Vanishing bile duct syndrome
Medical condition
From Wikipedia, the free encyclopedia
Vanishing bile duct syndrome is a loose collection of diseases leading to hepatic bile duct injury and eventual ductopenia.[1]
Other namesDuctopenia
| Vanishing bile duct syndrome | |
|---|---|
| Other names | Ductopenia |
| Specialty | Gastroenterology |
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
Chromosomal associations
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
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