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Semin Liver Dis 2021; 41(03): 331-348
DOI: 10.1055/s-0041-1729972
Review Article

Drug-Induced Vanishing Bile Duct Syndrome: From Pathogenesis to Diagnosis and Therapeutics

Fernando Bessone
1   Hospital Provincial del Centenario, Facultad de Ciencias Médicas, Servicio de Gastroenterología y Hepatología, Universidad Nacional de Rosario, Rosario, Argentina
,
Nelia Hernández
2   Clínica de Gastroenterología, Hospital de Clínicas y Facultad de Medicina, Universidad de la República (UdelaR), Montevideo, Uruguay
,
Mario Tanno
1   Hospital Provincial del Centenario, Facultad de Ciencias Médicas, Servicio de Gastroenterología y Hepatología, Universidad Nacional de Rosario, Rosario, Argentina
,
Marcelo G. Roma
3   Instituto de Fisiología Experimental (CONICET-UNR), Facultad de Ciencias Bioquímicas y Farmacéuticas, Universidad Nacional de Rosario, Rosario, Argentina
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Erratum to: Drug-Induced Vanishing Bile Duct Syndrome: From Pathogenesis to Diagnosis and TherapeuticsSemin Liver Dis 2021; 41(03): e1-e1
DOI: 10.1055/s-0041-1732497

Abstract

The most concerned issue in the context of drug/herb-induced chronic cholestasis is vanishing bile duct syndrome. The progressive destruction of intrahepatic bile ducts leading to ductopenia is usually not dose dependent, and has a delayed onset that should be suspected when abnormal serum cholestasis enzyme levels persist despite drug withdrawal. Immune-mediated cholangiocyte injury, direct cholangiocyte damage by drugs or their metabolites once in bile, and sustained exposure to toxic bile salts when biliary epithelium protective defenses are impaired are the main mechanisms of cholangiolar damage. Current therapeutic alternatives are scarce and have not shown consistent beneficial effects so far. This review will summarize the current literature on the main diagnostic tools of ductopenia and its histological features, and the differential diagnostic with other ductopenic diseases. In addition, pathomechanisms will be addressed, as well as the connection between them and the supportive and curative strategies for ductopenia management.

Keywords

cholestasis - ductopenia - drug-induced liver injury - vanishing bile duct syndrome - hepatotoxicity

Financial Support and Disclosures

None.




Publication History

Article published online:
15 June 2021

© 2021. Thieme. All rights reserved.

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