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先天性心脏病患儿的血栓形成-出血倾向

Thrombo-hemorrhagic liability in children with congenital heart diseases.

作者信息

Shebl Shebl Said, El-Shehaby Walid Ahmed Naguib, Said Yasmin Shebl, Darwish Amira Hamed, Elfadaly Nabeh Helal, Amer Eman

机构信息

Pediatrics Department, Faculty of Medicine, Tanta University, Egypt.

Clinical Pathology Department, Faculty of Medicine, Tanta University, Tanta, Egypt.

出版信息

Hematol Oncol Stem Cell Ther. 2018 Sep;11(3):123-128. doi: 10.1016/j.hemonc.2017.07.001. Epub 2017 Aug 26.

Abstract

BACKGROUND

The precise mechanisms of the increased incidence of hemostatic abnormalities in congenital heart disease (CHD) have not been determined. The aim of the study was to evaluate some indicators of activation of platelets and vascular endothelial cells in patients with CHD, evaluation of bleeding liability of these patients, and correlation with the clinical presentation of these patients.

METHODS

This work was carried out on 20 patients with cyanotic congenital heart diseases (CCHD), 20 patients with acyanotic congenital heart diseases (ACHD), and 20 healthy children who served as the control group, aged between 1 and 10years. All were subjected to full clinical examination, complete blood count, oxygen saturation, echocardiography, bleeding and coagulation times, PT, PTT, FDPs, plasma soluble P-selectin, E-selectin, and platelet factor 4 (PF4).

RESULTS

There was significant prolongation of PT and PTT, and there was a significant lowering of platelet counts. These results were obtained in CCHD and ACHD, but were more significant in CCHD patients. There was a significant elevation in PF4 (55.0±25.5ng/mL), P-selectin (128.9±42.44ng/dL), and E-selectin (9461.5±1701.24pg/mL) levels in children with CCHD as compared to those with ACHD (PF4, 21±7.94ng/mL; P-selectin, 80.1±13.2ng/mL; E-selectin, 7969.6±2127.5pg/mL), and significant increase in both groups when compared to the control group (PF4, 8.1±4.7ng/mL; P-selectin, 27.83±9.73ng/mL; E-selectin, 6750.00±3204.00pg/mL). There was a significant negative correlation between oxygen saturation, plasma P-selectin (r=-0.865), E-selectin (r=-0.401), and PF4 (r=-0.792) in patients with CCHD.

CONCLUSION

Patients with CHD-both cyanotic and acyanotic-have variable degrees of increased liability for both thrombosis and hemorrhage that represents some sort of adaptation to preserve hemostasis and to protect these patients against the clinical presentation of both thrombosis and bleeding. This is to say that CHD patients have their own point of balance between thrombogenicity and bleeding liability. Wide-scale studies are needed to detect the normal levels of different thrombohemorrhagic parameters of these patients.

摘要

背景

先天性心脏病(CHD)患者止血异常发生率增加的确切机制尚未明确。本研究旨在评估CHD患者血小板和血管内皮细胞活化的一些指标,评估这些患者的出血倾向,并分析其与患者临床表现的相关性。

方法

本研究纳入20例青紫型先天性心脏病(CCHD)患者、20例非青紫型先天性心脏病(ACHD)患者以及20名健康儿童作为对照组,年龄在1至10岁之间。所有受试者均接受全面临床检查、全血细胞计数、血氧饱和度、超声心动图、出血及凝血时间、PT、PTT、纤维蛋白降解产物(FDPs)、血浆可溶性P-选择素、E-选择素和血小板因子4(PF4)检测。

结果

CCHD和ACHD患者的PT和PTT均显著延长,血小板计数显著降低,CCHD患者更为明显。与ACHD患者(PF4:21±7.94ng/mL;P-选择素:80.1±13.2ng/mL;E-选择素:7969.6±2127.5pg/mL)相比,CCHD患儿的PF4(55.0±25.5ng/mL)、P-选择素(128.9±42.44ng/dL)和E-选择素(9461.5±1701.24pg/mL)水平显著升高,与对照组(PF4:8.1±4.7ng/mL;P-选择素:27.83±9.73ng/mL;E-选择素:6750.00±3204.00pg/mL)相比,两组均显著升高。CCHD患者的血氧饱和度与血浆P-选择素(r=-0.865)、E-选择素(r=-0.401)和PF4(r=-0.792)之间存在显著负相关。

结论

CHD患者,无论是青紫型还是非青紫型,均有不同程度的血栓形成和出血倾向增加,这是一种为维持止血和保护患者免受血栓形成和出血临床表现影响的适应性反应。也就是说,CHD患者在血栓形成倾向和出血倾向之间有自身的平衡。需要进行大规模研究以检测这些患者不同血栓出血参数的正常水平。

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