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Volumn 47, Issue 7-8, 2014, Pages 539-546

Lung inflammation in cystic fibrosis: Pathogenesis and novel therapies

Author keywords

CFTR; Cystic fibrosis; Cytokines; Inflammation; New therapies

Indexed keywords

ALPHA 1 ANTITRYPSIN; AMITRIPTYLINE; ANTIINFLAMMATORY AGENT; AZITHROMYCIN; CORTICOSTEROID; IBUPROFEN; INTERLEUKIN 1BETA; INTERLEUKIN 6; INTERLEUKIN 8; KB 001A; KB001A; MIGLUSTAT; N 6022; N6022; OMEGA 3 FATTY ACID; SILDENAFIL; UNCLASSIFIED DRUG; CYTOKINE;

EID: 84900315796     PISSN: 00099120     EISSN: 18732933     Source Type: Journal    
DOI: 10.1016/j.clinbiochem.2013.12.020     Document Type: Review
Times cited : (42)

References (110)
  • 1
    • 33845992179 scopus 로고    scopus 로고
    • Evidence for airway surface dehydration as the initiating event in CF airway disease
    • Boucher R.C. Evidence for airway surface dehydration as the initiating event in CF airway disease. J Intern Med 2007, 261:5-16.
    • (2007) J Intern Med , vol.261 , pp. 5-16
    • Boucher, R.C.1
  • 2
    • 27444444705 scopus 로고    scopus 로고
    • Normal and cystic fibrosis airway surface liquid homeostasis. The effects of phasic shear stress and viral infections
    • Tarran R., Button B., Picher M., Paradiso A.M., Ribeiro C.M., Lazarowski E.R., et al. Normal and cystic fibrosis airway surface liquid homeostasis. The effects of phasic shear stress and viral infections. J Biol Chem 2005, 280:35751-35759.
    • (2005) J Biol Chem , vol.280 , pp. 35751-35759
    • Tarran, R.1    Button, B.2    Picher, M.3    Paradiso, A.M.4    Ribeiro, C.M.5    Lazarowski, E.R.6
  • 3
    • 0030970422 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator inverts protein kinase A-mediated regulation of epithelial sodium channel single channel kinetics
    • Stutts M.J., Rossier B.C., Boucher R.C. Cystic fibrosis transmembrane conductance regulator inverts protein kinase A-mediated regulation of epithelial sodium channel single channel kinetics. J Biol Chem 1997, 272:14307-14340.
    • (1997) J Biol Chem , vol.272 , pp. 14307-14340
    • Stutts, M.J.1    Rossier, B.C.2    Boucher, R.C.3
  • 4
    • 0029912194 scopus 로고    scopus 로고
    • Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator
    • Ismailov I.I., Awayda M.S., Jovov B., Berdiev B.K., Fuller C.M., Dedman J.R., et al. Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator. J Biol Chem 1996, 271:4725-4732.
    • (1996) J Biol Chem , vol.271 , pp. 4725-4732
    • Ismailov, I.I.1    Awayda, M.S.2    Jovov, B.3    Berdiev, B.K.4    Fuller, C.M.5    Dedman, J.R.6
  • 6
    • 54049116737 scopus 로고    scopus 로고
    • Role of mechanical stress in regulating airway surface hydration and mucus clearance rates
    • Button B., Boucher R.C. Role of mechanical stress in regulating airway surface hydration and mucus clearance rates. Respir Physiol Neurobiol 2008, 163:189-201.
    • (2008) Respir Physiol Neurobiol , vol.163 , pp. 189-201
    • Button, B.1    Boucher, R.C.2
  • 7
    • 34047185781 scopus 로고    scopus 로고
    • Mucus structure and properties in cystic fibrosis
    • Rubin B.K. Mucus structure and properties in cystic fibrosis. Paediatr Respir Rev 2007, 8:4-7.
    • (2007) Paediatr Respir Rev , vol.8 , pp. 4-7
    • Rubin, B.K.1
  • 8
    • 77953402872 scopus 로고    scopus 로고
    • Expression of DeltaF508 Cystic Fibrosis Transmembrane Regulator (CFTR) decreases membrane sialylation
    • Dosanjh A., Muchmore E.A. Expression of DeltaF508 Cystic Fibrosis Transmembrane Regulator (CFTR) decreases membrane sialylation. Open Respir Med J 2009, 3:79-84.
    • (2009) Open Respir Med J , vol.3 , pp. 79-84
    • Dosanjh, A.1    Muchmore, E.A.2
  • 9
    • 0028920643 scopus 로고
    • Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface
    • Imundo L., Barasch J., Prince A., Al-Awqati Q. Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. Proc Natl Acad Sci U S A 1995, 92:3019-3023.
    • (1995) Proc Natl Acad Sci U S A , vol.92 , pp. 3019-3023
    • Imundo, L.1    Barasch, J.2    Prince, A.3    Al-Awqati, Q.4
  • 10
    • 0035145463 scopus 로고    scopus 로고
    • Lack of adherence of clinical isolates of Pseudomonas aeruginosa to asialo-GM(1) on epithelial cells
    • Schroeder T.H., Zaidi T., Pier G.B. Lack of adherence of clinical isolates of Pseudomonas aeruginosa to asialo-GM(1) on epithelial cells. Infect Immun 2001, 69:719-729.
