长期口服小剂量伊马替尼治疗可遗传性肺动脉高压1例  

A case of heritable pulmonary arterial hypertension treated with long-term oral low-dose imatinib

作　　者：高爱丽 龚娟妮[1] 李积凤[1] 杨媛华[1] Gao Aili;Gong Juanni;Li Jifeng;Yang Yuanhua(Department of Respiratory and Critical Care Medicine,Beijing Institute of Respiratory Medicine and Beijing Chao-Yang Hospital,Capital Medical University,Beijing 100020,China;Department of Respiratory and Critical Care Medicine,Hospital of Shunyi District,Beijing101300,China)

机构地区：[1]首都医科大学附属北京朝阳医院北京呼吸疾病研究所,呼吸与危重症医学科,北京100020 [2]北京市顺义区医院呼吸与危重症医学科,北京101300

出　　处：《中华结核和呼吸杂志》2024年第1期36-38,共3页Chinese Journal of Tuberculosis and Respiratory Diseases

基　　金：北京市研究型病房建设项目(第二批);中国医学科学院医学与健康科技创新工程(2021-I2M-1-049)。

摘　　要：可遗传性肺动脉高压是临床上少见的一种动脉型肺动脉高压,常表现为进行性加重的劳力性呼吸困难,目前尚无特效药物。现报道本院收治的1例可遗传性肺动脉高压患者,该患者存在骨形成蛋白受体2(bone morphogenetic protein 2,BMPR2)基因突变,口服伊马替尼100 mg,1次/d,治疗45个月后,临床症状和血流动力学均明显改善,未见明显不良反应。本文对该例临床特点进行总结并整理相关文献报道,尝试为临床提供治疗肺动脉高压的其他可行方案。Heritable pulmonary arterial hypertension(HPAH)is a rare type of pulmonary arterial hypertension that often presents with progressive exertional dyspnea and for which there is no significant effective drug.A HPAH patient was admitted to our hospital more than three years ago,and the gene mutation was bone morphogenetic protein 2(BMPR2).For the first 45 months,she was given oral imatinib 100 mg once daily,and her symptoms and hemodynamics improved significantly,with no apparent side effects.It is reported that,in combination with the characteristics of the case and related literatures,it provides clinicians with other feasible treatment options for HPAH.

关 键 词：伊马替尼 进行性加重 劳力性呼吸困难 肺动脉高压 基因突变 血流动力学 特效药物 可遗传性 

分 类 号：R544.1[医药卫生—心血管疾病]