<?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>1028-4818</journal-id>
<journal-title><![CDATA[Multimed]]></journal-title>
<abbrev-journal-title><![CDATA[Multimed]]></abbrev-journal-title>
<issn>1028-4818</issn>
<publisher>
<publisher-name><![CDATA[Centro Provincial de Información de Ciencias Médicas]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1028-48182022000600005</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Metodología para la atención integral a pacientes con genodermatosis]]></article-title>
<article-title xml:lang="en"><![CDATA[Methodology for the integral attention to patient with genodermatoses]]></article-title>
<article-title xml:lang="pt"><![CDATA[Metodologia para a atenção integral ao paciente com genodermatose]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Velázquez Ávila]]></surname>
<given-names><![CDATA[Yordania]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Batista Romagosa]]></surname>
<given-names><![CDATA[Maritza]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Espinosa Reyes]]></surname>
<given-names><![CDATA[Dayana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Orraca Castillo]]></surname>
<given-names><![CDATA[Miladys]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pérez Bruzón]]></surname>
<given-names><![CDATA[Dailin]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad de Ciencias Médicas de Las Tunas Hospital Pediátrico Provincial Docente Mártires de Las Tunas ]]></institution>
<addr-line><![CDATA[Las Tunas ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital Militar Dr. Joaquín Castillo Duany  ]]></institution>
<addr-line><![CDATA[Santiago de Cuba ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Departamento provincial de Genética Médica  ]]></institution>
<addr-line><![CDATA[Pinar del Río ]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2022</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2022</year>
</pub-date>
<volume>26</volume>
<numero>6</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S1028-48182022000600005&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S1028-48182022000600005&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S1028-48182022000600005&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN Las genodermatosis constituyen un grupo de enfermedades genéticas con afectación de la piel y sus anexos. En Cuba, el Programa Nacional de Diagnóstico, Atención y Prevención de Enfermedades Genéticas, en relación a las genodermatosis, no cuenta con protocolos para su diagnóstico, tratamiento y seguimiento. El objetivo del estudio es evaluar una metodología para la atención a los pacientes con genodermatosis. Se realizó en Las Tunas, provincia oriental de Cuba, un estudio cuasi-experimental, aplicándose la variante Delphy del método de expertos, siendo consultados un grupo de especialistas cubanos de dermatología, genética médica clínica y pediatría, con alto nivel científico y experiencia en el trabajo con pacientes con genodermatosis. Diseñándose la metodología que propone el protocolo del diagnóstico, tratamiento y algoritmo de seguimiento para estos pacientes. Se estudiaron 395 pacientes atendidos en el Departamento provincial de Genética Médica. Se estudiaron la tasa de prevalencia, la media de casos diagnosticados por año, la proporción de complicaciones presentadas, el índice de supervivencia e índice de letalidad y para relacionar las variables referentes a la mejoría del estado dermatológico y manifestaciones extracutáneas se utilizó la prueba estadística de Chi cuadrado de Mc-Nemar, con una significación estadística p&#8804;0,05. Después de implementada la metodología, predominó la neurofibromatosis 1, síndrome de Ehlers Danlos clásico e ictiosis vulgar, la media de casos diagnosticados por año aumentó; disminuyeron las complicaciones, predominando las piodermitis (6.13 %); el índice de mortalidad fue bajo (1.27%) con alto índice de supervivencia (98.73%) y mejoría de las manifestaciones dermatológicas (MCNemar X2=90.41558, P=0.000000) y extracutáneas (McNemar X2=24.083334, P=0.000001). La metodología diseñada para la atención a pacientes con genodermatosis fortalece el Programa Nacional de Diagnóstico, Atención y Prevención de Enfermedades Genéticas, demostrando ser efectiva, con mayor número de casos diagnosticados, menor proporción de complicaciones, alta supervivencia, baja letalidad y mejoría clínica de las manifestaciones dermatológicas y extracutáneas.