Authors:
Sly PD, Gangell CL, Chen L, Ware RS, Ranganathan S, Mott LS, Murray CP, Stick SM
Authors notes:
New England Journal of Medicine. 2013;368(21):1963-1970
Keywords:
Bronchiectasis, cystic fibrosis, neutrophil elastase, risk factors, children, AREST CF
Abstract:
Bronchiectasis develops early in the course of cystic fibrosis, being detectable in infants as young as 10 weeks of age, and is persistent and progressive.
We sought to determine risk factors for the onset of bronchiectasis, using data collected by the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) intensive surveillance program.
The point prevalence of bronchiectasis at each visit increased from 29.3% at 3 months of age to 61.5% at 3 years of age.
In multivariate analyses, risk factors for bronchiectasis were presentation with meconium ileus, respiratory symptoms at the time of CT and BAL, free neutrophil elastase activity in BAL fluid, and gas trapping on expiratory CT.
Free neutrophil elastase activity in BAL fluid at 3 months of age was associated with persistent bronchiectasis (present on two or more sequential scans), with the odds seven times as high at 12 months of age and four times as high at 3 years of age.
Neutrophil elastase activity in BAL fluid in early life was associated with early bronchiectasis in children with cystic fibrosis.