<?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>S2341-45452022000300042</article-id>
<article-id pub-id-type="doi">10.1159/000515469</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Pediatric Acute Liver Failure in Sickle Cell Disease]]></article-title>
<article-title xml:lang="pt"><![CDATA[Falência Hepática Aguda em Idade Pediátrica na Doença de Células Falciformes]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Rodrigues]]></surname>
<given-names><![CDATA[Luís]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Almeida]]></surname>
<given-names><![CDATA[Sofia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Salgado]]></surname>
<given-names><![CDATA[Catarina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gonçalves]]></surname>
<given-names><![CDATA[Cristina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte Department of Pediatrics Pediatric Intensive Care Unit]]></institution>
<addr-line><![CDATA[Lisbon ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital do Espírito Santo de Évora Department of Pediatrics ]]></institution>
<addr-line><![CDATA[Évora ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte Department of Pediatrics Hematology Unit]]></institution>
<addr-line><![CDATA[Lisbon ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte Department of Pediatrics Gastroenterology Unit]]></institution>
<addr-line><![CDATA[Lisbon ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>30</day>
<month>06</month>
<year>2022</year>
</pub-date>
<pub-date pub-type="epub">
<day>30</day>
<month>06</month>
<year>2022</year>
</pub-date>
<volume>29</volume>
<numero>3</numero>
<fpage>42</fpage>
<lpage>46</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S2341-45452022000300042&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S2341-45452022000300042&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S2341-45452022000300042&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract  Introduction:  Sickle cell intrahepatic cholestasis (SCIC) is one of the rarest and the most severe acute hepatic manifestations of sickle cell disease (SCD) and it can rapidly progress to acute liver failure. It is associated with a high mortality rate, demanding prompt recognition and management.  Case Presentation:  We report a case of a 7-year-old boy with a history of homozygous HbS SCD who presented to the emergency department with fever, increasing abdominal pain, and jaundice. His course was complicated by acute liver injury (AST 9,472 IU/L, ALT 2,683 IU/L, total bilirubin 15.4 mg/dL; conjugated bilirubin 8.69 mg/dL, hypoalbuminemia 2.6 g/dL, and persistent hypoglycemia), with acute liver failure (coagulopathy not corrected by vitamin K administration with INR 3.26, decreased factors V 10% and VII 28%, and West Haven grade I hepatic encephalopathy associated with mild hyperammonemia of 71 µmol/L). After excluding other causes of acute liver failure, the patient was diagnosed as having SCIC and was successfully treated with manual exchange transfusion.  Conclusion:  This case reinforces that exchange transfusion is an effective treatment for SCIC and that it should be introduced promptly to prevent fulminant and potentially fatal liver failure.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo  Introdução:  A colestase intra-hepática falciforme (CIHF) é uma das manifestações hepáticas agudas mais raras e graves da doença de células falciformes (DCF), que pode progredir rapidamente para falência hepática aguda. Associa-se a uma elevada taxa de mortalidade, que exige identificação e intervenção imediatas.  Caso clínico:  Descreve-se o caso de um rapaz de 7 anos com DCF, homozigótico para a HbS, que recorreu ao serviço de urgência por febre, dor abdominal de agravamento progressivo e icterícia. Verificou-se evolução para lesão hepática aguda (AST 9,472 UI/L, ALT 2,683 UI/L, bilirrubina total 15.4 mg/dL; bilirrubina conjugada 8.69 mg/dL, hipoalbuminemia 2.6 g/dL, hipoglicemia persistente), com falência hepática aguda (coagulopatia não corrigida por administração de vitamina K com INR 3.26, diminuição dos fatores V 10% e VII 28%, assim como encefalopatia grau I (critérios de West Haven) e hiperamoniemia ligeira 71 µmol/L). Após exclusão de outras etiologias de falência hepática aguda, o doente foi diagnosticado com CIHF e realiza-da exsanguineo-transfusão manual, com sucesso.  Discussão/Conclusão:  Este caso reforça que a exsanguineo-transfusão é uma terapêutica eficaz na CIHF, que deve ser instituída precocemente para prevenir falência hepática fulminante e potencialmente fatal.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Acute liver failure]]></kwd>
<kwd lng="en"><![CDATA[Exchange transfusion]]></kwd>
<kwd lng="en"><![CDATA[Intrahepatic cholestasis]]></kwd>
<kwd lng="en"><![CDATA[Pediatrics]]></kwd>
<kwd lng="en"><![CDATA[Sickle cell disease]]></kwd>
<kwd lng="pt"><![CDATA[Falência hepática aguda]]></kwd>
<kwd lng="pt"><![CDATA[Exsanguineotransfusão]]></kwd>
<kwd lng="pt"><![CDATA[Colestase intrahepática]]></kwd>
<kwd lng="pt"><![CDATA[Pediatria]]></kwd>
<kwd lng="pt"><![CDATA[Doença de células falciformes]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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