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休斯-斯托文综合征:它是不完全型白塞病吗?两例报告及文献复习

Hughes-Stovin syndrome: is it incomplete Behçet's? Report of two cases and review of the literature.

作者信息

Emad Yasser, Ragab Yasser, Shawki Abd El-Hakim, Gheita Tamer, El-Marakbi Ahmed, Salama Mohamed Hanfi

机构信息

Rheumatology and Rehabilitation Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

出版信息

Clin Rheumatol. 2007 Nov;26(11):1993-6. doi: 10.1007/s10067-007-0609-y. Epub 2007 Apr 25.

Abstract

In this report, we described two male patients with Hughes-Stovin syndrome. The first patient was a 26-year-old male who initially presented with deep vein thrombosis (DVT) in the right lower limb followed shortly by hemoptysis. Pulmonary CT angiography revealed bilateral pulmonary artery aneurysms secondary to underlying pulmonary vasculitis. While the second case was a 16-year-old male patient who initially presented with generalized fits due to sagittal sinus thrombosis and later followed by DVT in the left lower limb. Pulmonary CT angiography showed small pulmonary artery aneurysms with thrombosis of peripheral pulmonary artery branches. Both patients were treated successfully with steroids and immunosuppressive therapy and showed clinical improvement.

摘要

在本报告中,我们描述了两名患有休斯-斯托文综合征的男性患者。首例患者为一名26岁男性,最初表现为右下肢深静脉血栓形成(DVT),随后不久出现咯血。肺部CT血管造影显示双侧肺动脉瘤继发于潜在的肺血管炎。而第二例是一名16岁男性患者,最初因矢状窦血栓形成出现全身性惊厥,后来左下肢出现DVT。肺部CT血管造影显示小的肺动脉瘤伴外周肺动脉分支血栓形成。两名患者均接受了类固醇和免疫抑制治疗,治疗成功且临床症状改善。

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