<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>0872-671X</journal-id>
<journal-title><![CDATA[Medicina Interna]]></journal-title>
<abbrev-journal-title><![CDATA[Medicina Interna]]></abbrev-journal-title>
<issn>0872-671X</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Medicina Interna]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0872-671X2024000500050</article-id>
<article-id pub-id-type="doi">10.24950/rspmi.2578</article-id>
<title-group>
<article-title xml:lang="pt"><![CDATA[Hemocromatose]]></article-title>
<article-title xml:lang="en"><![CDATA[Hemochromatosis]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Araújo]]></surname>
<given-names><![CDATA[Margarida]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Antunes]]></surname>
<given-names><![CDATA[Eulália]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Machado]]></surname>
<given-names><![CDATA[Ana Isabel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gonçalves]]></surname>
<given-names><![CDATA[Francisco]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Unidade Local de Saúde de Braga Serviço de Medicina Interna ]]></institution>
<addr-line><![CDATA[Braga ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2024</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2024</year>
</pub-date>
<volume>31</volume>
<numero>4</numero>
<fpage>50</fpage>
<lpage>58</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.pt/scielo.php?script=sci_arttext&amp;pid=S0872-671X2024000500050&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.pt/scielo.php?script=sci_abstract&amp;pid=S0872-671X2024000500050&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.pt/scielo.php?script=sci_pdf&amp;pid=S0872-671X2024000500050&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo A hemocromatose é uma doença hereditária do metabolismo do ferro que resulta de defeitos genéticos no eixo hepcidina-ferroportina, com penetrância variável e, portanto, heterogeneidade clínica. Ocorre uma absorção descontrolada do ferro e risco de sobrecarga a nível do fígado, coração, pâncreas, articulações e órgãos endócrinos. Os doentes estão em risco de evolução para cirrose e carcinoma hepático. Deve ser suspeitada na presença de sintomas, história familiar ou elevação dos marcadores séricos do ferro, em especial da saturação da transferrina. O diagnóstico é desafiante pela inespecificidade de manifestações clínicas e dos marcadores séricos do ferro. Há vários genes implicados, mas o HFE é o mais frequentemente afetado; a homozigotia C282Y associada a sobrecarga de ferro faz diagnóstico; outras variantes genéticas podem necessitar de estudo complementar com ressonância magnética ou biópsia hepática. O tratamento preferencial é a flebotomia, ou, em alternativa, eritrocitaferese ou quelação do ferro. Apresentam elevada morbimortalidade que pode ser reduzida com o início atempado do tratamento.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Hemochromatosis is a hereditary disease of iron metabolism resulting from genetic defects in the hepcidin-ferroportin axis, exhibiting variable penetrance and, therefore, clinical heterogeneity. This condition leads to uncontrolled iron absorption, posing a risk of overload in vital organs such as the liver, heart, pancreas, joints, and endocrine organs. Patients face an increased risk of cirrhosis and hepatocellular carcinoma. Suspect hemochromatosis in the presence of symptoms, a family history, or elevated serum iron markers, especially transferrin saturation. Diagnosis is challenging due to the non-specificity of clinical manifestations and serum iron markers. There are several genes implicated, but HFE is the most frequently affected; C282Y homozygosity associated with iron overload makes the diagnosis; other genetic variants may require additional study with magnetic resonance imaging or liver biopsy The preferred treatment includes phlebotomy; alternatively, erythrocytapheresis or iron chelation can be considered. Early initiation of treatment is crucial to reduce the high morbidity and mortality associated with the disease.]]></p></abstract>
<kwd-group>
<kwd lng="pt"><![CDATA[Hemocromatose/classificação]]></kwd>
<kwd lng="pt"><![CDATA[Hemocromatose/diagnóstico]]></kwd>
<kwd lng="pt"><![CDATA[Hemocromatose/epidemiologia]]></kwd>
<kwd lng="pt"><![CDATA[Hemocromatose/fisiopatologia]]></kwd>
<kwd lng="pt"><![CDATA[Hemocromatose/terapia.]]></kwd>
<kwd lng="en"><![CDATA[Hemochromatosis/classification]]></kwd>
<kwd lng="en"><![CDATA[Hemochromatosis/diagnosis]]></kwd>
<kwd lng="en"><![CDATA[Hemochromatosis/epidemiology]]></kwd>
<kwd lng="en"><![CDATA[Hemochromatosis/physiopathology]]></kwd>
<kwd lng="en"><![CDATA[Hemochromatosis/therapy.]]></kwd>
</kwd-group>
</article-meta>
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