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December 10, 1997, Vol 278, No. 22 >
ARTICLE | December 10, 1997

Immunologic Aspects of Lung Diseases and Cystic Fibrosis FREE

Paul A. Greenberger, MD
JAMA. 1997;278(22):1924-1930. doi:10.1001/jama.1997.03550220130017
Text Size: A A A
Published online
Article
References

Immunologic lung disorders are accompanied by an array of laboratory abnormalities, some of which contribute to disease pathogenesis. Allergic bronchopulmonary aspergillosis, which complicates asthma and cystic fibrosis, causes mucous plugging of airways, eosinophilic pneumonia, and bronchiolitis obliterans. Aspergillus fumigatus, growing saprophytically in bronchial mucus, is responsible for most cases, and prednisone, not antifungal agents, is the drug of choice because it controls the immunologic responses of the lung. In cystic fibrosis, epithelial surface fluid from the lung does not kill Pseudomonas aeruginosa, in part because antibodies to P aeruginosa are plentiful but ineffective in opsonizing bacteria. Neutrophil-derived elastase cleaves immunoglobulins and digests the C3b receptor on neutrophils, which limits phagocytosis of pathogens. In helminth infections and infestations, pulmonary and peripheral blood eosinophilia can be accompanied by increases in total and antiparasite IgE concentrations and generate TH2 CD4+ T-lymphocyte responses. Understanding the immunologic abnormalities of lung disorders may lead to more effective therapies.