    • (2001) Infect Immun , vol.69 , pp. 719-729
    • Schroeder, T.H.1    Zaidi, T.2    Pier, G.B.3
  • 11
    • 0030691027 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung
    • Pier G.B., Grout M., Zaidi T.S. Cystic fibrosis transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung. Proc Natl Acad Sci U S A 1997, 94:12088-12093.
    • (1997) Proc Natl Acad Sci U S A , vol.94 , pp. 12088-12093
    • Pier, G.B.1    Grout, M.2    Zaidi, T.S.3
  • 12
    • 0027203886 scopus 로고
    • Eicosapentaenoic acid in cystic fibrosis: evidence of a pathogenetic role for leukotriene B4
    • Lawrence R., Sorrell T. Eicosapentaenoic acid in cystic fibrosis: evidence of a pathogenetic role for leukotriene B4. Lancet 1993, 342:465-469.
    • (1993) Lancet , vol.342 , pp. 465-469
    • Lawrence, R.1    Sorrell, T.2
  • 13
    • 0028519279 scopus 로고
    • The absorption and effect of dietary supplementation with omega-3 fatty acids on serum leukotriene B4 in patients with cystic fibrosis
    • Kurlandsky L.E., Bennink M.R., Webb P.M., Ulrich P.J., Baer L.J. The absorption and effect of dietary supplementation with omega-3 fatty acids on serum leukotriene B4 in patients with cystic fibrosis. Pediatr Pulmonol 1994, 18:211-217.
    • (1994) Pediatr Pulmonol , vol.18 , pp. 211-217
    • Kurlandsky, L.E.1    Bennink, M.R.2    Webb, P.M.3    Ulrich, P.J.4    Baer, L.J.5
  • 14
    • 16344381319 scopus 로고    scopus 로고
    • Antiinflammatory therapies for cystic fibrosis: past, present, and future
    • Prescott W.A., Johnson C.E. Antiinflammatory therapies for cystic fibrosis: past, present, and future. Pharmacotherapy 2005, 25:555-573.
    • (2005) Pharmacotherapy , vol.25 , pp. 555-573
    • Prescott, W.A.1    Johnson, C.E.2
  • 15
    • 39749133582 scopus 로고    scopus 로고
    • Influence of pancreatic status and sex on polyunsaturated fatty acid profiles in cystic fibrosis
    • Coste T.C., Deumer G., Reychler G., Lebecque P., Wallemacq P., Leal T. Influence of pancreatic status and sex on polyunsaturated fatty acid profiles in cystic fibrosis. Clin Chem 2008, 54:388-395.
    • (2008) Clin Chem , vol.54 , pp. 388-395
    • Coste, T.C.1    Deumer, G.2    Reychler, G.3    Lebecque, P.4    Wallemacq, P.5    Leal, T.6
  • 16
    • 0033819711 scopus 로고    scopus 로고
    • Energy supplements rich in linoleic acid improve body weight and essential fatty acid status of cystic fibrosis patients
    • Steinkamp G., Demmelmair H., Ruhl-Bagheri I., von der Hardt H., Koletzko B. Energy supplements rich in linoleic acid improve body weight and essential fatty acid status of cystic fibrosis patients. J Pediatr Gastroenterol Nutr 2000, 31:418-423.
    • (2000) J Pediatr Gastroenterol Nutr , vol.31 , pp. 418-423
    • Steinkamp, G.1    Demmelmair, H.2    Ruhl-Bagheri, I.3    von der Hardt, H.4    Koletzko, B.5
  • 17
    • 0019505626 scopus 로고
    • Essential fatty acid status in cystic fibrosis and the effects of safflower oil supplementation
    • Lloyd-Still J.D., Johnson S.B., Holman R.T. Essential fatty acid status in cystic fibrosis and the effects of safflower oil supplementation. Am J Clin Nutr 1981, 34:1-7.
    • (1981) Am J Clin Nutr , vol.34 , pp. 1-7
    • Lloyd-Still, J.D.1    Johnson, S.B.2    Holman, R.T.3
  • 18
    • 0031453452 scopus 로고    scopus 로고
    • Essential fatty acid deficiency in well nourished young cystic fibrosis patients
    • Roulet M., Frascarolo P., Rappaz I., Pilet M. Essential fatty acid deficiency in well nourished young cystic fibrosis patients. Eur J Pediatr 1997, 156:952-956.
    • (1997) Eur J Pediatr , vol.156 , pp. 952-956
    • Roulet, M.1    Frascarolo, P.2    Rappaz, I.3    Pilet, M.4
  • 19
    • 34247169193 scopus 로고    scopus 로고
    • An overview of monitoring and supplementation of omega 3 fatty acids in cystic fibrosis
    • Coste T.C., Armand M., Lebacq J., Lebecque P., Wallemacq P., Leal T. An overview of monitoring and supplementation of omega 3 fatty acids in cystic fibrosis. Clin Biochem 2007, 40:511-520.
    • (2007) Clin Biochem , vol.40 , pp. 511-520
    • Coste, T.C.1    Armand, M.2    Lebacq, J.3    Lebecque, P.4    Wallemacq, P.5    Leal, T.6
  • 21
    • 0033598714 scopus 로고    scopus 로고
    • A membrane lipid imbalance plays a role in the phenotypic expression of cystic fibrosis in cftr (-/-) mice
    • Freedman S.D., Katz M.H., Parker E.M., Laposata M., Urman M.Y., Alvarez J.G. A membrane lipid imbalance plays a role in the phenotypic expression of cystic fibrosis in cftr (-/-) mice. Proc Natl Acad Sci U S A 1999, 96:13995-40000.
