Pediatric Original

Intensive Care Medicine

, Volume 35, Issue 10, pp 1754-1760

First online:

Open Access This content is freely available online to anyone, anywhere at any time.

Decreased surfactant phosphatidylcholine synthesis in neonates with congenital diaphragmatic hernia during extracorporeal membrane oxygenation

  • Daphne J. JanssenAffiliated withDepartment of Pediatrics and Pediatric Surgery, Intensive Care Erasmus MC-Sophia Children’s Hospital
  • , Luc J. ZimmermannAffiliated withDivision of Neonatology, University Hospital Maastricht
  • , Paola CogoAffiliated withDepartment of Pediatrics, University of Padova
  • , Aaron HamvasAffiliated withDepartment of Pediatrics, Washington University/St. Louis Children’s Hospital
  • , Kajsa BohlinAffiliated withDepartment of Pediatrics, Washington University/St. Louis Children’s HospitalDivision of Pediatrics, Karolinska University Hospital Huddinge and the Karolinska Institute
  • , Ingrid H. LuijendijkAffiliated withDepartment of Pediatrics and Pediatric Surgery, Intensive Care Erasmus MC-Sophia Children’s Hospital
  • , Darcos WattimenaAffiliated withInternal Medicine, Erasmus MC-Dijkzigt
  • , Virgilio P. CarnielliAffiliated withDivision of Neonatology, Children’s Hospital Ancona
  • , Dick TibboelAffiliated withDepartment of Pediatrics and Pediatric Surgery, Intensive Care Erasmus MC-Sophia Children’s Hospital Email author 

Abstract

Purpose

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-13C] glucose. The 13C-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.

Conclusion

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.

Keywords

Surfactant metabolism Lung injury Stable isotopes Surfactant phosphatidylcholine Congenital diaphragmatic hernia