Impaired oxygen extraction in metabolic myopathies: detection and quantification by near-infrared spectroscopy

Mauro Marzorati, Paola Vago, Bruno Grassi, Francesca Lanfranconi, Alessandra Ferri, Miriam Longaretti, Andrea Stucchi, Claudio Marconi, Lucia Morandi

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review

75 Citazioni (Scopus)

Abstract

Patients with mitochondrial myopathies (MM) or myophosphorylase deficiency (McArdle's disease, McA) show impaired capacity for O(2) extraction, low maximal aerobic power, and reduced exercise tolerance. Non-invasive tools are needed to quantify the metabolic impairment. Six patients with MM, 6 with McA, 25 with symptoms of metabolic myopathy but negative biopsy (patient-controls, P-CTRL) and 20 controls (CTRL) underwent an incremental cycloergometric test. Pulmonary O(2) uptake (VO(2)) and vastus lateralis oxygenation indices (by near-infrared spectroscopy, NIRS) were determined. Concentration changes of deoxygenated hemoglobin and myoglobin (Delta[deoxy(Hb + Mb)]) were considered an index of O(2) extraction. Delta[deoxy(Hb + Mb)] peak (percent limb ischemia) was lower in MM (25.3 +/- 12.0%) and McA (18.7 +/- 7.3) than in P-CTRL (62.4 +/- 3.9) and CTRL (71.3 +/- 3.9) subjects. VO(2) peak and Delta[deoxy(Hb + Mb)] peak were linearly related (r(2) = 0.83). In these patients, NIRS is a tool to detect and quantify non-invasively the metabolic impairment, which may be useful in the follow-up of patients and in the assessment of therapies and interventions.
Lingua originaleEnglish
pagine (da-a)510-520
Numero di pagine11
RivistaMUSCLE & NERVE
Volume35
DOI
Stato di pubblicazionePubblicato - 2007

Keywords

  • Adult
  • Aged
  • Energy Metabolism
  • Exercise
  • Exercise Test
  • Exercise Tolerance
  • Female
  • Glycogen Storage Disease Type V
  • Heart Rate
  • Hemoglobins
  • Humans
  • Male
  • Miopatie metaboliche
  • Mitochondrial Myopathies
  • Muscle, Skeletal
  • Myoglobin
  • Oxidative Phosphorylation
  • Oxygen Consumption
  • Predictive Value of Tests
  • Reference Values
  • Spectroscopy, Near-Infrared
  • consumo d'ossigeno

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