Bone health in facioscapulohumeral muscular dystrophy: A cross‐sectional study. Issue 6 (5th June 2017)

• Record Type: Journal Article
• Authors: Chagarlamudi, Hema; Corbett, Alastair; Stoll, Marion; Bibat, Genila; Grosmann, Carla; Matichak Stock, Carly; Stinson, Nikia; Shapiro, Jay; Wagner, Kathryn R.
• Is Part Of: Muscle & nerve. Volume 56:Issue 6(2017)
• Page Start: 1108
• Page End: 1113
• Publication Date: 2017-06-05
• Subjects: bone density -- bone health -- bone mineral density -- muscular dystrophy -- facioscapulohumeral muscular dystrophy; Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.25619
• Languages: English
• ISSNs: 0148-639X

Abstract

ABSTRACT: Introduction: Herein we provide a comprehensive overview of bone health in facioscapulohumeral muscular dystrophy (FSHD). Methods : Ninety‐four adult individuals with FSHD type 1 from 2 sites were included in this cross‐sectional study. Clinical characteristics and determinants of bone health were examined. Relationships between bone mineral density (BMD), strength, and function were explored. Results : Nearly a third of subjects were deficient in vitamin D3 . Mean whole‐body BMD z ‐score was −0.7; 11% of subjects had greater than age‐related reductions in whole‐body BMD ( z ‐score < −2.0). Whole‐body and regional BMDs were associated with strength and function. Thirty‐six percent had a history of fractures. Likelihood of fractures was reduced for those with normal whole‐body BMD (odds ratio = 0.25, 95% confidence interval 0.04–0.78). Discussion : A diagnosis of FSHD is not necessarily predictive of reduced BMD or increased fracture rate. Given the considerable variability of bone health in the FSHD population, strength and function can serve as predictors of BMD. Muscle Nerve 56 : 1108–1113, 2017