Liver transplantation for Wilson’s cirrhosis: one center’s experience

Abstract

KINNIER WILSON described Wilson’s disease as a familial nervous system disease that occurs in conjunction with cirrhosis and coined the term “progressive lenticular degeneration.” A defect in copper metabolism is the main problem in this condition. Cirrhosis occurs secondarily due to copper deposition in the liver; lenticular degeneration results from copper deposition at basal ganglia; and the so-called “Kayser-Fleischer ring” can appear due to copper deposition in the peripheral cornea. Previous reports have stated that Wilson’s disease may be a valid indication for liver transplantation in cases where there are neurologic manifestations with stable liver function, as well as in those with hepatic manifestations of cirrhosis or acute liver failure. The 1997 conference organized by the American Society of Transplant Physicians and the American Association for the Study of Liver Diseases discussed unusual or rare indications for liver transplantation, including Budd-Chiari syndrome, Wilson’s disease, and other hereditary disorders. It was agreed at this meeting that there should be no absolute contraindications to placement of patients on the liver transplant waiting list, and that the criteria should be open to regular review to accommodate advances in the field. In Turkey, the most common etiologic factor in end-stage liver disease is postnecrotic cirrhosis, followed by congenital diseases of the liver. Our center has been the pioneer of organ transplantation in our region, we performed the first liver transplantation in Turkey and in the Middle East as a whole. Between May 1988 and October 1998 we carried out 46 liver transplantations, 38 orthotopic (21 segmental living-related and 17 full-size cadaveric) and eight auxiliary heterotopic (three segmental living-related and five splitliver cadaveric). In this study, we report our experience with liver transplantation as a treatment for Wilson’s cirrhosis, the second most common etiologic factor in our series (8 of 46) after postnecrotic cirrhosis (19 of 46).