Student or educator? Sign up with your school email and get 6 months of Pro free.
Cited by 378

The DOI matches its Crossref record; 16 of the 16 references it cites were found in a registry or an index.

Cystic fibrosis lung environment and Pseudomonas aeruginosa infection

Open access
Bhagirath, Anjali Y., Li, Yanqi, Somayajula, Deepti, Dadashi, Maryam, Badr, Sara, Duan, Kangmin·2016·BMC Pulmonary Medicine

Abstract

BACKGROUND: The airways of patients with cystic fibrosis (CF) are highly complex, subject to various environmental conditions as well as a distinct microbiota. Pseudomonas aeruginosa is recognized as one of the most important pulmonary pathogens and the predominant cause of morbidity and mortality in CF. A multifarious interplay between the host, pathogens, microbiota, and the environment shapes the course of the disease. There have been several excellent reviews detailing CF pathology, Pseudomonas and the role of environment in CF but only a few reviews connect these entities with regards to influence on the overall course of the disease. A holistic understanding of contributing factors is pertinent to inform new research and therapeutics. DISCUSSION: In this article, we discuss the deterministic alterations in lung physiology as a result of CF. We also revisit the impact of those changes on the microbiota, with special emphasis on P. aeruginosa and the influence of other non-genetic factors on CF. Substantial past and current research on various genetic and non-genetic aspects of cystic fibrosis has been reviewed to assess the effect of different factors on CF pulmonary infection. A thorough review of contributing factors in CF and the alterations in lung physiology indicate that CF lung infection is multi-factorial with no isolated cause that should be solely targeted to control disease progression. A combinatorial approach may be required to ensure better disease outcomes. CONCLUSION: CF lung infection is a complex disease and requires a broad multidisciplinary approach to improve CF disease outcomes. A holistic understanding of the underlying mechanisms and non-genetic contributing factors in CF is central to development of new and targeted therapeutic strategies.

Overview

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

A few free checks a day · papers already verified open instantly · results are public

Integrity signals

Registry

Crossref · 10.1186/s12890-016-0339-5

Cites

16 of 16 references verified0 citation errors · 0 not checkable

Statistics

Verification

Claims the paper makes

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

A few free checks a day · papers already verified open instantly · results are public

Self-check

Percentages, sums and reported differences re-computed from the paper’s own numbers.

The self-check needs the paper’s full text.

