Proteinuria y síndrome nefrótico / Proteinuria and Nephrotic Syndrome

Introducción: El síndrome nefrótico es una patología que afecta el complejo glomerular del riñón, se caracteriza por una proteinuria mayor 3500 mg/d. De acuerdo a la respuesta de los esteroides se puede clasificar en síndrome nefrótico en esteroide resistente o esteroide sensible. Objetivo: Determinar la relación que existe entre la proteinuria y las variantes del síndrome nefrótico en adultos. Métodos: Se realizó un estudio descriptivo, retrospectivo, tipo serie de casos, con una población de 28 pacientes. Se recolectaron y se procesaron los datos a través del software Epi-Info 7,2TM; la frecuencia simple, la media estadística, prueba t de Student, y el coeficiente de correlación de Pearson. Resultados: En el análisis combinatorio de los fármacos adyuvantes para síndrome nefrótico, el grupo que utilizó antiproteinúricos pero no estatinas, demostró una diferencia estadísticamente significativa entre la proteinuria postratamiento media del grupo de síndrome nefrótico esteroideo resistente (6202 mg/d) vs síndrome nefrótico esteroideo sensible (65,9 mg/d) (valor de p 0,418). Existe una correlación negativa entre los niveles proteinuria postratamiento y el nivel de albúmina sérica postratamiento (r = - 0,7 valor de p < 0,00001). Conclusiones: Se demostró la ausencia de asociación entre la proteinuria inicial y las variantes de síndrome nefrótico esteroide sensible y esteroide resistente (valor de p = 0,8)(AU)

Introduction: Nephrotic syndrome is a pathology that affects the glomerular complex of the kidney, characterized by proteinuria greater than 3500 mg/d. According to the response to steroids, nephrotic syndrome can be classified as steroid-resistant or steroid-sensitive. Objective: To determine the relationship between proteinuria and the variants of the nephrotic syndrome in adults. Methods: A descriptive, retrospective, case series type study was carried out with a population of 28 patients. The data was collected and processed through Epi-Info 7.2TM software; simple frequency, statistical mean, student's t-test, and Pearson's correlation coefficient. Results: The statistically significant difference was obtained in the antiproteinuric and non-statin group, between the mean post-treatment proteinuria of the steroid resistant nephrotic syndrome group (6202 mg/d) in comparison to steroid sensitive nephrotic syndrome (65.9 mg/d) (p value 0.0418). There is negative correlation between post-treatment proteinuria levels and post-treatment serum albumin level (r= -0.7 p value <0.00001). Conclusions: The absence of association between initial proteinuria and steroid-sensitive and steroid-resistant variants of nephrotic syndrome was demonstrated (p value=0.8)(AU)

Drug-induced nephrotoxicity

SUMMARY Acute kidney injury is a very common diagnosis, present in up to 60% of critical patients, and its third main cause is drug toxicity. Nephrotoxicity can be defined as any renal injury caused directly or indirectly by medications, with acute renal failure, tubulopathies, and glomerulopathies as common clinical presentations. Some examples of drugs commonly associated with the acute reduction of glomerular filtration rate are anti-inflammatories, antibiotics, such as vancomycin and aminoglycosides, and chemotherapeutic agents, such as cisplatin and methotrexate. Cases of tubulopathy are very common with amphotericin B, polymyxins, and tenofovir, and cases of glomerulopathies are common with VEGF inhibitors, bisphosphonates, and immunotherapy, and it is also common to have more than one clinical presentation related to a single agent. Early diagnosis is essential for the good evolution of the patient, with a reduction of renal exposure to the toxic agent, which requires knowing the risk factors and biomarkers. General measures such as correcting hydroelectrolytic disorders and hypovolemia, monitoring the serum level, avoiding combinations with the synergy of renal injury, and looking for similar options that are less toxic are the foundations for the treatment of complications that are still common and often preventable.

