메뉴 건너뛰기




Volumn 3, Issue 1, 2010, Pages 61-74

Potential of anti-inflammatory treatment for cystic fibrosis lung disease

Author keywords

Cystic fibrosis; Inflammation; Survival; Therapy

Indexed keywords

ANTIINFLAMMATORY AGENT; AZITHROMYCIN; BIOLOGICAL MARKER; CHEMOKINE RECEPTOR ANTAGONIST; CORTICOSTEROID; GLUTATHIONE; HYDROXYCHOROQUINE; IBUPROFEN; IMMUNOGLOBULIN ENHANCER BINDING PROTEIN; IMMUNOSUPPRESSIVE AGENT; INTERLEUKIN 10; INTERLEUKIN 17; INTERLEUKIN 23; LIPOPOLYSACCHARIDE; NITRIC OXIDE; NONSTEROID ANTIINFLAMMATORY AGENT; PIOGLITAZONE; PLACEBO; PREDNISONE; SB 656933; SILDENAFIL; SIMVASTATIN; TRANSCRIPTION FACTOR NRF2; TUMOR NECROSIS FACTOR ALPHA; UNCLASSIFIED DRUG;

EID: 77955536419     PISSN: None     EISSN: 11787031     Source Type: Book Series    
DOI: None     Document Type: Article
Times cited : (16)

References (168)
  • 2
    • 0017198208 scopus 로고
    • The lung in cystic fibrosis. A quantitative study including prevalence or pathologic findings among different age groups
    • Bedrossian CW, Greenberg SD, Singer DB, et al. The lung in cystic fibrosis. A quantitative study including prevalence or pathologic findings among different age groups. Hum Pathol. 1976;7(2):195-204.
    • (1976) Hum Pathol , vol.7 , Issue.2 , pp. 195-204
    • Bedrossian, C.W.1    Greenberg, S.D.2    Singer, D.B.3
  • 3
    • 0028914085 scopus 로고
    • Early pulmonary inflammation in infants with cystic fibrosis
    • Khan TZ, Wagener JS, Bost T, et al. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med. 1995;151:1075-1082.
    • (1995) Am J Respir Crit Care Med , vol.151 , pp. 1075-1082
    • Khan, T.Z.1    Wagener, J.S.2    Bost, T.3
  • 5
    • 0028015883 scopus 로고
    • Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation
    • Konstan MW, Hilliard KA, Norvell TM, et al. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am J Respir Crit Care Med. 1994;150:448-454.
    • (1994) Am J Respir Crit Care Med , vol.150 , pp. 448-454
    • Konstan, M.W.1    Hilliard, K.A.2    Norvell, T.M.3
  • 6
    • 0029346937 scopus 로고
    • The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis
    • Balough K, McCubbin M, Weinberger M, et al. The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis. Pediatr Pulmonol. 1995;20:63-70.
    • (1995) Pediatr Pulmonol , vol.20 , pp. 63-70
    • Balough, K.1    McCubbin, M.2    Weinberger, M.3
  • 7
    • 0030768159 scopus 로고    scopus 로고
    • Lower airway inflammation in infants and young children with cystic fibrosis
    • Armstrong DS, Grimwood K, Carlin JB, et al. Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med. 1997;156:1197-1204.
    • (1997) Am J Respir Crit Care Med , vol.156 , pp. 1197-1204
    • Armstrong, D.S.1    Grimwood, K.2    Carlin, J.B.3
  • 8
    • 0032770489 scopus 로고    scopus 로고
    • Quantitation of inflammatory response to bacteria in young cystic fibrosis and control patients
    • Muhlebach MS, Stewart PW, Leigh MW, et al. Quantitation of inflammatory response to bacteria in young cystic fibrosis and control patients. Am J Respir Crit Care Med. 1999;160:186-191.
    • (1999) Am J Respir Crit Care Med , vol.160 , pp. 186-191
    • Muhlebach, M.S.1    Stewart, P.W.2    Leigh, M.W.3
  • 9
    • 0021970474 scopus 로고
    • Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis
    • Auerbach HS, Williams M, Kirkpatrick JA, et al. Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet. 1985;2(8457):686-688.
    • (1985) Lancet , vol.2 , Issue.8457 , pp. 686-688
    • Auerbach, H.S.1    Williams, M.2    Kirkpatrick, J.A.3
  • 10
    • 0028969594 scopus 로고
    • A multicenter study of alternate- day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group
    • Eigen H, Rosenstein BJ, FitzSimmons S, et al. A multicenter study of alternate- day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group. J Pediatr. 1995;126(4):515-523.
    • (1995) J Pediatr , vol.126 , Issue.4 , pp. 515-523
    • Eigen, H.1    Rosenstein, B.J.2    Fitzsimmons, S.3
  • 11
    • 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;332:848-844.
    • (1995) N Engl J Med , vol.332 , pp. 848-844
    • Konstan, M.W.1    Byard, P.J.2    Hoppel, C.L.3
  • 12
    • 0000713023 scopus 로고
    • Long-term alternate day prednisone therapy in cystic fibrosis
    • Donati MA, Haver K, Gerson W, et al. Long-term alternate day prednisone therapy in cystic fibrosis. Pediatr Pulmonol. 1990;5:A322.
    • (1990) Pediatr Pulmonol , vol.5
    • Donati, M.A.1    Haver, K.2    Gerson, W.3
  • 13
    • 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;151:228-230.
    • (2007) J Pediatr , vol.151 , pp. 228-230
    • Lands, L.C.1    Milner, R.2    Cantin, A.M.3
  • 14
    • 36848999782 scopus 로고    scopus 로고
    • Clinical use of ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis
    • Konstan MW, Schluchter MD, Xue W, et al. Clinical use of ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis. Am J Respir Crit Care Med. 2007;176:1084-1089.
    • (2007) Am J Respir Crit Care Med , vol.176 , pp. 1084-1089
    • Konstan, M.W.1    Schluchter, M.D.2    Xue, W.3
  • 15
    • 0031035884 scopus 로고    scopus 로고
    • Nasal and bronchoalveolar lavage fluid cytokines in early cystic fibrosis
    • Noah TL, Black HR, Cheng PW, et al. Nasal and bronchoalveolar lavage fluid cytokines in early cystic fibrosis. J Infect Dis. 1997;175:638-647.
    • (1997) J Infect Dis , vol.175 , pp. 638-647
    • Noah, T.L.1    Black, H.R.2    Cheng, P.W.3
  • 17
    • 34247629486 scopus 로고    scopus 로고
    • MPB-07 reduces the inflammatory response to Pseudomonas aeruginosa in cystic fibrosis bronchial cells
    • Dechecchi MC, Nicolis E, Bezzerri V, et al. MPB-07 reduces the inflammatory response to Pseudomonas aeruginosa in cystic fibrosis bronchial cells. Am J Respir Cell Mol Biol. 2007;36: 615-624.
    • (2007) Am J Respir Cell Mol Biol , vol.36 , pp. 615-624
    • Dechecchi, M.C.1    Nicolis, E.2    Bezzerri, V.3
  • 18
    • 0031452350 scopus 로고    scopus 로고
    • Excessive inflammatory response on cystic fibrosis mice to bronchopulmonary infection with P. aeruginosa
    • van Heeckeren A, Walenga R, Konstan MW, et al. Excessive inflammatory response on cystic fibrosis mice to bronchopulmonary infection with P. aeruginosa. J Clin Invest. 1997;100:2810-2815.
    • (1997) J Clin Invest , vol.100 , pp. 2810-2815
    • van Heeckeren, A.1    Walenga, R.2    Konstan, M.W.3
  • 19
    • 0034822263 scopus 로고    scopus 로고
    • G551D CF mice display an abnormal host response and have impaired clearance of Pseudomonas lung disease
    • McMorran BJ, Palmer JS, Lunn DP, et al. G551D CF mice display an abnormal host response and have impaired clearance of Pseudomonas lung disease. Am J Physiol Lung Cel Mol Physiol. 2001;281:L740-L747.
    • (2001) Am J Physiol Lung Cel Mol Physiol , vol.281
    • McMorran, B.J.1    Palmer, J.S.2    Lunn, D.P.3
  • 20
    • 34247557545 scopus 로고    scopus 로고
    • Role of IKK and ERK pathways in intrinsic inflammation of cystic fibrosis airways
    • Verhaeghe C, Remouchamps C, Hennuy B, et al. Role of IKK and ERK pathways in intrinsic inflammation of cystic fibrosis airways. Biochem Pharmacol. 2007;73:1982-1994.
    • (2007) Biochem Pharmacol , vol.73 , pp. 1982-1994
    • Verhaeghe, C.1    Remouchamps, C.2    Hennuy, B.3
  • 21
    • 0037308173 scopus 로고    scopus 로고
    • Signaling intermediates required for NFκB activation and IL-8 expression in CF bronchial epithelial cells
    • Li J, Johnson XD, Lazvovskaia S, et al. Signaling intermediates required for NFκB activation and IL-8 expression in CF bronchial epithelial cells. Am J Physiol Lung Cell Mol Physiol. 2003;284:L307-L315.
    • (2003) Am J Physiol Lung Cell Mol Physiol , vol.284
    • Li, J.1    Johnson, X.D.2    Lazvovskaia, S.3
  • 22
    • 0037385638 scopus 로고    scopus 로고
    • Fluticasone reduces IL-6 and IL-8 production of cystic fibrosis bronchial epithelial cells via IKK-beta kinase pathway
    • Escotte S, Tabary O, Dusser D, et al. Fluticasone reduces IL-6 and IL-8 production of cystic fibrosis bronchial epithelial cells via IKK-beta kinase pathway. Eur Respir J. 2003;21:574-581.
