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Growth hormone—Insulin‐like growth factor 1 axis hyperactivity on bone fibrous dysplasia in McCune‐Albright Syndrome
Author(s) -
Tessaris Daniele,
Boyce Alison M,
Zacharin Margaret,
Matarazzo Patrizia,
Lala Roberto,
De Sanctis Luisa,
Collins Michael T
Publication year - 2018
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1111/cen.13722
Subject(s) - medicine , odds ratio , endocrinology , risk factor , acromegaly , gastroenterology , hormone , growth hormone
Summary Context In fibrous dysplasia ( BFD ), normal bone and bone marrow are replaced by fibro‐osseous tissue, leading to fracture, deformity and pain. BFD may be isolated, or in association with cutaneous hyperpigmentation and/or hyperfunctioning endocrinopathies, termed McCune‐Albright syndrome ( MAS ). GH hypersecretion has been described in 10%‐20% of MAS ‐ BFD patients. Aim of the study was to determine the impact of GH ‐insulin like growth factor 1 ( IGF 1) axis hyperactivity on MAS ‐ BFD morbidities and the efficacy of GH excess therapy. Design and patients A multicentric cross‐sectional analysis was conducted on three different MAS cohorts. From 195 MAS patients, 37 subjects (19%) with GH excess were identified and compared with 34 MAS controls without GH hypersecretion. Results Mean head circumference SDS was significantly higher in GH excess: 4.025 SDS vs 0.683 SDS ( P < .0001). The risk of optic neuropathy (Odds ratio 4.231; P = .039), hearing deficit (Odds ratio 2.961; P = .0481), facial asymmetry (Odds ratio 6.563; P = .0192), malignancies (Odds ratio 15.24; P = .0173) were higher in GH excess group. Overall, pharmacotherapy (octreotide alone 10‐30 mg/mo or with pegvisomant 10‐20 mg/d) was effective in IGF 1 normalization ( IGF 1 Z‐score between −2 and +2 SDS ) in 21/29 patients (72.4%) with good compliance to the regimen. Late diagnosis and GH excess treatment after 16 years old of age was associated with an increased risk of optic neuropathy (Odds ratio 4.500; P = .0491) and growth of pituitary adenomas (Odds ratio 7.846; P = .050). Conclusions GH ‐ IGF 1 hyperactivity increases risk of morbidities in MAS . Medical therapy is effective in normalizing IGF 1 in most patients, and early treatment during paediatric age is associated with a decreased risk of optic neuropathy and GH ‐secreting adenomas growth.