Citations

Cites

Show all 16 references

Research works printed but not linked: 217

  1. Davies JC, Ebdon AM, Orchard C. Recent advances in the management of cystic fibrosis. Arch Dis Child. 2014;99(11):1033–6. · DOI
  2. Scotet V, Duguépéroux I, Saliou P, Rault G, Roussey M, Audrézet M, Férec C. Evidence for decline in the incidence of cystic fibrosis: a 35-year observational study in Brittany, France. Orphanet J Rare Dis. 2012;7:14. · DOI
  3. Sawicki GS, Ren CL, Konstan MW, Millar SJ, Pasta DJ, Quittner AL. Treatment complexity in cystic fibrosis: trends over time and associations with site-specific outcomes. J Cyst Fibros. 2013;12(5):461–7. · DOI
  4. Dewulf J, Vermeulen F, Wanyama S, Thomas M, Proesmans M, Dupont L, De Boeck K. Treatment burden in patients with at least one class IV or V CFTR mutation. Pediatr Pulmonol. 2015;50(12):1230–6. · DOI
  5. Sawicki GS, Goss CH. Tackling the increasing complexity of CF care. Pediatr Pulmonol. 2015;50(S40):S74–9. · DOI
  6. Sawicki GS, Tiddens H. Managing treatment complexity in cystic fibrosis: challenges and opportunities. Pediatr Pulmonol. 2012;47(6):523–33. · DOI
  7. Cuthbertson L, Rogers GB, Walker AW, Oliver A, Green LE, Daniels TW, Carroll MP, Parkhill J, Bruce KD, Van Der Gast CJ. Respiratory microbiota resistance and resilience to pulmonary exacerbation and subsequent antimicrobial intervention. ISME J. 2015;10:1081–91. · DOI
  8. Sosnay PR, Siklosi KR, Van Goor F, Kaniecki K, Yu H, Sharma N, Ramalho AS, Amaral MD, Dorfman R, Zielenski J, et al. Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Genet. 2013;45(10):1160–7. · DOI · Paper page
  9. Crawford I, Maloney PC, Zeitlin PL, Guggino WB, Hyde SC, Turley H, Gatter KC, Harris A, Higgins CF. Immunocytochemical localization of the cystic fibrosis gene product CFTR. Proc Natl Acad Sci U S A. 1991;88(20):9262–6. · DOI · Paper page
  10. Ji HL, Chalfant ML, Jovov B, Lockhart JP, Parker SB, Fuller CM, Stanton BA, Benos DJ. The cytosolic termini of the beta- and gamma-ENaC subunits are involved in the functional interactions between cystic fibrosis transmembrane conductance regulator and epithelial sodium channel. J Biol Chem. 2000;275(36):27947–56. · DOI
  11. Qadri YJ, Cormet-Boyaka E, Benos DJ, Berdiev BK. CFTR regulation of epithelial sodium channel. Methods Mol Biol. 2011;742:35–50. · doi:10.1007/978-1-61779-120-8_3
  12. Nagel G, Szellas T, Riordan JR, Friedrich T, Hartung K. Non-specific activation of the epithelial sodium channel by the CFTR chloride channel. EMBO Rep. 2001;2(3):249–54. · DOI
  13. Leroy C, Prive A, Bourret JC, Berthiaume Y, Ferraro P, Brochiero E. Regulation of ENaC and CFTR expression with K+ channel modulators and effect on fluid absorption across alveolar epithelial cells. Am J Physiol Lung Cell Mol Physiol. 2006;291(6):L1207–19. · doi:10.1152/ajplung.00376.2005
  14. Lu M, Leng Q, Egan ME, Caplan MJ, Boulpaep EL, Giebisch GH, Hebert SC. CFTR is required for PKA-regulated ATP sensitivity of Kir1.1 potassium channels in mouse kidney. J Clin Invest. 2006;116(3):797–807. · doi:10.1172/jci26961
  15. Wang J, Haanes KA, Novak I. Purinergic regulation of CFTR and Ca2+-activated Cl− channels and K+ channels in human pancreatic duct epithelium. Am J Physiol Cell Physiol. 2013;304(7):C673–84. · DOI
  16. Gabriel SE, Clarke LL, Boucher RC, Stutts MJ. CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship. Nature. 1993;363(6426):263–6. · DOI · Paper page
  17. Egan ME, Schwiebert EM, Guggino WB. Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cells. Am J Physiol Lung Cell Mol Physiol. 1995;268(1 Pt 1):C243–51. · DOI
  18. Fischer H, Illek B, Sachs L, Finkbeiner WE, Widdicombe JH. CFTR and calcium-activated chloride channels in primary cultures of human airway gland cells of serous or mucous phenotype. Am J Physiol Lung Cell Mol Physiol. 2010;299(4):L585–94. · doi:10.1152/ajplung.00421.2009
  19. Hendrick SM, Mroz MS, Greene CM, Keely SJ, Harvey BJ. Bile acids stimulate chloride secretion through CFTR and calcium-activated Cl− channels in Calu-3 airway epithelial cells. Am J Physiol Lung Cell Mol Physiol. 2014;307(5):L407–18. · DOI
  20. Borowitz D. CFTR, bicarbonate, and the pathophysiology of cystic fibrosis. Pediatr Pulmonol. 2015;50 Suppl 40:S24–30. · DOI
  21. Xie R, Dong X, Wong C, Vallon V, Tang B, Sun J, Yang S, Dong H. Molecular mechanisms of calcium-sensing receptor-mediated calcium signaling in the modulation of epithelial ion transport and bicarbonate secretion. J Biol Chem. 2014;289(50):34642–53. · DOI
  22. Cheung KH, Leung CT, Leung GP, Wong PY. Synergistic effects of cystic fibrosis transmembrane conductance regulator and aquaporin-9 in the rat epididymis. Reprod Biol Endocrinol. 2003;68(5):1505–10. · doi:10.1095/biolreprod.102.010017
  23. Clancy JP. Cystic fibrosis transmembrane conductance regulator function in airway smooth muscle. A novel role in cystic fibrosis airway obstruction. Am J Respir Crit Care Med. 2016;193(4):352–3. · doi:10.1164/rccm.201510-2088ed
  24. Mornon JP, Lehn P, Callebaut I. Molecular models of the open and closed states of the whole human CFTR protein. Cell Mol Life Sci. 2009;66(21):3469–86. · DOI
  25. Dawson RJ, Locher KP. Structure of a bacterial multidrug ABC transporter. Nature. 2006;443(7108):180–5. · DOI · Paper page
  26. Dalton J, Kalid O, Schushan M, Ben-Tal N, Villà-Freixa J. New model of cystic fibrosis transmembrane conductance regulator proposes active channel-like conformation. J Chem Inf Model. 2012;52(7):1842–53. · DOI
  27. Serohijos AW, Hegedus T, Aleksandrov AA, He L, Cui L, Dokholyan NV, Riordan JR. Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function. Proc Natl Acad Sci U S A. 2008;105(9):3256–61. · DOI · Paper page
  28. Harris WT, Kirk KL. CFTR and cystic fibrosis. In: Ion channels and transporters of epithelia in health and disease. Edited by Hamilton KL, Devor DC. New York, NY: Springer New York; 2016: 519-52. · DOI
  29. Molinski SV, Ahmadi S, Hung M, Bear CE. Facilitating structure-function studies of CFTR modulator sites with efficiencies in mutagenesis and functional screening. J Biomol Screen. 2015;20(10):1204–17. · DOI
  30. Lewis HA, Zhao X, Wang C, Sauder JM, Rooney I, Noland BW, Lorimer D, Kearins MC, Conners K, Condon B, et al. Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure. J Biol Chem. 2005;280(2):1346–53. · DOI · Paper page
  31. Vergani P, Lockless SW, Nairn AC, Gadsby DC. CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains. Nature. 2005;433(7028):876–80. · DOI · Paper page
  32. Csanády L, Vergani P, Gadsby DC. Strict coupling between CFTR’s catalytic cycle and gating of its Cl− ion pore revealed by distributions of open channel burst durations. Proc Natl Acad Sci. 2010;107(3):1241–6. · DOI
  33. Hwang TC, Sheppard DN. Gating of the CFTR Cl− channel by ATP-driven nucleotide-binding domain dimerisation. J Physiol. 2009;587(Pt 10):2151–61. · DOI · Paper page
  34. Protasevich I, Yang Z, Wang C, Atwell S, Zhao X, Emtage S, Wetmore D, Hunt JF, Brouillette CG. Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1. Protein Sci. 2010;19(10):1917–31. · DOI
  35. Scott-Ward TS, Amaral MD. Deletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperone. FEBS J. 2009;276(23):7097–109. · DOI
