Reduced muscle strength in barth syndrome may be improved by resistance exercise training: A pilot study

Adam J. Bittel, Kathryn L. Bohnert, Dominic N. Reeds, Linda R. Peterson, Lisa de las Fuentes, Manuela Corti, Carolyn L. Taylor, Barry J. Byrne, W. Todd Cade

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Scopus citations

Abstract

Background: Cardioskeletal myopathy is thought to contribute to exercise intolerance, and reduced quality of life (QOL) in Barth syndrome (BTHS). The objectives of this study were to examine: (1) skeletal muscle strength/performance in adolescents and young adults with BTHS and (2) the safety, feasibility, and initial efficacy of 12 weeks of progressive resistance exercise training (RET) on muscle strength, mass, and performance, bone mineral density, exercise tolerance, cardiac function, and QOL in individuals with BTHS. Methods: Individuals with BTHS (n = 9, 23 ± 6 years), and age-, sex-, and activity level-matched unaffected Controls (n = 7, 26 ± 5 years) underwent baseline testing to assess muscle performance, exercise capacity, cardiac structure and function, body composition, and health-related QOL. Subsequently, n = 3 participants with BTHS performed 12 weeks of supervised RET (60 min per session, 3 sessions/week). All testing was repeated post-RET. Results: BTHS had lower strength and lean muscle mass compared to Controls (all p < 0.05). BTHS also had diminished lower extremity, upper extremity, thoracic spine, lumbar spine, and pelvic bone mineral density (all p < 0.05) and reduced exercise capacity (p < 0.001) compared to Controls. RET was well-tolerated and attended, was not associated with any adverse events, and significantly increased muscle strength (p < 0.05). Conclusions: Individuals with BTHS demonstrate reduced muscle strength and mass, bone mineral density, and exercise capacity. RET appears safe and well-tolerated in BTHS and promotes increased muscle strength. Larger studies are needed to confirm these improvements and to fully determine the effects of RET in individuals with BTHS.

Original languageEnglish
Title of host publicationJIMD Reports
PublisherSpringer
Pages63-72
Number of pages10
DOIs
StatePublished - Jan 1 2018

Publication series

NameJIMD Reports
Volume41
ISSN (Print)2192-8304
ISSN (Electronic)2192-8312

Keywords

  • Barth syndrome
  • Mitochondria
  • Muscle
  • Resistance exercise

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    Bittel, A. J., Bohnert, K. L., Reeds, D. N., Peterson, L. R., de las Fuentes, L., Corti, M., Taylor, C. L., Byrne, B. J., & Todd Cade, W. (2018). Reduced muscle strength in barth syndrome may be improved by resistance exercise training: A pilot study. In JIMD Reports (pp. 63-72). (JIMD Reports; Vol. 41). Springer. https://doi.org/10.1007/8904_2018_102