Supriya Ghosh (Editor)

Vanishing bile duct syndrome

Updated on
Edit
Like
Comment
Share on FacebookTweet on TwitterShare on LinkedInShare on Reddit

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

Contents

Signs and symptoms

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

Symptoms

  • Fatigue
  • Anorexia
  • Abdominal pain
  • Weight loss
  • Pruritus

    • Signs

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

    • Congenital

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

    Atretic causes

  • Intrahepatic bile duct atresia (Alagille syndrome) (ALGS2 MIM:610205 and ALGS1 MIM:118450)
  • Extrahepatic bile duct atresia

    • Fibrocystic causes

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

    • Chromosomal associations

  • Trisomy 17, 18 and 21

    • 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 lymphoma
  • Chronic graft-versus-host disease
  • Drugs(chlorpromazine)/Toxins
  • Ischemia

    • Treatment

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

    Medical therapies

  • Ursodeoxycholic acid
  • Immunosuppression
  • General consensus is that more studies are needed before this can be considered
  • Organ transplant

    • References

    Vanishing bile duct syndrome Wikipedia
    (Text) CC BY-SA