Exercise testing in late-onset glycogen storage disease type II patients undergoing enzyme replacement therapy. (Articolo in rivista)

Type

• Articolo in rivista (Classe)
• Prodotto della ricerca (Classe)

Label

• Exercise testing in late-onset glycogen storage disease type II patients undergoing enzyme replacement therapy. (Articolo in rivista) (literal)

Anno

• 2012-01-01T00:00:00+01:00 (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi

• 10.1016/j.nmd.2012.10.017 (literal)

Alternative label

• Mauro Marzorati; Simone Porcelli; Giuseppe Bellistri; Lucia Morandi; Bruno Grassi. (2012)
  Exercise testing in late-onset glycogen storage disease type II patients undergoing enzyme replacement therapy.
  in Neuromuscular disorders
  (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori

• Mauro Marzorati; Simone Porcelli; Giuseppe Bellistri; Lucia Morandi; Bruno Grassi. (literal)

Pagina inizio

• S230 (literal)

Pagina fine

• S234 (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume

• 22 (literal)

Rivista

• Neuromuscular disorders (Rivista)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni

• Institute of Bioimaging and Molecular Physiology, CNR, Segrate Milano, Italy. Department of Medical and Biological Sciences, University of Udine, Udine, Italy Neuromuscular Diseases and Neuroimmunology Unit, Fondazione IRCCS Istituto Neurologico \"Carlo Besta\", Milano, Italy (literal)

Titolo

• Exercise testing in late-onset glycogen storage disease type II patients undergoing enzyme replacement therapy. (literal)

Abstract

• Enzyme replacement therapy (ERT) has recently became available for patients with glycogen storage disease type II. Previous studies have demonstrated clinical efficacy of enzyme replacement therapy, however, data on physiological variables related to exercise tolerance are scarce. Four glycogen storage disease type II late-onset patients (45 ± 6 years) performed an incremental exercise on a cycle ergometer, up to voluntary exhaustion, before (BEFORE) and after 12 months of ERT (AFTER). Peak workload, oxygen uptake, heart rate, cardiac output (by impedance cardiography) and vastus lateralis oxygenation indices (by continuous-wave near-infrared spectroscopy, NIRS) were determined. Peak workload and oxygen uptake values significantly increased during ERT (54 ± 30 vs. 63 ± 31 watt, and 17.2 ± 4.4 vs. 19.7 ± 3.5 ml/kg/min, respectively, in BEFORE vs. AFTER). On the other hand, for both peak cardiac output (12.3 ± 5.3 vs. 14.8 ± 4.5 L/min) and the NIRS-determined peak skeletal muscle fractional O2 extraction, expressed as a percentage of the maximal values during a transient limb ischemia (30 ± 39% vs. 38 ± 28%), the observed increases were not statistically significant. Our findings suggest that in glycogen storage disease type II patients enzyme replacement therapy is associated with a mild improvement of exercise tolerance. The findings need to be validated during a longer follow-up on a larger group of patients. (literal)

Prodotto di

• Fattori cardiovascolari e muscolari limitanti l'esercizio: modelli clinici (ME.P01.002.008) (Modulo)
• Institute of molecular bioimaging and physiology (IBFM) (Istituto)

Autore CNR

• MAURO MARZORATI (Unità di personale interno)
• SIMONE PORCELLI (Unità di personale esterno)

Insieme di parole chiave

• Parole chiave di "Exercise testing in late-onset glycogen storage disease type II patients undergoing enzyme replacement therapy." (Insieme di parole chiave)

Incoming links:

Autore CNR di

• MAURO MARZORATI (Unità di personale interno)
• SIMONE PORCELLI (Unità di personale esterno)

Prodotto

• Institute of molecular bioimaging and physiology (IBFM) (Istituto)
• Fattori cardiovascolari e muscolari limitanti l'esercizio: modelli clinici (ME.P01.002.008) (Modulo)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#rivistaDi

• Neuromuscular disorders (Rivista)

Insieme di parole chiave di

• Parole chiave di "Exercise testing in late-onset glycogen storage disease type II patients undergoing enzyme replacement therapy." (Insieme di parole chiave)