Dataset Information

Reduced Muscle Strength in Barth Syndrome May Be Improved by Resistance Exercise Training: A Pilot Study.

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.

SUBMITTER: Bittel AJ

PROVIDER: S-EPMC6122057 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Reduced Muscle Strength in Barth Syndrome May Be Improved by Resistance Exercise Training: A Pilot Study.

Bittel Adam J AJ Bohnert Kathryn L KL Reeds Dominic N DN Peterson Linda R LR de Las Fuentes Lisa L Corti Manuela M Taylor Carolyn L CL Byrne Barry J BJ Cade W Todd WT

JIMD reports 20180414

<h4>Background</h4>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, ...[more]

PMID: 29654548

Similar Datasets

Project description:BACKGROUND:Barth syndrome (BTHS) is an X-linked disorder caused by defects in TAZ with key clinical features including cardiomyopathy, neutropenia and skeletal myopathy. In order to gain a better understanding of the range of clinical features, identify targets for monitoring, and increase knowledge of natural history of the disease, we conducted muscle strength testing, functional exercise capacity testing, physical activity assessment, balance assessment and motion reaction time testing in 33 affected individuals and 14 controls. We analyzed data points to provide a cross-sectional quantitative spectrum of disease characteristics. We also compared these data points to the matched data points collected two years prior to provide insight into effects of BTHS over time. RESULTS:In comparison to controls, pediatric subjects with BTHS present with significantly impaired balance and motion reaction time while adult subjects with BTHS present with significantly impaired motion reaction time. In comparison to controls, subjects with BTHS presented with decreased functional exercise capacity (assessed via 6 MWT), knee extensor strength (both assessed via handheld dynamometry and five times sit-to-stand (5 TSTS)), and self-reported physical activity. Comparison of functional exercise capacity, knee extensor strength and self-reported physical activity from identical cohorts in 2014 and 2016 BTHS showed that the deficits in 6 MWT do not change significantly over the 2?year time span. CONCLUSION:In this comprehensive assessment of musculoskeletal parameters in a cross-section of individuals with BTHS, we uncovered deficits in motion reaction time and balance, which were previously not known to be abnormal in in BTHS. We also confirmed results of our previous study showing that pediatric and adult subjects with BTHS have decreased functional exercise capacity, knee extensor strength, and physical activity in comparison to controls, r, verifying the importance of including these measures as part of the regular clinical assessment in individuals with BTHS, as well as introducing 5 TSTS as an additional testing parameter. Perhaps most importantly, we demonstrated that 6 MWT results do not significantly vary in pediatric and adult cohorts with BTHS over a 2-year period, supporting this as a reliable quantitative measure of therapeutic outcomes in clinical studies and for clinical monitoring.

Project description:Background:In addition to generally high levels of physical activity, multi-component exercise training is recommended for the maintenance of health and fitness in older adults, including the prevention of falls and frailty. This training often encompasses serial sequencing of balance, strength, endurance and other types of exercise. Exercise training featuring integrative training of these components (i.e. agility training) has been proposed, as it more likely reflects real life challenges like stop-and-go patterns, cutting manoeuvers, turns and decision-making. In this study, we compared the efficacy of an agility-based training to the traditional strength and balance training approach with regard to selected risk factors for falls and frailty. Methods:We trained twenty-seven community-dwelling healthy seniors (16?; 11?; age: 69.5 ± 5.3 y; BMI: 26.4 ± 3.7 kg/m2) for 8 weeks in a group setting with 3 sessions per week, each lasting 50 minutes. Participants were randomized into either the agility group (AGI; n = 12), that used the integrative multi-component training, or the traditional strength and balance group (TSB; n = 15). TSB performed balance and strength exercises separately, albeit within the same session. The training of both groups progressively increased in difficulty. Outcomes were static and dynamic balance (single leg eyes open stand, Y-balance test, reactive balance), lower limb (plantar flexion and dorsal extension) and trunk flexion and extension maximum strength and rate of torque development (RTD). In addition, we tested endurance by the six-minute walk test (6MWT). We calculated linear mixed effects models for between-groups comparisons as well as effect sizes (ES) with 95 % confidence intervals. Results:Small ES in favor of AGI were found for plantar flexion strength (ES > 0.18[-0.27;0.89]) and RTD (ES > 0.43[-0.19;1.36]) as well as trunk extension RTD (ES = 0.35[-0.05;0.75]). No other parameters showed notable between group differences. Compliance was high in both groups (AGI: 90 ± 8% of sessions; TSB: 91 ± 7% of sessions). Discussion:Agility-based exercise training seems at least as efficacious as traditional strength and balance training in affecting selected physical performance indicators among community-dwelling healthy seniors. In particular, lower limb and trunk extension explosive strength seem to benefit from the agility training.

Dataset Information

Reduced Muscle Strength in Barth Syndrome May Be Improved by Resistance Exercise Training: A Pilot Study.

Background

Methods

Results

Conclusions

Publications

Reduced Muscle Strength in Barth Syndrome May Be Improved by Resistance Exercise Training: A Pilot Study.

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