Iron Overload and Chelation Therapy

Kenneth R Bridges, Brigham and Women's Hospital, Boston, Massachusetts, USA

Published online: April 2001

DOI: 10.1038/npg.els.0002278

Iron overload (haemochromatosis) is a disorder of iron metabolism commonly due to hereditary haemochromatosis or chronic transfusions. Chelation is the only available treatment for transfusional iron overload.

Keywords: iron; haemochromatosis; chelation; transfusions; ferritin

 References
    Bassett M, Halliday J and Powell L (1986) Value of hepatic iron measurements in early hemochromatosis and determination of the critical iron level associated with fibrosis. Hepatology 6: 24–28.
    Celada A (1982) Iron overload in a non-transfused patient with thalassaemia intermedia. Scandinavian Journal of Haematology 28: 169–174.
    Feder NJ, Penny DM, Irrinki A et al. (1998) The hemochromatosis gene product complexes with the transferrin receptor and lowers its affinity for ligand binding. Proceedings of the National Academy of Sciences of the USA 95: 1472–1477.
    Gordeuk V, Boyd R and Brittenham G (1986) Dietary iron overload persists in rural sub-Saharan Africa. Lancet i: 1310–1313.
    Olivieri NF, Brittenham GM, McLaren CE et al. (1998) Long-term safety and effectiveness of iron-chelation therapy with deferiprone for thalassaemia major. New England Journal of Medicine 339(7): 417–423.
    Powell LW, George DK, McDonnell SM and Kowdley KV (1998) Diagnosis of hemochromatosis. Annals of Internal Medicine 129(11): 925–931.
    Propper R, Cooper B, Rufo R et al. (1977) Continuous subcutaneous administration of deferoxamine in patients with iron overload. New England Journal of Medicine 297: 418–423.
    Schafer A, Rabinowe S, Le Boff M et al. (1985) Long-term efficacy of deferoxamine iron chelation therapy in adults with acquired transfusional iron overload. Archives of Internal Medicine 45: 1217–1221.
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    Wolfe L, Oliver N, Sallan D et al. (1985) Prevention of cardiac disease by subcutaneous deferoxamine in patients with thalassaemia major. New England Journal of Medicine 312: 1600–1603.
 Further Reading
    Bonkovsky HL (1991) Iron and the liver. American Journal of Medical Science 301: 32–43.
    Nielsen P, Carpinteiro S, Fischer R et al. (1998) Prevalence of the C282Y and H63D mutations in the HFE gene in patients with hereditary haemochromatosis and in control subjects from northern Germany. British Journal of Haematology 103(3): 842–845.
    Pippard MJ (1987) Iron overload and iron chelation therapy in thalassaemia and sickle cell haemoglobinopathies. Acta Haematologica 78: 206–211.

Related Sub-Topics:

Specific Genetic Disorders | Mutational Mechanisms of Genetic Disease | Biomolecular Interactions | Proteins: Structure, Function, Metabolism
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Article Title: Iron Overload and Chelation Therapy
 
How to Cite close
Bridges, Kenneth R(Apr 2001) Iron Overload and Chelation Therapy. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1038/npg.els.0002278]