  36. Wang C, Protasevich I, Yang Z, Seehausen D, Skalak T, Zhao X, Atwell S, Spencer Emtage J, Wetmore DR, Brouillette CG, et al. Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis. Protein Sci. 2010;19(10):1932–47. · DOI
  37. Mendoza JL, Schmidt A, Li Q, Nuvaga E, Barrett T, Bridges RJ, Feranchak AP, Brautigam CA, Thomas PJ. Requirements for efficient correction of deltaF508 CFTR revealed by analyses of evolved sequences. Cell. 2012;148(1–2):164–74. · DOI · Paper page
  38. Rabeh WM, Bossard F, Xu H, Okiyoneda T, Bagdany M, Mulvihill CM, Du K, Di Bernardo S, Liu Y, Konermann L, et al. Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function. Cell. 2012;148(1–2):150–63. · DOI · Paper page
  39. Meyerholz DK. Lessons learned from the cystic fibrosis pig. Theriogenology. 2016;86(1):427–32. · DOI
  40. Denny RA, Gavrin LK, Saiah E. Recent developments in targeting protein misfolding diseases. Bioorg Med Chem Lett. 2013;23(7):1935–44. · DOI
  41. Esposito S, Tosco A, Villella VR, Raia V, Kroemer G, Maiuri L. Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis. Mol Cell Pediatr. 2016;3(1):13. · DOI
  42. Rudashevskaya EL, Stockner T, Trauner M, Freissmuth M, Chiba P. Pharmacological correction of misfolding of ABC proteins. Drug Discov Today. 2014;12:e87–94. · DOI
  43. Pier GB, Grout M, Zaidi TS. 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(22):12088–93. · DOI · Paper page
  44. Jolly AL, Takawira D, Oke OO, Whiteside SA, Chang SW, Wen ER, Quach K, Evans DJ, Fleiszig SMJ. Pseudomonas aeruginosa-induced Bleb-Niche formation in epithelial cells is independent of actinomyosin contraction and enhanced by loss of cystic fibrosis transmembrane-conductance regulator osmoregulatory function. MBio. 2015;6(2):e02533–14. · doi:10.1128/mbio.02533-14
  45. Abdulrahman BA, Khweek AA, Akhter A, Caution K, Tazi M, Hassan H, Zhang Y, Rowland PD, Malhotra S, Aeffner F, et al. Depletion of the ubiquitin-binding adaptor molecule SQSTM1/p62 from macrophages harboring cftr ΔF508 mutation improves the delivery of Burkholderia cenocepacia to the autophagic machinery. J Biol Chem. 2013;288(3):2049–58. · DOI
  46. Assani K, Tazi MF, Amer AO, Kopp BT. IFN-γ stimulates autophagy-mediated clearance of Burkholderia cenocepacia in human cystic fibrosis macrophages. PLoS One. 2014;9(5):e96681. · DOI
  47. Bruscia EM, Zhang P, Barone C, Scholte BJ, Homer R, Krause DS, Egan ME. Increased susceptibility of Cftr−/− mice to LPS-induced lung remodeling. Am J Physiol Lung Cell Mol Physiol. 2016;310(8):L711–9. · DOI
  48. Shah VS, Meyerholz DK, Tang XX, Reznikov L, Abou Alaiwa M, Ernst SE, Karp PH, Wohlford-Lenane CL, Heilmann KP, Leidinger MR, et al. Airway acidification initiates host defense abnormalities in cystic fibrosis mice. Science. 2016;351(6272):503–7. · DOI · Paper page
  49. Montaudon M, Berger P, Cangini-Sacher A, De Dietrich G, Tunon-De-Lara JM, Marthan R, Laurent F. Bronchial measurement with three-dimensional quantitative thin-section CT in patients with cystic fibrosis. Radiology. 2007;242(2):573–81. · doi:10.1148/radiol.2422060030
  50. Meyerholz DK, Stoltz DA, Namati E, Ramachandran S, Pezzulo AA, Smith AR, Rector MV, Suter MJ, Kao S, Mclennan G, et al. Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children. Am J Respir Crit Care Med. 2010;182(10):1251–61. · DOI · Paper page
  51. Long FR, Williams RS, Castile RG. Structural airway abnormalities in infants and young children with cystic fibrosis. J Pediatr. 2004;144(2):154–61. · DOI · Paper page
  52. Engelhardt JF, Yankaskas JR, Ernst SA, Yang Y, Marino CR, Boucher RC, Cohn JA, Wilson JM. Submucosal glands are the predominant site of CFTR expression in the human bronchus. Nat Genet. 1992;2(3):240–8. · DOI · Paper page
  53. Basbaum CB, Jany B, Finkbeiner WE. The serous cell. Annu Rev Physiol. 1990;52(1):97–113. · DOI · Paper page
  54. Meyrick B, Reid L. Ultrastructure of cells in the human bronchial submucosal glands. J Anat. 1970;107(Pt 2):281.
  55. Meyrick B, Sturgess JM, Reid L. A reconstruction of the duct system and secretory tubules of the human bronchial submucosal gland. Thorax. 1969;24(6):729–36. · DOI · Paper page
  56. Widdicombe JH, Wine JJ. Airway gland structure and function. Physiol Rev. 2015;95(4):1241–319. · DOI · Paper page
  57. Ballard ST, Inglis SK. Liquid secretion properties of airway submucosal glands. J Physiol. 2004;556(Pt 1):1–10. · doi:10.1113/jphysiol.2003.052779
  58. Ballard ST, Trout L, Bebok Z, Sorscher EJ, Crews A. CFTR involvement in chloride, bicarbonate, and liquid secretion by airway submucosal glands. Am J Physiol. 1999;277(4 Pt 1):L694–9.
  59. Joo NS, Saenz Y, Krouse ME, Wine JJ. Mucus secretion from single submucosal glands of pig. Stimulation by carbachol and vasoactive intestinal peptide. J Biol Chem. 2002;277(31):28167–75. · DOI
  60. Inglis SK, Corboz MR, Taylor AE, Ballard ST. Effect of anion transport inhibition on mucus secretion by airway submucosal glands. Am J Physiol. 1997;272(2 Pt 1):L372–7.
  61. Trout L, Gatzy JT, Ballard ST. Acetylcholine-induced liquid secretion by bronchial epithelium: role of Cl− and HCO3− transport. Am J Physiol. 1998;275(6 Pt 1):L1095–9.
  62. Ferroni A, Guillemot D, Moumile K, Bernede C, Le Bourgeois M, Waernessyckle S, Descamps P, Sermet-Gaudelus I, Lenoir G, Berche P, et al. Effect of mutator Pseudomonas aeruginosa on antibiotic resistance acquisition and respiratory function in cystic fibrosis. Pediatr Pulmonol. 2009;44(8):820–5. · doi:10.1002/ppul.21076
  63. Hogardt M, Heesemann J. Microevolution of Pseudomonas aeruginosa to a chronic pathogen of the cystic fibrosis lung. Curr Top Microbiol Immunol. 2013;358:91–118.
  64. Oliver A. Mutators in cystic fibrosis chronic lung infection: prevalence, mechanisms, and consequences for antimicrobial therapy. Int J Med Microbiol. 2010;300(8):563–72. · DOI
  65. Oliver A, Mena A. Bacterial hypermutation in cystic fibrosis, not only for antibiotic resistance. Clin Microbiol Infect. 2010;16(7):798–808. · DOI · Paper page
  66. Warren AE, Boulianne-Larsen CM, Chandler CB, Chiotti K, Kroll E, Miller SR, Taddei F, Sermet-Gaudelus I, Ferroni A, Mcinnerney K, et al. Genotypic and phenotypic variation in Pseudomonas aeruginosa reveals signatures of secondary infection and mutator activity in certain cystic fibrosis patients with chronic lung infections. Infect Immun. 2011;79(12):4802–18. · DOI
  67. López-Causapé C, Rojo-Molinero E, Macià MD, Oliver A. The problems of antibiotic resistance in cystic fibrosis and solutions. Expert Rev Respir Med. 2015;9(1):73–88. · DOI
  68. Boutin S, Graeber SY, Weitnauer M, Panitz J, Stahl M, Clausznitzer D, Kaderali L, Einarsson G, Tunney MM, Elborn JS, et al. Comparison of microbiomes from different niches of upper and lower airways in children and adolescents with cystic fibrosis. PLoS One. 2015;10(1):e0116029. · DOI · Paper page
  69. Fodor AA, Klem ER, Gilpin DF, Elborn JS, Boucher RC, Tunney MM, Wolfgang MC. The adult cystic fibrosis airway microbiota is stable over time and infection type, and highly resilient to antibiotic treatment of exacerbations. PLoS One. 2012;7(9):e45001. · DOI · Paper page