    • (2003) Eur Respir J , vol.21 , pp. 574-581
    • Escotte, S.1    Tabary, O.2    Dusser, D.3
  • 23
    • 24044466139 scopus 로고    scopus 로고
    • Chronic airway infection/ inflammation induces a Ca2+i-dependent hyperinflammatory response in human cystic fibrosis airway epithelia
    • Ribeiro CM, Paradiso AM, Schwab U, et al. Chronic airway infection/ inflammation induces a Ca2+i-dependent hyperinflammatory response in human cystic fibrosis airway epithelia. J Biol Chem. 2005;280:17798-17806.
    • (2005) J Biol Chem , vol.280 , pp. 17798-17806
    • Ribeiro, C.M.1    Paradiso, A.M.2    Schwab, U.3
  • 24
    • 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;13:257-261.
    • (1995) Am J Respir Cell Mol Biol , vol.13 , pp. 257-261
    • Bonfield, T.L.1    Konstan, M.W.2    Burfeind, P.3
  • 25
    • 0032817772 scopus 로고    scopus 로고
    • Altered respiratory epithelial cell cytokine production in cystic fibrosis
    • Bonfield TL, Konstan MW, Berger M. Altered respiratory epithelial cell cytokine production in cystic fibrosis. J Allergy Clin Immunol. 1999;104(1):72-78.
    • (1999) J Allergy Clin Immunol , vol.104 , Issue.1 , pp. 72-78
    • Bonfield, T.L.1    Konstan, M.W.2    Berger, M.3
  • 27
    • 0032864036 scopus 로고    scopus 로고
    • Distinct sputum cytokine profiles in cystic fibrosis and other chronic inflammatory airway disease
    • Osika E, Cavaillon JM, Chadelat K, et al. Distinct sputum cytokine profiles in cystic fibrosis and other chronic inflammatory airway disease. Eur Respir J. 1999;14:339-346.
    • (1999) Eur Respir J , vol.14 , pp. 339-346
    • Osika, E.1    Cavaillon, J.M.2    Chadelat, K.3
  • 28
    • 0030781140 scopus 로고    scopus 로고
    • In vivo suppression of NFkB and preservation of IkBalpha by interleukin-10 and interleukin-13
    • Lentsch AB, Shanley TP, Sarma V, et al. In vivo suppression of NFkB and preservation of IkBalpha by interleukin-10 and interleukin-13. J Clin Invest. 1997;100:2443-2448.
    • (1997) J Clin Invest , vol.100 , pp. 2443-2448
    • Lentsch, A.B.1    Shanley, T.P.2    Sarma, V.3
  • 29
    • 0033527549 scopus 로고    scopus 로고
    • Interleukin-10 signaling blocks inhibitor of kB kinase activity and nuclear factor kB DNA binding
    • Schottelius AJG, Mayo MW, Sartor RB, et al. Interleukin-10 signaling blocks inhibitor of kB kinase activity and nuclear factor kB DNA binding. J Biol Chem. 1999;274:31868-31874.
    • (1999) J Biol Chem , vol.274 , pp. 31868-31874
    • Schottelius, A.J.G.1    Mayo, M.W.2    Sartor, R.B.3
  • 30
    • 38049091190 scopus 로고    scopus 로고
    • Alterations in immune respnse and PPAR/LXR regulation in cystic fibrosis macrophages
    • Andersson C, Zaman MM, Jones AB, et al. Alterations in immune respnse and PPAR/LXR regulation in cystic fibrosis macrophages. J Cyst Fibros. 2008;7(1):68-78.
    • (2008) J Cyst Fibros , vol.7 , Issue.1 , pp. 68-78
    • Andersson, C.1    Zaman, M.M.2    Jones, A.B.3
  • 31
    • 3042856185 scopus 로고    scopus 로고
    • Decreased expression of peroxisome proliferator activated receptor gamma in cftr-/- mice
    • Ollero M, Junaidi O, Zaman MM, et al. Decreased expression of peroxisome proliferator activated receptor gamma in cftr-/- mice. J Cell Physiol. 2004;200:235-244.
    • (2004) J Cell Physiol , vol.200 , pp. 235-244
    • Ollero, M.1    Junaidi, O.2    Zaman, M.M.3
  • 32
    • 4644272735 scopus 로고    scopus 로고
    • Defective lipoxin-mediated antiinflammatory activity in the cystic fibrosis airway
    • Karp CL, Flick LM, Park KW, et al. Defective lipoxin-mediated antiinflammatory activity in the cystic fibrosis airway. Nat Immunol. 2004;5:357-358.
    • (2004) Nat Immunol , vol.5 , pp. 357-358
    • Karp, C.L.1    Flick, L.M.2    Park, K.W.3
  • 33
    • 33751288084 scopus 로고    scopus 로고
    • Anti-inflammatory cytokines in cystic fibrosis lung disease
    • Starosta V, Ratjen F, Rietschel E, et al. Anti-inflammatory cytokines in cystic fibrosis lung disease. Eur Respir J. 2006;28:581-587.
    • (2006) Eur Respir J , vol.28 , pp. 581-587
    • Starosta, V.1    Ratjen, F.2    Rietschel, E.3
  • 35
    • 33847043963 scopus 로고    scopus 로고
    • IL-23 mediates inflammatory responses to mucoid pseudomonas aeruginosa lung infection in mice
    • Dubin PJ, Kolls JK. IL-23 mediates inflammatory responses to mucoid pseudomonas aeruginosa lung infection in mice. Am J Physiol Lung Cel Mol Physiol. 2007;292:L519-L523.
    • (2007) Am J Physiol Lung Cel Mol Physiol , vol.292
    • Dubin, P.J.1    Kolls, J.K.2
  • 36
    • 21244455537 scopus 로고    scopus 로고
    • Role of IL-17A, IL-17F, and the IL-17 receptor in regulating growth related oncogene-alpha and granulocyte colony-stimulating factor in bronchial epithelium: Implications for airway inflammation in cystic fibrosis
    • McAllister F, Henry A, Kreindler JL, et al. Role of IL-17A, IL-17F, and the IL-17 receptor in regulating growth related oncogene-alpha and granulocyte colony-stimulating factor in bronchial epithelium: implications for airway inflammation in cystic fibrosis. J Immunol. 2005;175:404-412.
    • (2005) J Immunol , vol.175 , pp. 404-412
    • McAllister, F.1    Henry, A.2    Kreindler, J.L.3
  • 37
    • 72949095117 scopus 로고    scopus 로고
    • IL-17 primes airway epithelial cells lacking functional CFTR in increase NOD1 responses
    • Roussel L, Rousseau S. IL-17 primes airway epithelial cells lacking functional CFTR in increase NOD1 responses. Biochem Biophys Res Commun. 2010;391(1):505-509.
    • (2010) Biochem Biophys Res Commun , vol.391 , Issue.1 , pp. 505-509
    • Roussel, L.1    Rousseau, S.2
  • 38
    • 77954239363 scopus 로고    scopus 로고
    • Regulation of airway MUC5AC expression by IL-1beta and IL-17A; the NF-kappaB paradigm
    • Fujisawa T, Velichko S, Thai P, et al. Regulation of airway MUC5AC expression by IL-1beta and IL-17A; the NF-kappaB paradigm. J Immunol. 2009;183(10):6236-6243.
    • (2009) J Immunol , vol.183 , Issue.10 , pp. 6236-6243
    • Fujisawa, T.1    Velichko, S.2    Thai, P.3
  • 39
    • 0037442137 scopus 로고    scopus 로고
    • IL-17, produced by lymphocytes and neutrophils, is necessary for lipopolysaccharide induced airway neutrophilia: IL-15 as a possible trigger
    • Ferretti S, Bonneau O, Dubois GR, et al. IL-17, produced by lymphocytes and neutrophils, is necessary for lipopolysaccharide induced airway neutrophilia: IL-15 as a possible trigger. J Immunol. 2003;170:2106-2112.
    • (2003) J Immunol , vol.170 , pp. 2106-2112
    • Ferretti, S.1    Bonneau, O.2    Dubois, G.R.3
  • 40
    • 54449093079 scopus 로고    scopus 로고
    • Dysfunction of Nrf-2 in CF epithelia leads to excess intracellular H2O2 and inflammatory cytokine production
    • Chen J, Kinter M, Shank S, et al. Dysfunction of Nrf-2 in CF epithelia leads to excess intracellular H2O2 and inflammatory cytokine production. PLoS ONE. 2008;3:e3367.
    • (2008) PLoS ONE , vol.3
    • Chen, J.1    Kinter, M.2    Shank, S.3
  • 41
    • 33644990327 scopus 로고    scopus 로고
    • Interleukin-1B induction of NFkB is partially regulated by H2O2-mediated activation of NFkB-inducing kinase
    • Li Q, Engelhardt JF. Interleukin-1B induction of NFkB is partially regulated by H2O2-mediated activation of NFkB-inducing kinase. J Biol Chem. 2006;281:1495-1505.
    • (2006) J Biol Chem , vol.281 , pp. 1495-1505
    • Li, Q.1    Engelhardt, J.F.2
  • 42
  • 43
    • 0030961127 scopus 로고    scopus 로고
    • Decreased concentration of exhaled nitric oxide (NO) in patients with cystic fibrosis
    • Grasemann H, Michler E, Wallot M, et al. Decreased concentration of exhaled nitric oxide (NO) in patients with cystic fibrosis. Pediatr Pulmonol. 1997;24:173-177.
    • (1997) Pediatr Pulmonol , vol.24 , pp. 173-177
    • Grasemann, H.1    Michler, E.2    Wallot, M.3
  • 44
    • 0027742895 scopus 로고
    • Systemic deficiency of glutathione in cystic fibrosis
    • Roum JH, Buhl R, McElvaney NG, et al. Systemic deficiency of glutathione in cystic fibrosis. J Appl Physiol. 1993;75:2419-2424.
