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A amiloidose renal familiar é uma doença incomum em cães, que afeta os rins e está associada ao acúmulo anormal de proteínas amiloides, com capacidade de promover danos orgânicos progressivos com comprometimento de funcionalidade. Caracterizada pela presença de conteúdo proteináceo glomerular, a amiloidose frequentemente está associada a quadros de falência renal, com presença de sinais clínicos variados, sendo uma condição grave e complexa. O presente artigo tem como objetivo descrever os achados clínico-laboratoriais, de imagem e histopatológicos de amiloidose familiar em dois cães da raça Shar-pei. Os animais apresentavam parentesco direto e evidenciavam sinais de cansaço, prostração e emagrecimento progressivo. As evidências clínico-laboratoriais e ultrassonográficas sugeriram a presença de glomerulonefropatia, sendo essa confirmada por exame histopatológico. Os dois cães, diante da gravidade do quadro, foram a óbito. A análise histopatológica evidenciou deposição de material proteináceo fibrilar na região glomerular e tubular, bem como infiltrado linfoplasmocítico, característicos de amiloidose renal. É essencial lembrar que a amiloidose renal familiar em cães é uma doença complexa e que as origens devem ser investigadas. O tratamento é desafiador, diante da inexistência de um manejo terapêutico definido para a doença, sendo este muitas vezes ineficaz. A empatia e o cuidado no manejo dessa condição podem ajudar a melhorar a qualidade de vida do paciente e fornecer conforto ao proprietário durante esse processo desafiador.

Family renal amyloidosis is an uncommon disease in dogs, which affects the kidneys and is associated with abnormal accumulation of amyloid proteins, capable of promoting progressive organic damage with impairment of functionality. Characterized by the presence of glomerular proteinaceous content, amyloidosis is often associated with renal failure, with the presence of varied clinical signs, being a serious and complex condition. This article aims to describe the clinical, laboratory, imaging and histopathological findings of familial amyloidosis in two Shar-pei dogs. The animals were directly related and evidenced signs of tiredness, prostration and progressive weight loss. Clinical, laboratory and ultrasonographic evidence suggested the presence of glomerulonephropathy, which was confirmed by histopathological examination. The two dogs, given the severity of the condition, died. Histopathological analysis showed deposition of fibrillar proteinaceous material in the glomerular and tubular region, as well as lymphoplasmocytic infiltrate, characteristic of renal amyloidosis. It is essential to remember that family renal amyloidosis in dogs is a complex disease and that the origins must be investigated. The treatment is challenging, given the lack of a defined therapeutic management for the disease, which is often ineffective. Empathy and care in managing this condition can help improve the patient's quality of life and provide comfort to the owner during this challenging process.

La amiloidosis renal familiar es una enfermedad poco común en perros, que afecta a los riñones y se asocia con la acumulación anormal de proteínas amiloides, con capacidad de promover daño orgánico progresivo con compromiso de la funcionalidad. Caracterizada por la presencia de contenido proteico glomerular, la amiloidosis suele asociarse a insuficiencia renal, con la presencia de signos clínicos variados, siendo una afección grave y compleja. El presente artículo tiene como objetivo describir los hallazgos clínico-laboratorios, imagenológicos e histopatológicos de la amiloidosis familiar en dos perros Sharpei. Los animales estaban directamente emparentados y presentaban signos de cansancio, postración y pérdida progresiva de peso. Los datos clínico-laboratorios y ecográficos sugirieron la presencia de glomerulonefropatía, la cual fue confirmada mediante examen histopatológico. Los dos perros, dada la gravedad del cuadro, fallecieron. El análisis histopatológico mostró depósito de material proteico fibrilar en la región glomerular y tubular, así como infiltrado linfoplasmocitario, característico de la amiloidosis renal. Es fundamental recordar que la amiloidosis renal familiar en perros es una enfermedad compleja y que es necesario investigar sus orígenes. El tratamiento es un desafío, dada la falta de un manejo terapéutico definido para la enfermedad, que muchas veces resulta ineficaz. La empatía y el cuidado en el manejo de esta afección pueden ayudar a mejorar la calidad de vida del paciente y brindar comodidad al propietario durante este desafiante proceso.

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RESUMEN Introducción: En México, la enfermedad renal crónica (ERC) representa un gran problema de salud y las glomerulopatías (GP) representan la tercera causa de ERC. Objetivo: Describir, desde una base de datos de biopsias renales (BR) nativas, los diferentes patrones morfológicos de GP en México. Métodos: Se analizaron registros de BR de riñón nativo en un centro de referencia en nefropatología, todas las BR fueron evaluadas por una única nefropatóloga (AVP). El diagnóstico final en cada caso se basó en parámetros clínicos e histopatológicos. Resultados: Fueron revisadas 2084 BR, con edad de 34.4 ± 17.6 años. 1085 BR (52.1%) en género femenino; el síndrome nefrótico fue más frecuente en hombres (p<0.001) y síndrome nefrítico fue más frecuente en mujeres (p<0.001). GP primarias y nefropatías túbulo-intersticiales fueron más diagnosticadas en hombres (p<0.01). Nefritis lúpica (NL) fue la GP secundaria más reportada. La glomeruloesclerosis focal y segmentaria (GEFS) fue la GP primaria diagnosticada con mayor frecuencia en ambos géneros. Vasculitis por inmunoglobulina A fue la enfermedad vascular más frecuentemente detectada. Síndrome nefrótico fue la indicación más frecuente de BR (42.9%), seguido de: síndrome nefrítico (23.9%), proteinuria aislada (16.4%), daño renal agudo (8.7%), alteraciones urinarias asintomáticas (6.2%) y ERC (1.8%). Conclusiones: La GP primarias con mayor frecuencia fueron GEFS. Las GP secundarias más frecuentemente reportadas fueron NL, predominantemente en mujeres. Se observó nefropatía IgA con mayor frecuencia en comparación con otras series publicadas en México. Hubo diferencias significativas en la presentación de GP en relación con el género y la edad del paciente.

ABSTRACT Introduction: In Mexico, chronic kidney disease (CKD) represents a major health problem, and glomerulopathies (GP) represent the third leading cause of CKD. Aim: From a database of native kidney biopsies (KB), describe the different morphological patterns of GP in Mexico. Methods: Records of native KB in a nephropathology referral center were evaluated by a single nephropathologist. The final diagnosis in each case was based on clinical parameters and histopathological findings. Results: 2084 KB were analyzed, patients were 34.4±17.6 years of age, there were 1085 KB (52.1%) in females; nephrotic syndrome was most frequent in males (p<0.001), and nephritic syndrome was more frequent in females (p <0.001). Primary GP and túbulo-interstitial diseases were most diagnosed in males (p <0.01). Lupus nephritis (LN) was the most-reported secondary GP. Focal and segmental glomerulosclerosis (FSGS) was the primary GP most often diagnosed in both genders. The most frequently detected vascular disease was immunoglobulin A vasculitis. Nephrotic syndrome was the most frequent indication for KB (42.9%), followed by: nephritic syndrome (23.9%), isolated proteinuria (16.4%), acute kidney injury (8.7%), asymptomatic urinary alterations (6.2%), and CKD (1.8%). Conclusions: The most frequently observed primary GP was FSGS, and LN was the most frequent secondary GP, predominantly in females, and IgA nephropathy was observed more frequently in comparison with other series published in Mexico. There were significant differences in GP presentation in relation to patient sex and age.

