Bone health in facioscapulohumeral muscular dystrophy: A cross-sectional study

Hema Chagarlamudi; Alastair Corbett; Marion Stoll; Genila Bibat; Carla Grosmann; Carly Matichak Stock; Nikia Stinson; Jay Shapiro; Kathryn R. Wagner

doi:10.1002/mus.25619

Bone health in facioscapulohumeral muscular dystrophy: A cross-sectional study

Hema Chagarlamudi, Alastair Corbett, Marion Stoll, Genila Bibat, Carla Grosmann, Carly Matichak Stock, Nikia Stinson, Jay Shapiro, Kathryn R. Wagner

Research output: Contribution to journal › Article › peer-review

3 Scopus citations

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.

Original language	English (US)
Pages (from-to)	1108-1113
Number of pages	6
Journal	Muscle and Nerve
Volume	56
Issue number	6
DOIs	https://doi.org/10.1002/mus.25619
State	Published - Dec 2017

Keywords

bone density
bone health
bone mineral density
facioscapulohumeral muscular dystrophy
muscular dystrophy

ASJC Scopus subject areas

Physiology
Clinical Neurology
Cellular and Molecular Neuroscience
Physiology (medical)

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title = "Bone health in facioscapulohumeral muscular dystrophy: A cross-sectional study",

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.",

keywords = "bone density, bone health, bone mineral density, facioscapulohumeral muscular dystrophy, muscular dystrophy",

author = "Hema Chagarlamudi and Alastair Corbett and Marion Stoll and Genila Bibat and Carla Grosmann and {Matichak Stock}, Carly and Nikia Stinson and Jay Shapiro and Wagner, {Kathryn R.}",

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AU - Chagarlamudi, Hema

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AU - Bibat, Genila

AU - Grosmann, Carla

AU - Matichak Stock, Carly

AU - Stinson, Nikia

AU - Shapiro, Jay

AU - Wagner, Kathryn R.

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N2 - 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.

AB - 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.

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Bone health in facioscapulohumeral muscular dystrophy: A cross-sectional study

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