  70. Whelan FJ, Surette MG. Clinical insights into pulmonary exacerbations in cystic fibrosis from the microbiome. What are we missing? Ann Am Thorac Soc. 2015;12 Suppl 2:S207–11. · DOI
  71. Lamblin G, Degroote S, Perini JM, Delmotte P, Scharfman A, Davril M, Lo-Guidice JM, Houdret N, Dumur V, Klein A, et al. Human airway mucin glycosylation: a combinatory of carbohydrate determinants which vary in cystic fibrosis. Glycoconj J. 2001;18(9):661–84. · DOI · Paper page
  72. Huffnagle GB, Dickson RP. The bacterial microbiota in inflammatory lung diseases. Clin Immunol. 2015;159(2):177–82. · DOI
  73. Boucher RC, Cotton CU, Gatzy JT, Knowles MR, Yankaskas JR. Evidence for reduced Cl− and increased Na+ permeability in cystic fibrosis human primary cell cultures. J Physiol. 1988;405:77–103. · DOI · Paper page
  74. Abou Alaiwa MH, Beer AM, Pezzulo AA, Launspach JL, Horan RA, Stoltz DA, Starner TD, Welsh MJ, Zabner J. Neonates with cystic fibrosis have a reduced nasal liquid pH; a small pilot study. J Cyst Fibros. 2014;13(4):373–7. · DOI
  75. Mcshane D, Davies JC, Davies MG, Bush A, Geddes DM, Alton EW. Airway surface pH in subjects with cystic fibrosis. Eur Respir J. 2003;21(1):37–42. · doi:10.1183/09031936.03.00027603
  76. Tang XX, Ostedgaard LS, Hoegger MJ, Moninger TO, Karp PH, Mcmenimen JD, Choudhury B, Varki A, Stoltz DA, Welsh MJ. Acidic pH increases airway surface liquid viscosity in cystic fibrosis. J Clin Invest. 2016;126(3):879–91. · DOI · Paper page
  77. Pezzulo AA, Tang XX, Hoegger MJ, Alaiwa MH, Ramachandran S, Moninger TO, Karp PH, Wohlford-Lenane CL, Haagsman HP, Van Eijk M, et al. Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. Nature. 2012;487(7405):109–13. · DOI · Paper page
  78. Stigliani M, Manniello MD, Zegarra-Moran O, Galietta L, Minicucci L, Casciaro R, Garofalo E, Incarnato L, Aquino RP, Del Gaudio P. Rheological properties of cystic fibrosis bronchial secretion and in vitro drug permeation study: the effect of sodium bicarbonate. J Aerosol Med Pulm Drug Deliv. 2016;29(4):337–45. · DOI
  79. Lai SK, Wang YY, Wirtz D, Hanes J. Micro and macrorheology of mucus. Adv Drug Del Rev. 2009;61(2):86–100. · DOI · Paper page
  80. Worlitzsch D, Tarran R, Ulrich M, Schwab U, Cekici A, Meyer KC, Birrer P, Bellon G, Berger J, Weiss T, et al. Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients. J Clin Invest. 2002;109(3):317–25. · DOI · Paper page
  81. Leeper-Woodford SK, Detmer K. Acute hypoxia increases alveolar macrophage tumor necrosis factor activity and alters NF-κB expression. Am J Physiol Lung Cell Mol Physiol. 1999;276(6 Pt 1):L909–16. · DOI
  82. Borriello G, Werner E, Roe F, Kim AM, Ehrlich GD, Stewart PS. Oxygen limitation contributes to antibiotic tolerance of Pseudomonas aeruginosa in biofilms. Antimicrob Agents Chemother. 2004;48(7):2659–64. · DOI · Paper page
  83. Zimakoff J, Hoiby N, Rosendal K, Guilbert JP. Epidemiology of Pseudomonas aeruginosa infection and the role of contamination of the environment in a cystic fibrosis clinic. J Hosp Infect. 1983;4(1):31–40. · doi:10.1016/0195-6701(83)90062-2
  84. Zembrzuska-Sadkowska E, Sneum M, Ojeniyi B, Heiden L, Hoiby N. Epidemiology of Pseudomonas aeruginosa infection and the role of contamination of the environment in the danish cystic fibrosis centre. J Hosp Infect. 1995;29(1):1–7. · doi:10.1016/0195-6701(95)90288-0
  85. Farrell PM, Shen G, Splaingard M, Colby CE, Laxova A, Kosorok MR, Rock MJ, Mischler EH. Acquisition of Pseudomonas aeruginosa in children with cystic fibrosis. Pediatrics. 1997;100(5):E2. · DOI
  86. Maselli JH, Sontag MK, Norris JM, Mackenzie T, Wagener JS, Accurso FJ. Risk factors for initial acquisition of Pseudomonas aeruginosa in children with cystic fibrosis identified by newborn screening. Pediatr Pulmonol. 2003;35(4):257–62. · DOI
  87. Koch C. Early infection and progression of cystic fibrosis lung disease. Pediatr Pulmonol. 2002;34(3):232–6. · DOI
  88. Tingpej P, Smith L, Rose B, Zhu H, Conibear T, Al Nassafi K, Manos J, Elkins M, Bye P, Willcox M, et al. Phenotypic characterization of clonal and nonclonal Pseudomonas aeruginosa strains isolated from lungs of adults with cystic fibrosis. J Clin Microbiol. 2007;45(6):1697–704. · DOI
  89. Emerson J, Rosenfeld M, Mcnamara S, Ramsey B, Gibson RL. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatr Pulmonol. 2002;34(2):91–100. · DOI · Paper page
  90. Hudson VL, Wielinski CL, Regelmann WE. Prognostic implications of initial oropharyngeal bacterial flora in patients with cystic fibrosis diagnosed before the age of two years. J Pediatr. 1993;122(6):854–60. · DOI
  91. Goodman AL, Kulasekara B, Rietsch A, Boyd D, Smith RS, Lory S. A signaling network reciprocally regulates genes associated with acute infection and chronic persistence in Pseudomonas aeruginosa. Dev Cell. 2004;7(5):745–54. · DOI · Paper page
  92. Del Campo R, Morosini MI, De La Pedrosa EG, Fenoll A, Munoz-Almagro C, Maiz L, Baquero F, Canton R. Population structure, antimicrobial resistance, and mutation frequencies of Streptococcus pneumoniae isolates from cystic fibrosis patients. J Clin Microbiol. 2005;43(5):2207–14. · DOI
  93. Nixon GM, Armstrong DS, Carzino R, Carlin JB, Olinsky A, Robertson CF, Grimwood K. Clinical outcome after early Pseudomonas aeruginosa infection in cystic fibrosis. J Pediatr. 2001;138(5):699–704. · DOI · Paper page
  94. Mccarthy RR, Mooij MJ, Reen FJ, Lesouhaitier O, O’gara F. A new regulator of pathogenicity (bvlR) is required for full virulence and tight microcolony formation in Pseudomonas aeruginosa. Microbiology. 2014;160(Pt 7):1488–500. · doi:10.1099/mic.0.075291-0
  95. Kong W, Chen L, Zhao J, Shen T, Surette MG, Shen L, Duan K. Hybrid sensor kinase PA1611 in Pseudomonas aeruginosa regulates transitions between acute and chronic infection through direct interaction with RetS. Mol Microbiol. 2013;88(4):784–97. · DOI
  96. Ventre I, Goodman AL, Vallet-Gely I, Vasseur P, Soscia C, Molin S, Bleves S, Lazdunski A, Lory S, Filloux A. Multiple sensors control reciprocal expression of Pseudomonas aeruginosa regulatory RNA and virulence genes. Proc Natl Acad Sci U S A. 2006;103(1):171–6. · DOI · Paper page
  97. Munck A, Bonacorsi S, Mariani-Kurkdjian P, Lebourgeois M, Gerardin M, Brahimi N, Navarro J, Bingen E. Genotypic characterization of Pseudomonas aeruginosa strains recovered from patients with cystic fibrosis after initial and subsequent colonization. Pediatr Pulmonol. 2001;32(4):288–92. · doi:10.1002/ppul.1121
  98. Johansen HK, Hoiby N. Seasonal onset of initial colonisation and chronic infection with Pseudomonas aeruginosa in patients with cystic fibrosis in Denmark. Thorax. 1992;47(2):109–11. · DOI · Paper page
  99. Kolpen M, Hansen CR, Bjarnsholt T, Moser C, Christensen LD, Van Gennip M, Ciofu O, Mandsberg L, Kharazmi A, Doring G, et al. Polymorphonuclear leucocytes consume oxygen in sputum from chronic Pseudomonas aeruginosa pneumonia in cystic fibrosis. Thorax. 2009;65(1):57–62. · DOI · Paper page
  100. Yang L, Chen L, Shen L, Surette M, Duan K. Inactivation of MuxABC-OpmB transporter system in Pseudomonas aeruginosa leads to increased ampicillin and carbenicillin resistance and decreased virulence. J Microbiol. 2011;49(1):107–14. · DOI
