Natural anticoagulants and the liver
Natural anticoagulants and the liver
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DOI：
10.1111/j.1440-1746.1997.tb00351.x
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发表时间：
1997-01-01
影响因子：
4.1
通讯作者：
Salem, HH
中科院分区：
文献类型：
--
作者：
Castelino, DJ;Salem, HH
凝血的调节取决于促凝血、抗凝血和纤溶蛋白之间复杂的相互作用。大多数这些蛋白质是在肝脏中合成的，并且它们的水平在肝病患者中发生改变。肝脏在活化凝血因子清除过程中的止血调节中也发挥着重要作用。因此，肝病患者的临界平衡凝血系统失调也就不足为奇了。在中度肝衰竭中，出血性疾病占主导地位，而在更严重的肝病中，通常观察到血管内凝血，并导致血液凝固的整体失调。在一些患者中，肝病主要是由凝血系统异常引起的。这些患者通常具有由于天然抗凝系统成分缺乏而导致的高凝状态。这些包括蛋白 C、蛋白 S 和抗凝血酶 m。最近，因子 V 基因点突变引起的活化蛋白 C 抵抗已被确定为血栓形成的重要危险因素。在这些患者中，异常的因子 V 能够抵抗活化蛋白 C 的裂解，从而导致持续不受控制的促凝血驱动。这些患者均报告有肝脏和门静脉血栓形成。对这些患者的适当管理应包括对其天然抗凝蛋白进行彻底评估，并排除活化蛋白 C 抵抗作为血栓性疾病的原因。
The regulation of blood coagulation is dependent on a complex interplay between procoagulant, anticoagulant and fibrinolytic proteins. Most of these proteins are synthesised in the liver and their levels are altered in patients with liver disease. The liver also plays an important role in the regulation of haemostasis throughout the clearance of activated clotting factors. It is therefore not surprising that the critically balanced coagulation system is dysregulated in patients with liver disease. In moderate liver failure bleeding disorders predominate, whereas in more advanced liver disease intravascular coagulation is commonly observed and contributes to the overall dysregulation of blood coagulation. In some patients, liver disease can be primarily caused by an abnormality of the coagulation system. These patients usually have a hypercoagulable state caused by a deficiency of a component of the natural anticoagulant system. These include protein C, protein S and antithrombin m. More recently, activated protein C resistance caused by a point mutation in the Factor V gene has been identified as an important risk factor for thrombosis. In these patients the abnormal Factor V is resistant to cleavage by activated protein C resulting in ongoing uncontrolled procoagulant drive. Both hepatic and portal vein thrombosis have been reported in these patients. Appropriate management of these patients should include a thorough assessment of their natural anticoagulant proteins and exclusion of activated protein C resistance as the cause of their thrombotic disorder.