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

BACKGROUND: 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. METHODS: 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. RESULTS: 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 < 0.05, for all four exercises). Functional tests showed small reductions in the time needed to climb four steps (2 · 4 sec to 2 · 1, [− 0 · 54 to −0 · 04 ]) and rise to standing position (5 · 8 sec to 4 · 8, [−2 · 0 to 0 · 0]), while time to run, the quick motor function test results and body composition remained unchanged. CONCLUSIONS: Our study shows that a combination of aerobic, strength and core stability exercises is feasible, safe and beneficial to adults with Pompe disease

Ten year follow-up study comparing conservative versus operative treatment of anterior cruciate ligament ruptures. A matched-pair analysis of high level athletes

Objective: To compare long term outcome of highly active patients with anterior cruciate ligament ruptures treated operatively versus non-operatively. Design: We reviewed high level athletes with an anterior cruciate ligament rupture on either MRI or arthroscopic evaluation more than 10 years previously, who were treated conservatively. They were pair-matched with patients who had had an anterior cruciate ligament reconstruction with bone-patella-tendon-bone, with respect to age, gender and Tegner activity score before injury. Participants: In total 50 patients were pair-matched. Results: We found no statistical difference between the patients treated conservatively or operatively with respect to osteoarthritis or meniscal lesions of the knee, as well as activity level, objective and subjective functional outcome. The patients who were treated operatively had a significantly better stability of the knee at examination. Conclusion: We conclude that the instability repair using a bone-patella-tendon-bone anterior cruciate ligament reconstruction is a good knee stabilising operation. Both treatment options however show similar patient outcome at 10 year follow up