[Hypersiderosis and dissiderosis in the context of data on hemochromatosis microelementosis].
The review summarizes the results of the state-of-the-art studies of hemochromatosis (HC): iron-regulatory genes (HFE, HJV, HAMP, TFR2, SLC40A1) have been discovered; the HC types caused by mutations in these genes (types 1, 2, 3, and 4 in the OMIM register) have been identified; the inflammation anemia (IA) mediator - the polypeptide hepatic hormone hepcidin that is an important constituent of the natural immunity system - has been found. This gives an idea of hypersiderosis and dissiderosis as types of iron microelementosis. Types 1, 2, and 3 HC in which iron absorption and its total reserves are increased in the body serve as examples of hypersiderosis. Dissiderosis is characterized by the redistribution of iron between the functional and spare funds inherent in type 4 HC and IA. By taking into account their findings, the authors briefly discuss the leading clinical and morphological manifestations of HC and IA, difficulties in differential diagnosis, and treatment ways and prospects.
Smirnov
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D000740:Anemia / Q000235:genetics / Q000378:metabolism* / Q000628:therapy; D000818:Animals; D023181:Antimicrobial Cationic Peptides / Q000235:genetics / Q000378:metabolism; D027682:Cation Transport Proteins / Q000235:genetics / Q000378:metabolism; D006432:Hemochromatosis / Q000235:genetics / Q000378:metabolism* / Q000628:therapy; D000071020:Hemochromatosis Protein; D064451:Hepcidins; D015395:Histocompatibility Antigens Class I / Q000235:genetics / Q000378:metabolism; D006801:Humans; D007501:Iron / Q000378:metabolism*; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism; D009154:Mutation; D011990:Receptors, Transferrin / Q000235:genetics / Q000378:metabolism; D000098742:Ferroportin
D023181:Antimicrobial Cationic Peptides; D027682:Cation Transport Proteins; C578702:HAMP protein, human; C103673:HFE protein, human; D000071020:Hemochromatosis Protein; D064451:Hepcidins; D015395:Histocompatibility Antigens Class I; D008565:Membrane Proteins; D011990:Receptors, Transferrin; C503640:TFR2 protein, human; D000098742:Ferroportin; D007501:Iron