메뉴 건너뛰기




Volumn 14, Issue 3, 2015, Pages 401-411

Efficacy and adverse effects of drugs used to treat adult cystic fibrosis

Author keywords

Antibiotics; Bronchodilators; Cystic fibrosis; Cystic fibrosis transmembrane conductance regulator modulators; New treatments

Indexed keywords

ANTIBIOTIC AGENT; ANTIINFLAMMATORY AGENT; AZITHROMYCIN; AZTREONAM; BRONCHODILATING AGENT; COLISTIMETHATE; CORTICOSTEROID; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DORNASE ALFA; IVACAFTOR; MANNITOL; MONTELUKAST; MUCOLYTIC AGENT; PANCREAS ENZYME; SODIUM CHLORIDE; TOBRAMYCIN; BIOLOGICAL MARKER;

EID: 84923330833     PISSN: 14740338     EISSN: 1744764X     Source Type: Journal    
DOI: 10.1517/14740338.2015.994503     Document Type: Review
Times cited : (7)

References (71)
  • 1
    • 84907343152 scopus 로고    scopus 로고
    • Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: Survival analysis of the cystic fibrosis foundation patient registry
    • MacKenzie T, Gifford AH, Sabadosa KA, et al. Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: survival analysis of the cystic fibrosis foundation patient registry. Ann Intern Med 2014;161(4):233-41
    • (2014) Ann Intern Med , vol.161 , Issue.4 , pp. 233-241
    • Mackenzie, T.1    Gifford, A.H.2    Sabadosa, K.A.3
  • 2
    • 79958104493 scopus 로고    scopus 로고
    • Pharmacological therapy for cystic fibrosis: From bench to bedside
    • Becq F, Mall MA, Sheppard DN, et al. Pharmacological therapy for cystic fibrosis: from bench to bedside. J Cyst Fibros 2011;10(Suppl 2):S129-45
    • (2011) J Cyst Fibros , vol.10 , pp. S129-S145
    • Becq, F.1    Mall, M.A.2    Sheppard, D.N.3
  • 3
    • 33745191871 scopus 로고    scopus 로고
    • What's new in CF airway inflammation: An update
    • Ratjen F. What's new in CF airway inflammation: an update. Paediatr Respir Rev 2006;7(Suppl 1):S70-2
    • (2006) Paediatr Respir Rev , vol.7 , pp. S70-S72
    • Ratjen, F.1
  • 4
    • 84871946692 scopus 로고    scopus 로고
    • Cystic fibrosis therapeutics: The road ahead
    • Hoffman LR, Ramsey BW. Cystic fibrosis therapeutics: the road ahead. Chest 2013;143(1):207-13
    • (2013) Chest , vol.143 , Issue.1 , pp. 207-213
    • Hoffman, L.R.1    Ramsey, B.W.2
  • 7
    • 66849142486 scopus 로고    scopus 로고
    • Aerosol antibiotics in cystic fibrosis
    • Geller DE. Aerosol antibiotics in cystic fibrosis. Respir Care 2009;54(5):658-70
    • (2009) Respir Care , vol.54 , Issue.5 , pp. 658-670
    • Geller, D.E.1
  • 8
    • 0027287651 scopus 로고
    • Efficacy of aerosolized tobramycin in patients with cystic fibrosis
    • Ramsey BW, Dorkin HL, Eisenberg JD, et al. Efficacy of aerosolized tobramycin in patients with cystic fibrosis. N Engl J Med 1993;328(24):1740-6
    • (1993) N Engl J Med , vol.328 , Issue.24 , pp. 1740-1746
    • Ramsey, B.W.1    Dorkin, H.L.2    Eisenberg, J.D.3
  • 9
    • 84911956623 scopus 로고    scopus 로고
    • Inhaled versus nebulised tobramycin: A real world comparison in adult cystic fibrosis (CF)
    • Harrison MJ, McCarthy M, Fleming C, et al. Inhaled versus nebulised tobramycin: A real world comparison in adult cystic fibrosis (CF). J Cyst Fibros 2014;13(6):692-8
    • (2014) J Cyst Fibros , vol.13 , Issue.6 , pp. 692-698
    • Harrison, M.J.1    McCarthy, M.2    Fleming, C.3
