title: Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening.
abstract: OBJECTIVE
  To determine the prevalence of bronchiectasis in young children with cystic fibrosis (CF) diagnosed after newborn screening (NBS) and the relationship of bronchiectasis to pulmonary inflammation and infection.
  
  
  METHODS
  Children were diagnosed with CF after NBS. Computed tomography and bronchoalveolar lavage were performed with anesthesia (n = 96). Scans were analyzed for the presence and extent of abnormalities.
  
  
  RESULTS
  The prevalence of bronchiectasis was 22% and increased with age (P = .001). Factors associated with bronchiectasis included absolute neutrophil count (P = .03), neutrophil elastase concentration (P = .001), and Pseudomonas aeruginosa infection (P = .03).
  
  
  CONCLUSIONS
  Pulmonary abnormalities are common in infants and young children with CF and relate to neutrophilic inflammation and infection with P. aeruginosa. Current models of care for infants with CF fail to prevent respiratory sequelae. Bronchiectasis is a clinically relevant endpoint that could be used for intervention trials that commence soon after CF is diagnosed after NBS.
authors.affiliation: Department of Respiratory Medicine, Princess Margaret Hospital for Children, School of Paediatrics and Child Health, University of Western Australia, Melbourne, Australia. stephen.stick@health.wa.gov.au
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Stick
authors.forename: Siobhain
authors.initials: S
authors.lastname: Brennan
authors.forename: Conor
authors.initials: C
authors.lastname: Murray
authors.forename: Tonia
authors.initials: T
authors.lastname: Douglas
authors.forename: Britta S
authors.initials: BS
authors.lastname: von Ungern-Sternberg
authors.forename: Luke W
authors.initials: LW
authors.lastname: Garratt
authors.forename: Catherine L
authors.initials: CL
authors.lastname: Gangell
authors.forename: Nicholas
authors.initials: N
authors.lastname: De Klerk
authors.forename: Barry
authors.initials: B
authors.lastname: Linnane
authors.forename: Sarath
authors.initials: S
authors.lastname: Ranganathan
authors.forename: Phillip
authors.initials: P
authors.lastname: Robinson
authors.forename: Colin
authors.initials: C
authors.lastname: Robertson
authors.forename: Peter D
authors.initials: PD
authors.lastname: Sly
mesh_terms: D017677:Age Distribution; D001987:Bronchiectasis / Q000175:diagnosis / Q000453:epidemiology*; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology*; D002675:Child, Preschool; D015897:Comorbidity; D016001:Confidence Intervals; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000453:epidemiology*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D015997:Neonatal Screening; D015995:Prevalence; D011336:Probability; D011379:Prognosis; D012129:Respiratory Function Tests; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D014057:Tomography, X-Ray Computed