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It was evaluated the different methodologies for glomerular enumeration described in the literature in a quantitative, double-blind study on the number of glomeruli present in kidney biopsy specimens obtained using a Tru-cut-type biopsy needle. Eighteen samples were taken (n=18) from various regions of canine kidneys harvested immediately after euthanasia. Sample collection was carried out using Tru-cut-type, semi-automated, 16 gauge needles. Reading and evaluation of the specimens were performed independently by four researchers by means of eye loupe inspection, light microscopy, light microscopy with a reduced condenser aperture, and dissection microscopy. Samples were also subjected to histopathological evaluation for comparison purposes. There was no inter-researcher variation for any of the tested methods. The glomerular counts obtained using light microscopy with reduced condenser aperture were similar to those reported in the histopathological evaluation (P=0.44);whereas, the other glomerular enumeration methods yielded significantly lower counts when compared to the histopathological analysis (eye loupe inspection: P<0.01; conventional light microscopy: P=0.02; dissection microscopy: P<0.01).Light microscope with lowering of the condenser lens delivers better results than conventional light microscopy and eye loupe inspection. Results obtained using dissection microscopy and eye loupe inspection did not correlate well with those provided by histopathological evaluation; consequently, these two methods should be avoided.(AU)

Avaliou-se as diferentes metodologias de quantificação glomerular descritas na literatura em um estudo quantitativo, duplo-cego, sobre o número de glomérulos presentes em amostras de biópsias renais obtidas com agulha de biópsia tipo Tru-cut. Dezoito amostras foram coletadas (n=18) de várias regiões de rins caninos colhidos imediatamente após a eutanásia. A coleta das amostras foi realizada com agulhas semi-automáticas de calibre 16, tipo Tru-cut. A leitura e avaliação das amostras foram realizadas de forma independente por quatro pesquisadores por meio de inspeção visual, microscopia de luz, microscopia de luz com redução da lente condensadora e microscopia de dissecção. As amostras também foram submetidas à avaliação histopatológica para fins de comparação e variação do pesquisador para qualquer um dos métodos testados. As contagens glomerulares obtidas por microscopia de luz com redução da lente condensadora foram semelhantes àquelas encontradas na avaliação histopatológica (P=0.44), enquanto que os outros métodos de quantificação glomerular produziram contagens significativamente mais baixas a análise histopatológica (inspeção da lupa dos olhos: P<0.01; microscopia de luz convencional: P=0.02; microscopia dissecação: P<0.01). Microscópio de luz com abaixamento da lente condensadora fornece melhores resultados do que microscopia de luz convencional e inspeção da lupa. As contagens obtidas usando microscopia de dissecção e inspeção de lupa de olho não se correlacionaram bem com aqueles fornecidos pela avaliação histopatológica; consequentemente, estes dois métodos devem ser evitados.(AU)

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ABSTRACT: It was evaluated the different methodologies for glomerular enumeration described in the literature in a quantitative, double-blind study on the number of glomeruli present in kidney biopsy specimens obtained using a Tru-cut-type biopsy needle. Eighteen samples were taken (n=18) from various regions of canine kidneys harvested immediately after euthanasia. Sample collection was carried out using Tru-cut-type, semi-automated, 16 gauge needles. Reading and evaluation of the specimens were performed independently by four researchers by means of eye loupe inspection, light microscopy, light microscopy with a reduced condenser aperture, and dissection microscopy. Samples were also subjected to histopathological evaluation for comparison purposes. There was no inter-researcher variation for any of the tested methods. The glomerular counts obtained using light microscopy with reduced condenser aperture were similar to those reported in the histopathological evaluation (P=0.44);whereas, the other glomerular enumeration methods yielded significantly lower counts when compared to the histopathological analysis (eye loupe inspection: P<0.01; conventional light microscopy: P=0.02; dissection microscopy: P<0.01).Light microscope with lowering of the condenser lens delivers better results than conventional light microscopy and eye loupe inspection. Results obtained using dissection microscopy and eye loupe inspection did not correlate well with those provided by histopathological evaluation; consequently, these two methods should be avoided.

RESUMO: Avaliou-se as diferentes metodologias de quantificação glomerular descritas na literatura em um estudo quantitativo, duplo-cego, sobre o número de glomérulos presentes em amostras de biópsias renais obtidas com agulha de biópsia tipo Tru-cut. Dezoito amostras foram coletadas (n=18) de várias regiões de rins caninos colhidos imediatamente após a eutanásia. A coleta das amostras foi realizada com agulhas semi-automáticas de calibre 16, tipo Tru-cut. A leitura e avaliação das amostras foram realizadas de forma independente por quatro pesquisadores por meio de inspeção visual, microscopia de luz, microscopia de luz com redução da lente condensadora e microscopia de dissecção. As amostras também foram submetidas à avaliação histopatológica para fins de comparação e variação do pesquisador para qualquer um dos métodos testados. As contagens glomerulares obtidas por microscopia de luz com redução da lente condensadora foram semelhantes àquelas encontradas na avaliação histopatológica (P=0.44), enquanto que os outros métodos de quantificação glomerular produziram contagens significativamente mais baixas a análise histopatológica (inspeção da lupa dos olhos: P<0.01; microscopia de luz convencional: P=0.02; microscopia dissecação: P<0.01). Microscópio de luz com abaixamento da lente condensadora fornece melhores resultados do que microscopia de luz convencional e inspeção da lupa. As contagens obtidas usando microscopia de dissecção e inspeção de lupa de olho não se correlacionaram bem com aqueles fornecidos pela avaliação histopatológica; consequentemente, estes dois métodos devem ser evitados.

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Abstract Introduction: Rheumatoid arthritis is one of the most common clinical syndromes within rheumatological conditions and its association with glomerular diseases is rare. Objective: To describe the histopathological findings in renal biopsies in patients with rheumatoid arthritis and to correlate them with the clinical and laboratory manifestations at the beginning, at 6 months and at one year of follow-up. Patients and Methods: This is a retrospective observational study conducted in the Hospital de Clinicas "Jose De San Martin" in Buenos Aires, Argentina; Where we included 41 patients diagnosed with RA (ACR 1987) in a period of 20 years. Histopathological diagnoses of membranous nephropathy (MN), minimal change disease (MCD), secondary amyloidosis (AA), focal and segmental glomerulosclerosis (FSGS); mesangial glomerulopathy (MGP) and glomerulonephritis with extracapillary proliferation (GNEC) were included. Histopathological description, different treatments, years of evolution of rheumatoid arthritis Clinical and laboratory characteristics were analyzed during the first 6 months and one year of follow-up in order to determine the progression of renal failure calculated through the formula of MDRD of 4 variables (Modification of diet in renal disease) and the increase of proteinuria. Results: The most frequent histological finding was amyloidosis with 34,1 % (n=14), followed by mesangial glomerulopathy 21,9 % (n=9), membranous nephropathy 19,5 % (n=8), glomerulonephritis with extracapillary proliferation 12,1 % (n=5), focal and segmental glomerulosclerosis 7,3 % (n=3) and minimal change disease 8,2 % (n=2). Nephrotic syndrome was the most frequent presentation in patients with amyloidosis in 85,7 %, microhematuria occurred in 100 % of patients with MPG and in 80 % of patients with GNEC. In patients with AA, moderate to severe interstitial fibrosis occurred in 85,7 %, followed by GNEC and NM with 80 % and 40 % respectively. The 24-hour proteinuria, creatinine and glomerular filtration rate estimated by MDRD at 6 months and 12 months were evaluated. Concluding, that patients with AA, FSGS and GNEC had greater progression of renal failure at 12 months; the opposite occurred in patients with minimal change disease (MCD) and mesangial glomerulopathy (MGP) who had a lower progression of renal failure at one year of follow-up; There was a correlation in the glomerulopathies that had greater deterioration of the renal function had greater interstitial tubule involvement as was the case of amyloidosis. The glomerulopathies that presented greater proteinuria at the beginning were membranous nephropathy, amyloidosis and minimal change disease. Both membranous nephropathy and minimal change disease had partial remission at one year, in contrast to amyloidosis, which showed progression of proteinuria at 12 months of follow-up. Conclusion: The glomerulopathies that presented greater progression of renal failure at 1 year based on the estimation by MDRD 4, had a higher renal tubular interstitial involvement in renal biopsy and these were amyloidosis (AA), segmental focal glomerulosclerosis (FSGS), glomerulonephritis with proliferation extracapillary On the other hand, those with the best evolution in relation to the degree of proteinuria and the glomerular filtration rate determined by the MDRD4 equation were mesangial glomerulopathy, minimal change disease, and membranous nephropathy.

