Journal article
Cardiac and Pulmonary Function Variability in Duchenne/Becker Muscular Dystrophy: An Initial Report
Journal of child neurology, Vol.25(9), pp.1110-1115
09/2010
DOI: 10.1177/0883073810371003
PMCID: PMC3674812
PMID: 20501885
Abstract
The Duchenne and Becker forms of muscular dystrophy are associated with dilated cardiomyopathy and are diseases in which pulmonary function peaks and then progressively declines. In this report, the authors quantify cardiopulmonary function variability among brothers. Brothers in 3 of 7 eligible sibships had discordant pulmonary function, with significant differences between the brothers’ peak forced vital capacities and their vital capacities at last comparable age. There was no relationship between pulmonary and cardiac function among the siblings. The authors concluded that despite identical genetic mutations, cardiac and pulmonary function variability was common among brothers in their clinic with Duchenne or Becker muscular dystrophy. If confirmed by larger studies, these results have negative implications for the use of genetic testing to predict cardiopulmonary course and response to therapies in Duchenne or Becker muscular dystrophy.
Details
- Title: Subtitle
- Cardiac and Pulmonary Function Variability in Duchenne/Becker Muscular Dystrophy: An Initial Report
- Creators
- David J. Birnkrant - MetroHealth Medical CenterMahi Lakshmi Ashwath - MetroHealth Medical CenterGarey H. Noritz - MetroHealth Medical CenterMichelle C. Merrill - Case Western Reserve UniversityTushar A. Shah - Cincinnati Children's Hospital Medical CenterCarol A. Crowe - Case Western Reserve UniversityRobert C. Bahler - MetroHealth Medical Center
- Resource Type
- Journal article
- Publication Details
- Journal of child neurology, Vol.25(9), pp.1110-1115
- Publisher
- SAGE Publications
- DOI
- 10.1177/0883073810371003
- PMID
- 20501885
- PMCID
- PMC3674812
- ISSN
- 0883-0738
- eISSN
- 1708-8283
- Language
- English
- Date published
- 09/2010
- Academic Unit
- Radiology; Cardiovascular Medicine; Internal Medicine
- Record Identifier
- 9984359568802771
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