Normal Iron Metabolism and the Pathophysiology of Iron Overload Disorders
Identifieur interne : 000837 ( Main/Exploration ); précédent : 000836; suivant : 000838Normal 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.
Url:
PubMed: 16886043
PubMed Central: 1390789
Affiliations:
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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><wicri:noRegion>WA</wicri:noRegion></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><wicri:noRegion>WA</wicri:noRegion></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><affiliations><list><country><li>Australie</li></country></list><tree><noCountry><name sortKey="Adams, Leon A" sort="Adams, Leon A" uniqKey="Adams L" first="Leon A" last="Adams">Leon A. Adams</name><name sortKey="Ombiga, John" sort="Ombiga, John" uniqKey="Ombiga J" first="John" last="Ombiga">John Ombiga</name><name sortKey="Siah, Chiang W" sort="Siah, Chiang W" uniqKey="Siah C" first="Chiang W" last="Siah">Chiang W. Siah</name></noCountry><country name="Australie"><noRegion><name sortKey="Trinder, Debbie" sort="Trinder, Debbie" uniqKey="Trinder D" first="Debbie" last="Trinder">Debbie Trinder</name></noRegion><name sortKey="Olynyk, John K" sort="Olynyk, John K" uniqKey="Olynyk J" first="John K" last="Olynyk">John K. 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