摘要
AIM： TO describe the socio-demographic features, etiology, and risk factors for Budd-Chiari syndrome （BCS） in Egyptian patients. METHODS： Ninety-four Egyptian patients with confirmed primary Budd-Chiari syndrome were presented to the Budd-Chiari Study Group （BCSG） and admitted to the Tropical Medicine Department of Ain Shams University Hospital （Cairo, Egypt）. Complete clinical evaluation and laboratory investigations, including a thrombophilia workup and full radiological assessment, were performed to determine underlying disease etiologies.RESULTS： BCS was chronic in 79.8% of patients, acute or subacute in 19.1%, and fulminant in 1.1%. Factor V Leiden mutation （FVLM） was the most common etiological cause of disease （53.1%）, followed by mutation of the gene encoding methylene tetrahydrofolate reductase （MTHFR） （51.6%）. Current or recent hormonal treatment was documented in 15.5% of females, and BCS associated with pregnancy was present in 17.2% of females. Etiology could not be determined in 8.5% of patients. Males had significantly higher rates of MTHFR gene mutation and Behcet＇ s disease, and females had significantly higher rates of secondary antiphospholipid antibody syndrome. A highly significant positive relationship was evident between the presence of Behcet＇s disease and inferior vena caval occlusion, either alone or combined with occlusion of the hepatic veins （,0 〈 0.0001）. CONCLUSION： FVLM is the most common disease etiology and MTHFR the second most common in Egyptian BCS patients. BCS etiology tends to vary with geographic region.
AIM:To describe the socio-demographic features,etiology,and risk factors for Budd-Chiari syndrome(BCS) in Egyptian patients.METHODS:Ninety-four Egyptian patients with confirmed primary Budd-Chiari syndrome were presented to the Budd-Chiari Study Group(BCSG) and admitted tothe Tropical Medicine Department of Ain Shams University Hospital(Cairo,Egypt) . Complete clinical evaluation and laboratory investigations,including a throm bophiliaworkup and full radiological assessment,were performed to determine underlying disease etiologies.RESULTS:BCS was chronic in 79.8% of patients,acute or subacute in 19.1%,and fulminant in 1.1%.Factor V Leiden mutation(FVLM) was the most common etiological cause of disease(53.1%) ,followed by mutation of the gene encoding methylene tetra-hydrofolate reductase(MTHFR) (51.6%) . Current orrecent hormonal treatment was documented in 15.5%of females,and BCS associated with pregnancy was present in 17.2% of females. Etiology could not be determined in 8.5% of patients. Males had significantly higher rates of MTHFR gene mutation and Beh et's disease,and females had significantly higher ratesof secondary antiphos pholipid antibody syndrome.A highly significant positive relation ship was evident between the presence of Beh et's disease and inferiorven a caval occlusion,either alone or combined with occlusion of the hepatic veins(P < 0.0001) .CONCLUSION:FVLM is the most common disease etiology and MTHFR the second most common in Egyptian BCS patients. BCS etiology tends to vary with geographic region.
