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Comment
. 2021 Aug 1;204(3):372-374.
doi: 10.1164/rccm.202103-0796LE.

Reply to Kuek et al.: Optimism with Caution: Elexacaftor-Tezacaftor-Ivacaftor in Patients with Advanced Pulmonary Disease

Pierre-Régis Burgel  1   2   3 , Jennifer Da Silva  2   3 , Jean-Louis Paillasseur  4 , Clémence Martin  1   2   3
Affiliations
Comment

Reply to Kuek et al.: Optimism with Caution: Elexacaftor-Tezacaftor-Ivacaftor in Patients with Advanced Pulmonary Disease

Pierre-Régis Burgel et al. Am J Respir Crit Care Med. .
No abstract available

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Figures

Figure 1.
Figure 1.
Distribution of the difference between the best ppFEV1 after starting elexacaftor–tezacaftor–ivacaftor compared with baseline ppFEV1 (n = 232 patients). Numbers of patients are indicated on top of the bars. Data are from Reference . ppFEV1 = percentage predicted FEV1.
See this image and copyright information in PMC

Comment on

References

    1. Burgel P-R, Durieu I, Chiron R, Ramel S, Danner-Boucher I, Prevotat A, et al. French Cystic Fibrosis Reference Network Study Group. Rapid improvement after starting elexacaftor–tezacaftor–ivacaftor in patients with cystic fibrosis and advanced pulmonary disease. Am J Respir Crit Care Med. 2021;204 - PubMed
    1. Burgel PR, Durieu I, Chiron R, Mely L, Prevotat A, Murris-Espin M, et al. French Cystic Fibrosis Reference Network study group. Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function. J Cyst Fibros. 2021;20:220–227. - PubMed
    1. McColley SA, Konstan MW, Ramsey BW, Stuart Elborn J, Boyle MP, Wainwright CE, et al. Lumacaftor/ivacaftor reduces pulmonary exacerbations in patients irrespective of initial changes in FEV1. J Cyst Fibros. 2019;18:94–101. - PMC - PubMed
    1. Middleton PG, Mall MA, Dřevínek P, Lands LC, McKone EF, Polineni D, et al. VX17-445-102 Study Group. Elexacaftor-tezacaftor-ivacaftor for cystic fibrosis with a single Phe508del allele. N Engl J Med. 2019;381:1809–1819. - PMC - PubMed
    1. Burgel PR, 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