RESUMO A lesão renal aguda é um diagnóstico muito comum, presente em até 60% dos pacientes críticos, e sua terceira maior causa é a toxicidade de medicamentos. A nefrotoxicidade pode ser definida como qualquer lesão renal causada por medicamentos, direta ou indiretamente, tendo a insuficiência renal aguda, tubulopatias e glomerulopatias como apresentações clínicas comuns. Alguns exemplos de drogas comumente associadas à redução aguda da taxa de filtração glomerular são anti-inflamatórios, antibióticos, como a vancomicina e aminoglicosídeos, e agentes quimioterápicos, tais como cisplatina e metotrexato. Casos de tubulopatia são muito comuns com anfotericina B, polimixinas e tenofovir, já casos de glomerulopatias são comuns com inibidores de VEGF, bisfosfonatos e imunoterapia; também é comum ocorrer mais de uma apresentação clínica relacionada a um único agente. O diagnóstico precoce é essencial para a boa evolução do paciente, com a redução da exposição ao agente tóxico, o que requer conhecimento dos fatores de risco e biomarcadores. Medidas gerais, tais como a correção de distúrbios hidreletrolíticos e da hipovolemia, o monitoramento do nível sérico, evitar combinações com sinergia de lesão renal e procurar opções semelhantes e menos tóxicas são os alicerces do tratamento de complicações que são comuns e, muitas vezes, evitáveis.

Trastornos glomerulares en la práctica médica / Glomerulopathies in the medical practice

RESUMEN Las glomerulopatías agrupan varias nefropatías con lesiones fundamentalmente del corpúsculo renal y que se expresan principalmente por proteinuria, hematuria, edemas e hipertensión arterial. La presentación clínica varía en dependencia del tipo de enfermedad de que se trate. Constituye la causa más frecuente de enfermedad renal crónica en adultos jóvenes, por lo que su estudio resulta imprescindible sobre todo para el nivel primario de salud. El propósito fue actualizar consideraciones pertinentes sobre la conducta diagnóstica y terapéutica integral ante una glomerulopatía y valorar emisión de recomendaciones al respecto. Se realizó una búsqueda, análisis y síntesis de información a través de Bases de datos ScieLO Cuba, ScieLO regional, Pubmed, Cumed, Clinical Key en el período 2012-2017 con las palabras clave: síndrome nefrótico, glomerulonefritis, diagnóstico, terapéutica, atención integral. El abordaje en las glomerulopatías es integral, multidisciplinario e individualizado. En Cuba constituyen la cuarta causa de enfermedad renal crónica y predomina el síndrome nefrítico agudo postinfeccioso. El método clínico juega en ello un papel trascendental a la hora de reconocer y registrar sus aspectos clínicos, su etiología, su fisiopatología, y los exámenes complementarios que confirman su presencia o sus complicaciones, así como un tratamiento oportuno que garanticen el perfeccionamiento asistencial. El arma más poderosa ante el reto de los trastornos glomerulares es la visión integradora y con enfoque individual y social protagonizado por el médico ante este grupo de nefropatías en adultos.

ABSTRACT Glomerulopathies encompass a group of several renal disorders with lesions, mainly in the renal corpuscle, expressed in proteinuria, hematuria, edemas and arterial hypertension. Their clinical manifestations change in dependence of the kind of disease. They are the most frequent cause of chronic renal disease in young adults; therefore their study is very important above all in the health care primary level. The aim was updating pertinent considerations on the diagnostic behavior and comprehensive therapy in the case of glomerulopathy, and evaluating the emission of recommendations regarding to them. A search, analysis and synthesis of information was carried out in the databases ScieLO Cuba, ScieLO regional, Pubmed, Cumed, and Clinical Key in the period 2012-2017, using the key words nephrotic syndrome, glomerulonephritis, diagnosis, therapeutics, comprehensive care. The approach to glomerulopathies is comprehensive, multidisciplinary and individualized. They are the fourth cause of chronic renal disease; the acute post-infectious nephritic syndrome predominates. The clinical method plays a transcendental role at the moment of recognizing and registering their clinical characteristics, etiology and physiopathology, while complementary tests confirm their presence or complications, and therefore an opportune treatment guarantying the healthcare improvement. The most powerful weapon against the challenge of the glomerular disorders is the integrated vision with an individual and social approach led by the physician in the case of these nephropathies in adults.

