Porcelli Simone, Marzorati Mauro, Morandi Lucia, Grassi Bruno
Institute of Molecular Bioimaging and Physiology, National Research Council, Segrate, Italy; Department of Medical and Biological Sciences, University of Udine, Udine, Italy
Institute of Molecular Bioimaging and Physiology, National Research Council, Segrate, Italy;
J Appl Physiol (1985). 2016 Sep 1;121(3):699-708. doi: 10.1152/japplphysiol.00885.2015. Epub 2016 Jul 21.
Aerobic training can be effective in patients with mitochondrial myopathies (MM) and McArdle's disease (McA). The aim of the study was to use noninvasive functional evaluation methods, specifically aimed at skeletal muscle oxidative metabolism, to evaluate the effects of an aerobic exercise training (cycle ergometer, 12 wk, 4 days/wk, ∼65-70% of maximal heart rate) in 6 MM and 7 McA. Oxygen uptake and skeletal muscle vastus lateralis fractional O2 extraction by near-infrared spectroscopy were assessed during incremental and low-intensity constant work rate (CWR) exercises before (BEFORE) and at the end (AFTER) of training. Peak O2 uptake increased significantly with training both in MM [14.7 ± 1.2 vs. 17.6 ± 1.4 ml·kg(-1)·min(-1) (mean ± SD)] and in McA (18.5 ± 1.8 ml·kg(-1)·min(-1) vs. 21.6 ± 1.9). Peak skeletal muscle fractional O2 extraction increased with training both in MM (22.0 ± 6.7 vs. 32.6 ± 5.9%) and in McA (18.5 ± 6.2 vs. 37.2 ± 7.2%). During low-intensity CWR in both MM and McA: V̇o2 kinetics became faster in AFTER, but only in the patients with slow V̇o2 kinetics in BEFORE; the transient overshoot in fractional O2 extraction kinetics disappeared. The level of habitual physical activity was not higher 3 mo after training (FOLLOW-UP vs. PRE). In MM and McA patients a home-based aerobic training program significantly attenuated the impairment of skeletal muscle oxidative metabolism and improved variables associated with exercise tolerance. Our findings indicate that in MM and McA patients near-infrared spectroscopy and V̇o2 kinetics can effectively detect the functional improvements obtained by training.
有氧运动训练对线粒体肌病（MM）和麦卡德尔病（McA）患者可能有效。本研究的目的是使用专门针对骨骼肌氧化代谢的非侵入性功能评估方法，来评估有氧运动训练（功率自行车，12周，每周4天，约为最大心率的65 - 70%）对6例MM患者和7例McA患者的影响。在训练前（BEFORE）和训练结束时（AFTER），通过递增运动和低强度恒定工作率（CWR）运动，利用近红外光谱评估摄氧量和骨骼肌股外侧肌的氧提取分数。在MM患者中，训练后峰值摄氧量显著增加[14.7 ± 1.2对17.6 ± 1.4 ml·kg⁻¹·min⁻¹（均值±标准差）]，在McA患者中也是如此（18.5 ± 1.8 ml·kg⁻¹·min⁻¹对21.6 ± 1.9）。在MM患者和McA患者中，训练后骨骼肌峰值氧提取分数均增加（MM患者：22.0 ± 6.7对32.6 ± 5.9%；McA患者：18.5 ± 6.2对37.2 ± 7.2%）。在MM患者和McA患者的低强度CWR运动期间：AFTER组的V̇o₂动力学变快，但仅在BEFORE组中V̇o₂动力学较慢的患者中出现；氧提取分数动力学中的短暂过冲消失。训练后3个月时习惯性身体活动水平并未更高（随访期与训练前相比）。在MM和McA患者中，居家有氧运动训练计划显著减轻了骨骼肌氧化代谢的损害，并改善了与运动耐力相关的变量。我们的研究结果表明，在MM和McA患者中，近红外光谱和V̇o₂动力学能够有效检测到训练所带来的功能改善。
