Vanishing bile duct syndrome

From Wikipedia, the free encyclopedia
Vanishing bile duct syndrome
Other namesDuctopenia
SpecialtyGastroenterology

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

Signs and symptoms[]

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

Signs and symptoms[]

  • 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[]

  • Primary biliary cirrhosis
  • Primary sclerosing cholangitis
  • Hodgkin's lymphoma[2]
  • Chronic graft-versus-host disease
  • Drugs(chlorpromazine)/Toxins
  • Ischemia

Diagnosis[]

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.

External links[]

Classification
Retrieved from ""