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Excess iron reacts with other substances in tissues leading to the
formation of reactive oxygen species (ROS). Uncontained ROS damages
cells, which leads to cell death, then breakdown of tissue and
eventually severe organ damage. One of the organs frequently
affected is the heart. As iron accumulates in the heart, the ability
of its cells to perform functions, such as contracting or
transmitting electrical impulses, is impaired. Over time, this can
lead to serious complications. Thus, in the case of patients with
iron overload, rescue of iron from the heart is key to survival. A
recent follow-up analysis of 1073 individuals with thalassaemia
major in 7 Italian teaching hospitals found that during the course
of the study 59.6% of deaths were due to heart failure, 6.7% to
arrhythmia, 6.7% to infection and 4% were attributed to liver
disease (Borgna-Pignatti, 2001).
The use of Ferriprox® in the management of iron overload has been
shown to have a beneficial effect on the heart (Anderson
2003; Piga, 2003).
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