    • (1993) J Appl Physiol , vol.75 , pp. 2419-2424
    • Roum, J.H.1    Buhl, R.2    McElvaney, N.G.3
  • 45
    • 0344258132 scopus 로고    scopus 로고
    • Abnormal glutathione transport in cystic fibrosis airway epithelia
    • Gao L, Kim KJ, Yankaskas JR, et al. Abnormal glutathione transport in cystic fibrosis airway epithelia. Am J Physiol. 1999;277:L113-L118.
    • (1999) Am J Physiol , vol.277
    • Gao, L.1    Kim, K.J.2    Yankaskas, J.R.3
  • 46
    • 72449174784 scopus 로고    scopus 로고
    • The triterpenoid CDDO limits inflammation in preclinical models of cystic fibrosis lung disease
    • Nichols DP, Ziady AG, Shank SL, et al. The triterpenoid CDDO limits inflammation in preclinical models of cystic fibrosis lung disease. Am J Physiol Lung Cel Mol Physiol. 2009;297(5):L828-L836.
    • (2009) Am J Physiol Lung Cel Mol Physiol , vol.297 , Issue.5
    • Nichols, D.P.1    Ziady, A.G.2    Shank, S.L.3
  • 47
    • 27744450995 scopus 로고    scopus 로고
    • Transcription factor Nrf2 plays a pivotal role in protection against elastase-induced pulmonary inflammation and emphysema
    • Ishii Y, Itoh K, Morishima Y, et al. Transcription factor Nrf2 plays a pivotal role in protection against elastase-induced pulmonary inflammation and emphysema. J Immunol. 2005;175:6968-6975.
    • (2005) J Immunol , vol.175 , pp. 6968-6975
    • Ishii, Y.1    Itoh, K.2    Morishima, Y.3
  • 48
    • 45949089501 scopus 로고    scopus 로고
    • A novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in chronic neutrophilic inflammation
    • Gaggar A, Jackson PL, Noerager BD, et al. A novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in chronic neutrophilic inflammation. J Immunol. 2008;180:5662-5669.
    • (2008) J Immunol , vol.180 , pp. 5662-5669
    • Gaggar, A.1    Jackson, P.L.2    Noerager, B.D.3
  • 49
    • 77951951482 scopus 로고    scopus 로고
    • Human neutrophil elastase-mediated cleavage sites on MMP-9 and TIMP-1: Implications to cystic fibrosis proteolytic dysfunction
    • Epub 2010 Jan 29
    • Jackson PL, Xu X, Wilson L, et al. Human neutrophil elastase-mediated cleavage sites on MMP-9 and TIMP-1: implications to cystic fibrosis proteolytic dysfunction. Mol Med. 2010. Epub 2010 Jan 29.
    • (2010) Mol Med , pp. 29
    • Jackson, P.L.1    Xu, X.2    Wilson, L.3
  • 50
    • 76249114693 scopus 로고    scopus 로고
    • Decreased levels of secretory leucoprotease inhibitor in the pseudomonas-infected cystic fibrosis lung are due to neutrophil elastase degredation
    • Weldon S, McNally P, McElvaney NG, et al. Decreased levels of secretory leucoprotease inhibitor in the pseudomonas-infected cystic fibrosis lung are due to neutrophil elastase degredation. J Immunol. 2009;183:8146-8156.
    • (2009) J Immunol , vol.183 , pp. 8146-8156
    • Weldon, S.1    McNally, P.2    McElvaney, N.G.3
  • 51
    • 57749104104 scopus 로고    scopus 로고
    • Elafin, an elastase-specific inhibitor, is cleaved by its cognate enzyme neutrophil elastase in sputum from individuals with cystic f ibrosis
    • Goyot N, Butler MW, McNally P, et al. Elafin, an elastase-specific inhibitor, is cleaved by its cognate enzyme neutrophil elastase in sputum from individuals with cystic f ibrosis. J Biol Chem. 2008;283(47):32377-32385.
    • (2008) J Biol Chem , vol.283 , Issue.47 , pp. 32377-32385
    • Goyot, N.1    Butler, M.W.2    McNally, P.3
  • 52
    • 0032931961 scopus 로고    scopus 로고
    • The use of anti-inflammatory medications in cystic fibrosis: Trends, and physician attitudes
    • Oermann CM, Sockrider MM, Konstan MW. The use of anti-inflammatory medications in cystic fibrosis: trends, and physician attitudes. Chest. 1999;115:1053-1058.
    • (1999) Chest , vol.115 , pp. 1053-1058
    • Oermann, C.M.1    Sockrider, M.M.2    Konstan, M.W.3
  • 53
    • 0018864505 scopus 로고
    • Hypogammaglobulinemia in patients with cystic fibrosis
    • Matthews WJ, Williams M, Oliphint B, et al. Hypogammaglobulinemia in patients with cystic fibrosis. N Engl J Med. 1980;302:245-249.
    • (1980) N Engl J Med , vol.302 , pp. 245-249
    • Matthews, W.J.1    Williams, M.2    Oliphint, B.3
  • 54
    • 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(4):515-523.
    • (1995) J Pediatr , vol.126 , Issue.4 , pp. 515-523
    • Eigen, H.1    Rosenstein, B.J.2    Fitzsimmons, S.3
  • 55
    • 0025977985 scopus 로고
    • Risks of alternate day prednisone in patients with cystic fibrosis
    • Rosenstein BJ, Eigen H. Risks of alternate day prednisone in patients with cystic fibrosis. Pediatr. 1991;87:245-246.
    • (1991) Pediatr , vol.87 , pp. 245-246
    • Rosenstein, B.J.1    Eigen, H.2
  • 56
    • 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. Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis. N Engl J Med. 2000;342:851-859.
    • (2000) N Engl J Med , vol.342 , pp. 851-859
    • Lai, H.C.1    Fitzsimmons, S.C.2    Allen, D.B.3
  • 57
    • 0028169953 scopus 로고
    • Interleukin-1alpha, soluble interleukin-2 receptor, and IgG concentrations in cystic fibrosis treated with prednisolone
    • Greally P, Hussain MJ, Vergani D, et al. Interleukin-1alpha, soluble interleukin-2 receptor, and IgG concentrations in cystic fibrosis treated with prednisolone. Arch Dis Child. 1994;71:35-39.
    • (1994) Arch Dis Child , vol.71 , pp. 35-39
    • Greally, P.1    Hussain, M.J.2    Vergani, D.3
  • 59
    • 0025019280 scopus 로고
    • Ibuprofen attenuates the inflammatory response to Pseudomonas aeruginosa in a rat model of chroic pulmonary infection
    • Konstan MW, Vargo KM, Davis PB. Ibuprofen attenuates the inflammatory response to Pseudomonas aeruginosa in a rat model of chroic pulmonary infection. Am Rev Respir Dis. 1990;141:186-192.
    • (1990) Am Rev Respir Dis , vol.141 , pp. 186-192
    • Konstan, M.W.1    Vargo, K.M.2    Davis, P.B.3
  • 60
    • 0031990814 scopus 로고    scopus 로고
    • Modulation of transcription factor NF-kappaB by enantiomers of the nonsteroidal drug ibuprofen
    • Scheuren N, Bang H, Munster T, et al. Modulation of transcription factor NF-kappaB by enantiomers of the nonsteroidal drug ibuprofen. Br J Pharmacol. 1998;123:645-652.
    • (1998) Br J Pharmacol , vol.123 , pp. 645-652
    • Scheuren, N.1    Bang, H.2    Munster, T.3
  • 61
    • 0035220745 scopus 로고    scopus 로고
    • Inhibition of NF-kB and AP-1 activation by R- and S-flurbiprofen
    • Tegeder I, Niederberger E, Israr E, et al. Inhibition of NF-kB and AP-1 activation by R- and S-flurbiprofen. FASEB J. 2001;15:595-597.
    • (2001) FASEB J , vol.15 , pp. 595-597
    • Tegeder, I.1    Niederberger, E.2    Israr, E.3
  • 62
    • 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;290:1749-1756.
    • (2003) JAMA , vol.290 , pp. 1749-1756
    • Saiman, L.1    Marshall, B.C.2    Mayer-Hamblett, N.3
  • 63
    • 71249138160 scopus 로고    scopus 로고
    • Effects of prolonged use of azithromycin in patients with cystic fibrosis: A meta-analysis
    • Florescu DF, Murphy PJ, Kalil AC. Effects of prolonged use of azithromycin in patients with cystic fibrosis: a meta-analysis. Pulm Pharmacol Ther. 2009;22(6):467-472.
    • (2009) Pulm Pharmacol Ther , vol.22 , Issue.6 , pp. 467-472
    • Florescu, D.F.1    Murphy, P.J.2    Kalil, A.C.3
  • 64
    • 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;360:978-984.
    • (2002) Lancet , vol.360 , pp. 978-984
    • Equi, A.1    Balfour-Lynn, I.M.2    Bush, A.3
  • 65
    • 33748307448 scopus 로고    scopus 로고
    • Azithromycin reduces airway neutrophilia and interleukin-8 in patients with bronchiolitis obliterans syndrome
    • Verleden GM, Vanaudenaerde BM, Dupont LJ, et al. Azithromycin reduces airway neutrophilia and interleukin-8 in patients with bronchiolitis obliterans syndrome. Am J Respir Crit Care Med. 2006;174:566-570.
    • (2006) Am J Respir Crit Care Med , vol.174 , pp. 566-570
    • Verleden, G.M.1    Vanaudenaerde, B.M.2    Dupont, L.J.3
  • 66
    • 10644283833 scopus 로고    scopus 로고
    • Azithromycin blocks neutrophil recruitment in pseudomonas endobronchial infection
    • Tsai WC, Rodriguez ML, Young KS, et al. Azithromycin blocks neutrophil recruitment in pseudomonas endobronchial infection. Am J Respir Crit Care Med. 2004;170:1331-1339.
