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Gastroenterology an illustrated colour text - part 8

Ghép gan có thể là một lựa chọn trong một số bệnh nhân. Mức độ thân đầu tiên nên được kiểm tra, đo lường của ferritin và bão hòa sắt, và thử nghiệm di truyền. Homozygotes có thể sẽ cần điều trị, như dị hợp tử với những nghiên cứu sắt bất thường. | DISORDERS OF IRON AND COPPER METABOLISM 85 reduce life expectancy. Liver transplantation may be an option in some patients. First-degree relatives should be screened with measurement of ferritin and iron saturation and genetic testing. Homozygotes will probably need treatment as will heterozygotes with abnormal iron studies. Heterozygotes with normal iron studies can just be monitored. OTHER IRON OVERLOAD STATES Other liver disorders can cause a rise in ferritin serum iron and hepatic iron content. Haemochromatosis is more severe in patients who drink excessive amounts of alcohol and alcohol abusers without genetic haemochromatosis often have elevated hepatic iron content. A hepatic iron concentration of 10 000 p g is suggestive of haemochromatosis but younger patients may not have such high liver concentrations in which case the hepatic iron index may be helpful. The hepatic iron concentration in pmol g dry weight is divided by the age - a value of 1.9 is consistent with haemochromatosis. Parenteral iron overload Patients who require multiple blood transfusion usually more than 100 units for haematological disorders or who have chronic increased red cell turnover as in chronic haemolysis e.g. p thalassaemia can develop secondary iron overload with iron initially deposited in the Kupffer cells of the liver. Chelation therapy may be necessary to treat this. Porphyria cutanea tarda This condition is characterised by photosensitive skin reactions with pigmentation blistering and scarring Fig. 2 Unlike acute intermittent porphyria there are no acute neurological psychological or abdominal pain attacks. The condition is associated with alcohol use. Liver enzymes are usually abnormal and hepatic iron overload occurs causing liver damage which can be complicated by hepatocellular carcinoma. Treatment is effective and is with venesection as for haemochromatosis. Table 1 Iron studies useful in the diagnosis of haemochromatosis Bantu siderosis This condition affects South .