Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening

Stephen M Stick; Siobhain Brennan; Conor Murray; Tonia Douglas; Britta S von Ungern-Sternberg; Luke W Garratt; Catherine L Gangell; Nicholas De Klerk; Barry Linnane; Sarath Ranganathan; Phillip Robinson; Colin Robertson; Peter D Sly; Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF)

doi:10.1016/j.jpeds.2009.05.005

Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening

J Pediatr. 2009 Nov;155(5):623-8.e1. doi: 10.1016/j.jpeds.2009.05.005. Epub 2009 Jul 19.

Authors

Stephen M Stick¹, Siobhain Brennan, Conor Murray, Tonia Douglas, Britta S von Ungern-Sternberg, Luke W Garratt, Catherine L Gangell, Nicholas De Klerk, Barry Linnane, Sarath Ranganathan, Phillip Robinson, Colin Robertson, Peter D Sly; Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF)

Collaborators

Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF):
Elizabeth Balding, Luke J Berry, Claudia Calogero, John B Carlin, Rosemary Carzino, Graham L Hall, Anthony Kicic, Ingrid A Laing, Karla M Logie, John Massie, Lauren S Mott, David Mullane, Gary Nolan, Naveen Pillarisetti, Srinivas R Poreddy, Roy Robins-Browne, Billy Skoric, Erika N Sutanto, Elizabeth Williamson

Affiliation

¹ 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

PMID: 19616787
DOI: 10.1016/j.jpeds.2009.05.005

Abstract

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.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Age Distribution
Bronchiectasis / diagnosis
Bronchiectasis / epidemiology*
Bronchoalveolar Lavage Fluid / cytology*
Child, Preschool
Comorbidity
Confidence Intervals
Cystic Fibrosis / diagnosis
Cystic Fibrosis / epidemiology*
Female
Humans
Infant
Infant, Newborn
Male
Neonatal Screening*
Prevalence
Probability
Prognosis
Respiratory Function Tests
Risk Assessment
Severity of Illness Index
Sex Distribution
Tomography, X-Ray Computed