<?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>S1665-11462022000400215</article-id>
<article-id pub-id-type="doi">10.24875/bmhim.21000128</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Fibrosis quística: patogenia bacteriana y moduladores del CFTR (regulador de conductancia transmembranal de la fibrosis quística)]]></article-title>
<article-title xml:lang="en"><![CDATA[Cystic fibrosis: bacterial pathogenesis and CFTR (cystic fibrosis transmembrane conductance regulator) modulators]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vargas-Roldán]]></surname>
<given-names><![CDATA[Silvia Y.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Lezana-Fernández]]></surname>
<given-names><![CDATA[José L.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cerna-Cortés]]></surname>
<given-names><![CDATA[Jorge F.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Partida-Sánchez]]></surname>
<given-names><![CDATA[Santiago]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Santos-Preciado]]></surname>
<given-names><![CDATA[José I.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Rosales-Reyes]]></surname>
<given-names><![CDATA[Roberto]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad Nacional Autónoma de México Facultad de Medicina Unidad de Investigación en Medicina Experimental]]></institution>
<addr-line><![CDATA[Ciudad de México ]]></addr-line>
<country>México</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Instituto Politécnico Nacional Escuela Nacional de Ciencias Biológicas Departamento de Microbiología]]></institution>
<addr-line><![CDATA[Ciudad de México ]]></addr-line>
<country>México</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Hospital Infantil de México Federico Gómez Clínica de Fibrosis Quística ]]></institution>
<addr-line><![CDATA[Ciudad de México ]]></addr-line>
<country>México</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Asociación Mexicana de Fibrosis Quística Dirección Médica ]]></institution>
<addr-line><![CDATA[Ciudad de México ]]></addr-line>
<country>México</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,Abigail Wexner Research Institute at Nationwide Children&#8217;s Hospita Center for Microbial Pathogenesis ]]></institution>
<addr-line><![CDATA[Columbus Ohio]]></addr-line>
<country>Estados Unidos de América</country>
</aff>
<aff id="Af6">
<institution><![CDATA[,The Ohio State University College of Medicine Department of Pediatrics]]></institution>
<addr-line><![CDATA[Columbus Ohio]]></addr-line>
<country>Estados Unidos de América</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>08</month>
<year>2022</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>08</month>
<year>2022</year>
</pub-date>
<volume>79</volume>
<numero>4</numero>
<fpage>215</fpage>
<lpage>221</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S1665-11462022000400215&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S1665-11462022000400215&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S1665-11462022000400215&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen La fibrosis quística es una enfermedad hereditaria autosómica recesiva que se origina por mutaciones en el gen regulador de conductancia transmembranal de la fibrosis quística (CFTR, cystic fibrosis transmembrane conductance regulator). El CFTR es una proteína que transporta iones a través de la membrana de las células epiteliales pulmonares. La pérdida de su función conlleva la producción de un moco pegajoso y espeso, donde se pueden establecer y adaptar diversos patógenos bacterianos que contribuyen a la pérdida gradual de la función pulmonar. En este artículo de revisión se dará evidencia de los mecanismos moleculares que utilizan Pseudomonas aeruginosa y Burkholderia cenocepacia para sobrevivir y persistir en el ambiente pulmonar. Adicionalmente, se describirán las nuevas estrategias de terapia a base de moduladores de la función del CFTR.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Cystic fibrosis is an autosomal recessive inherited disease caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). CFTR is a protein that transports ions across the membrane of lung epithelial cells. Loss of its function leads to the production of thick sticky mucus, where various bacterial pathogens can establish and adapt, contributing to the gradual loss of lung function. In this review, evidence of the molecular mechanisms used by Pseudomonas aeruginosa and Burkholderia cenocepacia to survive and persist in the pulmonary environment will be provided. Additionally, new therapeutic strategies based on CFTR function modulators will be described.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Fibrosis quística]]></kwd>
<kwd lng="es"><![CDATA[Pseudomonas aeruginosa]]></kwd>
<kwd lng="es"><![CDATA[Burkholderia cenocepacia]]></kwd>
<kwd lng="es"><![CDATA[Moduladores de CFTR]]></kwd>
<kwd lng="en"><![CDATA[Cystic fibrosis]]></kwd>
<kwd lng="en"><![CDATA[Pseudomonas aeruginosa]]></kwd>
<kwd lng="en"><![CDATA[Burkholderia cenocepacia]]></kwd>
<kwd lng="en"><![CDATA[CFTR modulators]]></kwd>
</kwd-group>
</article-meta>
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