Bronchiectasis in Infants and Preschool Children Diagnosed with Cystic Fibrosis after Newborn Screening
Received 29 October 2008; received in revised form 19 March 2009; accepted 5 May 2009. published online 21 July 2009.
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) Abstract |
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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.
aPrincess Margaret Hospital for Children, University of Western Australia, Melbourne, Australia
bTVW Institute for Child Health Research, University of Western Australia, Melbourne, Australia
cSchool of Paediatrics and Child Health, University of Western Australia, Melbourne, Australia
dPerth and Royal Children's Hospital, Melbourne, Australia
Reprint requests: Stephen Stick, Department of Respiratory Medicine, Princess Margaret Hospital for Children, Box D184, Perth 6010, Western Australia.
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.
ABSTRACT