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Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 2002 Oct;87(4):306–311. doi: 10.1136/adc.87.4.306

Early airway infection, inflammation, and lung function in cystic fibrosis

G Nixon 1, D Armstrong 1, R Carzino 1, J Carlin 1, A Olinsky 1, C Robertson 1, K Grimwood 1
PMCID: PMC1763045  PMID: 12244003

Abstract

Aims: To determine the relation between lower airway infection and inflammation, respiratory symptoms, and lung function in infants and young children with cystic fibrosis (CF).

Methods: A prospective study of children with CF aged younger than 3 years, diagnosed by a newborn screening programme. All were clinically stable and had testing as outpatients. Subjects underwent bronchial lavage (BL) and lung function testing by the raised volume rapid thoracoabdominal compression technique under general anaesthesia. BL fluid was cultured and analysed for neutrophil count, interleukin 8, and neutrophil elastase. Lung function was assessed by forced expiratory volume in 0.5, 0.75, and 1 second.

Results: Thirty six children with CF were tested on 54 occasions. Lower airway infection shown by BL was associated with a 10% reduction in FEV0.5 compared with subjects without infection. No relation was identified between airway inflammation and lung function. Daily moist cough within the week before testing was reported on 20/54 occasions, but in only seven (35%) was infection detected. Independent of either infection status or airway inflammation, those with daily cough had lower lung function than those without respiratory symptoms at the time of BL (mean adjusted FEV0.5 195 ml and 236 ml respectively).

Conclusions: In young children with CF, both respiratory symptoms and airway infection have independent, additive effects on lung function, unrelated to airway inflammation. Further studies are needed to understand the mechanisms of airway obstruction in these young patients.

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Figure 1 .

