Treatment of Hereditary Hemochromatosis
Liver Transplantation for Hereditary Hemochromatosis
The American Association for the Study of Liver Diseases Practice Guidelines for Hereditary Hemochromatosis recommends liver transplantation for carefully selected patients with hereditary hemochromatosis. This recommendation is based on the observation that patients with hereditary hemochromatosis who develop end-stage liver disease are at high risk for developing hepatocellular carcinoma (primary liver cancer). Liver transplantation is the only effective treatment for patients with end-stage liver disease due to cirrhosis of the liver with surgically resectable hepatocellular carcinoma. Studies have shown, however, that patients with hereditary hemochromatosis who undergo liver transplantation usually have a poorer average survival after transplantation compared to patients who undergo liver transplantation for other reasons, particularly if the iron overload cannot be adequately removed prior to transplantation. The most common causes of mortality in patients with hereditary hemochromatosis undergoing liver transplantation are fungal infections and cardiac complications.
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