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Loss of CFTR function exacerbates the phenotype of Na hyperabsorption in murine airways
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Evidence of the mechanism behind defective bacterial killing in CF neutrophils
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Zhou Y, Song K, Painter RG, et al. Cystic fibrosis transmembrane conductance regulator recruitment to phagosomes in neutrophils. J Innate Immun 2013; 5:219-230. Evidence of the mechanism behind defective bacterial killing in CF neutrophils.
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Suggestion of different 'sub-populations' of neutrophils in the airway lumen and blood
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Houston N, Stewart N, Smith DS, et al. Sputum neutrophils in cystic fibrosis patients display a reduced respiratory burst. J Cyst Fibros 2013; 12:352-362. Suggestion of different 'sub-populations' of neutrophils in the airway lumen and blood.
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Metabolic adaptation of neutrophils in cystic fibrosis airways involves distinct shifts in nutrient transporter expression
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A further advance to our understanding of how the neutrophil adapts to the unique inflammatory environment of the CF lung
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Laval J, Touhami J, Herzenberg LA, et al. Metabolic adaptation of neutrophils in cystic fibrosis airways involves distinct shifts in nutrient transporter expression. J Immunol 2013; 190:6043-6050. A further advance to our understanding of how the neutrophil adapts to the unique inflammatory environment of the CF lung.
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Stockley, R.A.1
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Risk factors for bronchiectasis in children with cystic fibrosis
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A significant finding, which suggests that neutrophil elastase activity in BAL fluid in early life is associated with the development of bronchiectasis and lung function decline in CF
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Sly PD, Gangell CL, Chen L, et al. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med 2013; 368:1963-1970. A significant finding, which suggests that neutrophil elastase activity in BAL fluid in early life is associated with the development of bronchiectasis and lung function decline in CF.
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Sly, P.D.1
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17
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Urinary desmosine: A biomarker of structural lung injury during CF pulmonary exacerbation
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The development of urine desmosine as a marker of systemic elastin degradation promises to allow non-invasive assessment of neutrophil elastase activity
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Laguna TA, Wagner BD, Starcher B, et al. Urinary desmosine: A biomarker of structural lung injury during CF pulmonary exacerbation. Pediatr Pulmonol 2012; 47:856-863. The development of urine desmosine as a marker of systemic elastin degradation promises to allow non-invasive assessment of neutrophil elastase activity.
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Further evidence for neutrophil elastase as a biomarker in CF, predicting lung function decline
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Sagel SD, Wagner BD, Anthony MM, et al. Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis. Am J Respir Crit Care Med 2012; 186:857-865. Further evidence for neutrophil elastase as a biomarker in CF, predicting lung function decline.
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Increased expression of senescence markers in cystic fibrosis airways
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Evidence suggesting that neutrophil elastase can induce senescence in CF airway epithelial cells in vitro and perhaps in vivo
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Fischer BM, Wong JK, Degan S. Increased expression of senescence markers in cystic fibrosis airways. Am J Physiol Lung Cell Mol Physiol 2013; 304:L394-L400. Evidence suggesting that neutrophil elastase can induce senescence in CF airway epithelial cells in vitro and perhaps in vivo.
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Fischer, B.M.1
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22
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High expression of midkine in the airways of patients with cystic fibrosis
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[Epub ahead of print]. Describes an epithelial derived antimicrobial elevated in the CF lung that is cleaved and inactivated by neutrophil elastase
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Nordin SL, Jovic S, Kurut A, et al. High expression of midkine in the airways of patients with cystic fibrosis. Am J Respir Cell Mol Biol 2013. [Epub ahead of print]. Describes an epithelial derived antimicrobial elevated in the CF lung that is cleaved and inactivated by neutrophil elastase.
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Am J Respir Cell Mol Biol
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Nordin, S.L.1
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Kurut, A.3
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23
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Cleavage of lymphocyte surface antigens CD2, CD4, and CD8 by polymorphonuclear leukocyte elastase and cathepsin G in patients with cystic fibrosis
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Do ring G, Frank F, Boudier C, et al. Cleavage of lymphocyte surface antigens CD2, CD4, and CD8 by polymorphonuclear leukocyte elastase and cathepsin G in patients with cystic fibrosis. J Immunol 1995; 154:4842-4850.