Resumen Introducción: La artritis reumatoidea (AR) es uno de los síndromes clínicos con mayor frecuencia dentro de las afecciones reumatológicas y su asociación con las enfermedades glomerulares es poco frecuente. Objetivo: Describir los hallazgos histopatológicos en las biopsias renales en pacientes con artritis reumatoidea y correlacionarlos con las manifestaciones clínicas y de laboratorio al inicio, a los 6 meses y al año de seguimiento. Pacientes y métodos: Es un estudio observacional retrospectivo realizado en un hospital Universitario en Buenos Aires, Argentina. Se incluyeron 41 pacientes con diagnóstico de artritis reumatoidea de acuerdo a los criterios establecidos por el Colegio Americano de Reumatología publicados en 1987; en un período de 20 años. Se incluyeron diagnósticos histopatológicos de nefropatía membranosa (NM), enfermedad de cambios mínimos (ECM), amiloidosis secundaria (AA), gloméruloesclerosis focal y segmentaria (GEFS); glomerulopatía mesangial (GPM) y glomerulonefritis con proliferación extracapilar (GNEC). Las características clínicas, de laboratorios, la descripción histopatológica, los años de evolución de la artritis reumatoidea y los diferentes tratamientos fueron analizados durante los primeros 6 meses y al año del seguimiento. Con esto, se buscó determinar la progresión de la insuficiencia renal, calculada a través de la fórmula de MDRD (Modification of Diet in Renal Disease) de 4 variables y el aumento de la proteinuria. Resultados: El hallazgo histológico más frecuente fue la amiloidosis, con un 34.1 % (n=14), seguido de la glomerulopatía mesangial (21,9 %, n=9), la nefropatía membranosa (19,5 %, n=8), la glomerulonefritis con proliferación extracapilar (12,1 %, n=5), la glomeruloesclerosis focal y segmentaria (7,3 %, n=3) y enfermedad de cambios mínimos (8,2 %, n=2). El síndrome nefrótico fue la forma de presentación más frecuente en los pacientes con amiloidosis (en un 85,7 % de los casos), la microhematuria se presentó en el 100 % de los pacientes con GPM y en el 80 % de los pacientes con GNEC. En el 85,7 % de los pacientes con AA, se presentó fibrosis intersticial moderada a severa, mientras que en la GNEC y la NM la fibrosis se observó en un 80 % y 40 % respectivamente. Se evaluó la proteinuria de 24 horas, la creatinina y la filtración glomerular estimada por MDRD a los 6 y a los 12 meses. Se concluyó que los pacientes con AA, GEFS y GNEC presentaron mayor progresión de la insuficiencia renal a los 12 meses. Lo contrario sucedió en los pacientes con enfermedad de cambios mínimos (ECM) y glomerulopatía mesangial (GPM), los cuales tenían una menor progresión de la insuficiencia renal al año de seguimiento. Hubo una correlación entre las glomerulopatías que tenían mayor deterioro de la función renal en las cuales se observó a su vez, mayor compromiso tubulointersti-cial, (este fue el caso de la amiloidosis). Las glomerulopatías que presentaban mayor proteinuria al inicio eran la nefropatía membranosa, la amiloidosis y la enfermedad de cambios mínimos. Tanto la nefropatía membranosa como la enfermedad de cambios mínimos, tenía remisión parcial tras un año, a diferencia de la amiloidosis, la cual presentaba progresión de la proteinuria a los 12 meses de seguimiento. Conclusión: Las glomerulopatías que presentaron mayor progresión de la insuficiencia renal al año, con base en la estimación por MDRD4, tenían en la biopsia renal mayor compromiso tubulointersticial. Estas fueron la amiloidosis secundaria, la glomeruloesclerosis focal y segmentaria, y glomerulonefritis con proliferación extracapilar. Por el contrario, las de mejor evolución respecto al grado de proteinuria y tasa de filtrado glomerular determinado por MDRD4, fueron la glomerulopatía mesangial, la enfermedad de cambios mínimos y la nefropatía membranosa.

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INTRODUCCIÓN: El sedimento de orina es una herramienta en la práctica clínica empleada desde hace muchos años para la evaluación de enfermedades renales. La detección de hematuria dismórfica es útil en el diagnóstico de enfermedades glomerulares. OBJETIVOS: Agrupar las hematurias dismórficas en los casos con presencia de acantocitos y los que no los presentan, y correlacionar estos dos grupos con los hallazgos histológicos de las biopsias renales. MATERIAL Y MÉTODOS: Estudio observacional, retrospectivo y analítico. Se incluyeron los sedimentos de orina de 276 pacientes. Se analizaron dos grupos de hematuria dismórfica: D1 (presencia de acantocitos) y D2 (sin acantocitos), y se correlacionó con los hallazgos histológicos de la biopsia renal (glomerulopatías proliferativas y no proliferativas). Se analizaron los diferentes elementos formes de la orina (cilindros hemáticos, leucocitarios, céreos, granulosos, grasos), la creatinina plasmática y la proteinuria de 24 hs en los dos grupos de glomerulopatías. Posteriormente se realizó una regresión logística para evaluar las variables independientes entre los hallazgos del sedimento de orina, con los correspondientes odds ratio (OR) e intervalos de confianza (IC 95%). RESULTADOS: Se contó con 172 muestras provenientes de mujeres (62.3%) y 104 de hombres (37,7%). La presencia de acantocitos (D1) en las enfermedades glomerulares proliferativas (GP) fue 17 veces más frecuente comparada con las no proliferativas (GNP) OR 17.7 IC 95% (9.6-32.5) p 0.001. La presencia de cilindros hemáticos es ocho veces más frecuente en las GP OR 8 IC 95% (3.1-20.9). Los pacientes con hematuria no acantocitica (D2) es 5 veces más frecuente en una GNP OR 5.2 IC (2.4-11.3) p 0,001. La presencia de cilindros grasos fue más frecuente en los pacientes con GNP a diferencia de los cilindros leucocitarios, cuya frecuencia fue mayor en la GP. CONCLUSIONES: La presencia de hematuria dismórfica no acantocitica (D2) se correlacionó en la histología renal con la presencia de glomerulopatías no proliferativas (GNP) en forma significativa, a diferencia de la hematuria acantocitica y cilindros hemáticos que se observaron en glomerulopatías proliferativas, por lo tanto se considera una herramienta útil para poder diferenciar clínicamente estos dos grupos, sin remplazar la biopsia renal para el diagnóstico preciso y el pronóstico

INTRODUCTION: The analysis of urine sediment is a tool that has been used for many years in clinical practice to evaluate kidney diseases. Detecting dysmorphic red blood cells (RBC's) in urine is useful for the diagnosis of glomerular diseases. OBJECTIVES: To divide the cases of glomerular hematuria into two groups, depending on the presence or absence of acanthocytes, and to compare this factor with the histological findings of renal biopsies. METHODS: In this observational, retrospective, analytical study, urine sediments of 276 patients were included. Two groups of subjects with glomerular hematuria were analyzed: D1 (presence of acanthocytes) and D2 (absence of acanthocytes). The results were compared with the renal biopsy histological findings, i.e. proliferative glomerulonephritis and non-proliferative glomerulonephritis, considered separately. The formed elements of the urine (red blood cell, white blood cell, waxy, granular and fatty casts), plasma creatinine concentration and 24-hour urinary protein were tested in the two groups. A logistic regression analysis was later performed to assess the independent variables among urine sediment findings, with the corresponding odds ratio (OR) and confidence intervals (CI 95%). RESULTS: The samples were collected from 172 women (62.3 %) and 104 men (37.7 %). The presence of acanthocytes (D1) was 17 times more frequent in proliferative glomerulonephritis (PGN) than in non-proliferative glomerulonephritis (NPGN) [OR 17.7, CI 95% (9.6-32.5), p 0.001]. The presence of red blood cell casts was 8 times more frequent in PGN [OR 8, CI 95% (3.1-20.9)]. Cases of hematuria with no acanthocytes (D2) were 5 times more frequent in NPGN [OR 5.2, CI (2.4-11.3), p 0.001]. Fatty casts appeared more frequently in patients with NPGN, whereas white blood cell casts were more common in PGN cases. CONCLUSIONS: Renal histological findings revealed a significant correlation between glomerular hematuria without acanthocytes (D2) and non-proliferative glomerulonephritis (NPGN), while the presence of acanthocytes and red blood cell casts was associated with proliferative glomerulonephritis (PGN). The existence of acanthocytes in urine constitutes a useful tool to make a clinical distinction between these two conditions, but it does not replace renal biopsy to establish an accurate diagnosis and prognosis