REFERENCES

1 +
Greenberger PA, Miller TP, Roberts M, Smith LL.  Allergic bronchopulmonary aspergillosis in patients with and without evidence of bronchiectasis . Ann Allergy . 1993;;70:333-338.
2 +
Patterson R, Rosenberg M, Roberts M.  Evidence that Aspergillus fumigatus growing in the airway of man can be a potent stimulus of specific and nonspecific IgE formation . Am J Med . 1977;;63:257-262.
3 +
Greenberger PA, Smith LJ, Hsu CCS, Roberts M, Liotta JL.  Analysis of bronchoalveolar lavage in allergic bronchopulmonary aspergillosis . J Allergy Clin Immunol . 1988;;82:164-170.
4 +
Brown JE, Greenberger PA, Yarnold PR.  Soluble interleukin 2 receptors in patients with asthma and allergic bronchopulmonary aspergillosis . Ann Allergy Asthma Immunol . 1995;;74:484-488.
5 +
Chauhan B, Knutsen AP, Hutcheson PS, Slavin RG, Bellone CJ.  T cell subsets, epitope mapping, and HLA-restriction in patients with allergic bronchopulmonary aspergillosis . J Clin Invest . 1996;;97:2324-2331.
6 +
Greenberger PA.  Allergic bronchopulmonary aspergillosis . In: Patterson R, Grammer LC, Greenberger PA, eds. Allergic Diseases . 5th ed. Philadelphia, Pa: Lippincott-Raven; 1997;:555-577.
7 +
Denning DW, Van Wye JE, Lewiston NJ, Stevens DA.  Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole . Chest . 1991;;100:813-819.
8 +
Allen JN, Davis WB.  Eosinophilic lung diseases . Am J Respir Crit Care Med . 1994;;150:1423-1438.
9 +
Backman KS, Zull D, Patterson R.  Diagnostic complexity in a patient with asthma, pulmonary infiltrates, and eosinophilia . Ann Allergy Asthma Immunol . 1995;;75:391-400.
10 +
Matteson EL, Gold KN, Bloch DA, Hunder GG.  Long-term survival of patients with Wegener's granulomatosis from the American College of Rheumatology Wegener's granulomatosis classification criteria cohort . Am J Med . 1996;;101:129-134.
11 +
Bajema IM, Hagen EC, Van Der Woude FJ, Bruijn JA.  Wegener's granulomatosis . J Lab Clin Med . 1997;;129:17-22.
12 +
Boehme MWJ, Schmitt WH, Youinou P, Stremmel WR, Gross WL.  Clinical relevance of elevated serum thrombomodulin and soluble E-selectin in patients with Wegener's granulomatosis and other systemic vasculitides . Am J Med . 1996;;101:387-394.
13 +
Phills, JA, Harrold AJ, Whiteman GV, Perelmutter L.  Pulmonary infiltrates, asthma, and eosinophilia due to Ascaris suum infestation in man . N Engl J Med . 1972;;286:965-970.
14 +
van Haelst Pisani C, Kovach JS, Kita H, et al.  Administration of interleukin-2 (IL-2) results in increased plasma concentrations of IL-5 and eosinophilia in patients with cancer . Blood . 1991;;9:1538-1544.
15 +
Lindermann A, Ganser A, Herrmann F, et al.  Biologic effects of recombinant human interleukin-3 in vivo . J Clin Oncol . 1991;;9:2120-2127.
16 +
Gonzales-Chambers R, Rosenfeld C, Winkelstein A, Dameshek L.  Eosinophilia resulting from administration of recombinant granulocyte-macrophage colony-stimulating factor (rh GM-CSF) in a patient with Tγlymphoproliferative disease . Am J Hematol . 1991;;36:157-159.
17 +
Goldiner PL, Carlon GC, Cvitkovic E, Schweizer 0, Howland WS.  Factors influencing postoperative morbidity and mortality in patients treated with bleomycin . BMJ . 1978;;1:1664-1667.
18 +
Holoye PY, Luna MA, MacKay B, Bedrossian CWM.  Bleomycin hypersensitivity pneumonitis . Ann Intern Med . 1978;;88:47-49.
19 +
Cogan E, Schandene L, Crusiaux A, Cochaux P, Velu T, Goldman M.  Clonal proliferation of type 2 helper T cells in a man with the hypereosinophilic syndrome . N Engl J Med . 1994;;330:535-538.
20 +
Prin L, Plumas J, Gruart V, et al.  Elevated serum levels of soluble interleukin-2 receptor . Blood . 1991;;78:2626-2632.
21 +
Webb JKG, Job CK, Gault EW.  Tropical eosinophilia . Lancet . 1960;;1:835-842.
22 +
Fink JN.  Hypersensitivity pneumonitis . In: Patterson R, Grammer LC, Greenberger PA, eds. Allergic Diseases . 5th ed. Philadelphia, Pa: Lippincott Raven; 1997;:543-553.
23 +
Zissel G. Ernst M, Schlaak M, Muller-Quernheim J.  Accessory function of alveolar macrophages from patients with sarcoidosis and other granulomatous and nongranulomatous lung diseases . J Invest Med . 1997;;45:75-86.
24 +
Trentin L, Migone N, Zambello R, et al.  Mechanisms accounting for lymphocytic alveolitis in hypersensitivity pneumonitis . J Immunol . 1990;;145:2147-2154.
25 +
Denis M, Ghadirian E.  Transforming growth factor-β is generated in the course of hypersensitivity pneumonitis . Am J Respir Cell Mol Biol . 1992;;7:156-160.
26 +
Nakagawa-Yoshida K, Ando M, Etches, Dosman JA.  Fatal cases of farmer's lung in a Canadian family . Chest . 1997;;111:245-248.
27 +
Greenberger PA, Pien LC, Patterson R, Robinson P, Roberts M.  End-stage lung and ultimately fatal disease in a bird-fancier . Am J Med . 1989;;86:119-122.
28 +
Malmberg P, Rask-Andersen A, Rosenhall L.  Exposure to microorganisms associated with allergic alveolitis and febrile reactions to mold dust in farmers . Chest . 1993;;103:1202-1209.
