<?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>1688-1249</journal-id>
<journal-title><![CDATA[Archivos de Pediatría del Uruguay]]></journal-title>
<abbrev-journal-title><![CDATA[Arch. Pediatr. Urug.]]></abbrev-journal-title>
<issn>1688-1249</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Uruguaya de Pediatría]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1688-12492021000101205</article-id>
<article-id pub-id-type="doi">10.31134/ap.92.1.12</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Características epidemiológicas y clínicas de los menores de 18 años con hemofilia asistidos en el Centro Hospitalario Pereira Rossell. 2016 - 2018]]></article-title>
<article-title xml:lang="en"><![CDATA[Epidemiologic and clinical characteristics of hemophilic patients of under 18 years of age assisted at the Pereira Rossell Hospital Center. 2016-2018]]></article-title>
<article-title xml:lang="pt"><![CDATA[Características epidemiológicas e clínicas de menores de 18 anos com hemofilia assistidos no Centro Hospitalar Pereira Rossell. 2016 - 2018]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Casuriaga]]></surname>
<given-names><![CDATA[Ana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Lemos]]></surname>
<given-names><![CDATA[Felipe]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Giachetto]]></surname>
<given-names><![CDATA[Gustavo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Jaime]]></surname>
<given-names><![CDATA[Juan Manuel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Martínez]]></surname>
<given-names><![CDATA[Camila]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Martínez]]></surname>
<given-names><![CDATA[Analía]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Massud]]></surname>
<given-names><![CDATA[Juliana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Mautone]]></surname>
<given-names><![CDATA[Keila]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Medina]]></surname>
<given-names><![CDATA[Santiago]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Boggia]]></surname>
<given-names><![CDATA[Beatriz]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,UDELAR Facultad de Medicina Clínica Pediátrica &#8220;C&#8221;]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,CHPR Depto. Medicina Transfusional ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="Af3">
<institution><![CDATA[,UDELAR Facultad de Medicina Clínica Pediátrica &#8220;C&#8221;]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,UDELAR Facultad de Medicina Ciclo Metodología 2]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,CHPR Depto. Medicina Transfusional ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>06</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>06</month>
<year>2021</year>
</pub-date>
<volume>92</volume>
<numero>1</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_arttext&amp;pid=S1688-12492021000101205&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_abstract&amp;pid=S1688-12492021000101205&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_pdf&amp;pid=S1688-12492021000101205&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen:  Introducción:  la hemofilia es una enfermedad hereditaria, ligada al cromosoma X, debida al déficit de factor VIII (tipo A) o IX (tipo B). La prevalencia estimada al nacimiento es de 24,6 casos cada 100.000 varones para hemofilia A y 5 casos cada 100.000 para hemofilia B. El Departamento de Medicina Transfusional (DMT) del Centro Hospitalario Pereira Rossell (CHPR) es el Centro de Referencia Nacional (CDRN) para los menores de 18 años. El abordaje integral, inter-disciplinario del paciente con hemofilia en un centro especializado disminuye la morbi-mortalidad y contribuye a mejorar la calidad de vida.  Objetivo:  describir las características epidemiológicas y clínicas de los menores de 18 años con hemofilia asistidos en el DMT-CHPR entre el 1 enero de 2016 y el 31 de diciembre de 2018.  Metodología:  estudio descriptivo, retrospectivo, de todos los menores de 18 años con hemofilia. Se describió: edad y circunstancias del diagnóstico, tipo y severidad de la hemofilia, controles en salud, estudios complementarios, complicaciones, frecuencia y motivos de hospitalización, tratamiento. El protocolo de estudio fue aprobado por el Comité de Ética Institucional.  Resultados:  se asistieron 67 pacientes, 57 con hemofilia A y 10 con hemofilia B. La mediana de edad fue 8 años. Presentaban hemofilia severa 61 pacientes, moderada 2 y leve 4. Presentaban antecedentes familiares de coagulopatía 41. La mediana de edad al diagnóstico fue 2 meses. Se diagnosticaron en el período neonatal 24 de los pacientes con hemofilia A y 5 con hemofilia B. Desarrollaron inhibidores 7 pacientes, todos con hemofilia severa.  