    • (1999) Proc Natl Acad Sci U S A , vol.96 , pp. 13995-40000
    • Freedman, S.D.1    Katz, M.H.2    Parker, E.M.3    Laposata, M.4    Urman, M.Y.5    Alvarez, J.G.6
  • 22
    • 72249084127 scopus 로고    scopus 로고
    • Increased tissue arachidonic acid and reduced linoleic acid in a mouse model of cystic fibrosis are reversed by supplemental glycerophospholipids enriched in docosahexaenoic acid
    • Mimoun M., Coste T.C., Lebacq J., Lebecque P., Wallemacq P., Leal T., et al. Increased tissue arachidonic acid and reduced linoleic acid in a mouse model of cystic fibrosis are reversed by supplemental glycerophospholipids enriched in docosahexaenoic acid. J Nutr 2009, 139:2358-2364.
    • (2009) J Nutr , vol.139 , pp. 2358-2364
    • Mimoun, M.1    Coste, T.C.2    Lebacq, J.3    Lebecque, P.4    Wallemacq, P.5    Leal, T.6
  • 23
    • 41849092177 scopus 로고    scopus 로고
    • Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis
    • Teichgräber V., Ulrich M., Endlich N., Riethmüller J., Wilker B., De Oliveira-Munding C.C., et al. Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis. Nat Med 2008, 14:382-391.
    • (2008) Nat Med , vol.14 , pp. 382-391
    • Teichgräber, V.1    Ulrich, M.2    Endlich, N.3    Riethmüller, J.4    Wilker, B.5    De Oliveira-Munding, C.C.6
  • 24
    • 84867112106 scopus 로고    scopus 로고
    • Ceramide in cystic fibrosis: a potential new target for therapeutic intervention
    • Wojewodka G., De Sanctis J.B., Radzioch D. Ceramide in cystic fibrosis: a potential new target for therapeutic intervention. J Lipids 2011, 2011:674968.
    • (2011) J Lipids , vol.2011 , pp. 674968
    • Wojewodka, G.1    De Sanctis, J.B.2    Radzioch, D.3
  • 27
    • 33745485358 scopus 로고    scopus 로고
    • CF-emerging therapies: modulation inflammation
    • Airway-Inflammation Group, Société Française de Mucoviscidose
    • Fayon M., Airway-Inflammation Group, Société Française de Mucoviscidose CF-emerging therapies: modulation inflammation. Paediatr Respir Rev 2006, 7:S170-S174.
    • (2006) Paediatr Respir Rev , vol.7
    • Fayon, M.1
  • 28
    • 84860110054 scopus 로고    scopus 로고
    • Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index
    • Belessis Y., Dixon B., Hawkins G., Pereira J., Peat J., Macdonald R., et al. Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index. Am J Respir Crit Care Med 2012, 185:862-873.
    • (2012) Am J Respir Crit Care Med , vol.185 , pp. 862-873
    • Belessis, Y.1    Dixon, B.2    Hawkins, G.3    Pereira, J.4    Peat, J.5    Macdonald, R.6
  • 30
    • 0034764166 scopus 로고    scopus 로고
    • Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis
    • Rosenfeld M., Gibson R.L., Mcnamara S., Emerson J., Burns J.L., Castile R., et al. Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis. Pediatr Pulmonol 2001, 32:356-366.
    • (2001) Pediatr Pulmonol , vol.32 , pp. 356-366
    • Rosenfeld, M.1    Gibson, R.L.2    Mcnamara, S.3    Emerson, J.4    Burns, J.L.5    Castile, R.6
  • 34
    • 84874780534 scopus 로고    scopus 로고
    • Allergic bronchopulmonary aspergillosis treated successfully for one year with omalizumab
    • Collins J., Devos G., Hudes G., Rosenstreich D. Allergic bronchopulmonary aspergillosis treated successfully for one year with omalizumab. J Asthma Allergy 2012, 5:65-70.
    • (2012) J Asthma Allergy , vol.5 , pp. 65-70
    • Collins, J.1    Devos, G.2    Hudes, G.3    Rosenstreich, D.4
  • 36
    • 79960641412 scopus 로고    scopus 로고
    • Defective CFTR expression and function are detectable in blood monocytes: development of a new blood test for cystic fibrosis
    • Sorio C., Buffelli M., Angiari C., Ettorre M., Johansson J., Vezzalini M., et al. Defective CFTR expression and function are detectable in blood monocytes: development of a new blood test for cystic fibrosis. PLoS One 2011, 6:e22212.
    • (2011) PLoS One , vol.6
    • Sorio, C.1    Buffelli, M.2    Angiari, C.3    Ettorre, M.4    Johansson, J.5    Vezzalini, M.6
  • 37
    • 36849020586 scopus 로고    scopus 로고
    • Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease
    • Hartl D., Latzin P., Hordijk P., Marcos V., Rudolph C., Woischnik M., et al. Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease. Nat Med 2007, 13:1423-1430.
    • (2007) Nat Med , vol.13 , pp. 1423-1430
    • Hartl, D.1    Latzin, P.2    Hordijk, P.3    Marcos, V.4    Rudolph, C.5    Woischnik, M.6
  • 38
  • 39
    • 84868296268 scopus 로고    scopus 로고
    • Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis
    • Sagel S.D., Wagner B.D., Anthony M.M., Emmett P., Zemanick E.T. Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis. Am J Respir Crit Care Med 2012, 186:857-865.