Tratamiento estomatológico del paciente con síndrome nefrótico congénito e hipoplasia generalizada del esmalte: informe de un caso / Stomatological treatment of the patient with congenital nephrotic syndrome and generalized enamel hypoplasia: report of a case

El síndrome nefrótico es la glomerulopatía que se presenta con mayor frecuencia a nivel mundial. La historia natural de la enfermedad, laevolución, la histología renal y la respuesta al tratamiento, va desde la remisión hasta el trasplante renal. Los pacientes con nefropatía presentan alteraciones estomatológicas propias de la enfermedad y secundariasal tratamiento. Los niños con enfermedad renal crónica presentan doscondiciones orales importantes: alta incidencia de anomalías dentarias(hipoplasia del esmalte, retraso de erupción, calcificaciones pulpares) ybaja actividad de caries. Objetivo: Describir la técnica de restauración dental a base de ionómero mediante un caso clínico de un paciente con nefropatía e hipoplasia del esmalte. Conclusión: El uso de ionómero devidrio como obturación semipermanente en pacientes con hipoplasia del esmalte es una eficaz alternativa de tratamiento cuando no se pueden explotar opciones como sistemas adhesivos, coronas de acero cromo o coronas para dientes permanentes.

Nephrotic syndrome is the glomerulopathy which occur mostfrequently in the world. The natural history of disease, evolution, renalhistology and response to treatment, ranging from referral to renaltransplantation. Patients with kidney disease have own stomatology alterations and secondary alterations related to treatment. Children with chronic renal failure have two oral conditions of interest: high incidence of dental anomalies (enamel hypoplasia, delayed eruption,pulp calcifications) and low caries activity. Objective: To describe the technique ionomer dental restoration by a clinical case of a patientwith nephropathy and enamel hypoplasia. Conclusion: The use of glass ionomer as semi-shutter in patients with enamel hypoplasia isan effective alternative of treatment when cannot be exploited options such as adhesive systems, steel crowns or crowns for permanent teeth.

Marcadores biológicos para necrose tubular aguda em pacientes com doença glomerular / Biological markers for acute tubular necrosis in patients with glomerular disease

INTRODUCTION: Acute kidney injury (AKI) is a common complication in patients with nephrotic syndrome (NS), and it is reported in 34% of adults with idiopathic nephrotic syndrome. Emergence of AKI in the course of nephrotic syndrome requires a prompt differential diagnosis between acute tubular necrosis (ATN) and proliferative glomerular lesions leading to rapidly progressive glomerulonephritis. Although clinical and conventional laboratory clues can be decisive in many cases, sometimes such distinctions rely on renal biopsy, which is an invasive procedure and is not available in many centers. Several new biomarkers have emerged, increasing the perspective on early diagnosis and the prognostic prediction of AKI. OBJECTIVES: In this work, we studied the use of tests based on the urinary concentrations of kidney injury molecule-1 (KIM-1)...

INTRODUÇÃO: A lesão renal aguda (LRA) é uma complicação frequente em pacientes com glomerulopatias, acomentendo até 34% dos adultos com síndrome nefrótica (SNO) idiopática. O diagnóstico diferencial de necrose tubular aguda (NTA) de glomeulonefrite proliferativa ou crescêntica em pacientes com SNO e LRA é fundamental, visto que a NTA pode mimetizar quadro de glomerulonefrite rapidamente progressiva. Dados clínicos e laboratoriais podem ser úteis no diagnóstico diferencial da LRA na SNO, entretanto a distinção entre NTA e glomerulonefrite proliferativa ou crescêntica é feito pela biópsia renal, procedimento invasivo e que não está disponível amplamente. Novos biomarcadores para diagnóstico precoce e preditores diagnósticos na LRA têm sido identificados. OBJETIVOS: Neste trabalho nós avaliamos o uso de testes baseados nas concentrações urinárias de kidney injury molecule-1 (KIM-1)...

Semiología renal y genitourinaria en pediatría: segunda parte / Nephrologoy and urology semiology in children: second part

Entre las patologías renales en niños, se encuentra la injuria renal aguda; que es la pérdida súbita de la función renal; el síndrome nefrótico que es el espectro más grave de proteinuria; el síndrome nefrítico caracterizado por la riada edema, hematuria macro o microscópica e hipertensión arterial.