  • 10
    • 84880473914 scopus 로고    scopus 로고
    • Tobramycin inhalation powder manufactured by improved process in cystic fibrosis: The randomized EDIT trial
    • Galeva I, Konstan MW, Higgins M, et al. Tobramycin inhalation powder manufactured by improved process in cystic fibrosis: the randomized EDIT trial. Curr Med Res Opin 2013;29(8):947-56
    • (2013) Curr Med Res Opin , vol.29 , Issue.8 , pp. 947-956
    • Galeva, I.1    Konstan, M.W.2    Higgins, M.3
  • 12
    • 78651412529 scopus 로고    scopus 로고
    • Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: The EAGER trial
    • Konstan MW, Flume PA, Kappler M, et al. Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: the EAGER trial. J Cyst Fibros 2011;10(1):54-61
    • (2011) J Cyst Fibros , vol.10 , Issue.1 , pp. 54-61
    • Konstan, M.W.1    Flume, P.A.2    Kappler, M.3
  • 13
    • 38349058630 scopus 로고    scopus 로고
    • A phase 2 study of aztreonam lysine for inhalation to treat patients with cystic fibrosis and Pseudomonas aeruginosa infection
    • Retsch-Bogart GZ, Burns JL, Otto KL, et al. A phase 2 study of aztreonam lysine for inhalation to treat patients with cystic fibrosis and Pseudomonas aeruginosa infection. Pediatr Pulmonol 2008;43(1):47-58
    • (2008) Pediatr Pulmonol , vol.43 , Issue.1 , pp. 47-58
    • Retsch-Bogart, G.Z.1    Burns, J.L.2    Otto, K.L.3
  • 14
    • 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
  • 15
    • 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
  • 16
    • 78449294500 scopus 로고    scopus 로고
    • An 18-month study of the safety and efficacy of repeated courses of inhaled aztreonam lysine in cystic fibrosis
    • Oermann CM, Retsch-Bogart GZ, Quittner AL, et al. An 18-month study of the safety and efficacy of repeated courses of inhaled aztreonam lysine in cystic fibrosis. Pediatr Pulmonol 2010;45(11):1121-34
    • (2010) Pediatr Pulmonol , vol.45 , Issue.11 , pp. 1121-1134
    • Oermann, C.M.1    Retsch-Bogart, G.Z.2    Quittner, A.L.3
  • 17
    • 78650199065 scopus 로고    scopus 로고
    • Resurgence of colistin: A review of resistance, toxicity, pharmacodynamics, and dosing
    • Lim LM, Ly N, Anderson D, et al. Resurgence of colistin: a review of resistance, toxicity, pharmacodynamics, and dosing. Pharmacotherapy 2010;30(12):1279-91
    • (2010) Pharmacotherapy , vol.30 , Issue.12 , pp. 1279-1291
    • Lim, L.M.1    Ly, N.2    Anderson, D.3
  • 18
    • 84875215401 scopus 로고    scopus 로고
    • Safety, efficacy and convenience of colistimethate sodium dry powder for inhalation (Colobreathe DPI) in patients with cystic fibrosis: A randomised study
    • Schuster A, Haliburn C, Doring G, et al. Safety, efficacy and convenience of colistimethate sodium dry powder for inhalation (Colobreathe DPI) in patients with cystic fibrosis: a randomised study. Thorax 2013;68(4):344-50
    • (2013) Thorax , vol.68 , Issue.4 , pp. 344-350
    • Schuster, A.1    Haliburn, C.2    Doring, G.3
  • 19
    • 1642358243 scopus 로고    scopus 로고
    • Effect of nebulized colistin sulphate and colistin sulphomethate on lung function in patients with cystic fibrosis: A pilot study
    • Westerman EM, Le Brun PP, Touw DJ, et al. Effect of nebulized colistin sulphate and colistin sulphomethate on lung function in patients with cystic fibrosis: a pilot study. J Cyst Fibros 2004;3(1):23-8
