Document Type





OBJECTIVE: To determine the clinical characteristics of Budd-Chiari syndrome (BCS), its causes and outcome at a tertiary care hospital.

STUDY DESIGN: An observational study

.PLACE AND DURATION OF STUDY: The Aga Khan University Hospital,Karachi, from 2004 to 2014.

METHODOLOGY: Aretrospective analysis of data was conducted. Apredesigned questionnaire was filled from medical records of patients with BCS. Clinical features, etiology, management and outcome was noted from 2004 to 2014. Descriptive statistics were determined.

RESULTS: Forty-five patients' charts were reviewed; 26 (57.8%) were male patients. The median (IQR) age at diagnosis was 26.0 (20.5 to 34.5) years. Primary BCS was seen in 27 (60.0%) patients. The most frequent clinical features included ascites (82.2%), abdominal pain (55.6%), and hepatomegaly (31.1%). Acombined hepatic vein/inferior vena cava block was found in 25 (55.6%) patients. Out of the 28 tested patients protein C and protein S deficiencies were detected in 22 (78.6%) and 17 (60.7%) patients, respectively. Antithrombin III deficiency was detected in 14 (58.3%) of those tested patients. Anticoagulants were used in 24 (53.3%) patients. TIPS was done in 11 (24.4%) patients. Mortality was 6.7% (n=3).

CONCLUSION: Congenital thrombophilia was a major causal factor. Age, clinical features, biochemistry and management are important factors in survival.


JCPSP:Journal of the College of Physicians and Surgeons--Pakistan.