Evolución del síndrome nefrótico idiopático del niño / Idiopathic nephrotic syndrome in children / Evolução da síndrome nefrótica idiopática da criança

resumen está disponible en el texto completo

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 >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 >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.

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 >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 >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.

Dermatomiositis juvenil: experiencia de 13 años en un hospital de atención terciaria. Análisis de 17 casos clínicos / Juvenile dermatomyositis: 13 years of experience in a tertiary care hospital. Analysis of 17 clinical cases / Dermatomiosite juvenil: 13 anos de experiência em hospital de nível terciário de atenção. Analise de 17 casos clinicos

La dermatomiositis juvenil (DMJ) es una miopatía inflamatoria adquirida de base inmunológica acompañada por alteraciones cutáneas características. El objetivo de este artículo es describir las características clínicas y exámenes complementarios de un grupo de 17 pacientes con diagnóstico de DMJ, su evolución y tratamiento. Material y método: se estudiaron los pacientes que se asistieron en la Policlínica de enfermedades de tejido conectivo del Centro Hospitalario Pereira Rossell (CHPR) en el período del 1 de octubre de 2003 al 1 de abril de 2017. Resultados: los rasgos clínicos más frecuentes de presentación fueron las manifestaciones cutáneas características, debilidad muscular, síntomas constitucionales, manifestaciones gastrointestinales y respiratorias. Las enzimas musculares estuvieron aumentadas en todos los casos. La resonancia nuclear magnética, electromiograma y la biopsia muscular fueron patológicos en todos los casos realizados. El tratamiento se realizó fundamentalmente en base a corticoides y fármacos inmunosupresores, siendo el metotrexate la droga de elección. En los casos graves o refractarios se asoció gamaglobulina, ciclofosfamida o ciclosporina. La duración del tratamiento tuvo una mediana de 3 años 10 meses. Se logró remisión en 47% de los pacientes. La evolución fue monofásica en 15,4%, de los casos, polifásica en 8% y crónica en 77%. No hubo fallecimientos registrados, ni enfermedad maligna asociada. Conclusiones: la DMJ es una enfermedad de baja incidencia. La mayoría de los pacientes tuvieron una evolución crónica. Esto determina la necesidad de un tratamiento inmunosupresor prolongado con los efectos adversos de la misma. Se logró la remisión en 47% de los pacientes. No se registraron fallecimientos en la serie estudiada.

Juvenile dermatomyositis (JDM) is an acquired inflammatory myopathy with an immunologic basis and characteristic cutaneous rash. The aim of this article is to describe clinical features and most important exams of a group of 17 patients with JDM their evolution and treatment. Methods: children with JDM recruited from Connective tissue Diseases Office of Pereira Rossell Hospital from 1/10/2003 through 1/4/2017 were studied. Results: the most frequent features were: characteristic cutaneous rash, muscle weakness, systemic symptoms, gastrointestinal and respiratory manifestations. The diagnostic investigations showed an increase serum muscle enzymes in all patients. The nuclear magnetic resonance, electromyogram and muscle biopsy resulted abnormal in all the investigated cases. Treatment was based on corticosteroids and immunosuppressive drugs being methotrexate the preferred drug. In severe or refractory cases cyclophosphamide, human gammaglobulin or cyclosporine were associated. Median treatment length was 3 years 10 months Remission was achieved in 47 percent. The evolution was monophasic in 15.4 percent, polyphasic in 7.7 and chronic in 77 percent. No deaths were registered neither malignant associated diseases Conclusions: JDM is an infrequent illness. Most of the patients had chronic evolution. This obliges to prolonged immunosuppression with its adverse effects. Remission was achieved in 47% of the cases. No deaths were registered in this population.

