Budd Chiari syndrome

Budd Chiari syndrome

A condition defined by obstruction of the hepatic veins and its clinical manifestations, regardless of the cause (except congestive heart failure), where the obstruction is either within the liver on in the inferior vena cava between the liver and the right atrium.
 
Aetiology
Thrombosis in the hepatic veins (majority of cases) due to coagulation secondary to polycythaemia vera, pregnancy, postpartum, oral contraceptive use, intravascular lesions including malignancy, myeloproliferative disorders, paroxysmal nocturnal haemoglobinuria (PNH), infection (e.g., schistosomiasis), intravascular webs.
 
Clinical findings
Abdominal pain, ascites, jaundice, hepatomegaly, and eventually cirrhosis, liver failure and hepatic encephalopathy.

Lab
Abnormal LFTs, increased liver enzymes.

DiffDx
Liver failure, GI bleeding, starvation.
 
Management
Unsatisfactory.
References in periodicals archive ?
Objective: To determine aetiology clinical presentation and predictors of survival in Budd Chiari Syndrome patients.
Methods: The prospective observational study based on non-probability convenient sampling was conducted at the Sindh Institute of Urology and Transplantation (SIUT) Karachi and comprised Budd Chiari Syndrome patients between January 2004 and December 2013.
Conclusion: Budd Chiari Syndrome affected young patients more frequently and was associated with high mortality.
A number of syndromes overlapped with SLE like Sjogren's syndrome, Scleroderma, Rheumatoid arthritis, Antiphospholipid syndrome and Budd Chiari syndrome.
A number of syndromes overlapped with SLE like Sjogren's syndrome, Scleroderma, Rheumatoid arthritis, Anti-phospholipid syndrome and Budd Chiari syndrome.
Budd Chiari syndrome (BCS) is a rare disorder that results from hepatic venous outflow tract obstruction, occurring anywhere from the small hepatic veins to the suprahepatic inferior vena cava.
Budd Chiari syndrome is a rare disorder resulting from hepatic venous outflow tract obstruction anywhere from the small hepatic veins to the suprahepatic inferior vena cava.
In September 2010, Anas Saleh was diagnosed with a liver disease, Budd Chiari Syndrome (a clinical syndrome resulting from obstruction of the veins in the liver).
Depending on these Post mortem findings, it was concluded that she had an acute type of Budd Chiari Syndrome from which she never recovered and succumbed to death.
The pathology might have started early in the pregnancy which remained latent until the uterine contractions set in and increased abdominal pressure might have compressed the retro hepatic Inferior vena cava accelerating the thrombotic process, causing complete obstruction of the Inferior vena cava, portal vein, leading to acute presentation of Budd Chiari Syndrome.
Budd Chiari syndrome (BCS) is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atrio cava junction.
Budd Chiari Syndrome in a Fifteen-Year Old Girl with Systemic Lupus Erythematosus.