The online version of this article (doi:10.1186/s12890-015-0023-1) contains supplementary material, which is available to authorized users.
Payal H Mandaliya and Matthew Morten contributed equally to this work.
The authors declare that they have no competing interests.
PHM and MM performed measurements, collected and analysed data, and wrote manuscript. JM conceived, designed and supervised study, analyzed data, wrote manuscript. RK, AJ, AD, VEM, PGG, BW contributed to design of study, interpreted data, and edited manuscript. PR reviewed nitrogen multiple breath washout data, analysed data, and edited manuscript. All authors read and approved the final manuscript.
Congenital thoracic malformations (CTM) are rare lung lesions that are managed with surgical resection or active surveillance.
The objective of this study was to comprehensively assess large and small airway function in children with CTM who underwent lobectomy in early life.
We hypothesise that sensitive measures of lung function will demonstrate residual impairments in CTM compared to healthy children.
Nitrogen lung clearance index (LCI), reactance and resistance (X5Hz and R5Hz), forced expiratory volume in 1 s and forced vital capacity (FEV1 and FVC) 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 17 healthy children (mean age/SD: 4.8/0.4). Total lung capacity (TLC) was also conducted in children older than 7 years of age with CTM (n = 8).
Mean LCI was 8.0 (95% CI 7.5 to 8.5) in the CTM group and 7.3 (95% CI 7.0 to 7.6) in healthy children (p = 0.016). Mean X5Hz was −0.44kPa/l/s (95% CI −0.58 to −0.31) in the CTM group and −0.31kPa/l/s (95% CI −0.35 to −0.27) in healthy children (p = 0.02). Mean Z score for X5Hz was −2.11 (95% CI −3.59 to −0.63) in the CTM group and −0.11 (95% CI −0.55 to 0.33) in healthy children (p = 0.0008). Mean FEV1 was 1.21 L (95% CI 0.97 to 1.45) in the CTM group and 1.02 L (95% CI 0.90 to 1.15) in healthy children (p = 0.22). Mean % predicted FEV1 was 83% (95% CI 74 to 92) in the CTM group and 97% (95% CI 87 to 107) in healthy children (p < 0.05). Mean % predicted TLC in CTM children was 121.3% (95% CI 88.45 to 154.1). 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 = 0.22, p = 0.4).
Children with CTM have impaired lung function as demonstrated by the significant differences in LCI, reactance and FEV1 but not FVC, resistance and TLC. These findings may be of clinical relevance as ventilation inhomogeneities are closely correlated with somatic growth in this study.
Additional file 1: Table S1. ISAAC questionnaire comparing the respiratory health of CTM v healthy children. Table S2. Details on the lung function of the healthy cohort of children.12890_2015_23_MOESM1_ESM.docx
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- Ventilation inhomogeneities in children with congenital thoracic malformations
Payal H Mandaliya
Vanessa E Murphy
Peter G Gibson
- BioMed Central
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