  101. Hauser AR, Jain M, Bar-Meir M, Mccolley SA. Clinical significance of microbial infection and adaptation in cystic fibrosis. Clin Microbiol Rev. 2011;24(1):29–70. · DOI · Paper page
  102. Wood LF, Ohman DE. Identification of genes in the σ22 regulon of Pseudomonas aeruginosa required for cell envelope homeostasis in either the planktonic or the sessile mode of growth. MBio. 2012;3(3):e00094–00012.
  103. Wu D, Wang J, Zhang M. Altered Th17/Treg ratio in nasal polyps with distinct cytokine profile: association with patterns of inflammation and mucosal remodeling. Medicine. 2016;95(10):e2998. · DOI
  104. Tai AS, Bell SC, Kidd TJ, Trembizki E, Buckley C, Ramsay KA, David M, Wainwright CE, Grimwood K, Whiley DM. Genotypic diversity within a single Pseudomonas aeruginosa strain commonly shared by australian patients with cystic fibrosis. PLoS One. 2015;10(12):e0144022. · DOI
  105. Workentine ML, Sibley CD, Glezerson B, Purighalla S, Norgaard-Gron JC, Parkins MD, Rabin HR, Surette MG. Phenotypic heterogeneity of Pseudomonas aeruginosa populations in a cystic fibrosis patient. PLoS One. 2013;8(4):e60225. · DOI · Paper page
  106. Feliziani S, Marvig RL, Luján AM, Moyano AJ, Di Rienzo JA, Krogh Johansen H, Molin S, Smania AM. Coexistence and within-host evolution of diversified lineages of hypermutable Pseudomonas aeruginosa in long-term cystic fibrosis infections. PLoS Genet. 2014;10(10):e1004651. · DOI
  107. Duan K, Dammel C, Stein J, Rabin H, Surette MG. Modulation of Pseudomonas aeruginosa gene expression by host microflora through interspecies communication. Mol Microbiol. 2003;50(5):1477–91. · DOI · Paper page
  108. Sibley CD, Duan K, Fischer C, Parkins MD, Storey DG, Rabin HR, Surette MG. Discerning the complexity of community interactions using a drosophila model of polymicrobial infections. PLoS Pathog. 2008;4(10):e1000184. · DOI · Paper page
  109. Sibley CD, Rabin H, Surette MG. Cystic fibrosis: a polymicrobial infectious disease. Future Microbiol. 2006;1(1):53–61. · DOI
  110. Pernet E, Guillemot L, Burgel PR, Martin C, Lambeau G, Sermet-Gaudelus I, Sands D, Leduc D, Morand PC, Jeammet L, et al. Pseudomonas aeruginosa eradicates Staphylococcus aureus by manipulating the host immunity. Nat Commun. 2014;5:5105. · DOI
  111. Filkins LM, Graber JA, Olson DG, Dolben EL, Lynd LR, Bhuju S, O’toole GA. Coculture of Staphylococcus aureus with Pseudomonas aeruginosa drives S. aureus towards fermentative metabolism and reduced viability in a cystic fibrosis model. J Bacteriol. 2015;197(14):2252–64. · DOI · Paper page
  112. Chekabab SM, Silverman RJ, Lafayette SL, Luo Y, Rousseau S, Nguyen D. Staphylococcus aureus inhibits IL-8 responses induced by Pseudomonas aeruginosa in airway epithelial cells. PLoS One. 2015;10(9):e0137753. · DOI
  113. Deng Y, Boon C, Chen S, Lim A, Zhang LH. Cis-2-dodecenoic acid signal modulates virulence of Pseudomonas aeruginosa through interference with quorum sensing systems and T3SS. BMC Microbiol. 2013;13:231. · DOI
  114. Stroobant J. Viral infection in cystic fibrosis. J R Soc Med. 1986;79 Suppl 12:19–22.
  115. Wat D, Gelder C, Hibbitts S, Cafferty F, Bowler I, Pierrepoint M, Evans R, Doull I. The role of respiratory viruses in cystic fibrosis. J Cyst Fibros. 2008;7(4):320–8. · DOI · Paper page
  116. Brownlee JW, Turner RB. New developments in the epidemiology and clinical spectrum of rhinovirus infections. Curr Opin Pediatr. 2008;20(1):67–71. · DOI
  117. De Voss JJ, Rutter K, Schroeder BG, Barry CE. Iron acquisition and metabolism by mycobacteria. J Bacteriol. 1999;181(15):4443–51. · DOI · Paper page
  118. Zoued A, Brunet YR, Durand E, Aschtgen MS, Logger L, Douzi B, Journet L, Cambillau C, Cascales E. Architecture and assembly of the type VI secretion system. Biochim Biophys Acta. 2014;1843(8):1664–73. · DOI · Paper page
  119. Eichner A, Gunther N, Arnold M, Schobert M, Heesemann J, Hogardt M. Marker genes for the metabolic adaptation of Pseudomonas aeruginosa to the hypoxic cystic fibrosis lung environment. Int J Med Microbiol. 2014;304(8):1050–61. · DOI
  120. Hood RD, Singh P, Hsu F, Guvener T, Carl MA, Trinidad RR, Silverman JM, Ohlson BB, Hicks KG, Plemel RL, et al. A type VI secretion system of Pseudomonas aeruginosa targets a toxin to bacteria. Cell Host Microbe. 2010;7(1):25–37. · DOI · Paper page
  121. Benz J, Sendlmeier C, Barends TR, Meinhart A. Structural insights into the effector-immunity system Tse1/Tsi1 from Pseudomonas aeruginosa. PLoS One. 2012;7(7):e40453. · DOI
  122. Russell AB, Hood RD, Bui NK, Leroux M, Vollmer W, Mougous JD. Type VI secretion delivers bacteriolytic effectors to target cells. Nature. 2011;475(7356):343–7. · DOI · Paper page
  123. Sibley CD, Surette MG. The polymicrobial nature of airway infections in cystic fibrosis: cangene gold medal lecture. Can J Microbiol. 2011;57(2):69–77. · DOI
  124. Green H, Jones AM. The microbiome and emerging pathogens in cystic fibrosis and non-cystic fibrosis bronchiectasis. Semin Respir Crit Care Med. 2015;36(2):225–35. · DOI
  125. Keravec M, Mounier J, Prestat E, Vallet S, Jansson JK, Burgaud G, Rosec S, Gouriou S, Rault G, Coton E, et al. Insights into the respiratory tract microbiota of patients with cystic fibrosis during early Pseudomonas aeruginosa colonization. Springerplus. 2015;4(1):405. · DOI
  126. Willger SD, Grim SL, Dolben EL, Shipunova A, Hampton TH, Morrison HG, Filkins LM, O’toole GA, Moulton LA, Ashare A, et al. Characterization and quantification of the fungal microbiome in serial samples from individuals with cystic fibrosis. Microbiome. 2014;2:40. · DOI
  127. Moran LP, Chouvarine P, Dorda M, Hedtfeld S, Mielke S, Schulz A, Wiehlmann L, Tummler B. The cystic fibrosis lower airways microbial metagenome. ERJ Open Res. 2016;2:2.
  128. Hoen AG, Li J, Moulton LA, O’toole GA, Housman ML, Koestler DC, Guill MF, Moore JH, Hibberd PL, Morrison HG, et al. Associations between gut microbial colonization in early life and respiratory outcomes in cystic fibrosis. J Pediatr. 2015;167(1):138–47. · DOI · Paper page
  129. Renwick J, Mcnally P, John B, Desantis T, Linnane B, Murphy P, On Behalf Of Shield Cf. The microbial community of the cystic fibrosis airway is disrupted in early life. PLoS One. 2014;9(12):e109798. · DOI
  130. Coutinho HD, Falcão-Silva VS, Gonçalves GF. Pulmonary bacterial pathogens in cystic fibrosis patients and antibiotic therapy: a tool for the health workers. Int Arch Med. 2008;1:24. · doi:10.1186/1755-7682-1-24
  131. Coburn B, Wang PW, Diaz Caballero J, Clark ST, Brahma V, Donaldson S, Zhang Y, Surendra A, Gong Y, Elizabeth Tullis D, et al. Lung microbiota across age and disease stage in cystic fibrosis. Sci Rep. 2015;5(5):10241. · DOI · Paper page
  132. Middleton PG, Chen SC, Meyer W. Fungal infections and treatment in cystic fibrosis. Curr Opin Pulm Med. 2013;19(6):670–5. · DOI
  133. Parize P, Billaud S, Bienvenu AL, Bourdy S, Le Pogam MA, Reix P, Picot S, Robert R, Lortholary O, Bouchara JP, et al. Impact of Scedosporium apiospermum complex seroprevalence in patients with cystic fibrosis. J Cyst Fibros. 2014;13(6):667–73. · DOI
  134. Pihet M, Carrere J, Cimon B, Chabasse D, Delhaes L, Symoens F, Bouchara JP. Occurrence and relevance of filamentous fungi in respiratory secretions of patients with cystic fibrosis-a review. Med Mycol. 2009;47(4):387–97. · DOI · Paper page
