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Budd-Chiari Syndrome (Budd Chiari Syndrome)

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A condition in which the hepatic venous outflow is obstructed anywhere from the small HEPATIC VEINS to the junction of the INFERIOR VENA CAVA and the RIGHT ATRIUM. Usually the blockage is extrahepatic and caused by blood clots (THROMBUS) or fibrous webs. Parenchymal FIBROSIS is uncommon.

Also Known As:

Budd Chiari Syndrome; Hepatic Vein Thrombosis; Chiari Syndrome; Chiaris Syndrome; Hepatic Vein Thromboses; Thromboses, Hepatic Vein; Thrombosis, Hepatic Vein; Vein Thromboses, Hepatic; Vein Thrombosis, Hepatic; Chiari's Syndrome; Hepatic Venous Outflow Obstruction

Networked: 634 relevant articles (24 outcomes, 27 trials/studies) for this Disease, Comments

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Thrombosis (Thrombus)
2.	Ascites
3.	Pathologic Constriction (Stenosis)
4.	Liver Diseases (Liver Disease)
5.	Antiphospholipid Syndrome (Antiphospholipid Antibody Syndrome)

Experts

1.	Burroughs, Andrew K: 6 articles (01/2009 - 06/2003)
2.	Plessier, Aurélie: 5 articles (11/2009 - 04/2005)
3.	Patch, David: 5 articles (01/2009 - 06/2003)
4.	Valla, Dominique: 4 articles (11/2009 - 02/2003)
5.	Molmenti, Ernesto P: 4 articles (06/2005 - 10/2002)
6.	Shibata, Toshiya: 3 articles (06/2009 - 04/2002)
7.	Egawa, Hiroto: 3 articles (06/2009 - 04/2002)
8.	Neuhaus, Peter: 3 articles (02/2008 - 05/2005)
9.	Darwish Murad, Sarwa: 3 articles (02/2008 - 02/2007)
10.	Belghiti, Jacques: 3 articles (01/2007 - 02/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Budd-Chiari Syndrome:

