BUDD-CHIARI SYNDROME - CLINICAL-PATTERNS AND THERAPY

  • 1 January 1982
    • journal article
    • research article
    • Vol. 51  (201) , 79-88
Abstract
Retrospective analysis of 36 patients (25 women, 11 men) with the Budd-Chiari syndrome diagnosed between 1971 and 1980 showed a wide range in ages at presentation (12-68 yr) with the peak incidence in the 3rd decade for women and in the 4th for men. The 11 patients below the age of 30 were women and 6 of these were taking oral contraceptive preparations. There was also a wide range in duration of illness before establishment of diagnosis ranging from 8 wk or less in 20 patients, to up to 6 yr in the other 16. Those in the former group had a high incidence of the classical clinical features (tender hepatomegaly with ascites) and most had severe abnormalities in liver function tests. Liver scintiscanning showed the characteristic pattern of maximum colloid uptake in the caudate lobe in only 46% of patients. Mistaken diagnosis in 8 cases led to early exploratory laparotomy with serious deterioration in 4. One year survival of 58.3% was unrelated to age, sex or etiology and little benefit was seen in the few cases treated early with fibrinolytic agents. Of the surgical measures employed only hepatic transplantation has proved worthwhile with 3 out of 4 cases alive at 14, 16 and 52 mo. respectively from the time of operation.

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