    • (2012) Am J Respir Crit Care Med , vol.186 , pp. 857-865
    • Sagel, S.D.1    Wagner, B.D.2    Anthony, M.M.3    Emmett, P.4    Zemanick, E.T.5
  • 40
    • 84887488043 scopus 로고    scopus 로고
    • Airway inflammation in cystic fibrosis: molecular mechanisms and clinical implications
    • Cohen-Cymberknoh M., Kerem E., Ferkol T., Elizur A. Airway inflammation in cystic fibrosis: molecular mechanisms and clinical implications. Thorax 2013, 68:1157-1162.
    • (2013) Thorax , vol.68 , pp. 1157-1162
    • Cohen-Cymberknoh, M.1    Kerem, E.2    Ferkol, T.3    Elizur, A.4
  • 41
    • 0035152203 scopus 로고    scopus 로고
    • Directed neutrophil migration to IL-8 is increased in cystic fibrosis: a study of the effect of erythromycin
    • Brennan S., Cooper D., Sly P.D. Directed neutrophil migration to IL-8 is increased in cystic fibrosis: a study of the effect of erythromycin. Thorax 2001, 56:62-64.
    • (2001) Thorax , vol.56 , pp. 62-64
    • Brennan, S.1    Cooper, D.2    Sly, P.D.3
  • 42
    • 0032817772 scopus 로고    scopus 로고
    • Altered respiratory epithelial cell cytokine production in cystic fibrosis
    • Bonfield T.L., Konstan M.W., Berger M. Altered respiratory epithelial cell cytokine production in cystic fibrosis. J Allergy Clin Immunol 1999, 104:72-78.
    • (1999) J Allergy Clin Immunol , vol.104 , pp. 72-78
    • Bonfield, T.L.1    Konstan, M.W.2    Berger, M.3
  • 43
    • 4644272735 scopus 로고    scopus 로고
    • Defective lipoxin-mediated anti-inflammatory activity in the cystic fibrosis airway
    • Karp C.L., Flick L.M., Park K.W., Softic S., Greer T.M., Keledjian R., et al. Defective lipoxin-mediated anti-inflammatory activity in the cystic fibrosis airway. Nat Immunol 2004, 5:388-392.
    • (2004) Nat Immunol , vol.5 , pp. 388-392
    • Karp, C.L.1    Flick, L.M.2    Park, K.W.3    Softic, S.4    Greer, T.M.5    Keledjian, R.6
  • 44
    • 31344442586 scopus 로고    scopus 로고
    • Attenuation of host defense function of lung phagocytes in young cystic fibrosis patients
    • Alexis N.E., Muhlebach M.S., Peden D.B., Noah T.L. Attenuation of host defense function of lung phagocytes in young cystic fibrosis patients. J Cyst Fibros 2006, 5:17-25.
    • (2006) J Cyst Fibros , vol.5 , pp. 17-25
    • Alexis, N.E.1    Muhlebach, M.S.2    Peden, D.B.3    Noah, T.L.4
  • 46
    • 34250853027 scopus 로고    scopus 로고
    • Chronic infection with Achromobacter xylosoxidans in cystic fibrosis patients; a retrospective case control study
    • Rønne Hansen C., Pressler T., Høiby N., Gormsen M. Chronic infection with Achromobacter xylosoxidans in cystic fibrosis patients; a retrospective case control study. J Cyst Fibros 2006, 5:245-251.
    • (2006) J Cyst Fibros , vol.5 , pp. 245-251
    • Rønne Hansen, C.1    Pressler, T.2    Høiby, N.3    Gormsen, M.4
  • 47
    • 0036225688 scopus 로고    scopus 로고
    • Lung infections associated with cystic fibrosis
    • Lyczak J.B., Cannon C.L., Pier G.B. Lung infections associated with cystic fibrosis. Clin Microbiol Rev 2002, 15:194-222.
    • (2002) Clin Microbiol Rev , vol.15 , pp. 194-222
    • Lyczak, J.B.1    Cannon, C.L.2    Pier, G.B.3
  • 48
    • 84871350905 scopus 로고    scopus 로고
    • Modelling co-infection of the cystic fibrosis lung by Pseudomonas aeruginosa and Burkholderia cenocepacia reveals influences on biofilm formation and host response
    • Bragonzi A., Farulla I., Paroni M., Twomey K.B., Pirone L., Lorè N.I., et al. Modelling co-infection of the cystic fibrosis lung by Pseudomonas aeruginosa and Burkholderia cenocepacia reveals influences on biofilm formation and host response. PLoS One 2012, 7:e52330.
    • (2012) PLoS One , vol.7
    • Bragonzi, A.1    Farulla, I.2    Paroni, M.3    Twomey, K.B.4    Pirone, L.5    Lorè, N.I.6
  • 49
    • 77958113699 scopus 로고    scopus 로고
    • Allergic bronchopulmonary aspergillosis and Aspergillus infection in cystic fibrosis
    • Moss R.B. Allergic bronchopulmonary aspergillosis and Aspergillus infection in cystic fibrosis. Curr Opin Pulm Med 2010, 16:598-603.
    • (2010) Curr Opin Pulm Med , vol.16 , pp. 598-603
    • Moss, R.B.1
  • 50
    • 68449101095 scopus 로고    scopus 로고
    • Immunological mechanisms behind the cystic fibrosis-ABPA link
    • Hartl D. Immunological mechanisms behind the cystic fibrosis-ABPA link. Med Mycol 2009, 47(Suppl. 1):S183-S191.
