Normal Iron Metabolism and the Pathophysiology of Iron Overload Disorders
Identifieur interne : 000481 ( Pmc/Curation ); précédent : 000480; suivant : 000482Normal Iron Metabolism and the Pathophysiology of Iron Overload Disorders
Auteurs : Chiang W. Siah ; John Ombiga ; Leon A. Adams ; Debbie Trinder [Australie] ; John K. Olynyk [Australie]Source :
- Clinical Biochemist Reviews [ 0159-8090 ] ; 2006.
Abstract
Iron overload disorders represent a heterogenous group of conditions resulting from inherited and acquired causes. If undiagnosed they can be progressive and fatal. Early detection and phlebotomy prior to the onset of cirrhosis can reduce morbidity and normalise life expectancy. We now have greater insight into the complex mechanisms of normal and disordered iron homeostasis following the discovery of new proteins and genetic defects. Here we review the normal mechanisms and regulation of gastrointestinal iron absorption and liver iron transport and their dysregulation in iron overload states. Advances in the understanding of the natural history of iron overload disorders and new methods for clinical detection and management of hereditary haemochromatosis are also reviewed. The current screening strategies target high-risk groups such as first-degree relatives of affected individuals and those with clinical features suggestive of iron loading. Potential ethical, legal and psychosocial issues arising through application of genetic screening programs need to be resolved prior to implementation of general population screening programs.
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PubMed: 16886043
PubMed Central: 1390789
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Chiang W. Siah<affiliation><nlm:aff id="af1-cbr27_1p005"> Department of Gastroenterology, Liverpool Hospital, Elizabeth Street, Liverpool, NSW 2170,</nlm:aff><wicri:noCountry code="subfield">NSW 2170</wicri:noCountry></affiliation><affiliation><nlm:aff id="af2-cbr27_1p005"> Department of Gastroenterology, Fremantle Hospital, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation><nlm:aff id="af2-cbr27_1p005"> Department of Gastroenterology, Fremantle Hospital, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation><nlm:aff id="af3-cbr27_1p005"> School of Medicine and Pharmacology, University of Western Australia, Fremantle Hospital Campus, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation><nlm:aff id="af3-cbr27_1p005"> School of Medicine and Pharmacology, University of Western Australia, Fremantle Hospital Campus, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation><nlm:aff id="af3-cbr27_1p005"> School of Medicine and Pharmacology, University of Western Australia, Fremantle Hospital Campus, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation wicri:level="1"><nlm:aff id="af4-cbr27_1p005"> Western Australian Institute of Medical Research, Perth, WA, Australia</nlm:aff><country xml:lang="fr">Australie</country><wicri:regionArea> Western Australian Institute of Medical Research, Perth, WA</wicri:regionArea></affiliation><affiliation><nlm:aff id="af2-cbr27_1p005"> Department of Gastroenterology, Fremantle Hospital, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation><nlm:aff id="af3-cbr27_1p005"> School of Medicine and Pharmacology, University of Western Australia, Fremantle Hospital Campus, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation wicri:level="1"><nlm:aff id="af4-cbr27_1p005"> Western Australian Institute of Medical Research, Perth, WA, Australia</nlm:aff><country xml:lang="fr">Australie</country><wicri:regionArea> Western Australian Institute of Medical Research, Perth, WA</wicri:regionArea></affiliation><affiliation><nlm:aff id="af3-cbr27_1p005"> School of Medicine and Pharmacology, University of Western Australia, Fremantle Hospital Campus, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation wicri:level="1"><nlm:aff id="af4-cbr27_1p005"> Western Australian Institute of Medical Research, Perth, WA, Australia</nlm:aff><country xml:lang="fr">Australie</country><wicri:regionArea> Western Australian Institute of Medical Research, Perth, WA</wicri:regionArea></affiliation>Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Normal Iron Metabolism and the Pathophysiology of Iron Overload Disorders</title><author><name sortKey="Siah, Chiang W" sort="Siah, Chiang W" uniqKey="Siah C" first="Chiang W" last="Siah">Chiang W. Siah</name><affiliation><nlm:aff id="af1-cbr27_1p005"> Department of Gastroenterology, Liverpool Hospital, Elizabeth Street, Liverpool, NSW 2170,</nlm:aff><wicri:noCountry code="subfield">NSW 2170</wicri:noCountry></affiliation></author><author><name sortKey="Ombiga, John" sort="Ombiga, John" uniqKey="Ombiga J" first="John" last="Ombiga">John Ombiga</name><affiliation><nlm:aff id="af2-cbr27_1p005"> Department of Gastroenterology, Fremantle Hospital, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation></author><author><name sortKey="Adams, Leon A" sort="Adams, Leon A" uniqKey="Adams L" first="Leon A" last="Adams">Leon A. 