[Diagnosis, treatment and evolution of the Budd-Chiari syndrome: a single center experience].
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Unidad de Trasplante Hepático, Hospital Italiano de Buenos Aires, Argentina. joaquin.solari@hospitalitaliano.org.ar
Joaquín
J
Solari
Juan Carlos
JC
Bandi
Omar
O
Galdame
Sebastián
S
Marciano
Eduardo
E
Mullen
Mónaco Ricardo
MR
Garcia
Elsa
E
Nucifora
Eduardo
E
de Santibañes
Adrián
A
Gadano
D000328:Adult; D006502:Budd-Chiari Syndrome / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D011446:Prospective Studies; D016896:Treatment Outcome; D055815:Young Adult