title: ["Adult form" of congenital liver fibrosis].
abstract: METHODS
  During a routine check-up, a 37-year-old woman was found to have elevated levels of serum gamma-glutamyl transferase (gamma GTP) and IgA- and IgM-antibodies. One of the patient's brothers had died at the age of six from acute liver failure. We found a palpably enlarged liver with normal consistency and no particular helpful laboratory results.
  
  
  METHODS
  The abdominal ultrasound and computed tomography (CT) showed segmental and saccular dilatations of the biliary tract, hepatofugal flow in the portal vein, multiple collateral vessels as well as a mild splenomegaly. Histopathology revealed fibrotic liver parenchyma, a dilatated and branched biliary tract lined by cubical epithelium.
  
  
  RESULTS
  Gastroscopy showed lowgrade esophageal varices. Seventeen months after the initial presentation there were no significant changes of the laboratory tests or the ultrasound.
  
  
  CONCLUSIONS
  The defective remodelling of the ductal plate ("ductal plate malformation") is associated with dysplasia of the biliary tract. Depending on the localisation of the lesions within the biliary tract and whether it is a more cystic or more fibrotic component, there are different malformations caused by ductal plate malformation. We diagnosed congenital hepatic fibrosis, because of the atypical age of presentation, it could be the "adult form" of congenital liver fibrosis.
authors.affiliation: Klinik und Poliklinik für Innere Medizin I der Universität Regensburg, Regensburg. doscha2@yahoo.com
authors.forename: D
authors.initials: D
authors.lastname: Schacherer
authors.forename: P
authors.initials: P
authors.lastname: Rümmele
authors.forename: J
authors.initials: J
authors.lastname: Schölmerich
mesh_terms: D000328:Adult; D001706:Biopsy; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood; D007075:Immunoglobulin M / Q000097:blood; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008103:Liver Cirrhosis / Q000151:congenital* / Q000175:diagnosis* / Q000196:embryology; D008111:Liver Function Tests; D011877:Radionuclide Imaging; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D005723:gamma-Glutamyltransferase / Q000097:blood
chemical_list: D007070:Immunoglobulin A; D007075:Immunoglobulin M; D005723:gamma-Glutamyltransferase