Improved Exercise Tolerance after Enzyme Replacement Therapy in Pompe Disease

• Autores: Mauro Marzorati, Simone Porcelli, Barbara Reggiori, Luca Morandi
• Localización: Medicine & Science in Sports & exercise: Official Journal of the American College of Sports Medicine, ISSN 0195-9131, Vol. 44, Nº. 5, 2012, págs. 771-775
• Idioma: inglés
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• Resumen

  • Purpose: Enzyme replacement therapy (ERT) has recently became available for Pompe disease. Data on the effects of ERT on physiological variables related to exercise tolerance have never been published.

    Methods: Pulmonary gas exchange, cardiac output (by impedance cardiography), and vastus lateralis muscle O2 extraction (by near-infrared spectroscopy) were determined during cycle ergometer exercise in a 50-yr-old patient before and after 1, 12, and 24 months of ERT.

    Results: At the same constant-workload submaximal exercise, RPE, R, pulmonary ventilation, and HR were lower during ERT versus before, suggesting an increased exercise tolerance. Peak oxygen uptake (V?O2peak) increased by ~35% from before (0.64 L·min-1 or 11.4 mL·kg-1·min-1) to 1 month (0.88 L·min-1 or 15.7 mL·kg-1·min-1) of treatment and did not significantly change thereafter. Also, peak cardiac output significantly increased during ERT, whereas peak skeletal muscle fractional O2 extraction was unchanged compared with before.

    Conclusions: Improvements of peak exercise capacity and exercise tolerance at submaximal workloads were observed in a patient with Pompe disease after 1 month of ERT, with no further changes during the ensuing treatment period (up to 24 months).