title: Ventilation inhomogeneities in children with congenital thoracic malformations.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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).
  
  
  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 7.6) 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Experimental & Translational Respiratory Medicine, Hunter Medical Research Institute, University of Newcastle, Newcastle, NSW, Australia. Payal.Mandaliya@hnehealth.nsw.gov.au.|Paediatric Respiratory and Sleep Medicine Department, Newcastle Children's Hospital, Newcastle, NSW, Australia. Payal.Mandaliya@hnehealth.nsw.gov.au.
authors.forename: Payal H
authors.initials: PH
authors.lastname: Mandaliya
authors.affiliation: Experimental & Translational Respiratory Medicine, Hunter Medical Research Institute, University of Newcastle, Newcastle, NSW, Australia. Matthew.Morten@uon.edu.au.
authors.forename: Matthew
authors.initials: M
authors.lastname: Morten
authors.affiliation: Paediatric Surgery Department, Newcastle Children's Hospital, Newcastle, NSW, Australia. Rajendra.Kumar@hnehealth.nsw.gov.au.
authors.forename: Rajendra
authors.initials: R
authors.lastname: Kumar
authors.affiliation: Surgery Department, John Hunter Hospital, Newcastle, NSW, Australia. Alan.James@hnehealth.nsw.gov.au.
authors.forename: Alan
authors.initials: A
authors.lastname: James
authors.affiliation: Paediatric Surgery Department, Newcastle Children's Hospital, Newcastle, NSW, Australia. Aniruddh.Deshpande1@hnehealth.nsw.gov.au.
authors.forename: Aniruddh
authors.initials: A
authors.lastname: Deshpande
authors.affiliation: Hunter Medical Research Institute, University of Newcastle, Newcastle, NSW, Australia. vanessa.murphy@newcastle.edu.au.
authors.forename: Vanessa E
authors.initials: VE
authors.lastname: Murphy
authors.affiliation: Hunter Medical Research Institute, University of Newcastle, Newcastle, NSW, Australia. Peter.Gibson@hnehealth.nsw.gov.au.
authors.forename: Peter G
authors.initials: PG
authors.lastname: Gibson
authors.affiliation: Paediatric Respiratory and Sleep Medicine Department, Newcastle Children's Hospital, Newcastle, NSW, Australia. Bruce.Whitehead@hnehealth.nsw.gov.au.
authors.forename: Bruce
authors.initials: B
authors.lastname: Whitehead
authors.affiliation: Paediatric Respiratory Department, Children's Hospital at Westmead, Sydney, NSW, Australia. paul.robinson1@health.nsw.gov.au.
authors.forename: Paul
authors.initials: P
authors.lastname: Robinson
authors.affiliation: Experimental & Translational Respiratory Medicine, Hunter Medical Research Institute, University of Newcastle, Newcastle, NSW, Australia. joerg.mattes@newcastle.edu.au.|Paediatric Respiratory and Sleep Medicine Department, Newcastle Children's Hospital, Newcastle, NSW, Australia. joerg.mattes@newcastle.edu.au.
authors.forename: Joerg
authors.initials: J
authors.lastname: Mattes
mesh_terms: D001944:Breath Tests; D001994:Bronchogenic Cyst / Q000503:physiopathology* / Q000601:surgery; D001998:Bronchopulmonary Sequestration / Q000503:physiopathology* / Q000601:surgery; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000503:physiopathology* / Q000601:surgery; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D008168:Lung / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D008297:Male; D009584:Nitrogen / Q000032:analysis; D011013:Pneumonectomy; D011656:Pulmonary Emphysema / Q000151:congenital* / Q000503:physiopathology / Q000601:surgery; D012123:Pulmonary Ventilation / Q000502:physiology*; D012129:Respiratory Function Tests; D014109:Total Lung Capacity / Q000502:physiology; D014797:Vital Capacity / Q000502:physiology*
chemical_list: D009584:Nitrogen