The Experts below are selected from a list of 267 Experts worldwide ranked by ideXlab platform
Simon Olliff - One of the best experts on this subject based on the ideXlab platform.
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favourable medium term outcome following hepatic vein recanalisation and or transjugular intrahepatic portosystemic shunt for budd chiari Syndrome
Gut, 2006Co-Authors: C E Eapen, Simon Olliff, Dimitris Velissaris, Mathis Heydtmann, Bridget Gunson, E. EliasAbstract:Background: We report our experience with management of patients with Budd Chiari Syndrome over the past two decades. In 1996 we described a novel approach involving recanalisation of hepatic veins by combined percutaneous and transvenous approaches. This was incorporated into an algorithm published in 1999 in which our preferred treatment for all cases of Budd Chiari Syndrome with short segment occlusion or stenosis of the hepatic veins involves recanalisation of the hepatic veins by transvenous or combined percutaneous-transvenous approaches. In symptomatic Budd Chiari Syndrome where recanalisation is not possible, we perform transjugular intrahepatic portosystemic shunts (TIPS) because TIPS decompresses the portal circulation directly in an adjustable way. In this series of patients with Budd Chiari Syndrome treated with radiological interventions alone, we assess their medium term outcome using two independent objective prognostic indices. Methods: We retrospectively studied 61 patients with non-malignant Budd Chiari Syndrome treated by radiological intervention alone in our centre. Results: Actuarial survival for the entire cohort at one year and five years was 94% and 87%, respectively. Survival of our patients with mild disease (according to the Murad classification) was 100% at one year and at five years, with intermediate disease severity 94% at one year and 86% at five years, and with severe disease 85% at one year and 77% at five years. Conclusion: Management of Budd Chiari Syndrome by interventional radiology resulted in excellent medium term survival for patients in all categories of disease severity.
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Transjugular intrahepatic portosystemic shunt in the management of Budd Chiari Syndrome
European journal of gastroenterology & hepatology, 2006Co-Authors: Simon OlliffAbstract:Budd Chiari Syndrome presents with a wide range of severity and duration of symptoms. Transjugular intrahepatic portosystemic shunt has been used to treat selected Budd Chiari Syndrome patients for several years. The technique of transjugular intrahepatic portosystemic shunt may be more challenging than in cirrhosis because of hepatic vein occlusion. Covered transjugular intrahepatic portosystemic shunt stents have reduced the requirement for follow-up interventions. Transjugular intrahepatic portosystemic shunt has been a successful bridge to liver transplant for Budd Chiari Syndrome but is the definitive treatment in many cases. Patient selection is important to determine who will benefit from transjugular intrahepatic portosystemic shunt or other treatments such as hepatic vein recanalization or liver transplant.
E. Elias - One of the best experts on this subject based on the ideXlab platform.
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favourable medium term outcome following hepatic vein recanalisation and or transjugular intrahepatic portosystemic shunt for budd chiari Syndrome
Gut, 2006Co-Authors: C E Eapen, Simon Olliff, Dimitris Velissaris, Mathis Heydtmann, Bridget Gunson, E. EliasAbstract:Background: We report our experience with management of patients with Budd Chiari Syndrome over the past two decades. In 1996 we described a novel approach involving recanalisation of hepatic veins by combined percutaneous and transvenous approaches. This was incorporated into an algorithm published in 1999 in which our preferred treatment for all cases of Budd Chiari Syndrome with short segment occlusion or stenosis of the hepatic veins involves recanalisation of the hepatic veins by transvenous or combined percutaneous-transvenous approaches. In symptomatic Budd Chiari Syndrome where recanalisation is not possible, we perform transjugular intrahepatic portosystemic shunts (TIPS) because TIPS decompresses the portal circulation directly in an adjustable way. In this series of patients with Budd Chiari Syndrome treated with radiological interventions alone, we assess their medium term outcome using two independent objective prognostic indices. Methods: We retrospectively studied 61 patients with non-malignant Budd Chiari Syndrome treated by radiological intervention alone in our centre. Results: Actuarial survival for the entire cohort at one year and five years was 94% and 87%, respectively. Survival of our patients with mild disease (according to the Murad classification) was 100% at one year and at five years, with intermediate disease severity 94% at one year and 86% at five years, and with severe disease 85% at one year and 77% at five years. Conclusion: Management of Budd Chiari Syndrome by interventional radiology resulted in excellent medium term survival for patients in all categories of disease severity.
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New approaches to the Budd‐Chiari Syndrome
Journal of gastroenterology and hepatology, 1996Co-Authors: A. E. A. Mahmoud, E. EliasAbstract:Recent research has led to an improved understanding of the aetiology of Budd-Chiari Syndrome in some patients. Fresh approaches and technical developments within methods of radiological intervention have added more effective options to its treatment. In this editorial we aim to summarize our understanding of the role of new aetiologies and new therapeutic approaches in the Budd-Chiari Syndrome.
Dominique-charles Valla - One of the best experts on this subject based on the ideXlab platform.
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Small hepatic veins Budd–Chiari Syndrome
Journal of Thrombosis and Thrombolysis, 2014Co-Authors: Oliviero Riggio, Aurélie Plessier, Dominique-charles Valla, Chiara Marzano, Alessia Papa, Chiara Pasquale, Maria Ludovica Gasperini, Antonietta Gigante, Antonio AmorosoAbstract:Budd–Chiari Syndrome is a rare disorder characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atrio-caval junction, in the absence of heart failure or constrictive pericarditis. Various imaging modalities are available for investigating the gross hepatic vascular anatomy but there are rare forms of this disease where the obstruction is limited to the small intrahepatic veins, with normal appearance of the large hepatic veins at imaging. In this cases only a liver biopsy can demonstrate the presence of a small vessels outflow block. We report two cases of small hepatic veins Budd–Chiari Syndrome.