    • (2004) J Cyst Fibros , vol.3 , Issue.1 , pp. 23-28
    • Westerman, E.M.1    Le Brun, P.P.2    Touw, D.J.3
  • 20
    • 33750078400 scopus 로고    scopus 로고
    • Anti-inflammatory effects of azithromycin in cystic fibrosis airway epithelial cells
    • Cigana C, Nicolis E, Pasetto M, et al. Anti-inflammatory effects of azithromycin in cystic fibrosis airway epithelial cells. Biochem Biophys Res Commun 2006;350(4):977-82
    • (2006) Biochem Biophys Res Commun , vol.350 , Issue.4 , pp. 977-982
    • Cigana, C.1    Nicolis, E.2    Pasetto, M.3
  • 21
    • 0344624839 scopus 로고    scopus 로고
    • Long-term azithromycin may improve lung function in children with cystic fibrosis
    • Jaffe A, Francis J, Rosenthal M, et al. Long-term azithromycin may improve lung function in children with cystic fibrosis. Lancet 1998;351(9100):420
    • (1998) Lancet , vol.351 , Issue.9100 , pp. 420
    • Jaffe, A.1    Francis, J.2    Rosenthal, M.3
  • 22
    • 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):212-16
    • (2002) Thorax , vol.57 , Issue.3 , pp. 212-216
    • Wolter, J.1    Seeney, S.2    Bell, S.3
  • 23
    • 26944446140 scopus 로고    scopus 로고
    • Heterogeneity of treatment response to azithromycin in patients with cystic fibrosis
    • Saiman L, Mayer-Hamblett N, Campbell P, et al. Heterogeneity of treatment response to azithromycin in patients with cystic fibrosis. Am J Respir Crit Care Med 2005;172(8):1008-12
    • (2005) Am J Respir Crit Care Med , vol.172 , Issue.8 , pp. 1008-1012
    • Saiman, L.1    Mayer-Hamblett, N.2    Campbell, P.3
  • 24
    • 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
  • 26
    • 38449096089 scopus 로고    scopus 로고
    • Daily versus weekly azithromycin in cystic fibrosis patients
    • McCormack J, Bell S, Senini S, et al. Daily versus weekly azithromycin in cystic fibrosis patients. Eur Respir J 2007;30(3):487-95
    • (2007) Eur Respir J , vol.30 , Issue.3 , pp. 487-495
    • McCormack, J.1    Bell, S.2    Senini, S.3
  • 27
    • 0028778427 scopus 로고
    • Ototoxicity with azithromycin
    • Wallace MR, Miller LK, Nguyen MT, et al. Ototoxicity with azithromycin. Lancet 1994;343(8891):241
    • (1994) Lancet , vol.343 , Issue.8891 , pp. 241
    • Wallace, M.R.1    Miller, L.K.2    Nguyen, M.T.3
  • 28
    • 80052378958 scopus 로고    scopus 로고
    • Azithromycin blocks autophagy and may predispose cystic fibrosis patients to mycobacterial infection
    • Renna M, Schaffner C, Brown K, et al. Azithromycin blocks autophagy and may predispose cystic fibrosis patients to mycobacterial infection. J Clin Invest 2011;121(9):3554-63
    • (2011) J Clin Invest , vol.121 , Issue.9 , pp. 3554-3563
    • Renna, M.1    Schaffner, C.2    Brown, K.3
  • 29
    • 84877254751 scopus 로고    scopus 로고
    • Use of azithromycin and death from cardiovascular causes
    • Svanstrom H, Pasternak B, Hviid A. Use of azithromycin and death from cardiovascular causes. N Engl J Med 2013;368(18):1704-12
    • (2013) N Engl J Med , vol.368 , Issue.18 , pp. 1704-1712
    • Svanstrom, H.1    Pasternak, B.2    Hviid, A.3
  • 30
    • 33845388432 scopus 로고    scopus 로고
    • Do inhaled corticosteroids impair long-term growth in prepubertal cystic fibrosis patients?
