Sickle cell anemia (SCA) is a genetic hemoglobinopathy associated with an impaired oxygen delivery to skeletal muscle that could alter ATP production processes and increase intramuscular acidosis. These alterations have been already reported in the Townes mouse model of SCA but the corresponding changes in humans have not been documented. In the present study, we used 31-phosphorus magnetic resonance spectroscopy in order to investigate in vivo the metabolic changes induced by a moderate-intensity exercise in twelve SCA patients, eight sickle cell trait (SCT) carriers and twelve controls women. The rest - exercise - recovery protocol disclosed slight differences regarding PCr consumption and lactate accumulation between SCA patients and controls but these differences did not reach a statistical significance. On that basis, the in vivo metabolic changes associated with a moderate-intensity muscle exercise were slightly altered in SCA patients and SCT carriers but within a normal range. The present results strongly support the fact that a moderate-intensity exercise is safe and could be recommended in stable SCA patients and SCT subjects.

中文翻译：

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镰状细胞性贫血或性状女性的体内肌肉功能和能量学：一项³¹ P磁共振波谱研究

镰状细胞性贫血（SCA）是一种遗传性血红蛋白病，与向骨骼肌的氧气输送受损有关，可能会改变ATP的产生过程并增加肌内酸中毒。这些变化已经在SCA的Townes小鼠模型中进行了报道，但尚未记录人类的相应变化。在本研究中，我们使用31磷磁共振波谱技术来研究体内中等强度运动在12位SCA患者，8位镰状细胞性状（SCT）携带者和12位对照女性中引起的代谢变化。休息-锻炼-恢复方案揭示了SCA患者与对照组之间在PCr消耗和乳酸积累方面的细微差别，但这些差异没有统计学意义。在此基础上，在SCA患者和SCT携带者中，与中等强度肌肉运动相关的体内代谢变化略有变化，但在正常范围内。本结果强烈支持以下事实：中等强度的运动是安全的，可以推荐给稳定的SCA患者和SCT受试者。