囊性纤维化的主要病变在肺部,表现为肺有粘稠的粘液积聚、细菌感染、气管上皮炎症和阻塞性肺部病变,导致呼吸衰竭和死亡。由于上皮炎症以中性白细胞浸润为主,肺的病变可能是由中性白细胞释放弹性蛋白酶对上皮的长期作用所致。该酶不仅损伤正常肺组织,也干扰了机体的防御能力。作 The main lesion of cystic fibrosis in the lungs, manifested as viscous mucus accumulation in the lungs, bacterial infections, tracheal epithelial inflammation and obstructive pulmonary disease, leading to respiratory failure and death. Epithelial inflammation due to neutrophil infiltration, lung disease may be caused by neutrophil release of elastase on the long-term effect on the epithelium. The enzyme not only damages normal lung tissue but also disrupts the body’s defenses. Make