    • (2009) Med Mycol , vol.47 , Issue.Suppl. 1
    • Hartl, D.1
  • 51
    • 45849115137 scopus 로고    scopus 로고
    • Comparison of serum markers for allergic bronchopulmonary aspergillosis in cystic fibrosis
    • Latzin P., Hartl D., Regamey N., Frey U., Schoeni M.H., Casaulta C. Comparison of serum markers for allergic bronchopulmonary aspergillosis in cystic fibrosis. Eur Respir J 2008, 31:36-42.
    • (2008) Eur Respir J , vol.31 , pp. 36-42
    • Latzin, P.1    Hartl, D.2    Regamey, N.3    Frey, U.4    Schoeni, M.H.5    Casaulta, C.6
  • 53
    • 84943778887 scopus 로고    scopus 로고
    • Cystic Fibrosis Foundation Patient Registry
    • Cystic Fibrosis Foundation, Bethesda, Maryland
    • Cystic Fibrosis Foundation Patient Registry. 2012 Annual Data Report 2013, Cystic Fibrosis Foundation, Bethesda, Maryland.
    • (2013) 2012 Annual Data Report
  • 54
    • 33847688940 scopus 로고    scopus 로고
    • Azithromycin selectively reduces tumor necrosis factor alpha levels in cystic fibrosis airway epithelial cells
    • Cigana C., Assael B.M., Melotti P. Azithromycin selectively reduces tumor necrosis factor alpha levels in cystic fibrosis airway epithelial cells. Antimicrob Agents Chemother 2007, 51:975-981.
    • (2007) Antimicrob Agents Chemother , vol.51 , pp. 975-981
    • Cigana, C.1    Assael, B.M.2    Melotti, P.3
  • 55
  • 58
    • 0024424270 scopus 로고
    • Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA
    • Riordan J.R., Rommens J.M., Kerem B., Alon N., Rozmahel R., Grzerlczak Z., et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989, 245:1066-1073.
    • (1989) Science , vol.245 , pp. 1066-1073
    • Riordan, J.R.1    Rommens, J.M.2    Kerem, B.3    Alon, N.4    Rozmahel, R.5    Grzerlczak, Z.6
  • 62
    • 84900302345 scopus 로고    scopus 로고
    • Belgisch Mucoviscidose Register -Registre Belge de la Mucoviscidose-2007
    • Belgisch Mucoviscidose Register -Registre Belge de la Mucoviscidose-2007. Annual Data Report, Belgium 2007.
    • (2007) Annual Data Report, Belgium
  • 65
    • 33751177801 scopus 로고    scopus 로고
    • Azithromycin reduces spontaneous and induced inflammation in DeltaF508 cystic fibrosis mice
    • Legssyer R., Huaux F., Lebacq J., Delos M., Marbaix E., Lebecque P., et al. Azithromycin reduces spontaneous and induced inflammation in DeltaF508 cystic fibrosis mice. Respir Res 2006, 7:134.
    • (2006) Respir Res , vol.7 , pp. 134
    • Legssyer, R.1    Huaux, F.2    Lebacq, J.3    Delos, M.4    Marbaix, E.5    Lebecque, P.6
  • 67
    • 39449133330 scopus 로고    scopus 로고
    • Airway inflammation in cystic fibrosis
    • Elizur A., Cannon C.L., Ferkol T.W. Airway inflammation in cystic fibrosis. Chest 2008, 133:489-495.
    • (2008) Chest , vol.133 , pp. 489-495
    • Elizur, A.1    Cannon, C.L.2    Ferkol, T.W.3
  • 68
    • 67349226398 scopus 로고    scopus 로고
    • Azithromycin fails to reduce increased expression of neutrophil-related cytokines in primary-cultured epithelial cells from cystic fibrosis mice
    • Gavilanes X., Huaux F., Meyer M., Lebecque P., Marbaix E., Lison D., et al. Azithromycin fails to reduce increased expression of neutrophil-related cytokines in primary-cultured epithelial cells from cystic fibrosis mice. J Cyst Fibros 2009, 8:203-210.
    • (2009) J Cyst Fibros , vol.8 , pp. 203-210
    • Gavilanes, X.1    Huaux, F.2    Meyer, M.3    Lebecque, P.4    Marbaix, E.5    Lison, D.6
  • 69
    • 84878464222 scopus 로고    scopus 로고
    • Dysregulated proinflammatory and fibrogenic phenotype of fibroblasts in cystic fibrosis
    • Huaux F., Noel S., Dhooghe B., Panin N., Lo Re S., Lison D., et al. Dysregulated proinflammatory and fibrogenic phenotype of fibroblasts in cystic fibrosis. PLoS One 2013, 8:e64341.
    • (2013) PLoS One , vol.8
    • Huaux, F.1    Noel, S.2    Dhooghe, B.3    Panin, N.4    Lo Re, S.5    Lison, D.6
  • 70
    • 84862503933 scopus 로고    scopus 로고
    • Immunomodulatory activity of vardenafil on induced lung inflammation in cystic fibrosis mice
    • Lubamba B., Huaux F., Lebacq J., Marbaix E., Dhooghe B., Panin N., et al. Immunomodulatory activity of vardenafil on induced lung inflammation in cystic fibrosis mice. J Cyst Fibros 2012, 11:266-273.
