title: The evolution of airway function in early childhood following clinical diagnosis of cystic fibrosis.
abstract: This study aimed to investigate the evolution of airway function in infants newly diagnosed with cystic fibrosis (CF). FEV(0.5) 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 FEV(0.5) 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 FEV(0.5) with growth between the two groups. When compared with published reference data, FEV(0.5) was reduced by an average of two z scores on both test occasions in those with CF, with 72% of individuals having an FEV(0.5) 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 FEV(0.5) 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.
authors.affiliation: Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, Centre for Paediatric Epidemiology and Biostatistics, Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. drsarath@clara.net
authors.forename: Sarath C
authors.initials: SC
authors.lastname: Ranganathan
authors.forename: Janet
authors.initials: J
authors.lastname: Stocks
authors.forename: Carol
authors.initials: C
authors.lastname: Dezateux
authors.forename: Andrew
authors.initials: A
authors.lastname: Bush
authors.forename: Angie
authors.initials: A
authors.lastname: Wade
authors.forename: Siobhán
authors.initials: S
authors.lastname: Carr
authors.forename: Rosemary
authors.initials: R
authors.lastname: Castle
authors.forename: Robert
authors.initials: R
authors.lastname: Dinwiddie
authors.forename: Ah-Fong
authors.initials: AF
authors.lastname: Hoo
authors.forename: Sooky
authors.initials: S
authors.lastname: Lum
authors.forename: John
authors.initials: J
authors.lastname: Price
authors.forename: John
authors.initials: J
authors.lastname: Stroobant
authors.forename: Colin
authors.initials: C
authors.lastname: Wallis
mesh_terms: D001827:Body Height; D001835:Body Weight; D016022:Case-Control Studies; D002648:Child; D002657:Child Development / Q000502:physiology*; D002675:Child, Preschool; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy*; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D007223:Infant; D008168:Lung / Q000503:physiopathology*; D008297:Male