메뉴 건너뛰기




Volumn 18, Issue 5, 2012, Pages 614-627

New therapies in cystic fibrosis

Author keywords

Cystic fibrosis; Lung disease; Therapy

Indexed keywords

4 PHENYLBUTYRIC ACID; ACETYLCYSTEINE; ALPHA 1 ANTITRYPSIN; AMIKACIN; AMILORIDE; ANTIBIOTIC AGENT; ATALUREN; AZITHROMYCIN; AZTREONAM; CIPROFLOXACIN; COLISTIN; CORTICOSTEROID; CPX; CURCUMIN; DENUFOSOL; DORNASE ALFA; FOSFOMYCIN; GENISTEIN; GENTAMICIN; IBUPROFEN; IVACAFTOR; LANCOVUTIDE; LEVOFLOXACIN; LUMACAFTOR; MANNITOL; MUCOLYTIC AGENT; SODIUM CHANNEL BLOCKING AGENT; TOBRAMYCIN; TRANSMEMBRANE CONDUCTANCE REGULATOR; UNCLASSIFIED DRUG; UNINDEXED DRUG;

EID: 84856445142     PISSN: 13816128     EISSN: 18734286     Source Type: Journal    
DOI: 10.2174/138161212799315984     Document Type: Article
Times cited : (22)

References (163)
  • 1
    • 0037460729 scopus 로고    scopus 로고
    • Cystic fibrosis
    • Ratjen F, Doring G. Cystic fibrosis. Lancet 2003. 361[9358]: 681-9.
    • (2003) Lancet , vol.361 , Issue.9358 , pp. 681-689
    • Ratjen, F.1    Doring, G.2
  • 2
    • 66849089934 scopus 로고    scopus 로고
    • Cystic fibrosis: Pathogenesis and future treatment strategies
    • Ratjen FA. Cystic fibrosis: pathogenesis and future treatment strategies. Respir Care 2009. 54[5]: 595-605.
    • (2009) Respir Care , vol.54 , Issue.5 , pp. 595-605
    • Ratjen, F.A.1
  • 3
    • 34848821636 scopus 로고    scopus 로고
    • New pulmonary therapies for cystic fibrosis
    • Ratjen F. New pulmonary therapies for cystic fibrosis. Curr Opin Pulm Med 2007. 13[6]: 541-6.
    • (2007) Curr Opin Pulm Med , vol.13 , Issue.6 , pp. 541-546
    • Ratjen, F.1
  • 5
    • 84856408603 scopus 로고    scopus 로고
    • Modifier gene studies to identify new therapeutic targets in cystic fibrosis
    • Dorfman R. Modifier gene studies to identify new therapeutic targets in cystic fibrosis. Curr Pharm Des 2012; 18(5): 674-82.
    • (2012) Curr Pharm Des , vol.18 , Issue.5 , pp. 674-682
    • Dorfman, R.1
  • 6
    • 0031851457 scopus 로고    scopus 로고
    • Glutathione permeability of CFTR
    • Linsdell P, Hanrahan JW. Glutathione permeability of CFTR. Am J Physiol 1998; 275[1 Pt 1]: C323-6.
    • (1998) Am J Physiol , vol.275 , Issue.1 Pt 1
    • Linsdell, P.1    Hanrahan, J.W.2
  • 7
    • 0036305756 scopus 로고    scopus 로고
    • Mucociliary transport and cough in humans
    • Foster WM. Mucociliary transport and cough in humans. Pulm Pharmacol Ther 2002; 15[3]: 277-82.
    • (2002) Pulm Pharmacol Ther , vol.15 , Issue.3 , pp. 277-282
    • Foster, W.M.1
  • 8
    • 34047166052 scopus 로고    scopus 로고
    • Airway surface dehydration in cystic fibrosis: Pathogenesis and therapy
    • Boucher RC. Airway surface dehydration in cystic fibrosis: pathogenesis and therapy. Annu Rev Med 2007; 58: 157-70.
    • (2007) Annu Rev Med , vol.58 , pp. 157-170
    • Boucher, R.C.1
  • 9
    • 33645964965 scopus 로고    scopus 로고
    • Regulation of normal and cystic fibrosis airway surface liquid volume by phasic shear stress
    • Tarran R, Button B, Boucher RC. Regulation of normal and cystic fibrosis airway surface liquid volume by phasic shear stress. Annu Rev Physiol 2006; 68: 543-61.
    • (2006) Annu Rev Physiol , vol.68 , pp. 543-561
    • Tarran, R.1    Button, B.2    Boucher, R.C.3
  • 10
    • 0036167851 scopus 로고    scopus 로고
    • Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients
    • Worlitzsch D, Tarran R, Ulrich M, et al. Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients. J Clin Invest 2002; 109[3]: 317-25.
    • (2002) J Clin Invest , vol.109 , Issue.3 , pp. 317-325
    • Worlitzsch, D.1    Tarran, R.2    Ulrich, M.3
  • 11
    • 84856488316 scopus 로고    scopus 로고
    • Progress in gene and cell therapy for cystic fibrosis lung disease
    • Griesenbach U, Eric WFW. Progress in gene and cell therapy for cystic fibrosis lung disease. Curr Pharm Des 2012; 18(5): 642-62.
    • (2012) Curr Pharm Des , vol.18 , Issue.5 , pp. 642-662
    • Griesenbach, U.1    Eric, W.F.W.2
  • 12
    • 0026781952 scopus 로고
    • Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
    • Denning GM, Anderson MP, Amara JF, et al. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 1992; 358[6389]: 761-4.
    • (1992) Nature , vol.358 , Issue.6389 , pp. 761-764
    • Denning, G.M.1    Anderson, M.P.2    Amara, J.F.3
  • 13
    • 43449091135 scopus 로고    scopus 로고
    • Interplay between exonic splicing enhancers, mRNA processing, and mRNA surveillance in the dystrophic Mdx mouse
    • Buvoli M, Buvoli A, Leinwand LA. Interplay between exonic splicing enhancers, mRNA processing, and mRNA surveillance in the dystrophic Mdx mouse. PLoS One 2007; 2[5]: e427.
    • (2007) PLoS One , vol.2 , Issue.5
    • Buvoli, M.1    Buvoli, A.2    Leinwand, L.A.3
  • 14
    • 28644433645 scopus 로고    scopus 로고
    • Mild cystic fibrosis revealed by persistent hyponatremia during the French 2003 heat wave, associated with the S1455X C-terminus CFTR mutation
    • Epaud R, Girodon E, Corvol H, et al. Mild cystic fibrosis revealed by persistent hyponatremia during the French 2003 heat wave, associated with the S1455X C-terminus CFTR mutation. Clin Genet 2005; 68[6]: 552-3.
    • (2005) Clin Genet , vol.68 , Issue.6 , pp. 552-553
    • Epaud, R.1    Girodon, E.2    Corvol, H.3
  • 15
    • 0141863491 scopus 로고    scopus 로고
    • Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations
    • Wilschanski M, Yahav Y, Yaacov Y, et al. Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations. N Engl J Med 2003; 349[15]: 1433-41.
    • (2003) N Engl J Med , vol.349 , Issue.15 , pp. 1433-1441
    • Wilschanski, M.1    Yahav, Y.2    Yaacov, Y.3
  • 16
    • 34347224756 scopus 로고    scopus 로고
    • No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations
    • Clancy JP, Rowe SM, Bebok Z, et.al. No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations. Am J Respir Cell Mol Biol 2007; 37[1]: 57-66.
    • (2007) Am J Respir Cell Mol Biol , vol.37 , Issue.1 , pp. 57-66
    • Clancy, J.P.1    Rowe, S.M.2
  • 17
    • 34247588271 scopus 로고    scopus 로고
    • PTC124 targets genetic disorders caused by nonsense mutations
    • Welch EM, Barton ER, Zhuo J, et al. PTC124 targets genetic disorders caused by nonsense mutations. Nature 2007; 447[7140]: 87-91.
    • (2007) Nature , vol.447 , Issue.7140 , pp. 87-91
    • Welch, E.M.1    Barton, E.R.2    Zhuo, J.3
  • 18
    • 33947529670 scopus 로고    scopus 로고
    • Safety, tolerability, and pharmacokinetics of PTC124, a nonaminoglycoside nonsense mutation suppressor, following single- and multiple-dose administration to healthy male and female adult volunteers
    • Hirawat S, Welch EM, Elfring GL, et al. Safety, tolerability, and pharmacokinetics of PTC124, a nonaminoglycoside nonsense mutation suppressor, following single- and multiple-dose administration to healthy male and female adult volunteers. J Clin Pharmacol 2007; 47[4]: 430-44.
    • (2007) J Clin Pharmacol , vol.47 , Issue.4 , pp. 430-444
    • Hirawat, S.1    Welch, E.M.2    Elfring, G.L.3
  • 19
    • 33847360602 scopus 로고    scopus 로고
    • Nonsense-mediated mRNA decay affects nonsense transcript levels and governs response of cystic fibrosis patients to gentamicin
    • Linde L, Boelz S, Nissim-Rafinia M, et al. Nonsense-mediated mRNA decay affects nonsense transcript levels and governs response of cystic fibrosis patients to gentamicin. J Clin Invest 2007; 117[3]: 683-92.
