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Vanishing bile duct syndrome is a loose collection of diseases leading to hepatic bile duct injury and eventual ductopenia.[1]
 | This article needs more reliable medical references for verification or relies too heavily on primary sources. (March 2022) |  |
| Vanishing bile duct syndrome | |
|---|---|
| Other names | Ductopenia |
| Specialty | Gastroenterology |
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
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
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![[icon]](#/media/File:Wiki_letter_w_cropped.svg){kind=small} | This section is empty. You can help by adding to it. (March 2018) |
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
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