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.

Most common histopathological patterns of the Minas Gerais Association of the Centers of Nephrology

SUMMARY INTRODUCTION: We analyzed the distribution and frequency of glomerular diseases in patients biopsied between 1992 and 2016 in centers that make up the AMICEN (Minas Gerais Association of Nephrology Centers). METHODS: We analyzed the biopsy reports of patients from 9 AMICEN nephrology centers. We took note of their age, gender, ultrasound use, post-biopsy resting time, whether the kidney was native or a graft, number of glomeruli and indication for the biopsy. The kidney biopsy findings were broken down into four categories: glomerular and non-glomerular diseases, normal kidneys and insufficient material for analysis. Those patients diagnosed with glomerular diseases were further divided into having primary or secondary glomerular diseases. RESULTS: We obtained 582 biopsy reports. The median age was 38 years (1 to 85). The number of glomeruli varied between 0 and 70 (median = 13.0). In total, 97.8% of the biopsies were ultrasound guided. The main indication was nephrotic syndrome (36.9%), followed by hematuria-proteinuria association (16.2%). Primary glomerular diseases proved to be the most frequent (75.3%), followed by secondary diseases (24.7%). Among the primary glomerular diseases, FSGS was found at a higher frequency (28.8%), while among the secondary diseases, SLE was the most prevalent (42.4%). Regarding prevalence findings, those for both primary and secondary diseases were similar to those found in the large Brazilian registries published thus far. CONCLUSION: Glomerular disease registries are an important tool to identify the prevalence of such disease in regions of interest and can serve as an instrument to guide public policy decisions concerning the prevention of terminal kidney diseases.

RESUMO INTRODUÇÃO: Analisamos a distribuição e frequência de doenças glomerulares de pacientes biopsiados entre 1992 e 2016 em centros que compõem a Amicen (Associação de Minas Gerais de Nefrologia). MÉTODOS: Analisamos os relatórios de biópsia de pacientes de nove centros de nefrologia da Amicen. Observamos idade, gênero, uso de ultrassom, tempo de descanso pós-biópsia, se o rim era nativo ou um enxerto, número de glomérulos e indicação para a biópsia. Os achados da biópsia do rim foram divididos em quatro categorias: doenças glomerulares e não glomerulares, rins normais e material insuficiente para análise. Os pacientes diagnosticados com doenças glomerulares foram ainda divididos em doenças glomerulares primárias ou secundárias. RESULTADOS: Obtivemos 582 relatórios de biópsia. A idade mediana foi de 38 anos (1 a 85). O número de glomérulos variou entre zero e 70 (mediana = 13,0). No total, 97,8% das biópsias foram guiadas por ultrassom. A principal indicação foi síndrome nefrótica (36,9%), seguida de associação hematúria-proteinúria (16,2%). As doenças glomerulares primárias revelaram-se as mais frequentes (75,3%), seguidas de doenças secundárias (24,7%). Entre as doenças glomerulares primárias, o FSGS foi encontrado em maior frequência (28,8%), enquanto nas doenças secundárias, o lúpus eritematoso sistêmico foi o mais prevalente (42,4%). Quanto aos achados de prevalência, aqueles para doenças primárias e secundárias foram semelhantes aos encontrados nos grandes registros brasileiros publicados até o momento. CONCLUSÃO: Os registros de doenças glomerulares são uma ferramenta importante para identificar a prevalência dessas doenças em regiões de interesse e pode servir como um instrumento para orientar decisões de políticas públicas relativas à prevenção de doenças renais terminais.

Comparative analysis of primary and secondary glomerulopathies in the northeast of Brazil: data from the Pernambuco Registry of Glomerulopathies - REPEG / Análise comparativa de glomerulopatias primária e secundária no nordeste do Brasil: dados do Registro Pernambucano de Glomerulopatias - REPEG

