[3] a Prospective Follow-Up Study on 163 Patients With Budd-Chiari Syndrome: Results From the European Network for Vascular Disorders of the Liver (En-Vie)

Murad, Sarwa Darwish; Plessier, Aurélie; Guerra, M.; Primignani, Massimo; Elias, E.; Bahr, Matthias J.; Hadengue, A.; Langlet, Philippe; Miranda, Helena; Pagán, Juan Carlos García; Valla, Dominique; Janssen, Harry L.A.

doi:10.1016/s0168-8278(07)61601-8

Cited by 19 publications

(12 citation statements)

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“…Contrasting with a high degree of heterogeneity in causes, presentation, and level of hepatic venous outflow block, it is remarkable that all the prognostic information appears to be accounted for by the components of the Child-Pugh score or the MELD score [7]. However, it should be kept in mind that these markers are poor predictors of the outcome in an individual patient.…”

Section: Natural Course and Prognosismentioning

confidence: 99%

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Primary Budd-Chiari syndrome

Valla

2009

Journal of Hepatology

269

236

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Primary Budd-Chiari syndrome is characterized by a blocked hepatic venous outflow tract at various levels from small hepatic veins to inferior vena cava, resulting from thrombosis or its fibrous sequellae. This rare disease affects mainly young adults. Multiple risk factors have been identified and are often combined in the same patient. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients; their diagnosis can be made by showing the V617F mutation in Janus tyrosine kinase-2 gene of peripheral blood granulocytes and, should this mutation be absent, by showing clusters of dystrophic megacaryocytes at bone marrow biopsy. Presentation and manifestations are extremely varied, so that the diagnosis must be considered in any patient with acute or chronic liver disease. Doppler-ultrasound, computed tomography or magnetic resonance imaging of hepatic veins and inferior vena cava are usually successful in demonstrating non-invasively the obstacle or its consequences, the collaterals to hepatic veins or inferior vena cava. The disease is considered to be spontaneously lethal within 3 years of first symptoms. A therapeutic strategy has been proposed where anticoagulation, correction of risk factors, diuretics and prophylaxis for portal hypertension are used first; then angioplasty for short-length venous stenoses; then TIPS; and ultimately liver transplantation. Treatment progression is dictated by the response to previous therapy. This strategy has achieved 5-year survival rates approaching 90%. Medium-term prognosis depends on the severity of liver disease. Long-term outcome might be jeopardized by transformation of underlying conditions and hepatocellular carcinoma.

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Section: Natural Course and Prognosismentioning

confidence: 99%

“…Most affected Western patients have been young females whereas in Asia, middle-aged patients of either sex were predominantly affected. Recent data from a European cohort however indicate a change in demographics as the male to female ratio is closer to 1 and mean age is about 45 years [7].…”

Section: Introductionmentioning

confidence: 97%

Primary Budd-Chiari syndrome

Valla

2009

Journal of Hepatology

269

236

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“…4 Thrombosis is the most frequent cause of venous obstruction, and this is mainly caused by MPD; dysfunction of the coagulation cascade, such as factor V Leiden or prothrombin (factor II) mutation; protein C or S deficiency; or other hypercoagulable conditions as malignancy or that induced by oral contraceptive use. [5][6][7] The typical presentation is generally a triad of abdominal pain, ascites, and hepatomegaly, and it may vary significantly, from the absence of symptoms to severe liver failure.…”

Section: Discussionmentioning

confidence: 99%

Living-donor Liver Transplant in 3 Patients With Budd-Chiari Syndrome: Case Report

Baş

Yaprak²,

Dayangaç³

et al. 2012

Exp Clin Transplant

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Budd-Chiari syndrome is a rare but life-threatening disorder characterized by obstruction of the hepatic venous outflow. Treatment depends on the underlying cause, the location, and extent of the obstruction, and the functional capacity of the liver. A stepwise therapeutic approach is commonly accepted. When all other therapy options are unsuccessful, or in case of end-stage liver disease, transplant should be considered. We present case reports of 3 patients with Budd-Chiari syndrome who underwent living-donor liver transplant. Characteristic features of Budd-Chiari syndrome, diagnostic and therapeutic interventions, complications, and overall outcomes are discussed. We believe that when a deceased donor graft is unavailable, a living-donor liver transplant can be a safe option for patients with end-stage liver disease associated with Budd-Chiari syndrome.

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“…Other possible symptoms are nausea, fever, and jaundice. [124][125][126] The severity of symptoms depends on the extent of thrombosis, the rapidity of onset, and the compensatory collateral circulation.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…[124][125][126] There is a high serum ascites albumin gradient. Despite the major hemodynamic changes involving the liver, synthetic function is often relatively spared.…”

Section: Clinical Featuresmentioning

confidence: 99%

Noncirrhotic Portal Hypertension

Rajekar¹,

Vasishta²,

Chawla³

et al. 2011

Journal of Clinical and Experimental Hepatology

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Portal hypertension is characterized by an increase in portal pressure (> 10 mmHg) and could be a result of cirrhosis of the liver or of noncirrhotic diseases. When portal hypertension occurs in the absence of liver cirrhosis, noncirrhotic portal hypertension (NCPH) must be considered. The prognosis of this disease is much better than that of cirrhosis. Noncirrhotic diseases are the common cause of portal hypertension in developing countries, especially in Asia. NCPH is a heterogeneous group of diseases that is due to intrahepatic or extrahepatic etiologies. In general, the lesions in NCPH are vascular in nature and can be classified based on the site of resistance to blood flow. In most cases, these disorders can be explained

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[3] a Prospective Follow-Up Study on 163 Patients With Budd-Chiari Syndrome: Results From the European Network for Vascular Disorders of the Liver (En-Vie)

Cited by 19 publications

References 0 publications

Primary Budd-Chiari syndrome

Primary Budd-Chiari syndrome

Living-donor Liver Transplant in 3 Patients With Budd-Chiari Syndrome: Case Report

Noncirrhotic Portal Hypertension

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