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Published ahead of print on January 30, 2004, doi:10.1164/rccm.200309-1344OC
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American Journal of Respiratory and Critical Care Medicine Vol 169. pp. 928-933, (2004)
© 2004 American Thoracic Society

Original Article

The Evolution of Airway Function in Early Childhood Following Clinical Diagnosis of Cystic Fibrosis

Sarath C. Ranganathan, Janet Stocks, Carol Dezateux, Andrew Bush, Angie Wade, Siobhán Carr, Rosemary Castle, Robert Dinwiddie, Ah-Fong Hoo, Sooky Lum, John Price, John Stroobant and Colin Wallis The London Collaborative Cystic Fibrosis Group

Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit; Centre for Paediatric Epidemiology and Biostatistics, Institute of Child Health; Department of Paediatric Respiratory Medicine, Royal Brompton Hospital; Department of Child Health, Royal London Hospital; Department of Paediatric Respiratory Medicine, Great Ormond Street Hospital; Department of Child Health, King's College Hospital; Department of Child Health, University Hospital Lewisham; and Neonatal Unit, Homerton University Hospital, London, United Kingdom

Correspondence and requests for reprints should be addressed to Sarath Ranganathan, M.B.Ch.B., M.R.C.P., M.R.C.P.C.H., Ph.D., Portex Unit, 6th Floor, Cardiac Wing, Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. E-mail: drsarath{at}clara.net

This study aimed to investigate the evolution of airway function in infants newly diagnosed with cystic fibrosis (CF). FEV0.5 was measured soon after diagnosis (median age of 28 weeks) and 6 months later in subjects with CF and on two occasions 6 months apart (median ages of 7.4 and 33.7 weeks) in healthy infants, using the raised-volume technique. Repeated measurements were successful in 34 CF and 32 healthy subjects. After adjustment for age, length, sex, and exposure to maternal smoking, mean FEV0.5 was significantly lower in infants with CF both shortly after diagnosis and at the second test, with no significant difference in rate of increase in FEV0.5 with growth between the two groups. When compared with published reference data, FEV0.5 was reduced by an average of two z scores on both test occasions in those with CF, with 72% of individuals having an FEV0.5 of less than 1.64 z-scores (i.e., less than the fifth percentile) on one or both test occasions. On longitudinal analysis, subjects with CF experienced a mean (95% confidence interval) reduction in FEV0.5 of 20% (11, 28). Airway function is diminished soon after diagnosis in infants with CF and does not catch up during infancy and early childhood. These findings have important implications for early interventions in CF.

Key Words: cystic fibrosis • respiratory function tests • infant • early intervention • forced expiration




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