A dermatomiosite juvenil (DMJ) é uma miopatia inflamatória imunológica adquirida acompanhada de alterações cutâneas características. O objetivo deste artigo é descrever as características clínicas e os exames complementares de um grupo de 17 pacientes diagnosticados com DMJ, sua evolução e tratamento. Materiais e métodos: foram estudados os pacientes que compareceram à Policlínica das Doenças do Tecido Conjuntivo no Centro Hospitalar Pereira Rossell (CHPR) no período de 01/10/2003 a 04/01/2017. Resultados: as características clínicas mais frequentes foram manifestações cutâneas características, fraqueza muscular, sintomas constitucionais, manifestações gastrointestinais e respiratórias. As enzimas musculares estiveram aumentadas em todos os casos. A Ressonância nuclear magnética, o eletromiograma e a biópsia muscular foram patológicos em todos os casos. O tratamento foi baseado principalmente em corticosteroides e drogas imunossupressoras, e o metotrexato foi a droga de escolha. Em casos graves ou refratários, também se administrou gamaglobulina, ciclofosfamida ou ciclosporina. A duração do tratamento teve uma mediana de 3 anos e 10 meses. A remissão foi alcançada em 47% dos pacientes. A evolução foi monofásica em 15,4% dos casos, polifásica em 8% e crônica em 77%. Não houve mortes registradas, nem doença maligna associada. Conclusões: a DMJ é uma doença de baixa incidência. A maioria dos pacientes teve evolução crônica. Isso determina a necessidade de um tratamento imunossupressor prolongado com os seus conseguintes efeitos adversos. A remissão foi alcançada em 47% dos pacientes. Nenhuma morte foi registrada na série estudada.

Correlación clínico patológica y evolución de la nefropatía lúpica en niños y adolescentes / Clinicopathologic correlation and evolution of lupus nephritis in children and adolescents

El lupus eritematoso sistémico (LES) tiene una prevalencia baja en la población general y es menor en la edad pediátrica. La nefropatía lúpica (NL) es más frecuente y de mayor severidad que en adultos, condicionando la morbimortalidad de la enfermedad. Se realizó un estudio descriptivo prospectivo de 20 niños y adolescentes con NL controlados en la Policlínica de Colagenopatías del Centro Hospitalario Pereira Rossell en el período desde octubre de 2003 hasta setiembre de 2013 con el objetivo de describir las características clínico-serológicas y evolutivas de pacientes con NL y correlacionarlas con los hallazgos anátomopatológicos. La NL se observó en el 52,6% de los casos con LES. El 70% fueron de sexo femenino, relación femenino/masculino de 2,3/1, 85% de raza blanca, la mediana del diagnóstico fue de 12 años. Las formas de presentación fueron: alteraciones urinarias menores (AUM) en 14 pacientes (0,7), en cuatro casos síndrome nefrótico (SN), con o sin insuficiencia renal (IR) y/o hipertensión arterial. Un paciente se manifestó con síndrome nefrítico. Un paciente tenía un examen de orina normal. Las formas histopatológicas proliferativas graves se presentaron en 18 (0,9); los casos con AUM presentaban NL grado III-IV en 13 (0,93); todos los casos con SN con o sin IR tenían NL III-IV. No hubo casos de NL aislada como forma de comienzo. En el momento del diagnóstico, los anticuerpos antinucleares fueron positivos en 19 (0,95) y los anti DNA doble cadena en 16 (0,8); C3 y C4 estuvieron descendidos en 19 (0,95) y en 15 (0,75) respectivamente. El seguimiento promedio fue 4,2 años. Al final del seguimiento estaban en remisión 16 pacientes (0,8), cuatro en remisión parcial, todos con función renal normal, excepto un caso que presentó IR extrema, fue trasplantado y tuvo una excelente evolución. Un paciente falleció con hemorragia pulmonar. La sobrevida de la función renal y la de los pacientes fue 0,95 respectivamente. El tratamiento se realizó en base a corticoides, hidroxicloroquina asociados a azatioprina o micofenolato mofetilo. En ocho pacientes con cuadros graves se usó la ciclofosfamida I/V. Esta serie constituye la primera serie nacional de nefropatía lúpica en niños y adolescentes. Conclusión: predominó la presentación clínica con AUM y formas histopatológicas severas, clases III y IV, evidenciando una disociación clínico anatomopatológica. A pesar del elevado porcentaje de NL severas, el manejo adecuado y oportuno y la adherencia al tratamiento y a los controles médicos fueron fundamentales para la evolución favorable de la NL.

