The Journal of Pediatrics
Volume 154, Issue 2 , Pages 183-188.e3 , February 2009

Impact of Pseudomonas and Staphylococcus Infection on Inflammation and Clinical Status in Young Children with Cystic Fibrosis

  • Scott D. Sagel, MD

      Affiliations

    • Department of Pediatrics, The Children's Hospital and University of Colorado Health Sciences Center, Aurora, CO
    • Corresponding Author InformationReprint requests: Scott Sagel, MD, The Children's Hospital, 13123 E 16th Ave, B-395, Aurora, CO 80045
    • ,
  • Ronald L. Gibson, MD, PhD

      Affiliations

    • Department of Pediatrics, Children's Hospital & Regional Medical Center and University of Washington, Seattle, WA
    • ,
  • Julia Emerson, MD, MPH

      Affiliations

    • Department of Pediatrics, Children's Hospital & Regional Medical Center and University of Washington, Seattle, WA
    • ,
  • Sharon McNamara, MN

      Affiliations

    • Department of Pediatrics, Children's Hospital & Regional Medical Center and University of Washington, Seattle, WA
    • ,
  • Jane L. Burns, MD

      Affiliations

    • Department of Pediatrics, Children's Hospital & Regional Medical Center and University of Washington, Seattle, WA
    • ,
  • Jeffrey S. Wagener, MD

      Affiliations

    • Department of Pediatrics, The Children's Hospital and University of Colorado Health Sciences Center, Aurora, CO
    • ,
  • Bonnie W. Ramsey, MD

      Affiliations

    • Department of Pediatrics, Children's Hospital & Regional Medical Center and University of Washington, Seattle, WA
    • Cystic Fibrosis Foundation Therapeutics Development Network Coordinating Center, Children's Hospital and Regional Medical Center, Seattle, WA
    • ,
  • Inhaled Tobramycin in Young Children Study Group

      Affiliations

    • A list of additional members of the Inhaled Tobramycin in Young Children Study Group is available in the appendix (available at www.jpeds.com).
    • ,
  • Cystic Fibrosis Foundation Therapeutics Development Network

Received 1 April 2008 ,Revised 23 June 2008 ,Accepted 1 August 2008.