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Inflammatory lung secretions inhibit dendritic cell maturation and function via neutrophil elastase
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25
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84872502797
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Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo
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Evidence that neutrophil elastase degrades wild-type and DF508-epithelial CFTR in vitro that has implications for other chronic lung diseases
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Le Gars M, Descamps D, Roussel D, et al. Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo. Am J Respir Crit Care Med 2013; 187:170-179. Evidence that neutrophil elastase degrades wild-type and DF508-epithelial CFTR in vitro that has implications for other chronic lung diseases.
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Am J Respir Crit Care Med
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Le Gars, M.1
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26
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84867114791
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Short and long term antibiotic therapy reduces airway and systemic inflammation in non-CF bronchiectasis
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Chalmers JD, Smith MP, McHugh B, et al. Short and long term antibiotic therapy reduces airway and systemic inflammation in non-CF bronchiectasis. Am J Respir Crit Care Med 2012; 186:657-665.
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Chalmers, J.D.1
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McHugh, B.3
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27
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Infection-induced NETosis is a dynamic process involving neutrophil multitasking in vivo
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Yipp BG, Petri B, Salina D, et al. Infection-induced NETosis is a dynamic process involving neutrophil multitasking in vivo. Nat Med 2012; 18:1386-1393.
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28
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84865049455
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Influences of chloride and hypochlorite on neutrophil extracellular trap formation
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Describes a potential link between CFTR and NET formation
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Akong-Moore K, Chow OA, von Kockritz-Blickwede M, Nizet V. Influences of chloride and hypochlorite on neutrophil extracellular trap formation. PloS One 2012; 7:e42984. Describes a potential link between CFTR and NET formation.
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PloS One
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Akong-Moore, K.1
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29
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Influence of DNA on the activities and inhibition of neutrophil serine proteases in cystic fibrosis sputum
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An important finding that DNA reduces the activity of neutrophil serine proteases, suggesting NETs function to limit protease induced damage
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Dubois AV, Gauthier A, Bréa D, et al. Influence of DNA on the activities and inhibition of neutrophil serine proteases in cystic fibrosis sputum. Am J Respir Cell Mol Biol 2012; 47:80-86. An important finding that DNA reduces the activity of neutrophil serine proteases, suggesting NETs function to limit protease induced damage.
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Am J Respir Cell Mol Biol
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Dubois, A.V.1
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Pseudomonas aeruginosa pyocyanin is critical for lung infection in mice
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31
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Pyocyanin-enhanced neutrophil extracellular trap formation requires the NADPH oxidase
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NET production induced by pyocyanin is a novel mechanism contributing to the immune dysfunction in CF
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Rada B, Jendrysik MA, Pang L, et al. Pyocyanin-enhanced neutrophil extracellular trap formation requires the NADPH oxidase. PLoS One 2013; 8:e54205. NET production induced by pyocyanin is a novel mechanism contributing to the immune dysfunction in CF.
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32
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84875158804
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Activation of conventional protein kinase C (PKC) is critical in the generation of human neutrophil extracellular traps
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33
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84867131143
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Efficacy, safety and effect on biomarkers of AZD9668 in cystic fibrosis
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Phase IIa clinical study of a neutrophil elastase inhibitor in CF.
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Elborn JS, Perrett J, Forsman-Semb K, et al. Efficacy, safety and effect on biomarkers of AZD9668 in cystic fibrosis. Eur Respir J 2012; 40:969-976. Phase IIa clinical study of a neutrophil elastase inhibitor in CF.
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Elborn, J.S.1
Perrett J-Forsman-Semb, K.2
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Stevens T, Ekholm K, Granse M, et al. AZD9668: Pharmacological characterization of a novel oral inhibitor of neutrophil elastase. J Pharmacol Exp Ther 2011; 339:313-320.
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Saiman L, Marshall BC, Mayer-Hamblett N, et al. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: A randomised controlled trial. J Am Med Assoc 2003; 290:1749-1756.
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Effects of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: A randomized controlled trial
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Saiman L, Anstead M, Mayer-Hamblett N, et al. Effects of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: A randomized controlled trial. J Am Med Assoc 2010; 303:1707-1715.
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Mayer-Hamblett, N.3
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37
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84866076450
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Effect of azithromycin on systemic markers of inflammation in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa
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Recent trial that updates information on the use of azithromycin in CF without P. aeruginosa showing improvement in lung function and markers of infection
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Ratjen F, Saiman L, Mayer-Hamblett N, et al. Effect of azithromycin on systemic markers of inflammation in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa. Chest 2012; 142:1259-1266. Recent trial that updates information on the use of azithromycin in CF without P. aeruginosa showing improvement in lung function and markers of infection.