Systemic lupus erythematosus (SLE) has a low prevalence in the overall general population and this is lower in children. Child lupus nephropathy (LN) is more frequent and severe than in SLE adult patients, with greater disease morbidity and mortality. A prospective descriptive study of 20 children and adolescents with LN monitored in the Collagen Diseases Office of the Pereira Rossell Hospital between October, 2003 and September, 2013 was performed. The objective of this study was to describe clinical-serological features and the evolution of these patients and to correlate them with its anatomopathological findings. LN was diagnosed in 52,6% of the SLE patients, 70% were female with a female/male correlation of 2,3/1; 85% were Caucasian; median age at diagnosis was 12 years old. The clinical presentations were minor urinary findings (MUF) in 14 patients (0,7) and nephrotic syndrome (NS) in 4 (0,2), and another one nephritic syndrome. One patient presented no symptoms and had normal urinalysis. Severe proliferative classes predominated in18 patients (0.9); 13 (0.93) patients with MUF and all the patients with NS had LN classes III or IV. Isolated LN was not seen in the initial presentation. At the time of diagnosis antinuclear antibodies were positive in 19 patients (0.95); and anti DNA double stranded in 16 (0.8). Low C3 was found in 19 (0.95) and C4 in 15 (0.75), respectively. Average follow-up time was 4.2 years. At the end of follow-up 16 (0.8) were in remission, 4 of them in partial remission; all patients presented normal renal function except for one who evidenced severe renal failure and required hemodialysis and transplantation and had an excellent evolution. One patient died with pulmonary hemorrhage. The renal and patient survivals were 0.95 respectively. Treatment consisted in corticosteroids and hydroxychloroquine associated with azathioprine or mycophenolate mofetil. Cyclophosphamide was administered to 8 patients with severe illness. This is the first national report of LN in children and adolescents. Conclusions: the predominant clinical presentation of LN was MUF with severe anatomopathological findings, classes III and IV, showing a clinical-pathological dissociation. Despite the high percentage of severe LN, early and adequate treatment, as well as a good compliance to it with periodic medical follow-up, were essential to achieve a favorable outcome of LN.

Lupus eritematoso sistémico en niños y adolescentes / Systemic lupus erythematosus in children and adolescents

El lupus eritematoso sistémico (LES) es una enfermedad autoinmune de evolución crónica con períodos de exacerbación y remisiones. Puede afectar a todos los tejidos del organismo, dando origen a muy diversos cuadros clínicos. El 20% de los LES se presentan en la edad pediátrica con cuadros clínicos que en general son de mayor gravedad que en adultos. El objetivo de este estudio es describir las características clínico-serológicas y evolutivas de 39 casos de LES controlados en la Policlínica de Colagenopatías del Centro Hospitalario Pereira Rossell en el periodo octubre de 2003- mayo de 2014. El 84,6% fueron de sexo femenino. con relación femenino/masculino 5,5/1. El 80% fueron de raza blanca. El promedio de edad fue de 11 años con un rango 3-15 años. 10% tuvieron antecedentes familiares de LES. El 97,4% pertenecieron al grupo de LES grave. EL 82% presentaron manifestaciones generales y cutaneomucosas, 72% hematológicas, 67% articulares, 51% compromiso renal, 41% respiratorio. Las manifestaciones cardiovasculares se observaron en el 28%, las neurológicas en 21%, las gastrointestinales y hepáticas en 18%. Se realizó un seguimiento promedio de 3 años 6 meses. Se logró la remisión total o parcial en 67% de los pacientes. El tratamiento fue en base a corticosteroides, hidroxicloroquina; en la gran mayoría de los casos se asoció un inmunosupresor como azathioprina, micofenolato, ciclofosfamida de acuerdo al cuadro clínico. La mortalidad fue de 5%. Las complicaciones más frecuentes fueron las infecciosas. Un diagnóstico temprano, un tratamiento y seguimiento adecuado son importantes para mejorar el pronóstico de esta severa enfermedad.

