Cystic fibrosis: NHLBI workshop on the primary prevention of chronic lung diseases

Annals of the American Thoracic Society

Volumn 11, Issue SUPPL. 3, 2014, Pages

  Pittman, Jessica E ^a   Cutting, Garry ^b   Davis, Stephanie D ^c   Ferkol, Thomas ^a   Boucher, Richard ^d  

^a WASHINGTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c INDIANA UNIVERSITY SCHOOL OF MEDICINE   (United States)

^d UNIVERSITY OF NORTH CAROLINA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BIOLOGICAL MARKER;

CHILDHOOD DISEASE; CHRONIC LUNG DISEASE; CONFERENCE PAPER; CYSTIC FIBROSIS; GENETIC SCREENING; HUMAN; KNOWLEDGE BASE; LUNG DEVELOPMENT; MEDICAL RESEARCH; MOLECULAR INTERACTION; OUTCOME ASSESSMENT; PRIMARY PREVENTION; PROTEIN INTERACTION; WORKSHOP; CHRONIC DISEASE; LUNG DISEASES; NATIONAL HEALTH ORGANIZATION; ORGANIZATION; PROCEDURES; UNITED STATES;

CHRONIC DISEASE; CONGRESSES AS TOPIC; CYSTIC FIBROSIS; HUMANS; LUNG DISEASES; NATIONAL HEART, LUNG, AND BLOOD INSTITUTE (U.S.); PRIMARY PREVENTION; UNITED STATES;

EID: 84899046260     PISSN: 23256621     EISSN: None     Source Type: Journal    
DOI: 10.1513/AnnalsATS.201312-444LD     Document Type: Conference Paper

References (50)