29 +
Hogan MB, Patterson R, Pore S, Corder WT, Wilson NW.  Basement shower hypersensitivity pneumonitis secondary to Epicoccum nigrum . Chest . 1996;;110:855-856.
30 +
Ando M, Arima K, Yoneda R, Tamura M.  Japanese summer-type hypersensitivity pneumonitis: geographic distribution, home environment, and clinical characteristics of 621 cases . Am Rev Respir Dis . 1991;;144:765-769.
31 +
Grammer LC, Patterson R. Trimellitic anhydride. In: Rom WN, ed. Environmental and Occupational Medicine. 3rd ed. New York, NY: Little Brown & Co. In press.
32 +
Mapel DW, Samet JM, Coultas DB.  Corticosteroids and the treatment of idiopathic pulmonary fibrosis . Chest . 1996;;110:1058-1067.
33 +
Silicosis and Silicate Disease Committee.  Diseases associated with exposure to silica and nonfibrous silicate minerals . Arch Pathol Lab Med . 1988;;112:673-720.
34 +
Jagirdar J, Begin R, Dufresne A, Goswami S, Lee RC, Rom WN.  Transforming growth factor-β (TGF-β in silicosis . Am J Respir Crit Care Med . 1996;;154:1076-1081.
35 +
Balmes J, Cullen MR, Matthay RA.  Occupational and environmental lung disease . In: George RB, Light RW, Matthay MA, Matthay RA, eds. Chest Medicine . 2nd ed. Baltimore, Md: Williams & Wilkins; 1990;:277-306.
36 +
Wilson CB.  Goodpasture syndrome and other anti-basement membrane antibody diseases . In: Rich RR, Fleisher TA, Schwartz BD, Shearer WT, Strober W, eds. Clinical Immunology . St Louis, Mo: Mosby-Year Book; 1996;:1185-1191.
37 +
Kalluri R, Sun MJ, Hudson BG, Neilson EG.  The Goodpasture antigen . J Biol Chem . 1996;;271:9062-9068.
38 +
Semenzato G, Zambello R, Trentin L, Agostini C.  Cellular immunity in sarcoidosis and hypersensitivity pneumonitis . Chest . 1993;;103( (suppl) ):139S-143S.
39 +
Zissel G, HomolkaJ, Schlaak J, Schlaak M, Muller-Quernheim J.  Anti-inflammatory cytokine release by alveolar macrophages in pulmonary sarcoidosis . Am J Respir Crit Care Med . 1996;;154:713-719.
40 +
Hunninghake GW, Gilbert S, Pueringer R, et al.  Outcome of the treatment for sarcoidosis . Am J Respir Crit Care Med . 1994;;149:893-898.
41 +
Heatly T, Sekela M, Berger R.  Single lung transplantation involving a donor with documented pulmonary sarcoidosis . J Heart Lung Transplant . 1994;;13:720-723.
42 +
Weiner Miller P, Hamosh A, Macek JM Jr, et al.  Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in allergic bronchopulmonary aspergillosis . Am J Hum Genet . 1996;;59:45-51.
43 +
Davis PB, Drumm M, Konstan MW.  Cystic fibrosis . Am J Resp Crit Care Med . 1996;;154:1229-1256.
44 +
Steinbach S, Sun L, Jiang R-Z, et al.  Transmissibility of Pseudomonas cepacia infection in clinic patients and lung-transplant recipients with cystic fibrosis . N Engl J Med . 1994;;331:981-987.
45 +
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;;325:533-538.
46 +
Konstan MW, Walenga RW, Hilliard KA, Hilliard JB.  Leukotriene B4 markedly elevated in the epithelial lining fluid of patients with cystic fibrosis . Am Rev Respir Dis . 1993;;148:896-901.
47 +
Davis PB.  Cystic fibrosis from bench to bedside . N Engl J Med . 1991;;325:575-576.
48 +
Birrer P, McElvaney NG, Rudeberg A, et al.  Protease-antiprotease imbalance in the lungs of children with cystic fibrosis . Am J Respir Crit Care Med . 1994;;150:207-213.
49 +
Becker JW, Burke W, McDonald G, Greenberger PA, Henderson WR, Aitken ML.  Prevalence of allergic bronchopulmonary aspergillosis in adult patients with cystic fibrosis . Chest . 1996;;109:1536-1540.
50 +
Laufer P, Fink J, Bruns W, et al.  Allergic bronchopulmonary aspergillosis in cystic fibrosis . J Allergy Clin Immunol . 1984;;73:44-48.
51 +
Nikolaizik WH, Moser M, Crameri R, et al.  Identification of allergic bronchopulmonary aspergillosis in cystic fibrosis patients by recombinant Aspergillus fumigatus I/a-specific serology . Am J Resp Crit Care Med . 1995;;152:634-639.
52 +
Cystic Fibrosis Foundation Center Committee and Guidelines Subcommittee.  Cystic Fibrosis Foundation guidelines for patient services, evaluation, and monitoring in cystic fibrosis centers . AJDC . 1990;;144:1311-1312.
53 +
Auerbach HS, Williams M, Kirkpatrick JA, Colten HR.  Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis . Lancet . 1985;;2:686-688.
54 +
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.
55 +
Fuchs HJ, Borowitz DS, Christiansen DH, et al.  Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis . N Engl J Med . 1994;;331:637-642.
56 +
Knowles MR, Church NL, Waltner WE, et al.  A pilot study of aerosolized amiloride for the treatment of lung disease of cystic fibrosis . N Engl J Med . 1990;;322:1189-1194.
57 +
Regelmann WE, Elliott GR, Warwick WJ, Clawson CC.  Reduction of sputum Pseudomonas aeruginosa density by antibiotics improves lung function in cystic fibrosis more than do bronchodilators and chest physiotherapy alone . Am Rev Respir Dis . 1990;;141:914-921.
58 +
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.

