Döring G
Dept of General and Environmental Hygiene, University of Tubingen, Germany.
Monaldi Arch Chest Dis. 1997 Aug;52(4):363-6.
In cystic fibrosis, impaired mucociliary clearance leads to chronic endobronchial bacterial infection, mostly caused by Pseudomonas aeruginosa. In the early stage of infection, the pathogen produces several extracellular protein toxins which may contribute to its multifactorial virulence before specific antibodies are produced. P. aeruginosa successfully resists phagocytosis by neutrophils, which dominate the local inflammatory response, by switching from a nonmucoid variant to a mucoid phenotype. Chronic infection and inflammation are characterized by neutrophil-released proteinases which may provide favourable growth conditions for the bacterial opportunist. Aerosol therapy with serine proteinase inhibitors is being investigated in cystic fibrosis.
在囊性纤维化中，黏液纤毛清除功能受损会导致慢性支气管内细菌感染，主要由铜绿假单胞菌引起。在感染早期，病原体产生几种细胞外蛋白毒素，在特异性抗体产生之前，这些毒素可能导致其多因素致病性。铜绿假单胞菌通过从非黏液样变体转变为黏液样表型，成功抵抗了在局部炎症反应中占主导地位的中性粒细胞的吞噬作用。慢性感染和炎症的特征是中性粒细胞释放蛋白酶，这可能为机会性细菌提供有利的生长条件。目前正在对囊性纤维化患者进行丝氨酸蛋白酶抑制剂雾化治疗的研究。