    • (2004) Am J Respir Crit Care Med , vol.170 , pp. 1331-1339
    • Tsai, W.C.1    Rodriguez, M.L.2    Young, K.S.3
  • 67
    • 0037199689 scopus 로고    scopus 로고
    • Azithromycin modulates neutrophil function and circulating inflammatory mediators in healthy human subjects
    • Culic O, Erakovic V, Cepelak I, et al. Azithromycin modulates neutrophil function and circulating inflammatory mediators in healthy human subjects. Eur J Pharmacol. 2002;450:277-289.
    • (2002) Eur J Pharmacol , vol.450 , pp. 277-289
    • Culic, O.1    Erakovic, V.2    Cepelak, I.3
  • 68
    • 34948850804 scopus 로고    scopus 로고
    • Azithromycin blocks quorum sensing and alginate polymer formation an dincreases the sensitivity to serum and stationary-growth-phase killing of pseudomonas aeruginosa and attenuates chronic P. aeruginosa lung infection in cftr(-/-) mice
    • Hoffmann N, Lee B, Hentzer M, et al. Azithromycin blocks quorum sensing and alginate polymer formation an dincreases the sensitivity to serum and stationary-growth-phase killing of pseudomonas aeruginosa and attenuates chronic P. aeruginosa lung infection in cftr(-/-) mice. Antimicrob Agents Chemother. 2007;51:3677-3687.
    • (2007) Antimicrob Agents Chemother , vol.51 , pp. 3677-3687
    • Hoffmann, N.1    Lee, B.2    Hentzer, M.3
  • 69
    • 67349226398 scopus 로고    scopus 로고
    • Azithromycin fails to reduce increased expression of neutrophil-related cytokines in primarycultured epithelial cells from cystic fibrosis mice
    • Gavilanes X, Huaux F, Meyer M, et al. Azithromycin fails to reduce increased expression of neutrophil-related cytokines in primarycultured epithelial cells from cystic fibrosis mice. J Cyst Fibros. 2009;8(3):203-210.
    • (2009) J Cyst Fibros , vol.8 , Issue.3 , pp. 203-210
    • Gavilanes, X.1    Huaux, F.2    Meyer, M.3
  • 70
    • 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
  • 71
    • 33845975311 scopus 로고    scopus 로고
    • Maintenance azithromycin treatment in pediatric patients with cystic fibrosis: Long-term outcomes related to macrolide resistance and pulmonary function
    • Tramper-Stranders GA, Wolfs TF, Fleer A, et al. Maintenance azithromycin treatment in pediatric patients with cystic fibrosis: long-term outcomes related to macrolide resistance and pulmonary function. Pediatr Infect Dis J. 2007;26:8-12.
    • (2007) Pediatr Infect Dis J , vol.26 , pp. 8-12
    • Tramper-Stranders, G.A.1    Wolfs, T.F.2    Fleer, A.3
  • 72
    • 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;303:1707-1715.
    • (2010) JAMA , vol.303 , pp. 1707-1715
    • Saiman, L.1    Anstead, M.2    Mayer-Hamblett, N.3
  • 73
    • 11044227865 scopus 로고    scopus 로고
    • Established cell lines used in cystic fibrosis research
    • Gruenert DC, Willems M, Cassiman JJ, et al. Established cell lines used in cystic fibrosis research. J Cyst Fibros. 2004;3 Suppl 2:191-196.
    • (2004) J Cyst Fibros , vol.2 , Issue.3 SUPPL. , pp. 191-196
    • Gruenert, D.C.1    Willems, M.2    Cassiman, J.J.3
  • 75
    • 0032081281 scopus 로고    scopus 로고
    • Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells
    • DiMango E, Ratner AJ, Bryan R, et al. Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells. J Clin Invest. 1998; 101(11):2598-2605.
    • (1998) J Clin Invest , vol.101 , Issue.11 , pp. 2598-2605
    • Dimango, E.1    Ratner, A.J.2    Bryan, R.3
  • 76
    • 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(3):L493-L502.
    • (2001) Am J Physiol Lung Cell Mol Physiol , vol.280 , Issue.3
    • Kube, D.1    Sontich, U.2    Fletcher, D.3
  • 77
    • 0030776192 scopus 로고    scopus 로고
    • Altered cytokine production by cystic fibrosis tracheal gland serous cells
    • Kammouni W, Figarella C, Marchand S, et al. Altered cytokine production by cystic fibrosis tracheal gland serous cells. Infect Immun. 1997;65(12):5176-5183.
    • (1997) Infect Immun , vol.65 , Issue.12 , pp. 5176-5183
    • Kammouni, W.1    Figarella, C.2    Marchand, S.3
  • 78
    • 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(1): L71-L78.
    • (2001) Am J Physiol Lung Cell Mol Physiol , vol.281 , Issue.1
    • Weber, A.J.1    Soong, G.2    Bryan, R.3
  • 79
    • 34247891494 scopus 로고    scopus 로고
    • Induction of IL-6 gene expression in a CF bronchial epithelial cell line by Pseudomonas aeruginosa is dependent on transcription factors belonging to the Sp1 superfamily
    • Borgatti M, Bezzerri V, Mancini I, et al. Induction of IL-6 gene expression in a CF bronchial epithelial cell line by Pseudomonas aeruginosa is dependent on transcription factors belonging to the Sp1 superfamily. Biochem Biophys Res Commun. 2007;357(4): 977-983.
    • (2007) Biochem Biophys Res Commun , vol.357 , Issue.4 , pp. 977-983
    • Borgatti, M.1    Bezzerri, V.2    Mancini, I.3
  • 80
    • 0037308173 scopus 로고    scopus 로고
    • Signaling intermediates required for NF-kappa B activation and IL-8 expression in CF bronchial epithelial cells
    • Li J, Johnson XD, Iazvovskaia S, et al. Signaling intermediates required for NF-kappa B activation and IL-8 expression in CF bronchial epithelial cells. Am J Physiol Lung Cell Mol Physiol. 2003;284(2):L307-L315.
    • (2003) Am J Physiol Lung Cell Mol Physiol , vol.284 , Issue.2
    • Li, J.1    Johnson, X.D.2    Iazvovskaia, S.3
  • 81
    • 57649158936 scopus 로고    scopus 로고
    • Highlights of a workshop to discuss targeting inflammation in cystic fibrosis
    • Banner KH, De Jonge H, Elborn S, et al. Highlights of a workshop to discuss targeting inflammation in cystic fibrosis. J Cyst Fibros. 2009;8(1):1-8.
    • (2009) J Cyst Fibros , vol.8 , Issue.1 , pp. 1-8
    • Banner, K.H.1    de Jonge, H.2    Elborn, S.3
  • 82
    • 0033144638 scopus 로고    scopus 로고
    • Murine submucosal glands are clonally derived and show a cystic fibrosis gene-dependent distribution pattern
    • Borthwick DW, West JD, Keighren MA, et al. Murine submucosal glands are clonally derived and show a cystic fibrosis gene-dependent distribution pattern. Am J Respir Cell Mol Biol. 1999;20(6):1181-1189.
    • (1999) Am J Respir Cell Mol Biol , vol.20 , Issue.6 , pp. 1181-1189
    • Borthwick, D.W.1    West, J.D.2    Keighren, M.A.3
  • 83
    • 0019215134 scopus 로고
    • The distribution and structure of cells in the tracheal epithelium of the mouse
    • Pack RJ, Al-Ugaily LH, Morris G, et al. The distribution and structure of cells in the tracheal epithelium of the mouse. Cell Tissue Res. 1980;208(1):65-84.
    • (1980) Cell Tissue Res , vol.208 , Issue.1 , pp. 65-84
    • Pack, R.J.1    Al-Ugaily, L.H.2    Morris, G.3
  • 84
    • 11044233752 scopus 로고    scopus 로고
    • Animal models of cystic fibrosis
    • Scholte BJ, Davidson DJ, Wilke M, et al. Animal models of cystic fibrosis. J Cyst Fibros. 2004;3 Suppl 2:183-190.
    • (2004) J Cyst Fibros , vol.2 , Issue.3 SUPPL. , pp. 183-190
    • Scholte, B.J.1    Davidson, D.J.2    Wilke, M.3
  • 85
    • 0030727615 scopus 로고    scopus 로고
    • Towards an ovine model of cystic fibrosis
    • Harris A. Towards an ovine model of cystic fibrosis. Hum Mol Genet. 1997;6(13):2191-2194.
    • (1997) Hum Mol Genet , vol.6 , Issue.13 , pp. 2191-2194
    • Harris, A.1
  • 86
    • 41849102053 scopus 로고    scopus 로고
    • Adeno-associated virus-targeted disruption of the CFTR gene in cloned ferrets
    • Sun X, Yan Z, Yi Y, et al. Adeno-associated virus-targeted disruption of the CFTR gene in cloned ferrets. J Clin Invest. 2008;118(4):1578-1583.
    • (2008) J Clin Invest , vol.118 , Issue.4 , pp. 1578-1583
    • Sun, X.1    Yan, Z.2    Yi, Y.3
  • 87
    • 52949154301 scopus 로고    scopus 로고
    • Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs
    • Rogers CS, Stoltz DA, Meyerholz DK, et al. Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. Science. 26 2008;321(5897):1837-1841.
    • (2008) Science. 26 , vol.321 , Issue.5897 , pp. 1837-1841
    • Rogers, C.S.1    Stoltz, D.A.2    Meyerholz, D.K.3
  • 88
    • 41849148289 scopus 로고    scopus 로고
    • Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer
    • Rogers CS, Hao Y, Rokhlina T, et al. Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer. J Clin Invest. 2008;118(4):1571-1577.
