title: Decreased surfactant phosphatidylcholine synthesis in neonates with congenital diaphragmatic hernia during extracorporeal membrane oxygenation.
abstract: OBJECTIVE
  Congenital diaphragmatic hernia (CDH) may result in severe respiratory insufficiency with a high morbidity. The role of a disturbed surfactant metabolism in the pathogenesis of CDH is unclear. We therefore studied endogenous surfactant metabolism in the most severe CDH patients who required extracorporeal membrane oxygenation (ECMO).
  
  
  METHODS
  Eleven neonates with CDH who required ECMO and ten ventilated neonates without significant lung disease received a 24-h infusion of the stable isotope [U-(13)C] glucose. The (13)C-incorporation into palmitic acid in surfactant phosphatidylcholine (PC) isolated from serial tracheal aspirates was measured. Mean PC concentration in epithelial lining fluid (ELF) was measured during the first 4 days of the study.
  
  
  RESULTS
  Fractional surfactant PC synthesis was decreased in CDH-ECMO patients compared to controls (2.4 +/- 0.33 vs. 8.0 +/- 2.4%/day, p = 0.04). The control group had a higher maximal enrichment (0.18 +/- 0.03 vs. 0.09 +/- 0.02 APE, p = 0.04) and reached this maximal enrichment earlier (46.7 +/- 3.0 vs. 69.4 +/- 6.6 h, p = 0.004) compared to the CDH-ECMO group, which reflects higher and faster precursor incorporation in the control group. Surfactant PC concentration in ELF was similar in both groups.
  
  
  CONCLUSIONS
  These results show that CDH patients who require ECMO have a decreased surfactant PC synthesis, which may be part of the pathogenesis of severe pulmonary insufficiency and has a negative impact on weaning from ECMO.
authors: Department of Pediatrics and Pediatric Surgery, Intensive Care Erasmus MC-Sophia Children's Hospital, P.O. Box 2060, 3000 CB, Rotterdam, The Netherlands. d.janssen@erasmusmc.nl
authors: Daphne J
authors: DJ
authors: Janssen
authors: Luc J
authors: LJ
authors: Zimmermann
authors: Paola
authors: P
authors: Cogo
authors: Aaron
authors: A
authors: Hamvas
authors: Kajsa
authors: K
authors: Bohlin
authors: Ingrid H
authors: IH
authors: Luijendijk
authors: Darcos
authors: D
authors: Wattimena
authors: Virgilio P
authors: VP
authors: Carnielli
authors: Dick
authors: D
authors: Tibboel
mesh_terms: D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D006548:Hernia, Diaphragmatic / Q000378:metabolism* / Q000628:therapy; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010713:Phosphatidylcholines / Q000096:biosynthesis*; D037661:Pulmonary Surfactant-Associated Proteins / Q000096:biosynthesis*
chemical_list: D010713:Phosphatidylcholines; D037661:Pulmonary Surfactant-Associated Proteins