Abstract Introduction: In Brazil, glomerulopathies are the third leading cause of chronic renal disease, accounting for 11% of dialysis patients. Studies on the prevalence of this disease in Northeastern Brazil are scarce. Objective: The aim was to describe the findings of biopsies and to conduct a comparative analysis on the clinical laboratory presentation of primary glomerulopathies (PG) and secondary glomerulopathies (SG). Methods: This was a retrospective study conducted at two public teaching hospitals in the state of Pernambuco, Northeastern Brazil. Results: A total of 1151 biopsies performed between 1998 and 2016 were analyzed. The sample consisted of 670 biopsies of native kidneys, after excluding extra glomerular diseases and unsuitable material. PG were more frequent than SG (58% vs. 42%). There was a prevalence among PG of focal segmental glomerulosclerosis (43%). Membranoproliferative glomerulonephritis and collapsing glomerulopathy, accounted for 9% and 3% of the PG, respectively. For SG, the main etiologies were lupus nephritis (67%) and infections (10%). Female sex, hematuria and an elevated level of creatinine were related to a greater chance of SG, at multivariate analysis. An increase of proteinuria reduced this chance. Nephrotic syndrome was more common among the PG, while urinary abnormalities and nephritic syndrome prevailed in patients with SG. Conclusion: This is the first registry of glomerulopathies in Northeastern Brazil. It also presents a comparative analysis of the main clinical laboratory abnormalities of PG and SG, and includes the current classifications of glomerular diseases.

Resumo Introdução: No Brasil, glomerulopatias são a terceira causa de doença renal crônica terminal, responsáveis por 11% dos pacientes em diálise. Entretanto, estudos sobre a prevalência desta patologia no nordeste do Brasil são escassos. Objetivo: O objetivo foi descrever os achados das biópsias e analisar comparativamente a apresentação clínico laboratorial entre as glomerulopatias primárias (GP) e as glomerulopatias secundárias (GS). Métodos: Estudo retrospectivo, realizado em dois hospitais públicos de ensino do estado de Pernambuco, nordeste do Brasil. Resultados: Foram avaliadas 1.151 biópsias, de 1998 a 2016. A amostra foi composta por 670 biópsias de rins nativos, após exclusão de patologias extra glomerulares e materiais inadequados. GP foram mais frequentes do que GS (58% × 42%). Dentre as GP, houve predomínio de glomeruloesclerose segmentar e focal (GESF). Glomerulonefrite membranoproliferativa e glomerulopatia colapsante foram responsáveis por 9% e 3% das GP, respectivamente. Das GS, as etiologias principais foram nefrite lúpica (67%) e infecciosas (10%). Sexo feminino, hematúria e nível elevado de creatinina estiveram relacionadas a uma maior chance de GS na análise multivariada. Síndrome nefrótica foi mais comum dentre as GP, já anormalidades urinárias e síndrome nefrítica prevaleceram nos pacientes com GS. Conclusões: Este é o primeiro registro de glomerulopatias do nordeste do Brasil. Demonstrou-se também uma análise comparativa das principais alterações clínico laboratoriais das GP e GS, com classificações atualizadas das doenças glomerulares.

Pattern, clinical features and response to corticoids of glomerular diseases in a Brazilian population. An analytical cross-sectional study / Perfil, características clínicas e resposta a corticoides das doenças glomerulares em uma população brasileira. Um estudo transversal analítico

CONTEXT AND OBJECTIVE: Glomerular disease registries are increasing all around the world. The aim of this study was to evaluate the clinical characteristics and treatment response among patients with glomerular diseases followed up in a tertiary hospital in Brazil. DESIGN AND SETTING: Analytical cross-sectional study; tertiary-level public hospital. METHODS: This study included patients with glomerular diseases followed up at a tertiary hospital in Fortaleza, northeastern Brazil. Clinical and laboratory data on each patient were registered. The response to specific treatment was evaluated after 3, 6 and 12 months. RESULTS: The study included 168 patients of mean age 37 ± 14 years. The most prevalent glomerular diseases were focal segmental glomerulosclerosis FSGS] (19.6%), minimal change disease MCD] (17.9%), membranous nephropathy MN] (16.7%) and lupus nephritis LN] (11.9%). The main clinical presentations were nephrotic proteinuria (67.3%) and renal insufficiency (17.9%). The mean proteinuria value decreased after the treatment began. Regarding 24-hour proteinuria on admission, there was no significant difference between patients with a good response and those with no response (7,448 ± 5,056 versus 6,448 ± 4,251 mg/24 h, P = 0.29). The glomerular disease with the highest remission rate was MCD (92%). Absence ...

