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免疫抑制和溶栓治疗对一名患有白塞病所致心内血栓形成和肺动脉瘤的患者真的有效吗?

Is Immunosuppressive and Thrombolytic Therapy Really Effective in a Patient With Intracardiac Thrombosis and Pulmonary Artery Aneurysm due to Behçet's Disease?

作者信息

Aladağ Çiftdemir Nükhet, Gökalp Selman, Eren Tuba

机构信息

Department of Pediatrics, Trakya University Faculty of Medicine, Edirne, Turkey.

Department of Pediatrics, Cerrahpaşa University Faculty of Medicine, Istanbul, Turkey.

出版信息

Arch Rheumatol. 2019 May 22;34(4):451-456. doi: 10.5606/ArchRheumatol.2019.7169. eCollection 2019 Dec.

Abstract

Behçet's disease (BD) is a rare, multisystemic, chronic vasculitic disorder with unknown etiology. Intracardiac thrombus formation and pulmonary artery aneurysm in BD are very rare. The treatment protocol in patients with vascular involvement, particularly those with pulmonary artery aneurysm accompanied by thrombosis, have not been clearly defined. In this article, we report an exceptional case, who had been treated unsuccessfully with a combination of anti-inflammatory/immunosuppressive therapy and thrombolytic agents, to discuss the poor prognosis of pulmonary artery aneurysm accompanying intracardiac thrombosis in juvenile BD.

摘要

白塞病(BD)是一种病因不明的罕见的多系统慢性血管炎性疾病。白塞病中心腔内血栓形成和肺动脉瘤非常罕见。血管受累患者,尤其是伴有血栓形成的肺动脉瘤患者的治疗方案尚未明确界定。在本文中,我们报告了一例特殊病例,该病例接受抗炎/免疫抑制治疗和溶栓药物联合治疗未成功,以探讨青少年白塞病中心腔内血栓形成伴肺动脉瘤的不良预后。

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本文引用的文献

1
A review of current management of vasculo-Behcet's.血管型白塞病的治疗现状综述
Curr Opin Rheumatol. 2018 Jan;30(1):50-56. doi: 10.1097/BOR.0000000000000458.
3
Imaging pearls of pediatric Behçet's disease.儿童贝赫切特病的影像学表现。
Eur J Radiol. 2017 Sep;94:115-124. doi: 10.1016/j.ejrad.2017.06.016. Epub 2017 Jun 28.
4
Behçet's disease in children, an overview.儿童贝赫切特病概述
Pediatr Rheumatol Online J. 2016 Feb 18;14(1):10. doi: 10.1186/s12969-016-0070-z.
8
Behçet's syndrome: pulmonary vascular disease.白塞病:肺血管疾病
Curr Opin Rheumatol. 2015 Jan;27(1):18-23. doi: 10.1097/BOR.0000000000000131.

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