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在小儿体外循环心脏手术中,C1酯酶抑制剂在补体系统激活过程中起着至关重要的作用。

C1 esterase inhibitor in pediatric cardiac surgery with cardiopulmonary bypass plays a vital role in activation of the complement system.

作者信息

Miyamoto Takashi, Ozaki Shinichi, Inui Akitoshi, Tanaka Yuki, Yamada Yoshiyuki, Matsumoto Naoki

机构信息

Department of Cardiovascular Surgery, Gunma Children's Medical Center, 779 Shimohakoda, Hokkitsu, Shibukawa, Gunma, 377-8577, Japan.

Department of Allergy and Immunology, Gunma Children's Medical Center, Gunma, Japan.

出版信息

Heart Vessels. 2020 Jan;35(1):46-51. doi: 10.1007/s00380-019-01466-2. Epub 2019 Jul 5.

DOI:10.1007/s00380-019-01466-2
PMID:31278424
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7223185/
Abstract

Our prospective study was therefore designed to determine which part of the systemic inflammatory response after cardiac operations resulted from Cardiopulmonary bypass (CPB) in neonates and infants. After approval by the human ethical committee of the Gunma Children's Medical Center (GCMC) and informed consent of the parents, 40 consecutive term congenital heart disease patients aged until 1 year who underwent long CPB time (> 3 h) at surgery were included in the prospective study between January 2012 and December 2014. C1 esterase inhibitor (C1-inh) drug (@Berinert) was generously provided by CSL Behring (King of Prussia, PA). The C1-inh (20 IU/kg) was given intravenously 60 min after CPB. Blood samples for complement factors were obtained before and 48 h after administration of C1-inh. Six patients did not survive and their data were not included. Of 34 patients included, median age was 6.5 months, median body weight was 6050 g, and 16 (47%) were female. According to the Mann-Whitney U test, there were no differences between the two groups concerning demographic and intraoperative data, postoperative chemical data. C1q concentration was only significant lower in patients with C1-inh non-treated group than in patients with C1-inh treated group. But, the consumption of C1q, C3, C4, CH, and C1-inh in patients with C1-inhibitor non-treated group was observed early postoperatively. There is a significant difference in the values before and after C1-inh treatment between the two groups. The lower value in the C1-inh-treated group is explained by the activation of the classical pathway through the replenishment of complements by C1-inh treatment. This study proposes the administration of C1-inh is an effective therapy to reduce the activation and improve the clinical capillary leak syndrome.

摘要

因此,我们的前瞻性研究旨在确定新生儿和婴儿心脏手术后全身炎症反应的哪一部分是由体外循环(CPB)引起的。在获得群马儿童医学中心(GCMC)人类伦理委员会的批准并得到家长的知情同意后,2012年1月至2014年12月期间,40例年龄在1岁以下、手术中接受长时间CPB(>3小时)的足月先天性心脏病患者被纳入该前瞻性研究。C1酯酶抑制剂(C1-inh)药物(@Berinert)由CSL Behring(宾夕法尼亚州普鲁士王)慷慨提供。在CPB后60分钟静脉注射C1-inh(20 IU/kg)。在给予C1-inh之前和之后48小时采集用于检测补体因子的血样。6例患者未存活,其数据未纳入。在纳入的34例患者中,中位年龄为6.5个月,中位体重为6050克,16例(47%)为女性。根据曼-惠特尼U检验,两组在人口统计学和术中数据、术后化学数据方面没有差异。C1q浓度仅在未治疗C1-inh组患者中显著低于治疗C1-inh组患者。但是,在未治疗C1抑制剂组患者中,术后早期观察到C1q、C3、C4、CH和C1-inh的消耗。两组之间C1-inh治疗前后的值存在显著差异。C1-inh治疗组中较低的值可通过C1-inh治疗补充补体从而激活经典途径来解释。本研究提出给予C1-inh是一种有效的治疗方法,可减少激活并改善临床毛细血管渗漏综合征。

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本文引用的文献

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Pediatr Radiol. 2014 Mar;44(3):258-64. doi: 10.1007/s00247-013-2816-9. Epub 2013 Dec 21.
2
Early prediction of capillary leak syndrome in infants after cardiopulmonary bypass.体外循环后婴儿毛细血管渗漏综合征的早期预测。
Eur J Cardiothorac Surg. 2013 Aug;44(2):275-81. doi: 10.1093/ejcts/ezt028. Epub 2013 Feb 6.
3
The effect of C1 inhibitor on myocardial ischemia and reperfusion injury.C1 抑制剂对心肌缺血再灌注损伤的影响。
Cardiovasc Pathol. 2013 Jan-Feb;22(1):75-80. doi: 10.1016/j.carpath.2012.05.003. Epub 2012 Jun 16.
4
Perioperative serum interleukins in neonates with hypoplastic left-heart syndrome and transposition of the great arteries.患有左心发育不全综合征和大动脉转位的新生儿围手术期血清白细胞介素
J Cardiothorac Vasc Anesth. 2007 Apr;21(2):184-90. doi: 10.1053/j.jvca.2006.02.027. Epub 2006 May 30.
5
Complement factor 1 inhibitor improves cardiopulmonary function in neonatal cardiopulmonary bypass.补体因子1抑制剂可改善新生儿体外循环中的心肺功能。
Ann Thorac Surg. 2007 Apr;83(4):1477-82; discussion 1483. doi: 10.1016/j.athoracsur.2006.10.049.
6
Administration of C1 inhibitor reduces neutrophil activation in patients with sepsis.给予C1抑制剂可降低脓毒症患者的中性粒细胞活化。
Clin Diagn Lab Immunol. 2003 Jul;10(4):529-35. doi: 10.1128/cdli.10.4.529-535.2003.
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Production of proinflammatory cytokines and myocardial dysfunction after arterial switch operation in neonates with transposition of the great arteries.大动脉转位新生儿动脉调转术后促炎细胞因子的产生与心肌功能障碍
J Thorac Cardiovasc Surg. 2002 Oct;124(4):811-20. doi: 10.1067/mtc.2002.122308.
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9
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10
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