    • De Boeck K, De Baets F, Malfroot A, et al. Do inhaled corticosteroids impair long-term growth in prepubertal cystic fibrosis patients? Eur J Pediatr 2007;166(1):23-8
    • (2007) Eur J Pediatr , vol.166 , Issue.1 , pp. 23-28
    • De Boeck, K.1    De Baets, F.2    Malfroot, A.3
  • 32
    • 0020696950 scopus 로고
    • Chronic Pseudomonas aeruginosa lung infection in cystic fibrosis. A longitudinal study of immune complex activity and inflammatory response in sputum solphase of cystic fibrosis patients with chronic Pseudomonas aeruginosa lung infections: Influence of local steroid treatment
    • Schiotz PO, Jorgensen M, Flensborg EW, et al. Chronic Pseudomonas aeruginosa lung infection in cystic fibrosis. A longitudinal study of immune complex activity and inflammatory response in sputum solphase of cystic fibrosis patients with chronic Pseudomonas aeruginosa lung infections: influence of local steroid treatment. Acta Paediatr Scand 1983;72(2):283-7
    • (1983) Acta Paediatr Scand , vol.72 , Issue.2 , pp. 283-287
    • Schiotz, P.O.1    Jorgensen, M.2    Flensborg, E.W.3
  • 33
    • 0031907675 scopus 로고    scopus 로고
    • Serum eosinophil cationic protein, eosinophil protein X and eosinophil peroxidase in relation to pulmonary function in cystic fibrosis
    • Koller DY, Nilsson M, Enander I, et al. Serum eosinophil cationic protein, eosinophil protein X and eosinophil peroxidase in relation to pulmonary function in cystic fibrosis. Clin Exp Allergy 1998;28(2):241-8
    • (1998) Clin Exp Allergy , vol.28 , Issue.2 , pp. 241-248
    • Koller, D.Y.1    Nilsson, M.2    Enander, I.3
  • 34
    • 26944431603 scopus 로고    scopus 로고
    • Effects of montelukast treatment on clinical and inflammatory variables in patients with cystic fibrosis
    • Stelmach I, Korzeniewska A, Stelmach W, et al. Effects of montelukast treatment on clinical and inflammatory variables in patients with cystic fibrosis. Ann Allergy Asthma Immunol 2005;95(4):372-80
    • (2005) Ann Allergy Asthma Immunol , vol.95 , Issue.4 , pp. 372-380
    • Stelmach, I.1    Korzeniewska, A.2    Stelmach, W.3
  • 35
    • 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):245-78
    • (2006) Physiol Rev , vol.86 , Issue.1 , pp. 245-278
    • Rose, M.C.1    Voynow, J.A.2
  • 36
    • 3042709453 scopus 로고    scopus 로고
    • MUC5AC and MUC5B Mucins Are Decreased in Cystic Fibrosis Airway Secretions
    • Henke MO, Renner A, Huber RM, et al. MUC5AC and MUC5B Mucins Are Decreased in Cystic Fibrosis Airway Secretions. Am J Respir Cell Mol Biol 2004;31(1):86-91
    • (2004) Am J Respir Cell Mol Biol , vol.31 , Issue.1 , pp. 86-91
    • Henke, M.O.1    Renner, A.2    Huber, R.M.3
  • 37
    • 84857739601 scopus 로고    scopus 로고
    • Recombinant human DNase i decreases biofilm and increases antimicrobial susceptibility in staphylococci
    • Kaplan JB, LoVetri K, Cardona ST, et al. Recombinant human DNase I decreases biofilm and increases antimicrobial susceptibility in staphylococci. J Antibiot (Tokyo) 2012;65(2):73-7
    • (2012) J Antibiot (Tokyo) , vol.65 , Issue.2 , pp. 73-77
    • Kaplan, J.B.1    Lovetri, K.2    Cardona, S.T.3
  • 38
    • 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
  • 40
    • 79955909454 scopus 로고    scopus 로고
    • Clinical use of dornase alpha is associated with a slower rate of FEV1 decline in cystic fibrosis
    • Konstan MW, Wagener JS, Pasta DJ, et al. Clinical use of dornase alpha is associated with a slower rate of FEV1 decline in cystic fibrosis. Pediatr Pulmonol 2011;46(6):545-53
    • (2011) Pediatr Pulmonol , vol.46 , Issue.6 , pp. 545-553
