title: Safety and efficacy of perflubron-induced lung growth in neonates with congenital diaphragmatic hernia: Results of a prospective randomized trial.
abstract: BACKGROUND
  Mechanical transduction has been shown to promote fetal lung growth. We examined the safety and efficacy of perflubron-induced lung growth (PILG) in neonates with congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO).
  
  
  METHODS
  Infants with left-sided CDH requiring ECMO were eligible. Exclusion criteria included active air leak, intracranial hemorrhage, major congenital anomalies, and oxygenation index >25 for 24hours. Perflubron was instilled endotracheally and continuous positive airway pressure was applied without ventilation. Survival to discharge was the primary outcome. Daily chest radiographs were used to quantify lung size (the secondary outcome). Midway through the study our institutional practice shifted toward earlier repair of CDH.
  
  
  RESULTS
  Eight infants were randomized to each arm. In the conventional-ventilation arm, six survived to discharge (75%). In the perflubron arm, four survived (50%); the others succumbed to suprasystemic pulmonary hypertension. No adverse events related to perflubron occurred. Within the perflubron group, 4/8 patients had "late repair" (15-19days of life [DOL]) and 4 had "early repair" (2-3 DOL). "Early repair" patients had similar total lung growth, but accelerated growth and shorter ECMO runs.
  
  
  CONCLUSIONS
  PILG is safe in CDH and doubles the total lung size on average (accelerated with early repair). Despite amelioration of pulmonary hypoplasia with PILG, pulmonary hypertension persists.
authors: Section of Pediatric Surgery, Department of Surgery, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, MI. Electronic address: mychalis@med.umich.edu.
authors: George
authors: G
authors: Mychaliska
authors: Section of Pediatric Surgery, Department of Surgery, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Benjamin
authors: B
authors: Bryner
authors: Section of Pediatric Surgery, Department of Surgery, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Ronald
authors: R
authors: Dechert
authors: Section of Pediatric Surgery, Department of Surgery, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Jeannie
authors: J
authors: Kreutzman
authors: Section of Pediatric Surgery, Department of Surgery, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Mike
authors: M
authors: Becker
authors: Section of Pediatric Surgery, Department of Surgery, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Ronald
authors: R
authors: Hirschl
mesh_terms: D015199:Extracorporeal Membrane Oxygenation / Q000401:mortality; D005260:Female; D005466:Fluorocarbons / Q000008:administration & dosage*; D065630:Hernias, Diaphragmatic, Congenital / Q000150:complications* / Q000401:mortality; D006801:Humans; D006842:Hydrocarbons, Brominated; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000187:drug effects* / Q000254:growth & development; D008297:Male; D009929:Organ Size; D011175:Positive-Pressure Respiration / Q000401:mortality; D011446:Prospective Studies; D011859:Radiography
chemical_list: D005466:Fluorocarbons; D006842:Hydrocarbons, Brominated; C003072:perflubron
keywords: Congenital diaphragmatic hernia; Extracorporeal membrane oxygenation(ECMO); Lung development; Mechanotransduction; Perfluorocarbon; Pulmonary hypertension