title: Evidence of diminished FEV1 and FVC in 6-year-olds followed in the European Cystic Fibrosis Patient Registry, 2007-2009.
abstract: BACKGROUND
  Many infants with cystic fibrosis (CF) exhibit airway inflammation, gas trapping, bronchiectasis, and/or reduced flow, but by age 6 years have forced vital capacities (FVC) and expiratory volumes in 1 second (FEV1) within the variability range of the normal population. We sought evidence of diminished FVC and FEV1 in 6-year-olds with CF.
  
  
  METHODS
  GLI 2012 FVC and FEV1 Z-scores for 6-year-olds from the European CF Patient Registry were plotted against theoretical values from the Normal distribution.
  
  
  RESULTS
  Mean FVC and FEV1 Z-scores for 681 patients (322 females) were -0.43 (SD=1.41) and -0.65 (1.40). Z-scores were consistently lower than expected for the normative population by quantile-quantile plot.
  
  
  CONCLUSIONS
  Diminished FEV1, and to a lesser extent FVC, is found in a large majority of this population, consistent with an established body of evidence that loss of lung function begins early in life for most, if not all, children with CF.
authors: Case Western Reserve University School of Medicine, Cleveland, OH, United States. Electronic address: enigmaster@comcast.net.
authors: Donald R
authors: DR
authors: VanDevanter
authors: David J
authors: DJ
authors: Pasta
mesh_terms: D002648:Child; D003550:Cystic Fibrosis / Q000503:physiopathology*; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008297:Male; D012042:Registries; D014797:Vital Capacity
keywords: Early disease progression; Epidemiology; FEV1; Infants