Abstract：
A woman in her mid-20s, a known case of congenital afibrinogenaemia, presented with abdominal pain and distension. She was diagnosed with decompensated liver cirrhosis due to Budd-Chiari syndrome. She underwent deceased donor liver transplantation. Preoperatively, her serum fibrinogen level was undetectable and prothrombin time and international normalised ratio (INR) were unrecordable. Intraoperatively, she was given thromboelastography-guided human fibrinogen concentrate. Postoperatively, her fibrinogen, prothrombin time and INR normalised rapidly. This report summarises the rare occurrence of a complication of hypercoagulability (Budd-Chiari syndrome) in the setting of congenital hypocoagulability (congenital afibrinogenaemia). In this report, we discuss the simultaneous management of these two clinical problems and the curative role of liver transplantation.
摘要：
一个20多岁的女人，一个已知的先天性纤维蛋白原血症病例,表现为腹痛和腹胀。由于Budd-Chiari综合征，她被诊断为失代偿性肝硬化。她接受了已故供体肝移植。术前,她的血清纤维蛋白原水平无法检测到,凝血酶原时间和国际标准化比率(INR)无法记录.术中,她接受了血栓弹力图引导的人纤维蛋白原浓缩物.术后,她的纤维蛋白原,凝血酶原时间和INR迅速恢复正常。该报告总结了在先天性低凝（先天性纤维蛋白原血症）的情况下罕见的高凝并发症（Budd-Chiari综合征）。在这份报告中,我们讨论了这两个临床问题的同时处理和肝移植的治疗作用。
