<?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>0872-8178</journal-id>
<journal-title><![CDATA[Jornal Português de Gastrenterologia ]]></journal-title>
<abbrev-journal-title><![CDATA[J Port Gastrenterol.]]></abbrev-journal-title>
<issn>0872-8178</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Gastrenterologia]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0872-81782007000500003</article-id>
<title-group>
<article-title xml:lang="pt"><![CDATA[Colangite Esclerosante Primária: Uma Forma de Apresentação Potencialmente Fatal]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Bispo]]></surname>
<given-names><![CDATA[M.]]></given-names>
</name>
<xref ref-type="aff" rid="A01"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pina]]></surname>
<given-names><![CDATA[P. R.]]></given-names>
</name>
<xref ref-type="aff" rid="A02"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Sousa]]></surname>
<given-names><![CDATA[S.]]></given-names>
</name>
<xref ref-type="aff" rid="A02"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Seves]]></surname>
<given-names><![CDATA[I.]]></given-names>
</name>
<xref ref-type="aff" rid="A01"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Moura]]></surname>
<given-names><![CDATA[M. L. C.]]></given-names>
</name>
<xref ref-type="aff" rid="A03"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Graça]]></surname>
<given-names><![CDATA[J. P.]]></given-names>
</name>
<xref ref-type="aff" rid="A02"/>
</contrib>
</contrib-group>
<aff id="A01">
<institution><![CDATA[,E.P.E. - Hospital de Egas Moniz Centro Hospitalar de Lisboa Ocidental Serviço de Gastrenterologia]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="A02">
<institution><![CDATA[,E.P.E. - Hospital de Egas Moniz Centro Hospitalar de Lisboa Ocidental Serviço de Medicina II]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="A03">
<institution><![CDATA[,E.P.E. - Hospital de Egas Moniz Centro Hospitalar de Lisboa Ocidental Serviço de Anatomia Patológica]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2007</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2007</year>
</pub-date>
<volume>14</volume>
<numero>5</numero>
<fpage>236</fpage>
<lpage>240</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S0872-81782007000500003&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S0872-81782007000500003&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S0872-81782007000500003&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="pt"><p><![CDATA[A colangite esclerosante primária é uma doença idiopática, caracterizada por inflamação, esclerose e obliteração progressiva das vias biliares. É uma doença rara, mais prevalente nos homens e frequentemente associada à colite ulcerosa. A apresentação clínica habitual é um quadro colestático. Apesar da colangite aguda ser uma complicação conhecida, raramente constitui a forma de apresentação desta doença. Os autores descrevem o caso clínico de um homem de 67 anos, internado por sépsis, no contexto duma provável colangite aguda. São integrados os dados clínicos, laboratoriais, imagiológicos e histológicos que conduziram ao diagnóstico de colangite esclerosante primária. Salienta-se o achado laboratorial duma elevação significativa do CA19.9 associada à colangite. São ainda revistos alguns aspectos a ter em conta no prognóstico e seguimento desta doença.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Primary sclerosing cholangitis is an idiopathic disease, characterized by inflammation, progressive sclerosis and obliteration of bile ducts. It is a rare disease, with higher prevalence in males, often associated with ulcerative colitis. The most frequent clinical presentation is a cholestatic picture. Although acute cholangitis is a known complication, it seldom occurs as a form of presentation. The authors describe a case report of a 67 year-old male, who was admitted with sepsis, in a context of probable acute cholangitis. The clinical, laboratory, imaging and histologic data which lead to primary sclerosing cholangitis diagnosis are analyzed. The laboratory finding of markedly elevated CA19.9 associated with cholangitis is emphasized. Some features on the prognosis and follow-up of this disease are reviewed.]]></p></abstract>
