About this Research Topic
Budd-Chiari syndrome is a rare hypercoaguable disorder which often causes hepatic vein thrombosis leading to venous outflow obstruction, hepatocyte necrosis and liver failure. Patients may present with acute liver failure from extensive and irreversible necrosis requiring urgent liver transplantation whereas others may present subacutely with decompensated cirrhosis manifesting as jaundice, fatigue and ascites.
Polycythemia rubra vera is one of the most common causes of BCS with Janus kinase-2 mutations present in more than 90% of these patients. Budd Chiari syndrome is also more common is women, particularly if other risk factors for hypercoagulability are present such as oral contraceptive exposure, and usually presents in the third or fourth decade. However, no underlying risk factor is identified in the majority of patients who still require anticoagulation and /or antiplatelets as the absence of a pro-thrombotic state does not preclude its presence.
The management of Budd Chiari syndrome is multidisciplinary, complex and often entails a combination of medical and surgical interventions. This includes urgent anticoagulation, antiproliferative agents with/without anti-platelets for patients with underlying myeloproliferative disorders and portal systemic decompression by endovascular procedures, transjugular intrahepatic shunt, surgical portal-systemic shunting or liver transplantation. However, the choice of procedure often depends on the acuity and severity of the liver disease at presentation and availability of local expertise. For example, patients presenting in acute liver failure from Budd Chiari syndrome will require urgent liver transplantation whereas patients presenting subacutely may be able to be treated medically with anticoagulation, antiproliferative agents, venous decompression and diuretics reserving liver transplantation for those who do not respond to medical treatment.
The goals of this research topic are as follows:
1. Review the latest advances in the evaluation of underlying pro-thrombotic states for patients presenting with Budd Chiari syndrome and its treatment
2. Discuss the decision making process and review the evidence for determining the optimal long-term oral anticoagulation for these patients
3. Summarize the indications for the latest radiological and surgical interventions for Budd Chiari syndrome which include endovascular procedures, transjugular intrahepatic portal systemic shunts, surgical portal systemic shunts and liver transplantation.
We welcome the following articles for this research topic on Budd –Chiari syndrome:
1. Original research
3. Clinical trials
Case reports and short communications or minireviews are not acceptable.
Keywords: Vascular liver disease, Chronic liver disease, Portal hypertension, Thrombosis, Coagulation, MRI, MRCP, MRA, Ultrasound, CT, Imaging
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