Opportunities for the use of aerosolized α1-antitrypsin for the treatment of cystic fibrosis

Chest

Volumn 110, Issue 6 SUPPL., 1996, Pages 256S-260S

  Allen, Elizabeth D ^a,b  

^a OHIO STATE UNIVERSITY   (United States)

^b NATIONWIDE CHILDREN S HOSPITAL   (United States)

Author keywords

aerosolization; cystic fibrosis; neutrophil elastase; 1 antitrypsin

Indexed keywords

ALPHA 1 ANTITRYPSIN; LEUKOCYTE ELASTASE;

CYSTIC FIBROSIS; DRUG USE; GRAM NEGATIVE INFECTION; HUMAN; INHALATIONAL DRUG ADMINISTRATION; LYMPHOCYTIC INFILTRATION; PRIORITY JOURNAL; PSEUDOMONAS AERUGINOSA; REVIEW; STAPHYLOCOCCUS AUREUS; STAPHYLOCOCCUS INFECTION;

EID: 0030463020     PISSN: 00123692     EISSN: None     Source Type: Journal    
DOI: 10.1378/chest.110.6_Supplement.256S     Document Type: Article

References (61)

1. Tizzano EF, Buchwald M. Cystic fibrosis: beyond the gene to therapy. Pediatrics 1992; 120:337-49
2. Tsui L-C. The cystic fibrosis transmembrane conductance regulator gene. Am J Respir Crit Care Med 1995; 151:S47-53
3. FitzSimmons SC. The changing epidemiology of cystic fibrosis. J Pediatr 1993; 122:1-9
4. Zach MS. Lung disease in cystic fibrosis - an updated concept. Pediatr Pulmonol 1990; 8:188-202
5. Elborn JS, Shale DJ. Lung injury in cystic fibrosis. Thorax 1990; 45:970-73
6. Davis PB. Cystic fibrosis from bench to bedside. N Engl J Med 1991; 325:575-77
7. Orenstein DM. Cystic fibrosis. Curr Probl Pediatr 1993; 1:4-15
8. Dinwiddie R, Crawford O. Recent advances in cystic fibrosis research. J R Soc Med 1993; 86(suppl 20):7-10
9. Ramsey BW, Astley SJ, Aitken ML, et al. Efficacy and safety of short-term administration of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis. Am Rev Respir Dis 1993; 148:145-51
10. Knowles MR, Church NL, Waltner WE, et al. A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. N Engl J Med 1990; 322:1189-94
11. App E, King M, Helfesrieder R, et al. Acute and long-term amiloride inhalation in cystic fibrosis lung disease. Am Rev Respir Dis 1990; 141:605-12
12. Knowles MR, Clarke LL, Boucher RC. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N Engl J Med 1991; 325:533-38
13. Knowles MR, Olivier K, Noone P, et al. Pharmacologic modulation of salt and water in the airway epithelium in cystic fibrosis. Am J Respir Crit Care Med 1995; 151(3; pt 2):S65-9
14. Rosenfeld MA, Collins FS. Gene therapy for cystic fibrosis. Chest 1996; 109:241-52
15. Kerem E, Corey M, Gold R, et al. Pulmonary function and clinical course in patients with cystic fibrosis after pulmonary colonization with Pseudomonas aeruginosa. J Pediatr 1990; 116:714-19
16. Hoiby N, Koch C. Pseudomonas aeruginosa infection in cystic fibrosis and its management. Thorax 1990; 45:881-84
17. Do̊ring G, Albus A, Hoiby N. Immunologic aspects of cystic fibrosis. Chest 1988; 94:109S-14S
18. Auerbach HS, Kirkpatrick JA, Williams M, et al. Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet 1985; 2:686-88
19. Rosenstein BJ, Eigen H. Risks of alternate-day prednisone in patients with cystic fibrosis. Pediatrics 1991; 87:245-46
20. Konstan M, Vargo K, Davis P. Ibuprofen attenuates the inflammatory response to Pseudomonas aeruginosa in a rat model of chronic pulmonary infection: implications for antiinflammatory therapy in cystic fibrosis. Am Rev Respir Dis 1990; 141:186-92