    • (2007) J Clin Invest , vol.117 , Issue.3 , pp. 683-692
    • Linde, L.1    Boelz, S.2    Nissim-Rafinia, M.3
  • 20
    • 34247200483 scopus 로고    scopus 로고
    • In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: A pilot study
    • Sermet-Gaudelus I, Renouil M, Fajac A, et al. In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study. BMC Med 2007; 5: 5.
    • (2007) BMC Med , vol.5 , pp. 5
    • Sermet-Gaudelus, I.1    Renouil, M.2    Fajac, A.3
  • 21
    • 50149098401 scopus 로고    scopus 로고
    • Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: A prospective phase II trial
    • Kerem E, Hirawat S, Armoni S, et al. Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial. Lancet 2008; 372[9640]: 719-27.
    • (2008) Lancet , vol.372 , Issue.9640 , pp. 719-727
    • Kerem, E.1    Hirawat, S.2    Armoni, S.3
  • 22
    • 78349290383 scopus 로고    scopus 로고
    • Ataluren [PTC124] induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis
    • Sermet-Gaudelus I, Boeck KD, Casimir GJ, et al. Ataluren [PTC124] induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis. Am J Respir Crit Care Med 2010; 182[10]: 1262-72.
    • (2010) Am J Respir Crit Care Med , vol.182 , Issue.10 , pp. 1262-1272
    • Sermet-Gaudelus, I.1    Boeck, K.D.2    Casimir, G.J.3
  • 23
    • 79960187095 scopus 로고    scopus 로고
    • Chronic ataluren [PTC124] treatment of nonsense mutation cystic fibrosis
    • Wilschanski M, Miller LL, Shoseyov D, et al. Chronic ataluren [PTC124] treatment of nonsense mutation cystic fibrosis. Eur Respir J 2011; 38(1): 59-69.
    • (2011) Eur Respir J , vol.38 , Issue.1 , pp. 59-69
    • Wilschanski, M.1    Miller, L.L.2    Shoseyov, D.3
  • 24
    • 84856499552 scopus 로고    scopus 로고
    • NCT00803205
    • www.clinicaltrials.gov: NCT00803205.
  • 25
    • 0025242929 scopus 로고
    • Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
    • Cheng S, Gregory R, Marshall J, et al. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 1990; 63: 827-834.
    • (1990) Cell , vol.63 , pp. 827-834
    • Cheng, S.1    Gregory, R.2    Marshall, J.3
  • 26
    • 50649123290 scopus 로고    scopus 로고
    • CFTR function and prospects for therapy
    • Riordan JR. CFTR function and prospects for therapy. Annu Rev Biochem 2008; 77: 701-26.
    • (2008) Annu Rev Biochem , vol.77 , pp. 701-726
    • Riordan, J.R.1
  • 27
    • 77956237958 scopus 로고    scopus 로고
    • A chemical corrector modifies the channel function of F508del-CFTR
    • Epub
    • Kim Chiaw P, Wellhauser L, Huan LJ, et al. A chemical corrector modifies the channel function of F508del-CFTR. Mol Pharmacol 2010; 78[3]: 411-8. Epub.
    • (2010) Mol Pharmacol , vol.78 , Issue.3 , pp. 411-418
    • Kim, C.P.1    Wellhauser, L.2    Huan, L.J.3
  • 28
    • 77953133544 scopus 로고    scopus 로고
    • Repairing faulty genes by aminoglycosides: Development of new derivatives of geneticin [G418] with enhanced suppression of diseases-causing nonsense mutations
    • Nudelman I, Glikin D, Smolkin B, et al. Repairing faulty genes by aminoglycosides: development of new derivatives of geneticin [G418] with enhanced suppression of diseases-causing nonsense mutations. Bioorg Med Chem 2010; 18[11]: 3735-46.
    • (2010) Bioorg Med Chem , vol.18 , Issue.11 , pp. 3735-3746
    • Nudelman, I.1    Glikin, D.2    Smolkin, B.3
  • 29
    • 0037088577 scopus 로고    scopus 로고
    • Genistein restores functional interactions between Delta F508-CFTR and ENaC in Xenopus oocytes
    • Suaud L, Li J, Jiang Q, et al. Genistein restores functional interactions between Delta F508-CFTR and ENaC in Xenopus oocytes. J Biol Chem 2002; 277[11]: 8928-33.
    • (2002) J Biol Chem , vol.277 , Issue.11 , pp. 8928-8933
    • Suaud, L.1    Li, J.2    Jiang, Q.3
  • 30
    • 0036665609 scopus 로고    scopus 로고
    • Evidence of CFTR function in cystic fibrosis after systemic administration of 4- phenylbutyrate
    • Zeitlin P, Diener-West M, Rubenstein R, et al. Evidence of CFTR function in cystic fibrosis after systemic administration of 4- phenylbutyrate. Mol Ther 2002; 6: 119-126.
    • (2002) Mol Ther , vol.6 , pp. 119-126
    • Zeitlin, P.1    Diener-West, M.2    Rubenstein, R.3
  • 31
    • 11144355340 scopus 로고    scopus 로고
    • Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects
    • Egan M, Pearson M, Weiner SA, et al. Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects. Science 2004; 304: 600-602.
    • (2004) Science , vol.304 , pp. 600-602
    • Egan, M.1    Pearson, M.2    Weiner, S.A.3
  • 32
    • 6044268094 scopus 로고    scopus 로고
    • Curcumin does not stimulate cAMP-mediated chloride transport in cystic fibrosis airway epithelial cells
    • Dragomir A, Bjorstad J, Hjelte L, et al. Curcumin does not stimulate cAMP-mediated chloride transport in cystic fibrosis airway epithelial cells. Biochem Biophys Res Commun 2004; 322: 447-451.
    • (2004) Biochem Biophys Res Commun , vol.322 , pp. 447-451
    • Dragomir, A.1    Bjorstad, J.2    Hjelte, L.3
  • 33
    • 4644360693 scopus 로고    scopus 로고
    • Evidence against the rescue of defective DeltaF508-CFTR cellular processing by curcumin in cell culture and mouse models
    • Song Y, Sonawane N, Salinas D, et al. Evidence against the rescue of defective DeltaF508-CFTR cellular processing by curcumin in cell culture and mouse models. J Biol Chem 2004; 279: 40629-40623.
    • (2004) J Biol Chem , vol.279 , pp. 40623-40629
    • Song, Y.1    Sonawane, N.2    Salinas, D.3
  • 34
    • 33947543364 scopus 로고    scopus 로고
    • Curcumin opens cystic fibrosis transmembrane conductance regulator channels by a novel mechanism that requires neither ATP binding nor dimerization of the nucleotide-binding domains
    • Wang W, Bernard K, Li G, et al. Curcumin opens cystic fibrosis transmembrane conductance regulator channels by a novel mechanism that requires neither ATP binding nor dimerization of the nucleotide-binding domains. J Biol Chem 2006; 282: 4533-4544.
    • (2006) J Biol Chem , vol.282 , pp. 4533-4544
    • Wang, W.1    Bernard, K.2    Li, G.3
  • 35
    • 24644464284 scopus 로고    scopus 로고
    • Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by highthroughput screening
    • Pedemonte N, Lukacs G, Du K, et al. Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by highthroughput screening. J Clin Invest 2001; 115: 2564-2571.
    • (2001) J Clin Invest , vol.115 , pp. 2564-2571
    • Pedemonte, N.1    Lukacs, G.2    Du, K.3
  • 36
    • 33744831154 scopus 로고    scopus 로고
    • Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
    • Van Goor F, Straley K, Cao D, et al. Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am J Physiol Cell Physiol 2006; 290: L1117-L1130.
    • (2006) Am J Physiol Cell Physiol , vol.290
    • van Goor, F.1    Straley, K.2    Cao, D.3
  • 37
    • 84856507079 scopus 로고    scopus 로고
    • http://investor.shareholder.com/vrtx/releasedetail.cfm?ReleaseID=442429
  • 38
    • 0027380236 scopus 로고
    • The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells
    • Lukacs GL, Chang XB, Bear C, et al The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells. J Biol Chem 1993; 268[29]: 21592-8.
    • (1993) J Biol Chem , vol.268 , Issue.29 , pp. 21592-21598
    • Lukacs, G.L.1    Chang, X.B.2    Bear, C.3
  • 39
    • 78549279173 scopus 로고    scopus 로고
    • Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation
    • Accurso FJ, Rowe SM, Clancy JP, et al. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med 2010; 363[21]: 1991-2003.
    • (2010) N Engl J Med , vol.363 , Issue.21 , pp. 1991-2003
    • Accurso, F.J.1    Rowe, S.M.2    Clancy, J.P.3
  • 40
    • 80455162465 scopus 로고    scopus 로고
    • A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
    • VX08-770-102 Study Group
    • Ramsey BW, Davies J, McElvaney NG, et al. VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011; 365(18): 1663-72.
    • (2011) N Engl J Med , vol.365 , Issue.18 , pp. 1663-1672
    • Ramsey, B.W.1    Davies, J.2    McElvaney, N.G.3
  • 41
    • 77953122968 scopus 로고    scopus 로고
    • DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers
    • Rowe SM, Pyle LC, Jurkevante A, et al. DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers. Pulm Pharmacol Ther 2010; 23[4]: 268-78.
