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Items: 1 to 20 of 80

1.

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. doi: 10.1016/j.jcf.2017.08.002. Epub 2017 Oct 15.

PMID:
29042177
2.

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 Apr 8;10(4):e0124124. doi: 10.1371/journal.pone.0124124. eCollection 2015.

3.

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 26;(3):CD009841. doi: 10.1002/14651858.CD009841.pub2. Review.

PMID:
25811419
4.

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; GOAL Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network.

Am J Respir Crit Care Med. 2014 Jul 15;190(2):175-84. doi: 10.1164/rccm.201404-0703OC.

5.

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; VX08-770-102 Study Group.

N Engl J Med. 2011 Nov 3;365(18):1663-72. doi: 10.1056/NEJMoa1105185.

6.

Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation.

Davies JC, Wainwright CE, Canny GJ, Chilvers MA, Howenstine MS, Munck A, Mainz JG, Rodriguez S, Li H, Yen K, Ordoñez CL, Ahrens R; VX08-770-103 (ENVISION) Study Group.

Am J Respir Crit Care Med. 2013 Jun 1;187(11):1219-25. doi: 10.1164/rccm.201301-0153OC.

7.

Ivacaftor for the treatment of patients with cystic fibrosis and the G551D mutation: a systematic review and cost-effectiveness analysis.

Whiting P, Al M, Burgers L, Westwood M, Ryder S, Hoogendoorn M, Armstrong N, Allen A, Severens H, Kleijnen J.

Health Technol Assess. 2014 Mar;18(18):1-106. doi: 10.3310/hta18180. Review.

8.

A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial.

Boyle MP, Bell SC, Konstan MW, McColley SA, Rowe SM, Rietschel E, Huang X, Waltz D, Patel NR, Rodman D; VX09-809-102 study group.

Lancet Respir Med. 2014 Jul;2(7):527-38. doi: 10.1016/S2213-2600(14)70132-8. Epub 2014 Jun 24.

PMID:
24973281
9.

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. doi: 10.1177/1753465813502115. Epub 2013 Sep 3. Review.

PMID:
24004658
10.

Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial.

Moss RB, Flume PA, Elborn JS, Cooke J, Rowe SM, McColley SA, Rubenstein RC, Higgins M; VX11-770-110 (KONDUCT) Study Group.

Lancet Respir Med. 2015 Jul;3(7):524-33. doi: 10.1016/S2213-2600(15)00201-5. Epub 2015 Jun 9.

11.

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; VX 08-770-104 Study Group.

Chest. 2012 Sep;142(3):718-724. doi: 10.1378/chest.11-2672.

12.

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. doi: 10.1016/j.jcf.2015.01.010. Epub 2015 Feb 16.

13.

Ivacaftor improves appearance of sinus disease on computerised tomography in cystic fibrosis patients with G551D mutation.

Sheikh SI, Long FR, McCoy KS, Johnson T, Ryan-Wenger NA, Hayes D Jr.

Clin Otolaryngol. 2015 Feb;40(1):16-21. doi: 10.1111/coa.12310.

PMID:
25145599
14.

Sustained Benefit from ivacaftor demonstrated by combining clinical trial and cystic fibrosis patient registry data.

Sawicki GS, McKone EF, Pasta DJ, Millar SJ, Wagener JS, Johnson CA, Konstan MW.

Am J Respir Crit Care Med. 2015 Oct 1;192(7):836-42. doi: 10.1164/rccm.201503-0578OC. Erratum in: Am J Respir Crit Care Med. 2016 Jun 1;193(11):1317-20.

PMID:
26132840
15.

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; VX11-661-101 Study Group.

Am J Respir Crit Care Med. 2018 Jan 15;197(2):214-224. doi: 10.1164/rccm.201704-0717OC.

16.

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;61(1):198-207. doi: 10.1007/s10620-015-3834-2. Epub 2015 Aug 7.

PMID:
26250833
17.

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. doi: 10.1016/j.jcf.2014.09.005. Epub 2014 Sep 26.

18.

Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations.

Guimbellot J, Solomon GM, Baines A, Heltshe SL, VanDalfsen J, Joseloff E, Sagel SD, Rowe SM; GOALe(2) Investigators.

J Cyst Fibros. 2018 Apr 21. pii: S1569-1993(18)30090-0. doi: 10.1016/j.jcf.2018.04.004. [Epub ahead of print]

PMID:
29685811
19.

Cystic fibrosis transmembrane conductance regulator-modifying medications: the future of cystic fibrosis treatment.

Pettit RS.

Ann Pharmacother. 2012 Jul-Aug;46(7-8):1065-75. doi: 10.1345/aph.1R076. Epub 2012 Jun 26. Review.

PMID:
22739718
20.

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. doi: 10.1016/j.chest.2017.10.005. Epub 2017 Oct 14.

PMID:
29037527

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