TY -的T1 Elexacaftor / tezacaftor ivacaftor纠正单核细胞杀菌剂的不足在囊性纤维化JF -欧洲呼吸杂志》乔和J - 10.1183/13993003.00725 -2022欧元六世- 61 - 4 SP - 2200725 AU -卢卡Cavinato AU -弗朗西斯科·r·鲁非盟-瓦伦提娜帕斯托雷AU -丹尼尔·Chiappetta盟-格洛丽亚Sangiorgi AU - Eva费拉拉盟Pia Baiocchi AU -朱塞佩Mandarello AU -朱塞佩西米洛盟Paola Del波尔图盟佛罗伦萨Ascenzioni Y1 - 2023/04/01 UR - //www.qdcxjkg.com/content/61/4/2200725.abstract N2 -背景囊性纤维化(CF),这是由突变引起的CF跨膜电导调节器(雌性生殖道的特点是慢性细菌性肺部感染和炎症。在CF,单核细胞和monocyte-derived显示巨噬细胞已被证明有缺陷的吞噬作用和抗菌活性有关肺的病原体,包括铜绿假单胞菌。因此,我们解决的影响雌性生殖道三调制器疗法(elexacaftor / tezacaftor / ivacaftor (ETI))对铜绿假单胞菌对CF单核细胞的活动。方法从CF患者单核细胞(PWCF) 1和6个月的ETI治疗前后血中分离的铜绿假单胞菌感染评估吞噬活性和细胞内的细菌杀死。氧化破裂和白细胞介素- 6分泌也确定。单核细胞从健康对照组也包括在内。结果纵向分析的临床参数确认改善肺功能和肺部微生物指数。CF单核细胞的吞噬和杀菌剂的缺乏也明显改善,尽管不完全。此外,我们测量一个旺盛的氧化破裂在CF单核细胞疗法,大大减少了外星人。这导致了一个进步的活性氧生物杀菌活性。 Inflammatory response to bacterial stimuli was also lowered compared with pre-therapy.Conclusions PWCF on ETI therapy, in a real-life setting, in addition to clinical recovery, showed significant improvement in monocyte activity against P. aeruginosa, which may have contributed to the overall effect of ETI on pulmonary disease. This also suggests that CF monocyte dysfunctions may be specifically targeted to ameliorate lung function in CF.In people with cystic fibrosis, elexacaftor/tezacaftor/ivacaftor ameliorates the antimicrobial activity of monocytes against Pseudomonas aeruginosa by lowering their exuberant oxidative burst, thus contributing to the improvement of lung disease http://bit.ly/3hL2Z11 ER -