<?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>0186-4866</journal-id>
<journal-title><![CDATA[Medicina interna de México]]></journal-title>
<abbrev-journal-title><![CDATA[Med. interna Méx.]]></abbrev-journal-title>
<issn>0186-4866</issn>
<publisher>
<publisher-name><![CDATA[Edición y Farmacia S.A. de C.V.]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0186-48662017000500655</article-id>
<article-id pub-id-type="doi">10.24245/mim.v33i5.1405</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Avances en el diagnóstico y tratamiento de la porfiria intermitente aguda]]></article-title>
<article-title xml:lang="en"><![CDATA[Advances in diagnosis and treatment of acute intermittent porphyria.]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Jaramillo-Calle]]></surname>
<given-names><![CDATA[DA.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Zapata-Cárdenas]]></surname>
<given-names><![CDATA[A.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad de Antioquia  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Colombia</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>10</month>
<year>2017</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>10</month>
<year>2017</year>
</pub-date>
<volume>33</volume>
<numero>5</numero>
<fpage>655</fpage>
<lpage>667</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S0186-48662017000500655&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S0186-48662017000500655&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S0186-48662017000500655&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen: La porfiria intermitente aguda es la más frecuente de las porfirias hepáticas agudas. Ésta es causada por mutaciones de herencia autosómica dominante en el gen HMBS, que generan déficit de la enzima porfobilinógeno desaminasa en la vía biosintética del grupo hemo. Se caracteriza por síntomas neuroviscerales potencialmente letales, que incluyen dolor abdominal, vómito, estreñimiento, debilidad muscular, convulsiones. Estos ataques son desencadenados por medicamentos, ayuno, alcohol, cambios hormonales, estrés, entre otros. Su tratamiento incluye altas cargas de carbohidratos, hemina intravenosa y soporte médico. Algunos pacientes que padecen ataques agudos recurrentes pueden requerir hemina profiláctica o análogos de la hormona liberadora de gonadotropina. El trasplante hepático está indicado para los pacientes resistentes a esos tratamientos. Las complicaciones crónicas de la enfermedad incluyen carcinoma hepatocelular, hipertensión arterial, enfermedad renal crónica y dolor crónico.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract: Acute intermittent porphyria is the most frequent of the acute hepatic porphyrias. This is caused by mutations of autosomal dominant inheritance in the HMBS gene, which generate a deficiency of the enzyme porphobilinogen deaminase in the biosynthetic pathway of the heme group. It is characterized by potentially life-threatening neurovisceral symptoms, including abdominal pain, vomiting, constipation, muscle weakness and seizures. Such attacks are triggered by medications, fasting, alcohol, hormonal changes, stress, among others. Its treatment includes high carbohydrate loads, intravenous hemin, and medical support. Some patients who develop recurrent acute attacks may require prophylactic hemin or gonadotropin-releasing hormone analogs. Liver transplantation is indicated for patients refractory to those treatments. Chronic complications of the disease include hepatocellular carcinoma, hypertension, chronic kidney disease, and chronic pain.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[porfiria intermitente aguda]]></kwd>
<kwd lng="es"><![CDATA[diagnóstico]]></kwd>
<kwd lng="es"><![CDATA[tratamiento]]></kwd>
<kwd lng="es"><![CDATA[prevención]]></kwd>
<kwd lng="es"><![CDATA[síntomas neurológicos]]></kwd>
<kwd lng="es"><![CDATA[crisis]]></kwd>
<kwd lng="en"><![CDATA[acute intermittent porphyria]]></kwd>
<kwd lng="en"><![CDATA[diagnosis]]></kwd>
<kwd lng="en"><![CDATA[treatment]]></kwd>
<kwd lng="en"><![CDATA[prevention]]></kwd>
<kwd lng="en"><![CDATA[neurogical symptoms]]></kwd>
<kwd lng="en"><![CDATA[crisis]]></kwd>
</kwd-group>
</article-meta>
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