Among the renal pathology in children is acute kidney injury, which is the sudden loss of kidney function, the nephrotic syndrome is the most severe spectrum of proteinuria, the nephrotic syndrome characterized by the triad of edema, macro or microscopic hematuria and arterial hypertension.

Síndrome Nefrótico, un estudio de 18 años: Matanzas / Nephrotic Syndrome, an 18-years study: Matanzas

Se destaca un aumento significativo de la prevalencia de enfermedades renales crónicas en los países de América Latina, como indicador de la nueva epidemiología y del impacto de la tecnología en su determinismo. El síndrome nefrótico idiopático justifica alrededor del 90 por ciento de los casos de nefrosis en la infancia. Con el objetivo de conocer las características clínicas y epidemiológicas en nuestra provincia, se realizó un estudio aplicado, descriptivo y retroprospectivo sobre el comportamiento del Síndrome Nefrótico Primario, donde fueron estudiados 57 pacientes desde enero de 1990 a diciembre del 2008. Se caracterizaron los casos de acuerdo a las siguientes variables demográficas: edad de debut, sexo, se identificó en primario o secundario, se clasificó a los pacientes según su respuesta al tratamiento corticoesteroideo y se conoció la evolución y complicaciones más frecuentes. Los datos obtenidos fueron procesados utilizando métodos estadísticos (tasa de incidencia, números absolutos y por ciento). Se obtuvieron los siguientes resultados: La incidencia encontrada fue 3,6 por 100,000; el 56,1por ciento debutó entre los 3-5 años; 61,4 por ciento del sexo masculino; 92por ciento es de tipo primario; 59,6 por ciento son corticosensibles al inicio del tratamiento; las infecciones respiratorias fue la complicación más frecuente y la principal causa de recaída. Entre los pacientes biopsiados la Glomérulo Esclerosis Segmentaria Focal (GNFS) ocupó el 12,3 por ciento, seguida de Glomérido Nefritis Mesangial Difusa con 8,8 por ciento; el 78,9 por ciento se comportó como Síndrome Nefrótico Primario con Lesiones Mínimas. Estos resultados son comparables con otros reportes, donde se plantea una incidencia de 2 por 100000 niños menores de 16 años en Norteamérica.

Latin American countries show a significant increase of chronic kidney diseases prevalence, as indicator of the new epidemiology and then impact of technology in its determinism. The Idiopathic Nephrotic Syndrome justifies around 90 per cent of nephrosis cases in childhood. With the objective of knowing the clinical and epidemiological characteristics in our province, an applied, descriptive and retrospective study has been carried out on the behaviour of the Primary Nephrotic Syndrome, where 57 patients had been studied since January 1990 to December 2008. The cases were characterized according to the following demographic variables: onset age, gender; primary or secondary stages of the disease were identified; the patients were classified according to their response to the corticosteroid treatment and the evolution and more frequent complications were known. Obtained data were processed using statistic methods (incidence rate, absolute numbers and percentage). The following results were obtained: the incidence was 3,6 per 100 000; 56,1 per cent started between 3 and 5 years old; 61.4 per cent were males; 92 per cent is at the primary stage; 59,6 per cent are corticosensitive at the beginning of the treatment; respiratory infections were the most frequent complications and the main cause of relapse. Among the patients treated with biopsy GNFS (General nephrotic syndrome) occupied 12,3 per cent, followed by the Mesangial Diffuse Glomerulonephritis with 8,8 per cent; 78,9 per cent behaved as Primary Nephrotic Syndrome with Minimal Lesions. These results are comparable with other results, where an incidence of 2 per 100 000 children under 16 years old is stated in North America.

Mesangioproliferative glomerulonephritis: an important glomerulonephritis in nephrotic syndrome of young adult.