CONTEXTO E OBJETIVO: Registros de glomerulopatias estão aumentando em todo o mundo. O objetivo deste estudo é avaliar as características clínicas e a resposta do tratamento de pacientes com glomerulopatias acompanhados em um hospital terciário no Brasil. TIPO DE ESTUDO E LOCAL: Estudo transversal analítico. Hospital público terciário. MÉTODOS: O estudo incluiu pacientes com glomerulopatias acompanhados em um hospital terciário de Fortaleza, Ceará, Brasil. Foi realizado registro dos dados clínicos e laboratoriais para cada paciente. A resposta ao tratamento específico foi avaliada após 3, 6 e 12 meses. RESULTADOS: Foram incluídos 168 pacientes, com média de idade de 37 ± 14 anos. A glomerulopatia mais prevalente foi a glomerulosclerose segmentar e focal GESF] (19,6%), seguida pela doença de lesão mínima DLM] (17,9%), nefropatia membranosa NM] (16,7%) e nefrite lúpica NL] (11,9%). As principais manifestações clínicas foram proteinúria nefrótica (67,3%) e insuficiência renal (17,9%). A média dos valores de proteinúria diminuiu após o início do tratamento. Com relação à proteinúria de 24 horas na admissão, não houve diferença significativa entre os pacientes com boa resposta ao tratamento ...

Marcadores de necrose tubular aguda e lesão inflamatória glomerular no sedimento urinária de pacientes com síndrome / Acute tubular necrosis markers and inflammatory glomerular injury urinary sediment syndrome patients

Necrose tubular aguda (NTA) é a causa mais frequente de lesão renal aguda (LRA) em pacientes hospitalizados. Em pacientes com síndrome nefrótica (SNO), a NTA mimetiza, por vezes, quadro de glomerulonefrite rapidamente progressiva e requer instituição precoce de imunossupressores. A análise do sedimento urinário é uma ferramenta não invasiva, de baixo custo e ampla disponibilidade. O achado de células epiteliais no sedimento urinário de pacientes com LRA foi associado ao diagnóstico de NTA. Entretanto, estudos em pacientes com SNO associada são escassos. Técnicas de diagnóstico utilizando sedimento urinário corado normalmente não são utilizadas nesses casos. Além do mais, o sedimento urinário é uma importante fonte de proteínas; estudos proteômicos do sedimento urinário revelaram importantes frações de proteínas não encontradas em sobrenadante, que pode ser usado como potencial biomarcador de LRA. Nosso objetivo é identificar alterações citológicas e protéicas no sedimento urinário que permitam o diagnóstico diferencial entre NTA ou lesão inflamatória-proliferativa glomerular (INF) em pacientes com SNO. Trata-se de um estudo de corte transversal, onde foram incluídos 32 pacientes: 5 pacientes normais (grupo controle), 10 com NTA, 9 sem NTA e 8 com glomerulonefrites exsudativas. As células do sedimento urinário foram contadas, citocentrifugadas, coradas em hematoxilina/eosina ou Papanicolaou e contadas diferencialmente como pequenas (<30μm de diâmetro), médias (30-48μm)...

Acute tubular necrosis (ATN) is the most frequent cause of acute kidney injury (AKI) in hospitalized patients. In patients with nephrotic syndrome (NS), acute tubular necrosis mimic, sometimes, rapidly progressive glomerulonephritis and requires premature institution of immunosuppressive treatment. The analysis of urinary sediment is a noninvasive tool, low cost and wide availability. The found of epithelial cells in the urinary sediment of patients with AKI was associated to ATN diagnosis. However, studies in patients with AKI in the set of NS are scarce. Diagnostics techniques using stained urinary sediment are not ordinarily used in these cases. Furthermore, urinary sediment is an important source of proteins; proteomic studies revealed important fractions of proteins not found in urinary supernatant that could be used as potential biomarkers for AKI. Our goal is identify cytological alterations and protein in urinary sediment which allow the differential diagnosis between ATN and inflammatory-proliferative glomerular lesion (INF) in patients with NS. This is a cross sectional study, in which 32 patients were included: 5 normal patients (control group), 10 with ATN, 9 without ATN and 8 with exudative glomerulonephritis. The cells of urinary sediment were counted, cytocentrifuged, stained of hematoxylin/eosin or Papanicolaou and differentially counted as small (<30μm of diameter), medium (30-48μm)...

