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166 related items for PubMed ID: 28132845
1. Use of hyperpolarized helium-3 MRI to assess response to ivacaftor treatment in patients with cystic fibrosis. Altes TA, Johnson M, Fidler M, Botfield M, Tustison NJ, Leiva-Salinas C, de Lange EE, Froh D, Mugler JP. J Cyst Fibros; 2017 Mar; 16(2):267-274. PubMed ID: 28132845 [Abstract] [Full Text] [Related]
3. Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis. Elborn JS, Ramsey BW, Boyle MP, Konstan MW, Huang X, Marigowda G, Waltz D, Wainwright CE, VX-809 TRAFFIC and TRANSPORT study groups. Lancet Respir Med; 2016 Aug; 4(8):617-626. PubMed ID: 27298017 [Abstract] [Full Text] [Related]
5. Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor. Flume PA, Wainwright CE, Elizabeth Tullis D, Rodriguez S, Niknian M, Higgins M, Davies JC, Wagener JS. J Cyst Fibros; 2018 Jan; 17(1):83-88. PubMed ID: 28651844 [Abstract] [Full Text] [Related]
6. Antibiotic exposure and interpersonal variance mask the effect of ivacaftor on respiratory microbiota composition. Peleg AY, Choo JM, Langan KM, Edgeworth D, Keating D, Wilson J, Rogers GB, Kotsimbos T. J Cyst Fibros; 2018 Jan; 17(1):50-56. PubMed ID: 29042177 [Abstract] [Full Text] [Related]
7. Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial. Davies J, Sheridan H, Bell N, Cunningham S, Davis SD, Elborn JS, Milla CE, Starner TD, Weiner DJ, Lee PS, Ratjen F. Lancet Respir Med; 2013 Oct; 1(8):630-638. PubMed ID: 24461666 [Abstract] [Full Text] [Related]
8. GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1). Davies JC, Van de Steen O, van Koningsbruggen-Rietschel S, Drevinek P, Derichs N, McKone EF, Kanters D, Allamassey L, Namour F, de Kock H, Conrath K. J Cyst Fibros; 2019 Sep; 18(5):693-699. PubMed ID: 31147302 [Abstract] [Full Text] [Related]
9. Ivacaftor in cystic fibrosis with residual function: Lung function results from an N-of-1 study. Nick JA, St Clair C, Jones MC, Lan L, Higgins M, VX12-770-113 Study Team. J Cyst Fibros; 2020 Jan; 19(1):91-98. PubMed ID: 31784217 [Abstract] [Full Text] [Related]
10. Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, Colombo C, Davies JC, De Boeck K, Flume PA, Konstan MW, McColley SA, McCoy K, McKone EF, Munck A, Ratjen F, Rowe SM, Waltz D, Boyle MP, TRAFFIC Study Group, TRANSPORT Study Group. N Engl J Med; 2015 Jul 16; 373(3):220-31. PubMed ID: 25981758 [Abstract] [Full Text] [Related]
12. Effect of ivacaftor in patients with advanced cystic fibrosis and a G551D-CFTR mutation: Safety and efficacy in an expanded access program in the United States. Taylor-Cousar J, Niknian M, Gilmartin G, Pilewski JM, VX11-770-901 investigators. J Cyst Fibros; 2016 Jan 16; 15(1):116-22. PubMed ID: 25682022 [Abstract] [Full Text] [Related]
13. Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study. Davies JC, Cunningham S, Harris WT, Lapey A, Regelmann WE, Sawicki GS, Southern KW, Robertson S, Green Y, Cooke J, Rosenfeld M, KIWI Study Group. Lancet Respir Med; 2016 Feb 16; 4(2):107-15. PubMed ID: 26803277 [Abstract] [Full Text] [Related]
14. Positive clinical response to ivacaftor treatment in an individual with the CFTR genotype F508del/V456A. Bratcher PE, Hunt KC, Pickard K, Taylor-Cousar JL. J Cyst Fibros; 2019 Mar 16; 18(2):e9-e10. PubMed ID: 30348612 [No Abstract] [Full Text] [Related]
15. Nutritional Status Improved in Cystic Fibrosis Patients with the G551D Mutation After Treatment with Ivacaftor. Borowitz D, Lubarsky B, Wilschanski M, Munck A, Gelfond D, Bodewes F, Schwarzenberg SJ. Dig Dis Sci; 2016 Jan 16; 61(1):198-207. PubMed ID: 26250833 [Abstract] [Full Text] [Related]
16. Improvement in exercise duration, lung function and well-being in G551D-cystic fibrosis patients: a double-blind, placebo-controlled, randomized, cross-over study with ivacaftor treatment. Edgeworth D, Keating D, Ellis M, Button B, Williams E, Clark D, Tierney A, Heritier S, Kotsimbos T, Wilson J. Clin Sci (Lond); 2017 Aug 01; 131(15):2037-2045. PubMed ID: 28611235 [Abstract] [Full Text] [Related]