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dc.contributor.authorSly, Peter D
dc.contributor.authorGangell, Catherine L
dc.contributor.authorChen, Linping
dc.contributor.authorWare, Robert S
dc.contributor.authorRanganathan, Sarath
dc.contributor.authorMott, Lauren S
dc.contributor.authorMurray, Conor P
dc.contributor.authorStick, Stephen M
dc.date.accessioned2018-09-04T04:19:02Z
dc.date.available2018-09-04T04:19:02Z
dc.date.issued2013
dc.identifier.issn0028-4793
dc.identifier.doi10.1056/NEJMoa1301725
dc.identifier.urihttp://hdl.handle.net/10072/172347
dc.description.abstractBackground: 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. Methods: 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. Results: 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.)
dc.description.peerreviewedYes
dc.languageEnglish
dc.language.isoeng
dc.publisherMassachusetts Medical Society
dc.relation.ispartofpagefrom1963
dc.relation.ispartofpageto1970
dc.relation.ispartofissue21
dc.relation.ispartofjournalNew England Journal of Medicine
dc.relation.ispartofvolume368
dc.subject.fieldofresearchClinical Sciences not elsewhere classified
dc.subject.fieldofresearchMedical and Health Sciences
dc.subject.fieldofresearchcode110399
dc.subject.fieldofresearchcode11
dc.titleRisk factors for bronchiectasis in children with cystic fibrosis
dc.typeJournal article
dc.type.descriptionC1 - Articles
dc.type.codeC - Journal Articles
dc.description.versionAccepted Manuscript (AM)
gro.rights.copyright© 2013 Massachusetts Medical Society. The attached file is reproduced here in accordance with the copyright policy of the publisher. Please refer to the journal's website for access to the definitive, published version.
gro.hasfulltextFull Text
gro.griffith.authorWare, Robert


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