    • (2010) Pulm Pharmacol Ther , vol.23 , Issue.4 , pp. 268-278
    • Rowe, S.M.1    Pyle, L.C.2    Jurkevante, A.3
  • 42
    • 0025874599 scopus 로고
    • Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis
    • Knowles M, Clarke L, Boucher R. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N Engl J Med 1991; 325: 533-538.
    • (1991) N Engl J Med , vol.325 , pp. 533-538
    • Knowles, M.1    Clarke, L.2    Boucher, R.3
  • 43
    • 0035880177 scopus 로고    scopus 로고
    • Effect of aerosolized uridine 5'- triphosphate on mucociliary clearance in mild chronic bronchitis
    • Bennett W, Zeman K, Foy C, et al. Effect of aerosolized uridine 5'- triphosphate on mucociliary clearance in mild chronic bronchitis. Am J Respir Crit Care Med 2001; 164: 302-306.
    • (2001) Am J Respir Crit Care Med , vol.164 , pp. 302-306
    • Bennett, W.1    Zeman, K.2    Foy, C.3
  • 44
    • 20144363443 scopus 로고    scopus 로고
    • Cystic Fibrosis Foundation Therapeutics Development Network. Safety and tolerability of denufosol tetrasodium inhalation solution, a novel P2Y2 receptor agonist: Results of a phase 1/phase 2 multicenter study in mild to moderate cystic fibrosis
    • Deterding R, Retsch-Bogart G, Milgram L, et al. Cystic Fibrosis Foundation Therapeutics Development Network. Safety and tolerability of denufosol tetrasodium inhalation solution, a novel P2Y2 receptor agonist: results of a phase 1/phase 2 multicenter study in mild to moderate cystic fibrosis. Pediatr Pulmonol 2005; 39: 339-348.
    • (2005) Pediatr Pulmonol , vol.39 , pp. 339-348
    • Deterding, R.1    Retsch-Bogart, G.2    Milgram, L.3
  • 45
    • 34547949974 scopus 로고    scopus 로고
    • Phase Randomized Safety and Efficacy Trial of Nebulized Denufosol Tetrasodium in Cystic Fibrosis
    • Deterding R, Lavange L, Engels J, et al. Phase Randomized Safety and Efficacy Trial of Nebulized Denufosol Tetrasodium in Cystic Fibrosis. Am J Respir Crit Care Med 2007; 176[4]: 362-9
    • (2007) Am J Respir Crit Care Med , vol.176 , Issue.4 , pp. 362-369
    • Deterding, R.1    Lavange, L.2    Engels, J.3
  • 46
    • 79952211206 scopus 로고    scopus 로고
    • Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function
    • Accurso FJ, Moss RB, Wilmott RW, et al. Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function. Am J Respir Crit Care Med 2011; 183[5]: 627-34.
    • (2011) Am J Respir Crit Care Med , vol.183 , Issue.5 , pp. 627-634
    • Accurso, F.J.1    Moss, R.B.2    Wilmott, R.W.3
  • 47
    • 57149104917 scopus 로고    scopus 로고
    • Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in betaENaC-overexpressing mice
    • Zhou Z, Treis D, Schubert SC, et al. Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in betaENaC-overexpressing mice. Am J Respir Crit Care Med 2008; 178[12]: 1245-56.
    • (2008) Am J Respir Crit Care Med , vol.178 , Issue.12 , pp. 1245-1256
    • Zhou, Z.1    Treis, D.2    Schubert, S.C.3
  • 48
    • 0037324136 scopus 로고    scopus 로고
    • Pharmacokinetics and tissue distribution of the nonadecapeptide Moli1901 in rats and mice
    • McNulty MJ, Hutabarat RH, Findlay JW, et al. Pharmacokinetics and tissue distribution of the nonadecapeptide Moli1901 in rats and mice. Xenobiotica 2003; 33[2]: 197-210
    • (2003) Xenobiotica , vol.33 , Issue.2 , pp. 197-210
    • McNulty, M.J.1    Hutabarat, R.H.2    Findlay, J.W.3
  • 49
    • 20644458109 scopus 로고    scopus 로고
    • Determination of the tissue distribution and excretion by accelerator mass spectrometry of the nonadecapeptide 14C-Moli1901 in beagle dogs after intratracheal instillation
    • Rickert DE, Dingley K, Ubick E, et al. Determination of the tissue distribution and excretion by accelerator mass spectrometry of the nonadecapeptide 14C-Moli1901 in beagle dogs after intratracheal instillation. Chem Biol Interact 2005; 155[1-2]: 55-61.
    • (2005) Chem Biol Interact , vol.155 , Issue.1-2 , pp. 55-61
    • Rickert, D.E.1    Dingley, K.2    Ubick, E.3
  • 50
    • 1642493889 scopus 로고    scopus 로고
    • A phase I trial of intranasal Moli1901 for cystic fibrosis
    • Zeitlin P, Boyle M, Guggino W. A phase I trial of intranasal Moli1901 for cystic fibrosis. Chest 2005; 25: 143-149.
    • (2005) Chest , vol.25 , pp. 143-149
    • Zeitlin, P.1    Boyle, M.2    Guggino, W.3
  • 52
    • 84856501598 scopus 로고    scopus 로고
    • www.clinicaltrials.gov/ct2/show/NCT00671736.
  • 53
    • 33750842131 scopus 로고    scopus 로고
    • Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis
    • Wang X, Venable J, LaPointe P, et al. Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis. Cell 2006; 127[4]: 803-15.
    • (2006) Cell , vol.127 , Issue.4 , pp. 803-815
    • Wang, X.1    Venable, J.2    Lapointe, P.3
  • 54
    • 0033581885 scopus 로고    scopus 로고
    • Activation of the epithelial Na+ channel [ENaC] requires CFTR Cl-channel function
    • Reddy M, Light MJ, Quinton P. Activation of the epithelial Na+ channel [ENaC] requires CFTR Cl-channel function. Nature 1999; 402: 301-304.
    • (1999) Nature , vol.402 , pp. 301-304
    • Reddy, M.1    Light, M.J.2    Quinton, P.3
  • 55
    • 2442718786 scopus 로고    scopus 로고
    • Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice
    • Mall M, Grubb B, Harkema J, et al. Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice. Nat Med 2004; 10: 487-493.
    • (2004) Nat Med , vol.10 , pp. 487-493
    • Mall, M.1    Grubb, B.2    Harkema, J.3
  • 56
    • 77957592219 scopus 로고    scopus 로고
    • Effect of neutrophil elastase and its inhibitor EPI-hNE4 on transepithelial sodium transport across normal and cystic fibrosis human nasal epithelial cells
    • Prulière-Escabasse V, Clerici C, Vuagniaux G, et al. Effect of neutrophil elastase and its inhibitor EPI-hNE4 on transepithelial sodium transport across normal and cystic fibrosis human nasal epithelial cells. Respir Res 2010; 11: 141.
    • (2010) Respir Res , vol.11 , pp. 141
    • Prulière-Escabasse, V.1    Clerici, C.2    Vuagniaux, G.3
  • 57
    • 0027520191 scopus 로고
    • Amiloride inhalation therapy in cystic fibrosis. Influence on ion content, hydration, and rheology of sputum
    • Tomkiewicz RP, App EM, Zayas JG, et al. Amiloride inhalation therapy in cystic fibrosis. Influence on ion content, hydration, and rheology of sputum. Am Rev Respir Dis 1993; 148[4 Pt 1]: 1002-7.
    • (1993) Am Rev Respir Dis , vol.148 , Issue.4 Pt 1 , pp. 1002-1007
    • Tomkiewicz, R.P.1    App, E.M.2    Zayas, J.G.3
  • 58
    • 0027486593 scopus 로고
    • No added benefit from nebulized amiloride in patients with cystic fibrosis
    • Graham A, Hasani A, Alton EW, et al. No added benefit from nebulized amiloride in patients with cystic fibrosis. Eur Respir J 1993; 6[9]: 1243-8.
    • (1993) Eur Respir J , vol.6 , Issue.9 , pp. 1243-1248
    • Graham, A.1    Hasani, A.2    Alton, E.W.3
  • 59
    • 0028787073 scopus 로고
    • Nebulised amiloride in respiratory exacerbations of cystic fibrosis: A randomised controlled trial
    • Bowler IM, Kelman B, Worthington D, et al. Nebulised amiloride in respiratory exacerbations of cystic fibrosis: a randomised controlled trial. Arch Dis Child 1995; 73[5]: 427-30.
    • (1995) Arch Dis Child , vol.73 , Issue.5 , pp. 427-430
    • Bowler, I.M.1    Kelman, B.2    Worthington, D.3
  • 60
    • 0034047238 scopus 로고    scopus 로고
    • French multicenter randomized double-blind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients. The Amiloride-AFLM Collaborative Study Group
    • Pons G, Marchand MC, d'Athis P, et al. French multicenter randomized double-blind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients. The Amiloride-AFLM Collaborative Study Group. Pediatr Pulmonol 2000; 30[1]: 25-31.
    • (2000) Pediatr Pulmonol , vol.30 , Issue.1 , pp. 25-31
    • Pons, G.1    Marchand, M.C.2    D'Athis, P.3
  • 61
    • 57149094665 scopus 로고    scopus 로고
    • Amiloride: Still a viable treatment option in cystic fibrosis?
    • Ratjen F, Bush A. Amiloride: still a viable treatment option in cystic fibrosis? Am J Respir Crit Care Med 2008; 178[12]: 1191-2.
