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

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

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75 Citazioni (Scopus)


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
Stato di pubblicazionePubblicato - 2007
Pubblicato esternamente


  • 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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