References 

  1. Welsh MJ, Ramsey BW, Accurso FJ, Cutting GR. Cystic fibrosis. In:  Scriver CR,  Beaudet AL,  Valle D,  Sly WS editor. The Metabolic and Molecular Bases of Inherited Disease. New York: McGraw-Hill; 2001;p. 521–588
  2. Birrer P, McElvaney NG, Rudeberg A, Sommer CW, Liechti-Gallati S, Kraemer R, et al. Protease-antiprotease imbalance in the lungs of children with cystic fibrosis. Am J Respir Crit Care Med. 1994;150:207–213
  3. 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
  4. Balough K, McCubbin M, Weinberger M, Smits W, Ahrens 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
  5. Armstrong DS, Grimwood K, Carlin JB, Carzino R, Olinsky A, Phelan PD. Bronchoalveolar lavage or oropharyngeal cultures to identify lower respiratory pathogens in infants with cystic fibrosis. Pediatr Pulmonol. 1996;21:267–275
  6. Armstrong DS, Grimwood K, Carlin JB, Carzino R, Gutierrez JP, Hull J, et al. Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med. 1997;156:1197–1204
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  8. Muhlebach MS, Stewart PW, Leigh MW, Noah TL. Quantitation of inflammatory responses to bacteria in young cystic fibrosis and control patients. Am J Respir Crit Care Med. 1999;160:186–191
  9. Rosenfeld M, Gibson RL, McNamara S, Emerson J, Burns JL, Castile R, et al. Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis. Pediatr Pulmonol. 2001;32:356–366
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  11. Henry RL, Mellis CM, Petrovic L. Mucoid Pseudomonas aeruginosa is a marker of poor survival in cystic fibrosis. Pediatr Pulmonol. 1992;12:158–161
  12. Demko CA, Byard PJ, Davis PB. Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection. J Clin Epidemiol. 1995;48:1041–1049
  13. Parad RB, Gerard CJ, Zurakowski D, Nichols DP, Pier GB. Pulmonary outcome in cystic fibrosis is influenced primarily by mucoid Pseudomonas aeruginosa infection and immune status and only modestly by genotype. Infect Immun. 1999;67:4744–4750
  14. Abman SH, Ogle JW, Harbeck RJ, Butler-Simon N, Hammond KB, Accurso FJ. Early bacteriologic, immunologic, and clinical courses of young infants with cystic fibrosis identified by neonatal screening. J Pediatr. 1991;119:211–217
  15. Hudson VL, Wielinski CL, Regelmann WE. Prognostic implications of initial oropharyngeal bacterial flora in patients with cystic fibrosis diagnosed before the age of two years. J Pediatr. 1993;122:854–860
  16. Kerem E, Corey M, Gold R, Levison H. Pulmonary function and clinical course in patients with cystic fibrosis after pulmonary colonization with Pseudomonas aeruginosa. J Pediatr. 1990;116:714–719
  17. Kosorok MR, Zeng L, West SE, Rock MJ, Splaingard ML, Laxova A, et al. Acceleration of lung disease in children with cystic fibrosis after Pseudomonas aeruginosa acquisition. Pediatr Pulmonol. 2001;32:277–287
  18. Emerson J, Rosenfeld M, McNamara S, Ramsey B, Gibson RL. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatr Pulmonol. 2002;34:91–100
  19. Li Z, Kosorok MR, Farrell PM, Laxova A, West SE, Green CG, et al. Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis. JAMA. 2005;293:581–588
  20. Rosenfeld M, Emerson J, Accurso F, Armstrong D, Castile R, Grimwood K, et al. Diagnostic accuracy of oropharyngeal cultures in infants and young children with cystic fibrosis. Pediatr Pulmonol. 1999;28:321–328
  21. Jung A, Kleinau I, Schonian G, Bauernfeind A, Chen C, Griese M, et al. Sequential genotyping of Pseudomonas aeruginosa from upper and lower airways of cystic fibrosis patients. Eur Respir J. 2002;20:1457–1463
  22. Burns JL, Gibson RL, McNamara S, Yim D, Emerson J, Rosenfeld M, et al. Longitudinal assessment of Pseudomonas aeruginosa in young children with cystic fibrosis. J Infect Dis. 2001;183:444–452
  23. Gibson RL, Emerson J, McNamara S, Burns JL, Rosenfeld M, Yunker A, et al. Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis. Am J Respir Crit Care Med. 2003;167:841–849
  24. Gibson RL, Emerson J, Mayer-Hamblett N, Burns JL, McNamara S, Accurso FJ, et al. Duration of treatment effect after tobramycin solution for inhalation in young children with cystic fibrosis. Pediatr Pulmonol. 2007;42:610–623
  25. Doershuk CF, Matthews LW, Tucker AS, Nudleman H, Eddy G, Wise M, et al. A 5-year clinical evaluation of a therapeutic program for patients with cystic fibrosis. J Pediatr. 1964;65:677–693
  26. Nixon GM, Armstrong DS, Carzino R, Carlin JB, Olinsky A, Robertson CF, et al. Clinical outcome after early Pseudomonas aeruginosa infection in cystic fibrosis. J Pediatr. 2001;138:699–704
  27. Bruce MC, Poncz L, Klinger JD, Stern RC, Tomashefski JF, Dearborn DG. Biochemical and pathologic evidence for proteolytic destruction of lung connective tissue in cystic fibrosis. Am Rev Respir Dis. 1985;132:529–535
  28. Cystic Fibrosis Foundation Patient Registry, 2004 Annual Data Report to the Center Directors. Bethesda, MD: Cystic Fibrosis Foundation; 2005;
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  30. Meyer KC, Sharma A. Regional variability of lung inflammation in cystic fibrosis. Am J Respir Crit Care Med. 1997;156:1536–1540
  31. Gutierrez JP, Grimwood K, Armstrong DS, Carlin JB, Carzino R, Olinsky A, et al. Interlobar differences in bronchoalveolar lavage fluid from children with cystic fibrosis. Eur Respir J. 2001;17:281–286

 Supported by grants from the National Institutes of Health (1 RO1 DK 57755-01, -02, K23 RR018611-05, and U01 HL081335-01), US Food and Drug Administration (FD-R-001695-01), Cystic Fibrosis Foundation Therapeutics Development Center Network, Novartis Corporation, and General Clinical Research Centers Program, National Center for Research Resources (MO1-RR00037, RR00046, RR00052, RR00064, RR00069, RR00070, RR00080, RR00188, RR02172, and RR08084). The authors declare no conflicts of interest.

PII: S0022-3476(08)00677-X

doi: 10.1016/j.jpeds.2008.08.001

The Journal of Pediatrics
Volume 154, Issue 2 , Pages 183-188.e3 , February 2009

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