There is a genetic problem in living donor liver transplantat,ion, involvin
g Wilson's disease, because the majority of donors have a kinship relations
hip. Recently, it was reported that the serum ceruloplasmin level is insuff
icient in some persons with one allele mutation.
The recipient was a 13-year-old male child, and the donor was a 22-year-old
woman, who was his sister by a different father. The gene analysis for Wil
son's disease (ATP7B gene) was preoperatively carried out by the amplificat
ion refractory mutation system-PCR.
Homozygous and heterozygous deletion of 2871 cytosine (C) were detected in
the recipient and donor, respectively, in the ATP7B gene. Serum. ceruloplas
min level was sufficient in the donor. The right hepatic lobe graft was tra
nsplanted to the recipient. Immediately after the liver transplantation, th
e copper metabolism improved to increase the serum ceruloplasmin levels up
to the normal range, and decrease the urinary copper excretion. However, th
e serum ceruloplasmin levels gradually decreased below the normal base line
, although the urine copper levels continued to be low without any clinical
We should perform gene analyses and confirm the serum ceruloplasmin levels
in donors before living donor liver transplantation for Wilson's disease, t
o screen for their impairment of copper metabolism. After living donor live
r transplantation for Wilson's disease, we should carefully follow-up the t
ransition of serum ceruloplasmin levels in the recipient.