Lung function in infants with cystic fibrosis diagnosed by newborn screening

Linnane, Barry M., Hall, Graham L., Nolan, Gary, Brennan, Siobhan, Stick, Stephen M., Sly, Peter D., Robertson, Colin F., Robinson, Philip J., Franklin, Peter J., Turner, Stephen W. and Ranganatham, Sarath C. (2008) Lung function in infants with cystic fibrosis diagnosed by newborn screening. American Journal of Respiratory And Critical Care Medicine, 178 12: 1238-1244. doi:10.1164/rccm.200804-551OC

Author Linnane, Barry M.
Hall, Graham L.
Nolan, Gary
Brennan, Siobhan
Stick, Stephen M.
Sly, Peter D.
Robertson, Colin F.
Robinson, Philip J.
Franklin, Peter J.
Turner, Stephen W.
Ranganatham, Sarath C.
Title Lung function in infants with cystic fibrosis diagnosed by newborn screening
Journal name American Journal of Respiratory And Critical Care Medicine   Check publisher's open access policy
ISSN 1073-449X
Publication date 2008-12
Sub-type Article (original research)
DOI 10.1164/rccm.200804-551OC
Volume 178
Issue 12
Start page 1238
End page 1244
Total pages 7
Place of publication New York, NY, United States
Publisher American Thoracic Society
Language eng
Abstract Rationale: Progressive lung damage in cystic fibrosis (CF) starts in infancy, and early detection may aid preventative strategies. Objectives: To measure lung function in infants with CF diagnosed by newborn screening and describe its association with pulmonary infection and inflammation. Methods: Infants with CF (n = 68, 6 weeks to 30 months of age) and healthy infants without CF (n = 49) were studied. Forced vital capacity, FEV0.5, and forced expiratory flows at 75% of exhaled vital capacity (FEF75) were measured using the raised-volume rapid thoracoabdominal compression technique. Forty-eight hours later, infants with CF had bronchoalveolar lavage (BAL) for assessment of pulmonary infection and inflammation. Measurements and Main Results: In the CF group, the deficit in FEV0.5 z score increased by -0.77 (95% confidence interval, -1.14 to -0.41; P < 0.001) with each year of age. The mean FEV0.5 z score did not differ between infants with CF and healthy control subjects less than 6 months of age (-0.06 and 0.02, respectively; P = 0.87). However, the mean FEV0.5 z score was lower by 1.15 in infants with CF who were older than 6 months of age compared with healthy infants (P < 0.001). FVC and FEF75 followed a similar pattern. Pulmonary infection and inflammation in BAL samples did not explain the lung function results. Conclusions: Lung function, measured by forced expiration, is normal in infants with CF at the time of diagnosis by newborn screening but is diminished in older infants. These findings suggest that in CF the optimal timing of therapeutic interventions aimed at preserving lung function may be within the first 6 months of life.
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: Faculty of Health and Behavioural Sciences -- Publications
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Created: Wed, 17 Nov 2010, 11:37:26 EST