Pseudomonas Aeruginosa Infections in Individuals with Cystic Fibrosis

European PerspectiveS
  • Montserrat Bosque
  • Òscar Asensio
Part of the Perspectives on Critical Care Infectious Diseases book series (CCID, volume 7)


Pulmonary infection is the most common cause of morbidity and mortality in cystic fibrosis (CF). The lungs of the CF infant are close to normal at birth, but postnatally there is a rapid onset of infection and inflamation. The initial pathogens are often Staphylococcus aureus and Haemophilus influenzae, but bronchoscopic studies have confirmed that Pseudomonas aeruginosa infection may also be an early event. By adulthood about 80% of patients are chronically infected with P. aeruginosa.


Cystic Fibrosis Pseudomonas Aeruginosa Respiratory Syncytial Virus Cystic Fibrosis Transmembrane Conductance Regulator Paranasal Sinus 
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  1. 1.
    Frederiksen B, Cock CH, Hoiby N. Changing Epidemiology of Pseudomonas aeruginosa Infection in Danish Cystic Fibrosis Patients (1974–1995). Pediatr Pulmonol 1999; 28:159–166.PubMedCrossRefGoogle Scholar
  2. 2.
    FitzSimmons SC. The changing epidemiology of cystic fibrosis. J Pediatr 1996; 122:1–9.Google Scholar
  3. 3.
    Asensio 0, Cobos N, Seculi JL, Casals T, Marco MT, Gartner S, Puliol M, Prats R. Programa de cribaje neonatal para la FQ en Catalufia. Invest Clin 2001; 4:82–83.Google Scholar
  4. 4.
    Hoiby N. Epidemiological investigations of the respiratory tract in patients with cystic fibrosis. Acta Pathol Microbiolo Scand (B) 1974; 82:541.Google Scholar
  5. 5.
    Cystic Fibrosis Foundation USA. Patient Registry 1995. Annual Data Report. Bethesda, MD: Cystic Fibrosis Foundation USA, 1996.Google Scholar
  6. 6.
    Santis G. Basic molecular biology. In: Hodson ME, Geddes D, eds. Cystic Fibrosis. London, Chapman and Hall, 1995; pp. 15–39.Google Scholar
  7. 7.
    Anonymous. Cystic Fibrosis Foundation patient registry 1997 annual data report. Bethesda, MD, USA. Cystic Fibrosis Foundation.Google Scholar
  8. 8.
    Lewis PA. The epidemiology of cystic fibrosis. In: Hodson ME, Geddes D, eds. Cystic Fibrosis. London, Chapman and Hall, 1995; 1–13.Google Scholar
  9. 9.
    Johansen HK. Hoiby N. Seasonal onset of initial colonisation and chronic infection with Pseudomonas aeruginosa in patients with cystic fibrosis in Denmark. Thorax, 1992; 47:109–111.PubMedCrossRefGoogle Scholar
  10. 10.
    Hoiby N. Prevention and treatment of infection in cystic fibrosis. Int J Antimicrobiol Agents. 1992; 1:229–238.CrossRefGoogle Scholar
  11. 11.
    Armstrong DS, Grimwood K, Carlin JB, et al. Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med 1997; 156:1197–1204.PubMedGoogle Scholar
  12. 12.
    Kerem B, Rommens JM, Buchanan JA, Markiewicz D, Cox TK, Chakravarti A, buchwald M, Tsui LC. Identification of the cystic fibrosis gene: genetic analysis. Science 1989; 245:1073–1080.PubMedCrossRefGoogle Scholar
  13. 13.
    Kerem E, Kerem B. Genotype-phenotype correlations in cystic fibrosis. Pediatr Pulmonol 1996; 22:387–395.PubMedCrossRefGoogle Scholar
  14. 14.
    European Epidemiologic Registry of Cystic Fibrosis (ERCF): Comparison of Major Disease Manifestations Between Patients with Different Classes of Mutations. Koch C.Google Scholar
  15. 15.
    Rosenstein BJ, Cutting GR. The diagnosis of CF: A consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr 1998; 132:589.PubMedCrossRefGoogle Scholar
  16. 16.
    Stern RC. The diagnosis of cystic fibrosis. N Engl J Med. 1997; 336:487.PubMedCrossRefGoogle Scholar
  17. 17.
