A Case of Live Donor Liver Transplantation in Acute-on-Chronic Liver Failure with Budd-Chiari Syndrome: Donor and Recipient with Antiphospholipid Antibody Syndrome
Challenging differential diagnosis, Management of emergency care, Rare co-existance of disease or pathology
Aiman Obed, Abdalla Bashir, Anwar Jarrad
Department of Hepatobiliary and Transplant Surgery, Jordan Hospital, Amman, Jordan
Am J Case Rep 2018; 19:767-772
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.
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.
Keywords: Budd-Chiari syndrome, Liver Failure, Acute, Liver Transplantation, Pneumonia, Bacterial