Free Access
Issue
Med Sci (Paris)
Volume 35, Number 12, Décembre 2019
Anticorps monoclonaux en thérapeutique
Page(s) 1217 - 1220
Section Le Magazine
DOI https://doi.org/10.1051/medsci/2019234
Published online 06 January 2020
  1. Boucher RC. Cystic fibrosis: a disease of vulnerability to airway surface dehydration. Trends Mol Med 2007 ; 13: 231–240. [CrossRef] [PubMed] [Google Scholar]
  2. Quinton PM. Role of epithelial HCO3- transport in mucin secretion: lessons from cystic fibrosis. Am J Physiol Cell Physiol 2010 ; 299: C1222–C1233. [CrossRef] [PubMed] [Google Scholar]
  3. De Lisle RC, Borowitz D. The cystic fibrosis intestine. Cold Spring Harb Perspect Med 2013 ; 3: a009753. [Google Scholar]
  4. Chmiel JF, Davis PB. State of the art: why do the lungs of patients with cystic fibrosis become infected and why can’t they clear the infection?. Respir Res 2003 ; 4: 8. [CrossRef] [PubMed] [Google Scholar]
  5. Pezzulo AA, Tang XX, Hoegger MJ, et al. Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. Nature 2012 ; 487: 109–113. [CrossRef] [Google Scholar]
  6. Rosenow T, Oudraad MC, Murray CP, et al. PRAGMA-CF. A quantitative structural lung disease computed tomography outcome in young children with cystic fibrosis. Am J Respir Crit Care Med 2015 ; 191: 1158–1165. [CrossRef] [PubMed] [Google Scholar]
  7. Esther CR, Jr., Muhlebach MS, Ehre C, et al. Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis. Sci Transl Med 2019; 11. doi: 10.1126. [CrossRef] [Google Scholar]
  8. Button B, Cai LH, Ehre C, et al. A periciliary brush promotes the lung health by separating the mucus layer from airway epithelia. Science 2012 ; 337: 937–941. [CrossRef] [PubMed] [Google Scholar]
  9. Henderson AG, Ehre C, Button B, et al. Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure. J Clin Invest 2014 ; 124: 3047–3060. [CrossRef] [PubMed] [Google Scholar]
  10. Singh PK, Jia HP, Wiles K, et al. Production of b-defensins by human airway epithelia. Proc Natl Acad Sci USA 1998 ; 95: 14961–14966. [CrossRef] [Google Scholar]
  11. Muhlebach MS, Zorn BT, Esther CR, et al. Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children. PLoS Pathog 2018 ; 14: e1006798. [CrossRef] [PubMed] [Google Scholar]
  12. Okuda K, Chen G, Subramani DB, et al. Localization of secretory mucins MUC5AC and MUC5B in normal/healthy human airways. Am J Respir Crit Care Med 2019 ; 199: 715–727. [CrossRef] [PubMed] [Google Scholar]
  13. Verdugo P. Supramolecular dynamics of mucus. Cold Spring Harb Perspect Med 2012; 2. [CrossRef] [Google Scholar]
  14. Yuan S, Hollinger M, Lachowicz-Scroggins ME, et al. Oxidation increases mucin polymer cross-links to stiffen airway mucus gels. Sci Transl Med 2015; 7: 276ra27. [CrossRef] [Google Scholar]
  15. Shak S, Capon DJ, Hellmiss R, et al. Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum. Proc Natl Acad Sci USA 1990 ; 87: 9188–9192. [CrossRef] [Google Scholar]
  16. Fuchs HJ, Borowitz DS, Christiansen DH, et al. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme study group. N Engl J Med 1994 ; 331: 637–642. [CrossRef] [Google Scholar]
  17. Tam J, Nash EF, Ratjen F, et al. Nebulized and oral thiol derivatives for pulmonary disease in cystic fibrosis. Cochrane Database Syst Rev 2013: CD007168. [PubMed] [Google Scholar]
  18. Nash EF, Stephenson A, Ratjen F, Tullis E. Nebulized and oral thiol derivatives for pulmonary disease in cystic fibrosis. Cochrane Database Syst Rev 2009; CD007168. [PubMed] [Google Scholar]
  19. Ehre C, Rushton ZL, Wang B, et al. An improved inhaled mucolytic to treat airway muco-obstructive diseases. Am J Respir Crit Care Med 2019 ; 199: 171–180. [CrossRef] [PubMed] [Google Scholar]

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