    • (2008) Am J Respir Crit Care Med , vol.178 , Issue.12 , pp. 1191-1192
    • Ratjen, F.1    Bush, A.2
  • 63
    • 30944452384 scopus 로고    scopus 로고
    • Mucus clearance and lung function in cystic fibrosis with hypertonic saline
    • Donaldson S, Bennett W, Zeman K, et al. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med 2006; 354: 250.
    • (2006) N Engl J Med , vol.354 , pp. 250
    • Donaldson, S.1    Bennett, W.2    Zeman, K.3
  • 64
    • 33748744717 scopus 로고    scopus 로고
    • Hypertonic saline therapy in cystic fibrosis: Evidence against the proposed mechanism involving aquaporins
    • Levin M, Sullivan S, Nielson D, et al. Hypertonic saline therapy in cystic fibrosis: Evidence against the proposed mechanism involving aquaporins. J Biol Chem 2006; 281: 25803-12.
    • (2006) J Biol Chem , vol.281 , pp. 25803-25812
    • Levin, M.1    Sullivan, S.2    Nielson, D.3
  • 65
    • 10044235073 scopus 로고    scopus 로고
    • Evaluation of second generation amiloride analogs as therapy for cystic fibrosis lung disease
    • Hirsh AJ, Sabater JR, Zamurs A, et al. Evaluation of second generation amiloride analogs as therapy for cystic fibrosis lung disease. J Pharmacol Exp Ther 2004; 311[3]: 929-38.
    • (2004) J Pharmacol Exp Ther , vol.311 , Issue.3 , pp. 929-938
    • Hirsh, A.J.1    Sabater, J.R.2    Zamurs, A.3
  • 66
    • 33745838632 scopus 로고    scopus 로고
    • Design, synthesis, and structureactivity relationships of novel 2-substituted pyrazinoylguanidine epithelial sodium channel blockers: Drugs for cystic fibrosis and chronic bronchitis
    • Hirsh A, Molino B, Zhang J, et al. Design, synthesis, and structureactivity relationships of novel 2-substituted pyrazinoylguanidine epithelial sodium channel blockers: drugs for cystic fibrosis and chronic bronchitis. J Med Chem 2006; 49: 4098-4115.
    • (2006) J Med Chem , vol.49 , pp. 4098-4115
    • Hirsh, A.1    Molino, B.2    Zhang, J.3
  • 67
    • 30944452609 scopus 로고    scopus 로고
    • Restoring Airway Surface Liquid in Cystic Fibrosis
    • Ratjen F. Restoring Airway Surface Liquid in Cystic Fibrosis. N Engl J Med 2006; 354: 291-293.
    • (2006) N Engl J Med , vol.354 , pp. 291-293
    • Ratjen, F.1
  • 68
    • 0030784262 scopus 로고    scopus 로고
    • Effect of increasing doses of hypertonic saline on mucociliary clearance in patients with cystic fibrosis
    • Robinson M, Hemming A, Regnis J, et al. Effect of increasing doses of hypertonic saline on mucociliary clearance in patients with cystic fibrosis. Thorax 1997; 52: 900-903.
    • (1997) Thorax , vol.52 , pp. 900-903
    • Robinson, M.1    Hemming, A.2    Regnis, J.3
  • 69
    • 0035922668 scopus 로고    scopus 로고
    • Comparison of hypertonic saline and alternate-day or daily recombinant human deoxyribonuclease in children with cystic fibrosis: A randomised trial
    • Suri R, Metcalfe C, Lees B, et al. Comparison of hypertonic saline and alternate-day or daily recombinant human deoxyribonuclease in children with cystic fibrosis: a randomised trial. Lancet 2001; 358[9290]: 1316-21.
    • (2001) Lancet , vol.358 , Issue.9290 , pp. 1316-1321
    • Suri, R.1    Metcalfe, C.2    Lees, B.3
  • 70
    • 0036123981 scopus 로고    scopus 로고
    • Hypertonic saline and recombinant human DNase: A randomised cross-over pilot study in patients with cystic fibrosis
    • Ballmann M, von der Hardt H. Hypertonic saline and recombinant human DNase: a randomised cross-over pilot study in patients with cystic fibrosis. J Cyst Fibros 2002; 1[1]: 35-7.
    • (2002) J Cyst Fibros , vol.1 , Issue.1 , pp. 35-37
    • Ballmann, M.1    von der Hardt, H.2
  • 71
  • 72
    • 30944466084 scopus 로고    scopus 로고
    • National Hypertonic Saline in Cystic Fibrosis [NHSCF] Study Group. A controlled trial of longterm inhaled hypertonic saline in patients with cystic fibrosis
    • Elkins M, Robinson M, Rose B, et al. National Hypertonic Saline in Cystic Fibrosis [NHSCF] Study Group. A controlled trial of longterm inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006; 354: 229-240.
    • (2006) N Engl J Med , vol.354 , pp. 229-240
    • Elkins, M.1    Robinson, M.2    Rose, B.3
  • 73
    • 70349083003 scopus 로고    scopus 로고
    • Nebulised hypertonic saline for cystic fibrosis
    • Wark P, McDonald VM. Nebulised hypertonic saline for cystic fibrosis. Cochrane Database Syst Rev 2009; [2]: CD001506.
    • (2009) Cochrane Database Syst Rev , Issue.2
    • Wark, P.1    McDonald, V.M.2
  • 74
    • 77951988740 scopus 로고    scopus 로고
    • Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function
    • Amin R, Subbarao P, Jabar A, et al. Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function. Thorax 2010; 65[5]: 379-83.
    • (2010) Thorax , vol.65 , Issue.5 , pp. 379-383
    • Amin, R.1    Subbarao, P.2    Jabar, A.3
  • 75
    • 34247880282 scopus 로고    scopus 로고
    • Pilot study of safety and tolerability of inhaled hypertonic saline in infants with cystic fibrosis
    • Subbarao P, Balkovec S, Solomon M, et al. Pilot study of safety and tolerability of inhaled hypertonic saline in infants with cystic fibrosis. Pediatr Pulmonol 2007; 42: 471-476.
    • (2007) Pediatr Pulmonol , vol.42 , pp. 471-476
    • Subbarao, P.1    Balkovec, S.2    Solomon, M.3
  • 76
    • 57349158832 scopus 로고    scopus 로고
    • Safety and tolerability of inhaled hypertonic saline in young children with cystic fibrosis
    • Dellon EP, Donaldson SH, Johnson R, et al. Safety and tolerability of inhaled hypertonic saline in young children with cystic fibrosis. Pediatr Pulmonol 2008; 43[11]: 1100-6.
    • (2008) Pediatr Pulmonol , vol.43 , Issue.11 , pp. 1100-1106
    • Dellon, E.P.1    Donaldson, S.H.2    Johnson, R.3
  • 77
    • 79959828264 scopus 로고    scopus 로고
    • Inhaled hypertonic saline in infants and toddlers with cystic fibrosis: Short-term tolerability, adherence, and safety
    • Rosenfeld M, Davis S, Brumback L, et al. Inhaled hypertonic saline in infants and toddlers with cystic fibrosis: short-term tolerability, adherence, and safety. Pediatr Pulmonol 2011; 46(7): 666-71.
    • (2011) Pediatr Pulmonol , vol.46 , Issue.7 , pp. 666-671
    • Rosenfeld, M.1    Davis, S.2    Brumback, L.3
  • 78
    • 84856507081 scopus 로고    scopus 로고
    • NCT00709280
    • www.clinicaltrials.gov:: NCT00709280
  • 79
    • 57349176546 scopus 로고    scopus 로고
    • Bronchial provocation testing with dry powder mannitol in children with cystic fibrosis
    • Minasian C, Wallis C, Metcalfe C, et al. Bronchial provocation testing with dry powder mannitol in children with cystic fibrosis. Pediatr Pulmonol 2008; 43[11]: 1078-84.
    • (2008) Pediatr Pulmonol , vol.43 , Issue.11 , pp. 1078-1084
    • Minasian, C.1    Wallis, C.2    Metcalfe, C.3
  • 80
    • 77950787429 scopus 로고    scopus 로고
    • Inhaled mannitol improves the hydration and surface properties of sputum in patients with cystic fibrosis
    • Daviskas E, Anderson SD, Jaques A, et al. Inhaled mannitol improves the hydration and surface properties of sputum in patients with cystic fibrosis. Chest 2010; 137[4]: 861-8.
    • (2010) Chest , vol.137 , Issue.4 , pp. 861-868
    • Daviskas, E.1    Anderson, S.D.2    Jaques, A.3
  • 81
    • 78651452280 scopus 로고    scopus 로고
    • Inhaled mannitol in patients with cystic fibrosis: A randomised open-label dose response trial
    • Teper A, Jaques A, Charlton B. Inhaled mannitol in patients with cystic fibrosis: A randomised open-label dose response trial. J Cyst Fibros 2011; 10[1]: 1-8.
    • (2011) J Cyst Fibros , vol.10 , Issue.1 , pp. 1-8
    • Teper, A.1    Jaques, A.2    Charlton, B.3
  • 82
    • 44649166466 scopus 로고    scopus 로고
    • Inhaled mannitol improves lung function in cystic fibrosis
    • Jaques A, Daviskas E, Turton JA, et al. Inhaled mannitol improves lung function in cystic fibrosis. Chest 2008; 133[6]: 1388-96.
