Eitan Podgaetz, Carlos Chan
Background/Aims: Orthotopic liver transplantation is being used with more frequency as the treatment for Wilson´s disease. The experience at the Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran with orthotopic liver transplantation for Wilson´s disease is reported. We perform an extensive literature review for this treatment modality. Methods: Between january 2000 and june 2003, 23 orthotopic liver transplants were performed at our institution, 2 of them for Wilson´s disease. Both the patients presented with chronic advanced liver disease and one presented neurologic dysfunction. Results: Both the patients were trasplanted without any major complication and are alive 43 and 22 months after the transplant respectively. To our knowledge 370 liver transplants have been reported in the international literature since 1994 for the treatment of Wilson´s disease. Conclusions: Currently, orthotopic liver transplantation should be considered as a major option for the treatment of chronic liver disease in patients with Wilson´s disease. Although it is well known that the transplant only partially corrects the defective metabolism in patients with Wilson´s disease, it does convert the copper kinetics of a homozygous to that of a heterozigote, thus, providing an effective phenotypic cure.
Key words. Wilson¬¥s disease, hepatolenticular degeneration, copper, liver transplantation