LIVER-TRANSPLANTATION FOR HEREDITARY HEMOCHROMATOSIS

Although the experience of orthotopic liver transplantation (OLT) for hereditary hemochromatosis (HHC) is limited, the existing data indicat e that it carries a higher mortality when compared to transplantation for other causes of end-stage liver disease, Posttransplantation death s are usually related to infectious or cardiac complications. HHC is o ften not diagnosed prior to OLT and one series has shown a high incide nce of primary liver cancer diagnosed incidentally only at the time of transplantation. Factors that may account for the increase in postope rative mortality for HHC are the extent of iron deposition in extrahep atic sites in patients undiagnosed and thus untreated prior to transpl antation. A high index of suspicion in subjects with end-stage liver d isease should lead to improved diagnosis and allow for the prompt inst itution of either phlebotomy therapy or iron chelation therapy prior t o transplantation, It is expected that these changes would reduce post operative complications as well as improve long-term survival.