The Journal of Pediatrics
Volume 153, Issue 6 , Pages 752-757 , December 2008

Improvements in Lung Function Outcomes in Children with Cystic Fibrosis are Associated with Better Nutrition, Fewer Chronic Pseudomonas aeruginosa Infections, and Dornase Alfa Use

Received 8 November 2007 ,Revised 2 May 2008 ,Accepted 8 July 2008.

References 

  1. Kerem E, Reisman J, Corey M, Canny G, Levison H. Prediction of mortality in patients with cystic fibrosis. N Engl J Med. 1992;326:1187–1191
  2. Liou TG, Adler FR, FitzSimmons SC, Cahill B, Hibbs J, Marshall B. Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol. 2001;153:345–352
  3. Corey M, Edwards L, Levison H, Knowles M. Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis. J Pediatr. 1997;131:809–814
  4. Milla CE, Warwick WJ. Risk of death in cystic fibrosis patients with severely compromised lung function. Chest. 1998;113:1230–1234
  5. Cystic Fibrosis Foundation. Patient registry 2006: annual data report. http://www.cff.orgAccessed
  6. Que C, Cullinan P, Geddes D. Improving rate of decline of FEV1 in young adults with cystic fibrosis. Thorax. 2006;61:155–157
  7. Davis PB, Byard PJ, Konstan MW. Identifying treatments that halt progression of pulmonary disease in cystic fibrosis. Pediatr Res. 1997;41:161–165
  8. Konstan MW, Morgan WJ, Butler SM, Pasta D, Craib M, Silva S, et al. Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis. J Pediatr. 2007;151:134–139
  9. American Thoracic Society. Standardization of spirometry: 1994 update. Am J Respir Crit Care Med. 1995;152:1107–1136
  10. Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A, et al. Standardisation of spirometry. Eur Respir J. 2005;26:319–338
  11. Wang X, Dockery DW, Wypij D, Fay ME, Ferris BG, et al. Pulmonary function between 6 and 18 years of age. Pediatr Pulmonol. 1993;15:75–78
  12. Hamill PV, Drizd TA, Johnson CL, Reed RB, Roche AF, Moore WM. Physical growth: National Center for Health Statistics percentiles. Am J Clin Nutr. 1979;32:607–629
  13. Milla CE, Warwick WJ, Moran A. Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline. Am J Respir Crit Care Med. 2000;162:891–895
  14. Schaedel C, de Monestrol I, Hjelte L, Johannesson M, Kornfält R, Lindblad A, et al. Predictors of deterioration of lung function in cystic fibrosis. Pediatr Pulmonol. 2002;33:483–491
  15. Kosorok MR, Zeng L, West S, Rock M, Splaingard M, Laxova A, et al. Acceleration of lung disease in children with cystic fibrosis after Pseudomonas aeruginosa acquisition. Pediatr Pulmonol. 2001;32:277–287
  16. Konstan MW, Schluchter MD, Xue W, Davis PB. Clinical use of ibuprofen is associated with slower FEV1decline in children with cystic fibrosis. Am J Respir Crit Care Med. 2007;176:1084–1089
  17. Merkus PJFM, Tiddens HAWM, de Jonste JC. Annual lung function changes in young patients with chronic lung disease. Eur Respir J. 2002;19:886–891
  18. Fuchs HJ, Borowitz DS, Christiansen DH, Morris E, Nash M, Ramsey B, 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
  19. Quan JM, Tiddens HAWM, Sy JP, McKenzie S, Montgomery M, Robinson P, et al. A two-year randomized, placebo-controlled trial of dornase alpha in young patients with cystic fibrosis with mild lung function abnormalities. J Pediatr. 2001;139:813–820
  20. Padman R, McColley SA, Miller DP, Konstan M, Morgan W, Schechter M, et al. Infant care patterns at epidemiologic study of cystic fibrosis sites that achieve superior childhood lung function. Pediatrics. 2007;119:e531–e537
  21. Zemel BS, Jawad AF, FitzSimmons S, Stallings VA. Longitudinal relationship among growth, nutritional status, and pulmonary function in children with cystic fibrosis: analysis of the Cystic Fibrosis Foundation National CF Patient Registry. J Pediatr. 2000;137:374–380
  22. Konstan MW, Butler SM, Wohl ME, Stoddard M, Matousek R, Wagener J, et al. Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis. J Pediatr. 2003;142:624–630
  23. Ratjen F, Paul K, van Koningsbruggen S, Breitenstein S, Rietschel E, Nikolaizik W. DNA concentrations in BAL fluid of cystic fibrosis patients with early lung disease: influence of treatment with dornase alpha. Pediatr Pulmonol. 2005;39:1–4

 The authors have no conflicts of interest to disclose. There was no sponsor involvement in the design of this study or in the preparation of this manuscript for publication.

PII: S0022-3476(08)00591-X

doi: 10.1016/j.jpeds.2008.07.011

The Journal of Pediatrics
Volume 153, Issue 6 , Pages 752-757 , December 2008