    • (2008) Chest , vol.133 , Issue.6 , pp. 1388-1396
    • Jaques, A.1    Daviskas, E.2    Turton, J.A.3
  • 83
    • 80355127141 scopus 로고    scopus 로고
    • Inhaled dry powder mannitol in cystic fibrosis: An efficacy and safety study
    • Bilton D, Robinson P, Cooper P, et al. Inhaled dry powder mannitol in cystic fibrosis: an efficacy and safety study. Eur Respir J 2011; 38(5): 1071-80.
    • (2011) Eur Respir J , vol.38 , Issue.5 , pp. 1071-1080
    • Bilton, D.1    Robinson, P.2    Cooper, P.3
  • 84
    • 33644620356 scopus 로고    scopus 로고
    • Respiratory tract mucin genes and mucin glycoproteins in health and disease
    • Rose MC, Voynow JA. Respiratory tract mucin genes and mucin glycoproteins in health and disease. Physiol Rev 2006; 86[1]: 245-28.
    • (2006) Physiol Rev , vol.86 , Issue.1 , pp. 245-248
    • Rose, M.C.1    Voynow, J.A.2
  • 85
    • 0001609666 scopus 로고
    • The in vitro reduction in viscosity of human tracheobronchial secretions by acetylcysteine
    • Sheffner AL, Medler EM, Jacobs LW, et al. The in vitro reduction in viscosity of human tracheobronchial secretions by acetylcysteine. Am Rev Respir Dis 1964; 90: 721-9.
    • (1964) Am Rev Respir Dis , vol.90 , pp. 721-729
    • Sheffner, A.L.1    Medler, E.M.2    Jacobs, L.W.3
  • 86
    • 0017111053 scopus 로고
    • A controlled trial of intermittent oral acetylcysteine in the long-term treatment of chronic bronchitis
    • Grassi C, Morandini GC. A controlled trial of intermittent oral acetylcysteine in the long-term treatment of chronic bronchitis. Eur J Clin Pharmacol 1976; 09[5-6]: 393-6.
    • (1976) Eur J Clin Pharmacol , vol.9 , Issue.5-6 , pp. 393-396
    • Grassi, C.1    Morandini, G.C.2
  • 87
    • 0028040727 scopus 로고
    • Orally administered Nacetylcysteine may improve general well-being in patients with mild chronic bronchitis
    • Hansen NC, Skriver A, Brorsen-Riis L et al. Orally administered Nacetylcysteine may improve general well-being in patients with mild chronic bronchitis. Respir Med 1994; 88[7]: 531-535.
    • (1994) Respir Med , vol.88 , Issue.7 , pp. 531-535
    • Hansen, N.C.1    Skriver, A.2    Brorsen-Riis, L.3
  • 88
    • 0037718518 scopus 로고    scopus 로고
    • N-acetylcysteine reduces the risk of re-hospitalisation among patients with chronic obstructive pulmonary disease
    • Gerrits CM, Herings RM, Leufkens HG, et al. N-acetylcysteine reduces the risk of re-hospitalisation among patients with chronic obstructive pulmonary disease. Eur Respir J 2003; 21[5]: 795-798.
    • (2003) Eur Respir J , vol.21 , Issue.5 , pp. 795-798
    • Gerrits, C.M.1    Herings, R.M.2    Leufkens, H.G.3
  • 89
    • 13144274594 scopus 로고    scopus 로고
    • Randomised, controlled trial of N-acetylcysteine for treatment of acute exacerbations of chronic obstructive pulmonary disease
    • Black PN, Morgan-Day A, McMillan TE, et al. Randomised, controlled trial of N-acetylcysteine for treatment of acute exacerbations of chronic obstructive pulmonary disease. BMC Pulm Med 2004; 4[1]: 13.
    • (2004) BMC Pulm Med , vol.4 , Issue.1 , pp. 13
    • Black, P.N.1    Morgan-Day, A.2    McMillan, T.E.3
  • 90
    • 20944433417 scopus 로고    scopus 로고
    • Effects of N-acetylcysteine on outcomes in chronic obstructive pulmonary disease [Bronchitis Randomized on NAC Cost-Utility Study, BRONCUS]: A randomised placebo-controlled trial
    • Decramer M, Rutten-van Molken M, Dekhuijzen PN et al. Effects of N-acetylcysteine on outcomes in chronic obstructive pulmonary disease [Bronchitis Randomized on NAC Cost-Utility Study, BRONCUS]: a randomised placebo-controlled trial. Lancet 2005; 365[9470]: 1552-1560.
    • (2005) Lancet , vol.365 , Issue.9470 , pp. 1552-1560
    • Decramer, M.1    Rutten-Van, M.M.2    Dekhuijzen, P.N.3
  • 91
    • 0023096396 scopus 로고
    • No penetration of orally administered N-acetylcysteine into bronchoalveolar lavage fluid
    • Cotgreave IA, Eklund A, Larsson K, et al. No penetration of orally administered N-acetylcysteine into bronchoalveolar lavage fluid. Eur J Respir Dis 1987; 70[2]: 73-77.
    • (1987) Eur J Respir Dis , vol.70 , Issue.2 , pp. 73-77
    • Cotgreave, I.A.1    Eklund, A.2    Larsson, K.3
  • 92
    • 0026029981 scopus 로고
    • Cysteine and glutathione concentrations in plasma and bronchoalveolar lavage fluid after treatment with N-acetylcysteine
    • Bridgeman MM, Marsden M, MacNee W, et al. Cysteine and glutathione concentrations in plasma and bronchoalveolar lavage fluid after treatment with N-acetylcysteine. Thorax 1991; 46[1]: 39-42.
    • (1991) Thorax , vol.46 , Issue.1 , pp. 39-42
    • Bridgeman, M.M.1    Marsden, M.2    Macnee, W.3
  • 93
    • 33645229803 scopus 로고    scopus 로고
    • High-dose oral Nacetylcysteine, a glutathione prodrug, modulates inflammation in cystic fibrosis
    • Tirouvanziam R, Conrad CK, Bottiglieri T, et al. High-dose oral Nacetylcysteine, a glutathione prodrug, modulates inflammation in cystic fibrosis. Proc Natl Acad Sci USA 2006; 103[12]: 4628-4633.
    • (2006) Proc Natl Acad Sci USA , vol.103 , Issue.12 , pp. 4628-4633
    • Tirouvanziam, R.1    Conrad, C.K.2    Bottiglieri, T.3
  • 94
    • 0025647882 scopus 로고
    • Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum
    • Shak S, Capon DJ, Hellmiss R, et al. Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum. Proc Natl Acad Sci USA 1990; 87[23]: 9188-92.
    • (1990) Proc Natl Acad Sci USA , vol.87 , Issue.23 , pp. 9188-9192
    • Shak, S.1    Capon, D.J.2    Hellmiss, R.3
  • 95
    • 0030027655 scopus 로고    scopus 로고
    • In vivo effects of recombinant human DNase I on sputum in patients with cystic fibrosis
    • Shah PL, Scott SF, Knight RA, et al. In vivo effects of recombinant human DNase I on sputum in patients with cystic fibrosis. Thorax 1996; 51[2]: 119-25.
    • (1996) Thorax , vol.51 , Issue.2 , pp. 119-125
    • Shah, P.L.1    Scott, S.F.2    Knight, R.A.3
  • 96
    • 0026602608 scopus 로고
    • Recombinant human DNase inhalation in normal subjects and patients with cystic fibrosis. A phase 1 study
    • Aitken ML, Burke W, McDonald G, et al. Recombinant human DNase inhalation in normal subjects and patients with cystic fibrosis. A phase 1 study. JAMA 1992; 267[14]: 1947-51.
    • (1992) JAMA , vol.267 , Issue.14 , pp. 1947-1951
    • Aitken, M.L.1    Burke, W.2    McDonald, G.3
  • 97
    • 0028129568 scopus 로고
    • Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis
    • Fuchs HJ, Borowitz DS, Christiansen DH, et al. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. N Engl J Med 1994; 331: 637-42.
    • (1994) N Engl J Med , vol.331 , pp. 637-642
    • Fuchs, H.J.1    Borowitz, D.S.2    Christiansen, D.H.3
  • 98
    • 0029921953 scopus 로고    scopus 로고
    • Effects of 12-week administration of dornase alfa in patients with advanced cystic fibrosis lung disease. Pulmozyme Study Group
    • McCoy K, Hamilton S, Johnson C. Effects of 12-week administration of dornase alfa in patients with advanced cystic fibrosis lung disease. Pulmozyme Study Group. Chest 1996; 110[4]: 889-5.
    • (1996) Chest , vol.110 , Issue.4 , pp. 889-895
    • McCoy, K.1    Hamilton, S.2    Johnson, C.3
  • 99
    • 8944262191 scopus 로고    scopus 로고
    • Aerosolized recombinant human DNase in hospitalized cystic fibrosis patients with acute pulmonary exacerbations
    • Wilmott RW, Amin RS, Colin AA et al. Aerosolized recombinant human DNase in hospitalized cystic fibrosis patients with acute pulmonary exacerbations. Am J Respir Crit Care Med 1996; 153[6 Pt 1]: 1914-7.
    • (1996) Am J Respir Crit Care Med , vol.153 , Issue.6 Pt 1 , pp. 1914-1917
    • Wilmott, R.W.1    Amin, R.S.2    Colin, A.A.3
  • 100
    • 0035666058 scopus 로고    scopus 로고
    • A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities
    • Quan JM, Tiddens HA, Sy JP, et al. A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. J Pediatr 2001; 139: 813-20.
