Double-Blind Clinical Trial of Arginine Supplementation in the Treatment of Adult Patients with Sickle Cell Anaemia.
Double-Blind Clinical Trial of Arginine Supplementation in the Treatment of Adult Patients with Sickle Cell Anaemia.
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DOI：
10.1155/2019/4397150
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发表时间：
2019-01-01
影响因子：
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通讯作者：
Lemes, Romelia P G
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文献类型：
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作者：
Eleuterio, Renata M N;Nascimento, Francisco O;Lemes, Romelia P G
背景：镰状细胞贫血（SCA）是巴西最常见的单基因疾病。在SCA中，形成血红蛋白S（HbS），其改变红细胞形态。发生血管内溶血，其中游离Hb和自由基降解一氧化氮（NO）并释放精氨酸酶，从而降低精氨酸水平。因为精氨酸是NO形成的底物，这种减少导致NO（血管扩张剂）合成减少。SCA治疗使用的是羟基脲（HU），以保持高胎儿血红蛋白（HbF）水平，并减少HbS，以避免haemolytic episodes.OBJECTIVE：分析的疗效L-精氨酸作为辅助治疗SCA patients.SETTING：国家血液中心的塞阿拉，Brazil.METHODS：这是一个随机双盲的临床研究，成人SCA连续使用HU在国家血液中心的塞阿拉。临床研究招募了25名接受HU + L-精氨酸（500 mg）的患者和25名接受HU +安慰剂的患者。治疗进行了四个多月。进行实验室检查以确定以下各项的水平：（1）全血细胞计数;（2）亚硝酸盐+硝酸盐;（3）HbF;和（4）网织红细胞。临床实验由血液学家进行。主要结果的措施是亚硝酸盐和pain.RESULTS：统计分析表明，NO的水平增加的研究组，也有减少疼痛频率使用疼痛频率量表的天，周，月。在接受安慰剂加HU的组中，亚硝酸盐和硝酸盐的水平在评估的时间内没有变化（38.27 ± 17.27 mg/L，39.49 ± 12.84 mg/L，34.45 ± 11.25 mg/L，p >0.05），但在补充L-精氨酸加HU的患者中，在M0和M4之间观察到亚硝酸盐和硝酸盐水平显著增加（36.55 ± 20.23 mg/L vs 48.64 ± 20.63 mg/L，p =0.001）以及M2和M4（35.71 ± 15.11 mg/L vs 48.64 ± 20.63 mg/L，p
BACKGROUND: Sickle cell anaemia (SCA) is the most prevalent monogenic disease in Brazil. In SCA, haemoglobin S (HbS) is formed, which modifies red blood cell morphology. Intravascular haemolysis occurs, in which free Hb and free radicals degrade nitric oxide (NO) and release arginase, which reduces arginine levels. Because arginine is a substrate for NO formation, this decrease leads to reduced NO (vasodilator) synthesis. SCA treatment uses hydroxyurea (HU) to maintain high foetal haemoglobin (HbF) levels and reduces HbS to avoid haemolytic episodes.OBJECTIVE: To analyse the efficacy of L-arginine as an adjuvant in the treatment of SCA patients.SETTING: The State Blood Centre of Ceara, Brazil.METHODS: This was a randomized double-blind clinical study of adults with SCA with continuous use of HU at the State Blood Centre of Ceara. The clinical study enrolled 25 patients receiving HU + L-arginine (500 mg) and 25 patients receiving HU + placebo. The treatment was carried out over four months. Laboratory tests were performed to determine the levels of the following: (1) complete blood count; (2) nitrite + nitrate; (3) HbF; and (4) reticulocytes. The clinical experiments were performed by a haematologist. The main outcome measures were nitrite and pain.RESULTS: Statistical analysis showed that the levels of NO were increased in the study group, and there was also a reduction in pain frequency using a pain frequency scale by day, week, and month. The levels of nitrite plus nitrate in the group receiving placebo plus HU did not change among the times evaluated (38.27 ± 17.27 mg/L, 39.49 ± 12.84 mg/L, 34.45 ± 11.25 mg/L, p >0.05), but in the patients who received supplementation with L-arginine plus HU, a significant increase in nitrite plus nitrate levels was observed between M0 and M4 (36.55 ± 20.23 mg/L versus 48.64 ± 20.63 mg/L, p =0.001) and M2 and M4 (35.71 ± 15.11 mg/L versus 48.64 ± 20.63 mg/L, p