Mesangioproliferative glomerulonephritis (MesPGN) consists 10% of the total renal biopsy of glomerulonephritis. Aim of the present study was to find out clinicopathological changes in MesPGN and differences between diffuse and focal variety. MesPGN was seen mostly in young adults with mean age of 28.63 years for males and 26.3 years for females. Male predominance was noted (M:F ratio - 1.4:1). About 70.83% patient presented with edema feet, followed by hypertension (29.19%), fever (16.66%), oliguria, nausea and vomiting (10.41%). Urine analysis in 50 patients revealed that 70% patients presented with nephrotic-range proteinuria, 36% patients with microscopic hematuria and 56% patients with leukocyturia. Statistically, no significant difference was found in clinical features of diffuse and focal MesPGN. Microscopic comparison between diffuse and focal variety showed that significant increase of focal glomerular basement membrane thickening, focal endothelial cell proliferation, focal smooth muscle hyperplasia, hyaline sclerosis and vasculitis was more common in diffuse variety. In focal variety, Capillary loop congestion, periglomerulitis, cloudy swelling and vacuolar degeneration in tubules were significantly more as compared to diffuse variety. Details of the clinical features, special laboratory tests and histological details revealed that diffuse variety had systemic diseases, which included Wegner's granulomatosis, microscopic polyangitis, Henoch's schonlein purpura, systemic lupus erythematosus (two cases) and one case each of Kimura's disease, pyelonephritis and tuberculosis. Only one case of focal MesPGN showed tuberculosis. Thus, our study concludes that MesPGN is an important cause of nephrotic syndrome among young adults. Secondly, search for some other diseases should be made and thirdly, if biopsy shows focal mesangial cell proliferations in minimal change glomerulonephritis (MCGN), it should be diagnosed as focal MesPGN rather than MCGN because these cases show recurrences.

NPHS2 mutations.

OBJECTIVE: To uncover the frequency and the spectrum of NPHS2 mutations in Egyptian children with non familial steroid-resistant nephrotic syndrome (SRNS). METHODS: Sixteen patients were screened by PCR-single-strand conformation polymorphism analysis of NPHS2 gene followed by direct sequencing. RESULTS: NPHS2 mutations were evident in four patients (25%) who were bearing four novel mutations including two frame shift mutations (R238fs and P45fs) and two missense mutations (I136L and F216Y). There were no phenotypic or histological characteristics of patients bearing NPHS2 mutations, apart from the earlier onset of the disease, compared to those who were not bearing mutations. CONCLUSION: NPHS2 mutations are prevalent in Egyptian children with non-familial SRNS and this may in part explain the less favorable prognosis reported in these patients.

Perfil clínico-epidemiológico de glomerulopatia membranosa primária em brasileiros (71 casos) / Clinical-epidemiological profile of primary membranous glomerulopathy in Brazilian patients (71 cases)

Introdução: A glomerulopatia membranosa (GNM) é uma das causas mais comuns de síndrome nefrótica em adultos, podendo ser idiopática ousecundária. Inicialmente, a maioria dos pacientes apresenta função renal preservada. O curso clínico é variável, contemplando um espectro que vai desde a remissão espontânea da proteinúria até a progressão para insuficiência renal. Objetivo: O objetivo deste estudo foi traçar o perfil clínico-epidemiológico dos pacientes portadores de GNM primária no nosso Serviço e comparar nossos dados com o de levantamentos similares. Pacientes e Métodos: Foram avaliados, retrospectivamente, dados clínicos, epidemiológicos, laboratoriais e histopatológicos de 71 pacientes portadores de GNM primária, acompanhados no Ambulatório de Nefrites da UNIFESP-EPM, no período de 1976 a 2006. Resultados: Foram registrados 71 pacientes (43% do total)com a forma primária da doença, seguidos por pelo menos seis meses. Todos eram adultos, com idade mediana de 43 anos, predominantemente brancos e homens. Creatinina sérica e proteinúria medianas iniciais eram de, respectivamente, 1,1mg/dl e 6,4g/24h e as finais foram de 1,1mg/dl e 1,2g/24h. Hipertensão Arterial Sistêmica (HAS) e hematúria microscópica estiveram presentes em, respectivamente, 50% e 77,5% dos pacientes. A sobrevida livrede insuficiência renal em dez anos foi de 74,3%. Conclusões: No nosso serviço, as características clínicas, epidemiológicas, laboratoriais e morfológicas não diferiram, na sua grande maioria, de estudos semelhantes em todo o mundo, exceto por uma freqüência mais elevada de hematúria microscópica e HAS. Ao final de 30 anos, a probabilidade de apresentar algum grau de insuficiência renal foi de 28%.

