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Short-term clinical effect and safety of treprostinil on the treatment of severe pediatric idiopathic pulmonary arterial hypertension
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- Author:
- No author available
- Journal Title:
- China Medicine
- Issue:
- 11
- DOI:
- 10.3760/j.issn.1673-4777.2021.11.009
- Key Word:
- 特发性肺动脉高压;儿童;曲前列尼尔
Abstract： 目的 评价曲前列尼尔治疗重症特发性肺动脉高压(IPAH)患儿的短期临床效果和安全性.方法 收集2018年5月至2020年12月就诊于首都医科大学附属北京安贞医院小儿心脏中心年龄<18周岁的16例短期持续皮下泵入曲前列尼尔治疗的重症IPAH患儿的临床资料进行回顾性分析.收集其病史、临床症状、治疗策略及随访数据,比较随访及基线时的相关临床指标,观察不良反应发生情况.结果 入组患儿年龄为(9±5)岁,纽约心脏病协会(NYHA)心功能分级Ⅲ级11例、Ⅳ级5例,在为期(68±34)d的随访中,患儿心功能明显改善,Ⅱ级15例、Ⅲ级1例.患儿随访时B型脑钠肽、三尖瓣反流最大峰值速度、三尖瓣反流压差、主肺动脉内径与主动脉根部内径比值、右心室前后径与左心室舒张末期内径比值均较基线时明显下降[(135±98)ng/L比(380±353)ng/L、(426±58)cm/s比(467±49)cm/s、(73±19)mmHg(1 mmHg=0.133 kPa)比(88±19)mmHg、(1.32±0.22)比(1.62±0.47)、(0.6±0.3)比(0.9±0.6)],差异均有统计学意义(均P<0.05).起始用药5 d内,2例患儿诉输注部位疼痛,3例诉间断头痛,2例表现为面部潮红,调整用药剂量及对症支持治疗后均能耐受,无患儿因无法耐受而终止治疗.结论 曲前列尼尔治疗重症IPAH患儿安全有效,且耐受性良好.
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