Figures

Tables

Interactive Graphics

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

1 +
Greenberger PA, Miller TP, Roberts M, Smith LL.  Allergic bronchopulmonary aspergillosis in patients with and without evidence of bronchiectasis . Ann Allergy . 1993;;70:333-338.
2 +
Patterson R, Rosenberg M, Roberts M.  Evidence that Aspergillus fumigatus growing in the airway of man can be a potent stimulus of specific and nonspecific IgE formation . Am J Med . 1977;;63:257-262.
3 +
Greenberger PA, Smith LJ, Hsu CCS, Roberts M, Liotta JL.  Analysis of bronchoalveolar lavage in allergic bronchopulmonary aspergillosis . J Allergy Clin Immunol . 1988;;82:164-170.
4 +
Brown JE, Greenberger PA, Yarnold PR.  Soluble interleukin 2 receptors in patients with asthma and allergic bronchopulmonary aspergillosis . Ann Allergy Asthma Immunol . 1995;;74:484-488.
5 +
Chauhan B, Knutsen AP, Hutcheson PS, Slavin RG, Bellone CJ.  T cell subsets, epitope mapping, and HLA-restriction in patients with allergic bronchopulmonary aspergillosis . J Clin Invest . 1996;;97:2324-2331.
6 +
Greenberger PA.  Allergic bronchopulmonary aspergillosis . In: Patterson R, Grammer LC, Greenberger PA, eds. Allergic Diseases . 5th ed. Philadelphia, Pa: Lippincott-Raven; 1997;:555-577.
7 +
Denning DW, Van Wye JE, Lewiston NJ, Stevens DA.  Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole . Chest . 1991;;100:813-819.
8 +
Allen JN, Davis WB.  Eosinophilic lung diseases . Am J Respir Crit Care Med . 1994;;150:1423-1438.
9 +
Backman KS, Zull D, Patterson R.  Diagnostic complexity in a patient with asthma, pulmonary infiltrates, and eosinophilia . Ann Allergy Asthma Immunol . 1995;;75:391-400.
10 +
Matteson EL, Gold KN, Bloch DA, Hunder GG.  Long-term survival of patients with Wegener's granulomatosis from the American College of Rheumatology Wegener's granulomatosis classification criteria cohort . Am J Med . 1996;;101:129-134.
11 +
Bajema IM, Hagen EC, Van Der Woude FJ, Bruijn JA.  Wegener's granulomatosis . J Lab Clin Med . 1997;;129:17-22.
12 +
Boehme MWJ, Schmitt WH, Youinou P, Stremmel WR, Gross WL.  Clinical relevance of elevated serum thrombomodulin and soluble E-selectin in patients with Wegener's granulomatosis and other systemic vasculitides . Am J Med . 1996;;101:387-394.
13 +
Phills, JA, Harrold AJ, Whiteman GV, Perelmutter L.  Pulmonary infiltrates, asthma, and eosinophilia due to Ascaris suum infestation in man . N Engl J Med . 1972;;286:965-970.
14 +
van Haelst Pisani C, Kovach JS, Kita H, et al.  Administration of interleukin-2 (IL-2) results in increased plasma concentrations of IL-5 and eosinophilia in patients with cancer . Blood . 1991;;9:1538-1544.
15 +
Lindermann A, Ganser A, Herrmann F, et al.  Biologic effects of recombinant human interleukin-3 in vivo . J Clin Oncol . 1991;;9:2120-2127.
16 +
Gonzales-Chambers R, Rosenfeld C, Winkelstein A, Dameshek L.  Eosinophilia resulting from administration of recombinant granulocyte-macrophage colony-stimulating factor (rh GM-CSF) in a patient with Tγlymphoproliferative disease . Am J Hematol . 1991;;36:157-159.
17 +
Goldiner PL, Carlon GC, Cvitkovic E, Schweizer 0, Howland WS.  Factors influencing postoperative morbidity and mortality in patients treated with bleomycin . BMJ . 1978;;1:1664-1667.
18 +
