| A time course of hepcidin response to iron challenge in patients with HFE and TFR2 hemochromatosis. | |
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MedLine Citation:
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PMID: 21173098 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Inadequate hepcidin production leads to iron overload in nearly all types of hemochromatosis. We explored the acute response of hepcidin to iron challenge in 25 patients with HFE-hemochromatosis, in two with TFR2-hemochromatosis and in 13 controls. Sixteen patients (10 C282Y/C282Y homozygotes, 6 C282Y/H63D compound heterozygotes) had increased iron stores, while nine (6 C282Y/C282Y homozygotes, 3 C282Y/H63D compound heterozygotes) were studied after phlebotomy-induced normalization of iron stores. DESIGN AND METHODS: We analyzed serum iron, transferrin saturation, and serum hepcidin by both enzyme-linked immunosorbent assay and mass-spectrometry at baseline, and 4, 8, 12 and 24 hours after a single 65-mg dose of oral iron. RESULTS: Serum iron and transferrin saturation significantly increased at 4 hours and returned to baseline values at 8-12 hours in all groups, except in the iron-normalized patients who showed the highest and longest increase of both parameters. The level of hepcidin increased significantly at 4 hours and returned to baseline at 24 hours in controls and in the C282Y/H63D compound heterozygotes at diagnosis. The hepcidin response was smaller in C282Y-homozygotes than in controls, barely detectable in the patients with iron-depleted HFE-hemochromatosis and absent in those with TFR2-hemochromatosis. Conclusions Our results are consistent with a scenario in which TFR2 plays a prominent and HFE a contributory role in the hepcidin response to a dose of oral iron. In iron-normalized patients with HFE hemochromatosis, both the low baseline hepcidin level and the weak response to iron contribute to hyperabsorption of iron. |
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Authors:
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Domenico Girelli; Paola Trombini; Fabiana Busti; Natascia Campostrini; Marco Sandri; Sara Pelucchi; Mark Westerman; Tomas Ganz; Elizabeta Nemeth; Alberto Piperno; Clara Camaschella |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2010-12-20 |
Journal Detail:
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Title: Haematologica Volume: 96 ISSN: 1592-8721 ISO Abbreviation: Haematologica Publication Date: 2011 Apr |
Date Detail:
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Created Date: 2011-04-01 Completed Date: 2011-08-16 Revised Date: 2011-11-24 |
Medline Journal Info:
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Nlm Unique ID: 0417435 Medline TA: Haematologica Country: Italy |
Other Details:
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Languages: eng Pagination: 500-6 Citation Subset: IM |
Affiliation:
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Department of Medicine, University of Verona, Policlinico GB Rossi, 37134 Verona, Italy. domenico.girelli@univr.it |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Antimicrobial Cationic Peptides / blood* Ferritins / blood Genotype Hemochromatosis / blood*, genetics* Histocompatibility Antigens Class I / genetics Humans Iron / administration & dosage*, blood*, metabolism Male Membrane Proteins / genetics Middle Aged Receptors, Transferrin / genetics Time Factors |
| Grant Support | |
ID/Acronym/Agency:
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GGP06213//Telethon; GGP08089//Telethon |
| Chemical | |
Reg. No./Substance:
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0/Antimicrobial Cationic Peptides; 0/HFE protein, human; 0/Histocompatibility Antigens Class I; 0/Membrane Proteins; 0/Receptors, Transferrin; 0/TFR2 protein, human; 0/hepcidin; 7439-89-6/Iron; 9007-73-2/Ferritins |
| Comments/Corrections | |
Comment In:
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Haematologica. 2011 Apr;96(4):485-8
[PMID:
21454877
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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