Patterns of Glomerulonephritis in Uttarakhand State 13 Years Single Centre Experience at Teaching Institute.

Aims : Glomerular disease is considered to be a common cause of chronic kidney disease and its prevalence and histological pattern differ according to geographical area, race, age in different regions of the world. The present study was therefore conducted to study the pattern of different histological types of glomerulonephritis in this area of Uttarakhand region of India over an extended period of 13 years. Material and Methods : Retrospective study was conducted in the tertiary care centre of Uttarakhand state which included cases of different types of glomerulonephritis diagnosed on histopathology over a period of 13 years. Age, sex, clinical presentation, relevant investigations and the histopathological type of glomerulonephritis were noted for every case. Results : Histopathological examination due to various non-neoplastic renal diseases was done in total 218 cases and out of these glomerular diseases was diagnosed in 178 cases. Proteinurea and edema was the most common presentation. Primary glomerulonephritis was observed in 88.5% cases and secondary in 9.9%. Mesangioprolifrative glomerulonephritis was the most common primary glomerulonephritis and nephropathy due to amyloidosis was the most common type of secondary glomerulonephritis. Conclusion : The study concludes that primary glomerulonephritis is the most common renal disease with mesangioproliferative glomerulonephritis as the most common primary glomerulonephritis and amyloidosis nephropathy as the most common secondary glomerulonephritis which differs from other regions of world. The study may be useful to pathologists, nephrologists and health care providers to formulate a basic platform for effective diagnostic, therapeutic and research base for glomerular diseases so as to prevent its complications.

Prevalencia de enfermedad renal en pacientes adolescentes internados en el Servicio de Nefrología del Hospital de Niños Dr. Ricardo Gutierrez / Prevalence of renal disease in adolescents patients treated in Nephrology Unit of Children´s Hospital Dr. Ricardo Gutierrez

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.

Frequency of primary glomerular disease in northeastern China

Most frequently reported Chinese renal biopsy data have originated from southeastern China. The present study analyzed the renal biopsy data from northeastern China. The records of 1550 consecutive native patients who were diagnosed with primary glomerular diseases (PGD) after renal biopsy at our hospital during 2005-2009 were used. These patients were divided into four age groups for stratified analysis: <15, 15-44, 45-59, and ≥60 years old. Among PGD, minimal change disease (MCD) was the most common histologically diagnosed disease (30.7 percent), followed by IgA nephropathy (IgAN), mesangial proliferative glomerulonephritis (MsPGN), membranous nephropathy (MN), membranoproliferative glomerulonephritis (MPGN), focal segmental glomerulosclerosis (FSGS), and endocapillary proliferative glomerulonephritis (EnPGN). MCD was the disease most frequently observed (43.7 percent) in the <15-year-old group. MsPGN was the most common disease in the elderly group (38.1 percent). MsPGN was more prevalent in females (27.8 percent), whereas MCD was more prevalent in males (35.3 percent). Primary glomerular diseases constituted the most commonly encountered group of diseases with a high prevalence of MCD, which predominantly affected males and young adults. The prevalence of MCD was high in northeastern China. Further study is necessary to expand the epidemiologic data available for renal disease in China.

Immunoglobulin M nephropathy nephropathy in adults and adolescents in India: A single-center study of natural history.

