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布加综合征患者经颈静脉肝内门体分流术治疗后的长期生存情况

Long Term Survival of Patients Undergoing TIPS in Budd-Chiari Syndrome.

作者信息

Sonavane Amey D, Amarapurkar Deepak N, Rathod Krantikumar R, Punamiya Sundeep J

机构信息

Post-graduate Resident, Department of Gastroenterology, Bombay Hospital and Institute of Medical Sciences, Mumbai, India.

Consultant Gastroenterologist, Department of Gastroenterology, Bombay Hospital and Institute of Medical Sciences, Mumbai, India.

出版信息

J Clin Exp Hepatol. 2019 Jan-Feb;9(1):56-61. doi: 10.1016/j.jceh.2018.02.008. Epub 2018 Mar 1.

Abstract

BACKGROUND

There has been significant improvement in understanding the etiology and management of Budd-Chiari Syndrome (BCS). Patients with chronic or acute-on-chronic BCS need radiological interventions in the form of angioplasty, hepatic vein/inferior vena cava stenting or Transjugular Intrahepatic Portosystemic Shunt (TIPS). Data regarding the long term follow up of patients undergoing TIPS is limited. We thus prospectively followed-up BCS patients who underwent TIPS at our center.

METHODS

This study included 42 patients with BCS who underwent TIPS with a covered stent between 2004 and 2014. We analyzed the etiology, symptoms, severity, laboratory parameters and imaging pre and post TIPS. All patients underwent surveillance for hepatocellular carcinoma.

RESULTS

Patients demographics included 26 males and 16 females with a mean age of 40.5 years (19-68 years). The mean Model for End-Stage Liver Disease score of the entire cohort was 15.38 (range: 9-25). Thirty-four patients were grouped into Rotterdam Class 2 and remaining into Class 3. There was significant improvement in ascites, gastrointestinal bleed, renal function and transaminase levels post TIPS. There were 11 deaths over the follow-up period - 4 within one month, 2 within six months and the rest after 3 years following TIPS. Median duration from clinical presentation to TIPS was 2.1 weeks and median survival till follow-up was 45.5 months (0-130 months). 33/42 patients underwent TIPS prior to 2013, and their median survival till follow-up was 55 months. Six out of eleven deaths that occurred within six months post-TIPS were before 2006; when the technique of TIPS creation was evolving. The cumulative 1 year, 5 years and 10 years OLT-free survival was 86%, 81% and 76%, respectively. Two patients underwent a liver transplant at 4 and 7 years after TIPS.

CONCLUSION

Our results validate the role of TIPS in the management of patients with BCS. With the accessibility of TIPS, the requirement for liver transplantation has become rare.

摘要

背景

在布加综合征(BCS)的病因理解和治疗方面已有显著进展。慢性或慢性基础上急性发作的BCS患者需要血管成形术、肝静脉/下腔静脉支架置入术或经颈静脉肝内门体分流术(TIPS)等放射介入治疗。关于接受TIPS治疗患者的长期随访数据有限。因此,我们对在本中心接受TIPS治疗的BCS患者进行了前瞻性随访。

方法

本研究纳入了2004年至2014年间42例接受带覆膜支架TIPS治疗的BCS患者。我们分析了TIPS治疗前后的病因、症状、严重程度、实验室参数和影像学表现。所有患者均接受肝细胞癌监测。

结果

患者人口统计学资料包括26例男性和16例女性,平均年龄40.5岁(19 - 68岁)。整个队列的终末期肝病模型平均评分是15.38(范围:9 - 25)。34例患者被归入鹿特丹2级,其余归入3级。TIPS治疗后腹水、胃肠道出血、肾功能和转氨酶水平有显著改善。随访期间有11例死亡——4例在1个月内,2例在6个月内,其余在TIPS治疗后3年。从临床表现到TIPS的中位时间为2.1周,随访的中位生存期为45.5个月(0 - 130个月)。42例患者中有33例在2013年前接受TIPS治疗,其随访的中位生存期为55个月。TIPS治疗后6个月内发生的11例死亡中有6例发生在2006年前;当时TIPS创建技术正在发展。1年、5年和10年无肝移植生存率分别为86%、81%和76%。2例患者在TIPS治疗后4年和7年接受了肝移植。

结论

我们的结果证实了TIPS在BCS患者治疗中的作用。随着TIPS的可及性,肝移植的需求已变得罕见。

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本文引用的文献

1
Survival and prognostic indicators of Budd-Chiari syndrome: a systematic review of 79 studies.布加综合征的生存及预后指标:79项研究的系统评价
Expert Rev Gastroenterol Hepatol. 2015 Jun;9(6):865-75. doi: 10.1586/17474124.2015.1024224. Epub 2015 Mar 10.
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Etiology, management, and outcome of the Budd-Chiari syndrome.布加综合征的病因、治疗及预后
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Management of Budd-Chiari syndrome.布加综合征的管理
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The utility of TIPS in the management of Budd-Chiari syndrome.经颈静脉肝内门体分流术在布加综合征治疗中的应用
Ann Surg. 2005 Jun;241(6):978-81; discussion 982-3. doi: 10.1097/01.sla.0000164180.77824.12.

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