147 related articles for article (PubMed ID: 32855222)
1. Ivacaftor modifies cystic fibrosis neutrophil phenotype in subjects with R117H residual function CFTR mutations.
Hardisty GR; Law SM; Carter S; Grogan B; Singh PK; McKone EF; Gray RD
Eur Respir J; 2021 Jan; 57(1):. PubMed ID: 32855222
[No Abstract] [Full Text] [Related]
2. Ivacaftor as salvage therapy in a patient with cystic fibrosis genotype F508del/R117H/IVS8-5T.
Carter S; Kelly S; Caples E; Grogan B; Doyle J; Gallagher CG; McKone EF
J Cyst Fibros; 2015 Jul; 14(4):e4-5. PubMed ID: 25698453
[TBL] [Abstract][Full Text] [Related]
3. Improved clinical and radiographic outcomes after treatment with ivacaftor in a young adult with cystic fibrosis with the P67L CFTR mutation.
Yousef S; Solomon GM; Brody A; Rowe SM; Colin AA
Chest; 2015 Mar; 147(3):e79-e82. PubMed ID: 25732475
[TBL] [Abstract][Full Text] [Related]
4. Use of ivacaftor in late diagnosed cystic fibrosis monozygotic twins heterozygous for F508del and R117H-7T - a case report.
Welsner M; Straßburg S; Taube C; Sutharsan S
BMC Pulm Med; 2019 Apr; 19(1):76. PubMed ID: 30975115
[TBL] [Abstract][Full Text] [Related]
5. 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]
6. Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis.
Rowe SM; Daines C; Ringshausen FC; Kerem E; Wilson J; Tullis E; Nair N; Simard C; Han L; Ingenito EP; McKee C; Lekstrom-Himes J; Davies JC
N Engl J Med; 2017 Nov; 377(21):2024-2035. PubMed ID: 29099333
[TBL] [Abstract][Full Text] [Related]
7. Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.
Flume PA; Biner RF; Downey DG; Brown C; Jain M; Fischer R; De Boeck K; Sawicki GS; Chang P; Paz-Diaz H; Rubin JL; Yang Y; Hu X; Pasta DJ; Millar SJ; Campbell D; Wang X; Ahluwalia N; Owen CA; Wainwright CE;
Lancet Respir Med; 2021 Jul; 9(7):733-746. PubMed ID: 33581080
[TBL] [Abstract][Full Text] [Related]
8. 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]
9. 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]
10. 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]
11. The Role of Ivacaftor in Severe Cystic Fibrosis in a Patient With the R117H Mutation.
Ronan NJ; Fleming C; O'Callaghan G; Maher MM; Murphy DM; Plant BJ
Chest; 2015 Sep; 148(3):e72-e75. PubMed ID: 26324139
[TBL] [Abstract][Full Text] [Related]
12. 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]
13. 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]
14. Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects.
Kuk K; Taylor-Cousar JL
Ther Adv Respir Dis; 2015 Dec; 9(6):313-26. PubMed ID: 26416827
[TBL] [Abstract][Full Text] [Related]
15. Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis.
Southern KW; Patel S; Sinha IP; Nevitt SJ
Cochrane Database Syst Rev; 2018 Aug; 8(8):CD010966. PubMed ID: 30070364
[TBL] [Abstract][Full Text] [Related]
16. Tezacaftor/Ivacaftor in Subjects with Cystic Fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR.
Donaldson SH; Pilewski JM; Griese M; Cooke J; Viswanathan L; Tullis E; Davies JC; Lekstrom-Himes JA; Wang LT;
Am J Respir Crit Care Med; 2018 Jan; 197(2):214-224. PubMed ID: 28930490
[TBL] [Abstract][Full Text] [Related]
17. Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis.
McPhail GL; Clancy JP
Drugs Today (Barc); 2013 Apr; 49(4):253-60. PubMed ID: 23616952
[TBL] [Abstract][Full Text] [Related]
18. Treating the Underlying Cystic Fibrosis Transmembrane Conductance Regulator Defect in Patients with Cystic Fibrosis.
Cuyx S; De Boeck K
Semin Respir Crit Care Med; 2019 Dec; 40(6):762-774. PubMed ID: 31659727
[TBL] [Abstract][Full Text] [Related]
19. On the mechanism of gating defects caused by the R117H mutation in cystic fibrosis transmembrane conductance regulator.
Yu YC; Sohma Y; Hwang TC
J Physiol; 2016 Jun; 594(12):3227-44. PubMed ID: 26846474
[TBL] [Abstract][Full Text] [Related]
20. Intestinal Current Measurements Detect Activation of Mutant CFTR in Patients with Cystic Fibrosis with the G551D Mutation Treated with Ivacaftor.
Graeber SY; Hug MJ; Sommerburg O; Hirtz S; Hentschel J; Heinzmann A; Dopfer C; Schulz A; Mainz JG; Tümmler B; Mall MA
Am J Respir Crit Care Med; 2015 Nov; 192(10):1252-5. PubMed ID: 26568242
[No Abstract] [Full Text] [Related]
[Next] [New Search]
