环磷酰胺、利伐沙班和皮质类固醇联合应用对behet病相关Budd-Chiari综合征的影响

F. Enc, Celal Ulaşoğlu
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引用次数: 0

摘要

以复发性口腔和生殖器溃疡、皮肤损伤、葡萄膜炎、关节炎和血管炎为特征的溃疡性疾病由于血管炎和下肢深静脉受累导致的血栓形成可能是主要的发现。Budd-Chiari综合征(BCS)是由肝大静脉阻塞引起的。随着时间的推移,这些患者可能发展为肝功能衰竭和门静脉高压症在西方国家,5%的BCS与BD相关,土耳其为9%,埃及为13%。然而,BCS与BD相关的BCS患者常被误诊或延误诊断。5部分患者可能无症状,但经常出现腹痛、黄疸、呕吐、肝肿大和腹水。在这些患者中,不完整的双相障碍诊断标准不应延误治疗。在这里,我们报告了一位因肝酶升高和下肢血栓病史住院的患者,后来被诊断为bd相关性BCS,对联合治疗有反应。
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Behçet's Disease-Related Budd-Chiari Syndrome Responding to Combination of Cyclophosphamide, Rivaroxaban and Corticosteroids
culitic disease which is characterized by recurrent oral and genital aphtous ulcers, skin lesions, uveitis, arthritis and vasculitis.1 Thrombosis as a result of vasculitis and lower extremity deep vein involvement as the leading finding may be present. Budd-Chiari syndrome (BCS) is caused by obstruction of major hepatic veins. These patients may progress to liver failure and portal hypertension over time.2 BCS is related with BD in 5% of cases in western countries, while 9% in Turkey and 13% in Egypt.3,4 However, misdiagnosis or delay of diagnosis is common in patients with BD-related BCS.5 Some of the patients may be asymptomatic, while abdominal pain, jaundice, emesis, hepatomegaly and ascites are frequently present. In these patients, incomplete diagnostic criteria of BD should not delay the treatment. Here, we present a patient hospitalized for elevated liver enzymes and history of lower extremity thrombosis diagnosed later as BD-related BCS responding to combination therapy.
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