Vanishing bile duct syndrome explained

Vanishing bile duct syndrome
Synonyms:	Ductopenia
Field:	Gastroenterology

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.

• 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

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

External links

• Uptodate:Hepatic ductopenia and vanishing bile duct syndrome

Notes and References

1. Reau NS, Jensen DM . Vanishing bile duct syndrome . Clin Liver Dis . 12 . 1 . 203–17, x . February 2008 . 18242505 . 10.1016/j.cld.2007.11.007 .
2. 11018850. 2000. Rossini. M. S.. Vanishing bile duct syndrome in Hodgkin's disease: Case report. Sao Paulo Medical Journal . 118. 5. 154–7. Lorand-Metze. I. Oliveira. G. B.. Souza. C. A.. 10.1590/s1516-31802000000500008. free.