    Gilligan PH. Microbiology of cystic fibrosis lung disease. In: Yankaskas JR, Knowles MR, eds. Cystic Fibrosis in Adults. Philadelphia: Lippincott-Raven, 1998.Google Scholar
  18. 18.
    Kilby JM, Gilligan PH, Yankaskas JR, et al. Nontuberculous mycobacterial in adult patients with cystic fibrosis. Chest 1992; 102:70.PubMedCrossRefGoogle Scholar
  19. 19.
    Aitken ML, Burke W, McDonald G, et al. Nontuberculous mycobacterial disease in adult cystic fibrosis patients. Chest 1993; 103:1096.PubMedCrossRefGoogle Scholar
  20. 20.
    Olivier KN, Yankaskas JR, Knowles MR. Nontuberculous mycobacterial pulmonary disease in cystic fibrosis. Semin Respir Infect 1996; 11:272.PubMedGoogle Scholar
  21. 21.
    Hall CB, McBride JR, Gala CL, et al. Ribavirin treatment of respiratory syncytial viral infection in infants with underlying cardiopulmonary disease. JAMA 1985; 254:30–47.Google Scholar
  22. 22.
    McDonald NE, Hall CB, Suffin SC. Et al. Respiratory syncytial virus in infants with congenital heart disease. N Engl J Med 1982; 307:397.CrossRefGoogle Scholar
  23. 23.
    Hiatt P. The role of viral infections in cystic fibrosis. Peditr Pulmonol 1992; 8(Suppl):118–19.Google Scholar
  24. 24.
    Abman SH, Hogle JW, Butler-Simon N. Et al. Role of respiratory syncytial virus in early hospitalizations for respiratory distress of young infants with cystic fibrosis. J Pediatr 1988;113:826–30.PubMedCrossRefGoogle Scholar
  25. 25.
    Jaffe BR, Strome M, Khaw KT, Shwachman H. Nasal polypectomy and sinus surgery for cystic fibrosis-a 10 yeras review. Otolaryngol Clin North Am 1977; 10:81–90.PubMedGoogle Scholar
  26. 26.
    Zizmore J, Noyek AM, Chapnick IS. Mucocele of the paranasal sinuses. Can J Otolaryngol 1974; 3(suppl):1–30.Google Scholar
  27. 27.
    Siegel MJ, Shackelford GD, McAlister WH. Paranasal sinus mucoceles in children. Radiology 1979; 133:623–6.PubMedGoogle Scholar
  28. 28.
    Strauss RG, West PJ, Silverman FN. Unilateral proptosis in cystic fibrosis. Pediatrics 1969; 43:297–300.PubMedGoogle Scholar
  29. 29.
    Redding GJ, Restucia R, Cotton EK and Brooks JG. Serial changes in pulmonary functions in children hospitalized with cystic fibrosis. Am Rev Respir Dis 1982; 126:31–6.PubMedGoogle Scholar
  30. 30.
    Knudsen RJ, Slatin RC, Lebowitz MP, et al. The maximal expiratory flow-volume curve. Normal standards, variability and effects of age. Am Rev Resp Dis 1976; 113:587–600.Google Scholar
  31. 31.
    Wessel HU. Lung function in cystic fibrosis, In: Textbook of Cystic Fibrosis (ed. Lloyd-Still), John Wright, Boston, 1983, pp. 199–215.Google Scholar
  32. 32.
    Desmond KJ, Coates AL, and Beaudry PH. Relationship between the partial pressure of arterial oxygen and airflow limitation in children with cystic fibrosis. Can Med Assoc J 1984; 131:325–6.PubMedGoogle Scholar
  33. 33.
    Powers SK, Dodd S, Freeman J, et al. Accuracy of pulse oximetry to estimate Hb02 fraction of total Hb during exercise. J Appl Physiol 1989; 67:300–4.PubMedGoogle Scholar
  34. 34.
    Doring G, Conway SP, Heijerman HGM, Hodson ME, Hoiby N, Smith A, Touw DJ. Antibiotic therapy againstPseudomonas aeruginosain cystic fibrosis: a European consensus. Eur Respir J 2000; 16:749–767.PubMedCrossRefGoogle Scholar
  35. 35.
    Cystic Fibrosis: Antibiotic Therapy. Report of the meeting held at the Royal College of Pathologists, London. November 6, 1996.Google Scholar
  36. 36.
    SP Conway, Littlewood JM, Brownlee KG. Cystic Fibrosis in children and adults. The Leeds method of management. Revised Edition, number 5, 2000.Google Scholar
  37. 37.
    Ramsey BW, Pepe MS, Quan JM, Otto KL. Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. N Engl J Med 1999; 340:23–30.PubMedCrossRefGoogle Scholar
