BACKGROUND Budd-Chiari syndrome(BCS)is caused by obstruction of the hepatic veins or suprahepatic inferior vena cava,leading to portal hypertension and the development of gastroesophageal varices(GEVs),which are assoc...BACKGROUND Budd-Chiari syndrome(BCS)is caused by obstruction of the hepatic veins or suprahepatic inferior vena cava,leading to portal hypertension and the development of gastroesophageal varices(GEVs),which are associated with an increased risk of bleeding.Existing risk models for variceal bleeding in cirrhotic patients have limited applicability to BCS due to differences in pathophysiology.Radiomics,as a noninvasive technique,holds promise as a tool for more accurate prediction of bleeding risk in BCS-related GEVs.AIM To develop and validate a personalized risk model for predicting variceal bleeding in BCS patients with GEVs.METHODS We retrospectively analyzed clinical data from 444 BCS patients with GEVs in two centers.Radiomic features were extracted from portal venous phase computed tomography(CT)scans.A training cohort of 334 patients was used to develop the model,with 110 patients serving as an external validation cohort.LASSO Cox regression was used to select radiomic features for constructing a radiomics score(Radscore).Univariate and multivariate Cox regression identified independent clinical predictors.A combined radiomics+clinical(R+C)model was developed using stepwise regression.Model performance was assessed using the area under the receiver operating characteristic curve(AUC),calibration plots,and decision curve analysis(DCA),with external validation to evaluate generalizability.RESULTS The Radscore comprised four hepatic and six splenic CT features,which predicted the risk of variceal bleeding.Multivariate analysis identified invasive treatment to relieve hepatic venous outflow obstruction,anticoagulant therapy,and hemoglobin levels as independent clinical predictors.The R+C model achieved C-indices of 0.906(training)and 0.859(validation),outperforming the radiomics and clinical models alone(AUC:training 0.936 vs 0.845 vs 0.823;validation 0.876 vs 0.712 vs 0.713).DCA showed higher clinical net benefit across the thresholds.The model stratified patients into low-,medium-and high-risk groups with significant differences in bleeding rates(P<0.001).An online tool is available at https://bcsvh.shinyapps.io/BCS_Variceal_Bleeding_Risk_Tool/.CONCLUSION We developed and validated a novel radiomics-based model that noninvasively and conveniently predicted risk of variceal bleeding in BCS patients with GEVs,aiding early identification and management of high-risk patients.展开更多
BACKGROUND Hepatocellular carcinoma(HCC)with advanced features such as Budd-Chiari syndrome,chronic liver failure and multiple intrahepatic metastases poses significant therapeutic challenges.Yttrium-90(90Y)radioembol...BACKGROUND Hepatocellular carcinoma(HCC)with advanced features such as Budd-Chiari syndrome,chronic liver failure and multiple intrahepatic metastases poses significant therapeutic challenges.Yttrium-90(90Y)radioembolization is a locoregional treatment option with potential benefits in such complex cases.This case report explores the application of 90Y radioembolization in combination with systemic therapies,highlighting its potential role in managing advanced HCC.CASE SUMMARY A 51-year-old male presented with HCC characterized by massive intrahepatic lesions,multiple metastases,and chronic liver failure secondary to Budd-Chiari syndrome.The patient underwent 90Y radioembolization following hepatic arterial infusion chemotherapy and was subsequently combined with lenvatinib.Posttreatment follow-up revealed a significant reduction in tumor size,with the maximum diameter decreasing from 142.45 mm to 73.16 mm over six months.Liver function improved from Child-Pugh class B to A.However,new intrahepatic lesions emerged at ten months,and liver function deteriorated to Child-Pugh class C.The patient survived for 18 months after initial diagnosis.CONCLUSION Yttrium-90 radioembolization combined with systemic therapies demonstrated significant tumor regression and temporary liver function improvement in a patient with advanced HCC,suggesting its potential as a treatment option in complex cases.展开更多
This editorial narrative review discussed Budd-Chiari syndrome(BCS),which re-presents a rare but critical vascular liver disease resulting in an obstruction of he-patic venous outflow.Despite having a unifying mechani...This editorial narrative review discussed Budd-Chiari syndrome(BCS),which re-presents a rare but critical vascular liver disease resulting in an obstruction of he-patic venous outflow.Despite having a unifying mechanism,the syndrome shows a large heterogeneity across presentation,cause,and disease trajectory,compli-cating diagnosis and management.Based on established prognostic scoring systems,the New Clichy Score,the BCS-transjugular intrahepatic portosystemic shunt Index,the Zeitoun Score,and the Pediatric End-stage Liver Disease score were examined.These scoring systems are used for risk stratification and thera-peutic decision-making.Although these models deliver suitability information,their static parameters,narrow validation,and limited generalizability reduce their usefulness in diverse populations.Specific challenges are highlighted in pediatric patients,pregnant females,and individuals with myeloproliferative neoplasms for whom current tools often fall short.Moreover,there remains uncertainty regarding the durability of Pediatric End-stage Liver Disease score response and longer-term risks,such as hepatocellular carcinoma.There is a need to have a dynamic prognostic model that uses imaging and genetic factors in future studies.The article discussed enhancing recruitment to improve research.Overall,this article provided a contemporary,evidence-based approach for cli-nicians to aid in the evaluation and treatment of BCS.展开更多
基金Supported by Natural Science Foundation of Henan Province,China,No.232300420232Henan Provincial Key Research and Development Project,No.231111313500.
