Orthotopic liver transplantation for Wilson's disease

Abstract

An 11-year-old boy with terminal hepatic failure due to Wilson’s disease was treated 18 months ago with orthotopic liver transplantation. Postoperatively, there has been evidence of clearance of body copper stores but without accumulation of copper in biopsy specimens of the transplanted liver after 6 and 17 months. Further follow-up will be necessary before deciding whether the disorder has been cured by liver replacement and in turn whether this constitutes proof that Wilson’s disease is an inborn error of hepatic metabolism. The observations so far are consistent with these conclusions.

Fig. 1. Course after liver replacement

A.L.G. = horse antilymphocyte globulin.

Fig. 2

Urinary excretion of copper after orthotopic liver transplantation for Wilson’s disease.