AIM: To evaluate outcome of patients with Budd-Chiari syndrome after balloon angioplasty ± stenting or transjugular intrahepatic portosystemic shunt (TIPS). METHODS: Twenty five patients with Budd-Chiari syndrome...AIM: To evaluate outcome of patients with Budd-Chiari syndrome after balloon angioplasty ± stenting or transjugular intrahepatic portosystemic shunt (TIPS). METHODS: Twenty five patients with Budd-Chiari syndrome admitted to Ain Shams University Hospitals, Tropical Medicine Department were included. Twelve patients (48%) with short segment occlusion were candidates for angioplasty; with stenting in ten cases and without stenting in two. Thirteen patients (52%) had Transjugular Intrahepatic Portosystemic Shunt. Patients were followed up for 12-32 mo. RESULTS: Patency rate in patients who underwent angioplasty ± stenting was 83.3% at one year and at end of follow up. The need of revision was 41.6% with one year survival of 100%, dropped to 91.6% at end of follow up. In patients who had Transjugular Intrahepatic Portosystemic Shunt, patency rate was 92.3% at one year, dropped to 84.6% at end of follow up. The need of revision was 38.4% with one year and end of follow up survival of 100%. Patients with patent shunts showed marked improvement compared to those with occluded shunts. CONCLUSION: Morbidity and mortality following angioplasty ± stenting and TIPS are low with satisfactory outcome. Proper patient selection and management of shunt dysfunction are crucial in improvement.展开更多
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 w...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.展开更多
AIMTo compare predictive ability of Budd-Chiari syndrome (BCS) prognostic indices (PIs) for one-year survival and Transjugular intrahepatic portosystemic shunt (TIPS) patency.METHODSThis retrospective study enrolled 1...AIMTo compare predictive ability of Budd-Chiari syndrome (BCS) prognostic indices (PIs) for one-year survival and Transjugular intrahepatic portosystemic shunt (TIPS) patency.METHODSThis retrospective study enrolled 194 Egyptian patients with primary BCS who presented to the Budd-Chiari Study Group of Ain Shams University Hospital. Calculation of the available PIs was performed using Child-Pugh and model for end-stage liver disease scores, BCS-specific PIs (Clichy, New Clichy and Rotterdam) for all patients, and BCS-TIPS PI only for patients who underwent TIPS. The overall one-year survival rate and the one-year shunt patency rate for TIPS were reported.RESULTSThe overall one-year survival rate was 69.6%, and the New Clichy PI revealed the best validity for its prediction at a cut-off value of 3.75, with sensitivity and specificity of 78% and 73.3%, respectively [area under receiver operating characteristic curve (AUC) = 0.806]. The one-year survival rate post-TIPS was 89.7%, and the BCS-TIPS score demonstrated validity for its prediction at a cut-off value of 3.92 (sensitivity and specificity were 71.4% and 64.5%, respectively) (AUC = 0.715). Logistic regression analysis revealed that the New Clichy PI (P = 0.030), high serum total bilirubin (P = 0.047) and low albumin (P < 0.001) were independent factors for predicting mortality within one year. The one-year shunt patency rate in TIPS was 80.2%, and none of the PIs exhibited significant validity for its prediction.CONCLUSIONThe New Clichy score could independently predict the one-year survival in Egyptian BCS patients.展开更多
文摘AIM: To evaluate outcome of patients with Budd-Chiari syndrome after balloon angioplasty ± stenting or transjugular intrahepatic portosystemic shunt (TIPS). METHODS: Twenty five patients with Budd-Chiari syndrome admitted to Ain Shams University Hospitals, Tropical Medicine Department were included. Twelve patients (48%) with short segment occlusion were candidates for angioplasty; with stenting in ten cases and without stenting in two. Thirteen patients (52%) had Transjugular Intrahepatic Portosystemic Shunt. Patients were followed up for 12-32 mo. RESULTS: Patency rate in patients who underwent angioplasty ± stenting was 83.3% at one year and at end of follow up. The need of revision was 41.6% with one year survival of 100%, dropped to 91.6% at end of follow up. In patients who had Transjugular Intrahepatic Portosystemic Shunt, patency rate was 92.3% at one year, dropped to 84.6% at end of follow up. The need of revision was 38.4% with one year and end of follow up survival of 100%. Patients with patent shunts showed marked improvement compared to those with occluded shunts. CONCLUSION: Morbidity and mortality following angioplasty ± stenting and TIPS are low with satisfactory outcome. Proper patient selection and management of shunt dysfunction are crucial in improvement.
文摘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.
文摘AIMTo compare predictive ability of Budd-Chiari syndrome (BCS) prognostic indices (PIs) for one-year survival and Transjugular intrahepatic portosystemic shunt (TIPS) patency.METHODSThis retrospective study enrolled 194 Egyptian patients with primary BCS who presented to the Budd-Chiari Study Group of Ain Shams University Hospital. Calculation of the available PIs was performed using Child-Pugh and model for end-stage liver disease scores, BCS-specific PIs (Clichy, New Clichy and Rotterdam) for all patients, and BCS-TIPS PI only for patients who underwent TIPS. The overall one-year survival rate and the one-year shunt patency rate for TIPS were reported.RESULTSThe overall one-year survival rate was 69.6%, and the New Clichy PI revealed the best validity for its prediction at a cut-off value of 3.75, with sensitivity and specificity of 78% and 73.3%, respectively [area under receiver operating characteristic curve (AUC) = 0.806]. The one-year survival rate post-TIPS was 89.7%, and the BCS-TIPS score demonstrated validity for its prediction at a cut-off value of 3.92 (sensitivity and specificity were 71.4% and 64.5%, respectively) (AUC = 0.715). Logistic regression analysis revealed that the New Clichy PI (P = 0.030), high serum total bilirubin (P = 0.047) and low albumin (P < 0.001) were independent factors for predicting mortality within one year. The one-year shunt patency rate in TIPS was 80.2%, and none of the PIs exhibited significant validity for its prediction.CONCLUSIONThe New Clichy score could independently predict the one-year survival in Egyptian BCS patients.
