波生坦治疗β地中海贫血合并多普勒定义的肺动脉高压患者后肺动脉压的改善情况。
The improvement of pulmonary artery pressure after bosentan therapy in patients with β-thalassemia and Doppler-defined pulmonary arterial hypertension.
作者信息
Karami Hossein, Darvishi-Khezri Hadi, Kosaryan Mehrnoush, Akbarzadeh Rosetta, Dabirian Mojdeh
机构信息
Department of Pediatrics, Thalassemia Research Center (TRC), Hemoglobinopathy Institute, Mazandaran University of Medical Sciences, Sari, Iran.
Student Research Committee, Thalassemia Research Center (TRC), Hemoglobinopathy Institute, Mazandaran University of Medical Sciences, Sari, Iran.
出版信息
Int Med Case Rep J. 2018 Dec 17;12:1-7. doi: 10.2147/IMCRJ.S180602. eCollection 2019.
INTRODUCTION
Pulmonary arterial hypertension (PAH) is relatively prevalent in patients with thalassemia. PAH treatment is necessary as the prevalence of Doppler-estimated PAH and the resultant mortality is high in such patients.
MATERIALS AND METHODS
This study aimed at evaluating the effect of bosentan therapy on patients with thalassemia suspected of PAH. Based on pulsed Doppler echocardiography, all the cases were suspected of severe PAH. Consequently, bosentan was initiated at a dose of 62.5 mg twice a day for 4 weeks, which was increased to 62.5-125 mg twice a day, if no adverse side effects were observed.
RESULTS
The results of this study showed that pulmonary artery pressure (PAP) decreased after the administration of bosentan in three cases, from 160 to 120, 110 to 65, and 60 to 25 mmHg; in other words, the PAP reduced in the mentioned cases by 25%, 36.4%, and 58.4%, respectively.
CONCLUSION
In this study, PAP improved after bosentan therapy in patients with β-thalassemia suspected of PAH; however, further studies are required to confirm the findings.
引言
肺动脉高压（PAH）在 thalassemia 患者中相对普遍。由于此类患者中多普勒估计的 PAH 患病率及由此导致的死亡率较高，因此 PAH 治疗是必要的。
材料与方法
本研究旨在评估波生坦治疗疑似 PAH 的 thalassemia 患者的效果。基于脉冲多普勒超声心动图，所有病例均疑似重度 PAH。因此，波生坦以每日两次 62.5 毫克的剂量起始服用 4 周，若未观察到不良副作用，则剂量增加至每日两次 62.5 - 125 毫克。
结果
本研究结果显示，3 例患者在服用波生坦后肺动脉压（PAP）下降，分别从 160 降至 120、110 降至 65、60 降至 25 mmHg；换言之，上述病例的 PAP 分别降低了 25%、36.4%和 58.4%。
结论
在本研究中，疑似 PAH 的β - thalassemia 患者在接受波生坦治疗后 PAP 有所改善；然而，需要进一步研究以证实这些发现。
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