中国实用内科杂志2017，Vol.37Issue(5)：391-394,4.DOI:10.19538/j.nk2017050104
遗传性出血性毛细血管扩张症与肺动脉高压
Hereditary haemorrhagic telangiectasia and pulmonary artery hypertension
王岚 1刘锦铭1
作者信息
- 1. 同济大学附属上海市肺科医院肺循环科,上海200433
- 折叠
摘要
Abstract
Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder,characterised by the presence of vascular malformations.Pulmonary hypertension (PH) is a rare complication of HHT,including PH associated with high-output heart failure and liver vascular malformations and pulmonary arterial hypertension secondary to HHT.In the present review,the authors describe the clinical presentation,diagnosis and management of both PH presentations of HHT,as well as the underlying genetics and pathophysiology.关键词
遗传性出血性毛细血管扩张症/肺高血压/肺动脉高压Key words
hereditary haemorrhagic telangiectasia/pulmonary hypertension/pulmonary artery hypertension分类
医药卫生引用本文复制引用
王岚,刘锦铭..遗传性出血性毛细血管扩张症与肺动脉高压[J].中国实用内科杂志,2017,37(5):391-394,4.