</article-meta>
</front><body><![CDATA[ <p><b>Colangite Esclerosante Prim&aacute;ria: Uma Forma de Apresenta&ccedil;&atilde;o    Potencialmente Fatal</b></p>     <p></p>     <p>M. Bispo <sup><a href="#1">1</a><a name="top1"></a></sup>, P. R. Pina <sup><a href="#2">2</a><a name="top2"></a></sup>,    S. Sousa <sup><a href="#2">2</a><a name="top2"></a></sup>, I. Seves <sup><a href="#1">1</a></sup><a name="top1"></a>,    M. L. C. Moura <sup><a href="#3">3</a><a name="top3"></a></sup>, J. P. Gra&ccedil;a    <sup><a href="#2">2</a></sup><a name="top2"></a> </p>     <p>&nbsp;</p>     <p>&nbsp;</p>      <p><b>Resumo</b></p>      <p>A colangite esclerosante primária é uma doença idiopática, caracterizada por    inflamação, esclerose e obliteração progressiva das vias biliares. É uma doença    rara, mais prevalente nos homens e frequentemente associada à colite ulcerosa.</p>      <p>A apresentação clínica habitual é um quadro colestático. Apesar da colangite aguda ser uma complicação conhecida, raramente constitui a forma de apresentação desta doença.</p>      <p>Os autores descrevem o caso clínico de um homem de 67 anos, internado por sépsis, no contexto duma provável colangite aguda. São integrados os dados clínicos, laboratoriais, imagiológicos e histológicos que conduziram ao diagnóstico de colangite esclerosante primária. Salienta-se o achado laboratorial duma elevação significativa do CA19.9 associada à colangite. São ainda revistos alguns aspectos a ter em conta no prognóstico e seguimento desta doença.</p>      <p>&nbsp;</p>     ]]></body>
<body><![CDATA[<p>&nbsp;</p>      <p><b>Summary</b></p>      <p>Primary sclerosing cholangitis is an idiopathic disease, characterized by inflammation,    progressive sclerosis and obliteration of bile ducts. It is a rare disease,    with higher prevalence in males, often associated with ulcerative colitis. The    most frequent clinical presentation is a cholestatic picture.</p>      <p>Although acute cholangitis is a known complication, it seldom occurs as a form of presentation. </p>      <p>The authors describe a case report of a 67 year-old male, who was admitted with sepsis, in a context of probable acute cholangitis. The clinical, laboratory, imaging and histologic data which lead to primary sclerosing cholangitis diagnosis are analyzed. The laboratory finding of markedly elevated CA19.9 associated with cholangitis is emphasized. Some features on the prognosis and follow-up of this disease are reviewed.</p>      <p>&nbsp;</p>     <p>&nbsp;</p>      <p>Texto Completo disponível apenas em PDF</p>      <p>Full text only available in PDF format</p>      <p>&nbsp;</p>      ]]></body>