Figure 1

(A) Neutrophil percentages in bronchial lavage fluid, by symptom and infection group. AU, asymptomatic/uninfected (n = 22); AI, asymptomatic/infected (n = 8); SU, symptomatic/uninfected (n = 13); SI, symptomatic/infected (n = 6). Open circles represent individual values; closed circles are geometric means following adjustment for repeated measures, with 95% CI. (B) Lung function by symptom and infection group. Open circles represent individual values for each subject; closed circles represent the mean FEV0.5 adjusted by analysis of variance for height for each group, with 95% CI. AU, n = 26; AI, n = 8; SU, n = 13; SI, n = 6).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Armstrong D. S., Grimwood K., Carlin J. B., Carzino R., Gutièrrez J. P., Hull J., Olinsky A., Phelan E. M., Robertson C. F., Phelan P. D. Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med. 1997 Oct;156(4 Pt 1):1197–1204. doi: 10.1164/ajrccm.156.4.96-11058. [DOI] [PubMed] [Google Scholar]
  2. Armstrong D. S., Grimwood K., Carlin J. B., Carzino R., Olinsky A., Phelan P. D. Bronchoalveolar lavage or oropharyngeal cultures to identify lower respiratory pathogens in infants with cystic fibrosis. Pediatr Pulmonol. 1996 May;21(5):267–275. doi: 10.1002/(SICI)1099-0496(199605)21:5<267::AID-PPUL1>3.0.CO;2-K. [DOI] [PubMed] [Google Scholar]
  3. Armstrong D. S., Grimwood K., Carzino R., Carlin J. B., Olinsky A., Phelan P. D. Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis. BMJ. 1995 Jun 17;310(6994):1571–1572. doi: 10.1136/bmj.310.6994.1571. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Beardsmore C. S., Bar-Yishay E., Maayan C., Yahav Y., Katznelson D., Godfrey S. Lung function in infants with cystic fibrosis. Thorax. 1988 Jul;43(7):545–551. doi: 10.1136/thx.43.7.545. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Beardsmore C. S. Lung function from infancy to school age in cystic fibrosis. Arch Dis Child. 1995 Dec;73(6):519–523. doi: 10.1136/adc.73.6.519. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Boat T. F., Cheng P. W., Iyer R. N., Carlson D. M., Polony I. Human respiratory tract secretion. Mucous glycoproteins of nonpurulent tracheobronchial secretions, and sputum of patients with bronchitis and cystic fibrosis. Arch Biochem Biophys. 1976 Nov;177(1):95–104. doi: 10.1016/0003-9861(76)90419-7. [DOI] [PubMed] [Google Scholar]
  7. Chace K. V., Leahy D. S., Martin R., Carubelli R., Flux M., Sachdev G. P. Respiratory mucous secretions in patients with cystic fibrosis: relationship between levels of highly sulfated mucin component and severity of the disease. Clin Chim Acta. 1983 Aug 15;132(2):143–155. doi: 10.1016/0009-8981(83)90242-5. [DOI] [PubMed] [Google Scholar]
  8. Chinn S. The assessment of methods of measurement. Stat Med. 1990 Apr;9(4):351–362. doi: 10.1002/sim.4780090402. [DOI] [PubMed] [Google Scholar]
  9. Chow C. W., Landau L. I., Taussig L. M. Bronchial mucous glands in the newborn with cystic fibrosis. Eur J Pediatr. 1982 Dec;139(4):240–243. doi: 10.1007/BF00442171. [DOI] [PubMed] [Google Scholar]
  10. Dakin Carolyn J., Numa Andrew H., Wang He, Morton John R., Vertzyas Calypso C., Henry Richard L. Inflammation, infection, and pulmonary function in infants and young children with cystic fibrosis. Am J Respir Crit Care Med. 2002 Apr 1;165(7):904–910. doi: 10.1164/ajrccm.165.7.2010139. [DOI] [PubMed] [Google Scholar]
  11. Dezateux C., Stocks J., Dundas I., Fletcher M. E. Impaired airway function and wheezing in infancy: the influence of maternal smoking and a genetic predisposition to asthma. Am J Respir Crit Care Med. 1999 Feb;159(2):403–410. doi: 10.1164/ajrccm.159.2.9712029. [DOI] [PubMed] [Google Scholar]
  12. Eid N. S., Accurso F. J., Stenzler A. Early asymptomatic pulmonary changes in infants with cystic fibrosis. Pediatr Pulmonol Suppl. 1997;16:276–277. doi: 10.1002/ppul.19502308143. [DOI] [PubMed] [Google Scholar]
  13. Gappa M., Ranganathan S. C., Stocks J. Lung function testing in infants with cystic fibrosis: lessons from the past and future directions. Pediatr Pulmonol. 2001 Sep;32(3):228–245. doi: 10.1002/ppul.1113. [DOI] [PubMed] [Google Scholar]
  14. Godfrey S., Mearns M., Howlett G. Serial lung function studies in cystic fibrosis in the first 5 years of life. Arch Dis Child. 1978 Jan;53(1):83–85. doi: 10.1136/adc.53.1.83. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Gutierrez J. P., Grimwood K., Armstrong D. S., Carlin J. B., Carzino R., Olinsky A., Robertson C. F., Phelan P. D. Interlobar differences in bronchoalveolar lavage fluid from children with cystic fibrosis. Eur Respir J. 2001 Feb;17(2):281–286. doi: 10.1183/09031936.01.17202810. [DOI] [PubMed] [Google Scholar]
  16. Habre W., Matsumoto I., Sly P. D. Propofol or halothane anaesthesia for children with asthma: effects on respiratory mechanics. Br J Anaesth. 1996 Dec;77(6):739–743. doi: 10.1093/bja/77.6.739. [DOI] [PubMed] [Google Scholar]
  17. Hanrahan J. P., Tager I. B., Segal M. R., Tosteson T. D., Castile R. G., Van Vunakis H., Weiss S. T., Speizer F. E. The effect of maternal smoking during pregnancy on early infant lung function. Am Rev Respir Dis. 1992 May;145(5):1129–1135. doi: 10.1164/ajrccm/145.5.1129. [DOI] [PubMed] [Google Scholar]
