Caracterización de enfermedades glomerulares: análisis de 22 años de biopsias renales / Frequency of glomerular diseases in an analysis of 550 kidney biopsies

BACKGROUND: The frequency of glomerular diseases is dynamic and varies according to geographic area. AIM: To evaluate the frequency of primary and secondary glomerulopathies, their demographic profile and main clinical characteristics. MATERIAL AND METHODS: Renal biopsies from native kidneys performed between 1999 and 2020 were retrospectively reviewed. Demographic characteristics, clinical presentation, most relevant laboratory tests, frequency of primary and secondary glomerulopathies were analyzed. RESULTS: We analyzed 550 kidney biopsies from patients with a median age of 48 years (64% females). Nephrotic syndrome was the main indication for renal biopsy. Primary and secondary glomerulopathies occurred with similar frequency. Within the primary glomerulopathies, membranous nephropathy (34.1%) was the most common, followed by IgA nephropathy (31.1%) and focal segmental glomerulosclerosis (14.1%). Among the secondary glomerulopathies, lupus nephropathy was the most common (41.7%), followed by pauciimmune glomerulonephritis (27.1%) and diabetic nephropathy (6.4%). When comparing the results with other regions, significant differences were observed with reported frequencies in United States, Europe, Asia and the rest of Latin America. CONCLUSIONS: The most common primary glomerulopathies were membranous nephropathy and IgA nephropathy. Among the secondary glomerulopathies lupus nephropathy and pauci-immune glomerulonephritis were the most common. Compared to international registries, we observed a high proportion of membranous nephropathy and pauci-immune glomerulonephritis.

A rare case of PLA2R- and THSD7A-positive idiopathic membranous nephropathy / Positividade tecidual dupla para PLA2R e THSD7A em nefropatia membranosa idiopática: raro relato de caso

ABSTRACT Idiopathic membranous nephropathy (IMN) is a frequent cause of nephrotic syndrome in adults. In terms of etiology, the condition may be categorized as primary/idiopathic or secondary. Literature on the pathophysiology of IMN has indicated the presence of autoantibodies (PLA2R and THSD7A) directed against podocyte antigens. The detection of antibodies against a domain favors IMN. The presence of autoantibodies against one of the domains would in theory exclude the possibility of there being autoantibodies against the other domain. However, cases of patients with PLA2R- and THSD7A-positive disease have been recently reported, showing that antibodies against two targets may be concomitantly produced via yet unknown pathophysiological mechanisms. This study reports the case of a 46-year-old male patient with nephrotic-range proteinuria, hematuria, hypoalbuminemia, and hypercholesterolemia submitted to biopsy and histopathology examination (LM, IF, IHC, and EM) eventually diagnosed with PLA2R- and THSD7A-positive IMN associated with IgA nephropathy, stressing our experience with the use of IgG subclasses, PLA2R, and THSD7A in the workup for MN and the relevance of adopting a broad and adequate approach to elucidating and acquiring knowledge of the pathophysiology of IMN.

RESUMO A Nefropatia Membranosa Idiopática (NMi) é uma frequente causa de síndrome nefrótica em adultos e sua etiologia pode ser estratificada em primária/idiopática ou secundária. O conhecimento da fisiopatologia da NMi sugeriu a presença de autoanticorpos (PLA2R e a THSD7A) direcionados contra antígenos existentes nos podócitos. A detecção de anticorpos contra um domínio favorece NMi. A presença de autoanticorpos contra um desses domínios autoexcluiria a possibilidade de autoanticorpos contra o outro domínio; no entanto, recentemente foram descritos casos que apresentaram dupla positividade para PLA2R e THSD7A, comprovando que, por mecanismos fisiopatológicos ainda não conhecidos, raramente pode existir produção concomitante de anticorpos contra os dois alvos. O presente estudo tem por objetivo relatar o caso de um paciente de 46 anos de idade, do sexo masculino, que apresentou quadro de proteinúria nefrótica, hematúria, hipoalbuminemia e hipercolesterolemia submetido a biópsia e exame histopatológico (ML, IF, IHQ e ME), confirmando um caso raro de NMi com positividade dupla para os anticorpos anti-PLA2R e anti-THSD7A e associação à nefropatia por IgA, mostrando nossa experiência com a utilização de subclasses de IgG, PLA2R e THSD7A na rotina laboratorial para a investigação da GNM e enfatizando a importância de uma abordagem ampla para adequada elucidação e conhecimento dos mecanismos fisiopatológicos na NMi.

