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抗凝血酶III缺乏症:布加综合征的一个病因。

Antithrombin III deficiency: an etiology of Budd-Chiari syndrome.

作者信息

Das M, Carroll S F

出版信息

Surgery. 1985 Feb;97(2):242-6.

PMID:3969629
Abstract

This report documents a unique case of Budd-Chiari syndrome associated with antithrombin III deficiency and massive thrombus in the superior vena cava and right atrium. This aberration of the coagulation mechanism is proposed as an etiologic factor in the pathogenesis of hepatic venous obstruction whenever the cause is obscure. Antithrombin III was restored to a level adequate to permit thrombus extraction with cardiopulmonary bypass and relief of portal hypertension with a mesoatrial shunt. The protocol for reversing this hypercoagulable state with fresh frozen plasma and warfarin (Coumadin) is discussed, and technical innovations employed in the management of this complex problem are described.

摘要

本报告记录了一例独特的布加综合征病例,该病例与抗凝血酶III缺乏以及上腔静脉和右心房内的大量血栓形成相关。当肝静脉阻塞病因不明时,这种凝血机制异常被认为是其发病机制中的一个病因。通过体外循环使抗凝血酶III恢复到足以允许取出血栓的水平,并通过中房分流术缓解门静脉高压。讨论了使用新鲜冰冻血浆和华法林(香豆素)逆转这种高凝状态的方案,并描述了处理这一复杂问题所采用的技术创新。

相似文献

1
Antithrombin III deficiency: an etiology of Budd-Chiari syndrome.抗凝血酶III缺乏症:布加综合征的一个病因。
Surgery. 1985 Feb;97(2):242-6.
2
Budd-Chiari Syndrome and antithrombin III deficiency.布加综合征与抗凝血酶III缺乏症
Am J Clin Pathol. 1982 Aug;78(2):236-41. doi: 10.1093/ajcp/78.2.236.
3
Budd-Chiari syndrome: a common complication of Behçet's disease.布加综合征:白塞病的常见并发症。
Am J Gastroenterol. 1997 May;92(5):858-62.
4
Budd-Chiari syndrome due to antithrombin III deficiency.抗凝血酶III缺乏所致的布加综合征
Indian J Pediatr. 2003 Dec;70(12):1003-5. doi: 10.1007/BF02723831.
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Budd-Chiari syndrome with inferior vena cava obstruction associated with systemic lupus erythematosus.布加综合征合并下腔静脉阻塞伴系统性红斑狼疮
J Clin Gastroenterol. 1984 Jun;6(3):253-6.
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Budd-Chiari syndrome attributed to protein C deficiency.布加综合征归因于蛋白C缺乏。
Am J Gastroenterol. 1996 Apr;91(4):777-9.
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[Budd-Chiari syndrome and congenital antithrombin III deficiency].布加综合征与先天性抗凝血酶III缺乏症
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Experience with mesocaval shunt with autologous jugular vein interposition in patients with Budd-Chiari syndrome.布加综合征患者采用自体颈静脉间置的门腔分流术的经验。
Hepatogastroenterology. 2005 May-Jun;52(63):662-5.
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Heterotaxy syndrome with azygous continuation-causing pseudo Budd-Chiari syndrome after cardiopulmonary bypass.心肺转流术后因奇静脉延续导致假性布加综合征的内脏异位综合征
Ann Thorac Surg. 2006 May;81(5):1890-2. doi: 10.1016/j.athoracsur.2005.05.090.
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Acute partial Budd-Chiari syndrome and portal vein thrombosis in cytomegalovirus primary infection: a case report.巨细胞病毒原发性感染中的急性部分性布加综合征和门静脉血栓形成:一例报告
BMC Gastroenterol. 2006 Mar 10;6:10. doi: 10.1186/1471-230X-6-10.

引用本文的文献

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Low burden of a JAK2-V617F mutated clone in monoclonal haematopoiesis in a Japanese woman with Budd-Chiari syndrome.一名患有布加综合征的日本女性单克隆造血中JAK2-V617F突变克隆的低负荷
Int J Hematol. 2009 May;89(4):517-522. doi: 10.1007/s12185-009-0280-y. Epub 2009 Mar 24.
2
Budd-Chiari syndrome: a case with a combination of hepatic vein and superior vena cava occlusion.布加综合征:一例肝静脉与上腔静脉联合闭塞病例
World J Gastroenterol. 2005 Jun 28;11(24):3797-9. doi: 10.3748/wjg.v11.i24.3797.
3
Budd-Chiari syndrome due to antithrombin III deficiency.
抗凝血酶III缺乏所致的布加综合征
Indian J Pediatr. 2003 Dec;70(12):1003-5. doi: 10.1007/BF02723831.
4
Suprahepatic Budd-Chiari syndrome treated with thrombectomy and cavoplasty.经血栓切除术和腔静脉成形术治疗的肝上型布加综合征
Dig Dis Sci. 2003 Aug;48(8):1637-41. doi: 10.1023/a:1024740512762.
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Factor V Leiden related Budd-Chiari syndrome.因子V莱顿突变相关的布加综合征
Gut. 2001 Feb;48(2):264-8. doi: 10.1136/gut.48.2.264.
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Budd-Chiari Syndrome.布加综合征
Curr Treat Options Gastroenterol. 1999 Dec;2(6):491-504. doi: 10.1007/s11938-999-0053-y.
7
Acute Budd-Chiari syndrome with hepatic failure and obstruction of the inferior vena cava as presenting manifestations of hereditary protein C deficiency.以急性布加综合征伴肝衰竭及下腔静脉梗阻为表现的遗传性蛋白C缺乏症
Gut. 1990 Aug;31(8):949-52. doi: 10.1136/gut.31.8.949.
8
Liver transplantation for the Budd-Chiari syndrome.布加综合征的肝移植治疗
Ann Surg. 1990 Jan;211(1):43-9. doi: 10.1097/00000658-199001000-00007.