title: Abnormalities in pulmonary function in infants with high-risk congenital diaphragmatic hernia.
abstract: OBJECTIVE
  The aim of the study was to analyze lung growth and abnormality of infant pulmonary function tests (IPFT) in congenital diaphragmatic hernia (CDH) survivors younger than three years of age with respect to unfavorable prognostic factors.
  
  
  METHODS
  Thirty high-risk CDH survivors at the age of 1.32±0.54 years, body weight 9.76±1.25 kg were examined using IPFT: tidal breathing analysis, baby resistance/compliance, whole baby body plethysmography and rapid thoraco-abdominal compression. Gore-Tex patch was used in 13% of patients (GORE group). Pulmonary hypertension was diagnosed and managed in 13% (iNO group). Standard protocols and appropriate reference values were used and obtained data were statistically analysed.
  
  
  RESULTS
  High incidence of peripheral airway obstruction (70%), increased value of functional residual capacity (FRCp) 191.3±24.5 mL (126.5±36.9 % predicted; P < 0.0005), increased value of effective airway resistance (Reff) 1.71±0.93 kPa.L(-1).s (144.4±80.1 % predicted; P < 0.01) and decreased specific compliance of the respiratory system (Crs/kg) 14.1±2.3 mL.kPa.kg(-1) (i.e., 76.1±20.1 % predicted, P < 0.0005) was noted in infants with CDH in comparison with reference values. Increased value of FRCp was found in GORE group (165.7±51.9 versus 120.4±31.2, P < 0.02) and in iNO group (183.1±52.6 versus 117.8±25.7 mL; P < 0.0005).
  
  
  CONCLUSIONS
  A high incidence of peripheral airway obstruction, an increased value of FRCp and decreased specific compliance of the respiratory system was noted in infants with CDH. Unfavorable prognostic factors (Gore-Tex patch, pulmonary hypertension) correlate with more severe alteration of pulmonary function in infants.
authors.affiliation: Department of Paediatric Surgery, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.
authors.forename: Michal
authors.initials: M
authors.lastname: Rygl
authors.affiliation: Department of Paediatric Surgery, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.
authors.forename: Petra
authors.initials: P
authors.lastname: Rounova
authors.affiliation: Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague.
authors.forename: Jan
authors.initials: J
authors.lastname: Sulc
authors.affiliation: Department of Rehabilitation and Sports Medicine, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague.
authors.forename: Krystof
authors.initials: K
authors.lastname: Slaby
authors.affiliation: Department of Neonatology, 3rd Faculty of Medicine, Charles University in Prague and Institute for the Care for Mother and Child, Prague.
authors.forename: Zbynek
authors.initials: Z
authors.lastname: Stranak
authors.affiliation: Department of Paediatric Surgery, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.
authors.forename: Karel
authors.initials: K
authors.lastname: Pycha
authors.affiliation: Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague.
authors.forename: Tamara
authors.initials: T
authors.lastname: Svobodova
authors.affiliation: Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague.
authors.forename: Petr
authors.initials: P
authors.lastname: Pohunek
authors.affiliation: Department of Paediatric Surgery, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.
authors.forename: Richard
authors.initials: R
authors.lastname: Skaba
mesh_terms: D018153:Czech Republic / Q000453:epidemiology; D005260:Female; D065630:Hernias, Diaphragmatic, Congenital / Q000150:complications / Q000453:epidemiology* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000503:physiopathology*; D007223:Infant; D008168:Lung / Q000503:physiopathology*; D008297:Male; D009017:Morbidity / Q000639:trends; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012307:Risk Factors
keywords: chronic lung morbidity; congenital diaphragmatic hernia; infant pulmonary function testing; lung dysfunction