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Histopathological Features Predicting Long-term Clinical Outcomes in Patients with Vanishing bile duct Syndrome

Journal of Clinical and Translational Hepatology 2023年第5期11卷 1161-1169页

作者： Tingting Lv Haitian Yu Xiao Han Aileen Wee Jimin Liu Min Li Jinghang Xu Xiaoli Hu Jia Li Weijia Duan Tailing Wang Jidong Jia Xinyan Zhao Liver Research Center Beijing Friendship HospitalCapital Medical UniversityNational Clinical Research Center for Digestive DiseasesBeijingChina Department of Pathology Yong Loo Lin School of MedicineNational University of SingaporeNational University HospitalSingapore Department of Pathology and Molecular Medicine Faculty of Health SciencesMcMaster UniversityHamiltonOntarioCanada Clinical Epidemiology and Evidence-Based Medicine Unit National Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijingChina Department of infectious disease Peking University First HospitalBeijingChina Department of infectious disease Heilongjiang Province HospitalHeilongjiangChina Department of Hepatology Tianjin Second People’s HospitalTianjinChina Department of Pathology China-Japan Friendship HospitalBeijingChina

Background and Aims:The clinicopathological features and long-term outcomes of patients with vanishing bile duct syndrome(VBDS)have yet to be *** study aims to investigate these features and identify factors associated with poor ***:This multicenter retrospective study recruited patients with liver biopsy-proven VBDS who were followed up at five hospitals in northern China from January 2003 to April *** and pathological data at time of biopsy were *** outcomes including cirrhosis,decompensation events,liver transplantation(LT),and liver-related death were *** regression analysis was used to identify the risk factors associated with poor ***:A total of 183 patients were *** median age was 47 years,with 77.6%being *** a median follow-up of 4.8 years,88 patients developed compensated or decompensated cirrhosis,27 died,and 15 received *** Cox regression analysis showed that hepatocellular cholestasis(HR 2.953,95%CI:1.437–6.069),foam cells(HR 2.349,95%CI:1.092–5.053),and advanced fibrosis(HR 2.524,95%CI:1.313–4.851)were independent predictors of LT or liver-related deaths.A nomogram formulated with the above factors showed good consistency with a concordance index of 0.746(95%CI:0.706–0.785).Conclusions:Nearly half of VBDS patients studied progressed to end-stage liver disease and 23%of them had LT or liver-related death within two years of *** cholestasis,foam cells and advanced fibrosis rather than the degree of bile duct loss or underlying etiologies were independently associated with poor prognosis in VBDS patients.

关键词： bile duct loss Prognosis Cholestasis Foam cells Advanced fibrosis

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