    • (2008) J Clin Invest , vol.118 , Issue.4 , pp. 1571-1577
    • Rogers, C.S.1    Hao, Y.2    Rokhlina, T.3
  • 90
    • 77952974496 scopus 로고    scopus 로고
    • Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth
    • 29ra31
    • 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;2:29ra31.
    • (2010) Sci Transl Med , vol.2
    • Stoltz, D.A.1    Meyerholz, D.K.2    Pezzulo, A.A.3
  • 92
    • 51349100398 scopus 로고    scopus 로고
    • The prevalence of cystic fibrosis in the European Union
    • Farrell PM. The prevalence of cystic fibrosis in the European Union. J Cyst Fibros. 2008;7(5):450-453.
    • (2008) J Cyst Fibros , vol.7 , Issue.5 , pp. 450-453
    • Farrell, P.M.1
  • 93
    • 77949728437 scopus 로고    scopus 로고
    • Comparative demographics of the European cystic fibrosis population: A cross-sectional database analysis
    • McCormick J, Mehta G, Olesen HV, et al. Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet. 20 2010;375(9719):1007-1013.
    • (2010) Lancet. 20 , vol.375 , Issue.9719 , pp. 1007-1013
    • McCormick, J.1    Mehta, G.2    Olesen, H.V.3
  • 94
    • 0030748946 scopus 로고    scopus 로고
    • Enhanced drug metabolism in young children with cystic f ibrosis
    • Parker AC, Pritchard P, Preston T, et al. Enhanced drug metabolism in young children with cystic f ibrosis. Arch Dis Child. 1997;77(3):239-241.
    • (1997) Arch Dis Child , vol.77 , Issue.3 , pp. 239-241
    • Parker, A.C.1    Pritchard, P.2    Preston, T.3
  • 95
    • 0023909666 scopus 로고
    • Clinical pharmacology of antibiotics and other drugs in cystic fibrosis
    • Prandota J. Clinical pharmacology of antibiotics and other drugs in cystic fibrosis. Drugs. 1988;35(5):542-578.
    • (1988) Drugs , vol.35 , Issue.5 , pp. 542-578
    • Prandota, J.1
  • 96
    • 0027356041 scopus 로고
    • Hepatic drug metabolism in cystic fibrosis: Recent developments and future directions
    • Kearns GL. Hepatic drug metabolism in cystic fibrosis: recent developments and future directions. Ann Pharmacother. 1993;27(1):74-79.
    • (1993) Ann Pharmacother , vol.27 , Issue.1 , pp. 74-79
    • Kearns, G.L.1
  • 97
    • 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. 28 2002;360(9338):978-984.
    • (2002) Lancet. 28 , vol.360 , Issue.9338 , pp. 978-984
    • Equi, A.1    Balfour-Lynn, I.M.2    Bush, A.3
  • 98
    • 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
  • 99
    • 56049116219 scopus 로고    scopus 로고
    • Improvements in lung function outcomes in children with cystic fibrosis are associated with better nutrition, fewer chronic pseudomonas aeruginosa infections, and dornase alfa use
    • McPhail GL, Acton JD, Fenchel MC, et al. Improvements in lung function outcomes in children with cystic fibrosis are associated with better nutrition, fewer chronic pseudomonas aeruginosa infections, and dornase alfa use. J Pediatr. 2008;153(6):752-757.
    • (2008) J Pediatr , vol.153 , Issue.6 , pp. 752-757
    • McPhail, G.L.1    Acton, J.D.2    Fenchel, M.C.3
  • 100
    • 2442480846 scopus 로고    scopus 로고
    • Pulmonary exacerbations in cystic fibrosis
    • Rabin HR, Butler SM, Wohl ME, et al. Pulmonary exacerbations in cystic fibrosis. Pediatr Pulmonol. 2004;37(5):400-406.
    • (2004) Pediatr Pulmonol , vol.37 , Issue.5 , pp. 400-406
    • Rabin, H.R.1    Butler, S.M.2    Wohl, M.E.3
  • 101
    • 34247390062 scopus 로고    scopus 로고
    • Association between pulmonary function and sputum biomarkers in cystic fibrosis
    • Mayer-Hamblett N, Aitken ML, Accurso FJ, et al. Association between pulmonary function and sputum biomarkers in cystic fibrosis. Am J Respir Crit Care Med. 2007;175(8):822-828.
    • (2007) Am J Respir Crit Care Med , vol.175 , Issue.8 , pp. 822-828
    • Mayer-Hamblett, N.1    Aitken, M.L.2    Accurso, F.J.3
  • 102
    • 27144479427 scopus 로고    scopus 로고
    • Development and validation of The Cystic Fibrosis Questionnaire in the United States: A health-related quality-of-life measure for cystic fibrosis
    • Quittner AL, Buu A, Messer MA, et al. Development and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosis. Chest. 2005;128(4):2347-2354.
    • (2005) Chest , vol.128 , Issue.4 , pp. 2347-2354
    • Quittner, A.L.1    Buu, A.2    Messer, M.A.3
  • 103
    • 34548273107 scopus 로고    scopus 로고
    • Advancing outcome measures for the new era of drug development in cystic fibrosis
    • Mayer-Hamblett N, Ramsey BW, Kronmal RA. Advancing outcome measures for the new era of drug development in cystic fibrosis. Proc Am Thorac Soc. 2007;4(4):370-377.
    • (2007) Proc Am Thorac Soc , vol.4 , Issue.4 , pp. 370-377
    • Mayer-Hamblett, N.1    Ramsey, B.W.2    Kronmal, R.A.3
  • 104
    • 0032872906 scopus 로고    scopus 로고
    • Cystic fibrosis: Inflammatory response to infection with Burkholderia cepacia and Pseudomonas aeruginosa
    • Hendry J, Elborn JS, Nixon L, et al. Cystic fibrosis: inflammatory response to infection with Burkholderia cepacia and Pseudomonas aeruginosa. Eur Respir J. 1999;14(2):435-438.
    • (1999) Eur Respir J , vol.14 , Issue.2 , pp. 435-438
    • Hendry, J.1    Elborn, J.S.2    Nixon, L.3
  • 105
    • 0030444674 scopus 로고    scopus 로고
    • Relationship between disease severity and inflammatory markers in cystic fibrosis
    • Koller DY, Gotz M, Wojnarowski C, et al. Relationship between disease severity and inflammatory markers in cystic fibrosis. Arch Dis Child. 1996;75(6):498-501.
    • (1996) Arch Dis Child , vol.75 , Issue.6 , pp. 498-501
    • Koller, D.Y.1    Gotz, M.2    Wojnarowski, C.3
  • 106
    • 0025860015 scopus 로고
    • Plasma tumour necrosis factor alpha in cystic fibrosis
    • Norman D, Elborn JS, Cordon SM, et al. Plasma tumour necrosis factor alpha in cystic fibrosis. Thorax. 1991;46(2):91-95.
    • (1991) Thorax , vol.46 , Issue.2 , pp. 91-95
    • Norman, D.1    Elborn, J.S.2    Cordon, S.M.3
  • 107
    • 0026036665 scopus 로고
    • Circulating levels of tumor necrosis factor and interleukin-1 in cystic fibrosis
    • Brown MA, Morgan WJ, Finley PR, et al. Circulating levels of tumor necrosis factor and interleukin-1 in cystic fibrosis. Pediatr Pulmonol. 1991;10(2):86-91.
    • (1991) Pediatr Pulmonol , vol.10 , Issue.2 , pp. 86-91
    • Brown, M.A.1    Morgan, W.J.2    Finley, P.R.3
  • 108
    • 4444364876 scopus 로고    scopus 로고
    • Anti-neutrophil cytoplasmatic antibodies and lung disease in cystic fibrosis
    • Dorlochter L, Carlsson M, Olafsdottir EJ, et al. Anti-neutrophil cytoplasmatic antibodies and lung disease in cystic fibrosis. J Cyst Fibros. 2004;3(3):179-183.
    • (2004) J Cyst Fibros , vol.3 , Issue.3 , pp. 179-183
    • Dorlochter, L.1    Carlsson, M.2    Olafsdottir, E.J.3
  • 109
    • 21444461227 scopus 로고    scopus 로고
    • Cytokine levels in sputum of cystic fibrosis patients before and after antibiotic therapy
    • Colombo C, Costantini D, Rocchi A, et al. Cytokine levels in sputum of cystic fibrosis patients before and after antibiotic therapy. Pediatr Pulmonol. 2005;40(1):15-21.
    • (2005) Pediatr Pulmonol , vol.40 , Issue.1 , pp. 15-21
    • Colombo, C.1    Costantini, D.2    Rocchi, A.3
  • 110
    • 69549105799 scopus 로고    scopus 로고
    • Serum matrix metalloproteinases in adult CF patients: Relation to pulmonary exacerbation
    • Roderfeld M, Rath T, Schulz R, et al. Serum matrix metalloproteinases in adult CF patients: relation to pulmonary exacerbation. J Cyst Fibros. 2009;8(5):338-347.
    • (2009) J Cyst Fibros , vol.8 , Issue.5 , pp. 338-347
    • Roderfeld, M.1    Rath, T.2    Schulz, R.3
  • 111
    • 0041878830 scopus 로고    scopus 로고
    • Inflammatory markers in cystic fibrosis patients with transmissible Pseudomonas aeruginosa
    • Jones AM, Martin L, Bright-Thomas RJ, et al. Inflammatory markers in cystic fibrosis patients with transmissible Pseudomonas aeruginosa. Eur Respir J. 2003;22(3):503-506.
    • (2003) Eur Respir J , vol.22 , Issue.3 , pp. 503-506
    • Jones, A.M.1    Martin, L.2    Bright-Thomas, R.J.3
  • 112
    • 0026524149 scopus 로고
    • Modif ication of some markers of inflammation during treatment for acute respiratory exacerbation in cystic fibrosis
    • Valletta EA, Rigo A, Bonazzi L, et al. Modif ication of some markers of inflammation during treatment for acute respiratory exacerbation in cystic fibrosis. Acta Paediatr. 1992;81(3): 227-230.
