Risk factors for bronchiectasis in children with cystic fibrosis

Sly, Peter D., Gangell, Catherine L., Chen, Linping, Ware, Robert S., Ranganathan, Sarath, Mott, Lauren S., Murray, Conor P. and Stick, Stephen M. (2013) Risk factors for bronchiectasis in children with cystic fibrosis. New England Journal of Medicine, 368 21: 1963-1970. doi:10.1056/NEJMoa1301725

Author Sly, Peter D.
Gangell, Catherine L.
Chen, Linping
Ware, Robert S.
Ranganathan, Sarath
Mott, Lauren S.
Murray, Conor P.
Stick, Stephen M.
Title Risk factors for bronchiectasis in children with cystic fibrosis
Journal name New England Journal of Medicine   Check publisher's open access policy
ISSN 0028-4793
Publication date 2013-05
Year available 2013
Sub-type Article (original research)
DOI 10.1056/NEJMoa1301725
Volume 368
Issue 21
Start page 1963
End page 1970
Total pages 8
Place of publication Boston, MA United States
Publisher Massachusetts Medical Society
Collection year 2014
Language eng
Formatted abstract
Background: 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.

We examined data from 127 consecutive infants who received a diagnosis of cystic fibrosis after newborn screening. Chest computed tomography (CT) and bronchoalveolar lavage (BAL) were performed, while the children were in stable clinical condition, at 3 months and 1, 2, and 3 years of age. Longitudinal data were used to determine risk factors associated with the detection of bronchiectasis from 3 months to 3 years of age.

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 (odds ratio, 3.17; 95% confidence interval [CI], 1.51 to 6.66; P = 0.002), respiratory symptoms at the time of CT and BAL (odds ratio, 2.27; 95% CI, 1.24 to 4.14; P = 0.008), free neutrophil elastase activity in BAL fluid (odds ratio, 3.02; 95% CI, 1.70 to 5.35; P<0.001), and gas trapping on expiratory CT (odds ratio, 2.05; 95% CI, 1.17 to 3.59; P = 0.01). 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.

Conclusions: Neutrophil elastase activity in BAL fluid in early life was associated with early bronchiectasis in children with cystic fibrosis. (Funded by the National Health and Medical Research Council of Australia and Cystic Fibrosis Foundation Therapeutics.)
Keyword Resolution Computed Tomography
Lower Airway Inflammation
Structural Lung Injury
Pulmonary Inflammation
Young Children
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ

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