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The microbiome in pediatric cystic fibrosis patients: the role of shared environment suggests a window of intervention.

Hampton TH, Green DM, Cutting GR, Morrison HG, Sogin ML, Gifford AH, Stanton BA, O'Toole GA.

Microbiome. 2014 Apr 28;2:14. doi: 10.1186/2049-2618-2-14. eCollection 2014.


The airway microbiome in cystic fibrosis and implications for treatment.

Zemanick ET, Sagel SD, Harris JK.

Curr Opin Pediatr. 2011 Jun;23(3):319-24. doi: 10.1097/MOP.0b013e32834604f2. Review.


The adult cystic fibrosis airway microbiota is stable over time and infection type, and highly resilient to antibiotic treatment of exacerbations.

Fodor AA, Klem ER, Gilpin DF, Elborn JS, Boucher RC, Tunney MM, Wolfgang MC.

PLoS One. 2012;7(9):e45001. doi: 10.1371/journal.pone.0045001. Epub 2012 Sep 26.


Genetic modifiers play a substantial role in diabetes complicating cystic fibrosis.

Blackman SM, Hsu S, Vanscoy LL, Collaco JM, Ritter SE, Naughton K, Cutting GR.

J Clin Endocrinol Metab. 2009 Apr;94(4):1302-9. doi: 10.1210/jc.2008-2186. Epub 2009 Jan 6.


Mouse models of chronic lung infection with Pseudomonas aeruginosa: models for the study of cystic fibrosis.

Stotland PK, Radzioch D, Stevenson MM.

Pediatr Pulmonol. 2000 Nov;30(5):413-24. Review.


Assessment of the Microbial Constituents of the Home Environment of Individuals with Cystic Fibrosis (CF) and Their Association with Lower Airways Infections.

Heirali A, McKeon S, Purighalla S, Storey DG, Rossi L, Costilhes G, Drews SJ, Rabin HR, Surette MG, Parkins MD.

PLoS One. 2016 Feb 9;11(2):e0148534. doi: 10.1371/journal.pone.0148534. eCollection 2016.


Quantification of the relative contribution of environmental and genetic factors to variation in cystic fibrosis lung function.

Collaco JM, Blackman SM, McGready J, Naughton KM, Cutting GR.

J Pediatr. 2010 Nov;157(5):802-7.e1-3. doi: 10.1016/j.jpeds.2010.05.018. Epub 2010 Jun 30.


Inflammation and airway microbiota during cystic fibrosis pulmonary exacerbations.

Zemanick ET, Harris JK, Wagner BD, Robertson CE, Sagel SD, Stevens MJ, Accurso FJ, Laguna TA.

PLoS One. 2013 Apr 30;8(4):e62917. doi: 10.1371/journal.pone.0062917. Print 2013.


Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis.

Li Z, Kosorok MR, Farrell PM, Laxova A, West SE, Green CG, Collins J, Rock MJ, Splaingard ML.

JAMA. 2005 Feb 2;293(5):581-8.


Comparison of microbiomes from different niches of upper and lower airways in children and adolescents with cystic fibrosis.

Boutin S, Graeber SY, Weitnauer M, Panitz J, Stahl M, Clausznitzer D, Kaderali L, Einarsson G, Tunney MM, Elborn JS, Mall MA, Dalpke AH.

PLoS One. 2015 Jan 28;10(1):e0116029. doi: 10.1371/journal.pone.0116029. eCollection 2015.


Serial analysis of the gut and respiratory microbiome in cystic fibrosis in infancy: interaction between intestinal and respiratory tracts and impact of nutritional exposures.

Madan JC, Koestler DC, Stanton BA, Davidson L, Moulton LA, Housman ML, Moore JH, Guill MF, Morrison HG, Sogin ML, Hampton TH, Karagas MR, Palumbo PE, Foster JA, Hibberd PL, O'Toole GA.

MBio. 2012 Aug 21;3(4). pii: e00251-12. doi: 10.1128/mBio.00251-12. Print 2012.


SLC6A14 Is a Genetic Modifier of Cystic Fibrosis That Regulates Pseudomonas aeruginosa Attachment to Human Bronchial Epithelial Cells.

Di Paola M, Park AJ, Ahmadi S, Roach EJ, Wu YS, Struder-Kypke M, Lam JS, Bear CE, Khursigara CM.

MBio. 2017 Dec 19;8(6). pii: e02073-17. doi: 10.1128/mBio.02073-17.


Reliability of quantitative real-time PCR for bacterial detection in cystic fibrosis airway specimens.

Zemanick ET, Wagner BD, Sagel SD, Stevens MJ, Accurso FJ, Harris JK.

PLoS One. 2010 Nov 30;5(11):e15101. doi: 10.1371/journal.pone.0015101.


Genetic modifiers of nutritional status in cystic fibrosis.

Bradley GM, Blackman SM, Watson CP, Doshi VK, Cutting GR.

Am J Clin Nutr. 2012 Dec;96(6):1299-308. doi: 10.3945/ajcn.112.043406. Epub 2012 Nov 7.


Infections with Pseudomonas aeruginosa in patients with cystic fibrosis.

Tümmler B, Bosshammer J, Breitenstein S, Brockhausen I, Gudowius P, Herrmann C, Herrmann S, Heuer T, Kubesch P, Mekus F, Römling U, Schmidt KD, Spangenberg C, Walter S.

Behring Inst Mitt. 1997 Feb;(98):249-55. Review.


Pediatric Cystic Fibrosis Sputum Can Be Chemically Dynamic, Anoxic, and Extremely Reduced Due to Hydrogen Sulfide Formation.

Cowley ES, Kopf SH, LaRiviere A, Ziebis W, Newman DK.

MBio. 2015 Jul 28;6(4):e00767. doi: 10.1128/mBio.00767-15.


Reactive-oxygen-species-mediated P. aeruginosa killing is functional in human cystic fibrosis macrophages.

Cifani N, Pompili B, Anile M, Patella M, Diso D, Venuta F, Cimino G, Quattrucci S, Di Domenico EG, Ascenzioni F, Del Porto P.

PLoS One. 2013 Aug 19;8(8):e71717. doi: 10.1371/journal.pone.0071717. eCollection 2013.


Assessment of airway microbiota and inflammation in cystic fibrosis using multiple sampling methods.

Zemanick ET, Wagner BD, Robertson CE, Stevens MJ, Szefler SJ, Accurso FJ, Sagel SD, Harris JK.

Ann Am Thorac Soc. 2015 Feb;12(2):221-9. doi: 10.1513/AnnalsATS.201407-310OC.


Biogeochemical forces shape the composition and physiology of polymicrobial communities in the cystic fibrosis lung.

Quinn RA, Lim YW, Maughan H, Conrad D, Rohwer F, Whiteson KL.

MBio. 2014 Mar 18;5(2):e00956-13. doi: 10.1128/mBio.00956-13.


Response of CFTR-deficient mice to long-term chronic Pseudomonas aeruginosa infection and PTX3 therapy.

Paroni M, Moalli F, Nebuloni M, Pasqualini F, Bonfield T, Nonis A, Mantovani A, Garlanda C, Bragonzi A.

J Infect Dis. 2013 Jul;208(1):130-8. doi: 10.1093/infdis/jis636. Epub 2012 Oct 18.

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