Exertional dyspnea in mitochondrial myopathy: clinical features and physiological mechanisms
Exertional dyspnea in mitochondrial myopathy: clinical features and physiological mechanisms
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DOI：
10.1152/ajpregu.00001.2011
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发表时间：
2011-10-01
影响因子：
2.8
通讯作者：
Haller, Ronald G.
中科院分区：
文献类型：
--
作者：
Heinicke, Katja;Taivassalo, Tanja;Haller, Ronald G.
Heinicke K，泰瓦萨洛T，Wyrick P，Wood H，Babb TG，Haller RG.线粒体肌病的呼吸困难：临床特征和生理机制。Am J Physiol Regul Integr Comp Physiol 301：R873-R884，2011.首次发表于2011年8月3日; doi：10.1152/ajpregu.00001.2011。呼吸困难限制了一些线粒体肌病（MM）患者的运动，但这种综合征的临床特征定义不清，其潜在机制尚不清楚。我们评估了5名患有劳力性呼吸困难和遗传性线粒体缺陷的MM患者和4名对照受试者（C）在周期运动和恢复期间的通气和动脉血气。患者休息时通气正常。在运动过程中，MM患者具有较低的（V）过点（O2峰值）（预测值的28 +/- 9%）和相对于O-2利用的过度全身O-2输送（即，高动力循环）。患者的高感知呼吸努力与过度通气相关，相对于代谢率，高（V）/（E）/（V）/O-2 peak，O-（MM = 104 +/- 18; C = 42 +/- 8，P
Heinicke K, Taivassalo T, Wyrick P, Wood H, Babb TG, Haller RG. Exertional dyspnea in mitochondrial myopathy: clinical features and physiological mechanisms. Am J Physiol Regul Integr Comp Physiol 301: R873-R884, 2011. First published August 3, 2011; doi:10.1152/ajpregu.00001.2011.-Exertional dyspnea limits exercise in some mitochondrial myopathy (MM) patients, but the clinical features of this syndrome are poorly defined, and its underlying mechanism is unknown. We evaluated ventilation and arterial blood gases during cycle exercise and recovery in five MM patients with exertional dyspnea and genetically defined mitochondrial defects, and in four control subjects (C). Patient ventilation was normal at rest. During exercise, MM patients had low (V) over dot (O2peak) (28 +/- 9% of predicted) and exaggerated systemic O-2 delivery relative to O-2 utilization (i.e., a hyperkinetic circulation). High perceived breathing effort in patients was associated with exaggerated ventilation relative to metabolic rate with high (V)over dot (E)/(V) over dot O-2peak,O- (MM = 104 +/- 18; C = 42 +/- 8, P