nocturnally on 5 nights per week. Simultaneous oral administration of ascorbic acid is to be avoided; it increases the availability of free iron for chelation but carries the risk of mobilising iron from relatively safe reticuloendothelial storage sites to a potentially toxic pool in parenchymal cells. This regimen can put a transfusion-dependent patient into the desired negative iron balance. Compliance is often a problem and is typically difficult during teenage years in those with lifelong transfusion-dependence. The expense of chelation therapy over a long period is currently enormous and raises serious ethical problems in economically poor countries where most patients with thalassaemia and haemoglobinopathies live.
A safe, effective, inexpensive, orally-absorbed iron chelating agent would improve compliance and the quality of life of affected patients. Deferiprone, which is the best of many agents examined, is less effective than desferrioxamine, carries a risk of agranulocytosis and may itself cause tissue fibrosis. It remains under clinical trial but may be too toxic for general use.
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