Abstract
BackgroundIn school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution. The primary objective of this analysis was to determine the relationships between ventilation distribution outcomes and the presence and extent of structural damage as assessed by chest CT in infants and young children with CF.MethodsData of infants and young children with CF diagnosed following newborn screening consecutively reviewed between August 2005 and December 2009 were analysed. Ventilation distribution (lung clearance index and the first and second moment ratios [LCI, M1/M0 and M2/M0, respectively]), chest CT and airway pathology from bronchoalveolar lavage were determined at diagnosis and then annually. The chest CT scans were evaluated for the presence or absence of bronchiectasis and air trapping.ResultsMatched lung function, chest CT and pathology outcomes were available in 49 infants (31 male) with bronchiectasis and air trapping present in 13 (27%) and 24 (49%) infants, respectively. The presence of bronchiectasis or air trapping was associated with increased M2/M0 but not LCI or M1/M0. There was a weak, but statistically significant association between the extent of air trapping and all ventilation distribution outcomes.ConclusionThese findings suggest that in early CF lung disease there are weak associations between ventilation distribution and lung damage from chest CT. These finding are in contrast to those reported in older children. These findings suggest that assessments of LCI could not be used to replace a chest CT scan for the assessment of structural lung disease in the first two years of life. Further research in which both MBW and chest CT outcomes are obtained is required to assess the role of ventilation distribution in tracking the progression of lung damage in infants with CF.
Highlights
The non-invasive assessment of respiratory function in infants and young children with cystic fibrosis (CF) remains a significant challenge
Reports of chest computed tomography (CT) scans in infants and preschool children with CF are limited; lung damage has been reported to be associated with infection and inflammation [3] and decreased lung function [4]
In infants and young children diagnosed with CF following newborn screening (NBS) we reported the presence of bronchiectasis soon after diagnosis with the presence and extent of lung damage being associated with increasing neutrophilic inflammation and the presence of Pseudomonas aeruginosa [5]
Summary
The non-invasive assessment of respiratory function in infants and young children with cystic fibrosis (CF) remains a significant challenge. Reports of chest CT scans in infants and preschool children with CF are limited; lung damage has been reported to be associated with infection and inflammation [3] and decreased lung function [4]. In infants and young children diagnosed with CF following newborn screening (NBS) we reported the presence of bronchiectasis soon after diagnosis with the presence and extent of lung damage being associated with increasing neutrophilic inflammation and the presence of Pseudomonas aeruginosa [5]. In school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution.
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