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT The genodermatoses constitutes a group of genetic diseases with affectation of the skin and their annexes. In Cuba, the National Program of Diagnostic, Attention and Prevention of Genetic diseases, in relation to the genodermatoses, don't have protocols for their diagnosis, treatment and pursuit. The objective of this study is to evaluate a methodology for the attention to the patients with genodermatosis. Was carried out in The Tunas, oriental county of Cuba, a quasi-experimental study, being applied the varying Delphy of the method of experts, being consulted a group of Cuban specialists of dermatology, genetics clinical doctor and pediatrics, with high scientific level and experience in the work with patient with genodermatoses. Being designed the methodology that proposes the protocol of the diagnosis, treatment and pursuit algorithm for these patients. 395 patients were studied assisted in the provincial Department of Medical Genetics. Were studied the prevalence rate, the stocking of cases diagnosed per year, the proportion of presented complications, the index of survival and lethality index and to relate the relating variables to the improvement of the state dermatologic and extracutaneous manifestations the statistical test of square Chi of Mc-Nemar was used, with a significance statistical p&#8804;0,05. After having implemented the methodology, prevailed the neurofibromatosis, Ehlers Danlos syndrome and ichthyosis vulgaris; the stocking of cases diagnosed per year increased; they diminished the complications, prevailing the piodermitis (6.13%); the index of mortality was low (1.27%) with high index of survival (98.73%) and improvement of the manifestations dermatologic (MCNemar X2=90.41558, P=0.000000) and extracutaneous (McNemar X2=24.083334, P=0.000001). The methodology designed for the attention to patient with genodermatoses strengthens the National Program of Diagnostic, Attention and Prevention of Genetic Illnesses, demonstrating to be effective, with bigger number of diagnosed cases, smaller proportion of complications, high survival, low lethality and clinical improvement of the dermatologic and extracutaneous manifestations.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[RESUMO As genodermatoses são um grupo de doenças genéticas com envolvimento da pele e seus anexos. Em Cuba, o Programa Nacional de Diagnóstico, Cuidado e Prevenção de Doenças Genéticas, em relação às genodermatoses, não possui protocolos para seu diagnóstico, tratamento e acompanhamento. O objetivo do estudo é avaliar uma metodologia para o cuidado de pacientes com genodermatose. Um estudo quase-experimental foi realizado em Las Tunas, província oriental de Cuba, aplicando a variante Delphy do método expert, sendo consultado um grupo de especialistas cubanos em dermatologia, genética médica clínica e pediatria, com alto nível científico e experiência no trabalho com pacientes com genodermatose. Desenho da metodologia que propõe o protocolo de diagnóstico, tratamento e algoritmo de acompanhamento para esses pacientes. Um total de 395 pacientes tratados no Departamento Provincial de Genética Médica foram estudados. Foram estudadas as prevalências, o número médio de casos diagnosticados por ano, a proporção de complicações apresentadas, a sobrevida e o índice de letalidade e para relacionar as variáveis referentes à melhora do estado dermatológico e das manifestações extracutâneas, utilizou-se o teste estatístico Qui-quadrado de Mc-Nemar, com significância estatística p&#8804;0, 05. Após a implementação da metodologia, predominaram a neurofibromatose 1, a síndrome clássica de Ehlers Danlos e a ictiose vulgar, aumentando-se o número médio de casos diagnosticados por ano; complicações diminuídas, predominantemente piodermite (6,13%); a taxa de mortalidade foi baixa (1,27%), com alta sobrevida (98,73%) e melhora dermatológica (MCNemar X2=90,41558, P=0,000000) e extracutânea (McNemar X2=24,083334, P=0,000001). A metodologia desenhada para o cuidado de pacientes com genodermatose fortalece o Programa Nacional de Diagnóstico, Cuidado e Prevenção de Doenças Genéticas, mostrando-se eficaz, com maior número de casos diagnosticados, menor proporção de complicações, alta sobrevida, baixa letalidade e melhora clínica das manifestações dermatológicas e extracutâneas.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Genodermatosis]]></kwd>
<kwd lng="es"><![CDATA[Genética médica]]></kwd>
<kwd lng="es"><![CDATA[Neurofibromatosis]]></kwd>
<kwd lng="es"><![CDATA[Síndrome de Ehlers Danlos]]></kwd>
<kwd lng="es"><![CDATA[Ictiosis vulgar]]></kwd>
<kwd lng="en"><![CDATA[Genodermatoses]]></kwd>
<kwd lng="en"><![CDATA[Medical genetics]]></kwd>
<kwd lng="en"><![CDATA[Neurofibromatosis]]></kwd>
<kwd lng="en"><![CDATA[Ehlers Danlos syndrome]]></kwd>
<kwd lng="en"><![CDATA[Ichthyosis vulgaris]]></kwd>
<kwd lng="pt"><![CDATA[Genodermatose]]></kwd>
<kwd lng="pt"><![CDATA[Genética médica]]></kwd>
<kwd lng="pt"><![CDATA[Neurofibromatose]]></kwd>
<kwd lng="pt"><![CDATA[Síndrome de Ehlers Danlos]]></kwd>
<kwd lng="pt"><![CDATA[Ictiose vulgar]]></kwd>
</kwd-group>
</article-meta>
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