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Long bone fractures in fibrous dysplasia/McCune-Albright Syndrome: Prevalence, natural history, and risk factors

Journal of Bone and Mineral Research Oct 27, 2021

Geels RES, Meier ME, Saikali A, et al. - Fibrous dysplasia/McCune-Albright Syndrome (FD/MAS) patients experience long bone fractures in relation to a higher skeletal burden score and MAS hyperthyroidism.

  • From 2 large, well-phenotyped cohorts (NIH and LUMC), data were analyzed for 419 patients to identify long bone fractures at FD sites.

  • Occurrence of ≥1 lifetime fracture was evident in 48 (59%) patients, and median age at first fracture was 8 years.

  • A peak in fracture rates occurred between 6–10 years of age, followed by a decline thereafter.

  • Lifetime fracture rate was found to be related to skeletal burden score and MAS hyperthyroidism.

  • Factors linked with a higher total number of lifetime long bone fractures were skeletal burden score > 25 and age at first fracture ≤7 years, and both these factors may predict a more severe clinical course.

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