<?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>1413-6538</journal-id>
<journal-title><![CDATA[Revista Brasileira de Educação Especial]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. bras. educ. espec.]]></abbrev-journal-title>
<issn>1413-6538</issn>
<publisher>
<publisher-name><![CDATA[Associação Brasileira de Pesquisadores em Educação Especial - ABPEE]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1413-65382012000300003</article-id>
<title-group>
<article-title xml:lang="pt"><![CDATA[Competências escolares e sociais em crianças e adolescentes com Síndrome de Williams]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Hayashiuchi]]></surname>
<given-names><![CDATA[Ana Yaemi]]></given-names>
</name>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Segin]]></surname>
<given-names><![CDATA[Miriam]]></given-names>
</name>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Schwartzman]]></surname>
<given-names><![CDATA[José Salomão]]></given-names>
</name>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Carreiro]]></surname>
<given-names><![CDATA[Luiz Renato Rodrigues]]></given-names>
</name>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Teixeira]]></surname>
<given-names><![CDATA[Maria Cristina Triguero Veloz]]></given-names>
</name>
</contrib>
</contrib-group>
<aff id="A03">
<institution><![CDATA[,Universidade Presbiteriana Mackenzie  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>09</month>
<year>2012</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>09</month>
<year>2012</year>
</pub-date>
<volume>18</volume>
<numero>03</numero>
<fpage>375</fpage>
<lpage>390</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://educa.fcc.org.br/scielo.php?script=sci_arttext&amp;pid=S1413-65382012000300003&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://educa.fcc.org.br/scielo.php?script=sci_abstract&amp;pid=S1413-65382012000300003&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://educa.fcc.org.br/scielo.php?script=sci_pdf&amp;pid=S1413-65382012000300003&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="pt"><p><![CDATA[A Síndrome de Williams (SW) é uma doença genética e neurocomportamental causada por uma deleção hemizigótica de múltiplos genes na região cromossômica 7q11-23. Caracteriza-se por alterações cognitivas e comportamentais que interferem no ajustamento psicossocial. O objetivo do estudo foi verificar indicadores comportamentais de habilidades nas áreas social, escolar e de realização de atividades de um grupo de crianças e adolescentes com SW e sinais de desatenção e hiperatividade. A amostra foi composta por 22 crianças e adolescentes com diagnóstico clínico e genético de SW entre sete e 18 anos, média de idade 11,6 (desvio padrão 3,7) e suas respectivas mães. Os instrumentos de coleta de dados foram a Escala de Inteligência Wechsler para Crianças, o Inventário dos Comportamentos de Crianças e Adolescentes de seis a 18 anos e um Questionário que avaliou presença de sinais de desatenção e hiperatividade baseado nos critérios clínicos para Transtorno de Déficit de Atenção e Hiperatividade do Manual de Classificação Estatística dos Transtornos Mentais da Associação Americana de Psiquiatria. Os principais resultados apontaram para um elevado número de sinais de desatenção e hiperatividade (90% do total da amostra pontuaram positivamente nesses sinais). Também foram verificados prejuízos graves nas habilidades e competências de desempenho escolar do grupo, diferentemente dos resultados positivos obtidos nas escalas de socialização e prática de esportes. Conclui-se que os resultados positivos encontrados na área social podem agir como fatores protetores para o desenvolvimento de problemas afetivos como isolamento, tristeza, sentimentos de solidão e baixa autoestima.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Williams Syndrome (WS) is a genetic and neurobehavioral disease caused by a hemizygous deletion of multiples genes in the chromosome region 7q11-23. It is characterized by cognitive and behavioral changes that interfere in psychosocial adjustment. The objective of the study was to verify behavioral indicators of competence in social, educational and performance activities in a group of children and adolescents with WS and signs of inattention and hyperactivity. The sample was composed of 22 children and adolescents with WS clinical and genetic diagnoses between 7 and 18 years, average age 11.6 (standard deviation 3.7) and their mothers. The data collection instruments were the Wechsler Intelligence Scale for Children, the Child Behavior Checklist for Children and Adolescents between 6 and 18 years (CBCL/6-18) and a questionnaire that assessed signs of inattention and hyperactivity based on clinical criteria for Attention Deficit Hyperactivity Disorder of the Manual of Classification of Mental Disorders of the American Psychiatric Association. The main results showed a high number of signs of inattention and hyperactivity (90% of the group scored positively). Severe underachievement in academic skills were also observed, as opposed to positive results in socialization and sports scales. In conclusion, positive results in social areas can be inhibiting factors for the development of emotional problems such as withdrawal, loneliness and low self-esteem.]]></p></abstract>
<kwd-group>
<kwd lng="pt"><![CDATA[Educação Especial]]></kwd>
<kwd lng="pt"><![CDATA[Competência Social]]></kwd>
<kwd lng="pt"><![CDATA[Desatenção]]></kwd>
<kwd lng="pt"><![CDATA[Hiperatividade]]></kwd>
<kwd lng="en"><![CDATA[Special Education]]></kwd>
<kwd lng="en"><![CDATA[Social Competence]]></kwd>
<kwd lng="en"><![CDATA[Inattention]]></kwd>
<kwd lng="en"><![CDATA[Hyperactivity]]></kwd>
</kwd-group>
</article-meta>
</front><body><![CDATA[ <font face="Arial, Helvetica, sans-serif"> <h2>Compet&ecirc;ncias escolares e sociais em crian&ccedil;as e  adolescentes com S&iacute;ndrome de Williams.</h2> <h3>School and social  competencies in children and adolescents with Williams Syndrome.</h3>     <p>&nbsp;</p> <h4>Ana Yaemi Hayashiuchi, Miriam Segin, Jos&eacute; Salom&atilde;o  Schwartzman, Luiz Renato Rodrigues Carreiro, Maria Cristina Triguero Veloz  Teixeira    <br> </h4> </font>      ]]></body><back>
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