Vanishing bile duct syndrome

Last updated
Vanishing bile duct syndrome
Other namesDuctopenia
Specialty Gastroenterology

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

Contents

Signs and symptoms

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

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

Chromosomal associations

Genetic associations

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

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 Journal. 118 (5): 154–7. doi: 10.1590/s1516-31802000000500008 . PMC   11175542 . PMID   11018850.