Focal and Segmental Glomerulosclerosis and Membranous Nephropathy overlapping in a patient with Nephrotic Syndrome: a case report / Glomeruloesclerose Segmentar e Focal e Nefropatia Membranosa sobrepostas em paciente com Síndrome Nefrótica: relato de caso

Abstract Introduction: Some cases of membranous nephropathy (MGN) present focal segmental glomerulosclerosis (FSGS) typically associated with disease progression. However, we report a case of a patient who seemed to have MGN and FSGS, both primary. Case presentation: A 17-year-old female, Caucasian, presenting lower extremity edema associated with episodes of foamy urine and high blood pressure, had physical and laboratorial exams indicating nephrotic syndrome. A renal biopsy was performed and focal and segmental glomerulosclerosis were observed under light microscopy in some glomeruli presented as tip lesion, and in others it was accompanied by podocyte hypertrophy and podocyte detachment in urinary space, compatible with podocytopathy FSGS. Besides, there were thickened capillary loops with basement membrane irregularities due to "spikes" compatible with MGN stage II. Immunofluorescence showed finely granular IgG, IgG4, and PLA2R deposits in capillary loops and, in electron microscopy, subepithelial deposits and foot process effacement. These morphological findings are compatible with FSGS and MGN stage II. Conclusions: In the present case, clinical and morphological characteristics showed a possible overlap of primary FSGS and MGN as focal and segmental glomerulosclerosis does not seem to be related with MGN progression but with the podocytopathy FSGS.

Resumo Introdução: Alguns casos de nefropatia membranosa (NM) apresentam glomeruloesclerose segmentar e focal (GESF) tipicamente associada a progressão da doença. Contudo, relatamos o caso de uma paciente que parece ter NM e GESF, ambas primárias. Apresentação do caso: Uma jovem branca de 17 anos de idade com edema de membros inferiores associado a episódios de urina espumosa e hipertensão apresentou-se com achados físicos e laboratoriais sugestivos de síndrome nefrótica. Foi realizada biópsia renal. GESF foi observada por microscopia de luz em alguns glomérulos que apresentavam lesões de ponta, enquanto em outros o achado era acompanhado por hipertrofia podocitária e descolamento de podócitos no espaço urinário, compatíveis com podocitopatia GESF. Além disso, as alças capilares estavam espessadas com irregularidades na membrana basal devido a "espículas" compatíveis com NM estágio II. Imunofluorescência revelou depósitos finamente granulares de IgG, IgG4 e PLA2R nas alças capilares. Microscopia eletrônica exibiu depósitos subepiteliais e apagamento de pedicelos. Tais achados morfológicos são compatíveis com GESF e NM estágio II. Conclusões: No presente caso, as características clínicas e morfológicas revelaram uma possível sobreposição de GESF primária e NM, uma vez que a glomeruloesclerose segmentar e focal não parece estar relacionada com a progressão da NM, mas com a podocitopatia GESF.

Histological changes and immunolabeling of Leishmania infantum in kidneys and urinary bladder of dogs / Alterações histológicas e imunomarcação de Leishmania infantum em rins e bexiga de cães

The aim of this study was to assess histological alterations and perform immunolabeling of Leishmania infantum in the kidneys and urinary bladder of naturally infected dogs. Twenty-five urinary bladder and kidney samples of serologically positive animals (ELISA S7® Biogene and IFAT ≥ 1:40 - Biomanguinhos/Fiocruz) were analyzed by means of immunohistochemical and histological techniques. Cystitis was found in 44% (11/25) of the bladder samples and membranoproliferative glomerulonephritis in 92% (23/25) of the kidney samples. Immunolabeling of the parasite revealed that 32% (8/25) of the bladders and 8% (2/25) of the kidneys were positive. In conclusion, the immunohistochemical technique is a useful tool for detecting amastigote forms of L. infantum in organs of infected dogs. In addition, this was the first report of detection of amastigote forms ofL. infantum in the bladders of dogs.

