Vanishing bile duct syndrome

Vanishing bile duct syndrome is a loose collection of diseases which leads to the injury to hepatic bile ducts and eventual ductopenia.[1]

Vanishing bile duct syndrome
Other namesDuctopenia
SpecialtyGastroenterology

Signs and symptoms

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

  • Fatigue
  • Anorexia
  • Abdominal pain
  • Weight loss
  • Pruritus
  • Hyperlipidemia
  • Malabsorption
  • Fat-soluble vitamin deficiencies
  • Elevated alkaline phosphatase
  • Elevated gamma-glutamyltransferase
  • Elevated conjugated bilirubin

Cause

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

Treatment

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

Medical therapies

References

  1. Reau NS, Jensen DM (February 2008). "Vanishing bile duct syndrome". Clin Liver Dis. 12 (1): 203–17, x. doi:10.1016/j.cld.2007.11.007. PMID 18242505.
  2. Rossini, M. S.; Lorand-Metze, I; Oliveira, G. B.; Souza, C. A. (2000). "Vanishing bile duct syndrome in Hodgkin's disease: Case report". Sao Paulo medical [Revista paulista de medicina]. 118 (5): 154–7. PMID 11018850.
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