  38. 38.
    De Gracia J, Maiz L, Prados C, Vendrell M, Baranda F, Escribano A, et al. Conferencia de Consenso. Antibioticos nebulizados en pacientes con Fibrosis Quistica. FundaciOn “Sira Carrasco” para la ayuda a la Fibrosis quistica. 1999.Google Scholar
  39. 39.
    Campbell PW, Saiman L. Use of aerosolized antibiotics in patients with cystic fibrosis. Consensus conference. Chest 1999; 116:775–788.Google Scholar
  40. 40.
    Ryan G, Mukhopadhhyay S, Singh M. Nebulised anti-pseudomonal antibiotics for cystic fibrosis. The Cochrane Library. Volume (Issue 2) 2002Google Scholar
  41. 41.
    Southern KW, Barker PM, Solis A. Macrolide antibiotics for cystic fibrosis. The Cochrane Library. Volume (Issue 2) 2002.Google Scholar
  42. 42.
    Sánchez-Solis M, Gartner S, Salcedo A, Vazquez C, Antelo MC, Cobos N, et al. Protocolo de diagnóstico y seguimiento de los enfermos con fibrosis quística. Sociedad española de Neumologia pediatra.Google Scholar
  43. 43.
    Elphick HE, Tan A. Single versus combination intravenous antibiotic therapy for people with cystic fibrosis. The Cochrane Library. Volume (Issue 2) 2002Google Scholar
  44. 44.
    Marco T, Asensio 0, Bosque M, de Gracia J, Serra C. Home intravenous antibiotics for cystic fibrosis. The Cochrane Library. Volume (Issue 2) 2002.Google Scholar
  45. 45.
    Elborn JS, Prescott RJ, Stack BHR, Goodchild MC, Bates J, et al. Elective versus symptomatic antibiotic treatment in cystic fibrosis patients with chronic Pseudomonas infection of the lungs. Thorax 2000; 55:355–358.PubMedCrossRefGoogle Scholar
  46. 46.
    Murphy TM, Rosenstein BJ. Cystic Fibrosis Lung Disease: Approaching the 21stCentury. University of Chicago. Pritzker School of Medicine, October 1995Google Scholar
  47. 47.
    Denis Shale, Diana Bilton, Steven Conway, Margaret Hodson, Aileen Redmond, Bryan Stack, Kevin Webb. Management of Cystic Fibrosis in Adults. Cystic Fibrosis Trust. February 1995.Google Scholar
  48. 48.
    Davis PB, Drumm M, Konstan W. Cystic Fibrosis. Am J Respir Crit Care Med 1996; 154:1229–1256.PubMedGoogle Scholar
  49. 49.
    Ramsey B. Management of pulmonary disease in patients with cystic fibrosis. N Engl J Med 1996; 335:179–188.PubMedCrossRefGoogle Scholar
  50. 50.
    Tam K, Bunn H. Once daily versus multiple daily dosing with intravenous aminoglycosides for cystic fibrosis. The Cochrane Library. Volume (Issue 2) 2002.Google Scholar
  51. 51.
    Koch C, Hoiby N. Diagnosis and Treatment of cystic fibrosis. Respiration 2000; 67:239–247.PubMedCrossRefGoogle Scholar
  52. 52.
    Smyth A, Walters S. Prophylactic antibiotics for cystic fibrosis. The Cochrane Library. Volume (Issue 2) 2002.Google Scholar
  53. 53.
    Smyth RL, Cheng K, Motley J. Systematic reviews in cystic fibrosis. J Royal Soc Med 1998; 91(suppl 34):19–24.Google Scholar
  54. 54.
    Keogan MT, Johansen HK. Vaccines for prevention of infection with Pseudomonas aeruginosa in people with cystic fibrosis. The Cochrane Library. Volume (Issue 2) 2002Google Scholar
  55. 55.
    Littlewood J, Connnet G, Conway S, Eagles C, Govan J, Hodson M, Livermore D. Smith R, Scott M, Webb K. Antibiotic treatment for cystic fibrosis. Report of the UK Cystic Fibrosis Trust’s Antibiotic Group. April 2000.Google Scholar
  56. 56.
    Banerjee D, Stableforth D. The treatment of respiratory Pseudomonas infection in cystic fibrosis. Drugs 2000; 60:1053–1064.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2003

Authors and Affiliations

  • Montserrat Bosque
    • 1
  • Òscar Asensio
    • 1
  1. 1.Hospital de SabadellCorporacio Sanitaria Parc TauliBarcelonaSpain

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