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-(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. 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.
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-(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. 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.
Authors: J E Bunt; V P Carnielli; D J Janssen; J L Wattimena; W C Hop; P J Sauer; L J Zimmermann Journal: Crit Care Med Date: 2000-10 Impact factor: 7.598
Authors: Daphne J M T Janssen; Dick Tibboel; Virgilio P Carnielli; Esther van Emmen; Ingrid H T Luijendijk; J L Darcos Wattimena; Luc J I Zimmermann Journal: J Pediatr Date: 2003-03 Impact factor: 4.406
Authors: Jose Luis Peiro; Marc Oria; Emrah Aydin; Rashika Joshi; Nichole Cabanas; Ronny Schmidt; Christoph Schroeder; Mario Marotta; Brian M Varisco Journal: Am J Physiol Lung Cell Mol Physiol Date: 2018-09-27 Impact factor: 5.464
Authors: Massimo Antonelli; Elie Azoulay; Marc Bonten; Jean Chastre; Giuseppe Citerio; Giorgio Conti; Daniel De Backer; François Lemaire; Herwig Gerlach; Goran Hedenstierna; Michael Joannidis; Duncan Macrae; Jordi Mancebo; Salvatore M Maggiore; Alexandre Mebazaa; Jean-Charles Preiser; Jerôme Pugin; Jan Wernerman; Haibo Zhang Journal: Intensive Care Med Date: 2010-02-23 Impact factor: 17.440