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 ( = 16), acute on chronic liver failure ( = 1) and acute liver failure ( = 1). The donors were parents ( = 2), siblings ( = 3), cousin ( = 1), daughter ( = 1), nephew ( = 1), spouse or relatives of spouse ( = 9) and from swap transplantation ( = 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.