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首页> 外文期刊>Hepatology: Official Journal of the American Association for the Study of Liver Diseases >An unbalance between von Willebrand factor and ADAMTS13 in acute liver failure: Implications for hemostasis and clinical outcome
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An unbalance between von Willebrand factor and ADAMTS13 in acute liver failure: Implications for hemostasis and clinical outcome

机译：急性肝衰竭中von Willebrand因子和ADAMTS13之间的不平衡：对止血和临床结局的影响

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摘要

Emerging evidence supports the concept of a rebalanced hemostatic state in liver disease as a result of a commensurate decline in prohemostatic and antihemostatic drivers. In the present study, we assessed levels and functionality of the platelet-adhesive protein von Willebrand factor (VWF) and its cleaving protease ADAMTS13 in the plasma of patients with acute liver injury and acute liver failure (ALI/ALF). Furthermore, we explored possible associations between VWF, ADAMTS13, and disease outcome. We analyzed the plasma of 50 patients taken on the day of admission for ALI/ALF. The plasma of 40 healthy volunteers served as controls. VWF antigen levels were highly elevated in patients with ALI/ALF. In contrast, the collagen-binding activity and the ratio of the VWF ristocetin cofactor activity and VWF antigen was significantly decreased when compared with healthy controls. Also, the proportion of high molecular weight VWF multimers was reduced, despite severely decreased ADAMTS13 levels. In spite of these functional defects, platelet adhesion and aggregation were better supported by plasma of patients with ALI/ALF when compared with control plasma. Low ADAMTS13 activity, but not high VWF antigen, was associated with poor outcome in patients with ALI/ALF as evidenced by higher grades of encephalopathy, higher transplantation rates, and lower survival. VWF or ADAMTS13 levels were not associated with bleeding or thrombotic complications. Conclusion: Highly elevated levels of VWF in plasma of patients with ALI/ALF support platelet adhesion, despite a relative loss of function of the molecule. Furthermore, low ADAMTS13 activity is associated with progressive liver failure in the patient cohort, which might be attributed to platelet-induced microthrombus formation in the diseased liver resulting from a substantially unbalanced VWF/ADAMTS13 ratio.
机译：越来越多的证据支持肝病中止血状态重新平衡的概念，这是由于止血和止血驱动剂的相应下降所致。在本研究中，我们评估了急性肝损伤和急性肝衰竭（ALI / ALF）患者血浆中的血小板粘附蛋白von Willebrand因子（VWF）及其裂解蛋白酶ADAMTS13的水平和功能。此外，我们探讨了VWF，ADAMTS13与疾病结果之间的可能关联。我们分析了入院当天接受ALI / ALF的50名患者的血浆。 40名健康志愿者的血浆作为对照。 ALI / ALF患者的VWF抗原水平高度升高。相反，与健康对照相比，胶原蛋白结合活性和VWF瑞斯托菌素辅助因子活性与VWF抗原的比率显着降低。同样，尽管ADAMTS13水平严重降低，高分子量VWF多聚体的比例也降低了。尽管存在这些功能缺陷，但与对照血浆相比，ALI / ALF患者的血浆能更好地支持血小板粘附和聚集。 ADAMTS13活性低但VWF抗原不高与ALI / ALF患者预后差有关，这由较高程度的脑病，较高的移植率和较低的生存率所证明。 VWF或ADAMTS13水平与出血或血栓形成并发症无关。结论：尽管该分子功能相对丧失，但ALI / ALF患者血浆中VWF的高度升高仍支持血小板粘附。此外，ADAMTS13活性低与患者队列中的进行性肝衰竭有关，这可能归因于VWF / ADAMTS13比例基本不平衡导致患病肝脏中血小板诱导的微血栓形成。

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