Tissue damage, cardiac and hepatic failure are the most frequent consequences of chronic iron accumulation within the body. A long term administration of chelating agents may prevent organ damage by surplus of iron as well as improve cardiac and liver function in iron overloaded patients. Desferrioxamine (Desferal) is the only one chelating agent for routine clinical use. To produce a therapeutic effect parenteral administration of the drug in prolonged infusions is needed and therefore many investigators try to search for orally active chelator with effect comparable to desferrioxamine. Hydroxypyridones, especially 1,2-dimethyl-3-hydroxypyrid-4-one (L 1-Deferiprone), are the most intensively studied oral iron chelators. In animal and clinical studies L 1 administration caused iron excretion comparable to that obtained by desferrioxamine, however, some serious adverse effects (including agranulocytosis) related to L 1 treatment were observed. This problem still precludes wide large-scale clinical application of L 1. Other compounds possessing chelating activity after oral administration, eg. 1,2-diethyl-3-hydroxypyridone, PIH or HBED are also currently under investigation. Development of a safe inexpensive and orally effective iron chelator is the main objective of ongoing animal and clinical studies.

Ustav hematologie a krevní transfuze Praha