    • (2012) J Cyst Fibros , vol.11 , pp. 266-273
    • Lubamba, B.1    Huaux, F.2    Lebacq, J.3    Marbaix, E.4    Dhooghe, B.5    Panin, N.6
  • 71
    • 2442718786 scopus 로고    scopus 로고
    • Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice
    • Mall M., Grubb B.R., Harkema J.R., O'neal W.K., Boucher R.C. Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice. Nat Med 2004, 10:487-493.
    • (2004) Nat Med , vol.10 , pp. 487-493
    • Mall, M.1    Grubb, B.R.2    Harkema, J.R.3    O'neal, W.K.4    Boucher, R.C.5
  • 72
    • 57149104917 scopus 로고    scopus 로고
    • Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in betaENaC-overexpressing mice
    • Zhou Z., Treis D., Schubert S.C., Harm M., Schatterny J., Hirtz S., et al. Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in betaENaC-overexpressing mice. Am J Respir Crit Care Med 2008, 178:1245-1256.
    • (2008) Am J Respir Crit Care Med , vol.178 , pp. 1245-1256
    • Zhou, Z.1    Treis, D.2    Schubert, S.C.3    Harm, M.4    Schatterny, J.5    Hirtz, S.6
  • 73
    • 0031862236 scopus 로고    scopus 로고
    • Effects of topically delivered benzamil and amiloride on nasal potential difference in cystic fibrosis
    • Hofmann T., Stutts M.J., Ziersch A., Rückes C., Weber W.M., Knowles M.R., et al. Effects of topically delivered benzamil and amiloride on nasal potential difference in cystic fibrosis. Am J Respir Crit Care Med 1998, 157:1844-1849.
    • (1998) Am J Respir Crit Care Med , vol.157 , pp. 1844-1849
    • Hofmann, T.1    Stutts, M.J.2    Ziersch, A.3    Rückes, C.4    Weber, W.M.5    Knowles, M.R.6
  • 74
    • 10044235073 scopus 로고    scopus 로고
    • Evaluation of second generation amiloride analogs as therapy for cystic fibrosis lung disease
    • Hirsh A.J., Sabater J.R., Zamurs A., Smith R.T., Paradiso A.M., Hopkins S., et al. Evaluation of second generation amiloride analogs as therapy for cystic fibrosis lung disease. J Pharmacol Exp Ther 2004, 311:929-938.
    • (2004) J Pharmacol Exp Ther , vol.311 , pp. 929-938
    • Hirsh, A.J.1    Sabater, J.R.2    Zamurs, A.3    Smith, R.T.4    Paradiso, A.M.5    Hopkins, S.6
  • 75
    • 4344663919 scopus 로고    scopus 로고
    • Progress toward generating a ferret model of cystic fibrosis by somatic cell nuclear transfer
    • Li Z., Engelhardt J.F. Progress toward generating a ferret model of cystic fibrosis by somatic cell nuclear transfer. Reprod Biol Endocrinol 2003, 1:83.
    • (2003) Reprod Biol Endocrinol , vol.1 , pp. 83
    • Li, Z.1    Engelhardt, J.F.2
  • 77
    • 80052538896 scopus 로고    scopus 로고
    • Comparative biology of cystic fibrosis animal models
    • Fisher J.T., Zhang Y., Engelhardt J.F. Comparative biology of cystic fibrosis animal models. Methods Mol Biol 2011, 742:311-334.
    • (2011) Methods Mol Biol , vol.742 , pp. 311-334
    • Fisher, J.T.1    Zhang, Y.2    Engelhardt, J.F.3
  • 79
    • 84863476402 scopus 로고    scopus 로고
    • Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung
    • Pezzulo A.A., Tang X.X., Hoegger M.J., Alaiwa M.H., Ramachandran S., Moninger T.O., et al. Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. Nature 2012, 487:109-113.
    • (2012) Nature , vol.487 , pp. 109-113
    • Pezzulo, A.A.1    Tang, X.X.2    Hoegger, M.J.3    Alaiwa, M.H.4    Ramachandran, S.5    Moninger, T.O.6
  • 81
    • 78649915334 scopus 로고    scopus 로고
    • Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia
    • Chen J.H., Stoltz D.A., Karp P.H., Ernst S.E., Pezzulo A.A., Moninger T.O., et al. Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. Cell 2010, 143:911-923.
    • (2010) Cell , vol.143 , pp. 911-923
    • Chen, J.H.1    Stoltz, D.A.2    Karp, P.H.3    Ernst, S.E.4    Pezzulo, A.A.5    Moninger, T.O.6
  • 82
    • 84900317294 scopus 로고    scopus 로고
    • http://www.cff.org/research/DrugDevelopmentPipeline/.
  • 83
    • 30944466084 scopus 로고    scopus 로고
    • A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis
    • Elkins M.R., Robinson M., Rose B.R., Harbour C., Moriarty C.P., Marks G.B., et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006, 354:229-240.
    • (2006) N Engl J Med , vol.354 , pp. 229-240
    • Elkins, M.R.1    Robinson, M.2    Rose, B.R.3    Harbour, C.4    Moriarty, C.P.5    Marks, G.B.6
  • 85
    • 0031076507 scopus 로고    scopus 로고
    • Recombinant human DNase (rhDNase) influences phospholipid composition, surface activity, rheology and consecutively clearance indices of cystic fibrosis sputum
    • Griese M., App E.M., Duroux A., Burkert A., Schams A. Recombinant human DNase (rhDNase) influences phospholipid composition, surface activity, rheology and consecutively clearance indices of cystic fibrosis sputum. Pulm Pharmacol Ther 1997, 10:21-27.