    • (1992) Acta Paediatr , vol.81 , Issue.3 , pp. 227-230
    • Valletta, E.A.1    Rigo, A.2    Bonazzi, L.3
  • 113
    • 33947263039 scopus 로고    scopus 로고
    • The relationship of clinical and inflammatory markers to outcome in stable patients with cystic fibrosis
    • Downey DG, Martin SL, Dempster M, et al. The relationship of clinical and inflammatory markers to outcome in stable patients with cystic fibrosis. Pediatr Pulmonol. 2007;42(3):216-220.
    • (2007) Pediatr Pulmonol , vol.42 , Issue.3 , pp. 216-220
    • Downey, D.G.1    Martin, S.L.2    Dempster, M.3
  • 114
    • 0028177145 scopus 로고
    • Plasma interleukin-1 alpha and beta, tumor necrosis factor-alpha, and lipopolysaccharide concentrations during pulmonary exacerbations of cystic fibrosis
    • Wilmott RW, Frenzke M, Kociela V, et al. Plasma interleukin-1 alpha and beta, tumor necrosis factor-alpha, and lipopolysaccharide concentrations during pulmonary exacerbations of cystic fibrosis. Pediatr Pulmonol. 1994;18(1):21-27.
    • (1994) Pediatr Pulmonol , vol.18 , Issue.1 , pp. 21-27
    • Wilmott, R.W.1    Frenzke, M.2    Kociela, V.3
  • 115
    • 0034047708 scopus 로고    scopus 로고
    • Serum vascular endothelial growth factor is elevated in cystic fibrosis and decreases with treatment of acute pulmonary exacerbation
    • McColley SA, Stellmach V, Boas SR, et al. Serum vascular endothelial growth factor is elevated in cystic fibrosis and decreases with treatment of acute pulmonary exacerbation. Am J Respir Crit Care Med. 2000;161(6):1877-1880.
    • (2000) Am J Respir Crit Care Med , vol.161 , Issue.6 , pp. 1877-1880
    • McColley, S.A.1    Stellmach, V.2    Boas, S.R.3
  • 116
    • 0027274608 scopus 로고
    • Interleukin-8 concentrations are elevated in bronchoalveolar lavage, sputum, and sera of children with cystic fibrosis
    • Dean TP, Dai Y, Shute JK, et al. Interleukin-8 concentrations are elevated in bronchoalveolar lavage, sputum, and sera of children with cystic fibrosis. Pediatr Res. 1993;34(2):159-161.
    • (1993) Pediatr Res , vol.34 , Issue.2 , pp. 159-161
    • Dean, T.P.1    Dai, Y.2    Shute, J.K.3
  • 117
    • 0027173625 scopus 로고
    • Serum interleukin-1 alpha and soluble interleukin-2 receptor concentrations in cystic fibrosis
    • Greally P, Hussain MJ, Vergani D, et al. Serum interleukin-1 alpha and soluble interleukin-2 receptor concentrations in cystic fibrosis. Arch Dis Child. 1993;68(6):785-787.
    • (1993) Arch Dis Child , vol.68 , Issue.6 , pp. 785-787
    • Greally, P.1    Hussain, M.J.2    Vergani, D.3
  • 118
    • 0027600805 scopus 로고
    • Cytokines in sputum and serum from patients with cystic fibrosis and chronic Pseudomonas aeruginosa infection as markers of destructive inflammation in the lungs
    • Kronborg G, Hansen MB, Svenson M, et al. Cytokines in sputum and serum from patients with cystic fibrosis and chronic Pseudomonas aeruginosa infection as markers of destructive inflammation in the lungs. Pediatr Pulmonol. 1993;15(5):292-297.
    • (1993) Pediatr Pulmonol , vol.15 , Issue.5 , pp. 292-297
    • Kronborg, G.1    Hansen, M.B.2    Svenson, M.3
  • 119
    • 0032720873 scopus 로고    scopus 로고
    • Human neutrophil lipocalin, a highly specific marker for acute exacerbation in cystic fibrosis
    • Eichler I, Nilsson M, Rath R, et al. Human neutrophil lipocalin, a highly specific marker for acute exacerbation in cystic fibrosis. Eur Respir J. 1999;14(5):1145-1149.
    • (1999) Eur Respir J , vol.14 , Issue.5 , pp. 1145-1149
    • Eichler, I.1    Nilsson, M.2    Rath, R.3
  • 120
    • 34547731349 scopus 로고    scopus 로고
    • Broncoscopy Study Group The effect of treatment of cystic fibrosis pulmonary exacerbations on airways and systemic inflammation
    • Downey DG, Brockbank S, Martin SL, et al. Broncoscopy Study Group The effect of treatment of cystic fibrosis pulmonary exacerbations on airways and systemic inflammation. Pediatr Pulmonol. 2007;42(8):729-735.
    • (2007) Pediatr Pulmonol , vol.42 , Issue.8 , pp. 729-735
    • Downey, D.G.1    Brockbank, S.2    Martin, S.L.3
  • 121
    • 34548282581 scopus 로고    scopus 로고
    • Sputum biomarkers of inflammation in cystic f ibrosis lung disease
    • Sagel SD, Chmiel JF, Konstan MW. Sputum biomarkers of inflammation in cystic f ibrosis lung disease. Proc Am Thorac Soc. 2007;4(4):406-417.
    • (2007) Proc Am Thorac Soc , vol.4 , Issue.4 , pp. 406-417
    • Sagel, S.D.1    Chmiel, J.F.2    Konstan, M.W.3
  • 122
    • 0031695344 scopus 로고    scopus 로고
    • Aerosol delivery and safety of recombinant human deoxyribonuclease in young children with cystic fibrosis: A bronchoscopic study. Pulmozyme Pediatric
    • Wagener JS, Rock MJ, McCubbin MM, et al. Aerosol delivery and safety of recombinant human deoxyribonuclease in young children with cystic fibrosis: a bronchoscopic study. Pulmozyme Pediatric. J Pediatr. 1998;133(4):486-491.
    • (1998) J Pediatr , vol.133 , Issue.4 , pp. 486-491
    • Wagener, J.S.1    Rock, M.J.2    McCubbin, M.M.3
  • 123
    • 0034790494 scopus 로고    scopus 로고
    • Beclomethasone diproprionate reduced airway inflammation without adrenal suppression in young children with cystic fibrosis: A pilot study
    • Wojtczak HA, Kerby GS, Wagener JS, et al. Beclomethasone diproprionate reduced airway inflammation without adrenal suppression in young children with cystic fibrosis: a pilot study. Pediatr Pulmonol. 2001;32(4):293-302.
    • (2001) Pediatr Pulmonol , vol.32 , Issue.4 , pp. 293-302
    • Wojtczak, H.A.1    Kerby, G.S.2    Wagener, J.S.3
  • 124
    • 59649123905 scopus 로고    scopus 로고
    • Acquisition and eradication of P. aeruginosa in young children with cystic fibrosis
    • Douglas TA, Brennan S, Gard S, et al. Acquisition and eradication of P. aeruginosa in young children with cystic fibrosis. Eur Respir J. 2009;33(2):305-311.
    • (2009) Eur Respir J , vol.33 , Issue.2 , pp. 305-311
    • Douglas, T.A.1    Brennan, S.2    Gard, S.3
  • 125
    • 34347370866 scopus 로고    scopus 로고
    • Duration of treatment effect after tobramycin solution for inhalation in young children with cystic fibrosis
    • Gibson RL, Emerson J, Mayer-Hamblett N, et al. Duration of treatment effect after tobramycin solution for inhalation in young children with cystic fibrosis. Pediatr Pulmonol. 2007;42(7):610-623.
    • (2007) Pediatr Pulmonol , vol.42 , Issue.7 , pp. 610-623
    • Gibson, R.L.1    Emerson, J.2    Mayer-Hamblett, N.3
  • 126
    • 10344264976 scopus 로고    scopus 로고
    • DNA concentrations in BAL fluid of cystic fibrosis patients with early lung disease: Influence of treatment with dornase alpha
    • Ratjen F, Paul K, van Koningsbruggen S, et al. DNA concentrations in BAL fluid of cystic fibrosis patients with early lung disease: influence of treatment with dornase alpha. Pediatr Pulmonol. 2005;39(1): 1-4.
    • (2005) Pediatr Pulmonol , vol.39 , Issue.1 , pp. 1-4
    • Ratjen, F.1    Paul, K.2    van Koningsbruggen, S.3
  • 127
    • 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):719-725.
    • (2004) Am J Respir Crit Care Med , vol.169 , Issue.6 , pp. 719-725
    • Paul, K.1    Rietschel, E.2    Ballmann, M.3
  • 128
    • 0036845446 scopus 로고    scopus 로고
    • Matrix metalloproteases in BAL fluid of patients with cystic fibrosis and their modulation by treatment with dornase alpha
    • Ratjen F, Hartog CM, Paul K, et al. Matrix metalloproteases in BAL fluid of patients with cystic fibrosis and their modulation by treatment with dornase alpha. Thorax. 2002;57(11):930-934.
    • (2002) Thorax , vol.57 , Issue.11 , pp. 930-934
    • Ratjen, F.1    Hartog, C.M.2    Paul, K.3
  • 129
    • 0025992671 scopus 로고
    • Human neutrophil elastase and elastase/alpha 1-antiprotease complex in cystic fibrosis. Comparison with interstitial lung disease and evaluation of the effect of intravenously administered antibiotic therapy
    • Meyer KC, Lewandoski JR, Zimmerman JJ, et al. Human neutrophil elastase and elastase/alpha 1-antiprotease complex in cystic fibrosis. Comparison with interstitial lung disease and evaluation of the effect of intravenously administered antibiotic therapy. Am Rev Respir Dis. 1991;144(3 Pt 1):580-585.
