By James C. Barton, Corwin Q. Edwards, Pradyumna D. Phatak, Robert S. Britton, Bruce R. Bacon
A realistic, clinically-oriented guide of iron overload issues giving a compact advisor to general iron metabolism, iron-related pathobiology, and the analysis and administration of heritable and bought iron overload issues. lots of those issues have been chanced on and characterised purely within the final decade, and are unmentioned or inadequately defined in such a lot texts. Written by means of clinicians for clinicians, this guide summarizes info on diversified iron overload stipulations, together with their background, indicators, signs, and actual exam findings, genetics, genotype-phenotype correlations, pathophysiology, differential analysis and remedy. such a lot physicians, despite distinctiveness, come upon sufferers with systemic or organ-specific iron overload stipulations. This e-book comprises crucial details for training grownup and pediatric clinical experts within the fields of hematology, gastroenterology, hepatology, rheumatology, endocrinology, diabetology, neurology, oncology, dermatology, and inner medication. Pathologists, pharmacologists, geneticists, genetic counselors, and epidemiologists also will locate gigantic, updated sections during this guide which are pertinent to their respective fields of curiosity.
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A pragmatic, clinically-oriented instruction manual of iron overload problems giving a compact advisor to general iron metabolism, iron-related pathobiology, and the prognosis and administration of heritable and purchased iron overload issues. lots of those issues have been stumbled on and characterised simply within the final decade, and are unmentioned or inadequately defined in such a lot texts.
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Additional info for Handbook of Iron Overload Disorders
8. Harrison PM, Arosio P. The ferritins: molecular properties, iron storage function and cellular regulation. Biochim Biophys Acta 1996; 1275: 161–203. 9. Wixom RL, Prutkin L, Munro HN. Hemosiderin: nature, formation, and significance. Int Rev Exp Pathol 1980; 22: 193–225. 10. de Domenico I, Vaughn MB, Li L, et al. Ferroportinmediated mobilization of ferritin iron precedes ferritin degradation by the proteasome. EMBO J 2006; 25: 5396–404. 11. Levy JE, Jin O, Fujiwara Y, Kuo F, Andrews NC. Transferrin receptor is necessary for development of erythrocytes and the nervous system.
32 Therefore, evidence has accumulated indicating that iron can produce injury to DNA in patients with hemochromatosis and in experimental animals. This damage might be an important step leading to carcinogenesis, especially when coupled with a proliferative stimulus such as that provided by concomitant cirrhosis of the liver. 2 summarizes several proposed mechanisms of iron-induced toxicity. It has long been suspected that free radicals may play a role in iron-induced cell toxicity because of the powerful pro-oxidant action of iron salts in vitro.
Biochem J 1987; 243: 715–22. 187. Bastin JM, Jones M, O’Callaghan CA, Schimanski L, Mason DY, Townsend AR. Kupffer cell staining by an HFE-specific monoclonal antibody: implications for hereditary haemochromatosis. Br J Haematol 1998; 103: 931–41. 1–5 When storage mechanisms are overwhelmed, iron in low molecular weight forms can play a catalytic role in the initiation of free radical reactions. The resulting oxyradicals have the potential to damage cellular lipids, nucleic acids, proteins, and carbohydrates, resulting in wideranging impairment in cellular function and integrity.