Safety and efficacy of exercise training in adults with Pompe disease: evalution of endurance, muscle strength and core stability before and after a 12 week training program.

ABSTRACT: Pompe disease is a proximal myopathy. We investigated whether exercise training is a safe and useful adjuvant therapy for adult Pompe patients, receiving enzyme replacement therapy.Training comprised 36 sessions of standardized aerobic, resistance and core stability exercises over 12 weeks. Before and after, the primary outcome measures safety, endurance (aerobic exercise capacity and distance walked on the 6 min walk test) and muscle strength, and secondary outcome measures core stability, muscle function and body composition, were evaluated.Of 25 patients enrolled, 23 successfully completed the training. Improvements in endurance were shown by increases in maximum workload capacity (110 W before to 122 W after training, [95 % CI of the difference 6?·?0 to 19?·?7]), maximal oxygen uptake capacity (69?·?4 % and 75?·?9 % of normal, [2?·?5 to 10?·?4]), and maximum walking distance (6 min walk test: 492 meters and 508, [-4?·?4 to 27?·?7] ). There were increases in muscle strength of the hip flexors (156?·?4 N to 180?·?7 N [1?·?6 to 13?·?6) and shoulder abductors (143?·?1 N to 150?·?7 N [13?·?2 to 35?·?2]). As an important finding in secondary outcome measures the number of patients who were able to perform the core stability exercises rose, as did the core stability balancing time (p?

SUBMITTER: van den Berg LE 

PROVIDER: S-EPMC4506616 | biostudies-literature | 2015

REPOSITORIES: biostudies-literature