| 高波 周荣富 徐勇 李萍.遗传性蛋白C缺乏症的研究进展[J].,2015,15(2):362-364 |
| 遗传性蛋白C缺乏症的研究进展 |
| A Review of Research on Inherited Protein C Deficiency Syndrome |
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| DOI： |
| 中文关键词: 蛋白C 蛋白S 血栓 基因检测 |
| 英文关键词: Protein C Protein S Thrombus Gene detection |
| 基金项目: |
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| 中文摘要: |
| 蛋白C（Protein C）是一种维生素K 依赖性糖蛋白，它在凝血酶或凝血酶- 血栓调节蛋白复合物的作用下转变为活化蛋白
C，即APC（activated protein C），APC有灭活Va、VIIIa 及增加纤溶的活性，因此具有抗凝作用。蛋白S是蛋白C系统的重要组成
部分，蛋白C/S缺乏是由编码蛋白C/S 的基因突变或多态性引起的常染色体遗传性疾病，易产生深静脉血栓，特点是首次发生血
栓的年龄小，血栓发生次数多，且静脉血栓形成易造成肺栓塞，所以当临床上遇到有上述特点的静脉血栓患者时，应进行血浆蛋
白C系统检测，近年来随着分子生物技术的发展，对遗传性蛋白C/S基因的基因突变和基因多态性研究进入了一个新的阶段。治
疗上无临床症状的PC缺乏症者无需治疗，在术前或分娩前的抗栓治疗中，可输注PC 浓缩剂、新鲜血浆、凝血酶原复合物或肝素，
血栓形成时需做溶栓治疗。 |
| 英文摘要: |
| Protein C (PC) is a vitamin K-dependent plasma protein that is synthesized in the liver. With thrombin and
prothrombin complex, it becomes activated protein C (APC), which can inactivate factor Va and VIIIa and increase the fibrinolytic ability
with the help of protein S. Thus protein C is a kind of anticoagulant protein. Protein S is a member of protein C system. Inherited protein
C and/or S deficiency is an inherited disease due to mutation of the gene codes the protein. This disease is often manifested with
repeatedly deep vein thrombosis, especially with young individuals, which usually leads to pulmonary thrombosis. Thus, the detection of
protein C system is important. With the development of the technique in molecular medicine, there comes the new era of gene mutation
detection and polymorphism of protein C deficiency patients. The treatment for this disease includes the protein C concentrates, FFP,
prothrombin complex and heparin. Thrombolytic therapy is necessary when thrombus formed. |
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