<?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>2183-1351</journal-id>
<journal-title><![CDATA[Acta Radiológica Portuguesa]]></journal-title>
<abbrev-journal-title><![CDATA[Acta Radiol Port]]></abbrev-journal-title>
<issn>2183-1351</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Radiologia e Medicina Nuclear]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2183-13512025000200027</article-id>
<article-id pub-id-type="doi">10.25748/arp.39604</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Ischemic Cholangiopathy: A Rare Manifestation in Hereditary Hemorrhagic Telangiectasia]]></article-title>
<article-title xml:lang="pt"><![CDATA[Colangiopatia Isquémica: Uma Manifestação Rara da Telangiectasia Hemorrágica Hereditária]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Silva]]></surname>
<given-names><![CDATA[Maria J.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ferreira]]></surname>
<given-names><![CDATA[José M.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Torres]]></surname>
<given-names><![CDATA[Tiago P.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Simões]]></surname>
<given-names><![CDATA[Ana M.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Mendes]]></surname>
<given-names><![CDATA[Hugo T.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Oliveira]]></surname>
<given-names><![CDATA[Pedro G.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Unidade Local de Saúde de Trás-os-Montes e Alto Douro Serviço de Imagiologia ]]></institution>
<addr-line><![CDATA[Vila Real ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>08</month>
<year>2025</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>08</month>
<year>2025</year>
</pub-date>
<volume>37</volume>
<numero>2</numero>
<fpage>27</fpage>
<lpage>30</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S2183-13512025000200027&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S2183-13512025000200027&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S2183-13512025000200027&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disorder characterized by skin and mucosal telangiectasias and visceral arteriovenous malformations (AVMs). We report a case of ischemic cholangiopathy in a 60-year-old female with HHT who initially presented with intermittent biliary colic and no significant medical history. The absence of typical HHT symptoms made imaging crucial for diagnosis. Abdominal ultrasound, CT, and MRI revealed hepatic artery dilatation, hepatic AVMs and bilomas. In this setting, the bilomas result from biliary ductal necrosis due to arterial shunting induced by AVMs. Although the patient initially presented without common HHT manifestations such as epistaxis, a subsequent reassessment during the physical exam identified mucocutaneous telangiectasias, and genetic testing later confirmed the diagnosis. This case highlights the importance of recognizing specific imaging features to diagnose and understand the implications of ischemic cholangiopathy, a rare but distinct phenotype in patients with HHT.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo A Telangiectasia Hemorrágica Hereditária (THH) é uma doença autossómica dominante caracterizada por telangiectasias mucocutâneas e malformações arteriovenosas (MAV) viscerais. Apresentamos um caso de colangiopatia isquémica numa mulher de 60 anos com THH, com um quadro inicial de cólica biliar intermitente e sem antecedentes pessoais e familiares de relevo. Na ausência de sintomas típicos, os achados imagiológicos foram determinantes para o diagnóstico. A ecografia, TC e RM abdominal demonstraram dilatação da artéria hepática, MAVs hepáticas e bilomas. Neste contexto, os bilomas resultam de necrose dos ductos biliares devido ao shunt arterial induzido pelas MAVs. Embora a doente inicialmente não apresentasse manifestações típicas da THH, como epistaxis, uma reavaliação subsequente durante o exame físico identificou telangiectasias mucocutâneas, sendo o diagnóstico posteriormente confirmado através de testes genéticos. Este caso sublinha a importância das características imagiológicas para diagnosticar e compreender as implicações da colangiopatia isquémica, um fenótipo raro, mas distinto na THH.]]></p></abstract>
<kwd-group>
<kwd lng="pt"><![CDATA[Telangiectasia hemorrágica hereditária]]></kwd>
<kwd lng="pt"><![CDATA[Osler-Weber-Rendu]]></kwd>
<kwd lng="pt"><![CDATA[Fígado]]></kwd>
<kwd lng="pt"><![CDATA[Malformações arteriovenosas]]></kwd>
<kwd lng="pt"><![CDATA[Colangiopatia isquémica]]></kwd>
<kwd lng="pt"><![CDATA[biloma.]]></kwd>
<kwd lng="en"><![CDATA[Hereditary hemorrhagic telangiectasia]]></kwd>
<kwd lng="en"><![CDATA[Osler-Weber-Rendu]]></kwd>
<kwd lng="en"><![CDATA[Liver]]></kwd>
<kwd lng="en"><![CDATA[Arteriovenous malformations]]></kwd>
<kwd lng="en"><![CDATA[Ischemic cholangiopathy]]></kwd>
<kwd lng="en"><![CDATA[Biloma.]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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