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Chest
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Ratjen, F.1
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Mayer-Hamblett, N.3
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38
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84878665825
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Nonantibiotic macrolides prevent human neutrophil elastase-induced mucus stasis and airway surface liquid volume depletion
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Describes a novel macrolide anti-inflammatory with reduced antibacterial function
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Tarran R, Sabater JR, Clarke TC, et al. Nonantibiotic macrolides prevent human neutrophil elastase-induced mucus stasis and airway surface liquid volume depletion. Am J Physiol Lung Cell Mol Physiol 2013; 304:L746-L756. Describes a novel macrolide anti-inflammatory with reduced antibacterial function.
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Am J Physiol Lung Cell Mol Physiol
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Tarran, R.1
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39
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84865975368
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Doxycycline exhibits antiinflammatory activity in CF bronchial epithelial cells
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In-vitro demonstration of the anti-inflammatory properties of doxycycline, reducing IL-8 and MMPs
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Bensman TJ, Nguyen AN, Rao AP, Beringer PM. Doxycycline exhibits antiinflammatory activity in CF bronchial epithelial cells. Pulm Pharmacol Ther 2012; 25:377-382. In-vitro demonstration of the anti-inflammatory properties of doxycycline, reducing IL-8 and MMPs.
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Pulm Pharmacol Ther
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Bensman, T.J.1
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40
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36849020586
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Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease
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Hartl D, Latzin P, Hordijk P, et al. Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease. Nat Med 2007; 13:1423-1430.
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Hartl, D.1
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41
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84876987955
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Safety and early treatment effects of the CXCR2 antagonist SB-656933 in patients with cystic fibrosis
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An early stage trial of a CXCR2 inhibitor in cystic fibrosis
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Moss RB, Mistry SJ, Konstan MW, et al. Safety and early treatment effects of the CXCR2 antagonist SB-656933 in patients with cystic fibrosis. J Cyst Fibros 2013; 12:241-248. An early stage trial of a CXCR2 inhibitor in cystic fibrosis.
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Moss, R.B.1
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Impact of Mannose Binding Lectin (MBL) Insufficiency on the Course of Cystic Fibrosis: A Review and Metaanalysis
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Chalmers JD, Fleming GB, Hill AT, Kilpatrick DC. Impact of mannose binding lectin (MBL) insufficiency on the course of cystic fibrosis: A review and metaanalysis. Glycobiology 2011; 21:271-282.
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Chalmers, J.D.1
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43
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84867865314
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Polymorphisms in the lectin pathway genes as a possible cause of early chronic Pseudomonas aeruginosa colonization in cystic fibrosis patients
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Interesting small study relating genetic deficiency in the opsonins ficolin-1 and ficolin-2 with P. aeruginosa colonization in CF
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Haerynck F, Van Steen K, Cattaert T, et al. Polymorphisms in the lectin pathway genes as a possible cause of early chronic Pseudomonas aeruginosa colonization in cystic fibrosis patients. Hum Immunol 2012; 73:1175-1183. Interesting small study relating genetic deficiency in the opsonins ficolin-1 and ficolin-2 with P. aeruginosa colonization in CF.
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Haerynck, F.1
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Chiarini M, Sabelli C, Melotti P, et al. PTX3 genetic variations affect the risk of Pseudomonas aeruginosa airway colonization in cystic fibrosis patients. Genes Immun 2010; 11:665-670.
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Mannose binding lectin deficiency and disease severity in non-CF bronchiectasis: A prospective study
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Chalmers JD, McHugh BJ, Doherty CJ, et al. Mannose binding lectin deficiency and disease severity in non-CF bronchiectasis: A prospective study. Lancet Respir Med 2013; 1:175-274.
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Response of CFTR-deficient mice to long-term chronic Pseudomonas aeruginosa infection and PTX3 therapy
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Paroni M, Moalli F, Nebuloni M, et al. Response of CFTR-deficient mice to long-term chronic Pseudomonas aeruginosa infection and PTX3 therapy. J Infect Dis 2013; 208:130-138.
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Paroni, M.1
Moalli, F.2
Nebuloni, M.3
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