<?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>0871-9721</journal-id>
<journal-title><![CDATA[Revista Portuguesa de Imunoalergologia]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Port Imunoalergologia]]></abbrev-journal-title>
<issn>0871-9721</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Alergologia e Imunologia Clínica]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0871-97212021000200085</article-id>
<article-id pub-id-type="doi">10.32932/rpia.2021.07.056</article-id>
<title-group>
<article-title xml:lang="pt"><![CDATA[Mastocitose em idade pediátrica: Caracterização de um período de 5 anos num hospital central]]></article-title>
<article-title xml:lang="en"><![CDATA[Mastocytosis during pediatric age: 5-year characterization in a central hospital]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cunha]]></surname>
<given-names><![CDATA[Francisca]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ribeiro]]></surname>
<given-names><![CDATA[Carmelita]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ramos]]></surname>
<given-names><![CDATA[Leonor]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Carvalho]]></surname>
<given-names><![CDATA[Jóni]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Todo Bom]]></surname>
<given-names><![CDATA[Ana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Centro Hospitalar e Universitário de Coimbra Serviço de Imunoalergologia ]]></institution>
<addr-line><![CDATA[Coimbra ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Centro Hospitalar e Universitário de Coimbra Hospital Pediátrico Serviço de Dermatologia]]></institution>
<addr-line><![CDATA[Coimbra ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>30</day>
<month>06</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>30</day>
<month>06</month>
<year>2021</year>
</pub-date>
<volume>29</volume>
<numero>2</numero>
<fpage>85</fpage>
<lpage>93</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S0871-97212021000200085&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S0871-97212021000200085&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S0871-97212021000200085&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="pt"><p><![CDATA[RESUMO  Fundamentos: A mastocitose é um grupo de doenças raras caracterizada pela expansão e acumulação excessiva de mastócitos clonais, de que resulta o envolvimento cutâneo e/ou sistémico. Nas idades pediátricas, a forma mais comum de mastocitose é a mastocitose cutânea.  Objetivos: O presente trabalho tem como objetivo avaliar as características demográficas e os achados clínicos em crianças com diagnóstico de mastocitose.  Métodos: Foram revistos os processos clínicos, desde janeiro de 2014 a junho de 2019, de todos os doentes com diagnóstico de mastocitose no Hospital Pediátrico de Coimbra.  Resultados: Os 14 doentes foram diagnosticados com mastocitose cutânea. A idade média de início das lesões foi de 15,1 meses, sendo a mediana de 5 meses. A idade média de diagnóstico foi de 22,5 meses e a mediana de 9,5 meses. Dos 14 doentes, 8 eram do sexo masculino. 12 doentes tinham mastocitose cutânea maculopapular e 2 tinham mastocitoma cutâneo. No que diz respeito à subclassificação das variantes da MC maculopapular, 11 foram classificadas como polimórficas e 1 como monomórfica. O sinal de Darier foi positivo em 12 doentes, 10 tinham prurido, 2 apresentavam dor abdominal e 3 diarreia. Não foi constatado nenhum episódio de anafilaxia. Em todos os doentes foi realizado um estudo analítico inicial, a destacar o doseamento da triptase sérica, que variou entre 1,3&#8209;15,9 ng/mL na primeira avaliação. Em 8 doentes, o diagnóstico foi confirmado através de biópsia cutânea. A terapêutica baseou&#8209;se na evicção dos fatores desencadeantes, em anti&#8209;histaminicos H1 e corticosteroide tópico de potência moderada a forte. O curso das lesões permanece estável em 12 doentes.  Conclusões: O presente trabalho corrobora o que está descrito na literatura corrente sobre o tema. Realça a importância do conhecimento das características da doença em idade pediátrica para a prevenção de fatores desencadeantes, diagnóstico, investigação e tratamento dirigidos.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Background: Mastocytosis is a group of rare diseases, characterized by the excessive clonal mast cell proliferation and accumulation, having cutaneous and/or systemic involvement. During pediatric age, cutaneous mastocytosis is the most common presentation.  Aim: This descriptive analysis of children diagnosed with mastocytosis has the purpose of evaluating their demographic characteristics and clinical findings.  Methods: The clinical files of patients diagnosed with mastocytosis from January 2014 to June 2019 at Hospital Pediátrico de Coimbra were reviewed.  Results: Cutaneous mastocytosis was the final diagnosis for all 14 patients. The mean age for the starting lesions was 15.1 months, with a median of 5 months. The mean age for diagnosis was 22.5 months and the median 9.5 months. 8 patients were male, 12 had maculopapular cutaneous mastocytosis and 2 had mastocytoma. Regarding the subclassification of maculopapular cutaneous mastocytosis, 11 were classified as polymorphic and 1 as monomorphic. Darier&#8217;s sign was positive in 12 patients, 10 had pruritus, 2 had abdominal pain and 3 diarrhea. None of the patients had an anaphylactic episode. Every patient had an initial evaluation with tryptase dosage measurement, varying between 1.3&#8209;15.9 ng/mL at this point. 8 patients had the diagnosis confirmed through a skin biopsy. As medical therapy was included avoidance of triggers, H1 antihistamine and moderate to strong strength topical corticosteroid. The lesions progression remains stable in 12 patients.  Conclusions: This analysis reaffirms what is written about the theme in current literature. It emphasizes the importance of knowing the disease&#8217;s pediatric characteristics to ensure the prevention of triggers and an accurate diagnosis, investigation and treatment.]]></p></abstract>
<kwd-group>
<kwd lng="pt"><![CDATA[Mastocitose]]></kwd>
<kwd lng="pt"><![CDATA[mastocitose cutânea]]></kwd>
<kwd lng="pt"><![CDATA[mastocitose pediátrica.]]></kwd>
<kwd lng="en"><![CDATA[Cutaneous mastocytosis]]></kwd>
<kwd lng="en"><![CDATA[mastocytosis]]></kwd>
<kwd lng="en"><![CDATA[pediatric mastocytosis.]]></kwd>
</kwd-group>
</article-meta>
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