    • (2001) J Pediatr , vol.139 , pp. 813-820
    • Quan, J.M.1    Tiddens, H.A.2    Sy, J.P.3
  • 102
    • 79953287790 scopus 로고    scopus 로고
    • The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis
    • Amin R, Subbarao P, Lou W, et al. The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis. Eur Respir J 2011; 37[4]: 806-12.
    • (2011) Eur Respir J , vol.37 , Issue.4 , pp. 806-812
    • Amin, R.1    Subbarao, P.2    Lou, W.3
  • 103
    • 10344250594 scopus 로고    scopus 로고
    • Effect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosis
    • Paul K, Rietschel E, Ballmann M et al. Effect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosis. Am J Respir Crit Care Med 2004; 169[6]: 719-25.
    • (2004) Am J Respir Crit Care Med , vol.169 , Issue.6 , pp. 719-725
    • Paul, K.1    Rietschel, E.2    Ballmann, M.3
  • 104
    • 84856421613 scopus 로고    scopus 로고
    • New Treatments for Emerging Cystic Fibrosis Pathogens other than Pseudomonas
    • Waters V, New Treatments for Emerging Cystic Fibrosis Pathogens other than Pseudomonas. Curr Pharm Des 2012; 18(5): 696-725.
    • (2012) Curr Pharm Des , vol.18 , Issue.5 , pp. 696-725
    • Waters, V.1
  • 105
    • 84856455714 scopus 로고    scopus 로고
    • New developments in inhaled antibiotics for the treatment of
    • Hofmann T. New developments in inhaled antibiotics for the treatment of Pseudomonas aeruginosa. 2012; 18(5): 683-95.
    • (2012) Pseudomonas Aeruginosa , vol.18 , Issue.5 , pp. 683-695
    • Hofmann, T.1
  • 106
    • 70350449606 scopus 로고    scopus 로고
    • The approach to Pseudomonas aeruginosa in cystic fibrosis
    • Bendiak GN, Ratjen F. The approach to Pseudomonas aeruginosa in cystic fibrosis. Semin Respir Crit Care Med 2009; 30[5]: 587-95.
    • (2009) Semin Respir Crit Care Med , vol.30 , Issue.5 , pp. 587-595
    • Bendiak, G.N.1    Ratjen, F.2
  • 107
    • 0028920643 scopus 로고
    • Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface
    • Imundo L, Barasch J, Prince A, et al. Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. Proc Natl Acad Sci USA 1995; 92: 3019-23.
    • (1995) Proc Natl Acad Sci USA , vol.92 , pp. 3019-3023
    • Imundo, L.1    Barasch, J.2    Prince, A.3
  • 108
    • 0030021470 scopus 로고    scopus 로고
    • Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections
    • Pier GB, Grout M, Zaidi TS, et al. Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections. Science 1996; 271: 64-7.
    • (1996) Science , vol.271 , pp. 64-67
    • Pier, G.B.1    Grout, M.2    Zaidi, T.S.3
  • 109
    • 0029870085 scopus 로고    scopus 로고
    • Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid
    • Smith JJ, Travis SM, Greenberg EP, et al. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell 1996; 85: 229-36.
    • (1996) Cell , vol.85 , pp. 229-236
    • Smith, J.J.1    Travis, S.M.2    Greenberg, E.P.3
  • 110
    • 60249088927 scopus 로고    scopus 로고
    • Respiratory pathogens in children with and without respiratory symptoms
    • van der Zalm MM, van Ewijk BE, Wilbrink B, et al. Respiratory pathogens in children with and without respiratory symptoms. J Pediatr 2009; 154[3]: 396-400
    • (2009) J Pediatr , vol.154 , Issue.3 , pp. 396-400
    • van der Zalm, M.M.1    van Ewijk, B.E.2    Wilbrink, B.3
  • 111
    • 0026831341 scopus 로고
    • Mucoid Pseudomonas aeruginosa is a marker of poor survival in cystic fibrosis
    • Henry RL, Mellis CM, Petrovic L. Mucoid Pseudomonas aeruginosa is a marker of poor survival in cystic fibrosis. Pediatr Pulmonol 1992; 12[3]: 158-61.
    • (1992) Pediatr Pulmonol , vol.12 , Issue.3 , pp. 158-161
    • Henry, R.L.1    Mellis, C.M.2    Petrovic, L.3
  • 112
    • 0031661390 scopus 로고    scopus 로고
    • Long-term follow up of changes in FEV1 and treatment intensity during Pseudomonas aeruginosa colonisation in patients with cystic fibrosis
    • Sep
    • Ballmann M, Rabsch P, von der Hardt H. Long-term follow up of changes in FEV1 and treatment intensity during Pseudomonas aeruginosa colonisation in patients with cystic fibrosis. Thorax 1998 Sep; 53[9]: 732-7.
    • (1998) Thorax , vol.53 , Issue.9 , pp. 732-737
    • Ballmann, M.1    Rabsch, P.2    von der Hardt, H.3
  • 113
    • 0142043977 scopus 로고    scopus 로고
    • Pathophysiology and management of pulmonary infections in cystic fibrosis
    • Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med 2003; 168: 918-51.
    • (2003) Am J Respir Crit Care Med , vol.168 , pp. 918-951
    • Gibson, R.L.1    Burns, J.L.2    Ramsey, B.W.3
  • 114
    • 33750302487 scopus 로고    scopus 로고
    • Treatment of early Pseudomonas aeruginosa infection in patients with cystic fibrosis
    • Nov
    • Ratjen F. Treatment of early Pseudomonas aeruginosa infection in patients with cystic fibrosis. Curr Opin Pulm Med 2006 Nov; 12[6]: 428-32.
    • (2006) Curr Opin Pulm Med , vol.12 , Issue.6 , pp. 428-432
    • Ratjen, F.1
  • 115
    • 0025136898 scopus 로고
    • Mucoid Pseudomonas aeruginosa in cystic fibrosis: Mutations in the muc loci affect transcription of the algR and algD genes in response to environmental stimuli
    • Deretic V, Govan JR, Konyecsni WM, et al. Mucoid Pseudomonas aeruginosa in cystic fibrosis: mutations in the muc loci affect transcription of the algR and algD genes in response to environmental stimuli. Mol Microbiol 1990; 4: 189-96.
    • (1990) Mol Microbiol , vol.4 , pp. 189-196
    • Deretic, V.1    Govan, J.R.2    Konyecsni, W.M.3
  • 116
    • 0034641962 scopus 로고    scopus 로고
    • Quorum-sensing signals indicate that cystic fibrosis lungs are infected with bacterial biofilms
    • Singh PK, Schaefer AL, Parsek MR, et al. Quorum-sensing signals indicate that cystic fibrosis lungs are infected with bacterial biofilms. Nature 2000; 407: 762-4.
    • (2000) Nature , vol.407 , pp. 762-764
    • Singh, P.K.1    Schaefer, A.L.2    Parsek, M.R.3
  • 117
    • 0022503266 scopus 로고
    • Antibiotic activity in sputum
    • Levy J. Antibiotic activity in sputum. J Pediatr 1986; 108: 841-846.
    • (1986) J Pediatr , vol.108 , pp. 841-846
    • Levy, J.1
  • 118
    • 0034685940 scopus 로고    scopus 로고
    • High frequency of hypermutable Pseudomonas aeruginosa n cystic fibrosis lung infection
    • Oliver A, Canton R, Campo P, et al. High frequency of hypermutable Pseudomonas aeruginosa n cystic fibrosis lung infection. Science 2000; 288: 1251-4.
    • (2000) Science , vol.288 , pp. 1251-1254
    • Oliver, A.1    Canton, R.2    Campo, P.3
  • 120
    • 2642712510 scopus 로고    scopus 로고
    • Placebo-controlled double-blind randomized study of aerosolized tobramycin for early treatment of Pseudomonas aeruginosa colonisation in cystic fibrosis
    • Wiesemann HG, Steinkamp G, Ratjen F, et al. Placebo-controlled double-blind randomized study of aerosolized tobramycin for early treatment of Pseudomonas aeruginosa colonisation in cystic fibrosis. Pediatr Pulmonol 1998; 26: 88-92.
    • (1998) Pediatr Pulmonol , vol.26 , pp. 88-92
    • Wiesemann, H.G.1    Steinkamp, G.2    Ratjen, F.3
  • 121
    • 0035934545 scopus 로고    scopus 로고
    • Eradication of Pseudomonas aeruginosa with inhaled tobramycin in patients with cystic fibrosis
    • Ratjen F, Döring G, Nikolaizik W. Eradication of Pseudomonas aeruginosa with inhaled tobramycin in patients with cystic fibrosis. Lancet 2001; 358: 983-4.
    • (2001) Lancet , vol.358 , pp. 983-984
    • Ratjen, F.1    Döring, G.2    Nikolaizik, W.3
  • 122
    • 0025868478 scopus 로고
    • Prevention of chronic Pseudomonas aeruginosa colonisation by early treatment
    • Valerius NH, Koch C, Hoiby N. Prevention of chronic Pseudomonas aeruginosa colonisation by early treatment. Lancet 1991; 338: 725-6.