  135. Mouhajir A, Matray O, Giraud S, Mély L, Marguet C, Sermet-Gaudelus I, Le Gal S, Labbé F, Person C, Troussier F, et al. Long-term Rasamsonia argillacea complex species colonization revealed by PCR amplification of repetitive DNA sequences in cystic fibrosis patients. J Clin Microbiol. 2016;54(11):2804–12. · DOI
  136. Lim YW, Schmieder R, Haynes M, Willner D, Furlan M, Youle M, Abbott K, Edwards R, Evangelista J, Conrad D, et al. Metagenomics and metatranscriptomics: windows on CF-associated viral and microbial communities. J Cyst Fibros. 2013;12(2):154–64. · DOI · Paper page
  137. Ouennane S, Leprohon P, Moineau S. Diverse virulent pneumophages infect Streptococcus mitis. PLoS One. 2015;10(2):e0118807. · doi:10.1371/journal.pone.0118807
  138. Hector A, Frey N, Hartl D. Update on host-pathogen interactions in cystic fibrosis lung disease. Mol Cell Pediatr. 2016;3(1):12. · DOI
  139. Rogers GB, Van Der Gast CJ, Cuthbertson L, Thomson SK, Bruce KD, Martin ML, Serisier DJ. Clinical measures of disease in adult non-CF bronchiectasis correlate with airway microbiota composition. Thorax. 2013;68(8):731–7. · DOI · Paper page
  140. Stressmann FA, Rogers GB, Van Der Gast CJ, Marsh P, Vermeer LS, Carroll MP, Hoffman L, Daniels TW, Patel N, Forbes B, et al. Long-term cultivation-independent microbial diversity analysis demonstrates that bacterial communities infecting the adult cystic fibrosis lung show stability and resilience. Thorax. 2012;67(10):867–73. · DOI
  141. Cox MJ, Allgaier M, Taylor B, Baek MS, Huang YJ, Daly RA, Karaoz U, Andersen GL, Brown R, Fujimura KE, et al. Airway microbiota and pathogen abundance in age-stratified cystic fibrosis patients. PLoS One. 2010;5:e11044. · DOI · Paper page
  142. Hartl D, Gaggar A, Bruscia E, Hector A, Marcos V, Jung A, Greene C, Mcelvaney G, Mall M, Doring G. Innate immunity in cystic fibrosis lung disease. J Cyst Fibros. 2012;11(5):363–82. · DOI · Paper page
  143. Pohl K, Hayes E, Keenan J, Henry M, Meleady P, Molloy K, Jundi B, Bergin DA, Mccarthy C, Mcelvaney OJ, et al. A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy. Blood. 2014;124(7):999–1009. · DOI · Paper page
  144. Gao L, Kim KJ, Yankaskas JR, Forman HJ. Abnormal glutathione transport in cystic fibrosis airway epithelia. Am J Physiol. 1999;277(1 Pt 1):L113–8.
  145. Xu Y, Szep S, Lu Z. The antioxidant role of thiocyanate in the pathogenesis of cystic fibrosis and other inflammation-related diseases. Proc Natl Acad Sci U S A. 2009;106(48):20515–9. · DOI
  146. Branzk N, Lubojemska A, Hardison SE, Wang Q, Gutierrez MG, Brown GD, Papayannopoulos V. Neutrophils sense microbe size and selectively release neutrophil extracellular traps in response to large pathogens. Nat Immunol. 2014;15(11):1017–25. · DOI · Paper page
  147. Dubois AV, Gauthier A, Bréa D, Varaigne F, Diot P, Gauthier F, Attucci S. Influence of DNA on the activities and inhibition of neutrophil serine proteases in cystic fibrosis sputum. Am J Respir Cell Mol Biol. 2012;47(1):80–6. · DOI
  148. Gifford AM, Chalmers JD. The role of neutrophils in cystic fibrosis. Curr Opin Hematol. 2014;21(1):16–22. · DOI
  149. Wagner CJ, Schultz C, Mall MA. Neutrophil elastase and matrix metalloproteinase 12 in cystic fibrosis lung disease. Mol Cell Pediatr. 2016;3(1):25. · DOI
  150. Yoo DG, Winn M, Pang L, Moskowitz SM, Malech HL, Leto TL, Rada B. Release of cystic fibrosis airway inflammatory markers from Pseudomonas aeruginosa-stimulated human neutrophils involves NADPH oxidase-dependent extracellular DNA trap formation. J Immunol. 2014;192(10):4728–38. · DOI
  151. Descamps D, Le Gars M, Balloy V, Barbier D, Maschalidi S, Tohme M, Chignard M, Ramphal R, Manoury B, Sallenave JM. Toll-like receptor 5 (TLR5), IL-1β secretion, and asparagine endopeptidase are critical factors for alveolar macrophage phagocytosis and bacterial killing. Proc Natl Acad Sci. 2012;109(5):1619–24. · DOI
  152. Rieber N, Hector A, Carevic M, Hartl D. Current concepts of immune dysregulation in cystic fibrosis. Int J Biochem Cell Biol. 2014;52:108–12. · DOI
  153. Anil N, Singh M. CD4+ CD25 high FOXP3+ regulatory T cells correlate with FEV1 in north Indian children with cystic fibrosis. Immunol Invest. 2014;43(6):535–43. · doi:10.3109/08820139.2014.888447
  154. Ellis TN, Leiman SA, Kuehn MJ. Naturally produced outer membrane vesicles from Pseudomonas aeruginosa elicit a potent innate immune response via combined sensing of both lipopolysaccharide and protein components. Infect Immun. 2010;78(9):3822–31. · DOI · Paper page
  155. Hauser AR. The type III. secretion system of Pseudomonas aeruginosa: infection by injection. Nat Rev Microbiol. 2009;7(9):654–65. · DOI · Paper page
  156. Miao EA, Mao DP, Yudkovsky N, Bonneau R, Lorang CG, Warren SE, Leaf IA, Aderem A. Innate immune detection of the type III secretion apparatus through the NLRC4 inflammasome. Proc Natl Acad Sci. 2010;107(7):3076–80. · DOI · Paper page
  157. Von Moltke J, Ayres JS, Kofoed EM, Chavarria-Smith J, Vance RE. Recognition of bacteria by inflammasomes. Annu Rev Immunol. 2013;31:73–106. · DOI · Paper page
  158. Bomberger JM, Ely KH, Bangia N, Ye S, Green KA, Green WR, Enelow RI, Stanton BA. Pseudomonas aeruginosa Cif protein enhances the ubiquitination and proteasomal degradation of the transporter associated with antigen processing (TAP) and reduces major histocompatibility complex (MHC) class I antigen presentation. J Biol Chem. 2014;289(1):152–62. · DOI
  159. Siegmann N, Worbs D, Effinger F, Bormann T, Gebhardt M, Ulrich M, Wermeling F, Müller-Hermelink E, Biedermann T, Tighe M. Invariant natural killer T (iNKT) cells prevent autoimmunity, but induce pulmonary inflammation in cystic fibrosis. Cell Physiol Biochem. 2014;34(1):56–70. · doi:10.1159/000362984
  160. Hofer TP, Frankenberger M, Heimbeck I, Burggraf D, Wjst M, Wright AK, Kerscher M, Nahrig S, Huber RM, Fischer R, et al. Decreased expression of HLA-DQ and HLA-DR on cells of the monocytic lineage in cystic fibrosis. J Mol Med (Berl). 2014;92(12):1293–304. · doi:10.1007/s00109-014-1200-z
  161. Shijubo N, Itoh Y, Yamaguchi T, Imada A, Hirasawa M, Yamada T, Kawai T, Abe S. Clara cell protein–positive epithelial cells are reduced in small airways of asthmatics. Am J Respir Crit Care Med. 1999;160(3):930–3. · DOI
  162. Laguna TA, Williams CB, Brandy KR, Welchlin-Bradford C, Moen CE, Reilly CS, Wendt CH. Sputum club cell protein concentration is associated with pulmonary exacerbation in cystic fibrosis. J Cyst Fibros. 2015;14(3):334–40. · doi:10.1016/j.jcf.2014.10.002
  163. Cohen TS, Prince A. Cystic fibrosis: a mucosal immunodeficiency syndrome. Nat Med. 2012;18(4):509–19. · DOI · Paper page
  164. Collaco JM, Blackman SM, Mcgready J, Naughton KM, Cutting GR. Quantification of the relative contribution of environmental and genetic factors to variation in cystic fibrosis lung function. J Pediatr. 2010;157(5):802–7. · DOI
  165. Schechter MS. Non-genetic influences on cystic fibrosis lung disease: the role of sociodemographic characteristics, environmental exposures, and healthcare interventions. Semin Respir Crit Care Med. 2003;24(6):639–52. · doi:10.1055/s-2004-815660