1. Davies JC, Alton EW, Bush A. Cystic fibrosis. BMJ 2007;335:1255-1259.
2. Davis PB. Pulmonary disease in cystic fibrosis. In: Chernick V, Boat TF, Wilmott RW, Bush A, editor. Kendig's disorders of the respiratory tract in children. Philadelphia: Saunders Elsevier; 2006. pp 873-886.
3. Button B, Cai LH, Ehre C, Kesimer M, Hill DB, Sheehan JK, Boucher RC, Rubinstein M. A periciliary brush promotes the lung health by separating the mucus layer from airway epithelia. Science 2012;337:937-941.
4. Drumm ML, Ziady AG, Davis PB. Genetic variation and clinical heterogeneity in cystic fibrosis. Annu Rev Pathol 2012;7:267-282.
5. Wolfenden LL, Schechter MS. Genetic and non-genetic determinants of outcomes in cystic fibrosis. Paediatr Respir Rev 2009;10:32-36.
6. Vanscoy LL, Blackman SM, Collaco JM, Bowers A, Lai T, Naughton K, Algire M, McWilliams R, Beck S, Hoover-Fong J, et al. Heritability of lung disease severity in cystic fibrosis. Am J Respir Crit Care Med 2007;175:1036-1043. (Pubitemid 46741755)
7. 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;157:802-807.e1-3.
8. Cutting GR. Modifier genes in Mendelian disorders: the example of cystic fibrosis. Ann N Y Acad Sci 2010;1214:57-69.
9. Belessis Y, Dixon B, Hawkins G, Pereira J, Peat J, MacDonald R, Field P, Numa A, Morton J, Lui K, 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.
10. Armstrong DS, Hook SM, Jamsen KM, Nixon GM, Carzino R, Carlin JB, Robertson CF, Grimwood K. Lower airway inflammation in infants with cystic fibrosis detected by newborn screening. Pediatr Pulmonol 2005;40:500-510. (Pubitemid 41720706)
11. Stephenson AL, Mannik LA, Walsh S, Brotherwood M, Robert R, Darling PB, Nisenbaum R, Moerman J, Stanojevic S. Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study. Am J Clin Nutr 2013;97:872-877.
12. Callaghan M, McClean S. Bacterial host interactions in cystic fibrosis. Curr Opin Microbiol 2012;15:71-77.
13. Rosenfeld M, Emerson J, McNamara S, Joubran K, Retsch-Bogart G, Graff GR, Gutierrez HH, Kanga JF, Lahiri T, Noyes B, et al.; EPIC Study Group Participating Clinical Sites. Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohort. Pediatr Pulmonol 2010;45:934-944.
14. Konstan MW, Morgan WJ, Butler SM, Pasta DJ, Craib ML, Silva SJ, Stokes DC, Wohl ME, Wagener JS, Regelmann WE, Johnson CA. Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis. J Pediatr 2007;151:134-139, 139.e1.
15. Rabin HR, Surette MG. The cystic fibrosis airway microbiome. Curr Opin Pulm Med 2012;18:622-627.
16. Ranganathan SC, Dezateux C, Bush A, Carr SB, Castle RA, Madge S, Price J, Stroobant J, Wade A, Wallis C, et al.; London Collaborative Cystic Fibrosis Group. Airway function in infants newly diagnosed with cystic fibrosis. Lancet 2001;358:1964-1965. (Pubitemid 34072881)
17. Davis SD, Fordham LA, Brody AS, Noah TL, Retsch-Bogart GZ, Qaqish BF, Yankaskas BC, Johnson RC, Leigh MW. Computed tomography reflects lower airway inflammation and tracks changes in early cystic fibrosis. Am J Respir Crit Care Med 2007;175:943-950. (Pubitemid 46684705)
18. Hall GL, Logie KM, Parsons F, Schulzke SM, Nolan G, Murray C, Ranganathan S, Robinson P, Sly PD, Stick SM, et al.; AREST CF. Air trapping on chest CT is associated with worse ventilation distribution in infants with cystic fibrosis diagnosed following newborn screening. PLoS ONE 2011;6:e23932.
19. Ranganathan S, Linnane B, Nolan G, Gangell C, Hall G. Early detection of lung disease in children with cystic fibrosis using lung function. Paediatr Respir Rev 2008;9:160-167.
20. Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR, Durie PR, Legrys VA, Massie J, Parad RB, et al.; Cystic Fibrosis Foundation. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr 2008;153:S4-S14. (Pubitemid 351970581)
21. Goetzinger KR, Cahill AG. An update on cystic fibrosis screening. Clin Lab Med 2010;30:533-543.
22. Hale JE, Parad RB, Comeau AM. Newborn screening showing decreasing incidence of cystic fibrosis. N Engl J Med 2008;358:973-974. (Pubitemid 351397113)
23. Castellani C, Picci L, Tamanini A, Girardi P, Rizzotti P, Assael BM. Association between carrier screening and incidence of cystic fibrosis. JAMA 2009;302:2573-2579.
24. Matsui H, Grubb BR, Tarran R, Randell SH, Gatzy JT, Davis CW, Boucher RC. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 1998;95:1005-1015. (Pubitemid 29019052)
25. Knowles MR, Boucher RC. Mucus clearance as a primary innate defense mechanism for mammalian airways. J Clin Invest 2002;109:571-577. (Pubitemid 34204834)
26. Lipuma JJ. The changing microbial epidemiology in cystic fibrosis. Clin Microbiol Rev 2010;23:299-323.
27. Dasenbrook EC, Checkley W, Merlo CA, Konstan MW, Lechtzin N, Boyle MP. Association between respiratory tract methicillin-resistant Staphylococcus aureus and survival in cystic fibrosis. JAMA 2010;303:2386-2392.
