What Are Common Presentations for Budd-Chairi in Adolescents?

Discussion Budd-Chiari syndrome (BCS) is a rare liver disease caused by hepatic venous outflow obstruction (HVOTO). The obstruction can be anywhere from the small intrahepatic veins up to the inferior vena cava junction with the right atrium. The liver parenchyma itself is not directly affected but becomes compromised because of the increased hepatic sinusoidal pressure over time. The causes include: prothrombic events (35% of cases such as Protein C or Protein S deficiency, Factor V Leiden or antithrombin deficiency), myeloproliferative conditions, oral contraceptive use, and local factors. BCS can occur in any age but is more common in the adult population with it occurring more commonly in females than males. Clinically the presentation can be acute, subacute or chronic. Some patients remain asymptomatic (up to 15%) and others present in fulminant hepatic failure. It is believed this is due to the rapidity of the hepatic vein occlusion and the inability to develop collateral circulation. Classically BCS presents as abdominal pain, hepatomegaly and ascites in adult patients. Fever, esophageal bleeding or hepatic encephalopathy are much less common. Clinical presentation does not correlate with duration of disease. Asymptomatic patients have a good prognosis but symptomatic patients often don’t without treatment. “The reported life expectancy in these patient is 3 years after the first symptoms.” However treatment for underlying causes, angioplasty, tran...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news