title: Bone health in facioscapulohumeral muscular dystrophy: A cross-sectional study.
abstract: BACKGROUND
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.
authors.forename: Hema
authors.initials: H
authors.lastname: Chagarlamudi
authors.affiliation: The Concord Hospital, Concord, New South Wales, Australia.
authors.forename: Alastair
authors.initials: A
authors.lastname: Corbett
authors.affiliation: Molecular Medicine Laboratory, Concord Hospital, Concord, New South Wales, Australia.
authors.forename: Marion
authors.initials: M
authors.lastname: Stoll
authors.affiliation: Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.
authors.forename: Genila
authors.initials: G
authors.lastname: Bibat
authors.affiliation: Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.|Rady Children's Hospital and Department of Neurosciences, University of California San Diego School of Medicine, San Diego, California, USA.
authors.forename: Carla
authors.initials: C
authors.lastname: Grosmann
authors.affiliation: Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.
authors.forename: Carly
authors.initials: C
authors.lastname: Matichak Stock
authors.affiliation: Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.
authors.forename: Nikia
authors.initials: N
authors.lastname: Stinson
authors.affiliation: Osteogenesis Imperfecta Department, Kennedy Krieger Institute, Baltimore, Maryland, USA.
authors.forename: Jay
authors.initials: J
authors.lastname: Shapiro
authors.affiliation: Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.|Department of Neurology, The Johns Hopkins School of Medicine, Baltimore, Maryland, USA.|Department of Neuroscience, The Johns Hopkins School of Medicine, Baltimore, Maryland, USA.
authors.forename: Kathryn R
authors.initials: KR
authors.lastname: Wagner
mesh_terms: D015502:Absorptiometry, Photon / Q000379:methods; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015142:Baltimore / Q000453:epidemiology; D015519:Bone Density / Q000502:physiology*; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000000981:diagnostic imaging* / Q000453:epidemiology* / Q000503:physiopathology; D009517:New South Wales / Q000453:epidemiology; D055815:Young Adult
keywords: bone density; bone health; bone mineral density; facioscapulohumeral muscular dystrophy; muscular dystrophy