Shiraz Transplant Research Center, Shiraz University of Medical sciences, Shiraz, Islamic Republic of Iran.
Health Policy research center, School of Medicine, Institute of Health, Shiraz University of Medical Sciences, Shiraz, Islamic Republic of Iran.
Indian J Gastroenterol. 2021 Jun;40(3):281-286. doi: 10.1007/s12664-020-01139-3. Epub 2021 Mar 20.
BACKGROUND/PURPOSE: Budd-Chiari syndrome (BCS) is a rare, life-threatening disease characterized by hepatic venous outflow obstruction. Liver transplantation (LT) is widely accepted as an effective therapeutic measure for irreversible liver failure due to BCS. There is debate on differences in the post LT course and complications in patients with BCS as compared to non-Budd-Chiari (NBC) patients.
In this retrospective study, data on all patients who received a liver transplant for BCS at the Shiraz Organ Transplantation Center between January 1996 and September 2017 were reviewed and compared to data of a control group who had received liver transplants over the same period but due to other causes (NBC).
Out of 4225 patients who received liver transplants in the study period, 108 had BCS and an age- and gender-matched control group consisted of 108 NBC cases. The mean ± standard deviation (SD) of model for end-stage liver disease (MELD) scores were 19.1 ± 3 and 20 ± 3 for BCS and NBC groups, respectively (p = 0.33). One-, 3-, 5-, and 10-year survival rates in the BCS group were as follows: 82%, 78%, 76%, and 76% compared with the NBC rates of 83%, 83%, 83%, and 76%, respectively (p = 0.556). There was no difference between the two groups in complication rates after 6 months. In the later period, vascular thrombosis was more common in BCS.
Whole-organ LT from deceased donors in patients with BCS had comparable outcomes with LT due to other causes of end-stage liver disease. In most instances, these patients should receive lifelong anticoagulation.
背景/目的：布加氏综合征（BCS）是一种罕见的、危及生命的疾病，其特征为肝静脉流出道阻塞。由于 BCS 导致的不可逆转的肝衰竭，肝移植（LT）被广泛认为是一种有效的治疗措施。BCS 患者与非布加氏（NBC）患者在 LT 后的病程和并发症方面存在争议。
在这项回顾性研究中，回顾了 1996 年 1 月至 2017 年 9 月在设拉子器官移植中心接受 LT 治疗的所有 BCS 患者的数据，并与同期因其他原因（NBC）接受 LT 的对照组数据进行了比较。
在研究期间接受 LT 的 4225 例患者中，有 108 例患有 BCS，年龄和性别匹配的对照组有 108 例 NBC 患者。BCS 和 NBC 组的终末期肝病模型（MELD）评分的平均值±标准差（SD）分别为 19.1±3 和 20±3（p=0.33）。BCS 组的 1、3、5 和 10 年生存率分别为 82%、78%、76%和 76%，而 NBC 组的相应生存率分别为 83%、83%、83%和 76%（p=0.556）。两组患者在 6 个月后的并发症发生率无差异。在后期，BCS 中血管血栓形成更为常见。
来自已故供体的全器官 LT 在 BCS 患者中的结果与因其他原因导致的终末期肝病的 LT 相当。在大多数情况下，这些患者应接受终身抗凝治疗。
