Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening.
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.
Stick
Stephen M
SM
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
Brennan
Siobhain
S
Murray
Conor
C
Douglas
Tonia
T
von Ungern-Sternberg
Britta S
BS
Garratt
Luke W
LW
Gangell
Catherine L
CL
De Klerk
Nicholas
N
Linnane
Barry
B
Ranganathan
Sarath
S
Robinson
Phillip
P
Robertson
Colin
C
Sly
Peter D
PD
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