Published ahead of print on September 11, 2008, doi:10.1164/rccm.200804-551OC Am. J. Respir. Crit. Care Med., Volume 178, Number 12, December 2008, 1238-1244 A more recent version of this article appeared on December 15, 2008
Submitted on April 13, 2008 Lung Function in Infants with Cystic Fibrosis Diagnosed by Newborn ScreeningBarry M Linnane1,1 Department of Respiratory Medicine, Royal Children's Hospital, Melbourne, Australia; Infection, Immunity and Environment Theme, Murdoch Children's Research Institute, Melbourne, Australia, 2 Department of Respiratory Medicine, Princess Margaret Hospital, Perth, Australia; Clinical Sciences, Telethon Institute for Child Health Research, Centre for Child Health Research, University of Western Australia, Perth, Australia; School of Paediatric and Child Health, University of Western Australia, Perth, Australia, 3 Department of Respiratory Medicine, Princess Margaret Hospital, Perth, Australia; Clinical Sciences, Telethon Institute for Child Health Research, Centre for Child Health Research, University of Western Australia, Perth, Australia, 4 Clinical Sciences, Telethon Institute for Child Health Research, Centre for Child Health Research, University of Western Australia, Perth, Australia, 5 Department of Respiratory Medicine, Royal Children's Hospital, Melbourne, Australia; Infection, Immunity and Environment Theme, Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia, 6 School of Paediatric and Child Health, University of Western Australia, Perth, Australia, 7 Department of Respiratory Medicine, Princess Margaret Hospital, Perth, Australia * To whom correspondence should be addressed. E-mail: sarath.raganathan{at}rch.org.au.
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, age=6 weeks to 30 months old), and healthy infants without CF (n=49) were studied. Forced vital capacity (FVC), forced expired volume in 0.5s (FEV0.5) and forced-expiratory flows at 75% of exhaled VC (FEV75) were measured using the raised volume rapid thoracoabdominal compression technique. 48 hours later infants with CF had broncho-alveolar 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% CI: -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 controls < 6 months old (-0.06 and 0.02 respectively, p = 0.87). However, mean FEV0.5 z-score was lower by 1.15 in infants with CF who were > 6 months 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 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 six months of life. Key words: spirometry, bronchoalveolar lavage, inflammation, infection
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