Holoye PY, Luna MA, MacKay B, Bedrossian CWM.  Bleomycin hypersensitivity pneumonitis . Ann Intern Med . 1978;;88:47-49.
19 +
Cogan E, Schandene L, Crusiaux A, Cochaux P, Velu T, Goldman M.  Clonal proliferation of type 2 helper T cells in a man with the hypereosinophilic syndrome . N Engl J Med . 1994;;330:535-538.
20 +
Prin L, Plumas J, Gruart V, et al.  Elevated serum levels of soluble interleukin-2 receptor . Blood . 1991;;78:2626-2632.
21 +
Webb JKG, Job CK, Gault EW.  Tropical eosinophilia . Lancet . 1960;;1:835-842.
22 +
Fink JN.  Hypersensitivity pneumonitis . In: Patterson R, Grammer LC, Greenberger PA, eds. Allergic Diseases . 5th ed. Philadelphia, Pa: Lippincott Raven; 1997;:543-553.
23 +
Zissel G. Ernst M, Schlaak M, Muller-Quernheim J.  Accessory function of alveolar macrophages from patients with sarcoidosis and other granulomatous and nongranulomatous lung diseases . J Invest Med . 1997;;45:75-86.
24 +
Trentin L, Migone N, Zambello R, et al.  Mechanisms accounting for lymphocytic alveolitis in hypersensitivity pneumonitis . J Immunol . 1990;;145:2147-2154.
25 +
Denis M, Ghadirian E.  Transforming growth factor-β is generated in the course of hypersensitivity pneumonitis . Am J Respir Cell Mol Biol . 1992;;7:156-160.
26 +
Nakagawa-Yoshida K, Ando M, Etches, Dosman JA.  Fatal cases of farmer's lung in a Canadian family . Chest . 1997;;111:245-248.
27 +
Greenberger PA, Pien LC, Patterson R, Robinson P, Roberts M.  End-stage lung and ultimately fatal disease in a bird-fancier . Am J Med . 1989;;86:119-122.
28 +
Malmberg P, Rask-Andersen A, Rosenhall L.  Exposure to microorganisms associated with allergic alveolitis and febrile reactions to mold dust in farmers . Chest . 1993;;103:1202-1209.
29 +
Hogan MB, Patterson R, Pore S, Corder WT, Wilson NW.  Basement shower hypersensitivity pneumonitis secondary to Epicoccum nigrum . Chest . 1996;;110:855-856.
30 +
Ando M, Arima K, Yoneda R, Tamura M.  Japanese summer-type hypersensitivity pneumonitis: geographic distribution, home environment, and clinical characteristics of 621 cases . Am Rev Respir Dis . 1991;;144:765-769.
31 +
Grammer LC, Patterson R. Trimellitic anhydride. In: Rom WN, ed. Environmental and Occupational Medicine. 3rd ed. New York, NY: Little Brown & Co. In press.
32 +
Mapel DW, Samet JM, Coultas DB.  Corticosteroids and the treatment of idiopathic pulmonary fibrosis . Chest . 1996;;110:1058-1067.
33 +
Silicosis and Silicate Disease Committee.  Diseases associated with exposure to silica and nonfibrous silicate minerals . Arch Pathol Lab Med . 1988;;112:673-720.
34 +
Jagirdar J, Begin R, Dufresne A, Goswami S, Lee RC, Rom WN.  Transforming growth factor-β (TGF-β in silicosis . Am J Respir Crit Care Med . 1996;;154:1076-1081.
35 +
Balmes J, Cullen MR, Matthay RA.  Occupational and environmental lung disease . In: George RB, Light RW, Matthay MA, Matthay RA, eds. Chest Medicine . 2nd ed. Baltimore, Md: Williams & Wilkins; 1990;:277-306.
36 +
Wilson CB.  Goodpasture syndrome and other anti-basement membrane antibody diseases . In: Rich RR, Fleisher TA, Schwartz BD, Shearer WT, Strober W, eds. Clinical Immunology . St Louis, Mo: Mosby-Year Book; 1996;:1185-1191.
37 +
Kalluri R, Sun MJ, Hudson BG, Neilson EG.  The Goodpasture antigen . J Biol Chem . 1996;;271:9062-9068.
38 +