    • Konstan, M.W.1    Wagener, J.S.2    Pasta, D.J.3
  • 41
    • 84155165377 scopus 로고    scopus 로고
    • Reduced mortality in cystic fibrosis patients treated with tobramycin inhalation solution
    • Sawicki GS, Signorovitch JE, Zhang J, et al. Reduced mortality in cystic fibrosis patients treated with tobramycin inhalation solution. Pediatr Pulmonol 2012;47(1):44-52
    • (2012) Pediatr Pulmonol , vol.47 , Issue.1 , pp. 44-52
    • Sawicki, G.S.1    Signorovitch, J.E.2    Zhang, J.3
  • 43
    • 0029916071 scopus 로고    scopus 로고
    • Effect of hypertonic saline, amiloride, and cough on mucociliary clearance in patients with cystic fibrosis
    • Robinson M, Regnis JA, Bailey DL, et al. Effect of hypertonic saline, amiloride, and cough on mucociliary clearance in patients with cystic fibrosis. Am J Respir Crit Care Med 1996;153(5):1503-9
    • (1996) Am J Respir Crit Care Med , vol.153 , Issue.5 , pp. 1503-1509
    • Robinson, M.1    Regnis, J.A.2    Bailey, D.L.3
  • 44
    • 84862317515 scopus 로고    scopus 로고
    • Hypertonic saline in treatment of pulmonary disease in cystic fibrosis
    • Reeves EP, Molloy K, Pohl K, et al. Hypertonic saline in treatment of pulmonary disease in cystic fibrosis. ScientificWorldJournal 2012;2012:465230
    • (2012) ScientificWorldJournal , vol.2012 , pp. 465230
    • Reeves, E.P.1    Molloy, K.2    Pohl, K.3
  • 45
    • 30944466084 scopus 로고    scopus 로고
    • A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis
    • Elkins MR, Robinson M, Rose BR, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006;354(3):229-40
    • (2006) N Engl J Med , vol.354 , Issue.3 , pp. 229-240
    • Elkins, M.R.1    Robinson, M.2    Rose, B.R.3
  • 46
    • 84860907922 scopus 로고    scopus 로고
    • Stratified assessment of the role of inhaled hypertonic saline in reducing cystic fibrosis pulmonary exacerbations: A retrospective analysis
    • Dmello D, Nayak RP, Matuschak GM. Stratified assessment of the role of inhaled hypertonic saline in reducing cystic fibrosis pulmonary exacerbations: a retrospective analysis. BMJ Open 2011;1(1):e000019
    • (2011) BMJ Open , vol.1 , Issue.1 , pp. e000019
    • Dmello, D.1    Nayak, R.P.2    Matuschak, G.M.3
  • 47
    • 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
  • 48
    • 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(3):291-3
    • (2006) N Engl J Med , vol.354 , Issue.3 , pp. 291-293
    • Ratjen, F.1
  • 49
    • 84870196595 scopus 로고    scopus 로고
    • Nebulized hypertonic saline containing hyaluronic acid improves tolerability in patients with cystic fibrosis and lung disease compared with nebulized hypertonic saline alone: A prospective, randomized, double-blind, controlled study
    • Furnari ML, Termini L, Traverso G, et al. Nebulized hypertonic saline containing hyaluronic acid improves tolerability in patients with cystic fibrosis and lung disease compared with nebulized hypertonic saline alone: a prospective, randomized, double-blind, controlled study. Ther Adv Respir Dis 2012;6(6):315-22
    • (2012) Ther Adv Respir Dis , vol.6 , Issue.6 , pp. 315-322
    • Furnari, M.L.1    Termini, L.2    Traverso, G.3
  • 50
    • 0032834277 scopus 로고    scopus 로고
    • The effect of inhaled mannitol on bronchial mucus clearance in cystic fibrosis patients: A pilot study
    • Robinson M, Daviskas E, Eberl S, et al. The effect of inhaled mannitol on bronchial mucus clearance in cystic fibrosis patients: a pilot study. Eur Respir J 1999;14(3):678-85
    • (1999) Eur Respir J , vol.14 , Issue.3 , pp. 678-685
    • Robinson, M.1    Daviskas, E.2    Eberl, S.3
