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L-Glutamine in sickle cell disease.

L-谷氨酰胺在镰状细胞病中的应用.

  • 影响因子:1.08
  • DOI:10.1358/dot.2020.56.4.3110575
  • 作者列表:"Cox SE","Hart E","Kirkham FJ","Stotesbury H
  • 发表时间:2020-04-01
Abstract

:L-Glutamine is a conditionally essential amino acid required for synthesis of the pyridines for nucleotides, including nicotinamide adenine dinucleotide (NAD) and glutathione, as well as glutamate, and becomes essential during oxidative stress exposure. The NADH:[NAD⁺ + NADH] (redox) ratio in sickle red blood cells (RBCs) is lower than in normal RBCs, consistent with oxidative stress, therefore glutamine availability is important in sickle cell disease (SCD). RBC glutamine levels vary between SCD studies but the ratio glutamine:glutamate was inversely related to tricuspid regurgitant jet velocity in one. Oral L-glutamine was associated with an increase in NADH and reduction in RBC endothelium adhesion in small studies of SCD patients. In a sickle mouse model, glutamine levels were directly related to cerebral blood flow. Phase II and III randomized, double-blind, controlled trials of L-glutamine 0.6 g/kg/day compared with placebo in children and adults with SCD and = 2 episodes of pain in the previous year provide evidence that L-glutamine is safe and associated with a reduction in painful episodes and in hospitalizations. However, L-glutamine was only tolerated in two-thirds of patients, anemia and hemolysis did not improve and there are few data on mortality and organ complications. Future studies should investigate the effect of other amino acids and total protein intake.

摘要

L-谷氨酰胺是合成核苷酸的吡啶 (包括烟酰胺腺嘌呤二核苷酸 (NAD) 和谷胱甘肽) 以及谷氨酸所需的条件必需氨基酸,并且在氧化应激暴露期间变得必需。镰状红细胞 (rbc) 中的NADH:[NAD⁺ + NADH] (氧化还原) 比率低于正常rbc,与氧化应激一致,因此谷氨酰胺可用性在镰状细胞病 (SCD) 中很重要。RBC谷氨酰胺水平在SCD研究之间变化,但谷氨酰胺: 谷氨酸的比率与三尖瓣反流射流速度呈负相关。在SCD患者的小型研究中,口服L-谷氨酰胺与NADH的增加和RBC内皮粘附的减少相关。在镰状小鼠模型中,谷氨酰胺水平与脑血流量直接相关。II期和III期随机,双盲,L-谷氨酰胺0.6g/kg/天与安慰剂相比,在儿童和成人SCD患者中进行的对照试验提供了证据,证明L-谷氨酰胺是安全的,并且与疼痛发作和住院治疗的减少相关。然而,L-谷氨酰胺仅在3分之2的患者中耐受,贫血和溶血没有改善,并且关于死亡率和器官并发症的数据很少。未来的研究应该调查其他氨基酸和总蛋白摄入量的影响。

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影响因子:1.74
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翻译标题与摘要 下载文献
血红蛋白病方向

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