Both authors contributed equally.
Outcome and development of symptoms after orthotopic liver transplantation for Wilson disease
Article first published online: 9 OCT 2013
© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Volume 27, Issue 6, pages 914–922, November/December 2013
How to Cite
Outcome and development of symptoms after orthotopic liver transplantation for Wilson disease., , , , , , , , .
Conflict of interest: None.
- Issue published online: 4 DEC 2013
- Article first published online: 9 OCT 2013
- Manuscript Accepted: 5 SEP 2013
- Medical Faculty of the University of Heidelberg
- German Wilson disease patient organization
- neurologic deterioration;
- orthotopic liver transplantation;
- Wilson's disease
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.
To assess the course of neuropsychiatric and hepatic symptoms after liver transplantation for Wilson disease
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.
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.
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.