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En las glomerulopatías, las Microproteínas Urinarias (MU) se eliminan por distintos mecanismos fisiopatológicos. El objetivo del trabajo fue correlacionar las MU con el daño histológico evaluado en la punción biopsia renal (PBR) de pacientes con diversas glomerulopatías. Se estudiaron 44 orinas espontáneas (33 mujeres y 11 varones) entre 18 y 71 años de edad, por el método de electroforesis bidimensional de uso clínico (2D UC). Las proteínas identificadas se dividieron en 5 grupos y se compararon con lesiones vasculares, glomerulares y túbulointersticiales; estas dos últimas se dividieron en crónicas y agudas. Las del grupo identificado como las "Tres Marías" (fragmento de 35 kDa de la proteína inhibidor de tripsina cadena pesada H4, Prostaglandina H2 sintasa y fragmento de 23 kDa del Perlecan) resultaron no ser marcadoras de daño tubular, sino de alteraciones glomerulares crónicas. La presencia de las mismas, con proteinuria, se observa antes de la caída de la filtración glomerular (<60 mL/min) y correlaciona con el 30% de glomérulos totalmente esclerosados (p<0,001). El grupo Triángulo, en la glomerulopatía, contiene a la Alfa-1 microglobulina (A1m) y a las cadenas livianas libres de Inmunoglobulinas (CLL), e indica lesión glomerular activa. Por lo tanto, las MU en glomerulopatías, responden a lesiones glomerulares activas y crónicas. Los perfiles proteicos urinarios hallados por la 2D UC permitieron conocer el grado de lesión en los distintos compartimentos renales.

In Glomerulopathies, urinary microproteins (UM) are eliminated by different pathophysiological mechanisms. The objective of this work was to correlate the UM with the histological damage evaluated in the renal biopsy through puncture of patients with various glomerular diseases. Forty-four urine samples (33 females, 11 males) aged 18 to 71 years old were studied by the method of two-dimensional electrophoresis of clinical use (2D UC). The identified proteins were divided into 5 groups and were compared with vascular injury, glomerular and tubulointerstitial injury, the latter in chronic and acute cases. The group identified as the "Three Marias" (fragment 35 kDa protein trypsin inhibitor heavy chain H4, prostaglandin H2 synthase and 23 kDa fragment of Perlecan), was not found as marker of tubular damage, but it was found in chronic glomerular disorders. The presence of this same group -with proteinuria- is seen before the collapse of the glomerular filtration rate (<60 mL/min) and it is correlated with 30% of fully sclerotic glomeruli (p<0.001). In glomerulopathy, the Triangle group: Alpha-1 microglobulin (A1m) and free Immunoglobulin light chains (FLC), indicates active glomerular injury. Therefore, the UM in glomerular diseases, respond to active and chronic glomerular lesions. Urinary protein profiles found by the 2D UC made it possible to know the degree of renal injury in different renal compartments.

Nas glomerulopatias, as Micro proteínas Urinárias (MU) são eliminadas através de diferentes mecanismos fisiológicos. O objetivo do trabalho foi relacionar as MU com o dano histológico avaliado na punção biópsia renal (PBR) de pacientes com diversas glomerulopatias. Foram estudadas 44 urinas espontâneas (33 mulheres e 11 homens entre 18 e 71 anos de idade), pelo método de eletroforese bidimensional de uso clínico (2D UC). As proteínas identificadas foram divididas em 5 grupos e comparadas com lesões vasculares, glomerulares e túbulo-intersticiais, estas duas últimas classificadas em crônicas e agudas. O grupo identificado como as "Três Marias" (fragmento de 35 kDa da proteína inibidora de tripsina cadeia pesada H4, Prostaglandina H2 sintase e fragmento de 23 kDa do Perlecam), resultaram não ser marcadoras de dano tubular, mas de alterações glomerulares crônicas. A presença de tais proteínas, com proteinúria, observa-se antes da queda da filtragem glomerular (<60 mL/min) e correlaciona com 30% de glomérulos totalmente esclerosados (p<0,001). O grupo Triângulo, na glomerulopatia, que contém a Alfa-1 microglobulina (A1m) e as cadeias leves livres de Imunoglobulinas (CLL) indicam lesão glomerular ativa. Portanto, as MU em glomerulopatias respondem a lesões glomerulares ativas e crônicas. Os perfis proteicos urinários encontrados pela 2D UC permitiram conhecer o grau de lesão nos diferentes compartimentos renais.

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INTRODUCCIÓN: la biopsia renal percutánea constituye un elemento clave en el manejo de la enfermedad renal crónica, además de tener gran valor en la evaluación de la disfunción renal aguda y en el establecimiento de pronósticos. OBJETIVOS: conocer las indicaciones de la biopsia renal percutánea de riñones propios, en el Instituto de Nefrología entre 1988 y el 2007; las complicaciones relacionadas, los diagnósticos y su relación con la forma de presentación de la enfermedad renal a través de estudio descriptivo y transversal. MÉTODOS: los datos primarios para esta investigación fueron recolectados de la boleta de biopsia. Todas las biopsias renales durante los años de estudio fueron realizadas, siguiendo el protocolo del Departamento de Anatomía Patológica. La información fue procesada automatizada; paquete estadístico SPSS 22.0. Se utilizó análisis de distribución de frecuencias, promedio y desviación estándar en las variables cuantitativas y el test de independencia para identificar posibles relaciones entre variables. RESULTADOS: la edad media de los pacientes fue de 35,9 años. El 49,6 % eran hombres. Presentaron complicaciones 59 pacientes (3,9 %), fundamentalmente sangramientos. El síndrome nefrótico (47,0 %) y el síndrome nefrítico (11,2 %) fueron las indicaciones más comunes. Predominaron las enfermedades glomerulares (91,3 %) y dentro de estas las glomerulopatías primarias (75,3 %). Los diagnósticos más comunes fueron la glomeruloesclerosis segmentaria y focal (20,6 %) y la glomerulopatía proliferativa mesangial (16,8 %). CONCLUSIONES: la indicación más común de biopsia renal percutánea, es el síndrome nefrótico y la complicación más frecuente, el sangramiento. Los diagnósticos más usualmente realizados son: glomerulesclerosis segmentaria y focal, glomerulopatía proliferativa mesangial y nefritis lúpica.

INTRODUCTION: percutaneous renal biopsy is a key element in the management of the chronic kidney disease in addition to having great value in the evaluation of acute kidney dysfunction and in making prognoses. OBJECTIVE: to identify the indications for percutaneos renal biopsy of native kidney in the Nephrology Institute from 1988 to 2007; the related complications, the diagnoses and their relationship with the form of presentation of the renal disease through a descriptive cross-sectional study. METHODS: primary data for this research work were gathered from the biopsy form. All the renal biopsies performed during the study years followed the Pathological Anatomy Department protocol. The statistical package SPSS 22.0 was used for data processing. Frequency distribution analysis, averages and standard deviation were used in the quantitative variables whereas the independence test served to identify possible associations of variables. RESULTS: the average age of patients was 35.9 years. In the group, 49.6 % were men. Fifty nine patients presented (3.9 %) presented with complications, mainly bleeding. Nephrotic syndrome (47 %) and nephritic syndrome (11.2 %) were the most common indications for biopsy. Glomerular diseases (91.3 %) predominated and primary glomerulopathies prevailed among them (75.3 %). The most common diagnoses were focal and segmental glomerulosclerosis (20.6 %) and mesangial proliferative glomerulopathy (16.8 %). CONCLUSIONS: the most common indication of percutaneous renal biopsy is the nephrotic syndrome whereas the most frequent complication is bleeding. The usual diagnoses were focal and segmental glomerulosclerosis, mesangial proliferative glomerulopathy and lupus nephritis.

