CFTR modulators effect | References |
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Anti-inflammatory and significant reduction inflammatory mediators | Hisert KB et al. Restoring cystic fibrosis transmembrane conductance regulator function reduces airway bacteria and inflammation in people with cystic fibrosis and chronic lung infections. Am J Respir Crit CareMed. 2017; 195(12): 1617- 1628. https://doi.org/10.1164/rccm.201609-1954OC [61] |
Improve airways mucus clearance | Altes TA et al. Use of hyperpolarized helium-3 MRI to assess response to ivacaftor treatment in patients with cystic fibrosis. J Cyst Fibros. 2017; 16(2): 267- 274. https://doi.org/10.1016/j.jcf.2016.12.004 [62] |
Inhibition in vitro SARS-CoV-2 replication | Lotti V et al. CFTR Modulation Reduces SARS-CoV-2 Infection in Human Bronchial Epithelial Cells. Cells. 2022;11:1347. https://doi.org/10.3390/cells11081347 [63] |
Significantly improved ppFEV1 | Quittner AL et al. Development and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosis. Chest 2005 Oct;128(4):2347–54. https://doi.org/10.1378/chest.128.4.2347 [46] |
Significant improvements in respiratory function | Wainwright CE et al. Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N. Engl. J. Med. 373:220–31. https://doi.org/10.1056/NEJMoa1409547 [47] |