21. Konstan MW, Byard PJ, Hoppel CL, et al. Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med 1995; 332:848-54
22. Birrer P, McElvaney NG, Rüdeberg A, et al. Protease-antiprotease imbalance in the lungs of children with cystic fibrosis. Am J Respir Crit Care Med 1994; 150:207-13
23. 1-antitrypsin. J Lab Clin Med 1972; 80:88-101
24. Goldstein W, Döring G. Lysosomal enzymes from polymorphonuclear leukocytes and proteinase inhibitors in patients with cystic fibrosis. Am Rev Respir Dis 1986; 134:49-56
25. 1-Proteinase inhibitor, elastase activity, and lung disease severity in cystic fibrosis. Am Rev Respir Dis 1993; 148:1665-70
26. Suter S, Schaad UB, Tegner H, et al. Levels of free granulocyte elastase in bronchial secretions from patients with cystic fibrosis: effect of antimicrobial treatment against Pseudomonas aeruginosa. J Infect Dis 1986; 153:902-09
27. Konstan MW, Hilliard KA, Norvell TM, et al. 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; 150:448-54
28. McElvaney NG, Doujaiji B, Moan MJ, et al. Pharmacokinetics of recombinant secretory leukoprotease inhibitor aerosolized to normals and individuals with cystic fibrosis. Am Rev Respir Dis 1993; 148:1056-60
29. Meyer KC, Zimmerman J. Neutrophil mediators, Pseudomonas, and pulmonary dysfunction in cystic fibrosis. J Lab Clin Med 1993; 121:654-61
30. Nakamura H, Yoshimura K, McElvaney NG, et al. Neutrophil elastase in respiratory epithelial lining fluid of individuals with cystic fibrosis induces interleukin-8 gene expression in a human bronchial epithelial cell line. J Clin Invest 1992; 89:1478-84
31. 1-antiprotease complex in cystic fibrosis: comparison with interstitial lung disease and evaluation of the effect of intravenously administered antibiotic therapy. Am Rev Respir Dis 1991; 144:580-85
32. Brown MA, Morgan WJ, Finley PR, et al. Circulating levels of tumor necrosis factor and interleukin-1 in cystic fibrosis. Pediatr Pulmonol 1991; 10:86-91
33. Cantin AM, Lafrenaye S, Bégin RO. Antineutrophil elastase activity in cystic fibrosis serum. Pediatr Pulmonol 1991; 11:249-53
34. Elborn JS, Cordon SM, Western PH, et al. Tumour necrosis factor-alpha-1 resting energy expenditure and cachexia in cystic fibrosis. Clin Sci 1993; 85:563-68
35. 1-proteinase inhibitor in infected bronchial secretions from patients with cystic fibrosis. Eur Respir J 1991; 4:40-9
36. Khan TZ, Wagener JS, Bost T, et al. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med 1995; 151:1075-82
37. 1 antiproteinase complex in cystic fibrosis: sensitive plasma assay for monitoring pulmonary infections. J Pediatr 1987; 111:206-11
38. Norman D, Elborn JS, Cordon SM, et al. Plasma tumour necrosis factor alpha in cystic fibrosis. Thorax 1991; 46:91-5
39. Rayner RJ, Wiseman MS, Cordon SM, et al. Inflammatory markers in cystic fibrosis. Respir Med 1991; 85:139-45
40. Elborn JS, Cordon SM, Parker D, et al. The host inflammatory response prior to death in patients with cystic fibrosis and chronic Pseudomonas aeruginosa infection. Respir Med 1993; 87:603-07
41. Elborn JS, Cordon SM, Shale DJ. Host inflammatory responses to first isolation of Pseudomonas aeruginosa from sputum in cystic fibrosis. Pediatr Pulmonol 1993; 15:287-91
