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口服L-瓜氨酸苹果酸盐治疗特发性肺动脉高压和艾森曼格综合征患者:一项临床试验。

Oral l-citrulline malate in patients with idiopathic pulmonary arterial hypertension and Eisenmenger Syndrome: a clinical trial.

作者信息

Sharif Kashani Babak, Tahmaseb Pour Paritash, Malekmohammad Majid, Behzadnia Neda, Sheybani-Afshar Faezeh, Fakhri Mohammad, Chaibakhsh Samira, Naghashzadeh Farah, Aidenlou Salavan

机构信息

Cardiology Department, Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, PO Box 19575-154, Tehran 19569-444413, Iran.

Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, PO Box 19575-154, Tehran 19569-444413, Iran.

出版信息

J Cardiol. 2014 Sep;64(3):231-5. doi: 10.1016/j.jjcc.2014.01.003. Epub 2014 Feb 10.

Abstract

BACKGROUND AND PURPOSE

Citrulline is an amino acid which is produced by the urea cycle and also a precursor for NO, that is, a vasodilator for normal function of pulmonary vasculature. Thereby, enhancing l-citrulline malate in patients with idiopathic pulmonary arterial hypertension and those with congenital heart disease identified as Eisenmenger Syndrome results in reduction of pulmonary hypertension.

METHODS AND SUBJECTS

In this clinical trial before and after study, we assigned 25 patients with arterial pulmonary hypertension (idiopathic or Eisenmenger Syndrome) to receive l-citrulline malate 1g three times daily for two weeks. The primary measurement was the change in exercise capacity, as considered as a result of the total distance walked in six minutes, from baseline to week 2. We also assessed mean pulmonary artery pressure, the change in the quality of life, and the change in pro-brain natriuretic peptide (BNP) level. The study was not powered to evaluate mortality.

RESULTS

The mean walking distance in six minutes was significantly increased by about 44m (p=0.005) after receiving l-citrulline malate. Mean pulmonary artery pressure significantly reduced from 83.34mmHg before receiving l-citrulline malate to 79.1mmHg after that (p=0.01). All dimensions of the quality of life had statistical differences after receiving l-citrulline malate except limit due to physical health, limit due to emotional health and social functioning (p>0.05). Finally, pro-BNP difference was not statistically significant (p=0.9).

CONCLUSION

l-Citrulline malate improves the distance walk in six minutes and also the quality of life of patients with idiopathic arterial pulmonary hypertension and Eisenmenger Syndrome and also reduced mean arterial pulmonary hypertension.

摘要

背景与目的

瓜氨酸是一种由尿素循环产生的氨基酸,也是一氧化氮的前体,即一种对肺血管系统正常功能起作用的血管扩张剂。因此,在特发性肺动脉高压患者和被诊断为艾森曼格综合征的先天性心脏病患者中增加苹果酸左旋瓜氨酸可降低肺动脉高压。

方法与研究对象

在这项前后对照的临床试验中,我们将25例动脉性肺动脉高压(特发性或艾森曼格综合征)患者分配为接受每日三次、每次1克苹果酸左旋瓜氨酸,持续两周。主要测量指标是运动能力的变化,以从基线到第2周六分钟内行走的总距离来衡量。我们还评估了平均肺动脉压、生活质量的变化以及脑钠肽前体(BNP)水平的变化。该研究没有足够的能力来评估死亡率。

结果

接受苹果酸左旋瓜氨酸后,六分钟平均行走距离显著增加约44米(p = 0.005)。平均肺动脉压从接受苹果酸左旋瓜氨酸前的83.34mmHg显著降至之后的79.1mmHg(p = 0.01)。接受苹果酸左旋瓜氨酸后,除了因身体健康导致的限制、因情绪健康导致的限制和社会功能外,生活质量的所有维度均有统计学差异(p>0.05)。最后,脑钠肽前体的差异无统计学意义(p = 0.9)。

结论

苹果酸左旋瓜氨酸改善了特发性动脉性肺动脉高压和艾森曼格综合征患者的六分钟行走距离以及生活质量,还降低了平均动脉肺动脉压。

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