Introduction: Membranous glomerulopathy is one of the most frequent causes of nephrotic syndrome in adults. It can be idiopathic or secondary. Initially the majority of the patients has normal renal function. The clinical course is variable, i.e., it includes since spontaneous remission of proteinuria to chronic renal failure. Objective: The aim of this study was to determine the clinical and epidemiological profile of the patients with membranous glomerulopathy of our service and to compare these results to data from similar surveys. Patients and Methods: We evaluated retrospectively clinical, epidemiological, laboratorial and histological data from 71 patients with primary membranous glomerulopathy, followed up in the Glomerulopathies Section of the NephrologyService (UNIFESP-EPM), from 1976 to 2006. Results: Considering all cases of membranous glomerulopathy, 71 (43%) had the primary form of the disease and they were followed up for at least six months. All of them were adults, predominantly white males, with median age of 43 years. The median serum creatinine and proteinuria at presentation of disease were 1.1 mg/dl and 6.4 g/24h and at the end of follow-up were 1.1 mg/dl and 1.2 g/24h, respectively. Hypertension and microscopic hematuria were seen in 50 and 75% of the patients, respectively. Renal survival was 74.3% by 10 years of follow-up. Conclusions: In our service, the clinical, epidemiological, laboratorial and morphological features of membranous glomerulopathy were not different from those of other similar studies in the world, except for a higher frequency of hypertension and microscopic hematuria. At presentation patients had normal renal function and at the end of 30 years the renal survival was 28%.

Características clínicas e histopatológicas del síndrome nefrótico primario / Clinical and histopathological characteristics of primary nephrotic syndrome

Introducción: La presentación del síndrome nefrótico con proteinuria masiva, hipoalbuminemia, presencia de anasarca y el manejo con altas dosis de esteroides obliga al clínico a reconocerlo oportunamente, manejarlo interdisciplinariamente y tener la habilidad para predecir el curso y pronóstico de la enfermedad después del manejo inicial. Objetivos: Conocer el comportamiento del síndrome nefrótico primario en niños de Cali y área de influencia, sus características demográficas, la presentación clínica, respuesta a tratamiento y clasificación histopatológica y recomendaciones sobre indicación de biopsia renal. Material y métodos: Se revisaron las historias clínicas de 264 niños con síndrome nefrótico entre enero 1986 y julio 2002 con edades entre 1 y 15 años. Resultados: El promedio de edad al diagnóstico fue 4 años y 60/100 de los pacientes eran del sexo masculino. Todos presentaron edema y proteinuria, 97/100 hipoalbuminemia, 94/100 hipercolesterolemia y 54/100 oliguria; 89/100 fueron corticosensibles y sólo 11/100 corticorresistentes. En 65 pacientes con recaídas frecuentes que recibieron citotóxicos se logró remisión de la enfermedad en 40/100. Se realizó biopsia renal en 73 (28/100); en 29/100 la indicación fue por resistencia a esteroides y en 71/100 por recaídas frecuentes o corticodependencia. Los hallazgos histopatológicos mostraron cambios glomerulares mínimos en 36/100, proliferación mesangial en 46/100, esclerosis focal y segmentaria en 11/100 y otras enfermedades 7/100. Conclusiones: El porcentaje de casos resistentes a esteroides y la presencia de esclerosis segmentaria y focal son más bajos que los descritos en otras series. En niños con racaídas frecuentes o corticodependientes se recomienda intentar un ciclo con citotóxicos antes de programar biopsia renal, esta se debe reservar para los casos corticorresistentes. Sólo 40/100 del total son controlados en forma regular por el Servicio de Nefropediatría siendo necesario mejorar el sistema de seguimiento para evaluación de la función renal a largo plazo

Comportamiento clínico de los pacientes con síndrome nefrótico corticoresistente tratados con ciclofosfamida en el servicio de nefrología del Hospital Infantil Manuel de jesús Rivera / Patients clinical behavior with syndrome nefrótico corticoresistente tried with ciclofosfamida in the service of nefrology of the Infantile Hospital Manuel de Jesús Rivera

