en Vanishing bile duct syndrome

Vanishing bile duct syndrome
Vanishing bile duct syndrome
Other names	Ductopenia
Specialty	Gastroenterology

Vanishing bile duct syndrome is a loose collection of diseases leading to hepatic bile duct injury 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

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

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

References

^ 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.
^ 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.

External links

Uptodate:Hepatic ductopenia and vanishing bile duct syndrome

[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.

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