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Siah</name><affiliation><nlm:aff id="af1-cbr27_1p005"> Department of Gastroenterology, Liverpool Hospital, Elizabeth Street, Liverpool, NSW 2170,</nlm:aff><wicri:noCountry code="subfield">NSW 2170</wicri:noCountry></affiliation></author><author><name sortKey="Ombiga, John" sort="Ombiga, John" uniqKey="Ombiga J" first="John" last="Ombiga">John Ombiga</name><affiliation><nlm:aff id="af2-cbr27_1p005"> Department of Gastroenterology, Fremantle Hospital, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation></author><author><name sortKey="Adams, Leon A" sort="Adams, Leon A" uniqKey="Adams L" first="Leon A" last="Adams">Leon A. Adams</name><affiliation><nlm:aff id="af2-cbr27_1p005"> Department of Gastroenterology, Fremantle Hospital, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation><nlm:aff id="af3-cbr27_1p005"> School of Medicine and Pharmacology, University of Western Australia, Fremantle Hospital Campus, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation></author><author><name sortKey="Trinder, Debbie" sort="Trinder, Debbie" uniqKey="Trinder D" first="Debbie" last="Trinder">Debbie Trinder</name><affiliation><nlm:aff id="af3-cbr27_1p005"> School of Medicine and Pharmacology, University of Western Australia, Fremantle Hospital Campus, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation wicri:level="1"><nlm:aff id="af4-cbr27_1p005"> Western Australian Institute of Medical Research, Perth, WA, Australia</nlm:aff><country xml:lang="fr">Australie</country><wicri:regionArea> Western Australian Institute of Medical Research, Perth, WA</wicri:regionArea></affiliation></author><author><name sortKey="Olynyk, John K" sort="Olynyk, John K" uniqKey="Olynyk J" first="John K" last="Olynyk">John K. Olynyk</name><affiliation><nlm:aff id="af2-cbr27_1p005"> Department of Gastroenterology, Fremantle Hospital, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation><nlm:aff id="af3-cbr27_1p005"> School of Medicine and Pharmacology, University of Western Australia, Fremantle Hospital Campus, Alma Street, Fremantle, WA 6160,</nlm:aff><wicri:noCountry code="subfield">WA 6160</wicri:noCountry></affiliation><affiliation wicri:level="1"><nlm:aff id="af4-cbr27_1p005"> Western Australian Institute of Medical Research, Perth, WA, Australia</nlm:aff><country xml:lang="fr">Australie</country><wicri:regionArea> Western Australian Institute of Medical Research, Perth, WA</wicri:regionArea></affiliation></author></analytic><series><title level="j">Clinical Biochemist Reviews</title><idno type="ISSN">0159-8090</idno><imprint><date when="2006">2006</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Iron overload disorders represent a heterogenous group of conditions resulting from inherited and acquired causes. If undiagnosed they can be progressive and fatal. Early detection and phlebotomy prior to the onset of cirrhosis can reduce morbidity and normalise life expectancy. We now have greater insight into the complex mechanisms of normal and disordered iron homeostasis following the discovery of new proteins and genetic defects. Here we review the normal mechanisms and regulation of gastrointestinal iron absorption and liver iron transport and their dysregulation in iron overload states. Advances in the understanding of the natural history of iron overload disorders and new methods for clinical detection and management of hereditary haemochromatosis are also reviewed. The current screening strategies target high-risk groups such as first-degree relatives of affected individuals and those with clinical features suggestive of iron loading. Potential ethical, legal and psychosocial issues arising through application of genetic screening programs need to be resolved prior to implementation of general population screening programs.