Improved exercise tolerance after enzyme replacement therapy in Pompe’s disease

Mauro Marzorati

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review

8 Citazioni (Scopus)

Abstract

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. 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. 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 or 11.4 mL/kg/min) to 1 month (0.88 L/min or 15.7 mL/kg/min) 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. 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).
Lingua originaleEnglish
pagine (da-a)771-775
Numero di pagine5
RivistaMedicine and Science in Sports and Exercise
Volume44
DOI
Stato di pubblicazionePubblicato - 2012
Pubblicato esternamente

Keywords

  • Pompe disease
  • esercizio
  • exercise
  • malattia di Pompe

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