ABSTRACTPurposeWe determined the effects of an innovative 8-week exercise intervention (aerobic, resistance and inspiratory muscle training) for patients with mitochondrial disease (MD).MethodsSeveral endpoints were assessed in 12 patients (19–59 years, 4 female) at pre-training, post-training and after 4-week detraining: aerobic power, muscle strength/power and maximal inspiratory pressure (main endpoints), ability to perform activities of daily living (ADL), body composition, quality of life and blood myokines (secondary endpoints).ResultsThe program was safe with patients' adherence being 94±5%. A significant time-effect was found for virtually all main endpoints (P≤0.004), indicating a training improvement. Similar findings (P≤0.003) were found for ADL tests, total/trunk/leg lean mass, total fat mass, femoral fracture risk and general health perception. No differences were found for blood myokines, except for an acute exertional increase in interleukin-8 at post-training/detraining (P=0.002) and in fatty acid binding protein 3 at detraining (P=0.002).ConclusionAn intervention including novel exercises for MD patients (e.g., inspiratory muscle training) produced benefits in numerous indicators of physical capacity, and induced a previously unreported shift towards a healthier body composition phenotype. Purpose We determined the effects of an innovative 8-week exercise intervention (aerobic, resistance and inspiratory muscle training) for patients with mitochondrial disease (MD). Methods Several endpoints were assessed in 12 patients (19–59 years, 4 female) at pre-training, post-training and after 4-week detraining: aerobic power, muscle strength/power and maximal inspiratory pressure (main endpoints), ability to perform activities of daily living (ADL), body composition, quality of life and blood myokines (secondary endpoints). Results The program was safe with patients' adherence being 94±5%. A significant time-effect was found for virtually all main endpoints (P≤0.004), indicating a training improvement. Similar findings (P≤0.003) were found for ADL tests, total/trunk/leg lean mass, total fat mass, femoral fracture risk and general health perception. No differences were found for blood myokines, except for an acute exertional increase in interleukin-8 at post-training/detraining (P=0.002) and in fatty acid binding protein 3 at detraining (P=0.002). Conclusion An intervention including novel exercises for MD patients (e.g., inspiratory muscle training) produced benefits in numerous indicators of physical capacity, and induced a previously unreported shift towards a healthier body composition phenotype. First two authors contributed equally to this work, Carmen Fiuza-Luces, Jorge Díez-Bermejo Corresponding author: Dr. María Morán. Laboratorio de enfermedades raras: mitocondriales y neuromusculares. Instituto de Investigación Hospital Universitario 12 de Octubre (i+12). Centro de Actividades Ambulatorias, 6ª planta. Avenida de Córdoba s/n, 28041, Madrid. Phone: +34 91 779 2784, FAX: +34 91 390 8544, e-mail: mmoran@h12o.es This study was funded by FIS, Fondo de Investigaciones Sanitarias (grant numbers PI14/01085, PI15/00431;PI15/00558), and FEDER funds from the European Union. María Morán is supported by Miguel Servet contracts (CPII16/00023) by the Institute of Health Carlos III (ISCIII) and FEDER funds. Carmen Fiuza-Luces is supported by Sara Borrell contract (CD14/00005) by ISCIII. We acknowledge POWERbreath International Ltd., through BIOCORP EUROPA S.L./POWERbreath Spain for the kind support with equipment for inspiratory muscle training, as well as the blood bank of Hospital 12 de Octubre for facilitating patients' blood drawing. The authors report no conflict of interest and they affirm that the results of the study are presented clearly, honestly, and without fabrication, falsification, or inappropriate data manipulation. The results of the present study do not constitute endorsement by the American College of Sports Medicine. Accepted for Publication: 25 December 2017 © 2018 American College of Sports Medicine
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