Journal article
Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening
PD Sly, S Brennan, C Gangell, N De Klerk, C Murray, L Mott, SM Stick, PJ Robinson, CF Robertson, SC Ranganathan
American Journal of Respiratory and Critical Care Medicine | AMER THORACIC SOC | Published : 2009
Abstract
Rationale: The promise of newborn screening (NBS) for cystic fibrosis (CF) has not been fully realized, and the extent of improvement in respiratory outcomes is unclear. We hypothesized that significant lung disease was present at diagnosis. Objectives: To determine the extent of lung disease in a geographically defined population of infants with CF diagnosed after detection by NBS. Methods: Fifty-seven infants (median age, 3.6 mo) with CF underwent bronchoalveolar lavage and chest computed tomography (CT) using a three-slice inspiratory and expiratory protocol. Measurements and Main Results: Despite the absence of respiratory symptomsin 48 (84.2%)of infants, a substantial proportion had lun..
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Funding Acknowledgements
Supported by the Cysfic Fibrosis Foundation Trust, Inc.; the National Health and Medical Research Council; the Australian Cystic Fibrosis Research Trust; and The Murdoch Children's Research Institute and Princess Margaret Hospital Foundation.