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    Bone Health in Facioscapulohumeral Muscular Dystrophy: A Cross-Sectional Study

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    Authors
    Chagarlamudi, Hema
    Corbett, Alastair
    Stoll, Marion
    Bibat, Genila
    Grosmann, Carla
    Stock, Carly Matichak
    Stinson, Nikia
    Shapiro, Jay
    Wagner, Kathryn
    UMass Chan Affiliations
    Wellstone Center for FSHD
    Document Type
    Journal Article
    Publication Date
    2017-02-18
    Keywords
    Muscular Dystrophy
    Bone Density
    Bone Health
    Bone Mineral Density
    Facioscapulohumeral Muscular Dystrophy (FSHD)
    Cell Biology
    Developmental Biology
    Molecular Biology
    Molecular Genetics
    Musculoskeletal Diseases
    Nervous System Diseases
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    Link to Full Text
    https://doi.org/10.1002/mus.25619
    Abstract
    INTRODUCTION: We provide a comprehensive overview of bone health in facioscapulohumeral muscular dystrophy (FSHD). METHODS: Ninety-four adult individuals with FSHD1 from two 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% had greater than age-related reductions in whole body BMD (z-score < -2.0). Whole body and regional BMD were associated with strength and function. Thirty-six percent had a history of fractures. Likelihood for fractures was reduced for those with normal whole body BMD (OR=0.25, 95% CI: 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. This article is protected by copyright.
    Source
    Muscle Nerve. 2017 Feb 18. doi: 10.1002/mus.25619. Link to article on publisher's site
    DOI
    10.1002/mus.25619
    Permanent Link to this Item
    http://hdl.handle.net/20.500.14038/50567
    PubMed ID
    28214289
    Related Resources
    Link to Article in PubMed
    ae974a485f413a2113503eed53cd6c53
    10.1002/mus.25619
    Scopus Count
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    UMass Chan Faculty and Researcher Publications
    Wellstone Center for FSHD Publications

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