    • (1991) Am Rev Respir Dis , vol.144 , Issue.3 PART 1 , pp. 580-585
    • Meyer, K.C.1    Lewandoski, J.R.2    Zimmerman, J.J.3
  • 130
    • 0025966879 scopus 로고
    • Aerosol alpha 1-antitrypsin treatment for cystic fibrosis
    • McElvaney NG, Hubbard RC, Birrer P, et al. Aerosol alpha 1-antitrypsin treatment for cystic fibrosis. Lancet. 1991;337(8738):392-394.
    • (1991) Lancet , vol.337 , Issue.8738 , pp. 392-394
    • McElvaney, N.G.1    Hubbard, R.C.2    Birrer, P.3
  • 131
    • 0026474443 scopus 로고
    • Modulation of airway inflammation in cystic fibrosis. In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor
    • McElvaney NG, Nakamura H, Birrer P, et al. Modulation of airway inflammation in cystic fibrosis. In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor. J Clin Invest. 1992;90(4):1296-1301.
    • (1992) J Clin Invest , vol.90 , Issue.4 , pp. 1296-1301
    • McElvaney, N.G.1    Nakamura, H.2    Birrer, P.3
  • 132
    • 2942716728 scopus 로고    scopus 로고
    • Breath condensate pH in children with cystic fibrosis and asthma: A new noninvasive marker of airway inflammation?
    • Carpagnano GE, Barnes PJ, Francis J, et al. Breath condensate pH in children with cystic fibrosis and asthma: a new noninvasive marker of airway inflammation? Chest. 2004;125(6):2005-2010.
    • (2004) Chest , vol.125 , Issue.6 , pp. 2005-2010
    • Carpagnano, G.E.1    Barnes, P.J.2    Francis, J.3
  • 133
    • 70349518914 scopus 로고    scopus 로고
    • Exhaled breath condensate MMP-9 levels in children with bronchiectasis
    • Karakoc GB, Inal A, Yilmaz M, et al. Exhaled breath condensate MMP-9 levels in children with bronchiectasis. Pediatr Pulmonol. 2009;44(10):1010-1016.
    • (2009) Pediatr Pulmonol , vol.44 , Issue.10 , pp. 1010-1016
    • Karakoc, G.B.1    Inal, A.2    Yilmaz, M.3
  • 134
    • 54249153391 scopus 로고    scopus 로고
    • Biomarkers in exhaled breath condensate indicate presence and severity of cystic fibrosis in children
    • Robroeks CM, Rosias PP, van Vliet D, et al. Biomarkers in exhaled breath condensate indicate presence and severity of cystic fibrosis in children. Pediatr Allergy Immunol. 2008;19(7):652-659.
    • (2008) Pediatr Allergy Immunol , vol.19 , Issue.7 , pp. 652-659
    • Robroeks, C.M.1    Rosias, P.P.2    van Vliet, D.3
  • 135
    • 0034090782 scopus 로고    scopus 로고
    • Nasal and exhaled nitric oxide is reduced in adult patients with cystic fibrosis and does not correlate with cystic fibrosis genotype
    • Thomas SR, Kharitonov SA, Scott SF, et al. Nasal and exhaled nitric oxide is reduced in adult patients with cystic fibrosis and does not correlate with cystic fibrosis genotype. Chest. 2000;117(4):1085-1089.
    • (2000) Chest , vol.117 , Issue.4 , pp. 1085-1089
    • Thomas, S.R.1    Kharitonov, S.A.2    Scott, S.F.3
  • 136
    • 28344446191 scopus 로고    scopus 로고
    • Biomarkers of neutrophilic inflammation in exhaled air of cystic fibrosis children with bacterial airway infections
    • Bodini A, D'Orazio C, Peroni D, et al. Biomarkers of neutrophilic inflammation in exhaled air of cystic fibrosis children with bacterial airway infections. Pediatr Pulmonol. 2005;40(6):494-499.
    • (2005) Pediatr Pulmonol , vol.40 , Issue.6 , pp. 494-499
    • Bodini, A.1    D'orazio, C.2    Peroni, D.3
  • 137
    • 11944267738 scopus 로고    scopus 로고
    • Exhaled breath condensate pH and exhaled nitric oxide in allergic asthma and in cystic fibrosis
    • Ojoo JC, Mulrennan SA, Kastelik JA, et al. Exhaled breath condensate pH and exhaled nitric oxide in allergic asthma and in cystic fibrosis. Thorax. 2005;60(1):22-26.
    • (2005) Thorax , vol.60 , Issue.1 , pp. 22-26
    • Ojoo, J.C.1    Mulrennan, S.A.2    Kastelik, J.A.3
  • 138
    • 0036847496 scopus 로고    scopus 로고
    • Airways in cystic fibrosis are acidified: Detection by exhaled breath condensate
    • Tate S, MacGregor G, Davis M, et al. Airways in cystic fibrosis are acidified: detection by exhaled breath condensate. Thorax. 2002;57(11):926-929.
    • (2002) Thorax , vol.57 , Issue.11 , pp. 926-929
    • Tate, S.1    Macgregor, G.2    Davis, M.3
  • 139
    • 69749128729 scopus 로고    scopus 로고
    • Exhaled breath condensate pH and ammonia in cystic fibrosis and response to treatment of acute pulmonary exacerbations
    • Newport S, Amin N, Dozor AJ. Exhaled breath condensate pH and ammonia in cystic fibrosis and response to treatment of acute pulmonary exacerbations. Pediatr Pulmonol. 2009;44(9):866-872.
    • (2009) Pediatr Pulmonol , vol.44 , Issue.9 , pp. 866-872
    • Newport, S.1    Amin, N.2    Dozor, A.J.3
  • 140
    • 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. 15, 2003;168(12):1471-1475.
    • (2003) Am J Respir Crit Care Med. 15 , vol.168 , Issue.12 , pp. 1471-1475
    • Ordonez, C.L.1    Henig, N.R.2    Mayer-Hamblett, N.3
  • 141
    • 0022481020 scopus 로고
    • Conformity of bacterial growth in sputum and contamination free endobronchial samples in patients with cystic fibrosis
    • Gilljam H, Malmborg AS, Strandvik B. Conformity of bacterial growth in sputum and contamination free endobronchial samples in patients with cystic fibrosis. Thorax. 1986;41(8):641-646.
    • (1986) Thorax , vol.41 , Issue.8 , pp. 641-646
    • Gilljam, H.1    Malmborg, A.S.2    Strandvik, B.3
  • 142
    • 6444244008 scopus 로고    scopus 로고
    • Sputum versus bronchoscopy for diagnosis of Pseudomonas aeruginosa biofilms in cystic fibrosis
    • Aaron SD, Kottachchi D, Ferris WJ, et al. Sputum versus bronchoscopy for diagnosis of Pseudomonas aeruginosa biofilms in cystic fibrosis. Eur Respir J. 2004;24(4):631-637.
    • (2004) Eur Respir J , vol.24 , Issue.4 , pp. 631-637
    • Aaron, S.D.1    Kottachchi, D.2    Ferris, W.J.3
  • 143
    • 0035888715 scopus 로고    scopus 로고
    • Airway inflammation in children with cystic fibrosis and healthy children assessed by sputum induction
    • Sagel SD, Kapsner R, Osberg I, et al. Airway inflammation in children with cystic fibrosis and healthy children assessed by sputum induction. Am J Respir Crit Care Med. 15 2001;164(8 Pt 1):1425-1431.
    • (2001) Am J Respir Crit Care Med. 15 , vol.164 , Issue.8 PART 1 , pp. 1425-1431
    • Sagel, S.D.1    Kapsner, R.2    Osberg, I.3
  • 144
    • 0037385234 scopus 로고    scopus 로고
    • Safety and success of exhaled breath condensate collection in asthma
    • Baraldi E, Ghiro L, Piovan V, et al. Safety and success of exhaled breath condensate collection in asthma. Arch Dis Child. 2003;88(4):358-360.
    • (2003) Arch Dis Child , vol.88 , Issue.4 , pp. 358-360
    • Baraldi, E.1    Ghiro, L.2    Piovan, V.3
  • 145
    • 30844473744 scopus 로고    scopus 로고
    • Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis
    • de Jong PA, Lindblad A, Rubin L, et al. Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax. 2006;61(1):80-85.
    • (2006) Thorax , vol.61 , Issue.1 , pp. 80-85
    • de Jong, P.A.1    Lindblad, A.2    Rubin, L.3
  • 146
    • 0347766074 scopus 로고    scopus 로고
    • Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis
    • de Jong PA, Nakano Y, Lequin MH, et al. Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis. Eur Respir J. 2004;23(1):93-97.
    • (2004) Eur Respir J , vol.23 , Issue.1 , pp. 93-97
    • de Jong, P.A.1    Nakano, Y.2    Lequin, M.H.3
  • 147
    • 30344460714 scopus 로고    scopus 로고
    • Computed tomography correlates with pulmonary exacerbations in children with cystic fibrosis
    • Brody AS, Sucharew H, Campbell JD, et al. Computed tomography correlates with pulmonary exacerbations in children with cystic fibrosis. Am J Respir Crit Care Med. 2005;172(9):1128-1132.
    • (2005) Am J Respir Crit Care Med , vol.172 , Issue.9 , pp. 1128-1132
    • Brody, A.S.1    Sucharew, H.2    Campbell, J.D.3
  • 148
    • 0035021689 scopus 로고    scopus 로고
    • Use of computerized tomography and chest x-rays in evaluating efficacy of aerosolized recombinant human DNase in cystic fibrosis patients younger than age 5 years: A preliminary study
    • Nasr SZ, Kuhns LR, Brown RW, et al. Use of computerized tomography and chest x-rays in evaluating efficacy of aerosolized recombinant human DNase in cystic fibrosis patients younger than age 5 years: a preliminary study. Pediatr Pulmonol. 2001;31(5):377-382.