Systemic lupus erythematosus is a chronic autoinmune disease that evolves in relapses and remissions. It can affect every tissue of the body producing diverse clinical manifestations. Twenty per cent of SLE begin during childhood and are generally more severe than in adults. The objective of this study is to describe 39 patients with SLE controlled in the Connective tissue diseases clinic of the Pereira Rossell Hospital between October 2003 and May 2014. 84.6% were female with female ratio 5.51. 80% were Caucasian. Mean age at diagnosis was 11 years. Ten percent had family history of SLE. 97.4% of SLE were severe. 82% presented constitutional symptoms and mucocutaneous manifestations, 72% had hematological compromise. Arthritis was seen in 67%, renal involvement in 51%, respiratory tract was affected in 41%. 28% presented cardiovascular compromise and 21% had neurological involvement. The liver and gastrointestinal system was affected in 18%. Mean patient follow up was 3.6 years. Complete or partial remission was accomplished in 67%. Treatment included corticosteroids and hydroxychloroquine; other inmunosupressives as azathioprine, mycophenolate and cyclophosphamide were elected depending on the clinical pattern. Mortality was 5%. Infections were the most frequent complication. Early diagnosis, an adequate treatment and follow up are needed to improve the outcome of this severe illness.

Anomalías nefrourológicas congénitas en niños hospitalizados / Nephrourological congenital anomalies in children hospitalized

Introducción : las anomalías congénitas nefrourológicas se encuentran en segundo lugar en frecuencia entre las malformaciones detectadas por ecografía prenatal. La importancia del diagnóstico precoz radica en la posibilidad de prevenir infecciones urinarias, corregir la obstrucción urinaria y evitar o enlentecer el deterioro de la función renal. En nuestro país, el 30,4% de los menores de 15 años en diálisis crónica tiene una insuficiencia renal por nefrouropatía malformativa. Los objetivos fueron conocer la tasa de hospitalización, describir las características clínicas y evolutivas de los niños hospitalizados con diagnóstico ecográfico pre y postnatal de malformación nefrourológica. Material y métodos: estudio descriptivo, prospectivo. Se incluyeron los niños con ecografía del aparatourinario patológica hospitalizados en el sector de cuidados moderados del HP-CHPR entre 01/07/2012 y el 30/6/2013, se excluyeron aquellos con disfunción vesical o glomerulopatía crónica. Se valoró: edad, sexo, hallazgo ecográfico, diagnóstico nosológico, manifestaciones clínicas, evolución y tratamiento. Resultados: se incluyeron 44 niños, la tasa de hospitalización fue 3.9‰, relación masculino/femenino 1,9/1; la mediana de edad 23 meses (percentil 75: 91 meses). Se realizó ecografía prenatal en 36/44, de las cuales 18 fueron patológicas. Se controlaron ecográficamente luego del nacimiento 11/18. En los 26 pacientes restantes, el diagnóstico ecográfico se realizó en la etapa postnatal previo a la internación en 15 y durante la hospitalización en 11. El número de malformaciones halladas fue 66; 22 fueron bilaterales. Predominaron el reflujo vésicoureteral(n=19), las obstrucciones (n=15), y la agenesia/displasia renal (n=18). Requirieron tratamiento quirúrgico 21, endoscópico seis.(...)

