Elsevier

The Journal of Pediatrics

Volume 155, Issue 5, November 2009, Pages 623-628.e1
The Journal of Pediatrics

Original Article
Bronchiectasis in Infants and Preschool Children Diagnosed with Cystic Fibrosis after Newborn Screening

https://doi.org/10.1016/j.jpeds.2009.05.005Get rights and content

Objectives

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.

Study design

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.

Section snippets

Methods

The study was conducted by the Australian Respiratory Early Surveillance Team for CF (AREST CF) at the Princess Margaret Hospital for Children, Perth and the Royal Children's Hospital, Melbourne, and was approved by the Ethics Committees of both hospitals. Parents of children in the program provided written informed consent.

Cross-sectional data presented here are from a clinic survey of children from Perth and Melbourne who underwent high-resolution CT scanning during the period of August 2005

Results

The population characteristics are shown in Table I. Bronchiectasis and air trapping were commonly observed and easy to recognize in this population of children (Figure 2). Repeatability for reports of bronchiectasis, air trapping and BWT on CT scans were 82.5% (kappa = 0.64), 77.9% (kappa = 0.55) and 61.95% (kappa = 0.237) respectively. Nodules were very uncommon on the CT scans (identified in 7% of children assessed) and were only apparent in children with more severe bronchiectasis,

Discussion

Structural abnormalities of the lung in infants and young children with CF have been reported previously.14, 15, 16, 17 We report the prevalence of CT-defined bronchiectasis in a population of infants and preschool children with CF, diagnosed after NBS. We also report associations between structural changes on CT with airway inflammation and infection with P. aeruginosa. Compared with bronchiectasis and air-trapping the assessment of bronchial-wall thickening had poor repeatability. The poor

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  • Cited by (0)

    This study is supported by Cystic Fibrosis Foundation (USA) (Project grant support), National Health and Medical Research Council (Australia) (Project grant support and Fellowships [S.M.S., P.D.S.]), and) Cystic Fibrosis (Australia) (Project grant support). The authors declare no conflicts of interest.

    Additional members of AREST CF are available at www.jpeds.com (Appendix 1).

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