Obed Aiman, Bashir Abdalla, Jarrad Anwar
Department of Hepatobiliary and Transplant Surgery, Jordan Hospital, Amman, Jordan.
Department of General and Transplant Surgery, Jordan Hospital, Amman, Jordan.
Am J Case Rep. 2018 Jun 30;19:767-772. doi: 10.12659/AJCR.909694.
BACKGROUND Acute-on-chronic liver failure was first defined within the last 10 years as acute decompensation of chronic liver disease accompanied by multiorgan failure and poor outcome. Budd-Chiari syndrome is a rare and potentially deadly hepatic condition. To the best of our knowledge, this is the first case report of a live liver donor recipient with antiphospholipid antibody syndrome. CASE REPORT A 47-year-old woman from Sudan with acute-on-chronic liver failure and subacute Budd-Chiari syndrome triggered by active pneumonia was evacuated to Amman, Jordan. In Amman, she was transferred to our hospital for liver transplant evaluation. She presented with progressive liver failure, acute kidney failure, acute respiratory failure, and encephalopathy stage IV. Multidisciplinary therapy was initiated with IV anti-infective drugs and optimizing mechanical ventilation. Clinically, we stopped her progressive deterioration after 48 h and she improved slightly in our ICU. Accelerated work-up for donors and recipient was completed and her daughter was selected as a medically appropriate donor despite the fact that she was found to have heterozygote factor V Leiden mutation and antiphospholipid antibody syndrome, similar to her mother. A lifesaving live-donor liver transplantation was carried out after 72 h. Donor and recipient were discharged in good condition with normal liver function and both were discharged on anticoagulant Rivaroxaban 20 mg. CONCLUSIONS We present the first case of a patient with acute-on-chronic liver failure with subacute Budd-Chiari syndrome, which was triggered by bacterial pneumonia and was successfully treated by live-donor liver transplantation from a donor with antiphospholipid antibody syndrome.
急性慢性肝衰竭在过去10年内首次被定义为慢性肝病的急性失代偿,伴有多器官功能衰竭且预后不良。布加综合征是一种罕见且可能致命的肝脏疾病。据我们所知,这是首例关于抗磷脂抗体综合征活体肝供体受者的病例报告。
一名来自苏丹的47岁女性,因活动性肺炎引发急性慢性肝衰竭和亚急性布加综合征,被疏散至约旦安曼。在安曼,她被转至我院进行肝移植评估。她表现为进行性肝衰竭、急性肾衰竭、急性呼吸衰竭和IV期脑病。开始采用静脉注射抗感染药物和优化机械通气的多学科治疗。临床上,48小时后我们阻止了她的病情进展,她在我们的重症监护病房稍有好转。完成了供体和受体的快速检查,尽管发现她的女儿与母亲一样患有杂合子因子V莱顿突变和抗磷脂抗体综合征,但仍被选为合适的医学供体。72小时后进行了挽救生命的活体肝移植。供体和受体肝功能正常,状况良好出院,两人均出院时服用抗凝药利伐沙班20毫克。
我们报告了首例急性慢性肝衰竭合并亚急性布加综合征患者,该患者由细菌性肺炎引发,通过来自抗磷脂抗体综合征供体的活体肝移植成功治疗。