    • (1991) Lancet , vol.338 , pp. 725-726
    • Valerius, N.H.1    Koch, C.2    Hoiby, N.3
  • 123
    • 0034785372 scopus 로고    scopus 로고
    • Genotypic characterization of Pseudomonas aeruginosa strains recovered from patients with cystic fibrosis after initial and subsequent colonization
    • Munck A, Bonacorsi S, Mariani-Kurkdjian P, et al. Genotypic characterization of Pseudomonas aeruginosa strains recovered from patients with cystic fibrosis after initial and subsequent colonization. Pediatr Pulmonol 2001; 32: 288-92.
    • (2001) Pediatr Pulmonol , vol.32 , pp. 288-292
    • Munck, A.1    Bonacorsi, S.2    Mariani-Kurkdjian, P.3
  • 124
    • 0037445216 scopus 로고    scopus 로고
    • Cystic Fibrosis Therapeutics Development Network Study Group. Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis
    • Gibson RL, Emerson J, McNamara S, et al. Cystic Fibrosis Therapeutics Development Network Study Group. Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis. Am J Respir Crit Care Med 2003; 167: 841-9.
    • (2003) Am J Respir Crit Care Med , vol.167 , pp. 841-849
    • Gibson, R.L.1    Emerson, J.2    McNamara, S.3
  • 125
    • 77950914953 scopus 로고    scopus 로고
    • Treatment of early Pseudomonas aeruginosa infection in patients with cystic fibrosis: The ELITE trial
    • Ratjen F, Munck A, Kho P, et al. Treatment of early Pseudomonas aeruginosa infection in patients with cystic fibrosis: the ELITE trial. Thorax 2010; 65[4]: 286-91.
    • (2010) Thorax , vol.65 , Issue.4 , pp. 286-291
    • Ratjen, F.1    Munck, A.2    Kho, P.3
  • 126
    • 70449687936 scopus 로고    scopus 로고
    • Aminoglycoside therapy against Pseudomonas aeruginosa in cystic fibrosis: A review
    • Ratjen F, Brockhaus F, Angyalosi G. Aminoglycoside therapy against Pseudomonas aeruginosa in cystic fibrosis: a review. J Cyst Fibros 2009; 8[6]: 361-9.
    • (2009) J Cyst Fibros , vol.8 , Issue.6 , pp. 361-369
    • Ratjen, F.1    Brockhaus, F.2    Angyalosi, G.3
  • 127
    • 79955925867 scopus 로고    scopus 로고
    • The effect of early Pseudomonas aeruginosa treatment on lung function in pediatric cystic fibrosis
    • Feb
    • Amin R, Lam M, Dupuis A, et al. The effect of early Pseudomonas aeruginosa treatment on lung function in pediatric cystic fibrosis. Pediatr Pulmonol 2011 Feb 18.
    • (2011) Pediatr Pulmonol , pp. 18
    • Amin, R.1    Lam, M.2    Dupuis, A.3
  • 129
    • 84856501596 scopus 로고    scopus 로고
    • http://www.hta.ac.uk/1763.
  • 130
    • 84856495280 scopus 로고    scopus 로고
    • NCT01082367
    • www.clinicaltrials.gov: NCT01082367.
  • 131
    • 55549137086 scopus 로고    scopus 로고
    • Inhaled aztreonam lysine for chronic airway Pseudomonas aeruginosa in cystic fibrosis
    • McCoy KS, Quittner AL, Oermann CM, et al. Inhaled aztreonam lysine for chronic airway Pseudomonas aeruginosa in cystic fibrosis. Am J Respir Crit Care Med 2008; 178[9]: 921-8.
    • (2008) Am J Respir Crit Care Med , vol.178 , Issue.9 , pp. 921-928
    • McCoy, K.S.1    Quittner, A.L.2    Oermann, C.M.3
  • 132
    • 65949124667 scopus 로고    scopus 로고
    • Efficacy and safety of inhaled aztreonam lysine for airway pseudomonas in cystic fibrosis
    • Retsch-Bogart GZ, Quittner AL, Gibson RL, et al. Efficacy and safety of inhaled aztreonam lysine for airway pseudomonas in cystic fibrosis. Chest 2009; 135[5]: 1223-32.
    • (2009) Chest , vol.135 , Issue.5 , pp. 1223-1232
    • Retsch-Bogart, G.Z.1    Quittner, A.L.2    Gibson, R.L.3
  • 133
    • 2442526705 scopus 로고    scopus 로고
    • Clinically feasible biofilm susceptibility assay for isolates of Pseudomonas aeruginosa from patients with cystic fibrosis
    • Moskowitz SM, Foster JM, Emerson J, et al. Clinically feasible biofilm susceptibility assay for isolates of Pseudomonas aeruginosa from patients with cystic fibrosis. J Clin Microbiol 2004; 42[5]: 1915-22.
    • (2004) J Clin Microbiol , vol.42 , Issue.5 , pp. 1915-1922
    • Moskowitz, S.M.1    Foster, J.M.2    Emerson, J.3
  • 134
    • 67149133088 scopus 로고    scopus 로고
    • Azithromycin treatment alters gene expression in inflammatory, lipid metabolism, and cell cycle pathways in well-differentiated human airway epithelia
    • Ribeiro CM, Hurd H, Wu Y, et al. Azithromycin treatment alters gene expression in inflammatory, lipid metabolism, and cell cycle pathways in well-differentiated human airway epithelia. PLoS One 2009; 4[6]: e5806.
    • (2009) PLoS One , vol.4 , Issue.6
    • Ribeiro, C.M.1    Hurd, H.2    Wu, Y.3
  • 135
    • 0035991529 scopus 로고    scopus 로고
    • Use of modulators of airways inflammation in patients with CF
    • Ren CL. Use of modulators of airways inflammation in patients with CF. Clin Rev Allergy Immunol 2002; 23[1]: 29-39.
    • (2002) Clin Rev Allergy Immunol , vol.23 , Issue.1 , pp. 29-39
    • Ren, C.L.1
  • 136
    • 3142580825 scopus 로고    scopus 로고
    • Effects of a brief course of azithromycin on soluble cell adhesion molecules and markers of inflammation in survivors of an acute coronary syndrome: A double-blind, randomized, placebo-controlled study
    • Hillis GS, Pearson CV, Harding SA, et al. Effects of a brief course of azithromycin on soluble cell adhesion molecules and markers of inflammation in survivors of an acute coronary syndrome: A double-blind, randomized, placebo-controlled study. Am Heart J 2004; 148[1]: 72-9.
    • (2004) Am Heart J , vol.148 , Issue.1 , pp. 72-79
    • Hillis, G.S.1    Pearson, C.V.2    Harding, S.A.3
  • 137
    • 79251591304 scopus 로고    scopus 로고
    • A randomised controlled trial of azithromycin to prevent chronic rejection after lung transplantation
    • Vos R, Vanaudenaerde BM, Verleden SE, et al. A randomised controlled trial of azithromycin to prevent chronic rejection after lung transplantation. Eur Respir J 2011; 37[1]: 164-72.
    • (2011) Eur Respir J , vol.37 , Issue.1 , pp. 164-172
    • Vos, R.1    Vanaudenaerde, B.M.2    Verleden, S.E.3
  • 138
    • 0036185807 scopus 로고    scopus 로고
    • Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: A randomised trial
    • Wolter J, Seeney S, Bell S, et al. Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: a randomised trial. Thorax 2002; 57[3]: 212-216.
    • (2002) Thorax , vol.57 , Issue.3 , pp. 212-216
    • Wolter, J.1    Seeney, S.2    Bell, S.3
  • 139
    • 0037190575 scopus 로고    scopus 로고
    • Long term azithromycin in children with cystic fibrosis: A randomised, placebo-controlled crossover trial
    • Equi A, Balfour-Lynn IM, Bush A, et al. Long term azithromycin in children with cystic fibrosis: a randomised, placebo-controlled crossover trial. Lancet. 2002; 360[9338]: 978-984.
    • (2002) Lancet , vol.360 , Issue.9338 , pp. 978-984
    • Equi, A.1    Balfour-Lynn, I.M.2    Bush, A.3
  • 140
    • 0141816759 scopus 로고    scopus 로고
    • Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: A randomized controlled trial
    • Saiman L, Marshall BC, Mayer-Hamblett N, et al. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA 2003; 290[13]: 1749-1756.
    • (2003) JAMA , vol.290 , Issue.13 , pp. 1749-1756
    • Saiman, L.1    Marshall, B.C.2    Mayer-Hamblett, N.3
  • 141
    • 33749984264 scopus 로고    scopus 로고
    • Long term effects of azithromycin in patients with cystic fibrosis: A double blind, placebo controlled trial
    • Clement A, Tamalet A, Leroux E, et al. Long term effects of azithromycin in patients with cystic fibrosis: A double blind, placebo controlled trial. Thorax 2006; 61[10]: 895-902.
    • (2006) Thorax , vol.61 , Issue.10 , pp. 895-902
    • Clement, A.1    Tamalet, A.2    Leroux, E.3
  • 142
    • 77951887288 scopus 로고    scopus 로고
    • Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: A randomized controlled trial
    • Saiman L, Anstead M, Mayer-Hamblett N, et al. Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA 2010; 303[17]: 1707-15.
    • (2010) JAMA , vol.303 , Issue.17 , pp. 1707-1715
    • Saiman, L.1    Anstead, M.2    Mayer-Hamblett, N.3
  • 143
    • 84856495278 scopus 로고    scopus 로고
    • NCT01270074
    • www.clinicaltrials,.gov: NCT01270074.
