<?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>2341-4545</journal-id>
<journal-title><![CDATA[GE-Portuguese Journal of Gastroenterology]]></journal-title>
<abbrev-journal-title><![CDATA[GE Port J Gastroenterol]]></abbrev-journal-title>
<issn>2341-4545</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Gastrenterologia]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2341-45452024000400049</article-id>
<article-id pub-id-type="doi">10.1159/000533179</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Acute Abdominal Pain as the Initial Presentation of an Acquired C1 Inhibitor De&#64257;ciency]]></article-title>
<article-title xml:lang="pt"><![CDATA[Abdómen agudo como apresentação inicial de de&#64257;ciência adquirida de C1 inibidor]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pinto]]></surname>
<given-names><![CDATA[Ana Raquel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Carolino]]></surname>
<given-names><![CDATA[Fabrícia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Centro Hospitalar Universitário do Porto Allergy and Clinical Immunology Department ]]></institution>
<addr-line><![CDATA[Porto ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>08</month>
<year>2024</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>08</month>
<year>2024</year>
</pub-date>
<volume>31</volume>
<numero>4</numero>
<fpage>49</fpage>
<lpage>53</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S2341-45452024000400049&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S2341-45452024000400049&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S2341-45452024000400049&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract  Introduction:  Acquired angioedema (AAE), a rare cause of adult-onset non-urticarial mucocutaneous angioedema, can present as acute abdomen, a frequent complaint in the emergency room (ER), often leading to unnecessary and potentially harmful procedures.  Case Presentation:  We report a 47-year-old hypertense male, controlled with an angiotensin converting enzyme inhibitor (ACEI), who presented in the ER with progressively worsening abdominal pain, nausea, and vomiting, and a radiologic workup revealing small intestine thickening, initially diagnosed with ACEI-induced angioedema. However, further investigation revealed low serum levels of C4,C1q, and C1 inhibitors, with an abnormal function of the latter, favoring the diagnosis of AAE instead. The frequent association of this condition with lymphoproliferative disorders encouraged further studies, which unveiled a monoclonal gammopathy IgM/Kappa, representing an increased risk of Waldenström macroglobulinemia, non-Hodgkin lymphoma, and multiple myeloma.  Discussion:  AAE should be regarded as an important differential diagnosis in patients presenting with acute abdomen in the ER, especially when more common causes are excluded. A correct and early diagnosis may represent a chance for a better prognosis of underlying diseases.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo  Introdução:  O angioedema adquirido (AA), causa rara de angioedema mucocutâneo não urticariforme de início tardio, pode ter como apresentação inicial abdómen agudo, motivo frequente de admissão no serviço de urgência (SU), promovendo frequentemente procedimentos desnecessários e potencialmente prejudiciais.  Apresentação do caso:  Um homem de 47 anos, hipertenso e controlado com um inibidor da enzima conversora de angiotensina (IECA), recorreu ao SU por um quadro de dor abdominal com agravamento progressivo, náuseas e vómitos. A investigação radiológica inicial revelou espessamento do intestino delgado, culminando num diagnóstico preliminar de angioedema induzido por IECA. No entanto, uma investigação mais aprofundada em regime ambulatório revelou níveis séricos reduzidos de C4, C1q e de inibidor de C1, com função anormal deste último, favorecendo o diagnóstico de AA. A associação frequente desta condição com distúrbios linfoprolifer-ativos incentivou investigação adicional, que revelou uma gamopatia monoclonal IgM/Kappa, representando um risco aumentado de macroglobulinemia de Waldenström, linfoma não-Hodgkin e mieloma múltiplo.  Discussão:  O AA deve ser considerado um diagnóstico diferencial de abdómen agudo, principalmente após exclusão de causas mais frequentes. Um diagnóstico precoce pode contribuir para um melhor prognóstico da patologia subjacente.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Abdominal pain]]></kwd>
<kwd lng="en"><![CDATA[Angioedema]]></kwd>
<kwd lng="en"><![CDATA[C1 esterase inhibitor]]></kwd>
<kwd lng="en"><![CDATA[Lymphoproliferative disorders]]></kwd>
<kwd lng="en"><![CDATA[Monoclonal gammopathy]]></kwd>
<kwd lng="pt"><![CDATA[Dor abdominal]]></kwd>
<kwd lng="pt"><![CDATA[Angioedema]]></kwd>
<kwd lng="pt"><![CDATA[Inibidor da C1 esterase]]></kwd>
<kwd lng="pt"><![CDATA[Distúrbios linfoproliferativos]]></kwd>
<kwd lng="pt"><![CDATA[Gamopatia monoclonal]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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