<?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>0034-7531</journal-id>
<journal-title><![CDATA[Revista Cubana de Pediatría]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Cubana Pediatr]]></abbrev-journal-title>
<issn>0034-7531</issn>
<publisher>
<publisher-name><![CDATA[Centro Nacional de Información de Ciencias MédicasEditorial Ciencias Médicas]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0034-75312019000300005</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Fibrosis quística en niños y su seguimiento durante 40 años 1977-2017)]]></article-title>
<article-title xml:lang="en"><![CDATA[Cystic Fibrosis in children and its follow up during 40 years (1977-2017)]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Sánchez Infante]]></surname>
<given-names><![CDATA[Concepción]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Razón Behar]]></surname>
<given-names><![CDATA[Roberto]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ramos Carpenter]]></surname>
<given-names><![CDATA[Lidia Teresita]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Barreiro Paredes]]></surname>
<given-names><![CDATA[Beatriz]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Reyes López]]></surname>
<given-names><![CDATA[Cristina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cantillo Gámez]]></surname>
<given-names><![CDATA[Haydeé]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cuello Acuña]]></surname>
<given-names><![CDATA[Miyelkis]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital Pediátrico Universitario &#8220;William Soler&#8221;  ]]></institution>
<addr-line><![CDATA[La Habana ]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>09</month>
<year>2019</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>09</month>
<year>2019</year>
</pub-date>
<volume>91</volume>
<numero>3</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S0034-75312019000300005&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S0034-75312019000300005&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S0034-75312019000300005&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  Los enfermos de fibrosis quística tienen mayor supervivencia y calidad de vida en las últimas décadas, debido fundamentalmente a un diagnóstico precoz.  Objetivo:  Describir las características de los pacientes con fibrosis quística y el seguimiento de los vivos durante 40 años.  Métodos:  Estudio descriptivo longitudinal retrospectivo en 96 pacientes diagnosticados con fibrosis quística en el Hospital Pediátrico Universitario &#8220;William Soler&#8221; (1977-2017). Los datos se obtuvieron de las historias clínicas. Se analizaron variables demográficas, temporales, clínicas, genéticas y microbiológicas.  Resultados:  De la muestra, actualmente se mantienen con seguimiento 27, mayores de 19 años enviados a consulta de neumología 9, fallecieron 30, no se siguen en el centro 30. El 60,4 % se diagnosticó antes del primer año de vida, predominó el sexo masculino 62,5 % y el color de la piel blanco 88,5 %. Sugirió el diagnóstico la forma clínica respiratoria 39,6 %, mixta 38,5 %, digestiva 19,8 % y perdedora de sal 2,1 %. De los 27 casos seguidos actualmente 74,1 % son eutróficos y no hay desnutridos. En el primer aislamiento microbiológico predominó la Pseudomona aeruginosa en el menor de 1 año y el Staphilococcus aureus entre 1 y 4 años. Más del 37.0 % tuvo complicaciones respiratorias, el 14,8 % hepática y el 40,7 % sin complicaciones. La caracterización genética fue mayormente &#8710;F508 (70,3 %).  Conclusiones:  Es frecuente el aislamiento de Pseudomona aeruginosa en el menor de 1 año. Casi la mitad de los pacientes no tienen complicaciones, se consiguió mejorar el estado nutricional y disminuir la mortalidad.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  Patients with cystic fibrosis have had a greater survival and life quality in the last decades; this is due to a precocious diagnostic, a better nutrition state, and diminution of respiratory infections.  Objective: To describe the characteristics of cystic fibrosis patients and follow up process to survivors for the last 40 years.  Methods: Descriptive, longitudinal and retrospective study in 96 patients diagnosed with cystic fibrosis in &#8220;William Soler&#8221; Pediatric University Hospital (1977-2017). Data was obtained from clinical records. Demographic, temporary, clinical, genetic and microbiological variables were analized.  Results: From the sample, 27 patients keep under medical follow up; 9 patients older than 19 years old were remitted to the Neumology consultation; 30 died; and 30 patients are not followed up at the hospital. 60.4 % of the patients was diagnosed before the first year of life, t was predominant the male sex (62.5 %) and most of them were white skin (88.5 %). Diagnosis suggested; the respiratory clinical symptoms, 39.6 %; mixed, 38.5 %; digestive symptoms, 19.8 %; salt lost, 2.1 %. Out of the 27 cases followed at present, 74.1 % are eutrophics, and there are not patients with malnutrition. In the first microbiological isolation, Pseudomonas aeruginosa prevailed in patients under 1-year-old and Staphilococcus aureus in patients between 1 and 4 years old. More than 37.0 % of patients had respiratory complications, 14.8 % had hepatic complications, and 40.7 % had no complications. The genetic characterization was mostly of &#8710;F508 (59.2 %).  Conclusions: Pseudomonas aeruginosa is frequently in patients under 1-year-old. Almost half of the patients has no complications; and it is improved their nutritional state and mortality decreased.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[fibrosis quística]]></kwd>
<kwd lng="es"><![CDATA[Pseudomonas aeruginosa]]></kwd>
<kwd lng="es"><![CDATA[mutación genética fibrosis quística]]></kwd>
<kwd lng="en"><![CDATA[cystic fibrosis]]></kwd>
<kwd lng="en"><![CDATA[Pseudomonas aeruginosa]]></kwd>
<kwd lng="en"><![CDATA[genetic cystic fibrosis mutation]]></kwd>
</kwd-group>
</article-meta>
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