  • 144
    • 28344451370 scopus 로고    scopus 로고
    • Lower airway inflammation in infants with cystic fibrosis detected by newborn screening
    • Armstrong DS, Hook SM, Jamsen KM, et al. Lower airway inflammation in infants with cystic fibrosis detected by newborn screening. Pediatr Pulmonol 2005; 40: 500-10.
    • (2005) Pediatr Pulmonol , vol.40 , pp. 500-510
    • Armstrong, D.S.1    Hook, S.M.2    Jamsen, K.M.3
  • 145
    • 0034816052 scopus 로고    scopus 로고
    • Activation of NF-kappaB in airway epithelial cells is dependent on CFTR trafficking and Clchannel function
    • Weber AJ, Soong G, Bryan R, et al. Activation of NF-kappaB in airway epithelial cells is dependent on CFTR trafficking and Clchannel function. Am J Physiol Lung Cell Mol Physiol 2001; 281: L71-8.
    • (2001) Am J Physiol Lung Cell Mol Physiol , vol.281
    • Weber, A.J.1    Soong, G.2    Bryan, R.3
  • 146
    • 0034997191 scopus 로고    scopus 로고
    • Proinflammatory cytokine responses to P. aeruginosa infection in human airway epithelial cell lines
    • Kube D, Sontich U, Fletcher D, et al. Proinflammatory cytokine responses to P. aeruginosa infection in human airway epithelial cell lines. Am J Physiol Lung Cell Mol Physiol 2001; 280: L493-502.
    • (2001) Am J Physiol Lung Cell Mol Physiol , vol.280
    • Kube, D.1    Sontich, U.2    Fletcher, D.3
  • 147
    • 0036840478 scopus 로고    scopus 로고
    • Inflammatory response in airway epithelial cells isolated from patients with cystic fibrosis
    • Aldallal N, McNaughton EE, Manzel LJ, et al. Inflammatory response in airway epithelial cells isolated from patients with cystic fibrosis. Am J Respir Crit Care Med 2002; 166[9]: 1248-56.
    • (2002) Am J Respir Crit Care Med , vol.166 , Issue.9 , pp. 1248-1256
    • Aldallal, N.1    McNaughton, E.E.2    Manzel, L.J.3
  • 148
    • 1442325340 scopus 로고    scopus 로고
    • Cytokine secretion by cystic fibrosis airway epithelial cells
    • Becker MN, Sauer MS, Muhlebach MS, et al. Cytokine secretion by cystic fibrosis airway epithelial cells. Am J Respir Crit Care Med 2004; 169: 645-53.
    • (2004) Am J Respir Crit Care Med , vol.169 , pp. 645-653
    • Becker, M.N.1    Sauer, M.S.2    Muhlebach, M.S.3
  • 149
    • 0033844485 scopus 로고    scopus 로고
    • Inflammation and infection in naive human cystic fibrosis airway grafts
    • Tirouvanziam R, de Bentzmann S, Hubeau C, et al. Inflammation and infection in naive human cystic fibrosis airway grafts. Am J Respir Cell Mol Biol 2000; 23: 121-7.
    • (2000) Am J Respir Cell Mol Biol , vol.23 , pp. 121-127
    • Tirouvanziam, R.1    de Bentzmann, S.2    Hubeau, C.3
  • 150
    • 0034999076 scopus 로고    scopus 로고
    • Quantitative analysis of inflammatory cells infiltrating the cystic fibrosis airway mucosa
    • Hubeau C, Lorenzato M, Couetil JP, et al. Quantitative analysis of inflammatory cells infiltrating the cystic fibrosis airway mucosa. Clin Exp Immunol 2001; 124[1]: 69-76.
    • (2001) Clin Exp Immunol , vol.124 , Issue.1 , pp. 69-76
    • Hubeau, C.1    Lorenzato, M.2    Couetil, J.P.3
  • 152
    • 0029372481 scopus 로고
    • Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis
    • Bonfield TL, Konstan MW, Burfeind P, et al. Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis. Am J Respir Cell Mol Biol 1995; 13: 257-61.
    • (1995) Am J Respir Cell Mol Biol , vol.13 , pp. 257-261
    • Bonfield, T.L.1    Konstan, M.W.2    Burfeind, P.3
  • 153
    • 39449133330 scopus 로고    scopus 로고
    • Airway inflammation in cystic fibrosis
    • Elizur A, Cannon CL, Ferkol TW. Airway inflammation in cystic fibrosis. Chest 2008; 133[2]: 489-95.
    • (2008) Chest , vol.133 , Issue.2 , pp. 489-495
    • Elizur, A.1    Cannon, C.L.2    Ferkol, T.W.3
  • 154
    • 77952974496 scopus 로고    scopus 로고
    • Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth
    • Stoltz DA, Meyerholz DK, Pezzulo AA, et al. Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth. Sci Transl Med 2010; 2[29]: 29ra31.
    • (2010) Sci Transl Med , vol.2 , Issue.29
    • Stoltz, D.A.1    Meyerholz, D.K.2    Pezzulo, A.A.3
  • 155
    • 77956379893 scopus 로고    scopus 로고
    • Disease phenotype of a ferret CFTRknockout model of cystic fibrosis
    • Sun X, Sui H, Fisher JT, et al. Disease phenotype of a ferret CFTRknockout model of cystic fibrosis. J Clin Invest 2010; 120[9]: 3149-60.
    • (2010) J Clin Invest , vol.120 , Issue.9 , pp. 3149-3160
    • Sun, X.1    Sui, H.2    Fisher, J.T.3
  • 156
    • 9144237586 scopus 로고    scopus 로고
    • Inflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosis
    • Ordonez CL, Henig NR, Mayer-Hamblett N, et al. Inflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosis. Am J Respir Crit Care Med 2003; 168: 1471-1475.
    • (2003) Am J Respir Crit Care Med , vol.168 , pp. 1471-1475
    • Ordonez, C.L.1    Henig, N.R.2    Mayer-Hamblett, N.3
  • 157
    • 0028969594 scopus 로고
    • A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis
    • Eigen H, Rosenstein BJ, Fitzsimmons S, et al. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. J Pediatr 1995, 126: 515-523.
    • (1995) J Pediatr , vol.126 , pp. 515-523
    • Eigen, H.1    Rosenstein, B.J.2    Fitzsimmons, S.3
  • 158
    • 0034704799 scopus 로고    scopus 로고
    • Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis
    • Lai HC, FitzSimmons SC, Allen DB et al. Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis. N Engl J Med 2000; 342: 851-9.
    • (2000) N Engl J Med , vol.342 , pp. 851-859
    • Lai, H.C.1    Fitzsimmons, S.C.2    Allen, D.B.3
  • 159
    • 35448992094 scopus 로고    scopus 로고
    • Oral corticosteroid therapy in cystic fibrosis patients hospitalized for pulmonary exacerbation: A pilot study
    • Dovey M, Aitken ML, Emerson J, et al. Oral corticosteroid therapy in cystic fibrosis patients hospitalized for pulmonary exacerbation: a pilot study. Chest 2007; 132[4]: 1212-8.
    • (2007) Chest , vol.132 , Issue.4 , pp. 1212-1218
    • Dovey, M.1    Aitken, M.L.2    Emerson, J.3
  • 160
    • 33745130682 scopus 로고    scopus 로고
    • Multicenter randomized controlled trial of withdrawal of inhaled corticosteroids in cystic fibrosis
    • Balfour-Lynn IM, Lees B, Hall P, et al. Multicenter randomized controlled trial of withdrawal of inhaled corticosteroids in cystic fibrosis. Am J Respir Crit Care Med 2006; 173[12]: 1356-62.
    • (2006) Am J Respir Crit Care Med , vol.173 , Issue.12 , pp. 1356-1362
    • Balfour-Lynn, I.M.1    Lees, B.2    Hall, P.3
  • 161
    • 0028914667 scopus 로고
    • Effect of high-dose ibuprofen in patients with cystic fibrosis
    • Konstan MW, Byard PJ, Hoppel CL, et al. Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med 1995, 332: 848-54.
    • (1995) N Engl J Med , vol.332 , pp. 848-854
    • Konstan, M.W.1    Byard, P.J.2    Hoppel, C.L.3
  • 162
    • 34548038663 scopus 로고    scopus 로고
    • High-dose ibuprofen in cystic fibrosis: Canadian safety and effectiveness trial
    • Lands LC, Milner R, Cantin AM, et al. High-dose ibuprofen in cystic fibrosis: Canadian safety and effectiveness trial. J Pediatr 2007; 151[3]: 249-54.
    • (2007) J Pediatr , vol.151 , Issue.3 , pp. 249-254
    • Lands, L.C.1    Milner, R.2    Cantin, A.M.3
  • 163
    • 34548023098 scopus 로고    scopus 로고
    • Non! to non-steroidal anti-inflammatory therapy for inflammatory lung disease in cystic fibrosis [at least at the moment]
    • Bush A, Davies J. Non! to non-steroidal anti-inflammatory therapy for inflammatory lung disease in cystic fibrosis [at least at the moment]. J Pediatr 2007; 151[3]: 228-30.
    • (2007) J Pediatr , vol.151 , Issue.3 , pp. 228-230
    • Bush, A.1    Davies, J.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.