title: Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age?
abstract: BACKGROUND
  Sensitive outcome measures applicable in different centres to quantify and track early pulmonary abnormalities in infants with cystic fibrosis (CF) are needed both for clinical care and interventional trials. Chest CT has been advocated as such a measure yet there is no validated scoring system in infants.
  
  
  OBJECTIVE
  The objectives of this study were to standardise CT data collection across multiple sites; ascertain the incidence of bronchial dilatation and air trapping in newborn screened (NBS) infants with CF at 1 year; and assess the reproducibility of Brody-II, the most widely used scoring system in children with CF, during infancy.
  
  
  METHODS
  A multicentre observational study of early pulmonary lung disease in NBS infants with CF at age 1 year using volume-controlled chest CT performed under general anaesthetic.
  
  
  RESULTS
  65 infants with NBS-diagnosed CF had chest CT in three centres. Small insignificant variations in lung recruitment manoeuvres but significant centre differences in radiation exposures were found. Despite experienced scorers and prior training, with the exception of air trapping, inter- and intraobserver agreement on Brody-II score was poor to fair (eg, interobserver total score mean (95% CI) κ coefficient: 0.34 (0.20 to 0.49)). Only 7 (11%) infants had a total CT score ≥ 12 (ie, ≥ 5% maximum possible) by either scorer.
  
  
  CONCLUSIONS
  In NBS infants with CF, CT changes were very mild at 1 year, and assessment of air trapping was the only reproducible outcome. CT is thus of questionable value in infants of this age, unless an improved scoring system for use in mild CF disease can be developed.
authors.affiliation: Portex Unit: Respiratory Physiology and Medicine, UCL Institute of Child Health, , London, UK.
authors.forename: Lena P
authors.initials: LP
authors.lastname: Thia
authors.forename: Alistair
authors.initials: A
authors.lastname: Calder
authors.forename: Janet
authors.initials: J
authors.lastname: Stocks
authors.forename: Andrew
authors.initials: A
authors.lastname: Bush
authors.forename: Catherine M
authors.initials: CM
authors.lastname: Owens
authors.forename: Colin
authors.initials: C
authors.lastname: Wallis
authors.forename: Carolyn
authors.initials: C
authors.lastname: Young
authors.forename: Yvonne
authors.initials: Y
authors.lastname: Sullivan
authors.forename: Angie
authors.initials: A
authors.lastname: Wade
authors.forename: Angus
authors.initials: A
authors.lastname: McEwan
authors.forename: Alan S
authors.initials: AS
authors.lastname: Brody
mesh_terms: D003550:Cystic Fibrosis / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000000981:diagnostic imaging*; D008297:Male; D015997:Neonatal Screening / Q000379:methods*; D015203:Reproducibility of Results; D014057:Tomography, X-Ray Computed / Q000379:methods*