囊性纤维化儿童气道管理新进展

New progress of airway management in children with cystic fibrosis

囊性纤维化(CF)是一种多系统受累疾病,主要由囊性纤维化跨膜传导调节因子基因的致病性突变导致。近年来随着对本病认识的加深及基因检测技术的普及,我国CF儿童病例报道逐年增多,肺部受累影响本病的预后。肺部受累与气道密切相关,做好气道管理可延长患儿寿命。现从气道清除技术的选择、吸入药物等方面进行阐述,以期有助于我国CF患儿的长期气道管理。

Cystic fibrosis(CF)is a multisystem disease mainly caused by pathogenic mutation of the cystic fibrosis transmembrane conduction regulator gene.In recent years,with the deepened understanding of the disease and the popularization of gene detection technology,an increasing number of children are diagnosed with CF in China.Lung involvement is reported to affect the prognosis of the disease.Lung involvement is closely related to the airway,and good airway management can prolong the life of children.In this paper,the selection of airway clearance techniques and inhaled drugs were expounded,so as to improve the long-term airway management of children with CF in China.