vanishing bile duct syndrome
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vanishing bile duct syndromeAny of a group of acquired hepatopathies characterised by irreversible destructive cholangitis, loss of intrahepatic bile ducts and chronic cholestasis.
Fatigue, anorexia, right upper quadrant pain, malabsorption, weight loss, pruritis.
Hyperlipidaemia, fat-soluble vitamin (A, D, E, K) deficiencies, raised conjugated bilirubin, alkaline phosphatase, GGT-gamma-glutamyltransferase.
Vanishing bile duct syndrome
• Extrahepatic bile duct atresia.
• Autosomal recessive polycystic kidney disease;
• Congential hepatic fibrosis;
• Caroli's disease;
• von Meyenburg complex.
• Alpha-1 antitrypsin deficiency;
• Byler's disease;
• Cystic fibrosis;
• Trihydroxycoprostanic acidaemia;
• Trisomy 17, 18 and 21.
Bile duct injury and loss due to autoimmune destruction.
• Chronic graft-versus-host disease;
• Primary biliary cirrhosis;
• Primary sclerosing cholangitis;
• Hodgkin's disease;