  166. Schechter MS. Wealth as a disease modifier in cystic fibrosis. Lancet Respir Med. 2013;1(2):93–5. · doi:10.1016/s2213-2600(13)70014-6
  167. Schoni MH, Casaulta-Aebischer C. Nutrition and lung function in cystic fibrosis patients: review. Clin Nutr. 2000;19(2):79–85. · DOI
  168. Dror T, Dickstein Y, Dubourg G, Paul M. Microbiota manipulation for weight change. Microb Pathog. 2016. http://dx.doi.org/10.1016/j.micpath.2016.01.002 . Epub ahead of print. · DOI
  169. Psoter KJ, De Roos AJ, Mayer JD, Kaufman JD, Wakefield J, Rosenfeld M. Fine particulate matter exposure and initial Pseudomonas aeruginosa acquisition in cystic fibrosis. Ann Am Thorac Soc. 2015;12(3):385–91. · DOI
  170. Jassal MS, Yu AM, Bhatia R, Keens TG, Davidson Ward SL. Effect of residential proximity to major roadways on cystic fibrosis exacerbations. Int J Environ Health Res. 2013;23(2):119–31. · DOI
  171. Welsh MJ. Cigarette smoke inhibition of ion transport in canine tracheal epithelium. J Clin Invest. 1983;71(6):1614–23. · DOI
  172. Kopp B, Ortega-García J, Sadreameli S, Wellmerling J, Cormet-Boyaka E, Thompson R, Mcgrath-Morrow S, Groner J. The impact of secondhand smoke exposure on children with cystic fibrosis: a review. Int J Environ Res Public Health. 2016;13(10):1003. · DOI
  173. Kopp BT, Sarzynski L, Khalfoun S, Hayes Jr D, Thompson R, Nicholson L, Long F, Castile R, Groner J. Detrimental effects of secondhand smoke exposure on infants with cystic fibrosis. Pediatr Pulmonol. 2015;50(1):25–34. · DOI
  174. Rand S, Prasad SA. Exercise as part of a cystic fibrosis therapeutic routine. Expert Rev Respir Med. 2012;6(3):341–52. · DOI
  175. Collaco JM, Blackman SM, Raraigh KS, Morrow CB, Cutting GR, Paranjape SM. Self-reported exercise and longitudinal outcomes in cystic fibrosis: a retrospective cohort study. BMC Pulm Med. 2014;14(1):159. · doi:10.1186/1471-2466-14-159
  176. Grossoehme DH, Szczesniak RD, Mrug S, Dimitriou SM, Marshall A, Mcphail GL. Adolescents’ spirituality and cystic fibrosis airway clearance treatment adherence: examining mediators. J Pediatr Psychol. 2016;41(9):1022–32. · DOI
  177. Platten MJ, Newman E, Quayle E. Self-esteem and its relationship to mental health and quality of life in adults with cystic fibrosis. J Clin Psychol Med Settings. 2012;20(3):392–9. · DOI
  178. Besier S, Smaczny C, Mallinckrodt C, Krahl A, Ackermann H, Brade V. Prevalence and clinical significance of Staphylococcus aureus small-colony variants in cystic fibrosis lung disease. J Clin Microbiol. 2007;45(1):168–72. · DOI · Paper page
  179. Bodnar R, Kadar L, Szabo L, Hernadi M, Mikoczi M, Meszaros A. Health related quality of life of children with chronic respiratory conditions. Adv Clin Exp Med. 2015;24(3):487–95. · doi:10.17219/acem/24991
  180. Sutton MT, Fletcher D, Ghosh SK, Weinberg A, Van Heeckeren R, Kaur S, Sadeghi Z, Hijaz A, Reese J, Lazarus HM, et al. Antimicrobial properties of mesenchymal stem cells: therapeutic potential for cystic fibrosis infection, and treatment. Stem Cells Int. 2016;2016:1–12. · DOI · Paper page
  181. Martiniano SL, Sagel SD, Zemanick ET. Cystic fibrosis. Curr Opin Pediatr. 2016;28(3):312–7. · DOI
  182. Sinagoga KL, Wells JM. Generating human intestinal tissues from pluripotent stem cells to study development and disease. EMBO J. 2015;34(9):1149–63. · DOI
  183. Mcneer NA, Anandalingam K, Fields RJ, Caputo C, Kopic S, Gupta A, Quijano E, Polikoff L, Kong Y, Bahal R, et al. Nanoparticles that deliver triplex-forming peptide nucleic acid molecules correct F508del CFTR in airway epithelium. Nat Commun. 2015;6:6952. · DOI
  184. Maeder ML, Gersbach CA. Genome-editing technologies for gene and cell therapy. Mol Ther. 2016;24(3):430–46. · DOI · Paper page
  185. Paul-Smith MC, Bell RV, Alton WE, Alton EWFW, Griesenbach U. Gene therapy for cystic fibrosis: recent progress and current aims. Expert Opin Orphan Drugs. 2016;4(6):649–58. · DOI
  186. Mueller C, Flotte TR. Gene therapy for cystic fibrosis. Clin Rev Allergy Immunol. 2008;35(3):164–78. · DOI
  187. Harrison MJ, Murphy DM, Plant BJ. Ivacaftor in a G551D homozygote with cystic fibrosis. N Engl J Med. 2013;369(13):1280–2. · DOI
  188. Beringer PM, Bensman TJ, Ho H, Agnello M, Denovel N, Nguyen A, Wong-Beringer A, She R, Tran Dat Q, Moskowitz SM, et al. Rhesus θ-defensin-1 (RTD-1) exhibits in vitro and in vivo activity against cystic fibrosis strains of Pseudomonas aeruginosa. J Antimicrob Chemother. 2015;71(1):181–8. · DOI
  189. Mcmillan J, Batrakova E, Gendelman HE. Cell delivery of therapeutic nanoparticles. Prog Mol Biol Transl Sci. 2011;104:563–601. · DOI
  190. Friman VP, Soanes-Brown D, Sierocinski P, Molin S, Johansen HK, Merabishvili M, Pirnay JP, De Vos D, Buckling A. Pre-adapting parasitic phages to a pathogen leads to increased pathogen clearance and lowered resistance evolution with Pseudomonas aeruginosa cystic fibrosis bacterial isolates. J Evol Biol. 2015;29(1):188–98. · DOI
  191. Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, Colombo C, Davies JC, De Boeck K, Flume PA, et al. Lumacaftor–Ivacaftor in patients with cystic fibrosis homozygous for phe508del CFTR. N Engl J Med. 2015;373(3):220–31. · DOI · Paper page
  192. Waters V, Yau Y, Prasad S, Lu A, Atenafu E, Crandall I, Tom S, Tullis E, Ratjen F. Stenotrophomonas maltophilia in cystic fibrosis: serologic response and effect on lung disease. Am J Respir Crit Care Med. 2011;183(5):635–40. · DOI · Paper page
  193. Dasenbrook EC, Merlo CA, Diener-West M, Lechtzin N, Boyle MP. Persistent methicillin-resistant Staphylococcus aureus and rate of FEV1 decline in cystic fibrosis. Am J Respir Crit Care Med. 2008;178(8):814–21. · DOI · Paper page
  194. Chmiel JF, Aksamit TR, Chotirmall SH, Dasenbrook EC, Elborn JS, Lipuma JJ, Ranganathan SC, Waters VJ, Ratjen FA. Antibiotic management of lung infections in cystic fibrosis. I. The microbiome, methicillin-resistant staphylococcus aureus, gram-negative bacteria, and multiple infections. Ann Am Thorac Soc. 2014;11(7):1120–9. · DOI · Paper page
  195. Malouf MA, Glanville AR. The spectrum of mycobacterial infection after lung transplantation. Am J Respir Crit Care Med. 1999;160(5):1611–6. · DOI · Paper page
  196. Binder AM, Adjemian J, Olivier KN, Prevots DR. Epidemiology of nontuberculous mycobacterial infections and associated chronic macrolide use among persons with cystic fibrosis. Am J Respir Crit Care Med. 2013;188(7):807–912. · DOI
  197. Mahenthiralingam E. Emerging cystic fibrosis pathogens and the microbiome. Paediatr Respir Rev. 2014;15 Suppl 1:13–15. · DOI
  198. Zemanick ET, Sagel SD, Harris JK. The airway microbiome in cystic fibrosis and implications for treatment. Curr Opin Pediatr. 2011;23(3):319–24. · DOI
  199. Tunney MM, Field TR, Moriarty TF, Patrick S, Doering G, Muhlebach MS, Wolfgang MC, Boucher R, Gilpin DF, Mcdowell A, et al. Detection of anaerobic bacteria in high numbers in sputum from patients with cystic fibrosis. Am J Respir Crit Care Med. 2008;177(9):995–1001. · DOI · Paper page
  200. Sibley CD, Grinwis ME, Field TR, Parkins MD, Norgaard JC, Gregson DB, Rabin HR, Surette MG. McKay agar enables routine quantification of the ‘Streptococcus milleri’group in cystic fibrosis patients. J Med Microbiol. 2010;59(5):534–40. · DOI

