title: Lung function over the first 3 years of life in children with congenital diaphragmatic hernia.
abstract: OBJECTIVE
  Infants with congenital diaphragmatic hernia (CDH) have variable degrees of pulmonary hypoplasia at birth. Few reports of lung function over the first years of life exist in this group of children.
  
  
  OBJECTIVE
  Pulmonary function abnormalities correlate with severity of neonatal disease and intensity of neonatal therapies needed. We also hypothesized that longitudinal measurements of lung function over the usual period of rapid lung growth would lend some insight into how the lung remodels in CDH infants.
  
  
  METHODS
  Ninety-eight infants with CDH between 11 days and 44 months of age underwent pulmonary function testing (PFT) on 1-5 occasions using the raised volume rapid thoracic compression technique. Demographic data were also collected.
  
  
  RESULTS
  Forced expiratory flows were below normal. Total lung capacity was normal, but residual volume and functional residual capacity were elevated. Children requiring patch closure, ECMO, or pulmonary vasodilators generally had lower lung functions at follow up. Additionally, longer duration of mechanical ventilation correlated with worse lung function.
  
  
  CONCLUSIONS
  Lung functions of survivors of CDH remain abnormal throughout the first 3 years of life. The degree of pulmonary function impairment correlated both with markers of the initial degree of pulmonary hypoplasia and the duration of mechanical ventilation. Understanding the relationship between the phenotypic presentation of CDH and the potential for subsequent lung growth could help refine both pre- and postnatal therapies to optimize lung growth in CDH infants.
authors.affiliation: Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Howard B
authors.identifier: http://orcid.org/0000-0001-8440-283X
authors.initials: HB
authors.lastname: Panitch
authors.affiliation: Division of Pulmonary Medicine, The Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Weiner
authors.affiliation: Department of Biostatistics and Epidemiology, University of Pennsylvania School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Rui
authors.initials: R
authors.lastname: Feng
authors.affiliation: Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Myrza R
authors.initials: MR
authors.lastname: Perez
authors.affiliation: Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Fiona
authors.initials: F
authors.lastname: Healy
authors.affiliation: Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Joseph M
authors.initials: JM
authors.lastname: McDonough
authors.affiliation: Division of Neonatology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Natalie
authors.initials: N
authors.lastname: Rintoul
authors.affiliation: Department of General Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Holly L
authors.initials: HL
authors.lastname: Hedrick
mesh_terms: D002675:Child, Preschool; D015199:Extracorporeal Membrane Oxygenation / Q000706:statistics & numerical data; D005260:Female; D005500:Follow-Up Studies; D005652:Functional Residual Capacity / Q000502:physiology*; D065630:Hernias, Diaphragmatic, Congenital / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012115:Residual Volume / Q000502:physiology*; D012121:Respiration, Artificial / Q000706:statistics & numerical data; D014109:Total Lung Capacity / Q000502:physiology*; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D014665:Vasodilator Agents
keywords: compensatory lung growth; infant pulmonary function testing; neonatal lung disease; pulmonary hypoplasia