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

Journal of Pediatrics

Volumn 153, Issue 6, 2008, Pages 752-757

  McPhail, Gary L ^a   Acton, James D ^a   Fenchel, Matthew C ^a   Amin, Raouf S ^a   Seid, Michael ^a  

^a UNIVERSITY OF CINCINNATI   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

DORNASE ALFA;

ARTICLE; BODY MASS; CHRONIC DISEASE; CYSTIC FIBROSIS; DISEASE ASSOCIATION; FORCED EXPIRATORY VOLUME; GRAM NEGATIVE INFECTION; HUMAN; LUNG FUNCTION; NONHUMAN; NUTRITIONAL ASSESSMENT; PRIORITY JOURNAL; PSEUDOMONAS AERUGINOSA; TREATMENT OUTCOME;

BODY MASS INDEX; CHILD; CHILD, PRESCHOOL; CHRONIC DISEASE; COHORT STUDIES; CYSTIC FIBROSIS; DEOXYRIBONUCLEASE I; FEMALE; GROWTH; HUMANS; LONGITUDINAL STUDIES; MALE; MAXIMAL EXPIRATORY FLOW RATE; NUTRITIONAL SUPPORT; PREDICTIVE VALUE OF TESTS; PSEUDOMONAS INFECTIONS; TREATMENT OUTCOME;

EID: 56049116219     PISSN: 00223476     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.jpeds.2008.07.011     Document Type: Article

References (23)

1. Kerem E., Reisman J., Corey M., Canny G., and Levison H. Prediction of mortality in patients with cystic fibrosis. N Engl J Med 326 (1992) 1187-1191
2. Liou T.G., Adler F.R., FitzSimmons S.C., Cahill B., Hibbs J., and Marshall B. Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol 153 (2001) 345-352
3. Corey M., Edwards L., Levison H., and Knowles M. Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis. J Pediatr 131 (1997) 809-814
4. Milla C.E., and Warwick W.J. Risk of death in cystic fibrosis patients with severely compromised lung function. Chest 113 (1998) 1230-1234
5. Cystic Fibrosis Foundation. Patient registry 2006: annual data report. http://www.cff.org Accessed
6. 1 in young adults with cystic fibrosis. Thorax 61 (2006) 155-157
7. Davis P.B., Byard P.J., and Konstan M.W. Identifying treatments that halt progression of pulmonary disease in cystic fibrosis. Pediatr Res 41 (1997) 161-165
8. Konstan M.W., Morgan W.J., Butler S.M., 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 151 (2007) 134-139
9. American Thoracic Society. Standardization of spirometry: 1994 update. Am J Respir Crit Care Med 152 (1995) 1107-1136
10. Miller M.R., Hankinson J., Brusasco V., Burgos F., Casaburi R., Coates A., et al. Standardisation of spirometry. Eur Respir J 26 (2005) 319-338
11. Wang X., Dockery D.W., Wypij D., Fay M.E., Ferris B.G., et al. Pulmonary function between 6 and 18 years of age. Pediatr Pulmonol 15 (1993) 75-78
12. Hamill P.V., Drizd T.A., Johnson C.L., Reed R.B., Roche A.F., and Moore W.M. Physical growth: National Center for Health Statistics percentiles. Am J Clin Nutr 32 (1979) 607-629
13. Milla C.E., Warwick W.J., and 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 162 (2000) 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 33 (2002) 483-491
15. Kosorok M.R., 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 32 (2001) 277-287
16. 1decline in children with cystic fibrosis. Am J Respir Crit Care Med 176 (2007) 1084-1089
17. Merkus P.J.F.M., Tiddens H.A.W.M., and de Jonste J.C. Annual lung function changes in young patients with chronic lung disease. Eur Respir J 19 (2002) 886-891
18. Fuchs H.J., Borowitz D.S., Christiansen D.H., 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 331 (1994) 637-642
19. Quan J.M., Tiddens H.A.W.M., Sy J.P., 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 139 (2001) 813-820
20. Padman R., McColley S.A., Miller D.P., 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 119 (2007) e531-e537
21. Zemel B.S., Jawad A.F., FitzSimmons S., and Stallings V.A. 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 137 (2000) 374-380
22. Konstan M.W., Butler S.M., Wohl M.E., Stoddard M., Matousek R., Wagener J., et al. Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis. J Pediatr 142 (2003) 624-630
23. Ratjen F., Paul K., van Koningsbruggen S., Breitenstein S., Rietschel E., and Nikolaizik W. DNA concentrations in BAL fluid of cystic fibrosis patients with early lung disease: influence of treatment with dornase alpha. Pediatr Pulmonol 39 (2005) 1-4