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Journal Abstract Search

1147 related items for PubMed ID: 28068001

  • 1. 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
    [Abstract] [Full Text] [Related]

  • 2. 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
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  • 3. 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 01; 197(11):1433-1442. PubMed ID: 29327948
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  • 4. Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis.
    Skilton M, Krishan A, Patel S, Sinha IP, Southern KW.
    Cochrane Database Syst Rev; 2019 Jan 07; 1(1):CD009841. PubMed ID: 30616300
    [Abstract] [Full Text] [Related]

  • 5. 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, VX16-445-001 Study Group.
    N Engl J Med; 2018 Oct 25; 379(17):1612-1620. PubMed ID: 30334692
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  • 6. 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 25; 2(7):527-38. PubMed ID: 24973281
    [Abstract] [Full Text] [Related]

  • 7. 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, VX16-659-101 Study Group.
    N Engl J Med; 2018 Oct 25; 379(17):1599-1611. PubMed ID: 30334693
    [Abstract] [Full Text] [Related]

  • 8. 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 25; 143(1):14-18. PubMed ID: 23276841
    [Abstract] [Full Text] [Related]

  • 9. Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor.
    Mesbahi M, Shteinberg M, Wilschanski M, Hatton A, Nguyen-Khoa T, Friedman H, Cohen M, Escabasse V, Le Bourgeois M, Lucidi V, Sermet-Gaudelus I, Bassinet L, Livnat G.
    J Cyst Fibros; 2017 Jan 25; 16(1):45-48. PubMed ID: 27659740
    [Abstract] [Full Text] [Related]

  • 10. Efficacy and safety of lumacaftor and ivacaftor in patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR: a randomised, placebo-controlled phase 3 trial.
    Ratjen F, Hug C, Marigowda G, Tian S, Huang X, Stanojevic S, Milla CE, Robinson PD, Waltz D, Davies JC, VX14-809-109 investigator group.
    Lancet Respir Med; 2017 Jul 25; 5(7):557-567. PubMed ID: 28606620
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  • 12. 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. PubMed ID: 28930490
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  • 16. Ivacaftor restores CFTR-dependent sweat gland fluid secretion in cystic fibrosis subjects with S945L alleles.
    Kim J, Davies Z, Dunn C, Wine JJ, Milla C.
    J Cyst Fibros; 2018 Mar 15; 17(2):179-185. PubMed ID: 29279204
    [Abstract] [Full Text] [Related]

  • 17. A phase 3, double-blind, parallel-group study to evaluate the efficacy and safety of tezacaftor in combination with ivacaftor in participants 6 through 11 years of age with cystic fibrosis homozygous for F508del or heterozygous for the F508del-CFTR mutation and a residual function mutation.
    Davies JC, Sermet-Gaudelus I, Naehrlich L, Harris RS, Campbell D, Ahluwalia N, Short C, Haseltine E, Panorchan P, Saunders C, Owen CA, Wainwright CE, VX16-661-115 Investigator Group.
    J Cyst Fibros; 2021 Jan 15; 20(1):68-77. PubMed ID: 32967799
    [Abstract] [Full Text] [Related]

  • 18. 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 15; 4(2):107-15. PubMed ID: 26803277
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  • 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 15; 131():225-228. PubMed ID: 28947035
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