El presente estudio tuvo como objetivo determinar el comportamiento clínico y evolución de los pacientes con síndrome nefrótico esteride resistente tratados con ciclosfosfamida. Siendo un estudio de cohorte retrospectivo, constituido por 27 pacientes elegibles y que cumplieron los criterios para ser estudiados. El total de pacientes estudiados recibieron tratamiento inicialmente con corticoesteroides, presentando en su mayoría múltiples recaídas en el transcurso de su tratamiento, estableciéndose el diagnóstico de corticoresistencia al persistir la proteinuria después de 8 semanas en rangos no fisiológicos. El mayor porcentaje se encontró en edades comprendidas entre 11 a 15 años de edad, siendo el sexo predominante el femenino. Encontramos que únicamente 17 de los pacientes se pudo efectuar biosia renal y establecer la variedad histológica, siendo en el 35 porciento de la variedad de cambios mínimos y lesiones glomerulares focales, ambas comparten una clínica recurrente y respuesta variable a los esteroides...

Infections in chlidren with nephrotic syndrome

To determine the different types of infections in nephrotic children and their association with the activity of disease. Design: A descriptive study. Place and Duration of Study: It was carried out at National Institute of Child Health [NICH], Karachi from August 1995-July 1996. Patients and All children with nephrotic syndrome who had various infections during the study period were evaluated for the activity of disease and type of infections. Out of 62 children with nephrotic syndrome having infections, 45 [72.58%] were boys and 17 [27.42%] girls in a ratio of 2.5:1. Out of them 53 [85.45%] were known nephrotics and 36 [58%] of them were on steroid therapy. Nine were new cases and had not received steroids. A total of 74 episodes of infections were observed in 62 children. Acute respiratory infections [ARI] and skin infections were the most common [29.27% and 27.02% respectively] followed by diarrhoea [13.51%], UTI [12.5%] and peritonitis [10.81%]. Two patients had pulmonary tuberculosis and 3 patients had more than one infection [cellulitis, peritonitis and pneumonia]. All children with cellulitis and peritonitis were in active disease, whereas more than 80% of patients with ARI, diarrhoea and UTI were having either relapse or had recent onset of disease. The study showed that ARI, cellulitis and diarrhoea were most common infections followed by UTI and peritonitis. Majority of the infections [>78%] were associated with active disease

Síndrome nefrótico en infantes y adolescentes: revisión bibliográfica / Nefrótico syndrome in infants and adolescents: bibliographical overhaul

Presenta una revisión bibliográfica, describe el síndrome nefrótico durante la infancia y la adolescencia desde su definición, pasando por su clasificación y ciertas consideraciones generales, detallando su etiología, fisio e histopatología, consideraremos sus manifestaciones clínicas, finalizaremos con el esquema terapéutico y su pronóstico.

Está desaparecendo a glomerulopatia da esquistossomose mansônica? / Is glomerulopathy due to schistosomiasis mansoni disappearing?

As formas severas de esquistossomose mansônica, particularmente a hépato-esplênica, se acompanham de glomerulopatia geralmente manifesta por síndrome nefrótica. Nos últimos 10 anos reduziu-se muito o número de casos observados desta glomerulopatia no Hospital Universitário Prof. Edgard Santos, um hospital geral num estado onde esta parasitose é endêmica. O objetivo deste estudo foi verificar se estava havendo, de fato, desaparecimento desta patologia ou se este número decrescente refletia apenas diminuição marcante de casos graves de esquistossomose mansônica acompanhados neste Hospital. Para isso foram comparadas as prevalências de glomerulopatia em hepatosplênicos esquistossomóticos autopsiados no Hospital Universitário Prof. Edgard Santos em duas décadas: de 1960-70, antes da intervenção terapêutica nas áreas endêmicas de esquistossomose com oxamniquine, e de 1980-1990, após a adoção desta intervenção. Houve grande redução no número de doentes com hepatosplenomegalia esquistossomótica autopsiados no Hospital Universitário Prof. Edgard Santos quando comparadas a década antes (140 autópsias) e depois (31 autópsias) do tratamento com oxamniquine. A prevalência de glomerulopatia, entretanto, persistiu a mesma (11,4% comparada com 12,9%, respectivamente). A conclusão é que a redução do achado de glomerulopatia esquistossomótica em nossos dias reflete apenas a redução das formas graves da parasitose após a intervenção terapêutica nas áreas endêmicas.