Background: Immunoglobulin M nephropathy (IgMN) is an idiopathic glomerulonephritis (GN) usually presenting clinically as steroid resistant/dependent nephrotic syndrome (NS) with pathology of mesangial proliferative GN or focal and segmental glomerulosclerosis with diffuse predominant mesangial IgM deposits. Not much information is available about its natural history. This is the first Indian study to our knowledge on IgMN in adults and adolescents. Materials and Methods: We evaluated renal biopsies performed at our center between January,'04 to September,'09. Biopsies of all adolescents and adults were evaluated for IgMN and we studied their age, gender distribution, blood pressure (BP), disease duration, steroid/immunosuppressive management and serial serum creatinine (SCr), urinary proteins, and BP values. Patients with other systemic diseases/infections and children were excluded. Results: IgMN constituted 4.3% of 2702 adult renal biopsies. No significant gender predilection was noted. Males presented at average age of 23.1 years, females at 30 years. Steroid-dependent NS was the commonest presentation noted in 75% followed by steroid-resistant NS. Hypertension was noted in 10% patients. Mesangial proliferative GN (MePGN) was commonest histopathological finding noted in 74.4%, followed by focal segmental glomerulosclerosis (FSGS) in 16.2%, and minimal change disease (MCD) in 9.4% biopsies. Sole IgM deposits were noted in 88.5%. All MCD, 35.6% MePGN reached remission, FSGS progressed to renal failure by 1 year. Hypertension, proteinuria, interstitial fibrosis, and FSGS were bad prognosticators. Conclusions: This is the first Indian study of IgMN in adults and adolescents carried out over a period of 5.8 years, which has shown that hypertension, proteinuria, and interstitial fibrosis at presentation have bad prognosis.

Profile of glomerular diseases in a public hospital of Federal District, Brazil / Perfil das doenças glomerulares em um hospital público do Distrito Federal

INTRODUÇÃO: As doenças glomerulares são uma causa frequente de doença renal crônica, sobretudo nos países em desenvolvimento. OBJETIVO: O objetivo deste estudo foi determinar o perfil destas glomerulopatias em um hospital público da cidade de Brasília, Distrito Federal. MÉTODOS: Foram realizadas 121 biopsias renais pela equipe de nefrologia do Hospital Regional da Asa Norte (HRAN) entre agosto de 2005 e maio de 2009. Foram excluídas oito biopsias realizadas em pacientes transplantados renais e analisados os prontuários dos 113 pacientes restantes. Dados analisados: sexo, idade, exames laboratoriais, síndrome glomerular, diagnóstico clínico, grau de fibrose intersticial, uso de imunossupressores, necessidade de diálise e desfecho clínico. RESULTADOS: A média de idade foi 34,9 ± 16,2 anos, com predomínio masculino (51,3 por cento). As principais síndromes glomerulares foram: síndrome nefrótica (41,6 por cento) e glomerulonefrite rapidamente progressiva (35,4 por cento). Entre as glomerulopatias primárias, houve predomínio da glomeruloesclerose segmentar e focal (26,9 por cento) e da nefropatia por IgA (25 por cento) e entre as secundárias a nefrite lúpica (50 por cento) e a glomerulonefrite proliferativa exsudativa difusa (34,2 por cento). A maioria dos pacientes fez uso de imunossupressores (68,1 por cento) e quase um terço deles (29,2 por cento) necessitou de diálise durante a internação. Evoluíram para terapia dialítica crônica 13,3 por cento dos pacientes e 10,6 por cento evoluíram a óbito. CONCLUSÃO: Este estudo poderá contribuir para melhor entendimento epidemiológico das doenças glomerulares no Distrito Federal, orientando na adoção de políticas públicas visando permitir rápido diagnóstico e manejo clínico das mesmas.

INTRODUCTION: Glomerular diseases are a frequent etiology of chronic kidney disease, especially in the developing countries. OBJECTIVE: To determine the profile of such glomerulopathies in a public hospital located in the city of Brasilia, Federal District. METHODS: 121 renal biopsies in different patients were performed by the Renal Division of Hospital Regional da Asa Norte (HRAN) between August 2005 and May 2009. Eight renal biopsies in renal-transplant patients were excluded and the medical records of 113 remaining patients were analyzed. Analyzed data: sex, age, laboratory exams, glomerular syndrome, clinical diagnosis, degree of interstitial fibrosis, immunosuppressants use, need for dialysis and clinical outcome. RESULTS: The age average was 34.9 ± 16.2 years-old, a predominance of male patients (51.3 percent). Major glomerular syndromes were: nephrotic syndrome (41.6 percent) and the rapidly- progressive glomerulonephritis (35.4 percent). Among primary glomerulopathies focal glomerulosclerosis (26.8 percent) followed by IgA nephropathy (25 percent) were predominant; and among the most prevalent secondary glomerulopathies we had lupus nephritis (50 percent) and diffuse exudative proliferative glomerulonephritis (34.2 percent).The majority of the patients used immunosuppressants (68.1 percent) and almost one third of them (29.2 percent) needed dialysis during their hospitalization. Progressed to chronic dialysis therapy 13.3 percent of the patients and 10.6 percent died. CONCLUSION: This study may contribute to better epidemiological understanding of glomerular diseases in the Federal District, guiding the adoption of public policies aiming the quick clinical treatment of such diseases.

