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Vanishing bile duct syndrome (VBDS) (ductopenia)

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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Vanishing bile duct syndrome (VBDS) ("ductopenia")

  • refers to a group of disorders characterized by prolonged cholestasis as a result of destruction and disappearance of intrahepatic bile ducts
    • consists of a series of diseases characterized by the loss of >50% bile duct in portal areas (1)
  • multiple etiologies have been indentified including infections, neoplastic disorders, autoimmune conditions and drugs
    • exact mechanism underlying this syndrome is unknown; however, common causes include autoimmune disorders such as:
      • primary biliary cirrhosis (PBC),
      • neoplasms,
      • infections
      • drug toxicities (2)
        • for example cloxacillin, an antistaphylococcal penicillin (3)
  • natural history of this condition is variable and may involve resolution of cholestasis or progression with irreversible damage

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