Simoes Camila C, Ghouri Yezaz A, Merwat Shehzad N, Stevenson Heather L
Department of Pathology, University of Texas Medical Branch, Galveston, Texas, USA.
Department of Internal Medicine, University of Texas Medical Branch, Galveston, Texas, USA.
BMJ Case Rep. 2018 Jan 17;2018:bcr-2017-222946. doi: 10.1136/bcr-2017-222946.
Budd-Chiari syndrome (BCS) is characterised by obstruction of hepatic venous outflow and may be triggered by the prothrombotic state associated with inflammatory bowel disease (IBD). We reported a case of Crohn's disease (CD) that presented with anasarca, ascites, symptomatic hepatomegaly, elevated liver enzymes, increased prothrombin time and low albumin. Oesophagogastroduodenoscopy and colonoscopy confirmed active CD. Abdominal CT showed hepatic vein thrombosis. Liver biopsy revealed severe perivenular sinusoidal dilation with areas of hepatocyte dropout, bands of hepatocyte atrophy and centrizonal fibrosis, suggestive of BCS. The patient was treated with steroids for CD and systemic anticoagulants for BCS. His liver function and enzymes normalised, and he reported symptomatic improvement. The precise mechanism responsible for increased hypercoagulability in IBD remains unclear. Early recognition and treatment for possible thrombotic complications of CD is critical to prevent potentially fatal events like pulmonary embolism or liver failure.
布加综合征（BCS）的特征是肝静脉流出道梗阻，可能由与炎症性肠病（IBD）相关的血栓前状态引发。我们报告了一例克罗恩病（CD）患者，该患者出现全身性水肿、腹水、有症状的肝肿大、肝酶升高、凝血酶原时间延长和白蛋白降低。食管胃十二指肠镜检查和结肠镜检查确诊为活动性CD。腹部CT显示肝静脉血栓形成。肝活检显示严重的静脉周围窦性扩张，伴有肝细胞缺失区域、肝细胞萎缩带和中央区纤维化，提示布加综合征。该患者接受了针对CD的类固醇治疗和针对BCS的全身性抗凝治疗。他的肝功能和肝酶恢复正常，且报告症状有所改善。IBD中导致高凝状态增加的确切机制尚不清楚。早期识别和治疗CD可能的血栓并发症对于预防肺栓塞或肝衰竭等潜在致命事件至关重要。
