Abstract
We analysed the pulmonary evolution (radiological scores and pulmonary function) of 81 cystic fibrosis (CF) patients colonized by Pseudomonas aeruginosa (PA), by Burkholderia cepacia (BC) or by both these bacteria, compared to a control group. Pulmonary function was compared in the age bracket 6–13 years. Functional vital capacity (FVC) and forced expiratory volume (FEV1) values for PA colonized patients were significantly worse than for the control group but better than for children colonized by both organisms. In this last group, the evolution of radiological scores and pulmonary function showed a greater decline 2 years after the first colonization compared to the other groups. FVC and FEV1 values in patients colonized by BC were not worse than these of patients colonized by PA. Moreover, BC affected older patients with advanced lung disease and often previously colonized with PA. These results suggested that co-colonization by PA and BC could be a more deleterious factor on the pulmonary evolution than the isolated colonization by PA or BC, and that BC could be a severity marker rather than a cause. In addition, after starting the utilization of mouthpieces with filter at single use for spirometry in 1993 (without any other change in preventive measures already taken during hospitalization), incidence of BC decreased from 8.2% to zero, and no new case of BC colonization has been observed over the last 4 years.
Conclusion
Co-colonization of CF patients by PA and BC is more deleterious for pulmonary evolution than colonization by one of these bacteria alone. Re-inforcement of environmental measures during hospitalization (e.g. use of disposable mouthpieces for spirometry) was sufficient to reduce the transmission of BC.
Similar content being viewed by others
Abbreviations
- CF:
-
cystic fibrosis
- PA:
-
Pseudomonas aeruginosa
- BC:
-
Burkholderia cepacia
- FEV1 :
-
forced expiratory volume
- FVC:
-
functional vital capacity
References
Anonymous (1993) United States Cystic Fibrosis Foundation (letter). 18 January
Aronoff SC, Quinn FJ, Stern J, RC (1991) Longitudinal serum IgG response to Pseudomonas cepacia surface antigens in cystic fibrosis. Pediatr Pulmonol 11:289–293
Bingen EH, Weber M, Derelle J, Brahimi N, Lambert-Zechovsky NY, Vidailhet M, Navarro J, Elion J (1993) Arbitrarily primed polymerase chain reaction as a rapid method to differentiate crossed from independent Pseudomonas cepacia infections in cystic fibrosis patients. J Clin Microbiol 31, 10:2589–2593
Chrispin AR, Norman AP (1974) The systematic evaluation of the chest radiograph with cystic fibrosis. Pediatr Radiol 2:101–105
Gladman G, Connor PJ, Williams RF, David TJ (1992) Controlled study of Pseudomonas cepacia and Pseudomonas mal-tophilia in cystic fibrosis. Arch Dis Child 67:192–195
Govan JRW, Brown PH, Maddison J, Doherty CJ, Nelson JW, Dodd M, Greeming AP, Webb AK (1993) Evidence for transmission of Pseudomonas cepacia by social contact in cystic fibrosis. Lancet 342:15–19
Hardy KA, Mc Gowan KL, Fisher MC, Schidlow DV (1986) Pseudomonas cepacia in the hospital setting: lack of transmission between cystic fibrosis patients. J Pediatr 109:51–54
Hearst JE, Elliot KE (1995) Identifying the killer in cystic fibrosis. Nat Med 1, 7:626–627
Isles A, Maclusky I, Corey M, Gold R, Prober C, Fleming P, Levison H (1984) Pseudomonas cepacia infection in cystic fibrosis: an emerging problem. J Pediatr 104:206–210
JohansenHK, Kovesi T, Corey M, Levison H, Hoiby N (1994) P. aeruginosa and P. cepacia prevalences and correlation with pulmonary function differ in cystic fibrosis patients treated in Toronto and Copenhagen. Pediatr Pulmonol 18:254