[Underlying causes of acute renal failure in children admitted to Abuzar children's hospital in Ahvaz]

Acute renal failure [ARF] can be pre-renal, intrinsic renal or post-renal in etiology. The aim of the study was to assess the causes of ARF in patients admitted to Abuzar Children's Hospital in Ahvaz, a referral center in the South Western of Iran. The records of patients managed as ARF were reviewed in our center from 2003 to 2006. ARF was defined as doubling of baseline serum creatinine associated with oliguria. A patient with evidences of chronic kidney disease was excluded. Based on the data, these causes were categorized in 3 groups as: pre-renal, intrinsic renal and post-renal. Totally 113 admitted patients had the criteria for ARF. There were 77 [68%] males and 36 [32%] females [M/F: 2.2]. The mean age was 6.8 years [ranging from 10 days to 15 years], but most of the patients [34.5%] were 5-10 years old. The underlying causes of ARF were 74.4% intrinsic, renal 18.6% pre-renal and 7% post- renal. Among 84 patients with intrinsic renal failure, 63 [55.7%] had acute glomerulonephritis [AGN]. The underlying causes of ARF in our region were different from other parts of the world. The most common causes of ARF were AGN and ATN due to scorpion sting and severe dehydration secondary to acute gastroenteritis. Therefore, most of these cases can be preventable by improving primary health care and by early and prompt treatment of infection and dehydration

Chronic renal failure in Jamaican children: an update (2001-2006) / Insuficiencia renal crónica en niños jamaicanos: actualización (2001-2006)

OBJECTIVE: This study evaluated the incidence, epidemiology, aetiology and outcome of chronic renal failure (CRF) in Jamaican children < 12 years old between 2001 and 2006. METHODS: The required data on all children who fulfilled inclusion criteria were obtained from their medical records at the University Hospital of the West Indies, Bustamante Hospital for Children and from practitioners in hospitals serving children islandwide. RESULTS: Eighteen new children (72.2% male) presented with CRF. The cumulative annual incidence was 4.61/million child population under age 12 years or 1.14/million total population. Congenital urological disease (44.5%) was the commonest cause of CRF, followed by glomerulonephritis (33.3%). Half of the cases of glomerulonephritis were secondary to HIV-associated nephropathy. Although all children with posterior urethral valves were diagnosed before age 6 months and promptly treated, renal failure present at birth proved irreversible. The mean age at diagnosis of CRF was 6.72 years. Ten children (55.6%) were already in CRF at first presentation with renal disease. Of these, the five with non-urological disease were already in End Stage Renal Disease (ESRD). Mortality was 44.4%. Five children died in ESRD without the benefit of dialysis. CONCLUSION: The incidence of CRF has increased from the 1985-2000 local study and is mainly due to urological pathology which progresses despite early diagnosis and treatment. Non-urological renal disease is presenting too late for therapeutic intervention. Greater public awareness of symptoms of renal disease is needed. Children's access to dialysis is unpredictable. A paediatric dialysis and transplantation programme is needed.

