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Published ahead of print on January 30, 2004, doi:10.1164/rccm.200309-1344OC

Am. J. Respir. Crit. Care Med., Volume 169, Number 8, April 2004, 928-933

A more recent version of this article appeared on April 15, 2004
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Submitted on September 30, 2003
Accepted on January 25, 2004

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

Sarath C Ranganathan1*, Janet Stocks1, Carol Dezateux2, Andrew Bush3, Angie Wade2, Siobhan Carr4, Rosemary Castle1, Robert Dinwiddie5, Ah-Fong Hoo6, Sooky Lum6, John Price7, John Stroobant8, Colin Wallis5, and The London Collaborative Cystic Fibrosis Group

1 Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, Institute of Child Health, London, United Kingdom, 2 Centre for Paediatric Epidemiology and Biostatistics, Institute of Child Health, London, United Kingdom, 3 Paediatric Respiratory Medicine, Royal Brompton Hospital, London, United Kingdom, 4 Child Health, Royal London Hospital, London, United Kingdom, 5 Paediatric Respiratory Medicine, Great Ormond Street Hospital, London, United Kingdom, 6 Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, Institute of Child Health, London, United Kingdom; Neonatal Unit, Homerton University Hospital, London, United Kingdom, 7 Child Health, King's College Hospital, London, United Kingdom, 8 Child Health, University Hospital Lewisham, London, United Kingdom

* To whom correspondence should be addressed. 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 28 weeks) and six months later in subjects with CF, and on two occasions six months apart (median age 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 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 2 z scores on both test occasions in those with CF, with 72% of individuals having an FEV0.5 <1.64 z-scores (i.e. <5th percentile) on one or both test occasions. On longitudinal analysis, subjects with CF experienced a mean (95% CI) 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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