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[天然凝血抑制剂;肾病综合征所致高凝状态患儿的抗凝血酶III、蛋白C、蛋白S]

[Natural coagulation inhibitors; antithrombin III, protein C, protein S in children with hypercoagulation due to nephrotic syndrome].

作者信息

Wygledowska G, Grygalewicz J, Matuszewska E

机构信息

Klinika Pediatrii, CMKP, Czerniakowska, 00-416 Warszawa, Poland.

出版信息

Med Wieku Rozwoj. 2001 Oct-Dec;5(4):377-88.

Abstract

UNLABELLED

Activity of AT HI, protein C and protein S was evaluated in 58 children with nephrotic syndrome aged between 2-17 years and in 50 healthy children aged between 2-16 years serving as a control group. In children with nephrotic syndrome measurements were conducted at three different stages of disease: onset, improvement and remission. The aim was to assess the effect of steroid therapy on activity of the above inhibitors.

RESULTS

At the onset of the disease, activity of AT III and protein S were found to be significantly decreased whereas activity of protein C was significantly increased as compared with the control group. Activity of AT III lowered at the onset of the disease, was significantly higher during both the improvement and the remission stage. Activity of protein C, increased at the onset of the nephrotic syndrome, decreased progressively and significantly during the improvement stage and then again increased markedly during the remission. Activity of protein S, lowered at the onset and during the improvement stage of the nephrotic syndrome, significantly rose during the remission, but never reached levels observed in the control group. Activity of AT III and protein C within 72 hours after introduction of steroids increased without any marked alterations in the clinical and biochemical course of the nephrotic syndrome. Activity of protein S was not changed.

CONCLUSIONS

There are marked changes in AT III and protein C activity in children with nephrotic syndrome. It is expected that these proteins are involved in inhibition of the inflammatory process and of the local and systemic coagulopathy. Raised activity of protein C is not accompanied by increased activity of protein. This arouses doubts as to whether protein C-protein S complex is effective in inhibition of inflammation and limitation of coagulopathy. That role may be played AT III, which with its sufficient rise at the early stage of treatment may limit the risk of thromboembolic complications in children with nephrotic syndrome.

摘要

未标注

对58名年龄在2至17岁的肾病综合征患儿以及50名年龄在2至16岁的健康儿童(作为对照组)进行了抗凝血酶III(AT III)、蛋白C和蛋白S活性评估。对肾病综合征患儿在疾病的三个不同阶段进行测量:发病期、病情改善期和缓解期。目的是评估类固醇疗法对上述抑制剂活性的影响。

结果

在疾病发病期,与对照组相比,发现抗凝血酶III和蛋白S活性显著降低,而蛋白C活性显著升高。抗凝血酶III活性在疾病发病期降低,在病情改善期和缓解期均显著升高。蛋白C活性在肾病综合征发病期升高,在病情改善期逐渐且显著降低,然后在缓解期再次显著升高。蛋白S活性在肾病综合征发病期和病情改善期降低,在缓解期显著升高,但从未达到对照组观察到的水平。在引入类固醇后72小时内,抗凝血酶III和蛋白C活性增加,而肾病综合征的临床和生化过程没有任何明显变化。蛋白S活性未改变。

结论

肾病综合征患儿的抗凝血酶III和蛋白C活性有明显变化。预计这些蛋白质参与抑制炎症过程以及局部和全身凝血障碍。蛋白C活性升高并未伴随蛋白活性增加。这引发了关于蛋白C-蛋白S复合物是否能有效抑制炎症和限制凝血障碍的疑问。抗凝血酶III可能发挥该作用,其在治疗早期充分升高可能会降低肾病综合征患儿血栓栓塞并发症的风险。

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