<?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>S1688-12492020000600359</article-id>
<article-id pub-id-type="doi">10.31134/ap.91.6.4</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Evolución del síndrome nefrótico idiopático del niño]]></article-title>
<article-title xml:lang="en"><![CDATA[Idiopathic nephrotic syndrome in children]]></article-title>
<article-title xml:lang="pt"><![CDATA[Evolução da síndrome nefrótica idiopática da criança]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Halty]]></surname>
<given-names><![CDATA[Margarita]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Caggiani]]></surname>
<given-names><![CDATA[Marina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cocchi]]></surname>
<given-names><![CDATA[Valeria]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Noria]]></surname>
<given-names><![CDATA[Alejandro]]></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-group>
<aff id="Af1">
<institution><![CDATA[,UDELAR Facultad de Medicina Clínica Pediátrica]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,UDELAR Facultad de Medicina Clínica Pediátrica]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,ASSE HP.CHPR Depto. Emergencia]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="Af4">
<institution><![CDATA[,UDELAR Facultad de Medicina Depto. Métodos Cuantitativos]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,UDELAR Facultad de Medicina Clínica Pediatría]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>00</month>
<year>2020</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>00</month>
<year>2020</year>
</pub-date>
<volume>91</volume>
<numero>6</numero>
<fpage>359</fpage>
<lpage>369</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_arttext&amp;pid=S1688-12492020000600359&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_abstract&amp;pid=S1688-12492020000600359&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_pdf&amp;pid=S1688-12492020000600359&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[resumen está disponible en el texto completo]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Summary:  Introduction:  the idiopathic nephrotic syndrome (INS) is the most common glomerulopathy in pediatrics. The purpose of this study is to estimate the INS incidence in pediatric public health patients and to describe their response to treatment and their evolutionary characteristics and complications.  Methods:  a descriptive study was carried out of a historical cohort of patients under 15 years of age with first time INS assisted in the Nephrological Clinic of the Pereira Rossell Hospital Center between 01/01/2009 - 12/31/2013, monitored until 12/31/ 2015, users of the State Health Services (ASSE). Their annual incidence was estimated according to data obtained from the National Data System Report (SINADI) of the Ministry of Public Health. We recorded age, sex, height, body mass index, response to initial treatment and evolution and followed them up &gt;12 months.  Results: we included 42 patients with a male / female ratio 2.2 / 1; the SNI annual incidence in the ASSE population was 3.05 / 100,000 children under 15 years of age. 34 were cortico-sensitive (CS) and 8 cortico-resistant (CR). The mean age of the first incident was 5 years in CS and 2 years 10 months in CR. The evolution was analyzed in the 33 patients monitored for a period of time &gt;12 months, with a mean follow-up of 4 years ± 1 year 9 months; In these children, the frequency of CS was 78.7% (n = 26) and of RC 21.3% (n = 7). Within the CS, there were 10 (38.6%) cortico-dependents / frequent relapsers (DC), 8 had sporadic relapses and 8 did not relapse. Among the DCs, 5 received cyclophosphamide, in one patient prednisone was suspended at 3 months, in 2 of them the dose was lowered and the other 2 did not respond, one of them achieved sustained remission with cyclosporine. Of the 7 RCs, 4 had initial remission with cyclosporine, 1 partial remission and 2 were resistant; 3 received mycophenolate mofetil, 2 did not respond, and 1 had partial remission. The biopsy in all the RCs showed focal and segmental hyalinosis. The genetic study for NPHS2 and WT1 carried out on 4 patients was negative. At the end of follow-up, all CS patients were in remission, 16/26 without treatment. Among the RCs, 2 were in remission with cyclosporine, 4 maintained nephrotic proteinuria (one of them with a drop in glomerular filtration rate of 60 ml/min/1.73m2 sc), and another was on chronic dialysis. Serious infectious complications occurred in 5 CR and 2 CS patients. One CD and one CR patient developed chronic arterial hypertension. In CD and RC, a significant difference was observed in the Z score of the BMI and height between the beginning and the end of the follow-up.  