文摘BACKGROUND Budd-Chiari syndrome(BCS)is caused by obstruction of the hepatic veins or suprahepatic inferior vena cava,leading to portal hypertension and the development of gastroesophageal varices(GEVs),which are associated with an increased risk of bleeding.Existing risk models for variceal bleeding in cirrhotic patients have limited applicability to BCS due to differences in pathophysiology.Radiomics,as a noninvasive technique,holds promise as a tool for more accurate prediction of bleeding risk in BCS-related GEVs.AIM To develop and validate a personalized risk model for predicting variceal bleeding in BCS patients with GEVs.METHODS We retrospectively analyzed clinical data from 444 BCS patients with GEVs in two centers.Radiomic features were extracted from portal venous phase computed tomography(CT)scans.A training cohort of 334 patients was used to develop the model,with 110 patients serving as an external validation cohort.LASSO Cox regression was used to select radiomic features for constructing a radiomics score(Radscore).Univariate and multivariate Cox regression identified independent clinical predictors.A combined radiomics+clinical(R+C)model was developed using stepwise regression.Model performance was assessed using the area under the receiver operating characteristic curve(AUC),calibration plots,and decision curve analysis(DCA),with external validation to evaluate generalizability.RESULTS The Radscore comprised four hepatic and six splenic CT features,which predicted the risk of variceal bleeding.Multivariate analysis identified invasive treatment to relieve hepatic venous outflow obstruction,anticoagulant therapy,and hemoglobin levels as independent clinical predictors.The R+C model achieved C-indices of 0.906(training)and 0.859(validation),outperforming the radiomics and clinical models alone(AUC:training 0.936 vs 0.845 vs 0.823;validation 0.876 vs 0.712 vs 0.713).DCA showed higher clinical net benefit across the thresholds.The model stratified patients into low-,medium-and high-risk groups with significant differences in bleeding rates(P<0.001).An online tool is available at https://bcsvh.shinyapps.io/BCS_Variceal_Bleeding_Risk_Tool/.CONCLUSION We developed and validated a novel radiomics-based model that noninvasively and conveniently predicted risk of variceal bleeding in BCS patients with GEVs,aiding early identification and management of high-risk patients.
文摘BACKGROUND Hepatocellular carcinoma(HCC)with advanced features such as Budd-Chiari syndrome,chronic liver failure and multiple intrahepatic metastases poses significant therapeutic challenges.Yttrium-90(90Y)radioembolization is a locoregional treatment option with potential benefits in such complex cases.This case report explores the application of 90Y radioembolization in combination with systemic therapies,highlighting its potential role in managing advanced HCC.CASE SUMMARY A 51-year-old male presented with HCC characterized by massive intrahepatic lesions,multiple metastases,and chronic liver failure secondary to Budd-Chiari syndrome.The patient underwent 90Y radioembolization following hepatic arterial infusion chemotherapy and was subsequently combined with lenvatinib.Posttreatment follow-up revealed a significant reduction in tumor size,with the maximum diameter decreasing from 142.45 mm to 73.16 mm over six months.Liver function improved from Child-Pugh class B to A.However,new intrahepatic lesions emerged at ten months,and liver function deteriorated to Child-Pugh class C.The patient survived for 18 months after initial diagnosis.CONCLUSION Yttrium-90 radioembolization combined with systemic therapies demonstrated significant tumor regression and temporary liver function improvement in a patient with advanced HCC,suggesting its potential as a treatment option in complex cases.
文摘This editorial narrative review discussed Budd-Chiari syndrome(BCS),which re-presents a rare but critical vascular liver disease resulting in an obstruction of he-patic venous outflow.Despite having a unifying mechanism,the syndrome shows a large heterogeneity across presentation,cause,and disease trajectory,compli-cating diagnosis and management.Based on established prognostic scoring systems,the New Clichy Score,the BCS-transjugular intrahepatic portosystemic shunt Index,the Zeitoun Score,and the Pediatric End-stage Liver Disease score were examined.These scoring systems are used for risk stratification and thera-peutic decision-making.Although these models deliver suitability information,their static parameters,narrow validation,and limited generalizability reduce their usefulness in diverse populations.Specific challenges are highlighted in pediatric patients,pregnant females,and individuals with myeloproliferative neoplasms for whom current tools often fall short.Moreover,there remains uncertainty regarding the durability of Pediatric End-stage Liver Disease score response and longer-term risks,such as hepatocellular carcinoma.There is a need to have a dynamic prognostic model that uses imaging and genetic factors in future studies.The article discussed enhancing recruitment to improve research.Overall,this article provided a contemporary,evidence-based approach for cli-nicians to aid in the evaluation and treatment of BCS.
