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5. Effectivenesss of ivacaftor in severe cystic fibrosis patients and non-G551D gating mutations. Salvatore D; Carnovale V; Iacotucci P; Braggion C; Castellani C; Cimino G; Colangelo C; Francalanci M; Leonetti G; Lucidi V; Manca A; Vitullo P; Ferrara N Pediatr Pulmonol; 2019 Sep; 54(9):1398-1403. PubMed ID: 31237430 [TBL] [Abstract][Full Text] [Related]
6. Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation. Guimbellot JS; Baines A; Paynter A; Heltshe SL; VanDalfsen J; Jain M; Rowe SM; Sagel SD; J Cyst Fibros; 2021 Mar; 20(2):213-219. PubMed ID: 33249004 [TBL] [Abstract][Full Text] [Related]
7. CORK Study in Cystic Fibrosis: Sustained Improvements in Ultra-Low-Dose Chest CT Scores After CFTR Modulation With Ivacaftor. Ronan NJ; Einarsson GG; Twomey M; Mooney D; Mullane D; NiChroinin M; O'Callaghan G; Shanahan F; Murphy DM; O'Connor OJ; Shortt CA; Tunney MM; Eustace JA; Maher MM; Elborn JS; Plant BJ Chest; 2018 Feb; 153(2):395-403. PubMed ID: 29037527 [TBL] [Abstract][Full Text] [Related]
8. 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 [TBL] [Abstract][Full Text] [Related]
9. VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles. Davies JC; Moskowitz SM; Brown C; Horsley A; Mall MA; McKone EF; Plant BJ; Prais D; Ramsey BW; Taylor-Cousar JL; Tullis E; Uluer A; McKee CM; Robertson S; Shilling RA; Simard C; Van Goor F; Waltz D; Xuan F; Young T; Rowe SM; N Engl J Med; 2018 Oct; 379(17):1599-1611. PubMed ID: 30334693 [TBL] [Abstract][Full Text] [Related]
10. VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles. Keating D; Marigowda G; Burr L; Daines C; Mall MA; McKone EF; Ramsey BW; Rowe SM; Sass LA; Tullis E; McKee CM; Moskowitz SM; Robertson S; Savage J; Simard C; Van Goor F; Waltz D; Xuan F; Young T; Taylor-Cousar JL; N Engl J Med; 2018 Oct; 379(17):1612-1620. PubMed ID: 30334692 [TBL] [Abstract][Full Text] [Related]
11. CFTR-dependent chloride efflux in cystic fibrosis mononuclear cells is increased by ivacaftor therapy. Guerra L; D'Oria S; Favia M; Castellani S; Santostasi T; Polizzi AM; Mariggiò MA; Gallo C; Casavola V; Montemurro P; Leonetti G; Manca A; Conese M Pediatr Pulmonol; 2017 Jul; 52(7):900-908. PubMed ID: 28445004 [TBL] [Abstract][Full Text] [Related]
12. In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies. McGarry ME; Illek B; Ly NP; Zlock L; Olshansky S; Moreno C; Finkbeiner WE; Nielson DW Pediatr Pulmonol; 2017 Apr; 52(4):472-479. PubMed ID: 28068001 [TBL] [Abstract][Full Text] [Related]
13. [Real-world effectiveness of ivacaftor in children with cystic fibrosis and the G551D mutation]. Gomez-Pastrana D; Nwokoro C; McLean M; Brown S; Christiansen N; Pao CS An Pediatr (Engl Ed); 2019 Mar; 90(3):148-156. PubMed ID: 30093322 [TBL] [Abstract][Full Text] [Related]
14. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. Ramsey BW; Davies J; McElvaney NG; Tullis E; Bell SC; Dřevínek P; Griese M; McKone EF; Wainwright CE; Konstan MW; Moss R; Ratjen F; Sermet-Gaudelus I; Rowe SM; Dong Q; Rodriguez S; Yen K; Ordoñez C; Elborn JS; N Engl J Med; 2011 Nov; 365(18):1663-72. PubMed ID: 22047557 [TBL] [Abstract][Full Text] [Related]
15. IVACAFTOR restores FGF19 regulated bile acid homeostasis in cystic fibrosis patients with an S1251N or a G551D gating mutation. van de Peppel IP; Doktorova M; Berkers G; de Jonge HR; Houwen RHJ; Verkade HJ; Jonker JW; Bodewes FAJA J Cyst Fibros; 2019 Mar; 18(2):286-293. PubMed ID: 30279125 [TBL] [Abstract][Full Text] [Related]
16. Impact of the CFTR-potentiator ivacaftor on airway microbiota in cystic fibrosis patients carrying a G551D mutation. Bernarde C; Keravec M; Mounier J; Gouriou S; Rault G; Férec C; Barbier G; Héry-Arnaud G PLoS One; 2015; 10(4):e0124124. PubMed ID: 25853698 [TBL] [Abstract][Full Text] [Related]
17. Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence. Kotha K; Clancy JP Ther Adv Respir Dis; 2013 Oct; 7(5):288-96. PubMed ID: 24004658 [TBL] [Abstract][Full Text] [Related]
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19. Ivacaftor Reduces Inflammatory Mediators in Upper Airway Lining Fluid From Cystic Fibrosis Patients With a G551D Mutation: Serial Non-Invasive Home-Based Collection of Upper Airway Lining Fluid. Mainz JG; Arnold C; Wittstock K; Hipler UC; Lehmann T; Zagoya C; Duckstein F; Ellemunter H; Hentschel J Front Immunol; 2021; 12():642180. PubMed ID: 34025651 [TBL] [Abstract][Full Text] [Related]
20. Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation. De Boeck K; Munck A; Walker S; Faro A; Hiatt P; Gilmartin G; Higgins M J Cyst Fibros; 2014 Dec; 13(6):674-80. PubMed ID: 25266159 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]