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Muscle Nerve. 2017 Dec;56(6):1108-1113. doi: 10.1002/mus.25619. Epub 2017 Jun 5.

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

Author information

1
Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.
2
The Concord Hospital, Concord, New South Wales, Australia.
3
Molecular Medicine Laboratory, Concord Hospital, Concord, New South Wales, Australia.
4
Rady Children's Hospital and Department of Neurosciences, University of California San Diego School of Medicine, San Diego, California, USA.
5
Osteogenesis Imperfecta Department, Kennedy Krieger Institute, Baltimore, Maryland, USA.
6
Department of Neurology, The Johns Hopkins School of Medicine, Baltimore, Maryland, USA.
7
Department of Neuroscience, The Johns Hopkins School of Medicine, Baltimore, Maryland, USA.

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

PMID:
28214289
DOI:
10.1002/mus.25619
[Indexed for MEDLINE]

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