title: Outcome and development of symptoms after orthotopic liver transplantation for Wilson disease.
abstract: BACKGROUND
  Wilson disease (WD) is an autosomal recessive copper storage disease resulting in hepatic and neurologic dysfunction. Liver transplantation is an effective treatment for fulminant cases for patients with chronic liver disease. Reports on the outcome of neuropsychiatric symptoms after orthotopic liver transplantation (OLT) are limited.
  
  
  OBJECTIVE
  To assess the course of neuropsychiatric and hepatic symptoms after liver transplantation for Wilson disease
  
  
  METHODS
  Nineteen patients with Wilson disease received liver transplantation and were followed prospectively from 2005 to 2010 for the development of hepatic, neurological and psychiatric symptoms.
  
  
  RESULTS
  Eight patients (all female) were transplanted for acute liver failure and eleven patients for chronic liver failure. Patient survival rates one and five yr after transplantation were 78% and 65%, respectively. Of the surviving patients, hepatic symptom scores improved in all patients and neurological symptom scores improved in all but one patient after OLT compared to the time of initial diagnosis and compared to pre-OLT status. Psychiatric symptoms showed moderate improvements.
  
  
  CONCLUSIONS
  Survival after OLT for Wilson disease with end-stage liver disease is excellent. Overall, neuropsychiatric symptoms improved after transplantation, substantiating arguments for widening of the indication for liver transplantation in symptomatic neurologic Wilson disease patients with stable liver function.
authors.affiliation: Department of Gastroenterology, University Hospital Heidelberg, Heidelberg, Germany.
authors.forename: Karl Heinz
authors.initials: KH
authors.lastname: Weiss
authors.forename: Mark
authors.initials: M
authors.lastname: Schäfer
authors.forename: Daniel Nils
authors.initials: DN
authors.lastname: Gotthardt
authors.forename: Alexandra
authors.initials: A
authors.lastname: Angerer
authors.forename: Carolin
authors.initials: C
authors.lastname: Mogler
authors.forename: Peter
authors.initials: P
authors.lastname: Schirmacher
authors.forename: Peter
authors.initials: P
authors.lastname: Schemmer
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Stremmel
authors.forename: Peter
authors.initials: P
authors.lastname: Sauer
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D006527:Hepatolenticular Degeneration / Q000401:mortality / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation; D009422:Nervous System Diseases / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D009483:Neuropsychological Tests; D011183:Postoperative Complications; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies; D012307:Risk Factors; D055815:Young Adult
keywords: ATP7B; Wilson's disease; copper; neurologic deterioration; orthotopic liver transplantation