<?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>1665-1146</journal-id>
<journal-title><![CDATA[Boletín médico del Hospital Infantil de México]]></journal-title>
<abbrev-journal-title><![CDATA[Bol. Med. Hosp. Infant. Mex.]]></abbrev-journal-title>
<issn>1665-1146</issn>
<publisher>
<publisher-name><![CDATA[Instituto Nacional de Salud, Hospital Infantil de México Federico Gómez]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1665-11462016000400256</article-id>
<article-id pub-id-type="doi">10.1016/j.bmhimx.2016.04.002</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Disostosis espondilocostal y colangitis aguda en urgencias pediátricas]]></article-title>
<article-title xml:lang="en"><![CDATA[Spondylocostal dysostosis and acute cholangitis in pediatrics emergency room]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Avilés-Martínez]]></surname>
<given-names><![CDATA[Karla Isis]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="A01">
<institution><![CDATA[,Hospital Civil Fray Antonio Alcalde Urgencias Pediatría ]]></institution>
<addr-line><![CDATA[Guadalajara Jalisco]]></addr-line>
<country>México</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>08</month>
<year>2016</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>08</month>
<year>2016</year>
</pub-date>
<volume>73</volume>
<numero>4</numero>
<fpage>256</fpage>
<lpage>267</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S1665-11462016000400256&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S1665-11462016000400256&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S1665-11462016000400256&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen:  Introducción: Las malformaciones congénitas vertebrales y costales concomitantes comprenden un grupo heterogéneo de enfermedades denominadas disostosis espondilocostal. Tienen en común la alteración del desarrollo o morfología de las estructuras vertebrales y de la caja torácica con una expresividad variable: desde la deformidad leve sin consecuencias funcionales hasta lesiones que amenazan la vida. Se presenta el caso de una niña con disostosis espondilocostal y colangitis aguda.  Caso clínico: Paciente de sexo femenino de 13 meses de edad con desnutrición severa y antecedente de hidrocefalia y mielomeningocele quien ingresa al servicio de Urgencias por presentar dificultad respiratoria progresiva y fiebre. En la evaluación se encontraron malformaciones costovertebrales y colangitis aguda.  Conclusiones: Las anormalidades costales complejas consisten en malformaciones de la pared torácica sin un patrón determinado y son extremadamente raras. Cuando se presentan al mismo tiempo que las malformaciones vertebrales, puede considerarse como síndrome de disostosis espondilocostal ligado a herencia autosómica recesiva. El diagnóstico es clínico-radiográfico. La identificación de la disostosis espondilocostal y las complicaciones relacionadas con sus causas genético-moleculares implican un reto para el pediatra y el equipo multidisciplinario que los trata a lo largo de su vida.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract:  Background: Congenital malformations of the chest wall comprise a heterogeneous group of diseases denominated spondylocostal dysostosis. They have in common developmental abnormalities in the morphology of the structures of the chest and vertebrae with a broad characterization: from mild deformity without functional consequences to life-threatening injuries. We present the case of a girl with spondylocostal dysostosis and acute cholangitis.  Clinical case: A 13-month-old girl with severe malnutrition, history of hydrocephalus and myelomeningocele at birth was admitted in the emergency pediatric room with fever and progressive respiratory distress. Clinical assessment revealed ribs and vertebral malformations and acute cholangitis.  Conclusions: Complex rib abnormalities consist in deformities of the chest wall, which do not have a specific pattern and are extremely rare. When they are associated with myelomeningocele and hydrocephalus they may be considered as autosomal recessive inheritance spondylocostal dysostosis. The diagnosis is established by clinical assessment and X-rays. Spondylocostal dysostosis identification and complications related to their genetic and molecular causes are still a challenge for clinical pediatricians and the multidisciplinary medical team who treats these patients throughout lifetime.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Malformación costal]]></kwd>
<kwd lng="es"><![CDATA[Disostosis espondilocostal]]></kwd>
<kwd lng="es"><![CDATA[Defectos del desarrollo]]></kwd>
<kwd lng="es"><![CDATA[Mielomeningocele]]></kwd>
<kwd lng="es"><![CDATA[Hidrocefalia]]></kwd>
<kwd lng="es"><![CDATA[Colangitis]]></kwd>
<kwd lng="en"><![CDATA[Costal malformation]]></kwd>
<kwd lng="en"><![CDATA[Spondylocostal dysostosis]]></kwd>
<kwd lng="en"><![CDATA[Developmental defects]]></kwd>
<kwd lng="en"><![CDATA[Myelomeningocele]]></kwd>
<kwd lng="en"><![CDATA[Hydrocephalus]]></kwd>
<kwd lng="en"><![CDATA[Acute cholangitis]]></kwd>
</kwd-group>
</article-meta>
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