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血浆血管性血友病因子及含凝血酶原酶解聚蛋白和金属蛋白酶13在评估室间隔缺损患儿术后血管内皮损伤及预后中的应用价值

[Application Value of Plasma vWF and ADAMTS-13 in Evaluating Postoprative Vascular Endothelial Injury and Prognosis in Children with VSD].

作者信息

Wang Xin-Yi, Zhang Qiong-Wen, Shen Yong-Ming, Liu Jun-Feng, Li Wen-Zheng, Jia Ke-Gang

机构信息

Clinical College of Cardiovascular Diseases, Tianjin Medical University, Tianjin 300457, China.

Department of Clinical Laboratory, Tianjin Central Hospital of Gynecology Obstetrics, Tianjin 300457, China.

出版信息

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2023 Feb;31(1):189-196. doi: 10.19746/j.cnki.issn.1009-2137.2023.01.030.

Abstract

OBJECTIVE

To dynamically observe the levels and activities of von Willebrand factor (vWF) and ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) in plasma of children with congenital ventricular septal defect (VSD) during perioperative period, and explore the value of plasma vWF antigen (vWF:Ag) and ADAMTS-13 activity (ADAMTS-13: AC) in evaluating vascular endothelial injury and prognosis in children with VSD.

METHODS

In this cross-sectional study, a total of 74 children with VSD who underwent surgical treatment in TEDA International Cardiovascular Hospital from September 2018 to March 2019 were enrolled in the observation group. Among them, there were 28 cases of pure VSD, 32 cases of VSD combined with pulmonary hypertension, and 14 cases of VSD combined with valvular heart disease. 31 healthy children who underwent physical examination in Tianjin Children's Hospital during the same period were collected as the control group. The biochemical indexes of the children at admission were recorded. Peripheral plasma was collected at admission, postsurgery day 0 and day 1, respectively, and the levels of vWF activity (vWF:AC), vWF:Ag, ADAMTS-13 antigen (ADAMTS-13:Ag) and ADAMTS-13:AC were detected.

RESULTS

The level of plasma vWF:Ag and vWF:AC in the observation group before surgery were significantly lower than those in the control group (<0.001), and increased continuously, on postsurgery day 0 and day 1 (<0.001). The level of ADAMTS-13:Ag in the observation group before surgery was significantly higher than that in the control group (<0.001), which decreased significantly on postsurgery day 0 (<0.001), and increased significantly on postsurgery day 1 compared with postsurgery day 0 (P=0.033). The level of ADAMTS-13:AC in the observation group before surgery was significantly lower than that in the control group (P=0.015), which decreased significantly on postsurgery day 0 (P=0.037), and increased on postsurgery day 1, but the difference was not statistically significant (P=0.051). The changes of vWF and ADAMTS-13 in the three subgroups were basically similar to the observation group. vWF: Ag/ADAMTS-13: AC ratio on postsurgery day 0 and day 1 had high diagnostic value in vascular endothelial injury (AUC=0.80, <0.001; AUC=0.93, <0.001). Preoperative vWF and ADAMTS-13 levels, and related baseline indicators were not correlated with postoperative infection, bleeding, thrombosis,etc.

CONCLUSION

Preoperative vWF: Ag, vWF: AC and ADAMTS-13: AC levels in children with VSD are low, while the level of ADAMTS-13: Ag is high. After surgery, the levels of vWF: Ag and vWF: AC are increased and the level of ADAMTS-13: Ag is decreased. The postoperative vWF: Ag/ADAMTS-13: AC ratio shows high diagnostic value in evaluating vascular endothelial injury. There is no correlation between preoperative vWF and ADAMTS-13 levels with perioperative clinical events.

摘要

目的

动态观察先天性室间隔缺损(VSD)患儿围手术期血浆血管性血友病因子(vWF)及含Ⅰ型血小板反应蛋白基序的去整合素样金属蛋白酶13(ADAMTS - 13)水平及活性,探讨血浆vWF抗原(vWF:Ag)及ADAMTS - 13活性(ADAMTS - 13:AC)在评估VSD患儿血管内皮损伤及预后中的价值。

方法

本横断面研究选取2018年9月至2019年3月在泰达国际心血管病医院接受手术治疗的74例VSD患儿作为观察组。其中单纯VSD 28例,VSD合并肺动脉高压32例,VSD合并瓣膜性心脏病14例。同期在天津市儿童医院体检的31例健康儿童作为对照组。记录患儿入院时的生化指标。分别于入院时、术后第0天和第1天采集外周血浆,检测vWF活性(vWF:AC)、vWF:Ag、ADAMTS - 13抗原(ADAMTS - 13:Ag)及ADAMTS - 13:AC水平。

结果

观察组术前血浆vWF:Ag及vWF:AC水平显著低于对照组(<0.001),术后第0天和第1天持续升高(<0.001)。观察组术前ADAMTS - 13:Ag水平显著高于对照组(<0.001),术后第0天显著降低(<0.001),术后第1天较术后第0天显著升高(P = 0.033)。观察组术前ADAMTS - 13:AC水平显著低于对照组(P = 0.015),术后第0天显著降低(P = 0.037),术后第1天升高,但差异无统计学意义(P = 0.051)。三个亚组vWF和ADAMTS - 13的变化与观察组基本相似。术后第0天和第1天的vWF:Ag/ADAMTS - 13:AC比值对血管内皮损伤具有较高的诊断价值(AUC = 0.80,<0.001;AUC = 0.93,<0.001)。术前vWF和ADAMTS - 13水平及相关基线指标与术后感染、出血、血栓形成等无关。

结论

VSD患儿术前vWF:Ag、vWF:AC及ADAMTS - 13:AC水平较低,而ADAMTS - 13:Ag水平较高。术后vWF:Ag和vWF:AC水平升高,ADAMTS - 13:Ag水平降低。术后vWF:Ag/ADAMTS - 13:AC比值在评估血管内皮损伤方面显示出较高的诊断价值。术前vWF和ADAMTS - 13水平与围手术期临床事件无相关性。

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