Elexacaftor/Tezacaftor/Ivacaftor in Children with Cystic Fibrosis: No Longer "If" but a Question of "When" to Start
- PMID: 40680160
- PMCID: PMC12555054
- DOI: 10.1164/rccm.202505-1146ED
Elexacaftor/Tezacaftor/Ivacaftor in Children with Cystic Fibrosis: No Longer "If" but a Question of "When" to Start
Comment on
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Long-Term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Children ≥6 Years with Cystic Fibrosis and at Least One F508del Allele: A 192-Week, Phase 3, Open-Label Extension Study.Am J Respir Crit Care Med. 2025 Oct;211(10):1915-1925. doi: 10.1164/rccm.202502-0512OC. Am J Respir Crit Care Med. 2025. PMID: 40454869 Clinical Trial.
References
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- Wainwright C, McColley SA, McNally P, Powers M, Ratjen F, Rayment JH. et al. VX19-445-107 Study Group. Long-term safety and efficacy of elexacaftor/tezacaftor/ivacaftor in children ⩾6 years with cystic fibrosis and at least one F508del allele: a 192-week, phase 3, open-label extension study. Am J Respir Crit Care Med . 2025;211:1915–1925. - PubMed
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- Gabillard-Lefort C, Casey M, Glasgow AMA, Boland F, Kerr O, Marron E. et al. Trikafta rescues CFTR and lowers monocyte P2X7R-induced inflammasome activation in cystic fibrosis. Am J Respir Crit Care Med . 2022;205:783–794. - PubMed
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- Ratner D, Mueller C. Immune responses in cystic fibrosis: are they intrinsically defective? Am J Respir Cell Mol Biol . 2012 - PubMed
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