Hepatosplenic form of S. mansoni infection may be accompanied by a glomerulopathy in 12-15% of cases, manifested in the majority by a nephrotic. This type of renal involvement is becoming a rare occurrence in our University Hospital (Hospital Universitário Prof. Edgard Santos) a typical general hospital in an endemic state for this parasitic disease. To investigate this fact, autopsied cases with patients with hepatosplenic form of schistosomiasis mansoni during two decades in our Hospital-1960-70, (before a therapeutic intervention in endemic areas with oxamniquine) and 1980-1990 (after the intervention) were compared in reference to number of cases and the finding of glomerulonephritis by histological examination. Even though there was a striking decrease in number of patients with advanced forms of this disease (140 as compared to 31 autopsies in these two decades), the prevalence of glomerulonephritis diagnosed was 11.4 (16 cases) in the first and 12.9 (4 cases) in the second. As there was no change in pattern of attendance in this Hospital, the drastic decrease in number of severe forms of this parasitic infection following massive therapy of the endemic population with oxamniquine is the most likely explanation not only for the decrease in number of hepatosplenic cases but, also, and as a consequence, the scarcity of cases of the schistosomal glomerulopathy observed.

Nephrotic syndrome associated with hepatointestinal schistosomiasis

Schistosomal nephropathy has long been related to the hepatosplenic form of schistosomiasis. In the last few years, 24 patients with hepatointestinal schistosomiasis and the nephrotic syndrome were studied. Aiming at evaluating a possible etiologic participation of schistosomiasis in the development of the nephropathy, this group was comparatively studied with a group of 37 patients with idiopathic nephrotic syndrome. Both groups had a different distribution of the histologic lesions. In the group with schistosomiasis there was a statistically significant prevalence of proliferative mesangial glomerulonephritis (33.3%), whereas in the control group there was prevalence of membranous glomerulonephritis (32.4%). On immunofluorescence, IgM was positive in 94.4% of the patients with schistosomiasis versus 55.0% in the control group (P < 0.01). In the group with schistosomiasis, 8 patients evidenced mesangial proliferative glomerulonephritis and 5, membranoproliferative glomerulonephritis. In both histological types immunofluorescence showed IgM and C3 granular deposits in the glomeruli. The data in this study suggests that mesangial proliferative and membranoproliferative glomerulonephritis, with glomerular granular IgM and C3 deposits, represent the renal lesions of the schistosomiasis associated nephropathy

Síndrome nefrótico del lactante / Nephrotic syndrome of the infant

El SNL es raro, con un pronóstico estimado como desfavorable. Es más frecuente entre los 12 y 24 meses de edad. Según alteraciones de la biopsia renal (BR) se describen 5 tipos, que probablemente se relacionan con el pronóstico. Con el propósito de analizar esta patología se revisaron las fichas clínicas de los ingresos por SN al Hospital Roberto del Río en un período de 192 meses. De 66 niños con SN el 27,3% (n = 18) fueron lactantes, y de ellos el 16,7% (n = 3) y el 83,3% (n = 15) eran menores de 12 y 24 meses respectivamente. No se encontró registro de ancestro finlandés (AF) ni nefropatías familiares (NF) y 1/18 pacientes fue prematuro. Se hizo BR en 10 niños: de éstos 4 tenían lesión glomerular mínima (LGM), 2 SN finlandés (SNF), 2 glomeruloesclerosis segmentaria y focal (GESF) y 2 glomerulonefritis mesangioproliferativa (GNMP). Respecto a la respuesta al tratamiento esteroidal, 8 (44,4%) fueron corticosensibles (CS), 6 (33,3%) corticodependientes (CD) y 4 (22,2%) corticoresistentes (CR). En la evolución, con un seguimiento individual variable (1 a 16 años), se constató: remisión completa en 7 (38,9%), insuficiencia renal crónica en 1 (BR = GNMP) y no habría fallecimientos. Concluimos que el pronóstico parece menos favorable: hay remisiones completas y persistentes. Por tanto se requiere de un estudio que incluya BR para un diagnóstico histopatológico, que facilite la indicación de las terapias en uso, previniendo así complicaciones infecciosas y otras condicionantes de pronóstico definitivo