vanishing bile duct syndrome


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vanishing bile duct syndrome

Any of a group of acquired hepatopathies characterised by irreversible destructive cholangitis, loss of intrahepatic bile ducts and chronic cholestasis.

Clinical findings
Fatigue, anorexia, right upper quadrant pain, malabsorption, weight loss, pruritis.

Lab
Hyperlipidaemia, fat-soluble vitamin (A, D, E, K) deficiencies, raised conjugated bilirubin, alkaline phosphatase, GGT-gamma-glutamyltransferase.

Vanishing bile duct syndrome
Malformation
• Extrahepatic bile duct atresia.

Fibrosis
• Autosomal recessive polycystic kidney disease;
• Congential hepatic fibrosis;
• Caroli's disease;
• von Meyenburg complex.

Chromosomal/genetic associations
• Alpha-1 antitrypsin deficiency;
• Byler's disease;
• Cystic fibrosis;
• Trihydroxycoprostanic acidaemia;
• Trisomy 17, 18 and 21.

Immune associations
Bile duct injury and loss due to autoimmune destruction.

Others
• Chronic graft-versus-host disease;
• Drugs/Toxins;
• Ischaemia;
• Primary biliary cirrhosis;
• Primary sclerosing cholangitis;
• Hodgkin's disease;
 •Liver transplantation.
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