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Introducción: la biopsia renal percutánea constituye un elemento clave en el manejo de la enfermedad renal crónica, además de tener gran valor en la evaluación de la disfunción renal aguda y en el establecimiento de pronósticos. Objetivos: conocer las indicaciones de la biopsia renal percutánea de riñones propios, en el Instituto de Nefrología entre 1988 y el 2007; las complicaciones relacionadas, los diagnósticos y su relación con la forma de presentación de la enfermedad renal a través de estudio descriptivo y transversal. Métodos: los datos primarios para esta investigación fueron recolectados de la boleta de biopsia. Todas las biopsias renales durante los años de estudio fueron realizadas, siguiendo el protocolo del Departamento de Anatomía Patológica. La información fue procesada automatizada; paquete estadístico SPSS 22.0. Se utilizó análisis de distribución de frecuencias, promedio y desviación estándar en las variables cuantitativas y el test de independencia para identificar posibles relaciones entre variables. Resultados: la edad media de los pacientes fue de 35,9 años. El 49,6 por ciento eran hombres. Presentaron complicaciones 59 pacientes (3,9 por ciento), fundamentalmente sangramientos. El síndrome nefrótico (47,0 por ciento) y el síndrome nefrítico (11,2 por ciento) fueron las indicaciones más comunes. Predominaron las enfermedades glomerulares (91,3 por ciento) y dentro de estas las glomerulopatías primarias (75,3 por ciento). Los diagnósticos más comunes fueron la glomeruloesclerosis segmentaria y focal (20,6 por ciento) y la glomerulopatía proliferativa mesangial (16,8 por ciento). Conclusiones: la indicación más común de biopsia renal percutánea, es el síndrome nefrótico y la complicación más frecuente, el sangramiento. Los diagnósticos más usualmente realizados son: glomerulesclerosis segmentaria y focal, glomerulopatía proliferativa mesangial y nefritis lúpica(AU)

Introduction: percutaneous renal biopsy is a key element in the management of the chronic kidney disease in addition to having great value in the evaluation of acute kidney dysfunction and in making prognoses. Objective: to identify the indications for percutaneos renal biopsy of native kidney in the Nephrology Institute from 1988 to 2007; the related complications, the diagnoses and their relationship with the form of presentation of the renal disease through a descriptive cross-sectional study. Methods: primary data for this research work were gathered from the biopsy form. All the renal biopsies performed during the study years followed the Pathological Anatomy Department protocol. The statistical package SPSS 22.0 was used for data processing. Frequency distribution analysis, averages and standard deviation were used in the quantitative variables whereas the independence test served to identify possible associations of variables. Results: the average age of patients was 35.9 years. In the group, 49.6 percent were men. Fifty nine patients presented (3.9 percent) presented with complications, mainly bleeding. Nephrotic syndrome (47 percent) and nephritic syndrome (11.2 percent) were the most common indications for biopsy. Glomerular diseases (91.3 percent) predominated and primary glomerulopathies prevailed among them (75.3 percent). The most common diagnoses were focal and segmental glomerulosclerosis (20.6 percent) and mesangial proliferative glomerulopathy (16.8 percent). Conclusions: the most common indication of percutaneous renal biopsy is the nephrotic syndrome whereas the most frequent complication is bleeding. The usual diagnoses were focal and segmental glomerulosclerosis, mesangial proliferative glomerulopathy and lupus nephritis(AU)

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Introducción: La Nefritis lúpica (NL) es una complicación frecuente del Lupus Eritematoso Sistémico en niños y adolescentes; comparado con pacientes adultos, los daños son más severos y la remisión menos frecuente. Objetivo principal: describir aspectos clínicos y epidemiológicos de la NL en pacientes atendidos en el Servicio de Nefrología del Hospital de Niños de Valencia. Métodos: Investigación de tipo cuantitativa, no experimental, descriptiva y retrospectiva. Resultados: Muestra: 21 pacientes, 13 adolescentes (62%), 15 hembras (71%); lesión histopatológica más frecuente: glomerulonefritis Difusa (Clase IV de la clasificación de la OMS); clínica predominante: edema, afecciones de piel y artritis; en la mayoría de los pacientes se evidenciaron alteraciones hematológicas, inmunológicas y del examen de orina. Se observó co-morbilidad asociada en un número importante de casos con afectación más frecuente del sistema nefrourológico y hematológico. Fallecieron 4 pacientes y hubo remisión de la sintomatología, de grado variable, en los otros casos, durante un periodo de seguimiento de 7 meses a 8 años (X:4.2±3.62). Conclusiones: La presente serie de NL muestra que se presenta más frecuentemente en adolescentes, del sexo femenino, con clínica predominantemente en piel y articulaciones; La glomerulonefritis difusa es la forma histológica más frecuente. El tratamiento fue diverso y la sobrevida global fue de 17 pacientes (71 %) durante un periodo de seguimiento de 4.2 años. La NL es una patología compleja en la edad pediátrica y su diagnóstico precoz, clasificación y manejo son importantes para el pronóstico.

Introduction: Lupus nephritis (LN) is a common complication of Systemic Lupus in children and adolescents; in these age groups, kidney damage is more aggressive, symptoms are more severe and remission is lower, compared with what occurs in adults. Methods: The objective was to describe clinical and epidemiological aspects of LN in patients attended at the Pediatric Nephrology Service, Children’s Hospital of Valencia - Venezuela. The investigation was quantitative, not experimental, descriptive and retrospective. Results: 21 patients, 13 adolescents (62%), 15 female (71%). Diffuse Glomerulonephritis (Class IV Lupus Nephritis of WHO classification) was the most frequent lesion; clinical manifestations at presentation were edema, skin lesions and arthritis. Other manifestations: blood disorders, immune and urine alterations on admission. Co-morbidity associated was more frequent in the nephrourological and hematological systems. 4 patients died and remission of symptoms, of variable degree, was seen in the other cases. Conclusions: This series of LN in pediatrics shows that it is more frequent in female adolescents with clinical manifestations in skin and joints. The most frequent lesion is diffuse glomerulonephritis. Treatment was variable and the total survival of patients was 71% (17 patients) during a follow up of approximately 4.2 years. Lupus nephritis is a complex pathology in pediatric ages and its early diagnosis, proper histopathologic classification and adequate therapeutic management are important in terms of quality of life, prognosis and survival of patients.

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Introducción: Los pacientes adolescentes representan un desafío para el nefrólogo. Aunque la patología renal del mismo se asemeja más a la del adulto, tiene una epidemiología única a considerar. Métodos: Estudio descriptivo, retrospectivo. Se relevaron 43 historias clínicas de pacientes entre 10 Y 18 anos ingresados por primera vez en nefrología entre enero de 2009 y mayo de 2011, analizando datos demográficos, motivo de consulta, lugar de referencia, tiempo entre inicio de síntomas y la referencia al servicio, días de internación, diagnóstico de enfermedad renal y causas de ingreso a diálisis. Resultados: Se internaron 394 pacientes, 136 fueron adolescentes. 43 internaciones de primera vez, y de estos, 24 mujeres. Edad promedio 13,5 años ± 2,37. 27 pacientes de provincia de Bs. As., 11 CABA, y 5 resto del País. 27 derivados desde nuestro propio hospital y 16 de otros centros médicos. Los síntomas y signos más frecuentes fueron hematuria, edemas, proteinuria y disminución del filtrado glornerular. La media entre inido de síntomas y la consulta fue 15 días (rango 1-30). Promedio de días de internación 13,7 (rango 2-95). Las patologías prevalentes fueron las glomerulopatias y la nefropatía lúpica la enfermedad renal más frecuente. 11 pacientes requirieron diálisis, y 9 permanentemente. Conclusiones: Los adolescentes presentan prevalencia patológica diferente a la de pediatría. La presencia de IRCT sin diagnóstico previo fue significativa.(AU)

