Outcome of Living Donor Liver Transplantation for Wilson's Disease in Adults: A Single Center Experience.

Introduction: Although liver transplantation is a definitive cure for Wilson's disease (WD), there is limited data about results of living donor liver transplantation (LDLT) in adults.

Material and methods: 18 adults underwent LDLT for WD. The presentations before LDLT were decompensated cirrhosis ( n  = 16), acute on chronic liver failure ( n  = 1) and acute liver failure ( n  = 1). The donors were parents ( n  = 2), siblings ( n  = 3), cousin ( n  = 1), daughter ( n  = 1), nephew ( n  = 1), spouse or relatives of spouse ( n  = 9) and from swap transplantation ( n  = 1). All genetically related donors were negative for screening of WD.

Results: The study cohort comprised of 15 males and 3 females, aged 32 ± 10 years. Severity of liver disease (excluding acute liver failure patient) was as follows; Child's score 10 ± 2, model for end-stage liver disease (MELD) score 18 ± 6. The graft to recipient weight ratio was 1 ± 0.2. The ICU and hospital stay were 5.5 ± 0.9 and 15 ± 5 days. Two patients died in first month after liver transplantation, rest of patients are doing well at median 15 (8-38 months). Two patients had acute cellular rejection that responded to steroids, one had hepatic artery thrombosis and 2 had biliary strictures. Three patients had neurological symptoms; 2 of these patients had partial recovery while one had complete recovery. There was no significant difference between LDLT from genetically related or unrelated donors.

Conclusion: LDLT for WD in adults is associated with good outcomes.