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一位患有凝血因子V莱顿突变的患者出现下腔静脉膜性梗阻:血栓形成后病因学的证据。

Membranous obstruction of the inferior vena cava in a patient with factor V Leiden: evidence for a post-thrombotic aetiology.

作者信息

Blanshard C, Dodge G, Pasi J, Ormiston M, Dick R, Burroughs A K

机构信息

Department of Liver Transplantation and Hepatobiliary Medicine, Royal Free Hospital, London, UK.

出版信息

J Hepatol. 1997 Mar;26(3):731-5. doi: 10.1016/s0168-8278(97)80442-4.

DOI:10.1016/s0168-8278(97)80442-4
PMID:9075684
Abstract

BACKGROUND

Membranous obstruction of the inferior vena cava is a rare cause of hepatic venous outflow obstruction in Caucasians. There has been much debate in the literature about its aetiology.

METHODS AND RESULTS

We describe a Caucasian with hepatic venous outflow obstruction due to an inferior vena cava web, who was found to have hypercoagulability due to factor V Leiden. Following balloon rupture of the membrane and anticoagulation, his symptoms resolved and he has remained well for a year.

CONCLUSIONS

The age at presentation in this patient, the presence of hypercoagulability and the excellent response to membrane rupture and anticoagulation suggest that in this case the membrane may have been derived from organised thrombus. Balloon rupture of the membrane and anticoagulation appears to be an effective treatment in such cases.

摘要

背景

在高加索人群中,下腔静脉膜性梗阻是肝静脉流出道梗阻的罕见原因。关于其病因,文献中存在诸多争议。

方法与结果

我们描述了一名因下腔静脉蹼导致肝静脉流出道梗阻的高加索人,该患者被发现因凝血因子V莱顿突变而具有高凝状态。在对隔膜进行球囊破裂术及抗凝治疗后,他的症状得到缓解,且一年来状况良好。

结论

该患者的发病年龄、高凝状态的存在以及对隔膜破裂术和抗凝治疗的良好反应表明,在这种情况下,隔膜可能源自机化血栓。隔膜球囊破裂术和抗凝治疗在此类病例中似乎是一种有效的治疗方法。

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1
Membranous obstruction of the inferior vena cava in a patient with factor V Leiden: evidence for a post-thrombotic aetiology.一位患有凝血因子V莱顿突变的患者出现下腔静脉膜性梗阻:血栓形成后病因学的证据。
J Hepatol. 1997 Mar;26(3):731-5. doi: 10.1016/s0168-8278(97)80442-4.
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Liver cirrhosis in hepatic vena cava syndrome (or membranous obstruction of inferior vena cava).
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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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Long term complications of inferior vena cava thrombosis.下腔静脉血栓形成的长期并发症。
Arch Dis Child. 2001 Sep;85(3):228-33. doi: 10.1136/adc.85.3.228.