Abstract:
BACKGROUND:Budd-Chiari syndrome (BCS) is a rare but fatal disease caused by obstruction in the hepatic venous outflow tract. AIM:To provide an update of the pathophysiology, aetiology, diagnosis, management and follow-up of BCS. METHODS:Analysis of recent literature by using Medline, PubMed and EMBASE databases. RESULTS:Primary BCS is usually caused by thrombosis and is further classified into "classical BCS" type where obstruction occurs within the hepatic vein and "hepatic vena cava BCS" which involves thrombosis of the intra/suprahepatic portion of the inferior vena cava (IVC). BCS patients often have a combination of prothrombotic risk factors. Aetiology and presentation differ between Western and certain Asian countries. Myeloproliferative neoplasms are present in 35%-50% of European patients and are usually associated with the JAK2-V617F mutation. Clinical presentation is diverse and BCS should be excluded in any patient with acute or chronic liver disease. Non-invasive imaging (Doppler ultrasound, computed tomography, or magnetic resonance imaging) usually provides the diagnosis. Liver biopsy should be obtained if small vessel BCS is suspected. Stepwise management strategy includes anticoagulation, treatment of identified prothrombotic risk factors, percutaneous revascularisation and transjugular intrahepatic portosystemic stent shunt to re-establish hepatic venous drainage, and liver transplantation in unresponsive patients. This strategy provides a 5-year survival rate of nearly 90%. Long-term outcome is influenced by any underlying haematological condition and development of hepatocellular carcinoma. CONCLUSIONS:With the advent of newer treatment strategies and improved understanding of BCS, outcomes in this rare disease have improved over the last three decades. An underlying haematological disorder can be the major determinant of outcome.
journal_name
Aliment Pharmacol Therjournal_title
Alimentary pharmacology & therapeuticsauthors
Khan F,Armstrong MJ,Mehrzad H,Chen F,Neil D,Brown R,Cain O,Tripathi Ddoi
10.1111/apt.15149subject
Has Abstractpub_date
2019-04-01 00:00:00pages
840-863issue
7eissn
0269-2813issn
1365-2036journal_volume
49pub_type
杂志文章,评审abstract:BACKGROUND:Liver cirrhosis is characterized by a silent phase until decompensation, which is defined by ascites, bleeding from esophageal varices or hepatic encephalopathy. AIM:To compare the clinical course, patterns of survival and causes of death by etiology during long-term follow-up in a large population-based co...
journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章
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journal_title:Alimentary pharmacology & therapeutics
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章,多中心研究,随机对照试验
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章,随机对照试验
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 临床试验,杂志文章,随机对照试验
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abstract:BACKGROUND:Serum hepatitis B virus (HBV) RNA may reflect intrahepatic HBV replication. Novel anti-viral drugs have shown potent HBV RNA decline without concomitant hepatitis B surface antigen (HBsAg) decrease. How this relates to off-treatment response is yet unclear. AIM:To study the degree of on-treatment viral anti...
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章,多中心研究,随机对照试验
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章,评审
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章,meta分析,评审
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章,meta分析,评审
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更新日期:2013-12-01 00:00:00
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 临床试验,杂志文章,随机对照试验
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章
doi:10.1111/apt.12195
更新日期:2013-02-01 00:00:00
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pub_type: 杂志文章
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更新日期:2013-02-01 00:00:00
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章
doi:10.1046/j.1365-2036.2003.01534.x
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章
doi:10.1111/j.1365-2036.1990.tb00457.x
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章,meta分析
doi:10.1046/j.1365-2036.2001.00937.x
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journal_title:Alimentary pharmacology & therapeutics
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journal_title:Alimentary pharmacology & therapeutics
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 临床试验,杂志文章,随机对照试验
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章,多中心研究
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更新日期:2017-03-01 00:00:00
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pub_type: 杂志文章
doi:10.1111/j.1365-2036.2006.02958.x
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章
doi:10.1046/j.1365-2036.2002.01233.x
更新日期:2002-05-01 00:00:00
abstract:BACKGROUND:While current medications used to treat patients with chronic hepatitis C virus (HCV) effectively produce sustained viral response (SVR), postponement of therapy is often times attributed to patient perceptions of unfavourable outcomes. However, an instrument to assess patient perceptions of therapy (i.e. tr...
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pub_type: 杂志文章
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更新日期:2013-03-01 00:00:00
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章
doi:10.1046/j.1365-2036.2002.01327.x
更新日期:2002-10-01 00:00:00
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journal_title:Alimentary pharmacology & therapeutics
pub_type: 杂志文章,多中心研究,随机对照试验
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更新日期:2010-08-01 00:00:00