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[休斯-斯托文综合征,一例报告]

[Hughes-Stovin Syndrome, a case report].

作者信息

Pankl Sonia, Meraldi Ana, Pegoraro Paola, Earsman Geofredo, Bruetman Julio E, Ceresetto José, Bosio Martín, Lyons Gustavo, Young Pablo

机构信息

Servicio de Clínica Médica, Hospital Británico de Buenos Aires, Argentina.

出版信息

Medicina (B Aires). 2015;75(2):95-8.

Abstract

The Hughes-Stovin syndrome is a rare entity characterized by deep vein thrombosis and pulmonary artery aneurysms of unknown etiology and pathogenesis. Some authors considered a variant of Behcet's disease. Its natural course is usually fatal. The symptoms are cough, dyspnea, hemoptysis, chest pain and fever. The treatment goes from steroids and cytotoxic agents to surgery. We present the case of a 41 year old man who shows dyspnea, hemoptysis, and chest pain leading to the diagnosis of deep venous thrombosis of the right leg, lung thromboembolism and pulmonary artery aneurysms. He was treated with high-dose corticosteroids and 6 cyclophosphamide pulses of 1 gram each per 6 months with complete regression of aneurysms and symptomatology.

摘要

休斯-斯托文综合征是一种罕见病症,其特征为病因和发病机制不明的深静脉血栓形成和肺动脉瘤。一些作者认为它是白塞病的一种变体。其自然病程通常是致命的。症状包括咳嗽、呼吸困难、咯血、胸痛和发热。治疗方法从使用类固醇和细胞毒性药物到手术。我们报告一例41岁男性病例,该患者出现呼吸困难、咯血和胸痛,最终诊断为右腿深静脉血栓形成、肺血栓栓塞和肺动脉瘤。他接受了大剂量皮质类固醇治疗,并每6个月进行6次每次1克的环磷酰胺冲击治疗,动脉瘤和症状完全消退。

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