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-y...

Filling defects are often observed on 99mTc sulfur colloid liver scans. Hot spots are much less frequently seen. Superior vena cava syndrome is the most frequent cause of this finding. In this report 3 patients with S.V.C and budd - Chiari syndrome with HOT SPOT on liver scan are presented. Mechanisms of scintigraphic patterns are reviewed.

OBJECTIVES Budd-Chiari syndrome is a rare and serious complication of Behçet's disease, and is the result of occlusion of the major hepatic veins, the adjacent inferior vena cava, or both. The aim of this study was to determine the prevalence, clinical and laboratory findings, and treatment and clinical course of Budd-Chiari syndrome associated with Behçet's disease. METHODS We analyzed retro...

We describe a 38 year-old woman with polycythemia vera who presented with fulminant hepatic failure due to acute Budd-Chiari syndrome. She had a history of abdominal pain and distention for 4 days. Laboratory and clinical findings showed fulminant hepatic failure due to acute Budd-Chiari syndrome. Diagnosis was confirmed with abdominal ultrasonography and doppler ultrasonography showing ascites...

Budd-Chiari syndrome (BCS) represents a rare medical entity which has an estimated incidence of 0.1 to 10 people per million every year. It is defined by the obstruction flow in inferior vena cava or hepatic veins. Various classifications have been proposed. So, it can be acute chronic and primary secondary. The form more frequent characterized signs portal hypertension. Liver transplantation u...

background budd-chiari syndrome (bcs) is defined as hepatic venous outflow obstruction (hvoo). bcs is an uncommon, life-threatening liver disorder. this study describes the clinical and etiological characteristics in addition to the long-term outcome of bcs in a single referral center in tehran, iran. methods we reviewed long-term outcome of patients who were diagnosed with bcs between 1989 and...

Focal nodular hyperplasia and nodular regenerative hyperplasia are occasionally seen in patients with hepatic venous outflow obstruction as a consequence of circulatory stress in the liver. In addition, neoplastic processes such as hepatic adenoma, hepatocellular carcinoma, and metastatic disease may arise in these patients. Histologic evaluation is necessary when imaging modalities are unable ...