Right lobe liver transplantation for Wilson's disease from heterozygote donor: Case report

Abstract

Background: Use of liver grafts from heterozygote genetic carriers for Wilson's disease was considered safe. We present a 14 year-old male patient with Wilson's disease who was undergone right lobe liver transplantation from his heterozygote mother. Case report: The patient was admitted to our hospital with decompensated liver cirrhosis due to Wilson's disease as a liver recipient candidate. His Child score was 11 (Child C) and MELD score was 28. His weight was 52 kg. Total liver volume of his 40-year-old mother was calculated 1488 mL in computed tomography. Right and left liver lobe of the donor were calculated as 1044/444 (70.16%/29.84%) mL. Preoperative ceruloplasmin level, 24-h urinary copper excretion, serum copper level and liver biopsy of the donor were all normal. Right lobe liver transplant was performed. Recipient and donor were discharged from the hospital uneventfully. The patient's preoperative ceruloplasmin level was 15.3 mg/dL and increased to 22 mg/dL after liver transplant. Also urinary copper decreased from 1939 to 68 μg/day and serum copper improved from 72 to 82 mg/dL after liver transplant. There were no statistically difference between preoperative and postoperative serum copper, ceruloplasmin and 24 h urinary copper levels of the donor. Discussion: Equal outcomes of living related liver transplantation for Wilson's disease were reported for heterozygote and nonheterozygote donors previously. Usually left liver lobes were used for transplantation from heterozygous donors. The use of right liver lobe of the heterozygous donor has no negative impact on both the donor and recipient for Wilson's disease.