    • (1997) Pulm Pharmacol Ther , vol.10 , pp. 21-27
    • Griese, M.1    App, E.M.2    Duroux, A.3    Burkert, A.4    Schams, A.5
  • 86
    • 0029921953 scopus 로고    scopus 로고
    • Effects of 12-week administration of dornase alfa in patients with advanced cystic fibrosis lung disease. Pulmozyme Study Group
    • Mccoy K., Hamilton S., Johnson C. Effects of 12-week administration of dornase alfa in patients with advanced cystic fibrosis lung disease. Pulmozyme Study Group. Chest 1996, 110:889-895.
    • (1996) Chest , vol.110 , pp. 889-895
    • Mccoy, K.1    Hamilton, S.2    Johnson, C.3
  • 89
    • 0028914667 scopus 로고
    • Effect of high-dose ibuprofen in patients with cystic fibrosis
    • Konstan M.W., Byard P.J., Hoppel C.L., Davis P.B. Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med 1995, 332:848-854.
    • (1995) N Engl J Med , vol.332 , pp. 848-854
    • Konstan, M.W.1    Byard, P.J.2    Hoppel, C.L.3    Davis, P.B.4
  • 90
    • 0344624839 scopus 로고    scopus 로고
    • Long-term azithromycin may improve lung function in children with cystic fibrosis
    • Jaffé A., Francis J., Rosenthal M., Bush A. Long-term azithromycin may improve lung function in children with cystic fibrosis. Lancet 1998, 351:420.
    • (1998) Lancet , vol.351 , pp. 420
    • Jaffé, A.1    Francis, J.2    Rosenthal, M.3    Bush, A.4
  • 91
    • 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., Bowler S., Masel P., Mccormack J. Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: a randomised trial. Thorax 2002, 57:212-216.
    • (2002) Thorax , vol.57 , pp. 212-216
    • Wolter, J.1    Seeney, S.2    Bell, S.3    Bowler, S.4    Masel, P.5    Mccormack, J.6
  • 92
    • 0037190575 scopus 로고    scopus 로고
    • Long term azithromycin in children with cystic fibrosis: a randomised, placebo-controlled crossover trial
    • Equi A., Balfour-Lynn I.M., Bush A., Rosenthal M. 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    Rosenthal, M.4
  • 93
    • 33749984264 scopus 로고    scopus 로고
    • Long term effects of azithromycin in patients with cystic fibrosis: a double blind, placebo controlled trial
    • Clement A., Tamalet A., Leroux E., Ravilly S., Fauroux B., Jais J.P. Long term effects of azithromycin in patients with cystic fibrosis: a double blind, placebo controlled trial. Thorax 2006, 61:895-902.
    • (2006) Thorax , vol.61 , pp. 895-902
    • Clement, A.1    Tamalet, A.2    Leroux, E.3    Ravilly, S.4    Fauroux, B.5    Jais, J.P.6
  • 94
    • 84880296779 scopus 로고    scopus 로고
    • Impact of acute antibiotic therapy on the pulmonary exacerbation endpoint in cystic fibrosis clinical trials
    • Mayer-Hamblett N., Saiman L., Lands L.C., Anstead M., Rosenfeld M., Kloster M., et al. Impact of acute antibiotic therapy on the pulmonary exacerbation endpoint in cystic fibrosis clinical trials. Contemp Clin Trials 2013, 36:99-105.
    • (2013) Contemp Clin Trials , vol.36 , pp. 99-105
    • Mayer-Hamblett, N.1    Saiman, L.2    Lands, L.C.3    Anstead, M.4    Rosenfeld, M.5    Kloster, M.6
  • 95
    • 84862179762 scopus 로고    scopus 로고
    • Open-label, follow- study of azithromycin in pediatric patients with CF uninfected with Pseudomonas aeruginosa
    • Saiman L., Mayer-Hamblett N., Anstead M., Lands L.C., Kloster M., Goss C.H., et al. Open-label, follow- study of azithromycin in pediatric patients with CF uninfected with Pseudomonas aeruginosa. Pediatr Pulmonol 2012, 47:641-648.
    • (2012) Pediatr Pulmonol , vol.47 , pp. 641-648
    • Saiman, L.1    Mayer-Hamblett, N.2    Anstead, M.3    Lands, L.C.4    Kloster, M.5    Goss, C.H.6
  • 96
    • 84866076450 scopus 로고    scopus 로고
    • Effect of azithromycin on systemic markers of inflammation in cystic fibrosis patients uninfected with Pseudomonas aeruginosa
    • Ratjen F., Saiman L., Mayer-Hamblett N., Lands L.C., Kloster M., Thompson V., et al. Effect of azithromycin on systemic markers of inflammation in cystic fibrosis patients uninfected with Pseudomonas aeruginosa. Chest 2012, 142:1259-1266.
    • (2012) Chest , vol.142 , pp. 1259-1266
    • Ratjen, F.1    Saiman, L.2    Mayer-Hamblett, N.3    Lands, L.C.4    Kloster, M.5    Thompson, V.6
  • 97
  • 100
    • 0037242749 scopus 로고    scopus 로고
    • Effect of an 8-month treatment with omega-3 fatty acids (eicosapentaenoic and docosahexaenoic) in patients with cystic fibrosis
    • De Vizia B., Raia V., Spano C., Pavlidis C., Coruzzo A., Alessio M. Effect of an 8-month treatment with omega-3 fatty acids (eicosapentaenoic and docosahexaenoic) in patients with cystic fibrosis. J Parenter Enteral Nutr 2003, 27:52-57.