Objetivou-se neste estudo avaliar as alterações histológicas e realizar a imunomarcação de Leishmania infantum em rins e bexiga de cães naturalmente infectados. Vinte e cinco amostras de bexiga e rins de animais sorologicamente (ELISA S7® Biogene and IFAT ≥ 1:40 - Biomanguinhos/Fiocruz) positivas foram analisadas histologicamente e por meio da técnica de imuno-histoquímica. Os resultados revelaram cistite em 44% (11/25) das amostras de bexiga e glomerulonefrite membranoproliferativa em 92% (23/25) das amostras de rins. A imunomarcação do parasito revelou 32% (8/25) e 8% (2/25) de positividade em bexiga e rins, respectivamente. Conclui-se que a técnica de imunohistoquímica é uma útil ferramenta para detecção de formas amastigotas de L. infantum em órgãos de cães infectados. Além disso, o presente trabalho reporta a primeira descrição de formas amastigotas de L. infantum em bexiga de cães.

Glomerulopatias prevalentes na Bahia, um estudo baseado em biópsias / Glomerulopathies prevalent in Bahia, a study based on biopsies

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.

Clinicopathological features of atypical nephrotic syndrome in jamaican children / Características clínico-patológicas del síndrome nefrótico atípico en los niños jamaicanos

OBJECTIVES: To document the histopathological spectrum of atypical nephrotic syndrome in Jamaican children and to make clinicopathological correlations which will assist physicians in identifying patients needing nephrology consultation. METHODS: This was a retrospective review of renal biopsy data of Jamaican children who were referred to the University Hospital of the West Indies and the Bustamante Hospital for Children between January 1985 and December 2008. The study population consisted of children < 12 years old with atypical nephrotic syndrome. RESULTS: Biopsies were done in 157 children - 85 males and 72 females (mean age 8.91 ± 3.44 years). Indications for biopsy were steroid resistance (35%), frequent relapses (8.9%) and other atypical presentations (56.1%). Overall, mesangial proliferative glomerulonephritis (MesGN) was the commonest histology (49/157, 31.2%), followed by minimal change disease (MCD) (36/157, 22.9%) and diffuse proliferative glomerulonephritis (DPGN) (26/157, 16.6%). Infection was present in 38/157 (24%) cases. Diffuse proli ferative glomerulonephritis was the predominant type associated with streptococcal infection (52.9%) while Hepatitis B was seen in 83% ofcases ofmembranous nephropathy. CONCLUSION: Mesangial proliferative glomerulonephritis is the commonest histology seen in Jamaican children with atypical nephrotic syndrome. Most membranous nephropathy is Hepatitis B related. Hypertension with hypocomplementaemia, renal failure and anaemia are features ofmore serious renal disease (eg membranoproliferative glomerulonephritis and crescentic nephritis) rather than MCNS and should warrant urgent nephrology consultation for renal biopsy.

OBJETIVOS: Documentar el espectro histopatológico del síndrome nefrótico atípico en los niños jamaicanos y hacer correlaciones clínico-patológicas que ayuden a los médicos a identificar pacientes que necesitan la consulta de nefrología.. MÉTODOS: Se trata de un estudio retrospectivo de datos de biopsias renales de niños jamaicanos remitidos al Hospital Universitario de West Indies y al Hospital Pediátrico Bustamante, entre enero de 1985 y diciembre de 2008. La población del estudio consistió en niños < 12 años de edad que padecían el síndrome nefrótico atípico. RESULTADOS: Se realizaron biopsias a 157 niños - 85 varones y 72 hembras (edad promedio 8.91 + 3.44 años). Las indicaciones para la biopsia se debieron a resistencia a los esteroides (35%), recaídas frecuentes (8.9%) y otras manifestaciones atípicas (56.1%). En general, la glomerulonefritis proliferativa mesangial (GNMes) fue la histología más común con 49/157 (31.2%), seguida por la enfermedad de cambio mínimo (ECM) con 36/157(22.9%) y la glomerulonefritis proliferativa difusa (GNPD) con 26/157 (16.6%). La infección estuvo presente en 38/157 (24%) de los casos. La glomerulonefritis proliferativa difusa fue el tipo predominante asociado con la infección estreptocóccica (52.9%), mientras que Hepatitis B fue observada en el 83% de los casos de nefropatía membranosa. CONCLUSIÓN: La glomerulonefritis proliferativa mesangial es la histología que con mayor frecuencia se observa en los niños jamaicanos que padecen el síndrome nefrótico atípico. La mayoría de los casos de nefropatía membranosa guardan relación con la hepatitis B. La hipertensión con hipocomplementemia, la insuficiencia renal y la anemia son rasgos más bien de enfermedades renales más serias (p.ej, glomerulonefritis membranoproliferativa, nefritis crescéntica) que del síndrome nefrótico de cambios mínimos (SNCM) y debe asegurarse la consulta urgente con el nefrólogo para se realice una biopsia renal.

