恶性高热易感患者的骨骼肌代谢紊乱
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Skeletal Muscle Metabolic Dysfunction in Patients With Malignant Hyperthermia Susceptibility.
背景与目的
恶性高热（MH），是一种遗传性骨骼肌药理学障碍，病人对某些麻醉剂呈潜在的致命性高代谢反应。然而，一些MH易感患者在没有麻醉剂的情况下会出现肌肉无力、疲劳和不耐受运动。这项研究的目的是阐明在咖啡因氟烷挛缩试验检测MH阳性的患者不耐受运动的病理生理学。为此，我们使用了磷磁共振波谱、血氧水平依赖性功能磁共振成像（MRI）和传统的运动测试来比较MH阳性患者和健康对照者的骨骼肌代谢情况。
方 法
用磁共振波谱和血氧水平依赖性功能磁共振成像评估29例MH阳性患者和20例健康对照者的骨骼肌代谢情况。用传统的体能测量方法测量有氧代谢能力、无氧代谢能力和肌力。
结 果
在30和60秒的运动中，与健康对照组相比，MH阳性患者氧化途径ATP的产生量显著降低。与健康对照组相比，MH阳性患者的血氧水平依赖性功能磁共振成像恢复时间更长。运动试验显示MH阳性患者的有氧和无氧代谢能力低于健康对照组患者。
结 论
这项探索性研究的结果表明，与健康人相比，MH阳性患者的有氧代谢受影响。这可以对MH易感患者不耐受运动做出解释。
原始文献摘要
hompson SJ，Riazi S; Skeletal Muscle Metabolic Dysfunction in Patients With Malignant Hyperthermia Susceptibility; Anesth Analg. Aug 2017; 125(2): 434-441.
BACKGROUND:Malignant hyperthermia (MH), a pharmacogenetic disorder of skeletal muscle, presents with a potentially lethal hypermetabolic reaction to certain anesthetics. However, some MH-susceptible patients experience muscle weakness, fatigue, and exercise intolerance in the absence of anesthetic triggers. The objective of this exploratory study was to elucidate the pathophysiology of exercise intolerance in patients tested positive for MH with the caffeine-halothane contracture test. To this end, we used phosphorus magnetic resonance spectroscopy, blood oxygen level-dependent functional magnetic resonance imaging (MRI), and traditional exercise testing to compare skeletal muscle metabolism in MH-positive patients and healthy controls.
METHODS:Skeletal muscle metabolism was assessed using phosphorus magnetic resonance spectroscopy and blood oxygen level-dependent functional MRI in 29 MH-positive patients and 20 healthy controls. Traditional measures of physical capacity were employed to measure aerobic capacity, anaerobic capacity, and muscle strength.
RESULTS:During 30- and 60-second exercise, MH-positive patients had significantly lower ATP production via the oxidative pathway compared to healthy controls. MH-positive patients also had a longer recovery time with blood oxygen level-dependent functional MRI compared to healthy controls. Exercise testing revealed lower aerobic and anaerobic capacity in MH-positive patients compared to healthy controls.
CONCLUSIONS:Results of this exploratory study suggest that MH-positive patients have impaired aerobic metabolism compared to healthy individuals. This could explain the exercise intolerance exhibited in MH-susceptible patient population.
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