Current understanding of the inflammatory process in cystic fibrosis: Onset and Etiology

Pediatric Pulmonology

Volumn 24, Issue 2, 1997, Pages 137-142

  Konstan, Michael W ^a   Berger, Melvin ^a  

^a CASE WESTERN RESERVE UNIVERSITY   (United States)

Author keywords

Bronchoalveolar lavage; Cystic fibrosis; Inflammation; Lung disease; Pathogenesis

Indexed keywords

TRANSMEMBRANE CONDUCTANCE REGULATOR;

BRONCHOSCOPY; CONFERENCE PAPER; CYSTIC FIBROSIS; HUMAN; IMMUNOPATHOGENESIS; IMMUNOREGULATION; INFLAMMATION; LUNG LAVAGE; PATHOPHYSIOLOGY; PRIORITY JOURNAL;

BRONCHOALVEOLAR LAVAGE; BRONCHOSCOPY; CYSTIC FIBROSIS; CYTOKINES; HUMANS; INFLAMMATION; INTERCELLULAR ADHESION MOLECULE-1; NEUTROPHILS;

EID: 0030881846     PISSN: 87556863     EISSN: None     Source Type: Journal    
DOI: 10.1002/(SICI)1099-0496(199708)24:2<137::AID-PPUL13>3.0.CO;2-3     Document Type: Conference Paper

References (19)

1. Davis PB, Drumm M, Konstan MW. Cystic fibrosis: State of the art. Am J Respir Crit Care Med. 1996; 154:1229-1256.
2. Konstan MW, Berger M. Infection and inflammation in the lung in cystic fibrosis. In: Davis PB, ed. Cystic Fibrosis. New York: Marcel Dekker. 1993:219-276.
3. Tomashefski JF, Abramowsky CR, Dahms BB. The pathology of cystic fibrosis. In: Davis PB, ed. Cystic Fibrosis. New York: Marcel Dekker. 1993:435-489.
4. Smith AL, Redding G, Doershuk C, Goldmann D, Gore E, Hilman B, Marks M, Moss R, Ramsey B, Rubio T, Schwartz RH, Thomassen MJ, Williams-Warren J, Weber A, Wilmott RW, Wilson HD, Yogev R. Sputum changes associated with therapy for endobronchial exacerbation in cystic fibrosis. J Pediatr. 1988; 112: 547-554.
5. 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.
6. Armstrong DS, Grimwood K, Carzino R, Carlin JB, Olinsky A, Phelan PD. Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis. BMJ. 1995; 310:1571-1572.
7. Kirchner KK, Khan TZ, Wagener JS, Accurso FJ. Increased DNA levels in bronchoalveolar lavage fluid obtained from infants with cystic fibrosis. Am J Respir Crit Care Med. 1996; 154:1426-1429.
8. 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.
9. Birrer P, McElvaney NG, Rüdeberg A, Wirz Sommer C, Liechti-Gallati S, Kraemer R, Hubbard R, Crystal RG. Protease-antiprotease imbalance in the lungs of children with cystic fibrosis. Am J Respir Crit Care Med. 1994; 150:207-213.
10. Balough K, McCubbin M, Weinberger M, Smits W, Aherns R, Fick R. The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis. Pediatr Pulmonol. 1995; 20:63-70.
11. Cantin A. Cystic fibrosis lung inflammation: Early, sustained, and severe. Am J Respir Crit Care Med. 1995; 151:939-941.
12. Bonfield TL, Panuska JR, Konstan MW, Hilliard KA, Hilliard JB, Ghnaim H, Berger M. Inflammatory cytokines in cystic fibrosis lungs. Am J Respir Crit Care Med. 1995; 152:2111-2118.
13. Bonfield TL, Konstan MW, Burfeind P, Panuska JR, Hilliard JB, Berger M. Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine IL-10 which is down-regulated in cystic fibrosis. Am J Respir Cell Mol Biol. 1995; 13:257-261.
14. DiMango E, Zar HJ, Bryan R, Prince A. Diverse Pseudomonas aeruginosa gene products stimulate respiratory epithelial cells to produce interleukin-8. J Clin Invest. 1995; 96:2204-2210.
15. Mizgerd JP, Kobzik L, Warner AE, Brain JD. Effects of sodium concentration on human neutrophil bactericidal functions. Am J Physiol. 1995; 269:L388-L393.
16. Smith JJ, Travis SM, Greenberg EP, Welsh MJ. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell. 1996; 85:229-236.
17. Pier GB, Grout M, Zaidi TS, Olsen JC, Johnson LG, Yankaskas JR, Goldberg JB. Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections. Science. 1995; 271:64-67.
18. Konstan MW. Anti-inflammatory therapy in cystic fibrosis. In: New Insights into Cystic Fibrosis. New Jersey: Gardiner-Caldwell SynerMed, 1995; 3:7-11.
19. Konstan MW, Byard PJ, Hoppel CL, Davis PB. Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med. 1995; 332:848-854.