Introduction: congenital abnormalities of urinary tract andkidneys rank second in frequency of structuralabnormalities on antenatal ultrasound. Early diagnosisallows to prevent urinary tract infections, to treat urinarytract obstruction and to avoid or slow the decline in renalfunction. In our country 30,4% of patients younger than 15years old on chronic dialysis have a kidney failurebecause of a congenital abnormality.The aims of this study were to know the hospitalizationrate and to describe clinic and evolutionary characteristicsof hospitalized children with a congenital abnormality ofurinary tract and kidneys diagnosed by antenatal/postnatal ultrasound.Patients and methods: a descriptive and prospectivestudy was performed. Hospitalized children in HP-CHPR,between 01/07/2012 and 30/06/2013, with pathologicultrasound of kidneys or urinary tract, were included.Children with bladder dysfunction or glomerulopathy wereexcluded. We considered age, gender, ultrasoundfindings, definitive diagnosis, clinic manifestations,treatment and outcome.Results: we included 44 children. Hospitalization rate was3.9 0/00, the relation male/female was 1.9/1 and themedian age 23 months (75th percentile: 91 months).Antenatal ultrasound was performed in 36/44 patients; 18of them were pathologic; among these last ones, 11/18underwent postnatal ultrasound to control findings. In theother 26 patients, the diagnosis of pathologic ultrasoundwas made after birth: in 15 of them before thehospitalization and in 11 during the hospitalization. Wefound 66 affected kidneys; 22 were bilateral. The mostfrequent diagnosis were: vesicoureteric reflux (n=19),obstruction (n=15) and renal dysplasia/ agenesis (n=18).Twenty one patients underwent surgical treatment and 6underwent endoscopic treatment; 8 patients receivedmore than one invasive treatment...

Raquitismo vitamina D dependiente tipo I

Resumen La enfermedad ósea que se expresa como raquitismo deriva de diferentes desórdenes, desde la carencia de vitamina D, síndromes malabsortivos, enfermedad renal crónica, displasia metafisaria, hipofosfatasia y raquitismos resistentes, dentro de los cuales están los hipofosfatémicos y aquellos vitamina D dependientes. Presentamos una paciente de 2 años y 6 meses, con síntomas y signos de un raquitismo activo, hipocalcemia severa asintomática, normofosforemia, aumento de la fosfatasa alcalina y de la hormona paratiroidea y con niveles normales de 25 hidroxi vitamina D en sangre. Se diagnosticó como un probable raquitismo vitamina D dependiente tipo I dada la respuesta satisfactoria y rápida con calcio y 0,5 microgramos/día de calcitriol por vía oral.

Summary Rickets is a disease which disturbs normal bone formation through different methods, like vitamin D deficiency, malabsorption, chronic renal disease, metaphisary dysplasia, low phosphorus and resistant rickets. A two and a half year old patient with active rickets who had asymptomatic severe hypocalcemia, normal phosphorus level, high alkaline phosphatase and high parathyroid hormone and normal serum 25- hydroxycholecalciferol. Probable vitamin D dependence type I rickets was diagnosed due to her rapid recovery with the treatment given.

Pseudohipoaldosteronismo transitorio secundario a pielonefritis con reflujo vesicoureteral severo en un lactante

Resumen El pseudohipoaldosteronismo (PHA) secundario transitorio aparece como consecuencia de la resistencia del túbulo renal a la acción de la aldosterona en niños con infecciones del tránsito urinario y/o uropatía obstructiva. Presentamos el caso clínico de un lactante de 4 meses, que se presenta con un segundo episodio de deshidratación, hiperkalemia, hiponatremia y acidosis metabólica habiéndose descartado una hiperplasia suprarrenal congénita mediante valores plasmáticos de ACTH, 17-OH progesterona y cortisol normales. El urocultivo confirmó una infección del tracto urinario (ITU) a enterobacter, y los estudios anatómicos mostraron una ureterohidronefrosis izquierda con reflujo vesicoureteral (RVU) grado V. El centellograma con DMSA mostró un riñón izquierdo hipoplásico, con aporte de 13% a la funcionalidad renal total. El PHA secundario fue confirmado con niveles de renina y aldosterona plasmática elevados. Se realizó tratamiento médico-quirúrgico, con nefrectomía izquierda cursando con buena evolución clínica y normalización de los exámenes de laboratorio.