1.	Polytetrafluoroethylene (Teflon)FDA Link 11/01/2004 - "PTFE-covered stents are preferable in patients with Budd-Chiari Syndrome." 11/01/2004 - "PTFE-covered stents improve TIPS patency in Budd-Chiari syndrome." 09/01/1997 - "This report describes a patient with Budd-Chiari syndrome who was operated on successfully by means of shunt formation with polytetrafluoroethylene graft between the inferior vena cava (IVC) and right atrium. " 09/01/1997 - "Retrohepatic cavoatrial shunt with a ringed polytetrafluoroethylene graft for the Budd-Chiari syndrome. " 05/01/1989 - "Five patients with the Budd-Chiari syndrome and caval occlusion and/or obstruction have had mesoatrial shunts constructed using externally supported polytetrafluoroethylene grafts. " Order ALL the reference details at left...
2.	AnticoagulantsIBA 01/01/2007 - "A patient with the acute Budd-Chiari syndrome was administered myelosuppressants and anticoagulants on time so reperfusion was restored. " 01/01/2005 - "Putting all data together it could be speculated that weight reduction (along with anticoagulants) considerably activated endogenous fibrinolysis, resulting in spontaneous resolution of Budd-Chiari syndrome. " 01/01/2009 - "Hepatic function was stabilized in parallel to controlling ulcerative colitis and the patient is in good health until now receiving maintenance therapy for ulcerative colitis and anticoagulants for Budd-Chiari syndrome." 07/01/1994 - "In conclusion patients with Budd-Chiari syndrome should be tested for lupus anticoagulants and anticardiolipin antibodies, Budd-Chiari syndrome resulting from this cause may have a good response to treatment with oral anticoagulants; this treatment should be maintained permanently, and pregnancy in such patients may initiate serious difficulties." 03/01/2003 - "CONCLUSION: Hepatic synthesis of coagulation factors and anticoagulants is reduced in Budd-Chiari syndrome; this may play a role in recurrence of thrombosis." Order ALL the reference details at left...
3.	AldosteroneIBA 07/01/1964 - "VI. THE EFFECTS OF HEPATIC VENOUS OUTFLOW OBSTRUCTION AND ASCITES ON ALDOSTERONE SECRETION." 06/01/1997 - "RESULTS: In the experimental studies, side-to-side portacaval shunt permanently relieved severe ascites, reduced the 13-fold increase in thoracic duct lymph flow rate to almost normal, and abolished the aldosterone hypersecretory response to minimal hepatic venous outflow obstruction. " 06/01/1997 - "STUDY DESIGN: In the experimental studies, hepatic venous outflow obstruction and massive ascites were produced in dogs by ligation of the hepatic veins, and the effect of portacaval shunts on ascites, thoracic duct lymph flow, and aldosterone secretion were measured. " Order ALL the reference details at left...
4.	Lupus Coagulation Inhibitor (Lupus Anticoagulant)IBA 08/01/2003 - "The case of a 42-year-old female with Budd-Chiari syndrome (BCS) and lupus anticoagulant is reported. " 04/01/1991 - "Association of the Budd-Chiari syndrome with lupus anticoagulant. " 01/01/1986 - "An active search for the 'lupus' anticoagulant should be performed to lower the percentage of apparently idiopathic cases of Budd-Chiari syndrome." 01/01/1986 - "Three patients with Budd-Chiari syndrome in association with the 'lupus' anticoagulant are described. " 08/01/1985 - "[A case of Budd-Chiari syndrome with lupus anticoagulant]" Order ALL the reference details at left...
5.	Heparin (Liquaemin)FDA LinkGeneric 01/01/1997 - "Therapeutic options of Budd-Chiari syndrome include anticoagulation with heparin, fibrinolysis followed by oral anticoagulation, and appropriate treatment of the underlying PMD. " 04/01/2001 - "Budd--Chiari syndrome and heparin-induced thrombocytopenia." 01/01/2001 - "[Challenging perioperative anticoagulation in a patient with Budd-Chiari syndrome and heparin-induced thrombocytopenia]" 01/01/2000 - "This case shows that heparin-induced thrombocytopenia II is a possible cause of the Budd-Chiari syndrome." 01/01/2000 - "Segmental hepatic vein thrombosis associated with heparin-induced thrombocytopenia II." Order ALL the reference details at left...
6.	Anticardiolipin AntibodiesIBA 01/01/1998 - "Anticardiolipin antibody-related Budd-Chiari syndrome: report of a case." 07/01/1994 - "Budd-Chiari syndrome in a young patient with anticardiolipin antibodies: need for prolonged anticoagulant treatment." 03/01/2000 - "This is the first reported case of a young patient with ulcerative colitis who developed acute Budd-Chiari syndrome and was found positive for anticardiolipin antibodies. " 03/01/2000 - "Budd-Chiari syndrome in a patient with ulcerative colitis: association with anticardiolipin antibodies." 06/01/1998 - "Significance of elevated IgG anticardiolipin antibody levels in patients with Budd-Chiari syndrome." Order ALL the reference details at left...
7.	Cyclosporine (Ciclosporin)FDA LinkGeneric 01/01/2003 - "Case 2 was a female patient with advanced acute lymphoblastic leukaemia (ALL) with post bone marrow transplantation (BMT) acute haemolytic-uraemic syndrome (HUS) secondary to cyclosporin A (Cy A), cytomegalovirus (CMV) infection, severe nosocomial pneumonia, acute renal failure (ARF) treated with continuous haemofiltration and acute veno-occlusive disease resulting in Budd-Chiari syndrome. " 04/01/2004 - "Early treatment with heparin and cyclosporin administered intravenously induce a remission of intestinal disease and total disappearance of hepatic vein thrombosis. " Order ALL the reference details at left...
8.	Urokinase-Type Plasminogen Activator (Urokinase)FDA Link 11/01/1983 - "Urokinase treatment of Budd-Chiari syndrome." 09/01/1994 - "The hepatic venous outflow obstruction was relieved by angioplasty and thrombolytic therapy with use of local infusions of urokinase into the clot. " Order ALL the reference details at left...
9.	Factor VIIa (Activated Factor VII)IBA 04/01/2009 - "Additionally, therapeutic options have expanded with availability of drugs like activated factor VII and eltrombopeg and improved expertise in procedures like TIPSS for treatment of the Budd-Chiari syndrome. " Order ALL the reference details at left...
10.	ErythropoietinIBA 02/01/1997 - "BACKGROUND/AIMS: Erythropoietin-independent endogenous growth of erythroid colony from bone marrow cells has been shown in many patients with Budd Chiari syndrome in earlier studies. " 04/01/2000 - "Patients were considered as having occult PV if they had spontaneous erythroid cell growth in the absence of erythropoietin and with no features of overt PV. RESULTS: Cells from ten patients with Budd-Chiari syndrome demonstrated spontaneous erythroid cell growth; eight patients (32%) were found to have occult PV and two patients had overt PV. None of the controls had spontaneous erythroid growth. " 09/01/1985 - "High concentrations of serum erythropoietin were observed in 6 of 7 patients with acute primary Budd-Chiari syndrome. " 09/01/1985 - "Polycythemia and the Budd-Chiari syndrome: study of serum erythropoietin and bone marrow erythroid progenitors." 07/01/2006 - "Elevated serum erythropoietin levels in patients with Budd-Chiari syndrome secondary to polycythemia vera: clinical implications for the role of JAK2 mutation analysis." Order ALL the reference details at left...

