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Items: 1 to 20 of 175

1.

Ivacaftor in a G551D homozygote with cystic fibrosis.

Harrison MJ, Murphy DM, Plant BJ.

N Engl J Med. 2013 Sep 26;369(13):1280-2. doi: 10.1056/NEJMc1213681. No abstract available.

2.

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. doi: 10.1016/j.jcf.2013.03.004. Epub 2013 Apr 28.

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.

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.

5.

Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: the ivacaftor experience.

Durmowicz AG, Witzmann KA, Rosebraugh CJ, Chowdhury BA.

Chest. 2013 Jan;143(1):14-18. doi: 10.1378/chest.12-1430.

PMID:
23276841
6.

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.

7.

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.

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

PMID:
24461666
9.

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
10.

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; KIWI Study Group.

Lancet Respir Med. 2016 Feb;4(2):107-15. doi: 10.1016/S2213-2600(15)00545-7. Epub 2016 Jan 21.

PMID:
26803277
11.

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.

12.

Efficacy response in CF patients treated with ivacaftor: post-hoc analysis.

Konstan MW, Plant BJ, Elborn JS, Rodriguez S, Munck A, Ahrens R, Johnson C.

Pediatr Pulmonol. 2015 May;50(5):447-55. doi: 10.1002/ppul.23173. Epub 2015 Mar 9.

PMID:
25755212
13.

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 15;192(10):1252-5. doi: 10.1164/rccm.201507-1271LE. No abstract available.

PMID:
26568242
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.

Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.

Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordoñez CL, Campbell PW, Ashlock MA, Ramsey BW.

N Engl J Med. 2010 Nov 18;363(21):1991-2003. doi: 10.1056/NEJMoa0909825.

16.

Sweat chloride is not a useful marker of clinical response to Ivacaftor.

Barry PJ, Jones AM, Webb AK, Horsley AR.

Thorax. 2014 Jun;69(6):586-7. doi: 10.1136/thoraxjnl-2013-204532. Epub 2013 Nov 20.

PMID:
24258833
17.

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.

18.

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.

19.
20.

Lumacaftor/ivacaftor for patients homozygous for Phe508del-CFTR: should we curb our enthusiasm?

Jones AM, Barry PJ.

Thorax. 2015 Jul;70(7):615-6. doi: 10.1136/thoraxjnl-2015-207369. No abstract available.

PMID:
26071414

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