    • (2003) J Parenter Enteral Nutr , vol.27 , pp. 52-57
    • De Vizia, B.1    Raia, V.2    Spano, C.3    Pavlidis, C.4    Coruzzo, A.5    Alessio, M.6
  • 101
    • 33744904723 scopus 로고    scopus 로고
    • Biological effects of a dietary omega-3 polyunsaturated fatty acids supplementation in cystic fibrosis patients: a randomized, crossover placebo-controlled trial
    • Panchaud A., Sauty A., Kernen Y., Decosterd L.A., Buclin T., Boulat O., et al. Biological effects of a dietary omega-3 polyunsaturated fatty acids supplementation in cystic fibrosis patients: a randomized, crossover placebo-controlled trial. Clin Nutr 2006, 25:418-427.
    • (2006) Clin Nutr , vol.25 , pp. 418-427
    • Panchaud, A.1    Sauty, A.2    Kernen, Y.3    Decosterd, L.A.4    Buclin, T.5    Boulat, O.6
  • 102
    • 33645211759 scopus 로고    scopus 로고
    • Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat
    • Norez C., Noel S., Wilke M., Bijvelds M., Jorna H., Melin P., et al. Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat. FEBS Lett 2006, 508:2081-2086.
    • (2006) FEBS Lett , vol.508 , pp. 2081-2086
    • Norez, C.1    Noel, S.2    Wilke, M.3    Bijvelds, M.4    Jorna, H.5    Melin, P.6
  • 103
    • 44249097535 scopus 로고    scopus 로고
    • Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells
    • Noël S., Wilke M., Bot A.G., De Jonge H.R., Becq F. Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells. J Pharmacol Exp Ther 2008, 325:1016-1023.
    • (2008) J Pharmacol Exp Ther , vol.325 , pp. 1016-1023
    • Noël, S.1    Wilke, M.2    Bot, A.G.3    De Jonge, H.R.4    Becq, F.5
  • 104
    • 66249120003 scopus 로고    scopus 로고
    • Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice
    • Lubamba B., Lebacq J., Lebecque P., Vanbever R., Leonard A., Wallemacq P., et al. Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice. Am J Respir Crit Care Med 2009, 179:1022-1028.
    • (2009) Am J Respir Crit Care Med , vol.179 , pp. 1022-1028
    • Lubamba, B.1    Lebacq, J.2    Lebecque, P.3    Vanbever, R.4    Leonard, A.5    Wallemacq, P.6
  • 106
    • 33748944330 scopus 로고    scopus 로고
    • S-nitrosylating agents: a novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells
    • Zaman K., Carraro S., Doherty J., Henderson E.M., Lendermon E., Liu L., et al. S-nitrosylating agents: a novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells. Mol Pharmacol 2006, 70:1435-1442.
    • (2006) Mol Pharmacol , vol.70 , pp. 1435-1442
    • Zaman, K.1    Carraro, S.2    Doherty, J.3    Henderson, E.M.4    Lendermon, E.5    Liu, L.6
  • 107
    • 84941180107 scopus 로고    scopus 로고
    • Intestinal current measurement to assess modulation of F508del-CFTR function by GSNOR inhibitor treatment in vivo
    • Blonder J.P., Quinney N.L., Looker D., Sun X., Gentzsch M., Scoggin C., et al. Intestinal current measurement to assess modulation of F508del-CFTR function by GSNOR inhibitor treatment in vivo. Pediatr Pulmonol Suppl 2013, 36:209.
    • (2013) Pediatr Pulmonol Suppl , vol.36 , pp. 209
    • Blonder, J.P.1    Quinney, N.L.2    Looker, D.3    Sun, X.4    Gentzsch, M.5    Scoggin, C.6
  • 108
    • 84900318277 scopus 로고    scopus 로고
    • A novel GSNOR inhibitor with potent bronchodilatory effects and CFTR potentiation activity
    • Blonder J.P., Look K., Sun X., Sui J., Qiu J., Scoggin C., et al. A novel GSNOR inhibitor with potent bronchodilatory effects and CFTR potentiation activity. Pediatr Pulmonol Suppl 2013, 36:208.
    • (2013) Pediatr Pulmonol Suppl , vol.36 , pp. 208
    • Blonder, J.P.1    Look, K.2    Sun, X.3    Sui, J.4    Qiu, J.5    Scoggin, C.6
  • 109
    • 84926293024 scopus 로고    scopus 로고
    • Next generation F508del-CFTR correctors using a YFP based high throughput screening assay
    • Sun X., Sui J., Qiu J., Stout A.M., Verderber E.L., Blonder J.P., et al. Next generation F508del-CFTR correctors using a YFP based high throughput screening assay. Pediatr Pulmonol Suppl 2013, 36:216.
    • (2013) Pediatr Pulmonol Suppl , vol.36 , pp. 216
    • Sun, X.1    Sui, J.2    Qiu, J.3    Stout, A.M.4    Verderber, E.L.5    Blonder, J.P.6
  • 110
    • 84871510833 scopus 로고    scopus 로고
    • Novel therapeutic uses of alpha-1 antitrypsin: a window to the future
    • Wanner A., Arce A.D., Pardee E. Novel therapeutic uses of alpha-1 antitrypsin: a window to the future. COPD 2012, 9:583-588.
    • (2012) COPD , vol.9 , pp. 583-588
    • Wanner, A.1    Arce, A.D.2    Pardee, E.3


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