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血栓弹力图检测发现一名失代偿期肝病患者在接受肝活检时凝血块稳定性改变及补充凝血因子 XIII 的必要性。

Thromboelastometry Identified Alteration of Clot Stabilization and Factor XIII Supplementation Need in a Patient with Decompensated Liver Disease Undergoing Liver Biopsy.

作者信息

Crochemore Tomaz, Aragão Savioli Felicio

机构信息

Hospital Leforte, Department of Critical Care, São Paulo, SP, Brazil.

出版信息

Case Rep Gastrointest Med. 2018 Aug 29;2018:6360543. doi: 10.1155/2018/6360543. eCollection 2018.

DOI:10.1155/2018/6360543
PMID:30228917
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6136510/
Abstract

Liver disease has been considered the prototype of hemorrhagic disease. Disorder in any component of coagulation system can lead to hemorrhage. Deficiency of factor XIII may impair clot strength and clot stabilization and can be accessed by thromboelastometry. We report a case of a patient with a rapid evolution of liver disease who underwent a liver biopsy. Thromboelastometry was performed, evidencing impairment of clot stability. This clotting disorder was corrected with factor XIII concentrate after unsuccessful administration of antifibrinolytic drugs and hepatic biopsy was performed without hemorrhagic complications. . We report the case of a previously healthy 38-year-old man, who presented to our emergency department with clinical signs of rapid progression of acute liver failure. The laboratory tests revealed platelets of 142x10/mm3, plasma fibrinogen concentration of 221 mg/dl, increased international nationalized ratio (INR 1.9), total bilirubin of 3.9mg/dl, direct bilirubin of 2.3mg/dl, ALT 751U/l, and AST 540U/l without acute bleeding. A liver biopsy was indicated. Based on the results of the thromboelastometry, Tranexamic Acid was administered to correct hyperfibrinolysis followed by factor XIII concentrate to correct factor XIII deficiency. Thromboelastometry was normal despite conventional coagulation tests were still altered. So, liver biopsy was performed with no signs of bleeding and without need of further transfusion. . Thromboelastometry may be considered a useful, feasible, and safe tool to monitor and manage coagulopathy in patients with liver disease, with the potential advantage of helping avoid unnecessary transfusion in such patients.

摘要

肝脏疾病一直被视为出血性疾病的典型代表。凝血系统任何一个组成部分出现紊乱都可能导致出血。因子 XIII 缺乏可能会损害血凝块强度和血凝块稳定性,可通过血栓弹力图法进行评估。我们报告一例肝病迅速进展的患者接受肝脏活检的病例。进行了血栓弹力图检查,结果显示血凝块稳定性受损。在抗纤溶药物给药未成功后,使用因子 XIII 浓缩物纠正了这种凝血障碍,随后进行了肝脏活检,未出现出血并发症。我们报告一例既往健康的 38 岁男性,他因急性肝衰竭快速进展的临床症状就诊于我们的急诊科。实验室检查显示血小板计数为 142×10⁹/mm³,血浆纤维蛋白原浓度为 221mg/dl,国际标准化比值升高(INR 1.9),总胆红素为 3.9mg/dl,直接胆红素为 2.3mg/dl,谷丙转氨酶 751U/l,谷草转氨酶 540U/l,且无急性出血情况。遂进行肝脏活检。根据血栓弹力图检查结果,先给予氨甲环酸纠正高纤溶状态,随后给予因子 XIII 浓缩物纠正因子 XIII 缺乏。尽管传统凝血检查结果仍异常,但血栓弹力图检查结果已恢复正常。因此,进行肝脏活检时未出现出血迹象,也无需进一步输血。血栓弹力图法可被视为监测和管理肝病患者凝血障碍的一种有用、可行且安全的工具,其潜在优势在于有助于避免此类患者不必要的输血。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/977b/6136510/00c5ad194c9f/CRIGM2018-6360543.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/977b/6136510/00c5ad194c9f/CRIGM2018-6360543.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/977b/6136510/00c5ad194c9f/CRIGM2018-6360543.001.jpg

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本文引用的文献

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Factor XIII in plasma, but not in platelets, mediates red blood cell retention in clots and venous thrombus size in mice.血浆中的因子 XIII,而不是血小板,介导了红细胞在血栓中的滞留和静脉血栓的大小。
Blood Adv. 2018 Jan 3;2(1):25-35. doi: 10.1182/bloodadvances.2017011890. eCollection 2018 Jan 9.
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Inhibition of Fibrinolysis by Coagulation Factor XIII.凝血因子 XIII 对纤维蛋白溶解的抑制作用。
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A new era of thromboelastometry.血栓弹力图的新时代。
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Hemostasis in Acute and Chronic Liver Disease.急慢性肝病中的止血
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Effects of Factor XIII Deficiency on Thromboelastography. Thromboelastography with Calcium and Streptokinase Addition is more Sensitive than Solubility Tests.因子 XIII 缺乏对血栓弹力图的影响。添加钙和链激酶的血栓弹力图比溶解度试验更敏感。
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Comparison between thrombelastography and thromboelastometry in hyperfibrinolysis detection during adult liver transplantation.在成人肝移植期间检测高纤维蛋白溶解过程中,血栓弹力描记术与血栓弹力图的比较。
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Thromboelastometry-guided hemostatic therapy: an efficacious approach to manage bleeding risk in acute fatty liver of pregnancy: a case report.血栓弹力图引导的止血治疗:一种管理妊娠急性脂肪肝出血风险的有效方法:病例报告
J Med Case Rep. 2015 Sep 23;9:202. doi: 10.1186/s13256-015-0690-9.
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The antifibrinolytic function of factor XIII is exclusively expressed through α₂-antiplasmin cross-linking.因子 XIII 的抗纤维蛋白溶解功能仅通过 α₂-抗纤溶酶交联来表达。
Blood. 2011 Jun 9;117(23):6371-4. doi: 10.1182/blood-2011-02-333203. Epub 2011 Apr 6.
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