Vanishing bile duct syndrome is a loose collection of diseases which leads to of the injury to hepatic bile ducts and eventual ductopenia

Ductopenia

Ductopenia is the histological hallmark of Vanishing Bile Duct Syndrome.The ductopenia is arbitrarily defined as less than 0.5 bile ducts per portal triad.The most common cause of Ductopenia is Primary Biliary Cirrhosis....

.

Congenital/Developmental

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

Fibrocystic causes

• Autosomal recessive polycystic kidney disease
• Congential hepatic fibrosis
• Caroli's disease
• Von Meyenburg complex

Genetic associations

• Cystic fibrosis
• Alpha 1 antitrypsin deficiency
• Trihydroxycoprostanic acidemia
• Byler's disease

Immunologic associations

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

Other causes

• Primary biliary cirrhosis
• Primary sclerosing cholangitis
• Hodgkin's disease
• Chronic graft-versus-host disease
• Drugs/Toxins
• Ischemia

Clinical presentation

The presentation is dependant upon the underlying etiology. The course can be rapid or chronic.

Signs

• Hyperlipidemia
• Malabsorption
• Fat-soluble vitamin deficiencies
• Elevated alkaline phosphatase
• Elevated gamma-glutamyltransferase
• Elevated conjugated bilirubin

Treatment

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

Medical therapies

• Ursodeoxycholic acid
• Immunosuppression
  Immunosuppression
  Immunosuppression involves an act that reduces the activation or efficacy of the immune system. Some portions of the immune system itself have immuno-suppressive effects on other parts of the immune system, and immunosuppression may occur as an adverse reaction to treatment of other...
  
  
  • General consensus is that more studies are needed before this can be considered
• Organ transplant
  Organ transplant
  Organ transplantation is the moving of an organ from one body to another or from a donor site on the patient's own body, for the purpose of replacing the recipient's damaged or absent organ. The emerging field of regenerative medicine is allowing scientists and engineers to create organs to be...
  
  

External links

• Uptodate:Hepatic ductopenia and vanishing bile duct syndrome