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Items: 1 to 20 of 475

1.

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
2.

Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.

Sermet-Gaudelus I.

Eur Respir Rev. 2013 Mar 1;22(127):66-71. doi: 10.1183/09059180.00008512. Review.

3.

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. doi: 10.1358/dot.2013.49.4.1940984.

PMID:
23616952
4.

Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis.

Derichs N.

Eur Respir Rev. 2013 Mar 1;22(127):58-65. doi: 10.1183/09059180.00008412. Review.

5.

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
6.

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
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):630-8. doi: 10.1016/S2213-2600(13)70182-6. Epub 2013 Sep 10.

PMID:
24461666
8.

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.

9.

Development, clinical utility, and place of ivacaftor in the treatment of cystic fibrosis.

O'Reilly R, Elphick HE.

Drug Des Devel Ther. 2013 Aug 30;7:929-37. doi: 10.2147/DDDT.S30345. eCollection 2013. Review.

10.

Ivacaftor potentiation of multiple CFTR channels with gating mutations.

Yu H, Burton B, Huang CJ, Worley J, Cao D, Johnson JP Jr, Urrutia A, Joubran J, Seepersaud S, Sussky K, Hoffman BJ, Van Goor F.

J Cyst Fibros. 2012 May;11(3):237-45. doi: 10.1016/j.jcf.2011.12.005. Epub 2012 Jan 30.

11.

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.

PMID:
25698453
12.

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.

13.

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
14.

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.

15.

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.

PMID:
26132840
16.

The impact of personalised therapies on respiratory medicine.

Elborn JS.

Eur Respir Rev. 2013 Mar 1;22(127):72-4. doi: 10.1183/09059180.00008212.

17.

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):718-24.

18.

Lumacaftor alone and combined with ivacaftor: preclinical and clinical trial experience of F508del CFTR correction.

Brewington JJ, McPhail GL, Clancy JP.

Expert Rev Respir Med. 2016;10(1):5-17. doi: 10.1586/17476348.2016.1122527. Epub 2015 Dec 9.

PMID:
26581802
19.

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):e79-82. doi: 10.1378/chest.14-1198.

PMID:
25732475
20.

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.

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