囊性纤维化的治疗新进展  

Progress on the treatment strategies of cystic fibrosis

作　　者：宋亚亚[1,2] 高宝安[1,2] 

机构地区：[1]三峡大学第一临床学医学院 [2]宜昌市中心人民医院呼吸内科,湖北宜昌443003

出　　处：《海南医学》2015年第17期2572-2575,共4页Hainan Medical Journal

摘　　要：囊性纤维化(CF)是由编码囊性纤维化跨膜传导调节蛋白(CFTR)的基因突变引起的遗传性疾病。CF在我国发病率低,但是具有死亡率极高、中位生存期短的特点。本文介绍了CFRP在CF中的发病机制,并主要对CF治疗的最新进展做一综述。Cystic fibrosis （CF） is autosomal-recessive genetic disease, which is caused by mutations in the cystic fibrosis transmembrane conductance regulator （CFTR）. In China, the population of CF patients is small, but CF is life-limiting and is associated with high mortality. The present review primarily focuses on the mechanisms of CF, and makes a brief summary of new treatment strategies.

关 键 词：囊性纤维化 发病机制 囊性纤维化跨膜传导调节蛋白 治疗 

分 类 号：R394[医药卫生—医学遗传学]