Summary Secondary transitory pseudohypoaldosteronism (PHA) appears as a consequence of a tubular renal resistance to aldosterone in children with urinary infections and/or obstructive uropathy. The story of a 4 month-old child who had its second episode of dehydration, hyperkalemia, hyponatremia and metabolic acidosis without congenital suprarenal hyperplasia with normal ACTH, 17-hydroxiprogesterone and cortisol is presented. Urine culture showed Enterobacter and the studies revealed a grade V reflux. The DMSA renal scan showed a hypoplasic left kidney with an overall function of 13%. The secondary PHA was confirmed with elevated levels of renin and aldosterone. Medical and surgical treatment was realized with left nephrectomy with good evolution afterwards and normalization of lab studies.

Dermatomiositis juvenil.: A propósito de un caso con grave compromiso multisistémico

Se presenta un caso de dermatomiositis juvenil de presentación y evolución inhabitual en un paciente de 14 años de edad, que ingresa por una historia de 20 días de evolución caracterizada por manifestaciones cutáneas y musculares, constatándose al examen físico eritema en heliotropo, pápulas de Gottron y debilidad muscular proximal y simétrica. Las enzimas musculares elevadas y el electromiograma característico completaron el diagnóstico. Se destacó el grave compromiso respiratorio que obligó a la asistencia respiratoria mecánica, así como el toque multisistémico neurológico, digestivo y hematológico. La inmunosupresión enérgica con corticoides y ciclofosfamida intravenosa, y el uso de altas dosis de inmunoglobulina intravenosa permitieron una evolución favorable manteniéndose en remisión luego de un año y medio con corticoides a baja dosis y metrotexate.

A fourteen year old boy with an unusual presentation of juvenile dermatomyositis (JDM) is described. The illness was characterized by cutaneous manifestations like heliotrope eyelids, Gottron papules and proximal muscle weakness. Elevated serum levels of muscle-derived enzymes and the characteristic electromyogram completed the diagnosis of JDM. Respiratory assistance was needed due to the severe respiratory failure. Multisystemic failure involving the central nervous system, gastrointestinal tract and hematopoyetic system occurred. Aggressive immunosupression with corticosteroids, ciclophosphamide and intravenous immunoglobulin leaded to a favorable outcome. The disease has been in remission for 18 months with low doses of corticosteroids and methotrexate.

Infección urinaria en niños internados: características clínicas, bacteriológicas e imagenológicas

La infección urinaria es la enfermedad nefrológica que más frecuentemente motiva internación en niños. El objetivo de este estudio es realizar un análisis de características clínicas, bacteriológicas e imagenológicas en niños internados por esta enfermedad en un Servicio de Pediatría. En el período setiembre de 2001-mayo de 2002 ingresaron 60 niños de 0 a 14 años con diagnóstico de infección urinaria. 85% fueron menores de dos años, con predominio en el sexo femenino (65%). La fiebre constituyó el síntoma más frecuente, las manifestaciones digestivas fueron las segundas en jerarquía, el síndrome cistítico se observó en 10% de los casos. Escherichia coli constituyó el germen predominante: 81,7%, Klebsiella 10%, Proteus 3,3%, Enterobacter 1,7%. Se constató baja sensibilidad de estos gérmenes a ampicilina y cefalotina, intermedia para TMP-SMX y alta para cefuroxime. La ecografía renal fue patológica en 19,3% de los casos, con una baja sensibilidad para detección de anomalías parenquimatosas demostradas por estudio centellográfico: 12,5%, así como de reflujo vesicoureteral: 36%. El estudio con 99Tc DMSA mostró 65% de resultados patológicos. La asociación de fiebre, leucocitosis >15.000 elementos/mm3 y proteína C positiva fue más frecuente en casos de DMSA positivos: 44% frente a los casos de DMSA negativos: 12%. En dos pacientes con resultados dudosos del urocultivo, este estudio permitió realizar el diagnóstico de pielonefritis aguda. La uretocistografía retrógrada demostró reflujo vésico-ureteral en 40,7% de los pacientes. El alto porcentaje de niños en los que no se logró la realización de este estudio (55%), alerta acerca del subdiagnóstico de esta patología y conduce a plantear diferentes estrategias para su estudio.

