| Autor: |
Rania, Hefaiedh, Mariem, Cheikh, Lobna, Marsaoui, Rym, Ennaifer, Hayfa, Romdhane, Houda, Ben Nejma, Najet, Bel Hadj, Nafaa, Arfa, Mohamed Taher, Khalfallah |
| Rok vydání: |
2013 |
| Předmět: |
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| Zdroj: |
La Tunisie medicale. 91(6) |
| ISSN: |
0041-4131 |
| Popis: |
The Budd-Chiari syndrome is a rare disease, often fatal if not treated optimally. It is characterized by a blocked hepatic venous outflow tract. aim: This review attempted to present pathophysiology, aetiologies,diagnosis and therapeutic modalities of the Budd-Chiari syndrome.Review of literature.Budd-Chiari syndrome is a complex disease with a wide spectrum of aetiologies and presentations. Hematologic abnormalities, particularly myeloproliferative disorders, are the most common causes of the Budd-Chiari syndrome. The clinical presentation is governed by the extent and rapidity of the hepatic vein occlusion. 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. A therapeutic strategy has been proposed where anticoagulation, correction of risk factors, diuretics and prophylaxis for portal hypertension are used first; then angioplasty for shortlength venous stenosis; then Transjugular Intrahepatic Portosystemic Shunt (TIPS); and ultimately liver transplantation. Treatment progression is dictated by the response to previous therapy. This strategy has achieved 5-year survival rates approaching 70%. Medium-term prognosis depends on the severity of liver disease.The diagnosis of the Budd-Chiari syndrome must be considered in any patients with acute or chronic liver disease. Management of this syndrome should follow a step by step strategy. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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