Smith A
Department of Molecular Microbiology and Immunology, School of Medicine, University of Missouri, Columbia, USA.
Pediatr Infect Dis J. 1997 Jan;16(1):91-5; discussion 95-6, 123-6. doi: 10.1097/00006454-199701000-00030.
To describe the current understanding of the pathogenesis of infections in cystic fibrosis (CF).
The key element in the pathogenesis of infections in CF is the abnormal mucin resulting from a defective chloride channel. Abnormal mucin and the lack of hydration of respiratory secretions entraps bacteria, permitting colonization and subsequent infection. "Normal" physiologic insults, such as microaspiration, and pollution evoke mucin secretion with bacteria-mucin aggregates causing infection of small airways. Microcolonies of pulmotropic bacteria (i.e. Haemophilus and Staphylococcus) in the trapped mucin cause mucosal injury which predisposes the patient to Pseudomonas aeruginosa infection. Eventually there is obstruction of medium-sized and small airways by inflammatory exudate and mucus. Ultimately these chronic endobronchial bacterial infections cause significant loss of pulmonary function with morbidity and a decrease in the life span of patients with CF. Although antibiotic therapy is beneficial for the management of bacterial respiratory tract infections in CF patients, gene therapy may provide the ultimate cure.
New treatment strategies will emerge as the pathogenesis of cystic fibrosis is better elucidated.
描述目前对囊性纤维化（CF）感染发病机制的认识。
CF感染发病机制的关键因素是由有缺陷的氯离子通道导致的异常粘蛋白。异常粘蛋白以及呼吸道分泌物缺乏水合作用会困住细菌，使其得以定植并随后引发感染。“正常”的生理刺激，如微量误吸和污染，会引发粘蛋白分泌，细菌 - 粘蛋白聚集体导致小气道感染。被困粘蛋白中的嗜肺细菌（即嗜血杆菌和葡萄球菌）微菌落会造成黏膜损伤，使患者易患铜绿假单胞菌感染。最终，炎症渗出物和黏液会阻塞中小气道。这些慢性支气管内细菌感染最终会导致肺功能严重丧失，患者发病，并缩短CF患者的寿命。尽管抗生素治疗对CF患者细菌性呼吸道感染的管理有益，但基因治疗可能提供最终的治愈方法。
随着囊性纤维化发病机制得到更好的阐明，将会出现新的治疗策略。