<body><![CDATA[<p>&nbsp;</p>      <p><b>Bibliografia</b></p>      <p>&nbsp;</p>      <!-- ref --><p>1. Lee YM, Kaplan MM. Primary sclerosing cholangitis. N Engl J Med 1995; 332: 924-933.&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;[&#160;<a href="javascript:void(0);" onclick="javascript: window.open('/scielo.php?script=sci_nlinks&ref=000026&pid=S0872-8178200700050000300001&lng=','','width=640,height=500,resizable=yes,scrollbars=1,menubar=yes,');">Links</a>&#160;]<!-- end-ref --><p>2. Tung BY, Kowdley KV. Sclerosing cholangitis and recurrent pyogenic cholangitis. In: Sleisenger and Fordtran&#8217;s Gastrointestinal and Liver Disease, 8th edition, 2006; vol. 1; 65:1461-1485.</p>      <p>3. Kaplan MM. Toward better treatment of primary sclerosing cholangitis. N Engl J Med 1997; 336: 719-721.</p>      <p>4. Macfaul GR, Chapman RW. Sclerosing Cholangitis. Curr Opin Gastroenterol 2006; 22(3): 288-93.</p>      <p>5. Lindor KD, for The Mayo Primary Sclerosing Cholangitis-Ursodeoxycholic Acid Study Group. Ursodiol for primary sclerosing cholangitis. N Engl J Med 1997; 336: 691-695.</p>      <p>6. Karani J. The biliary tract. In: Sutton D. Textbook of radiology and imaging. London: Churchill Livingstone 2003; vol. 1; p. 716.</p>      <p>7. Warren KW, Athanassiades S, Monge JI: Primary sclerosing cholangitis: A study of forty-two cases. Am J Surg 1966; 111:23.</p>      ]]></body>
<body><![CDATA[<p>8. Kaw M, Silverman WB, Rabinovitz M, et al: Biliary tract calculi in primary sclerosing cholangitis. Am J Gastroenterol 1995; 90:72.</p>      <p>9. Kaplan MM. Medical approaches to primary sclerosing cholangitis. Semin Liver Dis 1991; 11: 56-63.</p>      <p>10. Goetz M, Steen PD. False elevation of CA19.9 levels in a patient with a history of pancreatic cancer. Am J Gastroenterol 1997; 92(8):1390-1.</p>      <p>11. Berstad AE, Aabakken L, Smith HJ, Aasen S, Boberg KM, Schrumpf E. Diagnostic accuracy of magnetic resonance and endoscopic retrograde cholangiography in primary sclerosing cholangitis. Clin Gastroenterol Hepatol 2006; 4(4): 514-20.</p>      <p>12. Mitchell SA, Bansi DS, Hunt N, et al. A preliminary trial of highdose ursodeoxycholic acid in primary sclerosing cholangitis. Gastroenterology 2001; 121:900.</p>      <p>13. Harnois DM, Angulo P, Jorgensen RA, et al. High-dose ursodeoxycholic acid as a therapy for patients with primary sclerosing cholangitis. Am J Gastroenterol 2001; 96:1558.</p>      <p>14. Orellana I,Valera JM, Nei C, Poniachik J, Berger Z, Latorre R, et al. Primary sclerosing cholangitis: a twelve-year experience. Rev Med Chil 2005; 133(7): 776-80.</p>      <p>15. Tung, BY, Brentnall, T, Kowdley, KV, et al. Diagnosis and prevalence of    ulcerative colitis in patients with sclerosing cholangitis. Hepatology 1996;    24:169A.</p>     <p>&nbsp;</p>     <p>&nbsp;</p>     ]]></body>
<body><![CDATA[<p>(<a name="1"></a><a href="#top1">1</a>) Serviço de Gastrenterologia.</p>     <p>(<a name="2"></a><a href="#top2">2</a>) Serviço de Medicina II.</p>     <p>(<a name="3"></a><a href="#top3">3</a>) Serviço de Anatomia Patológica.</p>     <p>Centro Hospitalar de Lisboa Ocidental, E.P.E. &#8211; Hospital de Egas Moniz,</p>     <p>Lisboa, Portugal.</p>     <p>&nbsp;</p>     <p><b>Correspondência</b>:</p>     <p>Miguel Bispo</p>     <p>Serviço de Gastrenterologia</p>     <p>Centro Hospitalar de Lisboa Ocidental, E.P.E. &#8211; Hospital</p>     ]]></body>
<body><![CDATA[<p>de Egas Moniz</p>     <p>Rua da Junqueira, 126</p>     <p>1349-019 Lisboa</p>     <p>Tel.: 919 002 599</p>     <p>e-mail: <a href="mailto:bispomiguel@hotmail.com">bispomiguel@hotmail.com</a></p>      <p align="right"><i>Recebido para publicação: 00/00/2007</i></p>     <p align="right"><i>Aceite para publicação: 00/00/2007</i></p>         ]]></body><back>
<ref-list>
<ref id="B1">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lee]]></surname>
<given-names><![CDATA[YM]]></given-names>
</name>
<name>
<surname><![CDATA[Kaplan]]></surname>
<given-names><![CDATA[MM.]]></given-names>
</name>
</person-group>
<article-title xml:lang="en"><![CDATA[Primary sclerosing cholangitis.]]></article-title>
<source><![CDATA[N Engl J Med]]></source>
<year>1995</year>
<volume>332</volume>
<page-range>924-933</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