Therapies and Procedures

1.	Stents 10/01/2008 - "Early posttransplant hepatic venous outflow obstruction: Long-term efficacy of primary stent placement." 02/01/2003 - "Budd-Chiari syndrome due to prothrombotic disorder: mid-term patency and efficacy of endovascular stents." 01/01/1999 - "The object of this study was to evaluate use of ultrasound (US) as guidance for IVC stent placement for the management of Budd-Chiari syndrome. " 05/01/1991 - "This study presents a patient with clinical resolution of the Budd-Chiari syndrome after placement of expandable metallic stents in the inferior vena cava and hepatic veins. " 01/01/2010 - "Transjugular intrahepatic portocaval shunt placed through the strut of an inferior vena cava stent in a patient with Budd-Chiari syndrome: a technical modification." Order ALL the reference details at left...
2.	Surgical Portasystemic Shunt (Portosystemic Shunt) 09/01/2007 - "In patients with acute or subacute Budd-Chiari syndrome uncontrolled by medical therapy, transjugular intrahepatic portosystemic shunt is highly effective with no complications and an associated mid-term clinical success." 08/01/2008 - "PURPOSE: This study was performed to evaluate the safety and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of patients affected by Budd-Chiari syndrome (BCS). " 09/01/2007 - "The aim of our study was to present two patients with Budd-Chiari syndrome treated with transjugular intrahepatic portosystemic shunt. " 07/01/1999 - "The aim of this study was to assess the factors, including surgical portosystemic shunts, which affect survival in adults with Budd-Chiari syndrome. " 11/01/2008 - "Long-term follow-up after successful transjugular intrahepatic portosystemic shunt placement in a pediatric patient with Budd-Chiari syndrome." Order ALL the reference details at left...
3.	Balloon Angioplasty (Angioplasty, Transluminal) 05/01/2002 - "Percutaneous balloon angioplasty is a safe and effective therapy for Budd-Chiari syndrome caused by IVC obstruction, therefore should be the first choice of treatment for this condition." 05/01/1990 - "This experience suggests that balloon angioplasty is a safe and effective treatment for patients with Budd-Chiari syndrome. " 04/01/1987 - "It is concluded that percutaneous transluminal angioplasty is a safe and effective mode of therapy in the management of the Budd-Chiari syndrome due to membranous obstruction of the hepatic portion of the inferior vena cava or the hepatic veins." 05/01/2002 - "This study was to evaluate the clinical effects of percutaneous balloon angioplasty of Budd-Chiari syndrome (BCS) caused by inferior vena cava (IVC) obstruction. " 12/01/1996 - "OBJECTIVES: This study sought to report the long-term result (up to 8 years) of percutaneous transluminal balloon angioplasty (PTBA) for Budd-Chiari syndrome (BCS) caused by membranous obstruction of the inferior vena cava (MOVC). " Order ALL the reference details at left...
4.	Transplants (Transplant) 07/15/2008 - "OBJECTIVE: To evaluate the therapeutic efficacy of interposition of graft meso-cavo-atri shunt (MCAS) for the treatment of Budd-Chiari syndrome (BCS) with occlusion of both inferior cava vena (ICV) and hepatic veins (HVs). " 11/15/2009 - "Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survival." 09/01/2009 - "A three-decade experience of radical open endvenectomy with pericardial patch graft for correction of Budd-Chiari syndrome." 07/15/2008 - "[Treatment of Budd-Chiari syndrome with occlusion of both inferior vena cava and hepatic veins lesions by interposition of graft meso-caval-atrial shunt]" 11/01/2005 - "Acute Budd Chiari syndrome post-transplant can satisfactorily be resolved with the Belghiti technique, although it is preferable to take prophylactic measures to avoid it." Order ALL the reference details at left...
5.	Surgical Portacaval Shunt (Portacaval Anastomosis) 10/01/1978 - "It is concluded that side-to-side portacaval shunt, which decompresses the liver by converting the portal vein into an outflow tract, provides effective treatment of the Budd-Chiari syndrome when the occlusive process is confined to the hepatic veins." 01/01/2009 - "CASE PRESENTATION: A 37-year old lady underwent side-to-side portacaval shunt for Budd Chiari syndrome. " 04/01/1999 - "This report presents the largest reported experience of Behçet's disease-related Budd-Chiari syndrome confined to the hepatic veins, and results of treatment by side-to-side portacaval shunt (SSPCS). " 04/01/1999 - "Budd-Chiari syndrome caused by Behçet's disease: treatment by side-to-side portacaval shunt." 01/01/1989 - "This is a report of a long term prospective study of 13 seriously ill patients with Budd-Chiari syndrome as a result of occlusion of the hepatic veins who were treated by side to side portacaval shunt from four to 78 weeks after the onset of symptoms and who were under observation for three to 16 years. " Order ALL the reference details at left...

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