  18. Hardy K. A., Wolfson M. R., Schidlow D. V., Shaffer T. H. Mechanics and energetics of breathing in newly diagnosed infants with cystic fibrosis: effect of combined bronchodilator and chest physical therapy. Pediatr Pulmonol. 1989;6(2):103–108. doi: 10.1002/ppul.1950060209. [DOI] [PubMed] [Google Scholar]
  19. Hayden M. J., Sly P. D., Devadason S. G., Gurrin L. C., Wildhaber J. H., LeSouëf P. N. Influence of driving pressure on raised-volume forced expiration in infants. Am J Respir Crit Care Med. 1997 Dec;156(6):1876–1883. doi: 10.1164/ajrccm.156.6.9609003. [DOI] [PubMed] [Google Scholar]
  20. Heine R. G., Button B. M., Olinsky A., Phelan P. D., Catto-Smith A. G. Gastro-oesophageal reflux in infants under 6 months with cystic fibrosis. Arch Dis Child. 1998 Jan;78(1):44–48. doi: 10.1136/adc.78.1.44. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Hiatt P., Eigen H., Yu P., Tepper R. S. Bronchodilator responsiveness in infants and young children with cystic fibrosis. Am Rev Respir Dis. 1988 Jan;137(1):119–122. doi: 10.1164/ajrccm/137.1.119. [DOI] [PubMed] [Google Scholar]
  22. Khan T. Z., Wagener J. S., Bost T., Martinez J., Accurso F. J., Riches D. W. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med. 1995 Apr;151(4):1075–1082. doi: 10.1164/ajrccm/151.4.1075. [DOI] [PubMed] [Google Scholar]
  23. Lethem M. I., James S. L., Marriott C. The role of mucous glycoproteins in the rheologic properties of cystic fibrosis sputum. Am Rev Respir Dis. 1990 Nov;142(5):1053–1058. doi: 10.1164/ajrccm/142.5.1053. [DOI] [PubMed] [Google Scholar]
  24. Mohon R. T., Wagener J. S., Abman S. H., Seltzer W. K., Accurso F. J. Relationship of genotype to early pulmonary function in infants with cystic fibrosis identified through neonatal screening. J Pediatr. 1993 Apr;122(4):550–555. doi: 10.1016/s0022-3476(05)83534-6. [DOI] [PubMed] [Google Scholar]
  25. Nixon G. M., Armstrong D. S., Carzino R., Carlin J. B., Olinsky A., Robertson C. F., Grimwood K. Clinical outcome after early Pseudomonas aeruginosa infection in cystic fibrosis. J Pediatr. 2001 May;138(5):699–704. doi: 10.1067/mpd.2001.112897. [DOI] [PubMed] [Google Scholar]
  26. Phelan P. D., Gracey M., Williams H. E., Anderson C. M. Ventilatory function in infants with cystic fibrosis. Physiological assessment of halation therapy. Arch Dis Child. 1969 Jun;44(235):393–400. doi: 10.1136/adc.44.235.393. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Puchelle E., Jacquot J., Beck G., Zahm J. M., Galabert C. Rheological and transport properties of airway secretions in cystic fibrosis--relationships with the degree of infection and severity of the disease. Eur J Clin Invest. 1985 Dec;15(6):389–394. doi: 10.1111/j.1365-2362.1985.tb00290.x. [DOI] [PubMed] [Google Scholar]
  28. Ranganathan S. C., Dezateux C., Bush A., Carr S. B., Castle R. A., Madge S., Price J., Stroobant J., Wade A., Wallis C. Airway function in infants newly diagnosed with cystic fibrosis. Lancet. 2001 Dec 8;358(9297):1964–1965. doi: 10.1016/s0140-6736(01)06970-7. [DOI] [PubMed] [Google Scholar]
  29. Regnis J. A., Robinson M., Bailey D. L., Cook P., Hooper P., Chan H. K., Gonda I., Bautovich G., Bye P. T. Mucociliary clearance in patients with cystic fibrosis and in normal subjects. Am J Respir Crit Care Med. 1994 Jul;150(1):66–71. doi: 10.1164/ajrccm.150.1.8025774. [DOI] [PubMed] [Google Scholar]
  30. Sheikh S., Goldsmith L. J., Howell L., Parry L., Eid N. Comparison of lung function in infants exposed to maternal smoking and in infants with a family history of asthma. Chest. 1999 Jul;116(1):52–58. doi: 10.1378/chest.116.1.52. [DOI] [PubMed] [Google Scholar]
  31. Stick S. M., Burton P. R., Gurrin L., Sly P. D., LeSouëf P. N. Effects of maternal smoking during pregnancy and a family history of asthma on respiratory function in newborn infants. Lancet. 1996 Oct 19;348(9034):1060–1064. doi: 10.1016/s0140-6736(96)04446-7. [DOI] [PubMed] [Google Scholar]
  32. Tepper R. S., Hiatt P. W., Eigen H., Smith J. Total respiratory system compliance in asymptomatic infants with cystic fibrosis. Am Rev Respir Dis. 1987 May;135(5):1075–1079. doi: 10.1164/arrd.1987.135.5.1075. [DOI] [PubMed] [Google Scholar]
  33. Tepper R. S., Hiatt P., Eigen H., Scott P., Grosfeld J., Cohen M. Infants with cystic fibrosis: pulmonary function at diagnosis. Pediatr Pulmonol. 1988;5(1):15–18. doi: 10.1002/ppul.1950050105. [DOI] [PubMed] [Google Scholar]
  34. Turner D. J., Lanteri C. J., LeSouef P. N., Sly P. D. Improved detection of abnormal respiratory function using forced expiration from raised lung volume in infants with cystic fibrosis. Eur Respir J. 1994 Nov;7(11):1995–1999. [PubMed] [Google Scholar]
  35. Turner D. J., Stick S. M., Lesouëf K. L., Sly P. D., Lesouëf P. N. A new technique to generate and assess forced expiration from raised lung volume in infants. Am J Respir Crit Care Med. 1995 May;151(5):1441–1450. doi: 10.1164/ajrccm.151.5.7735598. [DOI] [PubMed] [Google Scholar]
  36. Zeger S. L., Liang K. Y. Longitudinal data analysis for discrete and continuous outcomes. Biometrics. 1986 Mar;42(1):121–130. [PubMed] [Google Scholar]

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