Liver transplantation for Wilson disease: Current knowledge and future perspectives

Area of Science:

• Hepatology
• Transplant Surgery
• Neurology

Background:

• Wilson disease (WD) is a genetic disorder of copper metabolism.
• Liver transplantation is established for end-stage liver disease and acute liver failure in WD.
• Recent focus is on expanding transplant indications for WD.

Purpose of the Study:

• To review the current role of liver transplantation in Wilson disease.
• To explore the potential of transplantation for neuropsychiatric Wilson disease.
• To identify challenges and propose a disease-specific approach for WD transplant evaluation.

Main Methods:

• Review of existing literature on liver transplantation for Wilson disease.
• Analysis of outcomes in patients with end-stage liver disease and acute liver failure.
• Discussion of evidence supporting transplantation for neuropsychiatric symptoms and compensated cirrhosis.

Main Results:

• Liver transplantation demonstrates excellent survival rates in WD patients, especially younger ones without recurrence.
• Transplantation may improve copper homeostasis and ameliorate neuropsychiatric symptoms in selected WD patients.
• Key questions remain regarding pre-transplant assessment and postoperative outcome definitions for neuropsychiatric WD.

Conclusions:

• Liver transplantation is a viable option for advanced liver disease in Wilson disease.
• Careful selection of patients with neuropsychiatric Wilson disease may benefit from transplantation.
• A standardized, disease-specific approach is needed for Wilson disease transplant evaluation and management.