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

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

doi:10.1016/j.jpeds.2009.05.005

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

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

Stephen M. Stick, MA, MB BChir, PhD
- Princess Margaret Hospital for Children, University of Western Australia, Melbourne, Australia
- TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
- School of Paediatrics and Child Health, University of Western Australia, Melbourne, Australia
- Reprint requests: Stephen Stick, Department of Respiratory Medicine, Princess Margaret Hospital for Children, Box D184, Perth 6010, Western Australia.
Siobhain Brennan, BSc (hons), PhD
- TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
Conor Murray, MBBS, Dip Ch, FRANZCR
- Princess Margaret Hospital for Children, University of Western Australia, Melbourne, Australia
Tonia Douglas, MBChB (hons), FRACP
- Princess Margaret Hospital for Children, University of Western Australia, Melbourne, Australia
- TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
Britta S. von Ungern-Sternberg, MD, DEAA
- Princess Margaret Hospital for Children, University of Western Australia, Melbourne, Australia
Luke W. Garratt, BSc (hons)
- TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
Catherine L. Gangell, BSc (hons), PhD
- TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
Nicholas De Klerk, BSc, MSc, F Ed TC, PhD
- TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
Barry Linnane, MD, PhD
- Perth and Royal Children's Hospital, Melbourne, Australia
Sarath Ranganathan, MB ChB, MRCP, PhD, FRCPCH, FRACP
- Perth and Royal Children's Hospital, Melbourne, Australia
Phillip Robinson, BMed Sc, MBBS, FRACP, MD, PhD
- Perth and Royal Children's Hospital, Melbourne, Australia
Colin Robertson, MBBS, FRACP, MSc (Epi), MD
- Perth and Royal Children's Hospital, Melbourne, Australia
Peter D. Sly, MBBS, FRACP, MD, DSci
- TVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF)
- Additional members of AREST CF are available at www.jpeds.com (Appendix 1).

Received 29 October 2008; received in revised form 19 March 2009; accepted 5 May 2009. published online 21 July 2009.

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.

AREST CF, Australian Respiratory Early Surveillance Team for Cystic Fibrosis, BAL, Bronchoalveolar lavage, BWT, Bronchial wall thickening, CF, Cystic fibrosis, CT, Computed tomography, ESP, Early surveillance program, NBS, Newborn screening

To access this article, please choose from the options below

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.

PII: S0022-3476(09)00474-0

doi:10.1016/j.jpeds.2009.05.005

Refers to article:

Gastric Acid Inhibition for Fat Malabsorption or Gastroesophageal Reflux Disease in Cystic Fibrosis: Longitudinal Effect on Bacterial Colonization and Pulmonary Function , 17 August 2009
Hubert P.J. van der Doef, Hubertus G.M. Arets, Steven P. Froeling, Paul Westers, Roderick H.J. Houwen
The Journal of Pediatrics November 2009 (Vol. 155, Issue 5, Pages 629-633)