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Neuromuscul Disord. 2014 Jun;24(6):482-91. doi: 10.1016/j.nmd.2014.03.008. Epub 2014 Mar 22.

A systematic review and meta-analysis on the epidemiology of Duchenne and Becker muscular dystrophy.

Author information

1
Department of Clinical Neurosciences, University of Calgary, Canada; Department of Pediatrics, Alberta Children's Hospital Research Institute, University of Calgary, Canada. Electronic address: jean.mah@albertahealthservices.ca.
2
Department of Clinical Neurosciences, University of Calgary, Canada; Hotchkiss Brain Institute, University of Calgary, Canada.
3
Department of Clinical Neurosciences, University of Calgary, Canada; Hotchkiss Brain Institute, University of Calgary, Canada; Department of Community Health Sciences and Institute for Public Health, University of Calgary, Canada; Clinical Research Unit, University of Calgary, Canada.
4
Department of Clinical Neurosciences, University of Calgary, Canada.
5
Department of Clinical Neurosciences, University of Calgary, Canada; Department of Pediatrics, Alberta Children's Hospital Research Institute, University of Calgary, Canada.
6
Department of Clinical Neurosciences, University of Calgary, Canada; Hotchkiss Brain Institute, University of Calgary, Canada; Department of Community Health Sciences and Institute for Public Health, University of Calgary, Canada.

Abstract

The muscular dystrophies are a broad group of hereditary muscle diseases with variable severity. Population-based prevalence estimates have been reported but pooled estimates are not available. We performed a systematic review of worldwide population-based studies reporting muscular dystrophies prevalence and/or incidence using MEDLINE and EMBASE databases. The search strategy included key terms related to muscular dystrophies, incidence, prevalence and epidemiology. Two reviewers independently reviewed all abstracts, full text articles and abstracted data using standardized forms. Pooling of prevalence estimates was performed using random effect models. 1104 abstracts and 167 full text articles were reviewed. Thirty-one studies met all eligibility criteria and were included in the final analysis. The studies differed widely in their approaches to case ascertainment, resulting in significant methodological heterogeneity and varied data quality. The pooled prevalence of DMD and BMD was 4.78 (95% CI 1.94-11.81) and 1.53 (95% CI 0.26-8.94) per 100,000 males respectively. The incidence of DMD ranged from 10.71 to 27.78 per 100,000. This is the first meta-analysis of worldwide prevalence estimates for muscular dystrophies. There is a need for more epidemiological studies addressing global estimates on incidence and prevalence of muscular dystrophies, utilizing standardized diagnostic criteria as well as multiple sources of case ascertainment.

KEYWORDS:

Epidemiology; Incidence; Muscular dystrophy; Population-based; Prevalence

PMID:
24780148
DOI:
10.1016/j.nmd.2014.03.008
[Indexed for MEDLINE]

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