Pattern of renal pathology in fatal envenomation by indian cobra (naja naja).

Deaths due to poisonous snakebite are a significant health related problem especially the rural heartland of in tropical countries. Renal involvement in snakebite is well documented especially so in bites by the Viperidae group. The Elapidae family consisting of cobra and kraits among other varieties are mainly considered neurotoxic. The venom of neurotoxic variety predominantly has direct depressing action on the respiratory center and neuromuscular junction. We investigated the renal changes at autopsy and histology of fatal cobra bites. This series included autopsy examination of 14 cases of fatal cobra bite in our hospital-based study. Dissected kidneys were sectioned, stained with hematoxylin & eosin stain and histological examination was done under light microscope. Five cases from head injury subject were used as control. The study reveals renal involvement in 64.28 %of fatal bites by Indian cobra (Naja naja) primarily considered neurotoxic. The major renal changes were tubular necrosis 1(7.14%), cortical necrosis 3 (21.42%) and interstitial nephritis 3(21.42%). This fact is worth giving due consideration during management and monitoring of cases of envenomation by cobra.

Renal histological findings in adults in Jamaica / Hallazgos histológicos renales en adultos en Jamaica

BACKGROUND: In 2006, it was reported that Focal and Segmental Glomerulosclerosis (FSGS), Minimal Change Disease (MCD) and Membranous Glomerulonephritis (MGN) were the commonest primary glomerular diseases identified from percutaneous kidney biopsies done in Jamaica for that year (n = 76). The sample size was thought to be small and might have affected the reported findings. So a threeyear review of percutaneous kidney biopsies in Jamaica was carried out. METHODS: Histology reports and clinical data were reviewed for percutaneous kidney biopsies performed from January 2005 to December 2007. Demographic data (age, gender), laboratory investigations such as serum urea, serum creatinine, proteinuria, haematuria, 24-hour urinary protein, and creatinine clearance, and clinical diagnosis were collected from the histology requisition form. RESULTS: There was a total of 224 native kidney biopsies performed. There were 91 males (40.6%) and 133 females (59.4%). Age distribution showed a total number of 25 paediatric cases (11.2%) and 199 adult cases (88.8%). Proteinuria was present in 171 cases (76.3%) and haematuria in 86 cases (38.4%). Of the total biopsies done, 78 cases (39.2%) had primary glomerular diseases, 110 cases (55.3%) had secondary glomerular diseases and 11 (5.5%) biopsies were reported as either normal or inadequate for histological diagnosis. The most common reasons indicated for percutaneous kidney biopsy were proteinuria, haematuria and staging of lupus nephritis. Most common histological findings for primary glomerular disease after percutaneous kidney biopsy were FSGS (n = 34), MGN (n = 15) and MCD (n = 12). In secondary glomerular diseases (n = 110), there were more females (70.8%) than males. Systemic lupus erythematosus was present in 63.3%. Histology of lupus nephritis according to the International Society of Nephrologists classification shows Membranous Lupus Nephritis [MLN] (40.2%), Diffuse Lupus Nephritis [DLN] (19.5%) and Minimal Mesangial Lupus Nephritis [MMLN] (14.3%) as the common histological types. CONCLUSIONS: The most common histological finding for primary glomerular disease following percutaneous kidney biopsy was FSGS, MCD and MGN. Membranous Lupus Nephritis was the commonest histological type for lupus nephritis in this series.

