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蛋白C缺乏所致布加综合征中国患者以急性肝衰竭为首发表现:一例报告及文献复习

Acute liver failure as initial presentation in a Chinese patient with Budd-Chiari syndrome due to protein C deficiency: A case report and literature review.

作者信息

Xu Wanling, Tang Wenjing, Yang Weiying, Sun Lichao, Li Wei, Wang Shouqing, Zang Xiuxian

机构信息

Department of Emergency Medicine, The First Hospital of Jilin University, Changchun, Jilin, PR China.

Department of Ultrasound, The First Hospital of Jilin University, Changchun, Jilin, PR China.

出版信息

Heliyon. 2024 Apr 25;10(9):e29776. doi: 10.1016/j.heliyon.2024.e29776. eCollection 2024 May 15.

Abstract

Acute liver failure is an uncommon presentation in the clinic. Common causes for acute liver failure include viral hepatitis and drug-related hepatotoxicity. However, acute liver failure due to Budd-Chiari syndrome is rare. This case highlights the importance of necessary constrast-enhanced imaging studies to rule out vascular etiologies of acute liver failure, in addition to common causes like viral or drug-induced hepatic failure. We present a case of a male Chinese patient who presented with nausea, vomiting, fatigue, and fever after eating a large amount of fatty food. Six days after hospitalization, the patient developed acute liver failure and hepatic encephalopathy. Contrast-enhanced computerized tomography and ultrasound examinations revealed thromboses in the hepatic veins and inferior vena cava. Further testing also showed decreased protein C activity. Therefore, a diagnosis of Budd-Chiari syndrome secondary to protein C deficiency was made. He received supportive care and a transjugular intrahepatic portal shunt. Hepatic function, coagulation panel results, and clinical presentations gradually returned to normal. Budd-Chiari syndrome from protein C deficiency could be a rare but valid cause of acute liver failure in Chinese patients.

摘要

急性肝衰竭在临床上并不常见。急性肝衰竭的常见病因包括病毒性肝炎和药物性肝毒性。然而,由布加综合征导致的急性肝衰竭较为罕见。该病例强调了除病毒性或药物性肝衰竭等常见病因外,进行必要的增强影像学检查以排除急性肝衰竭血管病因的重要性。我们报告一例中国男性患者,该患者在大量进食油腻食物后出现恶心、呕吐、乏力和发热症状。住院六天后,患者发生急性肝衰竭和肝性脑病。增强计算机断层扫描和超声检查显示肝静脉和下腔静脉血栓形成。进一步检查还显示蛋白C活性降低。因此,诊断为继发于蛋白C缺乏的布加综合征。患者接受了支持治疗和经颈静脉肝内门体分流术。肝功能、凝血指标结果及临床表现逐渐恢复正常。蛋白C缺乏所致的布加综合征可能是中国患者急性肝衰竭的一种罕见但确切的病因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/60d6/11068517/1e3268a6b77a/gr1.jpg

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