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利用 P 磁共振光谱研究镰状细胞贫血或特征的女性体内肌肉功能和能量代谢。

In vivo muscle function and energetics in women with sickle cell anemia or trait: a P-magnetic resonance spectroscopy study.

机构信息

Aix-Marseille Université, CNRS, CRMBM, Marseille, France.

CellMade, Le-Bourget-du-Lac, France.

出版信息

J Appl Physiol (1985). 2021 Mar 1;130(3):737-745. doi: 10.1152/japplphysiol.00790.2020. Epub 2020 Dec 10.

Abstract

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 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 phosphocreatine (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. The main finding of the present study was that the metabolic changes associated with a moderate-intensity muscle exercise were slightly modified in stable sickle cell anemia patients and sickle cell trait carriers as compared to controls but still in the normal range. The present results strongly support the safety of a moderate-intensity exercise for stable sickle cell anemia patients and sickle cell trait carriers.

摘要

镰状细胞贫血症(SCA)是一种遗传性血红蛋白病,与骨骼肌供氧受损有关,这可能会改变 ATP 生成过程并增加肌肉内酸中毒。这些改变已经在 SCA 的 Townes 小鼠模型中得到报道,但在人类中相应的变化尚未记录。在本研究中,我们使用 31 磷磁共振波谱技术在 12 名 SCA 患者、8 名镰状细胞特征(SCT)携带者和 12 名对照女性中,研究了中等强度运动引起的代谢变化。休息-运动-恢复方案揭示了 SCA 患者和对照组之间在磷酸肌酸(PCr)消耗和乳酸积累方面的细微差异,但这些差异没有达到统计学意义。在此基础上,SCA 患者和 SCT 携带者的中等强度肌肉运动相关的体内代谢变化略有改变,但仍在正常范围内。本研究结果有力地证明了中等强度运动是安全的,可以推荐给稳定的 SCA 患者和 SCT 患者。本研究的主要发现是,与对照组相比,稳定的镰状细胞贫血症患者和镰状细胞特征携带者的中等强度肌肉运动相关的代谢变化略有改变,但仍在正常范围内。本研究结果有力地支持了中等强度运动对稳定的镰状细胞贫血症患者和镰状细胞特征携带者的安全性。

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