facial asymmetry (Odds ratio 6.563; P<\/jats:italic>\u00a0=\u00a0.0192), malignancies (Odds ratio 15.24; P<\/jats:italic>\u00a0=\u00a0.0173) were higher in GH<\/jats:styled-content> excess group. Overall, pharmacotherapy (octreotide alone 10\u201030\u00a0mg\/mo or with pegvisomant 10\u201020\u00a0mg\/d) was effective in IGF<\/jats:styled-content>1 normalization (IGF<\/jats:styled-content>1 Z\u2010score between \u22122 and +2 SDS<\/jats:styled-content>) in 21\/29 patients (72.4%) with good compliance to the regimen. Late diagnosis and GH<\/jats:styled-content> excess treatment after 16\u00a0years old of age was associated with an increased risk of optic neuropathy (Odds ratio 4.500; P<\/jats:italic>\u00a0=\u00a0.0491) and growth of pituitary adenomas (Odds ratio 7.846; P<\/jats:italic>\u00a0=\u00a0.050).<\/jats:p><\/jats:sec>Conclusions<\/jats:title>GH<\/jats:styled-content>\u2010IGF<\/jats:styled-content>1 hyperactivity increases risk of morbidities in MAS<\/jats:styled-content>. Medical therapy is effective in normalizing IGF<\/jats:styled-content>1 in most patients, and early treatment during paediatric age is associated with a decreased risk of optic neuropathy and GH<\/jats:styled-content>\u2010secreting adenomas growth.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cen.13722","type":"journal-article","created":{"date-parts":[[2018,4,19]],"date-time":"2018-04-19T20:02:54Z","timestamp":1524168174000},"page":"56-64","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Growth hormone\u2014Insulin\u2010like growth factor 1 axis hyperactivity on bone fibrous dysplasia in McCune\u2010Albright Syndrome"],"prefix":"10.1111","volume":"89","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6484-4371","authenticated-orcid":false,"given":"Daniele","family":"Tessaris","sequence":"first","affiliation":[{"name":"Department of Pediatric Endocrinology Regina Margherita Children's Hospital University of Torino Torino Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alison M","family":"Boyce","sequence":"additional","affiliation":[{"name":"Section on Skeletal Disorders and Mineral Homeostasis NIDCR, NIH Bethesda MD USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margaret","family":"Zacharin","sequence":"additional","affiliation":[{"name":"Department of Endocrinology Murdoch Childrens Research Institute Royal Children's Hospital Parkville VIC Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrizia","family":"Matarazzo","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology Regina Margherita Children's Hospital University of Torino Torino Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberto","family":"Lala","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology Regina Margherita Children's Hospital University of Torino Torino Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luisa","family":"De Sanctis","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology Regina Margherita Children's Hospital University of Torino Torino Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael T","family":"Collins","sequence":"additional","affiliation":[{"name":"Section on Skeletal Disorders and Mineral Homeostasis NIDCR, NIH Bethesda MD USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,5,17]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"crossref","unstructured":"CollinsMT RiminucciM BiancoP.Fibrous dysplasia. 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