  • 51
    • 84859392118 scopus 로고    scopus 로고
    • The direct effect of hyperosmolar agents on ciliary beating of human bronchial epithelial cells
    • Yaghi A, Zaman A, Dolovich MB. The direct effect of hyperosmolar agents on ciliary beating of human bronchial epithelial cells. J Aerosol Med Pulm Drug Deliv 2012;25(2):88-95
    • (2012) J Aerosol Med Pulm Drug Deliv , vol.25 , Issue.2 , pp. 88-95
    • Yaghi, A.1    Zaman, A.2    Dolovich, M.B.3
  • 52
    • 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
  • 53
    • 84858234304 scopus 로고    scopus 로고
    • Long-term inhaled dry powder mannitol in cystic fibrosis: An international randomized study
    • Aitken ML, Bellon G, De Boeck K, et al. Long-term inhaled dry powder mannitol in cystic fibrosis: an international randomized study. Am J Respir Crit Care Med 2012;185(6):645-52
    • (2012) Am J Respir Crit Care Med , vol.185 , Issue.6 , pp. 645-652
    • Aitken, M.L.1    Bellon, G.2    De Boeck, K.3
  • 54
    • 84863186511 scopus 로고    scopus 로고
    • Mannitol dry powder for inhalation: In patients with cystic fibrosis
    • Burness CB and Keating GM. Mannitol dry powder for inhalation: in patients with cystic fibrosis. Drugs 2012;72(10):1411-21
    • (2012) Drugs , vol.72 , Issue.10 , pp. 1411-1421
    • Burness, C.B.1    Keating, G.M.2
  • 55
    • 13944279989 scopus 로고    scopus 로고
    • Early decline of pancreatic function in cystic fibrosis patients with class 1 or 2 CFTR mutations
    • Walkowiak J, Sands D, Nowakowska A, et al. Early decline of pancreatic function in cystic fibrosis patients with class 1 or 2 CFTR mutations. J Pediatr Gastroenterol Nutr 2005;40(2):199-201
    • (2005) J Pediatr Gastroenterol Nutr , vol.40 , Issue.2 , pp. 199-201
    • Walkowiak, J.1    Sands, D.2    Nowakowska, A.3
  • 56
    • 21444443190 scopus 로고    scopus 로고
    • Pancreatic exocrine function in patients with cystic fibrosis
    • Baker SS, Borowitz D, Baker RD. Pancreatic exocrine function in patients with cystic fibrosis. Curr Gastroenterol Rep 2005;7(3):227-33
    • (2005) Curr Gastroenterol Rep , vol.7 , Issue.3 , pp. 227-233
    • Baker, S.S.1    Borowitz, D.2    Baker, R.D.3
  • 57
    • 42649103313 scopus 로고    scopus 로고
    • Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: Results of a systematic review
    • Stallings VA, Stark LJ, Robinson KA, et al. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc 2008;108(5):832-9
    • (2008) J Am Diet Assoc , vol.108 , Issue.5 , pp. 832-839
    • Stallings, V.A.1    Stark, L.J.2    Robinson, K.A.3
  • 60
    • 84897556769 scopus 로고    scopus 로고
    • Ivacaftor: A review of its use in patients with cystic fibrosis
    • Deeks ED. Ivacaftor: a review of its use in patients with cystic fibrosis. Drugs 2013;73(14):1595-604
    • (2013) Drugs , vol.73 , Issue.14 , pp. 1595-1604
    • Deeks, E.D.1
  • 61
    • 84864757410 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator-modifying medications: The future of cystic fibrosis treatment
    • Pettit RS. Cystic fibrosis transmembrane conductance regulator-modifying medications: the future of cystic fibrosis treatment. Ann Pharmacother 2012;46(7-8):1065-75
    • (2012) Ann Pharmacother , vol.46 , Issue.7-8 , pp. 1065-1075
    • Pettit, R.S.1
  • 62
    • 79958746554 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment
    • Rogan MP, Stoltz DA, Hornick DB. Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment. Chest 2011;139(6):1480-90
    • (2011) Chest , vol.139 , Issue.6 , pp. 1480-1490
    • Rogan, M.P.1    Stoltz, D.A.2    Hornick, D.B.3
