Cystic fibrosis: a rare disease emerging in China

作     者：Tengyue Zhang Xinlun Tian Kai-Feng Xu Tengyue Zhang;Xinlun Tian;Kai-Feng Xu

作者机构：Department of Respiratory MedicinePeking Union Medical College HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing 100730China 

出 版 物：《Science China(Life Sciences)》 (中国科学（生命科学英文版）)

年 卷 期：2020年第63卷第7期

页      面：1082-1084页

核心收录：

学科分类：0710[理学-生物学] 1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学] 

主　　题：a rare disease emerging in China Cystic fibrosis 

摘      要：Cystic fibrosis (CF) is an autosomal recessive disease that is caused by mutations in the CF transmembrane conductance regulator (CFTR) protein, an anion channel expressed on the epithelial surface (Rowe et al., 2005). The dysfunction of Cl–anion channels leads to pathophysiological changes such as airway surface liquid (ASL) decrement, delayed mucociliary clearance and defective bacterial killing, resulting in infection, mucus obstruction, inflammation and bronchiectasis(Rowe et al., 2005).