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Published on: February 27, 2009
[Diagnosis, treatment and evolution of the Budd-Chiari syndrome: a single center experience]
Joaquín Solari1, Juan Carlos Bandi, Omar Galdame
1Unidad de Trasplante Hepático, Hospital Italiano de Buenos Aires, Argentina. joaquin.solari@hospitalitaliano.org.ar
Insights
Budd-Chiari syndrome, a liver outflow obstruction, is linked to a procoagulant status. Transjugular intrahepatic portosystemic shunt (TIPS) effectively treats this condition, with liver transplantation reserved for refractory cases.
Area of Science:
- Hepatology
- Vascular Medicine
- Hematology
Context:
- Budd-Chiari syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction.
- It is frequently associated with a procoagulant state, increasing the risk of thrombosis.
- Liver transplantation is a recognized treatment option for advanced BCS.
Purpose:
- To investigate the causes, clinical presentations, management strategies, and outcomes of patients diagnosed with Budd-Chiari syndrome.
- To assess the role of procoagulant status in BCS.
- To evaluate the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) and liver transplantation in BCS management.
Summary:
- This study prospectively analyzed 10 adult patients with BCS over a median of 32.4 months.
- Common clinical manifestations included splenomegaly, malnutrition, ascites, and encephalopathy.
- A procoagulant status was identified in all patients, with polycythemia vera being a frequent finding. TIPS was effective in 6 patients, and 2 underwent liver transplantation.
Impact:
- The findings highlight the consistent procoagulant status in BCS patients.
- Transjugular intrahepatic portosystemic shunt (TIPS) demonstrates significant efficacy in managing Budd-Chiari syndrome.
- Liver transplantation should be considered a treatment of last resort for patients unresponsive to other therapies.
Introduction:
The Budd-Chiari syndrome is a low-prevalence disease due to an hepatic outflow obstruction. It is associated with procoagulant status and liver transplantation is one of the therapeutic tools for the treatment.
Objective:
To evaluate the etiology, presenting form, treatment and evolution of patients with Budd-Chiari syndrome.
Patients And Method:
Ten consecutive adult patients with Budd-Chiari syndrome evaluated from January 1998 to June 2009 were prospectively included. The median follow up was 32.4 months (4-108 months).
Results:
The mean age of patients was 34 +/- 12 years old. Presentation was acute in 1 patient, chronic in 2 and subacute in 7. The mean time from consultation to diagnosis was 4 +/- 2 days. Clinical manifestations were splenomegaly in 8 patients, malnutrition in 7, ascites in 6 and encephalopathy in 4. Diagnosis was confirmed by angiography in all cases. Initial prothrombin concentration was < 30% in 3 patients, 31% to 50% in 5, and > 50% in 2; hematocrit was > 45% in 5 patients and platelet count was > 400.000/mm3 in 6. MELD distribution at diagnosis was < or = 13 points in 4 patients, between 14 and 16 points in 5 and > or = 17 points in 1. Policytemia vera was detected in 7 patients, essential thrombocythemia in 1 and positive lupus inhibitor in 4. Nine patients were anticoagulated after diagnosis. Angioplasthy was required in 1 patient and 6 were treated with a transjugular intrahepatic portosystemic shunt. Death occurred in 1 patient due to gastrointestinal bleeding. Two patients were transplanted.
Conclusion:
In our experience all patients with Budd-Chiari syndrome have a procoagulant status. The transjugular intrahepatic portosystemic shunt is effective in treating this syndrome and liver transplantation should be reserved for patients who are refractory to other therapeutics.
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