Introduction: Teenager patients represent a challenge for Nephrologists. Although renal pathology of such patients is similar to adults, it has a unique epidemiology which must be considered. Methods: Retrospective, descriptive study. 43 clinical histories of patients between 10 and 18 years old admitted for the first time in Nephrology division between January 2009 and May 2011 were reported. We analyzed demographic data, consultation cause, reference place, time between the beginning of symptoms and the reference to the division, days of hospitalization, renal disease diagnosis and dialysis admission causes. Results: 394 patients were admitted, 136 were teenagers, 43 admissions for the first time and from these 24 women. Average age 13,5 years old +-2,37.27 patients from Buenos Aires province, 11 Capital City of Buenos Aires and 5 from the rest of the country. 27 referred from our own hospital and 16 from other medical centers. The 139 most frequent symptoms and signs were hematuria, edema, proteinuria and decrease of glomerular filtration. The average between the beginning of symptoms and consultation was 15 days (range1-30). Admission day average 13,7(range 2-95). Prevalent pathologies were glomerulopathies and lupic nephropathy the most frequent renal disease. 11 patients required dialysis. Conclusions: Teenagers show different pathological evidence in comparison to pediatrics. The presence of IRCT without previous diagnosis was significant.(AU)

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Introducción: Los pacientes adolescentes representan un desafío para el nefrólogo. Aunque la patología renal del mismo se asemeja más a la del adulto, tiene una epidemiología única a considerar. Métodos: Estudio descriptivo, retrospectivo. Se relevaron 43 historias clínicas de pacientes entre 10 Y 18 anos ingresados por primera vez en nefrología entre enero de 2009 y mayo de 2011, analizando datos demográficos, motivo de consulta, lugar de referencia, tiempo entre inicio de síntomas y la referencia al servicio, días de internación, diagnóstico de enfermedad renal y causas de ingreso a diálisis. Resultados: Se internaron 394 pacientes, 136 fueron adolescentes. 43 internaciones de primera vez, y de estos, 24 mujeres. Edad promedio 13,5 años ± 2,37. 27 pacientes de provincia de Bs. As., 11 CABA, y 5 resto del País. 27 derivados desde nuestro propio hospital y 16 de otros centros médicos. Los síntomas y signos más frecuentes fueron hematuria, edemas, proteinuria y disminución del filtrado glornerular. La media entre inido de síntomas y la consulta fue 15 días (rango 1-30). Promedio de días de internación 13,7 (rango 2-95). Las patologías prevalentes fueron las glomerulopatias y la nefropatía lúpica la enfermedad renal más frecuente. 11 pacientes requirieron diálisis, y 9 permanentemente. Conclusiones: Los adolescentes presentan prevalencia patológica diferente a la de pediatría. La presencia de IRCT sin diagnóstico previo fue significativa.

Introduction: Teenager patients represent a challenge for Nephrologists. Although renal pathology of such patients is similar to adults, it has a unique epidemiology which must be considered. Methods: Retrospective, descriptive study. 43 clinical histories of patients between 10 and 18 years old admitted for the first time in Nephrology division between January 2009 and May 2011 were reported. We analyzed demographic data, consultation cause, reference place, time between the beginning of symptoms and the reference to the division, days of hospitalization, renal disease diagnosis and dialysis admission causes. Results: 394 patients were admitted, 136 were teenagers, 43 admissions for the first time and from these 24 women. Average age 13,5 years old +-2,37.27 patients from Buenos Aires province, 11 Capital City of Buenos Aires and 5 from the rest of the country. 27 referred from our own hospital and 16 from other medical centers. The 139 most frequent symptoms and signs were hematuria, edema, proteinuria and decrease of glomerular filtration. The average between the beginning of symptoms and consultation was 15 days (range1-30). Admission day average 13,7(range 2-95). Prevalent pathologies were glomerulopathies and lupic nephropathy the most frequent renal disease. 11 patients required dialysis. Conclusions: Teenagers show different pathological evidence in comparison to pediatrics. The presence of IRCT without previous diagnosis was significant.

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A prevalência de doenças renais em pacientes encaminhados aos Serviços de Nefrologia em hospitais terciários em Salvador, Brasil, foi avaliada através de exames histopatológicos. Analisamos, retrospectivamente, 228 biópsias renais realizadas entre janeiro de 2003 a junho de 2006. Destes, 159 preencheram os critérios para inclusão no estudo. Foram examinados por microscopia óptica, imunofluorescência (arquivos de imagens digitais) e, quando necessário, por microscopia eletrônica. Compilamos informações sobre gênero, idade, etnicidade, a síndrome clínica e a duração da doença renal. A revisão histológica das biópsias foi realizada em três etapas. Inicialmente, por dois patologistas, simultaneamente, usando um microscópio multiobservador. Em seguida, os diagnósticos foram revistos por um observador independente. Ao final, os casos sem unanimidade no diagnóstico foram revistos pelos três patologistas em conjunto, para se chegar a um diagnóstico consensual. A nefropatia primária mais freqüente foi a esclerose glomerular focal e segmentar, somando 27% dos casos. Outros 15% foram identificados como parte do espectro alteração mínima-esclerose segmentar focal desta doença. Encontramos glomerulopatia membranosa em 9%, glomerulonefrite membranoproliferativa em 7%, e nefropatia por imunoglobulina A em 5%. A nefropatia secundaria mais freqüente foi a nefrite lúpica, constituindo 14% do total. Concordância entre observadores no diagnóstico das nefropatias foi de 93%, com kapa 0,919, DP 0,03 e p<0,01. Este é o primeiro estudo descritivo da prevalência das glomerulopatias em Salvador, Brasil, com utilização de imunofluorescência e microscopia eletrônica. As glomerulopatias primárias e secundárias mais freqüentes foram glomeruloesclerose focal e segmentar e nefrite lúpica. Estes achados representam uma alteração da prevalência das glomerulopatias na Bahia, antes mais influenciada pela infestação por Schistosoma mansoni.

The prevalence of renal diseases in patients referred to tertiary hospitals in Salvador, Brazil was evaluated by histopathological examination. 228 biopsies of native kidneys, performed from January, 2003 through June, 2006, were retrospectively analyzed; 159 of these fulfilled the criteria for inclusion in this study. They were reviewed by light microscopy, immunofluorescence (digital image archives) and, whenever necessary, by electron microscopy. Gender, age ethnicity, duration of the renal disease and clinical syndrome were studied. Histological revision of the biopsies was performed in three rounds: 1st, by two pathologists using a multiobserver microscopy; 2nd, an independent revision by an external examiner and 3rd, the cases given discrepant diagnosis were revised by all the observers working together. Focal and segmental glomerular sclerosis was the most frequent primary nephropathy, encountered in 27% of the cases. Another 15% were identified as part of the minimal change - focal segmental sclerosis spectrum of disease. Membranous glomerulopathy comprised 9%, membranoproliferative glomerulonephritis 7%, and immunoglobulin A nephropathy, 5% of the total. Lupus nephritis was the most common secondary nephropathy, corresponding to 14% of the cases. Interobserver concordance in the diagnosis of nephropathies was 93%, with Kappa 0.919, standard error 0.03 and P < 0.01. This is the first descriptive study of the prevalence of glomerulopathies in renal biopsies in Salvador, Brazil, using all the recourses of immunofluorescence and electron microscopy. Focal and segmental glomerulosclerosis and systemic lupus nephritis were identified as the most frequent primary and secondary glomerulopathies, respectively. This data may represent a shift in the patter of distribution of glomerulopaties in Bahia, formerly influenced by S. mansoni infection.

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O conceito e os critérios clínicos e laboratoriais para o diagnóstico da Síndrome Nefrótica (SN) são revisados neste artigo, assim como a definição de resposta ao seu tratamento. São abordadas as glomerulopatias de causa primariamente renal, particularmente o seu tratamento e prognóstico. Na fase inicial da SN a abordagem está centrada no diagnóstico e tratamento das complicações do estado nefrótico, muitas vezes graves e independentes da etiologia da SN, além da utilização de “medidas gerais” como restrição de sal, uso judicioso de diuréticos e de inibidores da enzima conversora e/ou bloqueadores do receptor da angiotensina. Procede-se o diagnóstico etiológico da doença, se de causa primária ou secundária. Nas glomerulopatias primárias, esse diagnóstico necessariamente dependerá da biópsia renal, a qual definirá qual o protocolo de “tratamento específico” de um ou mais imunossupressores a ser prescrito. Uma proporção significativa de pacientes pode não responder ao tratamento e permanecer com o estado nefrótico, e a decisão por “medidas gerais” e de nefroproteção em geral é a conduta mais adequada, pela baixa probabilidade de resposta e alto potencial de efeitos colaterais dos imunossupressores a longo prazo, como os corticosteróides, agentes citotóxicos e/ou inibidores da calcineurina. Futuramente o uso de drogas mais eficientes e com menos efeitos colaterais poderá ampliar as possibilidades de tratamento específico das glomerulopatias primárias.

