This article needs more reliable medical references for verification or relies too heavily on primary sources. (March 2022) |  |
Vanishing bile duct syndrome is a loose collection of diseases leading to hepatic bile duct injury and eventual ductopenia.[1]
| Vanishing bile duct syndrome | |
|---|---|
| Other names | Ductopenia |
| Specialty | Gastroenterology |
Signs and symptoms
editThe 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
editCongenital
editIn fetal and neonatal life the ductal plates are remodeled. The malformations can be atretic or fibrocystic.
Atretic causes
edit- Intrahepatic bile duct atresia (Alagille syndrome) (ALGS2 MIM:610205 and ALGS1 MIM:118450)
- Extrahepatic bile duct atresia
Fibrocystic causes
edit- Autosomal recessive polycystic kidney disease
- Congential hepatic fibrosis
- Caroli's disease
- Von Meyenburg complex
Chromosomal associations
edit- Trisomy 17, 18 and 21
Genetic associations
edit- Cystic fibrosis
- Alpha 1 antitrypsin deficiency
- Trihydroxycoprostanic acidemia
- Byler's disease
Immunologic associations
editBile duct injury and loss can result from autoimmune destruction. T cells recognize biliary epithelial cell antigens causing injury and eventual atresia.
Other causes
edit- Primary biliary cirrhosis
- Primary sclerosing cholangitis
- Hodgkin's lymphoma[2]
- Chronic graft-versus-host disease
- Drugs(chlorpromazine)/Toxins
- Ischemia
Diagnosis
editThis section is empty. You can help by adding to it. (March 2018) |
Treatment
editTreatment is dependent upon the underlying cause. Treatment is supportive as it is not possible to induce regrowth of lost ducts.
Medical therapies
edit- Ursodeoxycholic acid
- Immunosuppression
- General consensus is that more studies are needed before this can be considered
- Organ transplant
References
edit- ^ 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.