200 of 217 shown

Cited by

See all 378

Metadata

Authors
Bhagirath, A. Y., Li, Y., Somayajula, D., Dadashi, M., Badr, S., & Duan, K.
Venue
BMC Pulmonary Medicine
Published
2016-12
Volume
16
Issue
1
Pages
174-174
ISSN
1471-2466 (ISSN-L)
OpenAlex
W2560723352
Cited by
378
References
16
FWCI
19.60
Type
journal article
Language
en
Open access
gold
Funders
  • Natural Sciences and Engineering Research Council of Canada (402943-2011 RGPIN)
  • Research Manitoba (Establishment Grant)

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review

How to cite

Cite CoChat for the checks on this page, and the paper for its own findings.

This page
CoChat. (2026). Citation check: Cystic fibrosis lung environment and Pseudomonas aeruginosa infection. https://app.cochat.ai/papers/10.1186/s12890-016-0339-5
The paper
Bhagirath, A. Y., Li, Y., Somayajula, D., Dadashi, M., Badr, S., & Duan, K. (2016). Cystic fibrosis lung environment and Pseudomonas aeruginosa infection. BMC Pulmonary Medicine. https://doi.org/10.1186/s12890-016-0339-5

Data sources

Bibliographic data: Semantic Scholar, OpenAlex, Crossref.

Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.