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Clinical Trial
. 2017 Feb;32(2):204-209.
doi: 10.1177/0883073816677680. Epub 2016 Nov 22.

Childhood Activity on Progression in Limb Girdle Muscular Dystrophy 2I

Brianna N Brun  1 , Shelley R H Mockler  2 , Katie M Laubscher  2 , Carrie M Stephan  1 , Julia A Collison  1 , M Bridget Zimmerman  3 , Katherine D Mathews  1   4
Affiliations
Clinical Trial

Childhood Activity on Progression in Limb Girdle Muscular Dystrophy 2I

Brianna N Brun et al. J Child Neurol. 2017 Feb.

Abstract

Limb girdle muscular dystrophy 2I is a slowly progressive muscular dystrophy due to mutations in the Fukutin-related protein ( FKRP) gene. Clinicians are frequently asked if physical activity is harmful for pediatric patients with limb girdle muscular dystrophy 2I. The primary objective of this study was to determine if there is a relationship between self-reported childhood activity level and motor function and respiratory function in older children and adults with limb girdle muscular dystrophy 2I. We compared retrospective self-reported middle school activity level and sport participation with age at onset of weakness, 10-meter walk test, and forced vital capacity later in life in 41 participants with FKRP mutations. We found no relationship between activity level in childhood and disease course later in life, suggesting that self-directed physical activity in children with limb girdle muscular dystrophy 2I does not negatively affect disease progression and outcome.

Keywords: FKRP; dystroglycanopathy; exercise; muscular dystrophies; α-dystroglycan.

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Conflict of interest statement

Declaration of Conflicting Interests

The authors disclose no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1
Figure 1
Box plot of (A) 10-meter walk test time (10MWT) z-scores, (B) forced vital capacity (FVC) percent predicted, and (C) age of onset by middle school (MS) activity levels. Box plots show median values (middle line), 50th percentile (box outline), minimum/maximum (whiskers), and mean (diamond).
Figure 2
Figure 2
Bar graphs depicting number of individuals (A) not involved in sports/lessons, (B) involved in sports or lessons, and (C) involved in both sports and lessons across middle school activity levels.
See this image and copyright information in PMC

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