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Editorial
. 2020 Jan 15;201(2):133-134.
doi: 10.1164/rccm.201910-1894ED.

Combination Therapy with Lumacaftor-Ivacaftor in Cystic Fibrosis. Keeping It Real

Marc A Sala  1 , Manu Jain  1
Affiliations
Editorial

Combination Therapy with Lumacaftor-Ivacaftor in Cystic Fibrosis. Keeping It Real

Marc A Sala et al. Am J Respir Crit Care Med. .
No abstract available

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Comment on

  • Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis.
    Burgel PR, Munck A, Durieu I, Chiron R, Mely L, Prevotat A, Murris-Espin M, Porzio M, Abely M, Reix P, Marguet C, Macey J, Sermet-Gaudelus I, Corvol H, Bui S, Lemonnier L, Dehillotte C, Da Silva J, Paillasseur JL, Hubert D; French Cystic Fibrosis Reference Network Study Group. Burgel PR, et al. Am J Respir Crit Care Med. 2020 Jan 15;201(2):188-197. doi: 10.1164/rccm.201906-1227OC. Am J Respir Crit Care Med. 2020. PMID: 31601120

References

    1. Bessonova L, Volkova N, Higgins M, Bengtsson L, Tian S, Simard C, et al. Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor. Thorax. 2018;73:731–740. - PMC - PubMed
    1. Kirwan L, Fletcher G, Harrington M, Jeleniewska P, Zhou S, Casserly B, et al. Longitudinal trends in real-world outcomes after initiation of ivacaftor: a cohort study from the cystic fibrosis registry of Ireland. Ann Am Thorac Soc. 2019;16:209–216. - PubMed
    1. Hubert D, Dehillotte C, Munck A, David V, Baek J, Mely L, et al. Retrospective observational study of French patients with cystic fibrosis and a Gly551Asp-CFTR mutation after 1 and 2years of treatment with ivacaftor in a real-world setting. J Cyst Fibros. 2018;17:89–95. - PubMed
    1. Wainwright CE, Elborn JS, Ramsey BW. Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015;373:1783–1784. - PubMed
    1. Burgel P-R, Munck A, Durieu I, Chiron R, Mely L, Prevotat A, et al. French Cystic Fibrosis Reference Network Study Group. Real-life safety and effectiveness of lumacaftor–ivacaftor in patients with cystic fibrosis. Am J Respir Crit Care Med. 2020;201:188–197. - PubMed