Semenzato G, Zambello R, Trentin L, Agostini C.  Cellular immunity in sarcoidosis and hypersensitivity pneumonitis . Chest . 1993;;103( (suppl) ):139S-143S.
39 +
Zissel G, HomolkaJ, Schlaak J, Schlaak M, Muller-Quernheim J.  Anti-inflammatory cytokine release by alveolar macrophages in pulmonary sarcoidosis . Am J Respir Crit Care Med . 1996;;154:713-719.
40 +
Hunninghake GW, Gilbert S, Pueringer R, et al.  Outcome of the treatment for sarcoidosis . Am J Respir Crit Care Med . 1994;;149:893-898.
41 +
Heatly T, Sekela M, Berger R.  Single lung transplantation involving a donor with documented pulmonary sarcoidosis . J Heart Lung Transplant . 1994;;13:720-723.
42 +
Weiner Miller P, Hamosh A, Macek JM Jr, et al.  Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in allergic bronchopulmonary aspergillosis . Am J Hum Genet . 1996;;59:45-51.
43 +
Davis PB, Drumm M, Konstan MW.  Cystic fibrosis . Am J Resp Crit Care Med . 1996;;154:1229-1256.
44 +
Steinbach S, Sun L, Jiang R-Z, et al.  Transmissibility of Pseudomonas cepacia infection in clinic patients and lung-transplant recipients with cystic fibrosis . N Engl J Med . 1994;;331:981-987.
45 +
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;;325:533-538.
46 +
Konstan MW, Walenga RW, Hilliard KA, Hilliard JB.  Leukotriene B4 markedly elevated in the epithelial lining fluid of patients with cystic fibrosis . Am Rev Respir Dis . 1993;;148:896-901.
47 +
Davis PB.  Cystic fibrosis from bench to bedside . N Engl J Med . 1991;;325:575-576.
48 +
Birrer P, McElvaney NG, Rudeberg A, et al.  Protease-antiprotease imbalance in the lungs of children with cystic fibrosis . Am J Respir Crit Care Med . 1994;;150:207-213.
49 +
Becker JW, Burke W, McDonald G, Greenberger PA, Henderson WR, Aitken ML.  Prevalence of allergic bronchopulmonary aspergillosis in adult patients with cystic fibrosis . Chest . 1996;;109:1536-1540.
50 +
Laufer P, Fink J, Bruns W, et al.  Allergic bronchopulmonary aspergillosis in cystic fibrosis . J Allergy Clin Immunol . 1984;;73:44-48.
51 +
Nikolaizik WH, Moser M, Crameri R, et al.  Identification of allergic bronchopulmonary aspergillosis in cystic fibrosis patients by recombinant Aspergillus fumigatus I/a-specific serology . Am J Resp Crit Care Med . 1995;;152:634-639.
52 +
Cystic Fibrosis Foundation Center Committee and Guidelines Subcommittee.  Cystic Fibrosis Foundation guidelines for patient services, evaluation, and monitoring in cystic fibrosis centers . AJDC . 1990;;144:1311-1312.
53 +
Auerbach HS, Williams M, Kirkpatrick JA, Colten HR.  Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis . Lancet . 1985;;2:686-688.
54 +
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.
55 +
Fuchs HJ, Borowitz DS, Christiansen DH, et al.  Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis . N Engl J Med . 1994;;331:637-642.
56 +
Knowles MR, Church NL, Waltner WE, et al.  A pilot study of aerosolized amiloride for the treatment of lung disease of cystic fibrosis . N Engl J Med . 1990;;322:1189-1194.
57 +
Regelmann WE, Elliott GR, Warwick WJ, Clawson CC.  Reduction of sputum Pseudomonas aeruginosa density by antibiotics improves lung function in cystic fibrosis more than do bronchodilators and chest physiotherapy alone . Am Rev Respir Dis . 1990;;141:914-921.
58 +
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.

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