A IU é uma doença nefrológica que com mais freqüência motiva internação em crianças. O objetivo deste estudo é realizar uma análise de características clínicas, bacteriológicas e imagenológicas em crianças internadas devido a esta patologia num Serviço de Pediatria. No período 09/2001-05/2002 ingressaram 60 crianças de 0-14 anos com diagnóstico de IU. O 85% foram crianças como menos de 2 anos com predomínio no sexo feminino > 65%. A febre constitui o sintoma mais freqüente, as manifestações digestivas foram as segundas, a síndroma cistítico observou-se em 10% dos casos. Escheríchia coli constitui um germe predominante: 81,7%, Klebsiella Proteus 3,3%, Enterobacter 1,7%. Constatou-se baixa sensibilidade destes germes à ampicilina e cefalotina, intermédia para TMP- SMX e alta para cerufoxime. A ecografia renal foi patológica em 19,3% dos casos com a baixa sensibilidade para detectar anomalias parenquimatosas demostradas por estudo centellográfico: 12,5%, assim como de refluxo vesicoureteral: 36%. O estudo com 99Tc DMSA mostrou 65% de resultados patológicos. A associação de febre, leucocitosis > 15.000 e proteína C positiva foi mais freqüente em casos de DMSA positivos: 44% em comparação aos casos de DMSA negativos: 12%. Em dois pacientes com resultados duvidosos do urocultivo este estudo permitiu realizar o diagnóstico de pielonefritis aguda. A uretocisografía retrograda demostrou RVU em 40,7% dos pacientes. A alta percentagem de crianças nas que não se logrou a realização deste estudo: 55%, alerta referente ao subdiagnóstico desta patologia e conduz a estudar diferentes estratégias para seu estudo.

Urinary tract infection (UTI) is the nephrologic disease that most commonly determines hospital admission in children. The purpose of this prospective study is to analize the clinical, bacteriologic and imagenologic caratheristics of children with this disease in a Pediatric hospital setting. Sixty children were admitted with the diagnosis of UTI in the period 9/2001- 5/2002. 85% were less than 2 years old; 65% were females. Fever was the most frecuent symptom, gastrointestinal manifestations were second in importance, cystitis syndrome was observed in 10% of the patients. Escherichia coli was the predominant etiologic agent: 81,7%, Klebsiella in 10%, Proteus 3,3%, Enterobacter 1,7%. It was observed low sensitivity of these bacterias to ampicillin and cefalotine, intermediate to trimetroprim-sulfametoxasol and high sensitivity to cefuroxime. The renal ecography was abnormal in 19,3% with a low sensitivity to detect parenchymal anomalies observed in DMSA scintigraphy: 12,5% as well as vesicoureteral reflux(VUR): 36%. DMSA renal scans demonstrated pathological results in 65% of the patients. The association of fever, leukocytosis >15.000/mm3 and positive C reactive protein was more frecuent with positive DMSA scans (44%) than in cases of negative DMSA (12%). In two patients with equivocal urine cultures DMSA scan allowed the diagnosis of acute pyelonephritis. The voiding cystourethrography showed VUR in 40,7% of the patients. This study couldn’t be performed in a high number of children (55%) because of lack of compliance. In consequence, this important pathology must be underdiagnosed and obliges to plan different strategies to detect it.