  • 63
    • 80455162465 scopus 로고    scopus 로고
    • A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
    • Ramsey BW, Davies J, McElvaney NG, et al. 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
  • 64
    • 84878970875 scopus 로고    scopus 로고
    • 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, et al. Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation. Am J Respir Crit Care Med 2013;187(11):1219-25
    • (2013) Am J Respir Crit Care Med , vol.187 , Issue.11 , pp. 1219-1225
    • Davies, J.C.1    Wainwright, C.E.2    Canny, G.J.3
  • 65
    • 84871981271 scopus 로고    scopus 로고
    • Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: The ivacaftor experience
    • Durmowicz AG, Witzmann KA, Rosebraugh CJ, et al. Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: the ivacaftor experience. Chest 2013;143(1):14-18
    • (2013) Chest , vol.143 , Issue.1 , pp. 14-18
    • Durmowicz, A.G.1    Witzmann, K.A.2    Rosebraugh, C.J.3
  • 66
    • 0024424270 scopus 로고
    • Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
    • Riordan JR, Rommens JM, Kerem B, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989;245(4922):1066-73
    • (1989) Science , vol.245 , Issue.4922 , pp. 1066-1073
    • Riordan, J.R.1    Rommens, J.M.2    Kerem, B.3
  • 67
    • 84868266593 scopus 로고    scopus 로고
    • Assessment of F/HN-pseudotyped lentivirus as a clinically relevant vector for lung gene therapy
    • Griesenbach U, Inoue M, Meng C, et al. Assessment of F/HN-pseudotyped lentivirus as a clinically relevant vector for lung gene therapy. Am J Respir Crit Care Med 2012;186(9):846-56
    • (2012) Am J Respir Crit Care Med , vol.186 , Issue.9 , pp. 846-856
    • Griesenbach, U.1    Inoue, M.2    Meng, C.3
  • 68
    • 84858147126 scopus 로고    scopus 로고
    • Amniotic mesenchymal stem cells: A new source for hepatocyte-like cells and induction of CFTR expression by coculture with cystic fibrosis airway epithelial cells
    • Paracchini V, Carbone A, Colombo F, et al. Amniotic mesenchymal stem cells: a new source for hepatocyte-like cells and induction of CFTR expression by coculture with cystic fibrosis airway epithelial cells. J Biomed Biotechnol 2012;2012:575471
    • (2012) J Biomed Biotechnol , vol.2012 , pp. 575471
    • Paracchini, V.1    Carbone, A.2    Colombo, F.3
  • 69
    • 84885183918 scopus 로고    scopus 로고
    • Modifying disease in cystic fibrosis: Current and future therapies on the horizon
    • Ong T, Ramsey BW. Modifying disease in cystic fibrosis: current and future therapies on the horizon. Curr Opin Pulm Med 2013;19(6):645-51
    • (2013) Curr Opin Pulm Med , vol.19 , Issue.6 , pp. 645-651
    • Ong, T.1    Ramsey, B.W.2
  • 70
    • 81755163563 scopus 로고    scopus 로고
    • Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
    • Van Goor F, Hadida S, Grootenhuis PD, et al. Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc Natl Acad Sci USA 2011;108(46):18843-8
    • (2011) Proc Natl Acad Sci USA , vol.108 , Issue.46 , pp. 18843-18848
    • Van Goor, F.1    Hadida, S.2    Grootenhuis, P.D.3
  • 71
    • 84904016376 scopus 로고    scopus 로고
    • Ataluren for the treatment of nonsense-mutation cystic fibrosis: A randomised, double-blind, placebocontrolled phase 3 trial
    • Kerem E, Konstan MW, De Boeck K, et al. Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebocontrolled phase 3 trial. Lancet Respir Med 2014;2(7):539-47
    • (2014) Lancet Respir Med , vol.2 , Issue.7 , pp. 539-547
    • Kerem, E.1    Konstan, M.W.2    De Boeck, K.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.