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急诊科罕见的两例布加综合征病例:病例报告

A rare encounter with two cases of Budd‑Chiari syndrome in the Emergency Department: A case report.

作者信息

Stanciugelu Alexandra, Petrică Alina, Chiriac Sorin Dan, Iurciuc Mircea, Boruga Madalina Veronica, Balica Nicolae, Mederle Ovidiu Alexandru

机构信息

Department of Surgery, Faculty of Medicine, 'Victor Babes' University of Medicine and Pharmacy, 300041 Timisoara, Romania.

Department of Emergency, Emergency Clinical Municipal Hospital, 300079 Timisoara, Romania.

出版信息

Exp Ther Med. 2022 Oct 24;24(6):730. doi: 10.3892/etm.2022.11666. eCollection 2022 Dec.

DOI:10.3892/etm.2022.11666
PMID:36349062
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9634885/
Abstract

Budd-Chiari syndrome (BCS) is a rare disorder clinically characterized by abdominal pain, hepatomegaly and ascites. The condition is often related to thrombosis of the hepatic veins or the terminal portion of the inferior vena cava. A myeloproliferative disorder is the most identified underlying prothrombotic risk factor, although almost one-half of affected patients are now recognized as having multiple underlying prothrombotic risk factors. Doppler ultrasound may be enough to confirm the diagnosis of BCS; however, computed tomography or magnetic resonance imaging is often employed. Anticoagulant therapy is the cornerstone of BCS treatment, but most patients also need additional treatment strategies. Most patients with BCS are now treated by endovascular intervention, which has improved survival rate in those afflicted by this disease. The long-term course of the disease can be complicated by progression or recurrence of the underlying myeloproliferative disorder. The present study reports the cases of two patients with BCS with the aim of alerting healthcare workers in Emergency Departments of this less common diagnosis in patients presenting with frequent complaints of abdominal pain.

摘要

布加综合征(BCS)是一种罕见的疾病,临床特征为腹痛、肝肿大和腹水。该病常与肝静脉或下腔静脉末端血栓形成有关。骨髓增殖性疾病是最常见的潜在促血栓形成危险因素,尽管现在几乎一半的患者被认为有多种潜在促血栓形成危险因素。多普勒超声可能足以确诊BCS;然而,计算机断层扫描或磁共振成像也经常被采用。抗凝治疗是BCS治疗的基石,但大多数患者还需要其他治疗策略。现在大多数BCS患者通过血管内介入治疗,这提高了该病患者的生存率。该疾病的长期病程可能因潜在骨髓增殖性疾病的进展或复发而复杂化。本研究报告了两例BCS患者的病例,目的是提醒急诊科的医护人员,对于经常主诉腹痛的患者,这种不太常见的诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bc1f/9634885/4e4f31025027/etm-24-06-11666-g00.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bc1f/9634885/4e4f31025027/etm-24-06-11666-g00.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bc1f/9634885/4e4f31025027/etm-24-06-11666-g00.jpg

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本文引用的文献

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Review of Budd-Chiari Syndrome.布加综合征综述
J Interv Med. 2020 Mar 31;3(2):65-76. doi: 10.1016/j.jimed.2020.03.002. eCollection 2020 May.
2
Budd-Chiari Syndrome and Acute Liver Failure: An Uncommon Presentation of Acute Myeloid Leukaemia.布加综合征与急性肝衰竭:急性髓系白血病的一种罕见表现。
GE Port J Gastroenterol. 2020 Dec;28(1):62-66. doi: 10.1159/000507335. Epub 2020 May 27.
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Long-Term Outcomes of Endovascular Interventions in More than 500 patients with Budd-Chiari Syndrome.500 余例布加综合征患者血管内介入治疗的长期疗效。
布加综合征合并自身免疫性溶血性贫血、免疫性血小板减少性紫癜和狼疮性肾炎:病例报告
Cureus. 2024 Jul 22;16(7):e65160. doi: 10.7759/cureus.65160. eCollection 2024 Jul.
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An Update on the Management of Budd-Chiari Syndrome.布加综合征治疗进展。
Dig Dis Sci. 2021 Jun;66(6):1780-1790. doi: 10.1007/s10620-020-06485-y. Epub 2020 Jul 20.
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Imbalance of pro- vs. anti-coagulation factors in Chinese patients with Budd-Chiari syndrome and non-cirrhotic portal vein thrombosis.中国布加综合征和非肝硬化门静脉血栓形成患者促凝血与抗凝血因子失衡
PLoS One. 2015 Mar 30;10(3):e0119909. doi: 10.1371/journal.pone.0119909. eCollection 2015.
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Budd-Chiari syndrome: a single-center experience.布加综合征:单中心经验
World J Gastroenterol. 2014 Nov 21;20(43):16236-44. doi: 10.3748/wjg.v20.i43.16236.
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Long-term Outcome of Budd-Chiari Syndrome: A Single Center Experience.布加综合征的长期预后:单中心经验
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An update on management of Budd-Chiari syndrome.布加综合征治疗进展。
Ann Hepatol. 2014 May-Jun;13(3):323-6.
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Good long-term outcome of Budd-Chiari syndrome with a step-wise management.布加综合征的阶梯式管理具有良好的长期疗效。
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Acute promyelocytic leukemia-associated thrombosis.急性早幼粒细胞白血病相关性血栓形成。
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