<?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>1646-2890</journal-id>
<journal-title><![CDATA[Revista Portuguesa de Estomatologia, Medicina Dentária e Cirurgia Maxilofacial]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Port Estomatol Med Dent Cir Maxilofac]]></abbrev-journal-title>
<issn>1646-2890</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Estomatologia e Medicina Dentária]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1646-28902021000100042</article-id>
<article-id pub-id-type="doi">10.24873/j.rpemd.2021.03.826</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Oral findings in Coffin-Siris syndrome: A case report]]></article-title>
<article-title xml:lang="pt"><![CDATA[Abordagem odontológica na síndrome de Coffin Siris: Relato de caso]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Figueira]]></surname>
<given-names><![CDATA[Heliton Siqueira]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Medina]]></surname>
<given-names><![CDATA[Pollyanna Oliveira]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Jesus]]></surname>
<given-names><![CDATA[Giorge Pessoa de]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Hanan]]></surname>
<given-names><![CDATA[Aida Renée Assayag]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Sponchiado Júnior]]></surname>
<given-names><![CDATA[Emílio Carlos]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Hanan]]></surname>
<given-names><![CDATA[Simone Assayag]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Federal University of Amazonas Dental School ]]></institution>
<addr-line><![CDATA[Manaus Amazonas]]></addr-line>
<country>Brazil</country>
</aff>
<pub-date pub-type="pub">
<day>30</day>
<month>03</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>30</day>
<month>03</month>
<year>2021</year>
</pub-date>
<volume>62</volume>
<numero>1</numero>
<fpage>42</fpage>
<lpage>49</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S1646-28902021000100042&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S1646-28902021000100042&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S1646-28902021000100042&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract This paper reports the oral health approach of a child with Coffin-Siris syndrome. This syndrome is a multisystem congenital anomaly caused by mutations in genes of BRG- 1-associated factors complex. Individuals with this syndrome have been described with hypoplasia or aplasia of the fifth digit nails or phalanges. Other features include growth deficiency, developmental and intellectual delay, and other organ-system abnormalities. Clinical examination revealed gingival hyperplasia in the upper arch, dental biofilm, and dental caries on the lower deciduous and permanent molars. Guidelines on oral hygiene and dietary habits were provided to the guardians, and tooth extraction and restoration with glass-ionomer cement were performed. This case suggests that these patients require home care and periodic dental consultations for preventive and systematic dental treatment and quality of life improvement. The patient is being monitored, and her oral condition has improved.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo Este artigo relata a abordagem da saúde oral de uma criança com a síndrome de Coffin Siris. Esta síndrome é uma anomalia congénita multi-sistémica causada por mutações em genes do complexo BRG-1. Os indivíduos com esta síndrome apresentam hipo ou aplasia das unhas ou das falanges do quinto dedo. Outras características incluem deficiência de crescimento, atraso de desenvolvimento intelectual e anormalidades do sistema de órgãos. O exame clínico revelou hiperplasia gengival na arcada superior, presença de biofilme e cárie dentária nos molares decíduos e permanentes inferiores. Orientações sobre higiene oral e hábitos alimentares foram fornecidas aos responsáveis, além da extração e restauração dos dentes com cimento de ionómero de vidro. Este caso sugere que esses pacientes necessitam de atendimento domiciliar e consultas odontológicas periódicas para tratamento preventivo e sistemático para que se alcance a melhoria da qualidade de vida. A paciente está sendo monitorizada e sua condição oral melhorou.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Case report]]></kwd>
<kwd lng="en"><![CDATA[Coffin-Siris syndrome]]></kwd>
<kwd lng="en"><![CDATA[Dental care for children]]></kwd>
<kwd lng="en"><![CDATA[Fifth-digit syndrome]]></kwd>
<kwd lng="pt"><![CDATA[Relato de caso]]></kwd>
<kwd lng="pt"><![CDATA[Síndrome de Coffin Siris]]></kwd>
<kwd lng="pt"><![CDATA[Tratamento odontológico de crianças]]></kwd>
<kwd lng="pt"><![CDATA[Síndrome do quinto dígito]]></kwd>
</kwd-group>
</article-meta>
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