Vanishing bile duct syndrome

Vanishing bile duct syndrome
Classification and external resources
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Vanishing bile duct syndrome (or "ductopenia") is a loose collection of diseases which leads to the injury to hepatic bile ducts and eventual ductopenia.

The presentation is dependent upon the underlying cause. The course can be rapid or chronic.

In fetal and neonatal life the ductal plates are remodeled. The malformations can be atretic or fibrocystic.

Bile duct injury and loss can result from autoimmune destruction. T cells recognize biliary epithelial cell antigens causing injury and eventual atresia.

Treatment is dependent upon the underlying cause. Treatment is supportive as it is not possible to induce regrowth of lost ducts.

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