title: Effect of Perflubron-induced lung growth on pulmonary vascular remodeling in congenital diaphragmatic hernia.
abstract: OBJECTIVE
  Congenital diaphragmatic hernia (CDH) involves lung hypoplasia and pulmonary hypertension (PH). Post-natal Perflubron ventilation induces lung growth. This phenomenon is called Perflubon-induced lung growth (PILG). However, it does not appear to ameliorate PH in CDH. We aim to determine the effect of PILG on pulmonary vascular remodeling in neonates with CDH and PH requiring extracorporeal membrane oxygenation (ECMO).
  
  
  METHODS
  Lung tissue from four patients was obtained, three treated with PILG + ECMO, and one maintained on conventional ventilation + ECMO (control). The distribution of collagen was assessed with Masson's trichrome stain. Immunohistochemistry was done to assess cell proliferation and immunofluorescence to assess vascular morphology.
  
  
  RESULTS
  Comparing PILG vs. control, there was an increase in vessel wall diameter (6.85 μm, 10.28 μm, and 10.35 μm vs. 4.34 μm), increase in collagen thickness in two PILG patients (35.66 μm, 14.23 μm, and 38.46 μm vs. 22.16 μm), and decrease in lumen diameter despite similar total area (48.99 μm, 41.74 μm, and 36.32 μm vs. 51.56 μm) for each PILG patient vs. the control patient, respectively.
  
  
  CONCLUSIONS
  PILG does not appear to improve pulmonary vascular remodeling that occurs with PH. The findings are descriptive and will require larger samples to validate the significance of the findings. Overall, further studies will be required to identify the mechanistic causes of PH in CDH to create effective treatments.
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, University of North Carolina, Chapel Hill, NC, 27599, USA.
authors.forename: Mansi
authors.initials: M
authors.lastname: Shah
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, University of North Carolina, Chapel Hill, NC, 27599, USA.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Phillips
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, University of North Carolina, Chapel Hill, NC, 27599, USA.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Bryner
authors.affiliation: University of Michigan, Ann Arbor, MI, USA.
authors.forename: Ronald B
authors.initials: RB
authors.lastname: Hirschl
authors.affiliation: University of Michigan, Ann Arbor, MI, USA.
authors.forename: George B
authors.initials: GB
authors.lastname: Mychaliska
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, University of North Carolina, Chapel Hill, NC, 27599, USA. sean_mclean@med.unc.edu.
authors.forename: Sean E
authors.initials: SE
authors.lastname: McLean
mesh_terms: D015199:Extracorporeal Membrane Oxygenation / Q000379:methods*; D005260:Female; D005466:Fluorocarbons / Q000494:pharmacology*; D065630:Hernias, Diaphragmatic, Congenital / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D006842:Hydrocarbons, Brominated; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000098:blood supply / Q000000981:diagnostic imaging / Q000187:drug effects*; D008297:Male; D011651:Pulmonary Artery / Q000187:drug effects / Q000503:physiopathology*; D066253:Vascular Remodeling
chemical_list: D005466:Fluorocarbons; D006842:Hydrocarbons, Brominated; C003072:perflubron
keywords: Congenital diaphragmatic hernia; Extracorporeal membrane oxygenation (ECMO); Perflubron; Pulmonary hypertension; Pulmonary vascular remodeling