Metabolic Myopathies: Functional Evaluation by Analysis of Oxygen Uptake Kinetics
Metabolic Myopathies: Functional Evaluation by Analysis of Oxygen Uptake Kinetics
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DOI：
10.1249/mss.0b013e3181aae96b
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发表时间：
2009-12-01
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影响因子：
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通讯作者：
Morandi, Lucia
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文献类型：
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作者：
Grassi, Bruno;Porcelli, Simone;Morandi, Lucia
Marzorati，F.Lanfrconi，P.Vago，C.Marconi和L.Morandi。代谢性肌病：通过摄氧动力学分析进行功能评估。地中海医院。SCI。《体育运动》，第4卷第1期，第12期，第2120-2127页，2009年。目的：本研究的目的是寻找更多的非侵入性检测工具，以检测和量化线粒体肌病(MM)或McArdle病(MCA)患者的代谢损伤。方法：对15例MM、8例MCA、21例有代谢性肌病体征和/或症状但活检阴性的患者(P-CTRL)和22例健康对照(CTRL)在向恒定负荷中等强度功率自行车运动过渡过程中肺摄氧量(V)对Doto(2)动力学的调节动力学进行了测定。结果：(V)高于DOTO(2)MM和MCA较P-CTRL和CTRL慢，MCA较MM慢，P-CTRL和CTRL无显著差异。描述(V)对DOTO(2)动力学的单指数函数的时间常数(Tau)分别为(X)59.2+/-8.5 S(MM)、87.6+/-16.4 S(MCA)、36.9+/-3.1 S(P-CTRL)和35.4+/-1.9 S(CTRL)。在一组患者(8名MM和7名MCA)中，(V)对Doto(2)动力学的tau与先前研究中确定的两个变量(Grassi B、Marzorati M、Lanfrconi F等)呈负相关。代谢性肌病的氧摄取受损：近红外光谱检测和量化。肌肉神经。2007年；35：510-20)：a)通过近红外光谱获得的肌肉氧合指数，估计骨骼肌组分O(2)提取的峰容量；以及b)(V)超过Doto(2)峰。结论：在MM和MCA患者中，中等强度运动中肺(V)对DOTO(2)的动力学分析可以识别和非侵入性地量化骨骼肌氧化代谢的损害。在这些患者中，(V)比Doto(2)更慢的动力学可以被认为是运动耐量受损的标志。目前的数据对于需要对损伤进行客观、定量和纵向评估以用于这些患者的随访以及评估治疗干预措施的临床医生可能是有用的。
GRASSI, B., S. PORCELLI, M. MARZORATI, F. LANFRANCONI, P. VAGO, C. MARCONI, and L. MORANDI. Metabolic Myopathies: Functional Evaluation by Analysis of Oxygen Uptake Kinetics. Med. Sci. Sports Exerc., Vol. 4 1, No. 12, pp. 2120-2127, 2009. Purpose: The aim was to identify additional noninvasive tools allowing to detect and to quantify the metabolic impairment in patients with mitochondrial myopathies (MM) or McArdle's disease (McA). Methods: Kinetics of adjustment of pulmonary oxygen uptake ((V) over dotO(2) kinetics) during transitions to constant-load moderate-intensity cycle ergometer exercise were determined on 15 MM, 8 McA, 21 patients with signs and/or symptoms of metabolic myopathy but a negative biopsy ("patient controls"; P-CTRL), and 22 healthy untrained controls (CTRL). Results: (V) over dotO(2) kinetics were slower in MM and in McA versus P-CTRL and CTRL, slower in McA versus MM, and not significantly different between P-CTRL and CTRL. The time constants (tau) of the monoexponential function describing the (V) over dotO(2) kinetics were ((X) over bar +/- SE) 59.2 +/- 8.5 s in MM, 87.6 +/- 16.4 s in McA, 36.9 +/- 3.1 s in P-CTRL, and 35.4 +/- 1.9 s in CTRL. In a subgroup of the patients (eight MM and seven McA), tau of (V) over dotO(2) kinetics were negatively correlated with two variables determined in a previous study (Grassi B, Marzorati M, Lanfranconi F, et al. Impaired oxygen extraction in metabolic myopathies: detection and quantification by near-infrared spectroscopy. Muscle Nerve. 2007;35:510-20): a) a muscle oxygenation index, obtained by near-infrared spectroscopy, estimating the peak capacity of skeletal muscle fractional O(2) extraction; and b) (V) over dotO(2) peak. Conclusions: In MM and McA patients, analysis of pulmonary (V) over dotO(2) kinetics during moderate-intensity exercise allows to identify and to quantify, noninvasively, the impairment of skeletal muscle oxidative metabolism. In these patients, the slower (V) over dotO(2) kinetics can be considered a marker of the impaired exercise tolerance. The present data could be useful for clinicians who need an objective, quantitative, and longitudinal evaluation of the impairment to be used in the follow-up of these patients as well as in the assessment of therapeutic interventions.