Review article: a multidisciplinary approach to the diagnosis and management of Budd-Chiari syndrome.

Khan, Faisal and Armstrong, Matthew J and Mehrzad, Homoyon and Chen, Frederick and Neil, Desley and Brown, Rachel and Cain, Owen and Tripathi, Dhiraj (2019) Review article: a multidisciplinary approach to the diagnosis and management of Budd-Chiari syndrome. Alimentary pharmacology & therapeutics. ISSN 1365-2036. This article is available to all UHB staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their UHB Athens login IDs

Full text not available from this repository.
Official URL: https://onlinelibrary.wiley.com/doi/full/10.1111/a...

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.

Item Type: Article
Additional Information: This article is available to all UHB staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their UHB Athens login IDs
Subjects: WI Digestive system. Gastroenterology
Divisions: Planned IP Care > Gastroentrology
Related URLs:
Depositing User: Miss Emily Johnson
Date Deposited: 11 Mar 2019 10:47
Last Modified: 11 Mar 2019 10:47
URI: http://www.repository.heartofengland.nhs.uk/id/eprint/1902

Actions (login required)

View Item View Item