ESSENTIALS OF DIAGNOSIS in Objective-C

Creation QR in Objective-C ESSENTIALS OF DIAGNOSIS

Right upper quadrant pain and tenderness Ascites Imaging studies show occlusion/absence of flow in the hepatic vein(s) or inferior vena cava Clinical picture is similar in veno-occlusive disease but major hepatic veins are patent

Factors that predispose patients to hepatic vein obstruction, or Budd Chiari syndrome, including hereditary and acquired hypercoagulable states, can be identified in 75% of affected patients; multiple disorders are found in 25% Many cases are associated with polycythemia vera or other myeloproliferative disease, which is often associated with a specific mutation (V617F) in the gene that codes for JAK2 tyrosine kinase and may be subclinical In some cases, an underlying predisposition to thrombosis (eg, hyperprothrombinemia [factor II G20210A mutation], activated protein C resistance [factor V Leiden mutation], protein C or S or antithrombin deficiency, the methylenetetrahydrofolate reductase mutation, antiphospholipid antibodies) can be identified Hepatic vein obstruction may be associated with caval webs, right-sided heart failure or constrictive pericarditis, neoplasms that cause hepatic vein occlusion, paroxysmal nocturnal hemoglobinuria, Beh et s syndrome, blunt abdominal trauma, use of oral contraceptives, and pregnancy Some cytotoxic agents and pyrrolizidine alkaloids ( bush teas ) may cause sinusoidal obstruction syndrome (previously known as veno-occlusive disease because the terminal venules are often occluded), which mimics Budd Chiari syndrome clinically Sinusoidal obstruction syndrome is common in patients who have undergone bone marrow transplantation, particularly those with pretransplant aminotransferase elevations or fever during cytoreductive therapy with cyclophosphamide, azathioprine, carmustine, busulfan, or etoposide or those receiving high-dose cytoreductive therapy or high-dose total body irradiation In India, China, and South Africa, Budd Chiari syndrome is often the result of occlusion of the hepatic portion of the inferior vena cava, presumably due to prior

Percutaneous or transjugular liver biopsy frequently shows a characteristic centrilobular congestion and fibrosis and often multiple large regenerating nodules Liver biopsy is often contraindicated in sinusoidal obstruction syndrome because of thrombocytopenia, and the diagnosis is based on clinical findings

Ascites should be treated with fluid and salt restriction and diuretics Treatable causes of Budd Chiari syndrome should be sought Prompt recognition and treatment of an underlying hematologic disorder may avoid the need for surgery, but the optimal anticoagulation regimen is uncertain Infusion of a thrombolytic agent into recently occluded veins has been attempted with success In patients with sinusoidal obstruction syndrome, defibrotide, an adenosine receptor agonist that increases endogenous tissue plasminogen activator levels, has shown promise TIPS placement may be attempted in patients with persistent hepatic congestion or failed thrombolytic therapy, although late TIPS dysfunction is common TIPS is now preferred over surgical decompression (side-toside portacaval, mesocaval, or mesoatrial shunt), which has not been proven to improve long-term survival Balloon angioplasty, in some cases with placement of an intravascular metallic stent, is preferred in patients with an inferior vena caval web and is being performed increasingly in patients with a short segment of thrombosis in the hepatic vein Liver transplantation is considered in patients with fulminant hepatic failure, cirrhosis and hepatocellular dysfunction, and failure of a portosystemic shunt Patients often require lifelong anticoagulation and treatment of the underlying myeloproliferative disease; antiplatelet therapy with aspirin and

hydroxyurea has been suggested as an alternative to warfarin in patients with a myeloproliferative disorder The overall 5year survival rate is 50 90% Adverse prognostic factors in patients with Budd Chiari syndrome are older age, advanced Child-Turcotte-Pugh stage, ascites, encephalopathy, elevated total bilirubin, prolonged prothrombin time, elevated serum creatinine, concomitant portal vein thrombosis, and histologic features of acute liver disease superimposed on chronic liver injury

Helmy A Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome Aliment Pharmacol Ther 2006 Jan 1;23(1):11 25 [PMID: 16393276] Patel RK et al Prevalence of the activating JAK2 tyrosine kinase mutation V617F in the Budd-Chiari syndrome Gastroenterology 2006 Jun;130(7):2031 8 [PMID: 16762626] Plessier A et al Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome Hepatology 2006 Nov; 44(5):1308 16 [PMID: 17058215]