Diminished muscle oxygen uptake and fatigue in spinal muscular atrophy.
Diminished muscle oxygen uptake and fatigue in spinal muscular atrophy.
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DOI：
10.1002/acn3.51353
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发表时间：
2021-05
影响因子：
5.3
通讯作者：
De Vivo DC
中科院分区：
文献类型：
--
作者：
Montes J;Goodwin AM;McDermott MP;Uher D;Hernandez FM;Coutts K;Cocchi J;Hauschildt M;Cornett KM;Rao AK;Monani UR;Ewing Garber C;De Vivo DC
评估脊髓性肌萎缩症 (SMA) 患者和健康对照者运动期间的肌肉摄氧量并量化疲劳程度。通过心肺运动试验 (CPET) 测量 19 名动态 SMA 患者和 16 名健康对照者的峰值有氧能力 (VO2peak) 和工作负荷 (Wpeak)。在 40% Wpeak 下进行次最大运动 (SME) 10 分钟。通过近红外光谱测量股外侧肌脱氧血红蛋白的变化，确定静息时以及 CPET 和 SME 期间的肌肉摄氧量 (ΔHHb)。双能 X 射线吸收法评估去脂体重 (FFM%)。疲劳程度分别通过 SME (FatigueSME) 和六分钟步行测试 (Fatigue6MWT) 第一分钟与最后一分钟相比的工作量或距离变化百分比来确定。与对照组相比，SMA 中的 ΔHHb-PEAK、ΔHHb-SME、VO2peak、Wpeak、FFM% 和 6MWT 距离较低 (P < 0.001)，而 Fatigue6MWT 和 FatigueSME 较高 (P < 0.001)。仅在对照组中，ΔHHb-PEAK 与 FFM% (r = 0.50) 和 VO2peak (r = 0.41) 相关。仅在 SMA 中，Fatigue6MWT 与 Wpeak (r = -0.69) 呈负相关，FatigueSME 与 FFM% (r = -0.55) 和 VO2peak (r = -0.69) 呈负相关。这项研究为 SMA 患者的肌肉线粒体功能障碍提供了进一步的支持。在运动过程中，我们观察到肌肉摄氧量减少，但与有氧能力或身体成分没有相关性。我们还观察到疲劳增加与有氧能力、工作量和身体成分下降相关。了解 SMA 运动期间肌肉摄氧量减少和疲劳增加的机制可能会确定其他治疗目标，以挽救有症状的患者并减轻他们的残余疾病负担。
To estimate muscle oxygen uptake and quantify fatigue during exercise in ambulatory individuals with spinal muscular atrophy (SMA) and healthy controls. Peak aerobic capacity (VO2peak) and workload (Wpeak) were measured by cardiopulmonary exercise test (CPET) in 19 ambulatory SMA patients and 16 healthy controls. Submaximal exercise (SME) at 40% Wpeak was performed for 10 minutes. Change in vastus lateralis deoxygenated hemoglobin, measured by near‐infrared spectroscopy, determined muscle oxygen uptake (ΔHHb) at rest and during CPET and SME. Dual energy X‐ray absorptiometry assessed fat‐free mass (FFM%). Fatigue was determined by percent change in workload or distance in the first compared to the last minute of SME (FatigueSME) and six‐minute walk test (Fatigue6MWT), respectively. ΔHHb‐PEAK, ΔHHb‐SME, VO2peak, Wpeak, FFM%, and 6MWT distance were lower (P < 0.001), and Fatigue6MWT and FatigueSME were higher (P < 0.001) in SMA compared to controls. ΔHHb‐PEAK correlated with FFM% (r = 0.50) and VO2peak (r = 0.41) only in controls. Only in SMA, Fatigue6MWT was inversely correlated with Wpeak (r = −0.69), and FatigueSME was inversely correlated with FFM% (r = −0.55) and VO2peak (r = −0.69). This study provides further support for muscle mitochondrial dysfunction in SMA patients. During exercise, we observed diminished muscle oxygen uptake but no correlation with aerobic capacity or body composition. We also observed increased fatigue which correlated with decreased aerobic capacity, workload, and body composition. Understanding the mechanisms underlying diminished muscle oxygen uptake and increased fatigue during exercise in SMA may identify additional therapeutic targets that rescue symptomatic patients and mitigate their residual disease burden.
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