title: Successful liver transplantation in a child with Caroli's disease.
abstract: CD is a rare autosomal recessive disease, characterized by multifocal cystic dilatation of intrahepatic bile ducts. The course of the disease is characterized by intrahepatic cholelithiasis, recurrent episodes of cholangitis, because of cholelithiasis, hepatic abscesses often ending in death caused by uncontrolled infection. Other conditions such as choledochal cyst and renal cystic disease are frequently associated, and patients have a higher risk for the development of cholangiocarcinoma. Endoscopic drainage of the bile duct is palliative and ineffective. OLT appears to be the treatment of choice. In monolobar cases partial liver resection has been shown to be a curative therapeutic option. We report on the course of disease in a Turkish girl who was diagnosed with CD in the neonatal period. At the age of 8.2 yr, she received OLT and is in good health 57 months post-transplantation.
authors.affiliation: Hospital of Children and Adolescents, University of Leipzig Medical Centre, Leipzig, Germany. constance.meier@medizin.uni-leipzig.de
authors.forename: Constance
authors.initials: C
authors.lastname: Meier
authors.forename: Jens
authors.initials: J
authors.lastname: Deutscher
authors.forename: Susanna
authors.initials: S
authors.lastname: Müller
authors.forename: Karin
authors.initials: K
authors.lastname: Haluany
authors.forename: Josef
authors.initials: J
authors.lastname: Fangmann
authors.forename: Werner
authors.initials: W
authors.lastname: Siekmeyer
authors.forename: Thomas
authors.initials: T
authors.lastname: Richter
authors.forename: Wieland
authors.initials: W
authors.lastname: Kiess
mesh_terms: D001653:Bile Ducts, Intrahepatic / Q000473:pathology; D001706:Biopsy; D016767:Caroli Disease / Q000628:therapy*; D002648:Child; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D016031:Liver Transplantation / Q000379:methods*; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents