apc耐药儿童的凝血和纤溶:一项人群研究

U. Nowak-Göttl , H. Vielhaber , R. Schneppenheim , H.G. Koch
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引用次数: 6

摘要

目的:对活化蛋白C(APCR)的耐药性已成为静脉血栓栓塞症最重要的遗传原因。为了确定这种相对常见的基因突变在多大程度上影响总体止血平衡,在APC耐药儿童群体(n=46)中与年龄匹配的健康对照组(n=80)进行了凝血和纤溶研究,PAP和PAI1在APCR患儿中显著升高。此外,TM、D-二聚体、t-PA、u-PA、PAI 1和PAP与凝血酶生成量呈显著正相关。在进行这项研究之前,儿童和患有血管损伤的患者之间没有发现差异。结论:APCR患儿表现为活化凝血和纤溶的结合。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Coagulation and fibrinolysis in children with APC-resistance: a population study

Objective: resistance to activated protein C (APCR) has emerged as the most important hereditary cause of venous thromboembolism. To determine to what extent this relatively common gene mutation influences the overall haemostatic balance, coagulation and fibrinolysis were investigated in a population of APC resistant children (n=46) in comparison to an age-matched healthy control group (n=80).

Results: compared to the sex- and age-matched healthy control thrombomodulin (TM), F1+2, D-Dimer formation, t-PA, u-PA, PAP and PAI 1 were significantly increased in the APCR children. In addition, TM, D-Dimer, t-PA, u-PA, PAI 1 and PAP showed a significantly positive correlation to the amount of thrombin generated. No difference was found between children and patients who had suffered from vascular insults before this study was conducted.

Conclusions: children with APCR showed a combination of activated coagulation and fibrinolysis.

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