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PardoASelmanMIdiopathic pulmonary fibrosis: new insights in its pathogenesisInt J Biochem Cell Biol2002341534153812379275PardoASelmanMIdiopathic pulmonary fibrosis: new insights in its pathogenesisInt J Biochem Cell Biol2002341534153812379275, PardoASelmanMIdiopathic pulmonary fibrosis: new insights in its pathogenesisInt J Biochem Cell Biol2002341534153812379275
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EDITORIALS 4. Wainwright CE, Elborn JS, Ramsey BW. Lumacaftor-ivacaftor in triple-combination modulator therapy, which has shown greater efficacy patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl than either dual combination and has been approved by the U.S. Food JMed 2015;373:1783–1784. and Drug Administration. With the approval of the triple-combination 5. Burgel P-R, Munck A, Durieu I, Chiron R, Mely L, Prevotat A, et al.; drug, 90% of patients with CF will be eligible for a CFTR modulator French Cystic Fibrosis Reference Network Study Group. Real-life (13). It is plausible that in the not-too-distant future, most infants safety and effectiveness of lumacaftor–ivacaftor in patients with cystic fibrosis. Am J Respir Crit Care Med 2020;201:188–197. diagnosed with CF will begin a highly effective CFTR modulator, 6. Elborn JS, Ramsey BW, Boyle MP, Konstan MW, Huang X, Marigowda such as the triple-combination treatment, shortly after birth, and G, et al.; VX-809 TRAFFIC and TRANSPORT Study Groups. Efficacy continue receiving it indefinitely. This possibility highlights the and safety of lumacaftor/ivacaftor combination therapy in patients continuing need for postmarketing observational analyses, such as with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis. Lancet Respir Med 2016;4: this
American Journal of Respiratory and Critical Care Medicine – Pubmed Central
Published: Jan 15, 2020
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