28. Pittman JE, Johnson RC, Davis SD. Improvement in pulmonary function following antibiotics in infants with cystic fibrosis. Pediatr Pulmonol 2012;47:441-446.
29. Willner D, Haynes MR, Furlan M, Schmieder R, Lim YW, Rainey PB, Rohwer F, Conrad D. Spatial distribution of microbial communities in the cystic fibrosis lung. ISME J 2012;6:471-474.
30. Farberman MM, Ibricevic A, Joseph TD, Akers KT, Garcia-Medina R, Crosby S, Clarke LL, Brody SL, Ferkol TW. Effect of polarized release of CXC-chemokines from wild-type and cystic fibrosis murine airway epithelial cells. Am J Respir Cell Mol Biol 2011;45:221-228.
31. van Ewijk BE, van der Zalm MM, Wolfs TF, Fleer A, Kimpen JL, Wilbrink B, van der Ent CK. Prevalence and impact of respiratory viral infections in young children with cystic fibrosis: prospective cohort study. Pediatrics 2008;122:1171-1176.
32. 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;7:320-328.
33. Olesen HV, Nielsen LP, Schiotz PO. Viral and atypical bacterial infections in the outpatient pediatric cystic fibrosis clinic. Pediatr Pulmonol 2006;41:1197-1204. (Pubitemid 44893874)
34. 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;487:109-113.
35. Moskwa P, Lorentzen D, Excoffon KJ, Zabner J, McCray PB Jr, Nauseef WM, Dupuy C, Bánfi B. A novel host defense system of airways is defective in cystic fibrosis. Am J Respir Crit Care Med 2007;175:174-183. (Pubitemid 46115089)
36. Khan TZ, Wagener JS, Bost T, Martinez J, Accurso FJ, Riches DW. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med 1995;151:1075-1082.
37. Konstan MW, Hilliard KA, Norvell TM, Berger M. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am J Respir Crit Care Med 1994;150:448-454. (Pubitemid 24252357)
38. Sly PD, Brennan S, Gangell C, de Klerk N, Murray C, Mott L, Stick SM, Robinson PJ, Robertson CF, Ranganathan SC; Australian Respiratory Early Surveillance Team for Cystic Fibrosis (ARESTCF). Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening. Am J Respir Crit Care Med 2009;180:146-152.
39. Sly PD, Gangell CL, Chen L, Ware RS, Ranganathan S, Mott LS, Murray CP, Stick SM, Investigators AC; AREST CF Investigators. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med 2013;368:1963-1970.
40. Venkatakrishnan A, Stecenko AA, King G, Blackwell TR, Brigham KL, Christman JW, Blackwell TS. Exaggerated activation of nuclear factor-kappaB and altered IkappaB-beta processing in cystic fibrosis bronchial epithelial cells. Am J Respir Cell Mol Biol 2000;23:396-403.
41. Brodlie M, McKean MC, Johnson GE, Gray J, Fisher AJ, Corris PA, Lordan JL, Ward C. Ceramide is increased in the lower airway epithelium of people with advanced cystic fibrosis lung disease. Am J Respir Crit Care Med 2010;182:369-375.
42. DiMango E, Ratner AJ, Bryan R, Tabibi S, Prince A. Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells. J Clin Invest 1998;101:2598-2605.
43. Petit-Bertron AF, Tabary O, Corvol H, Jacquot J, Clément A, Cavaillon JM, Adib-Conquy M. Circulating and airway neutrophils in cystic fibrosis display different TLR expression and responsiveness to interleukin-10. Cytokine 2008;41:54-60.
44. Tan HL, Regamey N, Brown S, Bush A, Lloyd CM, Davies JC. The Th17 pathway in cystic fibrosis lung disease. Am J Respir Crit Care Med 2011;184:252-258.
45. Livraghi-Butrico A, Kelly EJ, Klem ER, Dang H, Wolfgang MC, Boucher RC, Randell SH, O'Neal WK. Mucus clearance, MyD88-dependent and MyD88-independent immunity modulate lung susceptibility to spontaneous bacterial infection and inflammation. Mucosal Immunol 2012;5:397-408.
46. Davis SD, Brody AS, Emond MJ, Brumback LC, Rosenfeld M. Endpoints for clinical trials in young children with cystic fibrosis. Proc Am Thorac Soc 2007;4:418-430. (Pubitemid 47333916)
47. Puderbach M, Eichinger M, Haeselbarth J, Ley S, Kopp-Schneider A, Tuengerthal S, Schmaehl A, Fink C, Plathow C, Wiebel M, et al. Assessment of morphological MRI for pulmonary changes in cystic fibrosis (CF) patients: comparison to thin-section CT and chest xray. Invest Radiol 2007;42:715-725. (Pubitemid 351325904)
48. Rosenfeld M, Ratjen F, Brumback L, Daniel S, Rowbotham R, McNamara S, Johnson R, Kronmal R, Davis SD, Group IS; ISIS Study Group. Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial. JAMA 2012;307:2269-2277.
49. Robinson PD, Latzin P, Verbanck S, Hall GL, Horsley A, Gappa M, Thamrin C, Arets HG, Aurora P, Fuchs SI, et al. Consensus statement for inert gas washout measurement using multiple- and single- breath tests. Eur Respir J 2013;41:507-522.
50. Quittner AL, Buu A, Messer MA, Modi AC, Watrous M. Development and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosis. Chest 2005;128:2347-2354. (Pubitemid 41507578)