Budd Chiari Syndrome General Discussion Budd-Chiari syndrome is a rare disorder characterized by narrowing and obstruction (occlusion) of the veins of the liver (hepatic veins). Symptoms associated with Budd Chiari syndrome include pain in the upper right part of the abdomen, an abnormally large liver (hepatomegaly), and/or accumulation of fluid in the space (peritoneal cavity) between the two layers of the membrane that lines the stomach (ascites). Additional findings that may be associated with the disorder include nausea, vomiting, and/or an abnormally large spleen (splenomegaly). The severity of the disorder varies from case to case, depending upon the site and number of affected veins. In some cases, if the major hepatic veins are involved, high blood pressure in the veins carrying blood from the gastrointestinal (GI) tract back to the heart through the liver (portal hypertension) may be present. In most cases, the exact cause of Budd-Chiari syndrome is unknown.>The cause for the disease cannot be found in about half of the patients. Primary Budd–Chiari syndrome (75%): thrombosis of the hepatic vein Secondary Budd–Chiari syndrome (25%): compression of the hepatic vein by an outside structure (e.g. a tumor) Hepatic vein thrombosis is associated with the following in decreasing order of frequency: Polycythemia vera Pregnancy Post partum state Use of oral contraceptives Paroxysmal nocturnal hemoglobinuria Hepatocellular carcinoma Lupus anticoagulants Budd–Chiari syndrome is also seen in Infection such as tuberculosis, congenital venous webs and occasionally in inferior vena caval stenosis. Often, the patient is known to have a tendency towards thrombosis, although Budd–Chiari syndrome can also be the first symptom of such a tendency. Examples of genetic tendencies include Protein C deficiency, Protein S deficiency, the Factor V Leiden mutation, Hereditary anti-thrombin deficiency and Prothrombin Mutation G20210A.[2] An important non-genetic risk factor is the use of estrogen-containing (combined) forms of hormonal contraception. Other risk factors include the antiphospholipid syndrome, aspergillosis, Behçets disease, dacarbazine, pregnancy, and trauma>>
Posted on: Thu, 02 Oct 2014 00:45:24 +0000
Trending Topics
Recently Viewed Topics
© 2015