Conclusions:  the calculated incidence includes only patients treated by the State Health Services in our country; it does not reflect the global incidence at national level, and it is comparable to that of other series. The evolution of CS and CR patients was also similar to those described. The growth of children exposed to long-term corticosteroid treatment was affected. For best results, these patients should be offered customized treatment according to current recommendations.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo:  Introdução:  a síndrome nefrótica idiopática (SNI) é a glomerulopatia mais comum em pediatria. O objetivo do estudo é estimar a incidência em pacientes usuários da assistência pública e descrever a resposta ao tratamento, as características evolutivas e as complicações.  Métodos: realizou-se um estudo descritivo de uma coorte histórica de pacientes menores de 15 anos de idade atendidos na Policlínica Nefrológica do Centro Hospitalar Pereira Rossell com SNI entre 01/01/2009 - 31/12/2013, controlados até 31/12 / 2015, usuários dos Serviços de Saúde do Estado (ASSE). Estimou-se a incidência anual em pacientes de ASSE menores de 15 anos de idade, segundo dados do relatório do Sistema Nacional de Dados (SINADI) do Ministério da Saúde Pública. Se registraram a idade, sexo, altura, índice de massa corporal, resposta ao tratamento inicial e evolução dos pacientes com seguimento &gt;12 meses.  Resultado: foram incluídos 42 pacientes com relação sexo masculino/feminino 2,2/1; a incidência anual de SNI na população ASSE foi de 3,05/100.000 crianças menores de 15 anos de idade. 34 eram córtico-sensíveis (CS) e 8 córtico-resistentes (CR). A idade média do começo da doença foi de 5 anos no CS e 2 anos e 10 meses no CR. A evolução foi analisada nos 33 pacientes controlados por um período de tempo &gt;12 meses, com seguimento médio de 4 anos ± 1 ano 9 meses; nessas crianças, a frequência de SC foi de 78,7% (n = 26) e de RC de 21,3% (n = 7). No CS, havia 10 (38,6%) corticodependentes/recidivas frequentes (CD), 8 tiveram recidivas esporádicas e 8 não tiveram recidiva. Dentre as CDs, 5 receberam ciclofosfamida, numa criança a prednisona foi suspensa aos 3 meses, em 2 delas a dose foi diminuída e as outras 2 não responderam, uma delas obteve remissão sustentada com ciclosporina. Dos 7 casos de CRs, 4 tiveram remissão inicial com ciclosporina, 1 remissão parcial e 2 foram resistentes; 3 receberam micofenolato de mofetil, 2 não responderam e 1 teve remissão parcial. A biópsia em todos os CRs mostrou hialinose focal e segmentar. O estudo genético para NPHS2 e WT1 realizado em 4 pacientes foi negativo. Ao final do acompanhamento, todos os pacientes com SC estavam em remissão, 16/26 sem tratamento. Entre os CRs, 2 estavam em remissão com ciclosporina, 4 mantinham a proteinúria nefrótica (um deles com queda na taxa de filtração glomerular de 60 ml/min/1,73 m2 sc) e outro estava em diálise crônica. Complicações infecciosas graves ocorreram em 5 pacientes com RC e 2 com SC. Um paciente com DC e um com RC desenvolveram hipertensão arterial crônica. Em DC e RC, foi observada diferença significativa no escore Z do IMC e da estatura entre o início e o final do seguimento.  Conclusões:  a incidência calculada corresponde apenas ao setor da assistência pública de nosso país, não reflete a incidência global em nível nacional, sendo comparável a outras séries. A evolução dos pacientes com SC e RC também foi semelhante às descritas. O crescimento das crianças expostas ao tratamento com corticosteróides a longo prazo se afetou. Esses pacientes devem receber tratamento individualizado de acordo com as recomendações atuais para obter os melhores resultados.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Síndrome nefrótico]]></kwd>
<kwd lng="es"><![CDATA[Niño]]></kwd>
<kwd lng="en"><![CDATA[Nephrotic syndrome]]></kwd>
<kwd lng="en"><![CDATA[Child]]></kwd>
<kwd lng="pt"><![CDATA[Síndrome nefrótica]]></kwd>
<kwd lng="pt"><![CDATA[Criança]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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