These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.
4. Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis. Patel S; Sinha IP; Dwan K; Echevarria C; Schechter M; Southern KW Cochrane Database Syst Rev; 2015 Mar; (3):CD009841. PubMed ID: 25811419 [TBL] [Abstract][Full Text] [Related]
5. 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]
6. Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation. Flume PA; Liou TG; Borowitz DS; Li H; Yen K; Ordoñez CL; Geller DE; Chest; 2012 Sep; 142(3):718-724. PubMed ID: 22383668 [TBL] [Abstract][Full Text] [Related]
7. 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]
8. 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]
9. A phase 3, randomized, double-blind, parallel-group study to evaluate tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for F508del-CFTR and a gating mutation. McKone EF; DiMango EA; Sutharsan S; Barto TL; Campbell D; Ahluwalia N; Higgins M; Owen CA; Tullis E J Cyst Fibros; 2021 Mar; 20(2):234-242. PubMed ID: 33339768 [TBL] [Abstract][Full Text] [Related]
10. [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]
11. 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; Lancet Respir Med; 2016 Feb; 4(2):107-15. PubMed ID: 26803277 [TBL] [Abstract][Full Text] [Related]
12. Lumacaftor/Ivacaftor Treatment of Patients with Cystic Fibrosis Heterozygous for F508del-CFTR. Rowe SM; McColley SA; Rietschel E; Li X; Bell SC; Konstan MW; Marigowda G; Waltz D; Boyle MP; Ann Am Thorac Soc; 2017 Feb; 14(2):213-219. PubMed ID: 27898234 [TBL] [Abstract][Full Text] [Related]
13. 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]
14. The predictive potential of the sweat chloride test in cystic fibrosis patients with the G551D mutation. Seliger VI; Rodman D; Van Goor F; Schmelz A; Mueller P J Cyst Fibros; 2013 Dec; 12(6):706-13. PubMed ID: 23628510 [TBL] [Abstract][Full Text] [Related]
15. 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]
16. Effects of Lumacaftor-Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis. Graeber SY; Dopfer C; Naehrlich L; Gyulumyan L; Scheuermann H; Hirtz S; Wege S; Mairbäurl H; Dorda M; Hyde R; Bagheri-Hanson A; Rueckes-Nilges C; Fischer S; Mall MA; Tümmler B Am J Respir Crit Care Med; 2018 Jun; 197(11):1433-1442. PubMed ID: 29327948 [TBL] [Abstract][Full Text] [Related]
17. Ivacaftor withdrawal syndrome in cystic fibrosis patients with the G551D mutation. Trimble AT; Donaldson SH J Cyst Fibros; 2018 Mar; 17(2):e13-e16. PubMed ID: 29079142 [TBL] [Abstract][Full Text] [Related]
18. 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]
19. Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis. Rowe SM; Heltshe SL; Gonska T; Donaldson SH; Borowitz D; Gelfond D; Sagel SD; Khan U; Mayer-Hamblett N; Van Dalfsen JM; Joseloff E; Ramsey BW; Am J Respir Crit Care Med; 2014 Jul; 190(2):175-84. PubMed ID: 24927234 [TBL] [Abstract][Full Text] [Related]
20. Ivacaftor for the p.Ser549Arg (S549R) gating mutation - The Israeli experience. Dagan A; Cohen-Cymberknoh M; Shteinberg M; Levine H; Vilozni D; Bezalel Y; Bar Aluma BE; Sarouk I; Ashkenazi M; Lavie M; Tsabari R; Blau H; Kerem E; Bentur L; Efrati O; Livnat G Respir Med; 2017 Oct; 131():225-228. PubMed ID: 28947035 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]