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Multimodal endovascular management of a Jehovah's Witness patient with Hugues-Stovin syndrome presenting with ruptured pulmonary artery aneurysm and cardiopulmonary thromboembolism.

作者信息

Kably Issam M, Reveron Carolina

机构信息

Department of Radiology, Section of Vascular and Interventional Radiology, Jackson Memorial Hospital, University of Miami Miller School of Medicine, Miami, FL, USA

Department of Radiology, University of Miami Miller School of Medicine, Miami, FL, USA.

出版信息

Eur J Cardiothorac Surg. 2015 Apr;47(4):e158-61. doi: 10.1093/ejcts/ezu529. Epub 2015 Feb 5.

DOI:10.1093/ejcts/ezu529
PMID:25656405
Abstract

Hughes-Stovin syndrome is a very rare disease with fewer than 40 cases reported in the literature. The disease is thought to be a variant of Behçet's disease that can manifest with massive haemoptysis due to ruptured pulmonary arterial aneurysms in the setting of deep venous thrombosis. This association might impose a therapeutic dilemma when anticoagulation has to be balanced with the excessive risk of bleeding, especially if the patient refuses any blood replacement, and is not a surgical candidate. We present a case of a 41-year-old Jehovah's Witness patient with Hughes-Stovin syndrome that presented to the emergency room with haemoptysis due to multiple ruptured pulmonary artery aneurysms. Her work-up showed concomitant cardio-venous thromboembolism and pulmonary infarction. She was successfully managed via a bloodless single-wall venous puncture, allowing endovascular treatment of bilateral pulmonary aneurysms with subsequent caval filtration. The course was uneventful, and the patient was discharged under immunosuppressant.

摘要

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引用本文的文献

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A Rare but Fatal Behçet Variant: The Hughes-Stovin Syndrome-Successful Case Report and New Evidence from Literature Review.一种罕见但致命的白塞病变体:休斯-斯托文综合征——成功病例报告及文献综述新证据
Aorta (Stamford). 2023 Aug;11(4):156-161. doi: 10.1055/s-0043-1777994. Epub 2024 Mar 26.
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Pulmonary vasculitis in Hughes-Stovin syndrome (HSS): a reference atlas and computed tomography pulmonary angiography guide-a report by the HSS International Study Group.休斯-斯托文综合征(HSS)中的肺血管炎:参考图谱和 CT 肺动脉造影指南——HSS 国际研究组报告。
Clin Rheumatol. 2021 Dec;40(12):4993-5008. doi: 10.1007/s10067-021-05912-3. Epub 2021 Sep 17.
3
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Z Rheumatol. 2019 May;78(4):365-371. doi: 10.1007/s00393-019-0618-7.
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Transcatheter embolotherapy of pulmonary artery aneurysms as emergency treatment of hemoptysis in Behcet patients: experience of a referral center and a review of the literature.经导管肺动脉瘤栓塞术治疗白塞病咯血的紧急治疗:一家转诊中心的经验和文献复习。
Intern Emerg Med. 2018 Jun;13(4):491-500. doi: 10.1007/s11739-018-1817-y. Epub 2018 Mar 7.