Clinical and laboratorial criteria for the definition of Nephrotic Syndrome (NS) are reviewed in this article, as well as characterization of response to its treatment. Primary glomerulopathies are specifically described, particularly their treatment and prognosis. In the initial phase of NS, the approach is mainly focused in the diagnosis and treatment of complications of the nephrotic state, many times severe and independent of the etiology of NS, when general measures such as salt restriction, judicious use of diuretics and angiotensin-enzyme inhibitors and/or angiotensin-receptor blockers are prescribed. Then the etiology of NS is determined, if associated to a primary or a secondary cause. In primary glomerulopathies, this diagnosis relies on renal biopsy that will define a specific immunosuppressive protocol to be prescribed. A significative proportion of these patients could not respond to treatment and remain nephrotic, when to keep general measures and nephroprotection should be the best approach, because immunosuppressors such as corticosteroids, cytotoxic agents and calcineurin inhibitors have a small probability of response and high potential for toxicity in the long term. In the future, more efficient drugs with less side-effects should broaden the options of specific treatments for primary glomerulopathies.

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El análisis de los resultados de casuísticas de biopsias renales es importante con fines diagnósticos, terapéuticos y de pronóstico. Evaluar la serie de biopsias renales en el Hospital de Niños de Valencia, Venezuela, durante el período 1978-2007. Fueron analizadas 421 biopsias renales practicadas en 395 pacientes, de 2 meses a 20 años, 57% varones. El material fue procesado por microscopía óptica, inmunofluorescencia y microscopía electrónica en 98% de los casos. Se obtuvo muestra adecuada (más de 10 glomérulos) en 93% de los casos (n=392). Indicaciones clínicas: síndrome nefrótico 199(50%), síndrome nefrítico atípico 53(13%), otro: (hematuria/proteinuria, proteinuria, trasplante, enfermedades sistémicas 143(37%). Resultados Histopatológicos: A.-Glomerulonefrítis primaria (GNP) 302 casos (77%), B.-Nefropatías secundarias (NS) 68 casos (17%), C.-Riñones trasplantados 28 casos (7%).-Diagnósticos en GNP: 1) Lesión de cambios mínimos 140(46%), 2) Glomeruloesclerosis segmentaria y focal 79 (26%), 3) GN proliferativa y/o mesangial 67(22%), 4) GN Membranosa 16 (5%). -Diagnósticos en NS: Nefritis lúpica: 20 casos (32.25%), Nefropatía IgA: 22 casos (35.50%); Otras: 20 casos (32.25%). -Diagnósticos en riñones trasplantados: rechazo agudo 50%, necrosis tubular aguda 25%, rechazo crónico 20%, enfermedad recurrente en trasplante 5%. Complicaciones: Hematuria transitoria: 21 casos (5%), hematoma perirenal: 3(<1%), perforación intestinal: 2 (<0.5%), hemorragia importante: 2 (<0.5%), nefrectomía: 1(0.2%). La presente es una de las primeras casuísticas de biopsias renales reportadas en Latinoamérica y una de las más grandes en el mundo y, de acuerdo a nuestros resultados, es un procedimiento seguro con gran utilidad diagnóstica, pocas complicaciones, sin mortalidad.

Evaluation and analysis of the results of renal biopsy are important for diagnostic, therapeutic and prognostic matters. To evaluate a series of renal biopsies performed during the period 1978-2007 in the Hospital de Niños de Valencia, Venezuela. All patients had history of either primary or secondary nephropathies. 421 biopsies were done in 377 patients, ages 2 months-20 years; 57% boys. 26 patients were re-biopsed. Percutaneous needle biopsy (PNB) was performed in all the patients, except in one who underwent open biopsy because of a solitary kidney. Renal tissue was processed for optical, inmunofluorescence and electronic microscopy in 98% of cases. The biopsy technique, clinical syndromes at presentation, hystopathological pattern, effectiveness and complications are described. Adequate sample was obtained in 392 cases (93%) (more than 10 glomeruli) and inadequate or failed biopsy in 29(7%). Clinical syndromes at presentation were: nephrotic syndrome: 199 cases (50%), atypical acute nephritic syndrome: 53 (13%), others: hematuria and proteinuria, isolated proteinuria, kidney transplant biopsy or systemic diseases: 143(37%). The hystopathological pattern obtained was as follows. A.-Primary glomerulonephritis (PG): 302 cases, 77%, B.-Secondary nephropathies: 68 cases, 17%, C.- Kidney transplant biopsies: 28 cases, 5 %. Primary Glomerulonephritis diagnosis: minimal change disease: 140 cases, 46%, Focal Segmental Glomerulosclerosis: 79(26%), diffuse proliferative glomerulonephritis/mesangial: 67(22%), membranous glomerulonephritis: 16(5%). Secondary nephropathies: lupus nephritis: 20 cases (32.25%), IgA Nephropathy: 22 cases (35.50%), others: 20 cases (32.25%).Transplant biopsies: rejection 50%, acute tubular necrosis 25%, chronic rejection 20%, and recurrent disease 5%. Complications: transient hematuria: 21(5%), perirenal hematoma: 3(<1%), gut perforation (<0.2%), bleeding which required blood transfusion: 2(<0.5%) and nephrectomy because of incontrollable...

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A biópsia renal percutânea constitui o padrão-ouro para o diagnóstico, prognóstico e tratamento das doenças glomerulares. Estudamos 51 pacientes que foram submetidos à biópsia renal no Hospital Geral César Cals, em fortaleza, Ceará Brasil, no período de janeiro de 2000 a dezembro de 2007. Houve predomínio do sexo feminino (57%). A faixa etária mais afetada foi de 20-40 anos. A principal forma de apresentação das glomerulopatias foi a síndrome nefrótica (49%), e o padrão histopatológico mais prevalente nas glomerulopatias secundárias (37%). Tivemos 5,8% de complicações atribuídas à biópsia renal, tendo sido a hematúria macroscópica a principal responsável (3,9%). Estudos mais abrangentes são necessários para traçar o perfil epidemiológico das doenças glomerulares no Estado do Ceará.

A percutaneous renal biopsy is the gold standard for diagnosis, prognosis and treatment of glomerular diseases. We studied 51 patients who underwent renal biopsy at the Hospital Geral César Cals, Fortaleza, Ceara, Brazil, from January 2000 to December 2007. There was predominance of females (57%). The most affected age group was 20-40 years. The main form of presentation was nephrotic syndrome glomerulopathies (49%), and histopathological pattern more prevalent in secondary glomerulopathies (37%). 5.8% had complications attributed to renal biopsy, macroscopic hematuria was the main cause (3.9%). Large studies are needed to delineate the epidemiological profile of glomerular diseases in Ceará.

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Objetivo: estabelecer o perfil epidemiológico dos pacientes submetidos à biópsia renal na fundação.

Objective: to establish the epidemiologic profile of Fundação Hospital de Clínicas Gaspar Vianna (FHCGV) patients submitted to renal biopsy and identify the most common kidney diseases biopsed and related clinical syndromes, formulating clinical and pathologic correlations. Methods: a retrospective study was undertaken of ali renal biopsy registries performed from 2002 to 2005 at Nephrology Service of FHCGV. Data were obtained from Kidney Biopsies Registry Book, which contained personal, clinical and histological data of 32 patients, of both sexes. Results: adults composed most part of the sample (94%) and 56% of ali patients were male. Only three subjects IInderwellt biopsies complicationsj. The most common clinical syndrome was nephritic syndrome (72%), followed by hematuria (25%). Focal segmental glomerulosclerosis (FSGS) (22%) and lupus nephritis (15%) were associated to nephrotic syndrome in most of cases. Conclusion: nephrotic syndrome and FSGS were the most frequent clinical and histological findings at FHCGV, respectively.

