Resistance exercise training with protein supplementation improves skeletal muscle strength and improves quality of life in late adolescents and young adults with Barth syndrome: A pilot study
Kathryn L. Bohnert,
Grace Ditzenberger,
Adam J. Bittel,
Lisa de lasFuentes,
Manuela Corti,
Christina A. Pacak,
Carolyn Taylor,
Barry J. Byrne,
Dominic N. Reeds,
W. Todd Cade
Affiliations
Kathryn L. Bohnert
Program in Physical Therapy Washington University School of Medicine St. Louis Missouri USA
Grace Ditzenberger
Doctor of Physical Therapy Division Duke University School of Medicine Durham North Carolina USA
Adam J. Bittel
Program in Physical Therapy Washington University School of Medicine St. Louis Missouri USA
Lisa de lasFuentes
Department of Medicine Washington University School of Medicine St. Louis Missouri USA
Manuela Corti
Department of Pediatrics University of Florida School of Medicine Gainesville Florida USA
Christina A. Pacak
Department of Pediatrics University of Florida School of Medicine Gainesville Florida USA
Carolyn Taylor
Department of Pediatrics Medical University of South Carolina Chaleston South Carolina USA
Barry J. Byrne
Department of Pediatrics University of Florida School of Medicine Gainesville Florida USA
Dominic N. Reeds
Department of Medicine Washington University School of Medicine St. Louis Missouri USA
W. Todd Cade
Program in Physical Therapy Washington University School of Medicine St. Louis Missouri USA
Abstract Background Muscle weakness and exercise intolerance contribute to reduced quality of life (QOL) in Barth syndrome (BTHS). Our group previously found that 12 weeks of resistance exercise training (RET) improved muscle strength, however, did not increase muscle (lean) mass or QOL in n = 3 young adults with BTHS. The overall objective of this pilot study was to examine the safety and effectiveness of RET plus daily protein supplementation (RET + protein) on muscle strength, skeletal muscle mass, exercise tolerance, cardiac function, and QOL in late adolescents/young adults with BTHS. Methods Participants with BTHS (n = 5, age 27 ± 7) performed 12 weeks of supervised RET (60 minutes per session, three sessions/week) and consumed 42 g/day of whey protein. Muscle strength, muscle mass, exercise capacity, cardiac function, and health‐related QOL were assessed pre‐post intervention. Results RET + protein was safe, increased muscle strength and quality of life, and tended to increase lean mass. Conclusions RET + protein appears safe, increases muscle strength and quality of life and tends to increase lean mass. Larger studies are needed to confirm these findings and to fully determine the effects of RET + protein in individuals with BTHS.