</p></div></front></TEI><pmc article-type="review-article"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Clin Biochem Rev</journal-id><journal-title>Clinical Biochemist Reviews</journal-title><issn pub-type="ppub">0159-8090</issn><publisher><publisher-name>The Australian Association of Clinical Biochemists</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">16886043</article-id><article-id pub-id-type="pmc">1390789</article-id><article-id pub-id-type="publisher-id">cbr27_1p005</article-id><article-categories><subj-group subj-group-type="heading"><subject>Review Article</subject></subj-group></article-categories><title-group><article-title>Normal Iron Metabolism and the Pathophysiology of Iron Overload Disorders</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Siah</surname><given-names>Chiang W</given-names></name><xref ref-type="aff" rid="af1-cbr27_1p005">1</xref></contrib><contrib contrib-type="author"><name><surname>Ombiga</surname><given-names>John</given-names></name><xref ref-type="aff" rid="af2-cbr27_1p005">2</xref></contrib><contrib contrib-type="author"><name><surname>Adams</surname><given-names>Leon A</given-names></name><xref ref-type="aff" rid="af2-cbr27_1p005">2</xref><xref ref-type="aff" rid="af3-cbr27_1p005">3</xref></contrib><contrib contrib-type="author"><name><surname>Trinder</surname><given-names>Debbie</given-names></name><xref ref-type="aff" rid="af3-cbr27_1p005">3</xref><xref ref-type="aff" rid="af4-cbr27_1p005">4</xref><xref ref-type="corresp" rid="c1-cbr27_1p005">*</xref></contrib><contrib contrib-type="author"><name><surname>Olynyk</surname><given-names>John K</given-names></name><xref ref-type="aff" rid="af2-cbr27_1p005">2</xref><xref ref-type="aff" rid="af3-cbr27_1p005">3</xref><xref ref-type="aff" rid="af4-cbr27_1p005">4</xref></contrib></contrib-group><aff id="af1-cbr27_1p005"><label>1</label> Department of Gastroenterology, Liverpool Hospital, Elizabeth Street, Liverpool, NSW 2170,</aff><aff id="af2-cbr27_1p005"><label>2</label> Department of Gastroenterology, Fremantle Hospital, Alma Street, Fremantle, WA 6160,</aff><aff id="af3-cbr27_1p005"><label>3</label> School of Medicine and Pharmacology, University of Western Australia, Fremantle Hospital Campus, Alma Street, Fremantle, WA 6160,</aff><aff id="af4-cbr27_1p005"><label>4</label> Western Australian Institute of Medical Research, Perth, WA, Australia</aff><author-notes><corresp id="c1-cbr27_1p005">* For correspondence: Professor John K Olynyk e-mail:
<email>jolynyk@meddent.uwa.edu.au</email></corresp></author-notes><pub-date pub-type="ppub"><month>2</month><year>2006</year></pub-date><volume>27</volume><issue>1</issue><fpage>5</fpage><lpage>16</lpage><copyright-statement>The contents of articles or advertisements in The Clinical Biochemist – Reviews are not to be construed as official statements, evaluations or endorsements by the AACB, its official bodies or its agents. Statements of opinion in AACB publications are those of the contributors. Print Post Approved - PP255003/01665. Copyright © 2006 The Australasian Association of Clinical Biochemists Inc. No literary matter in The Clinical Biochemist – Reviews is to be reproduced, stored in a retrieval system or transmitted in any form by electronic or mechanical means, photocopying or recording, without permission. Requests to do so should be addressed to the Editor. ISSN 0159 – 8090.</copyright-statement><copyright-year>2006</copyright-year><abstract><p>Iron overload disorders represent a heterogenous group of conditions resulting from inherited and acquired causes. If undiagnosed they can be progressive and fatal. Early detection and phlebotomy prior to the onset of cirrhosis can reduce morbidity and normalise life expectancy. We now have greater insight into the complex mechanisms of normal and disordered iron homeostasis following the discovery of new proteins and genetic defects. Here we review the normal mechanisms and regulation of gastrointestinal iron absorption and liver iron transport and their dysregulation in iron overload states. Advances in the understanding of the natural history of iron overload disorders and new methods for clinical detection and management of hereditary haemochromatosis are also reviewed. The current screening strategies target high-risk groups such as first-degree relatives of affected individuals and those with clinical features suggestive of iron loading. Potential ethical, legal and psychosocial issues arising through application of genetic screening programs need to be resolved prior to implementation of general population screening programs.</p></abstract></article-meta></front></pmc></record>Pour manipuler ce document sous Unix (Dilib)
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