Budd-Chiari syndrome: Etiology, pathogenesis and diagnosis

Abstract

Budd-Chiari syndrome is a congestive hepatopathy caused by blockage of hepatic veins. This syndrome occurs in 1/100000 in the general population. Hypercoagulable state could be identified in 75% of the patients; more than one etiologic factor may play a role in 25% of the patients. Primary myeloproliferative diseases are the leading cause of the disease. Two of the hepatic veins must be blocked for clinically evident disease. Liver congestion and hypoxic damage of hepatocytes eventually result in predominantly centrilobular fibrosis. Doppler ultrasonography of the liver should be the initial diagnostic procedure. Hepatic venography is the reference procedure if required. Additionally liver biopsy may be helpful for differential diagnosis. The prognosis of the chronic form is acceptable compared to other chronic liver diseases.

Diffuse Venous Thrombosis, Intracardiac Thrombus and Coronary-Pulmonary Artery Fistula in a Patient with Behçet\'s Disease: Case Report

“…5 Budd-Chi a ri syndro me may be de tec ted as a comp li ca ti on of Beh çet's di se a se. 4,6 In tra car di ac throm bus for ma ti on is not very com mon in Beh çet's di se a se. Mo gul koc and col lequ es had re wi e ved in tra car di ac throm bus in Beh çet's di se a se in 2000.…”

“…He pa tic ve in throm bo sis in Beh çet's di se a se is li kely se condary to in fe ri or ve na ca va throm bo sis. 4 A very ra re comp li ca ti on in pa ti ents with Behçet's di se a se is the de ve lop ment of ar te ri al fis tula. 5,19,20 Our pa ti ent had a con vo lu ted fis tu la de tected in the co ro nary an gi og raphy.…”

Case Report: A Rare and Unusual Cause of Acute Liver Failure

“…Serum transferase levels may be more than five times the upper limit of the normal range, especially in the fulminant and acute forms of BCS [8]. Serum alkaline phosphatase and bilirubin levels also increase but serum albumin level decreases moderately [8].…”

“…Hepatocytes then undergo hypoxic damage which eventually evolves into non-inflammatory centrilobular cell necrosis, which is found in 70% of cases of BCS [2,8]. Obstruction of the portal vein is also seen in 10%-20% of cases of BCS, due to underlying thrombophilic disorder and stagnant blood flow [8].…”

Budd-Chiari Syndrome

“…In 25% of patients, at least two underlying conditions can be identified (Aydinli and Bayraktar, 2007). Primary BCS requires laboratory test for factor V Leiden and factor II (prothrombin) mutations, for the presence of antiphospholipid antibodies…”