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

Impaired oxygen extraction in metabolic myopathies: Detection and quantification by near-infrared spectroscopy
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DOI:
10.1002/mus.20708
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发表时间:
2007-04-01
期刊:
影响因子:
3.4
通讯作者:
Morandi, Lucia
Morandi, Lucia
中科院分区:
医学3区
文献类型:
--
作者:
Grassi, Bruno;Marzorati, Mauro;Morandi, Lucia

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线粒体肌病(MM)或肌磷酸酶缺乏症(McArdle病,MCA)患者表现出0,提取能力受损,最大有氧能力降低,运动耐量降低。需要非侵入性的工具来量化代谢损伤。6例MM患者、6例MCA患者、25例代谢性肌病症状但活检阴性的患者(P-CTRL)和20名对照组(CTRL)接受了递增循环功率试验。测定肺摄氧量(VO2)和股外侧肌氧合指数(近红外光谱分析)。脱氧血红蛋白和肌红蛋白(Delta[Deoxy(Hb+Mb)])的浓度变化被认为是02提取的一个指标。MM(25.3±12.0%)和MCA(18.7+/-7.3)组的Delta[Deoxy(Hb+Mb)]峰(肢体缺血百分比)低于P-CTRL组(62.4+/-3.9)和CTRL组(71.3+/-3.9)。VO2峰与Delta[Deoxy(Hb+Mb)]峰呈线性相关(r(2)=0.83)。在这些患者中,NIRS是一种非侵入性检测和量化代谢损伤的工具,这可能对患者的随访以及对治疗和干预的评估有用。
Patients with mitochondrial myopathies (MM) or myophosphorylase deficiency (McArdle's disease, McA) show impaired capacity for 0, 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 (VO2) 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 02 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. VO2 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.