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Budd-Chiari Syndrome.
Seminars in Liver Disease, 2008Co-Authors: Aurélie Plessier, Dominique-charles VallaAbstract:Primary Budd-Chiari Syndrome is related to thrombosis of hepatic veins or the terminal portion of the inferior vena cava. This rare disease is usually caused by multiple concurrent factors, including acquired and inherited thrombophilias. Half of the patients with primary Budd-Chiari Syndrome are affected with a myeloproliferative disease, the recognition of which is largely based on the assessment of V617F Janus tyrosine kinase 2 (JAK2) mutation in peripheral granulocytes. A diagnosis of Budd-Chiari Syndrome should be considered in any patient presenting with acute or chronic liver disease, as clinical manifestations are extremely diverse. Spontaneous outcome in symptomatic patients is poor. Diagnosis can be made in most patients noninvasively when imaging shows venous obstruction and/or collaterals. A treatment strategy is recommended where anticoagulation is given first, followed by angioplasty when appropriate, then TIPS in patients not responding to previous measure, and finally liver transplantation. This strategy has achieved 5-year survival rates close to 90%.
Aurélie Plessier - One of the best experts on this subject based on the ideXlab platform.
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Small hepatic veins Budd–Chiari Syndrome
Journal of Thrombosis and Thrombolysis, 2014Co-Authors: Oliviero Riggio, Aurélie Plessier, Dominique-charles Valla, Chiara Marzano, Alessia Papa, Chiara Pasquale, Maria Ludovica Gasperini, Antonietta Gigante, Antonio AmorosoAbstract:Budd–Chiari Syndrome is a rare disorder characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atrio-caval junction, in the absence of heart failure or constrictive pericarditis. Various imaging modalities are available for investigating the gross hepatic vascular anatomy but there are rare forms of this disease where the obstruction is limited to the small intrahepatic veins, with normal appearance of the large hepatic veins at imaging. In this cases only a liver biopsy can demonstrate the presence of a small vessels outflow block. We report two cases of small hepatic veins Budd–Chiari Syndrome.
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Budd-Chiari Syndrome.
Seminars in Liver Disease, 2008Co-Authors: Aurélie Plessier, Dominique-charles VallaAbstract:Primary Budd-Chiari Syndrome is related to thrombosis of hepatic veins or the terminal portion of the inferior vena cava. This rare disease is usually caused by multiple concurrent factors, including acquired and inherited thrombophilias. Half of the patients with primary Budd-Chiari Syndrome are affected with a myeloproliferative disease, the recognition of which is largely based on the assessment of V617F Janus tyrosine kinase 2 (JAK2) mutation in peripheral granulocytes. A diagnosis of Budd-Chiari Syndrome should be considered in any patient presenting with acute or chronic liver disease, as clinical manifestations are extremely diverse. Spontaneous outcome in symptomatic patients is poor. Diagnosis can be made in most patients noninvasively when imaging shows venous obstruction and/or collaterals. A treatment strategy is recommended where anticoagulation is given first, followed by angioplasty when appropriate, then TIPS in patients not responding to previous measure, and finally liver transplantation. This strategy has achieved 5-year survival rates close to 90%.
Mariano Volpacchio - One of the best experts on this subject based on the ideXlab platform.
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Eculizumab in paroxysmal nocturnal hemoglobinuria with Budd-Chiari Syndrome progressing despite anticoagulation
Experimental Hematology & Oncology, 2012Co-Authors: Andrés Brodsky, Octavio Mazzocchi, Fabiana Sánchez, Gus Khursigara, Suneil Malhotra, Mariano VolpacchioAbstract:Paroxysmal nocturnal hemoglobinuria (PNH) is a progressive, life-threatening disorder characterized by chronic intravascular hemolysis caused by uncontrolled complement activation. Hepatic vein thrombosis (Budd-Chiari Syndrome) is common in PNH patients. This case report describes the response to eculizumab (a humanized monoclonal antibody that inhibits terminal complement activation) in a 25-year-old male with progressive liver function deterioration despite standard anticoagulation therapy and transjugular intrahepatic porto-systemic shunt. The patient presented with anemia, severe thrombocytopenia, headache, abdominal pain, and distention. He was diagnosed with PNH, cerebral vein thrombosis, and Budd-Chiari Syndrome. Despite adequate anticoagulation, diuretic administration, and placement of a transjugular shunt, additional thrombotic events and progressive liver damage were observed. Eculizumab therapy was initiated, resulting in rapid blockade of intravascular hemolysis, increased platelet counts, ascites resolution, and liver function recovery, all of which are presently sustained. Since starting eculizumab the patient has had no further thrombotic events and his quality of life has dramatically improved. This is the first report to confirm the role of complement-mediated injury in the progression of Budd-Chiari Syndrome in a patient with PNH. This case shows that terminal complement blockade with eculizumab can reverse progressive thromboses and hepatic failure that is unresponsive to anticoagulation therapy and suggests that early initiation of eculizumab should be included in the therapeutic regimen of patients with PNH-related Budd-Chiari Syndrome.
