中文摘要：

呼吸道上皮的离子和液体的转运功能失调，一直被认为是遗传性疾病囊性纤维化肺病的主要致病因素，但学术界对气道表面液转运缺陷如何引致囊性纤维化肺的感染和受损存在不同的观点。本文从解释气道表面液转运的细胞机制入手，综述了囊性纤维化肺病的病理和遗传特征和近年国际上对囊性纤维化肺病病因的研究进展。

英文摘要：

It has been considered that dysfunction of ions and liquid transport in airway epithelia is a main pathogenic factor of genetic cystic fibrosis lung disease. However, the problem about how airway surface liquid transport makes cystic fibrosis lung inflamed and impaired, has been argued in researches. Starting with explaining the cellular mechanisms of airway surface liquid transport, this article reviews the pathologic and genetic characteristics of cystic fibrosis lung disease and ihe research progress of late years in cystic fibrosis pathogenesis.