Childhood Activity on Progression in Limb Girdle Muscular Dystrophy 2I

Brianna N Brun; Shelley R H Mockler; Katie M Laubscher; Carrie M Stephan; Julia A Collison; M Bridget Zimmerman; Katherine D Mathews

doi:10.1177/0883073816677680

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Childhood Activity on Progression in Limb Girdle Muscular Dystrophy 2I

Journal article

Peer reviewed

Childhood Activity on Progression in Limb Girdle Muscular Dystrophy 2I

Brianna N Brun, Shelley R H Mockler, Katie M Laubscher, Carrie M Stephan, Julia A Collison, M Bridget Zimmerman and Katherine D Mathews

Journal of child neurology, Vol.32(2), pp.204-209

02/2017

DOI: 10.1177/0883073816677680

PMCID: PMC5464953

PMID: 27872178

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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.

Humans

Middle Aged

Muscular Dystrophies, Limb-Girdle - physiopathology

Self Report

Disease Progression

Proteins - genetics

Young Adult

Vital Capacity

Exercise

Muscular Dystrophies, Limb-Girdle - genetics

Adolescent

Age of Onset

Adult

Aged

Retrospective Studies

Walk Test

Child

Sports

Details

Title: Subtitle: Childhood Activity on Progression in Limb Girdle Muscular Dystrophy 2I
Creators: Brianna N Brun - 1 Department of Pediatrics, University of Iowa Hospitals and Clinics, Iowa City, IA, USA
Shelley R H Mockler - 2 Center for Disabilities and Development, University of Iowa Children's Hospital, Iowa City, IA, USA
Katie M Laubscher - 2 Center for Disabilities and Development, University of Iowa Children's Hospital, Iowa City, IA, USA
Carrie M Stephan - 1 Department of Pediatrics, University of Iowa Hospitals and Clinics, Iowa City, IA, USA
Julia A Collison - 1 Department of Pediatrics, University of Iowa Hospitals and Clinics, Iowa City, IA, USA
M Bridget Zimmerman - 3 Department of Biostatistics, College of Public Health, University of Iowa, Iowa City, IA, USA
Katherine D Mathews - 4 Department of Neurology, University of Iowa Hospitals and Clinics, Iowa City, IA, USA
Resource Type: Journal article
Publication Details: Journal of child neurology, Vol.32(2), pp.204-209
DOI: 10.1177/0883073816677680
PMID: 27872178
PMCID: PMC5464953
NLM abbreviation: J Child Neurol
ISSN: 0883-0738
eISSN: 1708-8283
Publisher: United States
Grant note: U54 NS053672 / NINDS NIH HHS U54 TR001356 / NCATS NIH HHS
Language: English
Date published: 02/2017
Academic Unit: Neurology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Biostatistics; Physical Therapy and Rehabilitation Science; Neurology (Pediatrics)
Record Identifier: 9983997323102771

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