Conclusiones:  en esta serie, predominaron los pacientes con hemofilia A, severa, antecedentes familiares conocidos de coagulopatía, en tratamiento profiláctico con factores de la coagulación. Esta comunicación aporta información valiosa sobre las características de estos pacientes, lo que contribuye a la gestión clínica y a planificar estrategias de mejora de la calidad asistencial.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Summary:  Introduction:  hemophilia is a hereditary disease, linked to chromosome X and caused by the deficit of factor VIII (type A) and IX (type B). Estimated prevalence at birth is 24.6 cases every 100,000 boys for hemophilia A and 5 cases every 100,000 cases for hemphilia B. The Transfusion Medical Department (TMD) of the Pereira Rossell Children&#8217;s Hospital Center (CHPR, acronym in Spanish) is the national reference center (NRC) for patients under 18 years of age. A comprehensive, inter-disciplinary approach to hemophilic patients at a specialized center decreases morbidity and mortality and contributes to improving quality of life.  Objective:  to describe the epidemiologic, clinical and progression characteristics of hemophilic patients of under 18 years of age assisted at the TMD-CHPR between January 1st 2016 and December 31st, 2018.  Methodology:  descriptive, retrospective study of all hemophilic patients of under 18 years of age. Variables described: age, circumstances of diagnosis, type and severity of hemophilia, health check-ups, tests, complications, frequency and reasons for hospital admittance, treatment. The study protocol was approved by the Institutional Ethics Committee.  Results:  67 patients were assisted, 57 with hemophilia A and 10 with hemophilia B. Median age was 8 years. Severe hemophilia was present in 61 patients, moderate in 2 and mild in 4. 41 had a family history of coagulopathy. Median age at diagnosis was 2 months. 24 patients with hemophilia A and 5 patients with hemophilia B were diagnosed during the neonatal period. 7 patients developed inhibitors, all of them with severe hemophilia.  Conclusions:  in this study there is a predominance of patients with severe hemophilia A, known family history of coagulopathy, under prophylactic treatment with coagulation factors. This study provides valuable information about the characteristics of these patients, which contributes to improved clinical management and planning strategies to improve their quality of care.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo:  Introdução:  o Departamento de Medicina Transfusional (DMT) do Centro Hospitalar Pereira Rossell (CHPR) é o Centro Nacional de Referência (CNR) para menores de 18 anos de idade. A abordagem abrangente e interdisciplinar do paciente com hemofilia em um centro especializado reduz a morbimortalidade e contribui para a melhoria da qualidade de vida.  Objetivo:  descrever as características epidemiológicas, clínicas e evolutivas em crianças menores de 18 anos com hemofilia atendidas no DMT-CHPR entre 1 de janeiro de 2016 e 31 de dezembro de 2018.  Metodologia:  estudo descritivo, retrospectivo, de todos os menores de 18 anos com hemofilia. Descrevemos: idade e circunstâncias do diagnóstico, tipo e gravidade da hemofilia, controles de saúde, estudos complementares, complicações, frequência e motivos de hospitalização, tratamento. O protocolo do estudo foi aprovado pelo Comitê de Ética Institucional.  Resultados:  67 pacientes foram atendidos, 57 com hemofilia A e 10 com hemofilia B. A media de idade foi de 8 anos. Houve 61 pacientes com hemofilia grave, moderada 2 e leve 4. 41 tiveram história familiar de coagulopatia. A media de idade no diagnóstico foi de 2 meses. 24 dos pacientes com hemofilia A e 5 com hemofilia B foram diagnosticados no período neonatal e 7 desenvolveram inibidores, todos com hemofilia grave.  Conclusões:  neste estudo, predominaram pacientes com hemofilia A grave, história familiar conhecida de coagulopatia, em tratamento profilático com fatores de coagulação. O estudo fornece informações valiosas sobre as características desses pacientes, o que contribui para o manejo clínico e estratégias de planejamento para melhorar a qualidade do atendimento deles.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Hemofilia A]]></kwd>
<kwd lng="es"><![CDATA[Hemofilia B]]></kwd>
<kwd lng="es"><![CDATA[Trastornos de la coagulación sanguínea]]></kwd>
<kwd lng="es"><![CDATA[Pediatría]]></kwd>
<kwd lng="en"><![CDATA[Hemophilia A]]></kwd>
<kwd lng="en"><![CDATA[Hemophilia B]]></kwd>
<kwd lng="en"><![CDATA[Blood coagulation disorders]]></kwd>
<kwd lng="en"><![CDATA[Pediatrics]]></kwd>
<kwd lng="pt"><![CDATA[Hemofilia A]]></kwd>
<kwd lng="pt"><![CDATA[Hemofilia B]]></kwd>
<kwd lng="pt"><![CDATA[Transtornos da coagulação sanguínea]]></kwd>
<kwd lng="pt"><![CDATA[Pediatria]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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