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.

Grassi, B., Marzorati, M., Lanfranconi, F., Ferri, A., Longaretti, M., Stucchi, A., Vago, P., Marconi, C., Morandi, L., Impaired oxygen extraction in metabolic myopathies: detection and quantification by near-infrared spectroscopy, <<MUSCLE & NERVE>>, 2007; 35 (4): 510-520. [doi:10.1002/mus.20708] [http://hdl.handle.net/10807/31392]

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

Marzorati, Mauro;Vago, Paola;
2007

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.
2007
Inglese
Grassi, B., Marzorati, M., Lanfranconi, F., Ferri, A., Longaretti, M., Stucchi, A., Vago, P., Marconi, C., Morandi, L., Impaired oxygen extraction in metabolic myopathies: detection and quantification by near-infrared spectroscopy, <<MUSCLE & NERVE>>, 2007; 35 (4): 510-520. [doi:10.1002/mus.20708] [http://hdl.handle.net/10807/31392]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10807/31392
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