OBJETIVO: Este estudio evaluó la incidencia, epidemiología, etiología y resultado de la insuficiencia renal crónica (IRC) en niños jamaicanos menores de 12 años entre 2001 y 2006. MÉTODOS: Los datos requeridos sobre los niños que satisfacían los criterios de inclusión, fueron obtenidos a partir de sus historias clínicas en el Hospital Universitario de West Indies y en el Hospital Pediátrico Bustamante, así como a partir de médicos en hospitales que prestan atención a niños en todo el país. RESULTADOS: Dieciocho nuevos niños (72.2% varones) presentaron IRC. La incidencia acumulada anual fue de 4.61 por millón de población infantil menor de 12 años de edad o 1.14 por millón de población total. La enfermedad urológica congénita (44.5%) fue la causa más común de IRC, seguida por la glomerulonefritis (33.3%). La mitad de los casos de glomerulonefritis fueron secundarios a la nefropatía asociada al VIH. Aunque todos los niños con válvulas uretrales posteriores fueron diagnosticados antes de los 6 meses de edad y puestos bajos tratamiento, la insuficiencia renal presente al nacer resultó irreversible. La edad promedio al momento del diagnóstico de la IRC fue 6.72 años. Diez niños (55.6%) se hallaban ya con IRC desde que se les presentara inicialmente la enfermedad renal. De estos, los cinco con enfermedad no urológica se hallaban ya en la fase terminal de la enfermedad renal (ERT). La mortalidad fue de 44.4%. Cinco niños murieron en la ERT sin el beneficio de la diálisis. CONCLUSIÓN: La incidencia de la IRC ha aumentado desde el estudio local de 1985-2000, principalmente debido a patologías urológicas que progresan a pesar del diagnóstico precoz y el tratamiento. La enfermedad renal no urológica se está presentando demasiado tarde para una intervención terapéutica. Se requiere mayor conciencia pública de los síntomas de la enfermedad renal. El acceso de los niños a la diálisis es imprescindible. Hace falta una diálisis infantil y un programa de transplante.

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.

Comportamiento clínico y manejo de glomerulonefritis postestreptoccocica en niños egresados del HFVP en el período de enero 2006 a enero 2007 / Behavior and clinical management of glomerulonephritis postestreptoccocica in children discharged from the HFVP in the period from January 2006 to January 2007

El presente estudio fue realizado en el Hospital Fernando Velez Paiz en el período de enero 2006 a enero 2007. El principal objetivo fue el de conocer el comportamiento clínico y manejo de los niños con síndrome nefrítico egresados en el período de estudio. La muestra estuvo constituida por 48 pacientes, manejados en nuestro centro asistencial, fueron referidos únicamente 7 pacientes que presentaron complicaciones renales. La mayoría de los pacientes fueron del sexo masculino. La totalidad de estos pacientes presentaron como mínimo un foco infeccioso previo a su ingreso, predominantemente a nivel de faringe y piel. EL comportamiento clínico fue predominantemente clásico como edema, hipertensión y alteraciones del sedimento urinario como muestra de afectación renal...

Varied presentations of acute glomerulonephritis in children: Single centre experience from a developing country

The objective of this prospective study, carried out at Manipal Teaching Hospital, Pokhara, was to document the various clinical presentations of children with acute glomerulonephritis and compare them with the available biological parameters in Western Nepal. Clinical and laboratory parameters of children with oedema and microscopic/macroscopic haematuria. For seven years [2000-2007], 92 cases of children were clinically diagnosed with acute glomerulonephritis [AGN]. Other clinical and laboratory anaylses were also eventful. The present study highlights the varied presentations of AGN, atypical presentations or complications of glomerulonephritis being more common than the classical presentation in the Western Region of Nepal

IgM nephropathy in adults: incidence and correlation with electron microscopic features.

IgM nephropathy is characterised on light microscopy (LM) by variable features of normal glomeruli to mesangial hypercellularity; and immunofluorescence (IF) deposits of LgM. Our aim was to study the incidence of IgM nephropathy in adults with primary glomerular disease, with correlation to electron microscopy (EM) features. All adults presenting with proteinuria glomerular hematuria underwent renal biopsy. We excluded patients with systemic diseases and post-infectious glomerulonephritis. All the specimens were evaluated by LM, IF and EM. Our series had 146 cases. Of the 42 cases diagnosed on LM as minimal change disease, mesangial deposition of IgM was present in 11 cases. In addition there were seven cases of mesangioproliferative glomerulonephritis with mesangial IgM deposition. Thus, there were a total of 18 cases of IgM nephropathy (12.3%). Only six of these 18 cases showed typical electron dense deposits in the mesangium on EM. We feel that IgM nephropathy is probably a separate pathological entity, comprising 12.3% of all adults with primary chronic glomerulopathy. Electron dense deposits are seen in only about a third of these cases.

