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囊性纤维化中性白细胞介导的呼吸道上皮炎症,导致呼吸道上皮分解酶(中性白细胞弹性蛋白酶)的负担长期过重。α1-抗胰蛋白酶(α1-AT)是正常肺内中性白细胞弹性蛋白酶的主要抑制剂,故作者将其用于治疗囊性纤维化患者.作者对17例肺容量和流率中度降低的囊性纤维化患者和12名正常人进行了探讨.两组均无吸烟史.通过支气管肺泡灌洗(BAL)获得呼吸道上皮衬液(ELF),囊性纤维化组BAL 提示有明显的中性白细胞为主的炎症。5例囊性纤维化患者
Cystic fibrosis Neutrophils mediate respiratory epithelial inflammation, resulting in long-term over-burden of respiratory epithelial lytic enzymes (neutrophil elastase). α1-antitrypsin (α1-AT) is a major inhibitor of normal intrapulmonary neutrophil elastase and is therefore used by authors in the treatment of patients with cystic fibrosis.17 The authors evaluated 17 patients with moderately reduced lung volume and flow rate Cystic fibrosis patients and 12 normal subjects were studied.There was no history of smoking in both groups.Respiratory epithelial lining fluid (ELF) was obtained by bronchoalveolar lavage (BAL). BAL in cystic fibrosis group showed obvious neutrality Leukocyte-based inflammation. 5 cases of cystic fibrosis patients