vanishing bile duct syndrome vanishing bile duct syndrome vanishing bile duct syndrome is a loose collection of diseases which leads to the injury to hepatic bile ducts and eventual ductopenia. vanishing bile duct syndromeother namesductopeniaspecialtygastroenterology 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 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 chronic graft-versus-host disease drugs(chlorpromazine)/toxins ischemia 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