Non-neoplastic renal diseases in Kerala, India--analysis of 1592 cases, a two year retrospective study.

A retrospective analysis of 1592 renal biopsies received from various hospitals all over Kerala over a period of two years was done. Of the 1544 native kidney biopsies majority of cases (300; 18.84%) were focal segmental glomerulosclerosis. Next infrequency was IgA nephropathy (227; 14.26%). The other diagnoses included post infectious glomerulonephritis (GN) (150; 9.42%), lupus nephritis (131; 8.23%), membranous glomerulonephritis (118; 7.41%), minimal change disease (94; 5.94%), interstitial nephritis 87(5.46%), membranoproliferative glomerulonephritis (MPGN) and severe glomerulosclerosis (74; 4.64% each), IgM nephropathy (72; 4.52%), acute tubular necrosis and diabetic glomerulopathy (24; 1.5% each), ANCA-related GN (21;1.32%), amyloidosis (13; 0.82%), Henoch Schonlien purpura (12; 0.75%) atd thrombotic microangiopathy (10; 0.63%). Afew others like myeloma cast nephropathy, cholesterol embolism, Clq nephropathy, oxalosis, mixed connective tissue disorder (MCTD, Wegner's granulomatosis etc. also were seen occasionally. 48 cases were allograft biopsies (3.02%).

Les glomerulonephrites aigues post-infectieuses de l'enfant : aspects epidemiologiques et cliniques dans le service de pediatrie du CHU Donka, Conakry

Il s'agissait d'une étude prospective et descriptive réaliséedu 1er janvier 2001 au 31 décembre 2002 dans le service dePédiatrie du CHU Donka de Conakry en Guinée, l'objectifétant de décrire les aspects épidémiologiques et cliniquesdes glomérulonéphrites post infectieuses. Quarante trois(43) enfants âgés de 0 à 14 ans ont été inclus sur des critèrescliniques et biologiques.La fréquence hospitalière était de 2,65%. L'âge moyenétait de 7,93 ± 3,46 ans (extrêmes 11 mois et 14 ans).L'échantillon était composé de 24 garçons et 19 filles. Lescas ont été observés toute l'année avec une plus grandefréquence en saison pluvieuse. Le syndrome œdémateux aété le motif principal de consultation. L'hypertensionartérielle était présente chez 17 enfants, modérée dans 11cas et sévère dans 6 cas avec un fond d'œil au stade III. Uneinsuffisance rénale fonctionnelle rapidement réversible aété observée chez 15 enfants (34,9%). Les infections étaientORL dans 33 cas dont 26 angines et 7 otites suppurées,cutanées dans 5 cas et stomatologiques (caries surinfectées)dans 5 cas. Les pathologies associées étaient principalementle paludisme et les parasitoses intestinales. 76,74% derémission complète et 18,60% de rémission incomplète et4,66% de décès ont été obtenus.Les facteurs de morbidité étaient liés au climat et surtoutau retard diagnostique

Unresolved issues and current concepts in management of primary glomerulonephritis

The successful treatment of primary glomerulonephritis [GN] presenting with nephrotic syndrome in adults depends heavily on an accurate diagnosis. A successful diagnosis depends on a correct approach, combining light microscopy, immunofluorescence, and other special staining of renal biopsy material examined by a trained nephropathologist. A good clinical history and serological tests easily rule out possible secondary causes [for example, infection, autoimmune, metabolic or toxic] in most cases. Unfortunately, these procedures are not put into practice in most cases in developing countries, resulting in missed diagnosis and unnecessary steroid and immunosuppressant therapy with its inherent morbidity. Following the emergence of IgA and IgM nephropathies as very common forms of glomerular disorders in some countries, immunofluorescence has become absolutely necessary for their diagnosis. Moreover, a recent meta-analysis has defined different treatment protocols for minimal change nephropathy, focal segmental glomerulosclerosis, membranous nephropathy, and IgA nephropathy for a better outcome. This article emphasizes and elaborates on these issues for proper management of primary GN