Medicine and science in sports and exercise · 2012
Improved exercise tolerance after enzyme replacement therapy in Pompe disease.
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Marzorati Mauro, Porcelli Simone, Reggiori Barbara, Morandi Lucia, Grassi Bruno
Аннотация
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·min⁻¹) to 1 month (0.88 L·min⁻¹ or 15.7 mL·kg·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).
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·min⁻¹) to 1 month (0.88 L·min⁻¹ or 15.7 mL·kg·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).