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目的:探讨曲前列尼尔治疗支气管肺发育不良合并肺动脉高压(pulmonary arterial hypertension associated with bronchopulmonary dysplasia,BPD-PH)的疗效。方法:回顾性分析2017年1月至2019年12月静脉输注曲前列尼尔治疗BPD-PH的患儿,收集患儿曲前列尼尔治疗时日龄、最大剂量、治疗持续时间、并发症、临床转归等,比较患儿曲前列尼尔治疗前、治疗1周后、药物达最大剂量后肺动脉收缩压与体循环收缩压之比(systolic pulmonary artery pressure/systolic blood pressure,sPAP/sBP)、氧合指数(oxygenation index,OI)变化,采用多个相关样本的非参数检验对数据进行统计学分析。结果:共纳入6例患儿,男5例,女1例,重度PH 5例,中度PH 1例,胎龄(26.0±1.0)周,出生体重(735±170) g。与用药前相比,曲前列尼尔治疗1周后sPAP/sBP较前下降[1.1(0.8,1.2)比1.3(1.1,1.4)],差异有统计学意义(n P0.05);曲前列尼尔达最大剂量后 sPAP/sBP较前下降明显[0.4(0.3,0.4)比1.1(0.8,1.2)],差异有统计学意义(n P<0.05)。2例发生呕吐,1例腹泻,1例轻度水肿,无低血压、肝肾功能损害等明显药物不良反应。5例患儿存活,1例死于晚发型败血症。n 结论:曲前列尼尔对BPD-PH患儿控制肺动脉压、改善临床预后有一定效果,但仅限于其他治疗效果差的BPD-PH患儿谨慎使用。“,”Objective:To study the effects of treprostinil in the treatment of pulmonary hypertension associated with bronchopulmonary dysplasia (BPD-PH) in preterm infants.Method:From January 2017 to December 2019, a retrospective analysis was conducted on preterm infants with BPD-PH receiving intravenous treprostanil therapy. The timing, dosage and duration of treprostanil, the general information, complications and clinical outcomes of the infants were collected. Ratio of pulmonary artery systolic pressure to systemic systolic pressure (sPAP/sBP) and oxygenation index (OI) of the infants were compared among different timepoints (before treprostinil treatment, one week after the treatment and after reaching the maximum dose). Nonparametric tests were used for statistical analysis.Result:A total of 6 infants were included, 5 males and 1 female, 5 with severe PH and 1 with moderate PH. The gestational age was (26.0±1.0) weeks and the birth weight was (735±170) g. sPAP/sBP was significantly decreased after 1 week and after reaching the maximum dose than before treprostinil treatment [1.1(0.8,1.2),0.4(0.3,0.4)vs. 1.3(1.1,1.4), n P0.05). 2 cases had vomiting, 1 case had diarrhea, 1 case had mild edema and no severe adverse effects such as hypotension, liver and/or kidney dysfunction occurred during the treatment. 5 patients survived and 1 died of late-onset sepsis.n Conclusion:Treprostinil may improve the clinical prognosis and the survival rate of refractory BPD-PH infants and may be considered when all other treatments show poor effects.