PT  - JOURNAL ARTICLE
AU  - Matthew Morten
AU  - Payal Mandaliya
AU  - Rajendra Kumar
AU  - Alan James
AU  - Aniruddh Deshpande
AU  - Vanessa Murphy
AU  - Peter Gibson
AU  - Bruce Whitehead
AU  - Paul Robinson
AU  - Joerg Mattes
TI  - Late-breaking abstract: Ventilation inhomogeneities in children with congenital thoracic malformations
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - 2934
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/2934.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/2934.full
SO  - Eur Respir J2014 Sep 01; 44
AB  - Background:Congenital thoracic malformations (CTM) are rare lung lesions that are managed with surgical resection or active surveillance.Aims and objectives:To assess large and small airway function in children with CTM by utilising pulmonary function tests that are better suited for the sensitive measure of disease in children.We hypothesise that more sensitive measures of peripheral airways will more likely demonstrate adverse pulmonary function in post-operative CTM children.Methods:Nitrogen lung clearance index (LCI), reactance and resistance (X5Hz and R5Hz) and forced expiratory volume in 1 s (FEV1) were prospectively measured in 10 children with CTM (mean age/SD: 7.6/1.3) who had undergone surgical resection in early life and in 16 healthy children (mean age/SD: 4.8/0.4).Results:Mean LCI was 8.0 (95% CI 7.5 to 8.5) in the CTM group and 7.3 (95% CI 7.0 to 7.8) in healthy children (p=0.02). Mean % predicted X5Hz was 175% (95% CI 122 to 228) in the CTM group and 104% (95% CI 89.4 to 118.3) in healthy children (p=0.001). Mean % predicted FEV1 was 83% (95% CI 74 to 92) in the CTM group and 98% (95% CI 87 to 108) in healthy children (p=0.043). Mean LCI was inversely correlated with height z-scores in the CTM group (rs=-0.88, p=0.002) but not in healthy children (rs=-0.19, p=0.49).Conclusions:Children with CTM appear to have impaired lung function as demonstrated by the significant differences in X5Hz and LCI scores. These findings are of clinical significance as ventilation inhomogeneities are closely correlated with somatic growth.