Lipuma JJ, Dasen SE, Nielson DW, Stern RC, Stull TL (1990) Person-to-person transmission of Pseudomonas cepacia between patients with cystic fibrosis. Lancet 336:1094–1096
Mc Kenney D, Brown KE, Allison DG (1995) Influence of Pseudomonas aeruginosa exoproducts on virulence factor production in Burkholderia cepacia: evidence of inter species communication. J Bacteriol 177, 23:6989–6992
Minicucci L, Tubino B, Pelegrini M, Zona S, Diana MC, Manno G, Ugolotti E, Marzullo L, Romano L, Romano C (1994) Influence of Pseudomonas cepacia chronic infection on lung function in cystic fibrosis (CF). Pediatr Pulmonol 18:253
Pegues DA, Carson LA, Tablan OC, Fitz Simmons SC, Roman SB, Miller JM, Jarvis WR, Summer Camp Study Group (1994) Acquisition of Pseudomonas cepacia at summer camps for patients with cystic fibrosis. J Pediatr 124:694–702
Sajjan SU, Forstner JF (1992) Identification of the mucin-binding adhesin of Pseudomonas cepacia isolated from patients with cystic fibrosis. Infect Immun 60, 4:1434–1440
Sajjan US, Corey M, Karmali MA, Forstner JF (1992) Binding of Pseudomonas cepacia to normal human intestinal mucin and respiratory mucin from patients with cystic fibrosis. J Clin Invest 89, 2:648–656
Simmonds EJ, Conway SP, Ghoneim ATM, Ross H, Little-wood JM (1990) Pseudomonas cepacia: a new pathogen in patients with cystic fibrosis referred to a large center in the United Kingdom. Arch Dis Child 65:874–877
Smith DL, Gumery LB, Smith EG, Stableforth DE, Kaufmann ME, Pitt TL (1993) Epidemic of Pseudomonas cepacia in an adult cystic fibrosis unit: evidence of person-to-person transmission. J Clin Microbiol 31:3017–3022
Snell GI, Hoyos A de, Krajden M, Winton T, Maurer JR (1993) Pseudomonas cepacia in lung transplant recipients with cystic fibrosis. chest 103:466–471
Sun L, Jiang RZ, Steinbach S, Holmes A, Campanelli C, Forstner J, Sajjan U, Tan Y, Riley M, Goldstein R (1995) The emergence of a highly transmissible lineage of cbl + Pseudomonas (Burkholderia) cepacia causing CF centre epidemics in North America and Britain. Nat Med 1, 7:661–666
Sylvester FA, Sajjan US, Forstner JF (1996) Burkholderia (basonym Pseudomonas) cepacia binding to lipid receptors. Infect Immun 64, 4:1420–1425
Tablan OC, Chorba TL, Schidlow DV, White JW, Hardy KA, Gilligan PH, Morgan WM, Carson LA, Martone WJ, Jason JM, Jarvis WR (1985) Pseudomonas cepacia colonization in patients with cystic fibrosis: risk factors and clinical outcome. J Pediatr 107:382–387
Tablan OC, Martone WJ, Doershuk CF, Stern RC, Thomassen MJ, Klinger JD, White JW, Carson LA, Jarvis WR (1987) Colonization of the respiratory tract with Pseudomonas cepacia in cystic fibrosis. Risk factors and outcomes. Chest 91:527–553
Taylor RF, Gaya H, Hodson ME (1993) Pseudomonas cepacia: pulmonary infection in patients with cystic fibrosis. Respir Med 87, 3:187–192
Thomassen MJ, Demko CA, Klinger JD, Stern RC (1985) Pseudomonas cepacia Colonization among Patients with Cystic Fibrosis. A new Opportunist. Am Rev Respir Dis 131:791–796
Thomassen MJ, Demko CA, Doershuk CF, Stern RC, Klinger JD (1986) Pseudomonas cepacia: decrease in colonization in patients with cystic fibrosis. Am Rev Respir Dis 134:669–671
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Jacques, I., Derelle, J., Weber, M. et al. Pulmonary evolution of cystic fibrosis patients colonized by Pseudomonas aeruginosa and/or Burkholderia cepacia . Eur J Pediatr 157, 427–431 (1998). https://doi.org/10.1007/s004310050844
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/s004310050844