Improved Exercise Tolerance after Enzyme Replacement Therapy in Pompe's Disease (Articolo in rivista)

Type
Label
  • Improved Exercise Tolerance after Enzyme Replacement Therapy in Pompe's Disease (Articolo in rivista) (literal)
Anno
  • 2012-01-01T00:00:00+01:00 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi
  • 10.1249/MSS.0b013e31823e6579 (literal)
Alternative label
  • Mauro Marzorati; Simone Porcelli; Barbara Reggiori; Lucia Morandi; Bruno Grassi B. (2012)
    Improved Exercise Tolerance after Enzyme Replacement Therapy in Pompe's Disease
    in Medicine and science in sports and exercise
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • Mauro Marzorati; Simone Porcelli; Barbara Reggiori; Lucia Morandi; Bruno Grassi B. (literal)
Pagina inizio
  • 771 (literal)
Pagina fine
  • 775 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#url
  • http://journals.lww.com/acsm-msse/pages/articleviewer.aspx?year=2012&issue=05000&article=00001&type=abstract (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
  • 44 (literal)
Rivista
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#note
  • Pubblicato on-line: 25 Ottobre 2011 Corresponding author: M. Marzorati (mauro.marzorati@ibfm.cnr.it) IF 2010: 4.106 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroFascicolo
  • 5 (literal)
Note
  • ISI Web of Science (WOS) (literal)
  • PubMe (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
  • IBFM-CNR, Segrate (MI). Università Telematica San Raffaele Roma. Movement Disorders Unit, Fondazione IRCCS Istituto Neurologico \"Carlo Besta\", Milano. Neuromuscular Diseases and Neuroimmunology Unit, Fondazione IRCCS Istituto Neurologico \"Carlo Besta\", Milano. Department of Medical and Biological Sciences, University of Udine, Udine. (literal)
Titolo
  • Improved Exercise Tolerance after Enzyme Replacement Therapy in Pompe's Disease (literal)
Abstract
  • PURPOSE: Enzyme replacement therapy (ERT) has recently became available for Pompe's 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), vastus lateralis muscle O2 extraction (by near-infrared spectroscopy) were determined during cycle ergometer exercise in a 50-year-old patient BEFORE and after 1, 12 and 24 months of ERT. RESULTS: At the same constant-workload submaximal exercise, rates of self-perceived exertion and values of respiratory exchange ratio, pulmonary ventilation and heart rate were lower during ERT vs.. BEFORE, suggesting an increased exercise tolerance. Peak oxygen uptake (VÙO2peak) 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 to BEFORE. CONCLUSIONS: Improvements of peak exercise capacity and exercise tolerance at submaximal workloads were observed in a patient with Pompe's disease after 1 month of ERT, with no further changes during the ensuing treatment period (up to 24 months). (literal)
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