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Neste artigo, apresentamos uma revisão geral dos aspectos clínicos e laboratoriais dopaciente com proteinúria. A proteinúria reflete um aumento na permeabilidade do capilarglomerular. São descritos três tipos básicos de proteinúria: glomerular, tubular ou por aumentoda produção. A proteinúria assintomática também pode ser dividida em três categorias: transitória,ortostática e persistente. A avaliação desses pacientes com proteinúria deve começar com oexame comum de urina, repetido pelo menos duas vezes. O sedimento urinário também deveser feito, procurando-se sinais de comprometimento glomerular, como: hematúria dismórfica,cilindros hemáticos ou lipidúria. Uma cuidadosa história médica é importante, buscando-se apresença de hipertensão, diabetes melito, insuficiência cardíaca congestiva ou história préviade doença renal. A proteinúria persistente pode ser avaliada através da medida na urina de 24horas ou medindo-se a proteinúria e a creatinina em amostra isolada de urina. A amostra deurina aleatória é um método simples, rápido e com boa correlação com a urina de 24 horas. Aquantidade de proteinúria excretada é importante, do ponto de vista prognóstico, nos pacientescom doença glomerular primária, tais como: glomerulonefrite membranosa, glomerulonefritepor IgA, glomeruloesclerose segmentar e focal. Nesses pacientes, quanto maior for a proteinúria,maior será o risco de lesão renal.

In this paper, we present a general review of clinical and laboratory approaches to patientswith proteinuria. Proteinuria reflects an increase in glomerular capillary permeability. There arethree basic types of proteinuria: glomerular, tubular, and overflow. Asymptomatic proteinuria canalso be divided into three categories: transient or intermittent, orthostatic, and persistent. Theevaluation of patients with proteinuria should begin by testing the urine on at least two differentoccasions. The urine sediment should also be examined, searching for other signs of glomerulardisease, such as dysmorphic hematuria, red cell casts or lipiduria. A careful medical history isalso indicated, searching for hypertension, diabetes mellitus, congestive heart failure or previoushistory of renal disease. Persistent proteinuria can be evaluated by a 24-hour urine collection ora random urine protein-to-creatinine ratio. The random urine sample can be simply and quicklyobtained and presents good correlation with the 24-hour urine evaluation. The degree of proteinuriais prognostically important in patients with primary glomerular disease, such as membranousglomerulonephritis, IgA glomerulonephritis or focal and segmental glomerulosclerosis. The higherthe proteinuria, the higher the risk of renal injury for these patients.

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La biopsia renal es el principal método de diagnóstico de las glomerulopatías. El Registro de biopsias renales es importante para conocer la situación epidemiológica y clínico­patológica de las enfermedades renales de un país y las variaciones geográficas entre los países o continentes. Para conocer la situación actual de las glomerulopatías del Paraguay se ha realizado el presente estudio en 1.365 biopsias renales registradas entre agosto de 1989 a junio de 2005 en el Departamento de Patología del IICS, que es el Centro de Referencia y Registro de Biopsias Renales del país, analizando sus aspectos histológicos, demográficos y las diferencias entre periodos quinquenales de estudio. Del total de casos, 1072 (75.5%) fueron glomerulopatías y, a diferencia de la mayoría de los países, se observó un predominio de glomerulonefritis secundarias (GNS) (57.3%) de las cuales 85.9% fueron nefritis lúpica (NL). En las glomerulonefritis primarias (GNP) la GN proliferativa mesangial (GNPM) y la membranoproliferativa (GNMP) fueron las mas frecuentes (39.8% y 28.2%), mientras que la Nefropatía por IgA (NIgA) y la Enfermedad de cambios mínimos (ECM) fueron raras (1.9% y 1.3%). No se observaron cambios significativos de incremento o disminución de tipos histológicos a través de los periodos de estudio tanto en las GNP como en las GNS. La existencia de este registro de biopsias renales fue la base para la creación del Registro de Glomerulopatías del Paraguay en el año 2004, cuyos datos son necesarios para establecer programas de tratamiento y prevención de las enfermedades glomerulares en nuestro país a fin de disminuir su progresión al estadío terminal.

Renal biopsy is the main diagnostic method of glomerulopathies. The registry of renal biopsies is important to know the epidemiological and clinical and pathological situation of renal diseases of a country and the geographic variations between countries or continents. In order to know the current situation of glomerulopathies in our country, we studied 1,365 renal biopsies registered between August 1989 to June 2005 at the Pathology Department of the IICS, the reference center and the Renal Biopsy Registry of the country, analyzing histological and demographic aspects as well as the differences between five­year periods of study. On the total cases, 1,072 (75.5%) were glomerulopathies while secondary glomerulonephritis (SGN) were more frequent (57.3 %) with 85.9% of lupus nephritis (LN) in contrast to most countries. Among primary glomerulonephritis (PGN), mesangioproliferative GN (MPGN) and membranoproliferative GN (MPGN) were predominant (39.8% and 28.2%) while IgA Nephropathy (IgAN) and Minimal Change Disease (ECM) were rare (1.9% and 1.3%). We did not observe significant changes of increases or decreases of histological types through the study periods in PGN as well as in SGN. The existence of this registry of renal biopsy was the base for the creation of the Paraguayan Registry of Glomerulopathies in 2004, which data are necessary to establish treatment and prevention programs of glomerular diseases in our country in order to decrease their progression to end­stage renal disease.

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La glomerulonefritis crónica es una de las causas principales de enfermedades renales en estadío terminal (ERET) en países en vías de desarrollo, con incremento de su incidencia y prevalencia. En este estadío solamente sobreviven los pacientes con posibilidades de acceso a la terapia de reemplazo renal (TRR) como la diálisis y el transplante renal, por su costo elevado, mientras que aquellos con enfermedades renales crónicas (ERC), con ingresos bajos y sin cobertura de seguro de salud, pueden progresar a ERET y fallecer por uremia, porque la TRR no es accesible para ellos. En el Paraguay un gran número de pacientes no tienen acceso a TRR y no existen datos nacionales sobre enfermedades renales para desarrollar programas de prevención y tratamiento. Para conocer la situación real de ERC en nuestro país, el primer paso fue la creación del Registro de Glomerulopatías del Paraguay, en septiembre de 2004, por Resolución de Ministerio de Salud Pública y Bienestar Social basado en el Registro de Biopsias renales del IICS de la Universidad Nacional de Asunción, que es el Centro de Referencia de biopsias renales del país. Este Registro nos proveerá de datos de incidencia, prevalencia, tipos, aspectos epidemiológicos y clínicopatológicos de las enfermedades renales y también nos permitirá realizar estudios epidemiológicos comparativos con otros Registros del mundo. La creación del Registro será un importante apoyo para implantar programas de diagnóstico precoz, prevención y tratamiento de las glomerulopatía a fin de disminuir la gran carga de ERET en pacientes renales y también en el aspecto financiero del país.

Chronic glomerulonephritis is one of the main causes of end­stage renal diseases (ESRD) in developing countries with increasing incidence and prevalence rates. In this stage, because of the high costs only patients with financial possibility that have access to renal replacement therapy (RRT) such as dialysis or renal transplant are alive; while people with chronic renal diseases (CRD), low income and without coverage of health insurance can progress to ESRD and death from uremia because RRT is out of their reach. In Paraguay, a large number of patients with CRD are not able to access to RRT and there is not a national data on renal disease to develop prevention and treatment programs of renal diseases. The first step to know the real situation of CRD was the creation of the Paraguayan Registry of Glomerulopathy on September 2004 by Resolution of the Ministry of Public Health based on the Renal Biopsy Registry of IICS of the National University of Asunción, the reference center of renal biopsy of the country. This National Registry of Glomerulopathies will provide us data about incidence, prevalence, types, epidemiological, clinical and pathological aspects of renal diseases and will also allow to carry out epidemiological studies in comparison with other registries of the world. The creation of the registry will be an important support to implement programs of early diagnosis, prevention and treatment of glomerulopathies in order to decrease the great burden of the ESRD on renal patients and also on the financial possibilities of the country.

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