Bas Koray, Yaprak Onur, Dayangac Murat, Ulusoy Onur Levent, Dogusoy Gulen Bulbul, Yuzer Yildiray, Tokat Yaman
Department of General Surgery, Florence Nightingale Hospital, Organ Transplantation Center, Istanbul, Turkey.
Exp Clin Transplant. 2012 Apr;10(2):172-5. doi: 10.6002/ect.2011.0044. Epub 2012 Jan 25.
Budd-Chiari syndrome is a rare but life-threatening disorder characterized by obstruction of the hepatic venous outflow. Treatment depends on the underlying cause, the location, and extent of the obstruction, and the functional capacity of the liver. A stepwise therapeutic approach is commonly accepted. When all other therapy options are unsuccessful, or in case of end-stage liver disease, transplant should be considered. We present case reports of 3 patients with Budd-Chiari syndrome who underwent living-donor liver transplant. Characteristic features of Budd-Chiari syndrome, diagnostic and therapeutic interventions, complications, and overall outcomes are discussed. We believe that when a deceased donor graft is unavailable, a living-donor liver transplant can be a safe option for patients with end-stage liver disease associated with Budd-Chiari syndrome.
布加综合征是一种罕见但危及生命的疾病,其特征为肝静脉流出道梗阻。治疗取决于潜在病因、梗阻的部位和范围以及肝脏的功能状态。通常采用逐步治疗方法。当所有其他治疗选择均无效或处于终末期肝病时,应考虑进行移植。我们报告了3例接受活体肝移植的布加综合征患者的病例。讨论了布加综合征的特征、诊断和治疗干预措施、并发症及总体结果。我们认为,当无法获得尸体供肝时,活体肝移植对于与布加综合征相关的终末期肝病患者可能是一种安全的选择。