Decreased surfactant phosphatidylcholine synthesis in neonates with congenital diaphragmatic hernia during extracorporeal membrane oxygenation.
Decreased surfactant phosphatidylcholine synthesis in neonates with congenital diaphragmatic hernia during extracorporeal membrane oxygenation.
复制标题
先天性膈疝新生儿在体外膜氧合过程中表面活性剂磷脂酰胆碱合成减少。
DOI：
10.1007/s00134-009-1564-7
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发表时间：
2009
影响因子：
38.9
通讯作者：
Tibboel,Dick
中科院分区：
文献类型：
--
作者：
Janssen,DaphneJ;Zimmermann,LucJ;Cogo,Paola;Hamvas,Aaron;Bohlin,Kajsa;Luijendijk,IngridH;Wattimena,Darcos;Carnielli,VirgilioP;Tibboel,Dick
目的先天性腹股沟疝（CDH）可导致严重的呼吸功能不全，发病率高。表面活性物质代谢紊乱在CDH发病机制中的作用尚不清楚。因此，我们研究了内源性表面活性物质代谢的最严重的CDH患者谁需要体外膜肺氧合（ECMO）。MethodsEleven新生儿CDH谁需要ECMO和10个通气的新生儿没有显着的肺部疾病接受了24小时输注的稳定同位素[U-13 C]葡萄糖。测定了从连续气管吸出物中分离的表面活性剂磷脂酰胆碱（PC）中13 C掺入棕榈酸的量。平均PC浓度在上皮衬里液（ELF）测定在第一个4 days of the study.ResultsFractional表面活性剂PC合成减少CDH-ECMO患者相比，对照组（2.4 ± 0.33与8.0 ± 2.4%/天，p= 0.04）。与CDH-ECMO组相比，对照组具有更高的最大富集（0.18 ± 0.03 vs. 0.09 ± 0.02 APE，p= 0.04），并且更早达到该最大富集（46.7 ± 3.0 vs. 69.4 ± 6.6 h，p= 0.004），这反映了对照组中更高和更快的前体掺入。在ELF中的表面活性剂PC浓度是相似的，在两个group.ConclusionThese结果表明，CDH患者谁需要ECMO有一个减少表面活性剂PC合成，这可能是严重肺功能不全的发病机制的一部分，并有一个负面影响，从ECMO脱机。
PurposeCongenital 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).MethodsEleven 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. The13C-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.ResultsFractional 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.ConclusionThese 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.