Hypersidéroses de l'enfant

Abstract

Iron overload in children is most often encountered in a context of chronic congenital anaemia requiring regular blood transfusions. Symptomatic organ disease related to hereditary haemochromatosis begins in the 4th or the 5th decade, except in the rare cases of juvenile phenotype. The use of parenteral desferrioxamine treatment that remains the standard regimen for iron chelation have resulted in a dramatic improvement in mortality and morbidity of patients suffering from major thalassaemia, the most frequent disease worldwide leading to severe transfusional iron overload. However, this therapy has 2 major limitations: its cost and the inability for some patients to cope with its cumbersome long-term use. Recent years have opened the era of orally active iron chelators.