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Acute-on-Chronic Liver Failure in Budd-Chiari Syndrome: Profile and Predictors of Outcome.
Digestive Diseases and Sciences ( IF 3.1 ) Pub Date : 2020-01-02 , DOI: 10.1007/s10620-019-06005-7
Shalimar 1 , Sanchit Sharma 1 , Shivanand R Gamanagatti 2 , Ashish Chauhan 1 , Sudheer Kumar Vuyyuru 1 , Anshuman Elhence 1 , Gyanranjan Rout 1 , Anoop Saraya 1 , Deepak Gunjan 1 , Baibaswata Nayak 1 , Ramesh Kumar 3 , Subrat Kumar Acharya 1
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BACKGROUND AND AIM There is a paucity of data on the clinical presentations and outcome of Budd-Chiari syndrome (BCS) patients presenting as acute-on-chronic liver failure (BCS-ACLF). We aimed to describe the profile and outcomes of endovascular interventions in patients with BCS-ACLF. METHODS All BCS-ACLF patients presenting between October 2007 and April 2019 satisfying the Asian Pacific Association for the Study of the Liver (APASL) definition were studied. We compared 30- , 90- and, 180-day survival among BCS-ACLF patients who underwent endovascular intervention with those who did not, and with a historical cohort of Child-C BCS patients without ACLF who underwent endovascular intervention. RESULTS Twenty-eight (5%) of 553 BCS patients presented as ACLF as per APASL definition. The majority (60.7%) were males, and mean age was 29.6 ± 11.2 years. The most common site of the block was isolated involvement of hepatic veins-HV (68%), followed by combined inferior vena cava (IVC) and HV block (25%) and isolated IVC block (7%). The acute precipitants were stent thrombosis (17.9%), acute HV thrombosis (10.7%), acute viral hepatitis (7.1%), and antituberculosis drug with hepatitis B virus reactivation (3.6%). In 60.7% patients, no acute precipitant could be identified. The 30- , 90- , and 180-day survival in BCS-ACLF post-endovascular intervention (n = 15), BCS-ACLF without endovascular intervention (n = 13), and Child-C BCS without ACLF who underwent endovascular intervention (n = 25) were (93%, 87%, and 87%), (46%, 28%, and 0%) and (96%, 92%, and 88%), respectively (log-rank test, p value < 0.001). On multivariate Cox proportional analysis, endovascular intervention and the presence of hepatic encephalopathy were independent predictors of mortality. CONCLUSION Budd-Chiari syndrome can present as acute-on-chronic liver failure. Endovascular intervention is associated with an improved outcome.

中文翻译:

Budd-Chiari 综合征中的急性慢性肝功能衰竭:结果的概况和预测因素。

背景和目的 关于以慢加急性肝衰竭 (BCS-ACLF) 为表现的布加综合征 (BCS) 患者的临床表现和结果的数据很少。我们旨在描述 BCS-ACLF 患者血管内干预的概况和结果。方法 研究了 2007 年 10 月至 2019 年 4 月期间满足亚太肝脏研究协会 (APASL) 定义的所有 BCS-ACLF 患者。我们比较了接受血管内介入治疗的 BCS-ACLF 患者与未接受血管内介入治疗的 BCS-ACLF 患者的 30 天、90 天和 180 天生存率,以及接受血管内介入治疗的未接受 ACLF 的 Child-C BCS 患者的历史队列。结果 根据 APASL 定义,553 名 BCS 患者中有 28 名 (5%) 表现为 ACLF。大多数 (60.7%) 为男性,平均年龄为 29.6 ± 11 岁。2年。最常见的阻滞部位是孤立的肝静脉-HV 受累(68%),其次是联合下腔静脉(IVC)和 HV 阻滞(25%)和孤立的 IVC 阻滞(7%)。急性诱发因素为支架内血栓形成(17.9%)、急性HV血栓形成(10.7%)、急性病毒性肝炎(7.1%)和乙肝病毒再激活的抗结核药物(3.6%)。在 60.7% 的患者中,没有发现急性沉淀物。BCS-ACLF 血管内介入治疗后(n = 15)、BCS-ACLF 无血管内介入治疗(n = 13)和 Child-C BCS 无 ACLF 接受血管内介入治疗后的 30、90 和 180 天生存率(n = 15)。 n = 25)分别为(93%、87% 和 87%)、(46%、28% 和 0%)和(96%、92% 和 88%)(对数秩检验,p 值< 0.001)。在多变量 Cox 比例分析中,血管内介入治疗和肝性脑病是死亡率的独立预测因素。结论 Budd-Chiari 综合征可表现为慢加急性肝功能衰竭。血管内干预与改善结果相关。
更新日期:2020-01-04
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