    • (2001) Pediatr Pulmonol , vol.31 , Issue.5 , pp. 377-382
    • Nasr, S.Z.1    Kuhns, L.R.2    Brown, R.W.3
  • 149
    • 27144512698 scopus 로고    scopus 로고
    • Dornase alfa reduces air trapping in children with mild cystic fibrosis lung disease: A quantitative analysis
    • Robinson TE, Goris ML, Zhu HJ, et al. Dornase alfa reduces air trapping in children with mild cystic fibrosis lung disease: a quantitative analysis. Chest. 2005;128(4):2327-2335.
    • (2005) Chest , vol.128 , Issue.4 , pp. 2327-2335
    • Robinson, T.E.1    Goris, M.L.2    Zhu, H.J.3
  • 150
    • 12144252928 scopus 로고    scopus 로고
    • The prediction of small airway dimensions using computed tomography
    • Nakano Y, Wong JC, de Jong PA, et al. The prediction of small airway dimensions using computed tomography. Am J Respir Crit Care Med. 2005;171(2):142-146.
    • (2005) Am J Respir Crit Care Med , vol.171 , Issue.2 , pp. 142-146
    • Nakano, Y.1    Wong, J.C.2    de Jong, P.A.3
  • 151
    • 12344263724 scopus 로고    scopus 로고
    • Chronic obstructive pulmonary disease: Thin-section CT measurement of airway wall thickness and lung attenuation
    • Orlandi I, Moroni C, Camiciottoli G, et al. Chronic obstructive pulmonary disease: thin-section CT measurement of airway wall thickness and lung attenuation. Radiology. 2005;234(2):604-610.
    • (2005) Radiology , vol.234 , Issue.2 , pp. 604-610
    • Orlandi, I.1    Moroni, C.2    Camiciottoli, G.3
  • 152
    • 21744447612 scopus 로고    scopus 로고
    • Changes in airway dimensions on computed tomography scans of children with cystic fibrosis
    • de Jong PA, Nakano Y, Hop WC, et al. Changes in airway dimensions on computed tomography scans of children with cystic fibrosis. Am J Respir Crit Care Med. 2005;172(2):218-224.
    • (2005) Am J Respir Crit Care Med , vol.172 , Issue.2 , pp. 218-224
    • de Jong, P.A.1    Nakano, Y.2    Hop, W.C.3
  • 153
    • 2442585161 scopus 로고    scopus 로고
    • Effect of short-term treatment with inhaled corticosteroid on airway wall thickening in asthma
    • Niimi A, Matsumoto H, Amitani R, et al. Effect of short-term treatment with inhaled corticosteroid on airway wall thickening in asthma. Am J Med. 2004;116(11):725-731.
    • (2004) Am J Med , vol.116 , Issue.11 , pp. 725-731
    • Niimi, A.1    Matsumoto, H.2    Amitani, R.3
  • 154
    • 33846967486 scopus 로고    scopus 로고
    • Proton MRI appearance of cystic fibrosis: Comparison to CT
    • Puderbach M, Eichinger M, Gahr J, et al. Proton MRI appearance of cystic fibrosis: comparison to CT. Eur Radiol. 2007;17(3): 716-724.
    • (2007) Eur Radiol , vol.17 , Issue.3 , pp. 716-724
    • Puderbach, M.1    Eichinger, M.2    Gahr, J.3
  • 155
    • 33748452022 scopus 로고    scopus 로고
    • Contrast-enhanced 3D MRI of lung perfusion in children with cystic fibrosis - initial results
    • Eichinger M, Puderbach M, Fink C, et al. Contrast-enhanced 3D MRI of lung perfusion in children with cystic fibrosis - initial results. Eur Radiol. 2006;16(10):2147-2152.
    • (2006) Eur Radiol , vol.16 , Issue.10 , pp. 2147-2152
    • Eichinger, M.1    Puderbach, M.2    Fink, C.3
  • 156
    • 23344438882 scopus 로고    scopus 로고
    • Assessment of hemodynamic changes in the systemic and pulmonary arterial circulation in patients with cystic fibrosis using phase-contrast MRI
    • Ley S, Puderbach M, Fink C, et al. Assessment of hemodynamic changes in the systemic and pulmonary arterial circulation in patients with cystic fibrosis using phase-contrast MRI. Eur Radiol. 2005;15(8):1575-1580.
    • (2005) Eur Radiol , vol.15 , Issue.8 , pp. 1575-1580
    • Ley, S.1    Puderbach, M.2    Fink, C.3
  • 157
    • 34548203879 scopus 로고    scopus 로고
    • Magnetic resonance imaging of the lung in cystic f ibrosis
    • Altes TA, Eichinger M, Puderbach M. Magnetic resonance imaging of the lung in cystic f ibrosis. Proc Am Thorac Soc. 2007;4(4):321-327.
    • (2007) Proc Am Thorac Soc , vol.4 , Issue.4 , pp. 321-327
    • Altes, T.A.1    Eichinger, M.2    Puderbach, M.3
  • 158
    • 27244452469 scopus 로고    scopus 로고
    • Hyperpolarized HHe 3 MRI of the lung in cystic fibrosis: Assessment at baseline and after bronchodilator and airway clearance treatment
    • Mentore K, Froh DK, de Lange EE, et al. Hyperpolarized HHe 3 MRI of the lung in cystic fibrosis: assessment at baseline and after bronchodilator and airway clearance treatment. Acad Radiol. 2005;12(11):1423-1429.
    • (2005) Acad Radiol , vol.12 , Issue.11 , pp. 1423-1429
    • Mentore, K.1    Froh, D.K.2    de Lange, E.E.3
  • 159
    • 34548246514 scopus 로고    scopus 로고
    • Use of lung imaging studies as outcome measures for development of new therapies in cystic fibrosis
    • Ramsey BW. Use of lung imaging studies as outcome measures for development of new therapies in cystic fibrosis. Proc Am Thorac Soc. 2007;4(4):359-363.
    • (2007) Proc Am Thorac Soc , vol.4 , Issue.4 , pp. 359-363
    • Ramsey, B.W.1
  • 162
    • 70350506537 scopus 로고    scopus 로고
    • Effect of simvastatin on exhaled nitric oxide and inflammatory markers in sputum in patients with cystic fibrosis
    • Kraynack NC, Xue W, et al. Effect of simvastatin on exhaled nitric oxide and inflammatory markers in sputum in patients with cystic fibrosis. Pediatr Pulmonol. 2008;(31):A300.
    • (2008) Pediatr Pulmonol , Issue.31
    • Kraynack, N.C.1    Xue, W.2
  • 163
    • 70350497079 scopus 로고    scopus 로고
    • Effect of pioglitazone on sputum markers of inflammation in cystic fibrosis
    • Konstan MW, Bucur C, et al. Effect of pioglitazone on sputum markers of inflammation in cystic fibrosis. Pediatr Pulmonol. 2008;(31):A310.
    • (2008) Pediatr Pulmonol , Issue.31
    • Konstan, M.W.1    Bucur, C.2
  • 165
    • 1642493925 scopus 로고    scopus 로고
    • Effect of phosphodiesterase-5 inhibitor, sildenafil (Viagra), in animal models of airways disease
    • Toward TJ, Smith N, Broadley KJ. Effect of phosphodiesterase-5 inhibitor, sildenafil (Viagra), in animal models of airways disease. Am J Respir Crit Care Med. 2004;169(2):227-234.
    • (2004) Am J Respir Crit Care Med , vol.169 , Issue.2 , pp. 227-234
    • Toward, T.J.1    Smith, N.2    Broadley, K.J.3
  • 166
    • 33745196486 scopus 로고    scopus 로고
    • Endosomal hyperacidification in cystic fibrosis is due to defective nitric oxide-cylic GMP signalling cascade
    • Poschet JF, Fazio JA, Timmins GS, et al. Endosomal hyperacidification in cystic fibrosis is due to defective nitric oxide-cylic GMP signalling cascade. EMBO Rep. 2006;7(5):553-559.
    • (2006) EMBO Rep , vol.7 , Issue.5 , pp. 553-559
    • Poschet, J.F.1    Fazio, J.A.2    Timmins, G.S.3
  • 167
    • 34548407741 scopus 로고    scopus 로고
    • Pharmacological modulation of cGMP levels by phosphodiesterase 5 inhibitors as a therapeutic strategy for treatment of respiratory pathology in cystic fibrosis
    • Poschet JF, Timmins GS, Taylor-Cousar JL, et al. Pharmacological modulation of cGMP levels by phosphodiesterase 5 inhibitors as a therapeutic strategy for treatment of respiratory pathology in cystic fibrosis. Am J Physiol Lung Cell Mol Physiol. 2007;293(3):L712-L719.
    • (2007) Am J Physiol Lung Cell Mol Physiol , vol.293 , Issue.3
    • Poschet, J.F.1    Timmins, G.S.2    Taylor-Cousar, J.L.3
  • 168
    • 33645229803 scopus 로고    scopus 로고
    • High-dose oral N-acetylcysteine, a glutathione prodrug, modulates inflammation in cystic fibrosis
    • Tirouvanziam R, Conrad CK, Bottiglieri T, et al. High-dose oral N-acetylcysteine, a glutathione prodrug, modulates inflammation in cystic fibrosis. Proc Natl Acad Sci U S A. 2006; 103(12):4628-4633.
    • (2006) Proc Natl Acad Sci U S A , vol.103 , Issue.12 , pp. 4628-4633
    • Tirouvanziam, R.1    Conrad, C.K.2    Bottiglieri, T.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.