遗传性出血性毛细血管扩张症的药物治疗进展  

Advances in drug therapy of hereditary hemorrhagic telangiectasia

作　　者：赵琳[1] 周宪梁[1] Zhao Lin;Zhou Xianliang(Chinese Academy of Medical Sciences and Peking Union Medical College,National Center for Cardiovascular Disease,Department of Cardiology,Fuwai Hospital,Beijing 100037,China)

机构地区：[1]中国医学科学院/北京协和医学院/国家心血管病中心阜外医院心内科,北京100037

出　　处：《疑难病杂志》2022年第10期1101-1104,共4页Chinese Journal of Difficult and Complicated Cases

基　　金：国家重点研发计划专项(2016YFC1300100);临床与转化医学研究基金项目(2019XK320057)。

摘　　要：遗传性出血性毛细血管扩张症(HHT)是一种常染色体显性遗传的血管病。典型的临床表现是存在血管畸形,最常见的临床表现是鼻出血、消化道出血、缺铁性贫血及特征性的皮肤黏膜毛细血管扩张。HHT的诊断主要依靠血管畸形的证据及基因检测。现阶段的治疗目标主要在于缓解症状。近年来关于HHT的药物治疗取得了一定进展,可考虑使用的药物包括抗血管生成药物、免疫调节剂、抗纤溶药物、激素、β受体阻滞剂等,为HHT患者的治疗提供了更多的选择。Hereditary hemorrhagic telangiectasia(HHT) is an autosomal dominant vascular disease.The typical clinical manifestation is the presence of vascular malformation.The most common clinical manifestations are epistaxis,gastrointestinal bleeding,iron deficiency anemia and characteristic telangiectasia of skin and mucosa.The diagnosis of HHT mainly depends on the evidence of vascular malformation and gene detection.The current treatment goal is mainly to alleviate symptoms.In recent years,the drug treatment of HHT has made some progress.The drugs that can be considered include anti angiogenic drugs,immunomodulators,anti-fibrinolytic drugs,hormones β Receptor blockers provide more options for the treatment of HHT patients.

关 键 词：遗传性出血性毛细血管扩张症 毛细血管扩张 药物治疗 

分 类 号：R543.7[医药卫生—心血管疾病]