42. Döring G, Drogh-Johansen H, Weidinger S, et al. Allotypes of alpha-1-antitrypsin in patients with cystic fibrosis, homozygous and heterozygous for deltaF508. Pediatr Pulmonol 1994; 18:3-7
43. Pier GB. Pulmonary disease associated with Pseudomonas aeruginosa in cystic fibrosis: current status of the host-bacterium interaction. J Infect Dis 1985; 151:575-80
44. Matthews WJ, Williams M, Oliphint B, et al. Hypogammaglobulinemia in patients with cystic fibrosis. N Engl J Med 1980; 302:245-49
45. Bruce MC, Poncz L, Klinger JD, et al. Biochemical and pathologic evidence for proteolytic destruction of lung connective tissue in cystic fibrosis. Am Rev Respir Dis 1985; 132:529-35
46. Nadel JA. Protease actions on airway secretions: relevance to cystic fibrosis. Ann NY Acad Sci 1991; 624:286-96
47. Sommerhoff CP, Nadel JA, Basbaum CB, et al. Neutrophil elastase and cathepsin G stimulate secretion from cultured bovine airway gland serous cells. J Clin Invest 1990; 85:682-89
48. Amitani R, Wilson R, Rutman A, et al. Effects of human neutrophil elastase and Pseudomonas aeruginosa proteinases on human respiratory epithelium. Am J Respir Cell Mol Biol 1991; 4:26-32
49. Ruef C, Jefferson DM, Schlegel-Haueter SE, et al. Regulation of cytokine secretion by cystic fibrosis airway epithelial cells. Eur Respir J 1993; 6:1429-36
50. Ulich TR, Yin S, Guo K, et al. Intratracheal injection of endotoxin and cytokines: II. Interleukin-6 and transforming growth factor-β inhibit acute inflammation. Am J Pathol 1991; 138:1097-1101
51. 4 by alveolar macrophages. J Clin Invest 1991; 88:891-97
52. 4 markedly elevated in the epithelial lining fluid of patients with cystic fibrosis. Am Rev Respir Dis 1993; 148:896-901
53. Woods DE, Straus DC, Johanson WG, et al. Role of fibronectin in the prevention of adherence of Pseudomonas aeruginosa to buccal cells. J Infect Dis 1981; 143:784-90
54. Plotkowski MC, Veck G, Toumier JM, et al. Adherence of Pseudomonas aeruginosa to respiratory epithelium and the effect of leucocyte elastase. J Med Microbiol 1989; 30:285-93
55. Fick RB, Naegel GP, Squier S, et al. Proteins of the cystic fibrosis respiratory tract: fragmented immunoglobulin G opsonic antibody causing defective opsonophagocytosis. J Clin Invest 1984; 74:236-48
56. Berger M, Sorensen RJ, Tosi MF, et al. Complement receptor expression on neutrophils at an inflammatory site, the pseudomona infected lung in cystic fibrosis. J Clin Invest 1989; 84:1302-13
57. McElvaney NG, Nakamura H, Birrer P, et al. Modulation of airway inflammation in cystic fibrosis: in vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor. J Clin Invest 1992; 90:1296-1301
58. Stolk J, Camps J, Feitsma HIJ, et al. Pulmonary deposition and disappearance of aerosolized secretory leukocyte protease inhibitor. Thorax 1995; 50:645-50
59. Hansen G, Schuster A, Zubrod C, et al. α-Proteinase inhibitor abrogates proteolytic and secretagogue activity of cystic fibrosis sputum. Respiration 1995; 62:117-24
60. McElvaney N, Hubbard R, Fells G, et al. Intravenous α1-antitrypsin therapy to reestablish anti-neutrophil elastase defenses of the pulmonary epithelial surface in cystic fibrosis. Am Rev Respir Dis 1990; 140:A83
61. McElvaney NG, Birrer P, Caplan DB, et al. Aerosol α1-antitrypsin treatment for cystic fibrosis. Lancet 1991; 337:392-94