Wilson disease is an autosomal recessive disorder characterized by the progressive accumulation of copper in the body. The failure of hepatocytes to excrete copper into bile and the decreased copper incorporation into ceruloplasmin causes the metal to accumulate in the body (1-5). In 1993, Wilson disease gene, ATP7B, was identified by separate groups (6-8). ATP7B encodes a metal-transporting P-...

Wilson disease is associated with multisystem involvement. We describe a patient of Wilson disease with severe arthropathy, which completely reversed following liver transplantation. This is the first case report in literature describing the complete reversal of Wilson disease related arthropathy by liver transplantation.

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 20...