ANTECEDENTES: En 2006, se reportó que la Glomeruloesclerosis Segmentaria y Focal (GESF), la Enfermedad de Cambios Mínimos (ECM) y la Glomerulonefritis Membranosa (GNM) fueron las enfermedades glomerulares primarias más comunes identificadas a partir de las biopsias renales percutáneas realizadas en Jamaica ese año (n = 76). El tamaño de la muestra se consideró pequeño y pudo haber afectado los hallazgos reportados. De manera que se realizó un examen de tres años, de las biopsias renales percutáneas en Jamaica. MÉTODOS: Se revisaron los reportes de histología y los datos clínicos correspondientes a las biopsias renales percutáneas realizadas desde enero de 2005 a diciembre de 2007. RESULTADOS: Hubo un total de 224 biopsias de riñón nativo. Se realizaron 74, 78 y 72 biopsias renales en 2005, 2006 y 2007 respectivamente. Hubo 91 varones (40.6%) y 133 hembras (59.4%). La distribución por edades mostró un total de 25 casos pediátricos (11.2%) y 119 casos de adultos (88.8%). La proteinuria estuvo presente en 171 casos (76.3%) y la hematuria en 86 casos (38.4%). Del total de biopsias realizadas, 78 casos (39.2%) tenían enfermedades glomerulares primarias, 110 casos (55.3%) tenían enfermedades glomerulares secundarias y 11 (5.5%) biopsias fueron reportadas como normales, o como inadecuadas para el diagnóstico histológico. Las razones más comunes señaladas para la biopsia renal percutánea fueron la proteinuria, la hematuria y la estadificación de la nefritis por lupuso nefritis lúpica. Los hallazgos histológicos más comunes para la enfermedad glomerular primaria tras la biopsia renal percutánea fueron GESF (n = 34), GNM (n = 15) y ECM (n = 12). En relación con las enfermedades glomerulares secundarias (n = 110), hubo más hembras (70.8%) que varones. El lupus eritematoso sistémico estuvo presente en 63.3%. De acuerdo con la clasificación de la Sociedad Internacional de Nefrología, la histología de la nefritis por lupus muestra la nefritis lúpica membranosa (NLM) [40.2%], la nefritis lúpica difusa (NLD) [19.5%], y la nefritis lúpica mesangial mínima (NLMM) [14.3%], como los tipos histológicos más comunes. CONCLUSIÓN: Los hallazgos histológicos más comunes para la enfermedad glomerular primaria tras la biopsia renal percutánea, fueron GESF, ECM y GNM. La nefritis lúpica membranosa fue el tipo de histología más común para la nefritis por lupus en esta serie.

Identifi cation of novel variants in the COL4A4 gene in Korean patients with thin basement membrane nephropathy.

Background & objectives: The α4 chain of the type 4 collagen family is an important component of the glomerular basement membrane (GBM) in the kidney. It is encoded by the COL4A4 gene, and mutations of this gene are known to be associated with thin basement membrane nephropathy (TBMN). To better understand the contribution of variants in the COL4A4 gene to TBMN, we investigated the sequence of the complete COL4A4 gene in 45 Korean patients with TBMN. Method: Genomic DNA was obtained from the peripheral blood lymphocytes. For the analysis of the COL4A4 gene, all the exons including splicing sites were amplififi ed by PCR and screened by direct sequencing analysis. Results: Eight novel COL4A4 sequence variants were found in these patients. Two of these variants, G199R and G1606E, were possibly pathogenic variants affecting the phenotype. None of these variants were observed in 286 chromosomes from normal Korean control subjects. In addition, 39 polymorphisms including 7 novel SNPs were identifi ed in this study. Interpretation & conclusion: The frequency of COL4A4 mutations in Korean patients with TBMN is low and the other cases may have mutations in other genes like COL4A3. Screening of the COL4A3 gene and fi nding a novel causative gene for TBMN will help clarify the pathogenesis of this disorder and perhaps for distinguishing TBMN from Alport syndrome.

Von Recklinghausen's neurofibromatosis associated with membranous glomerulonephritis

A 70-year old woman, known case of von Recklinghausen's neurofibromatosis presented with nephrotic syndrome and mild azotemia. Renal biopsy revealed membranous nephropathy. After ruling out secondary causes of membranous nephropathy, a possible coexistence of von Recklinghausen's neurofibromatosis and membranous nephropathy were thought of. This association has rarely been reported

Curso clínico de la nefropatía membranosa lúpica pura / Long-Term outcome of type V lupus membranous glomerulonephritis

Background: The long-term outcome of the pure form of WHO type V lupus membranous glomerulonephritis is apparently more benign than that of other forms of lupus glomerulonephritis. However 12percent of such patients progress to terminal renal failure. The presence of proteinuria may be an indication of cytotoxic agents. Aim: To study the clinical long-term outcome of WHO type V lupus membranous glomerulonephritis. Material and methods: A retrospective analysis of all kidney biopsies of a University Pathology Department, with the diagnosis of WHO type V lupus membranous glomerulonephritis. Review of medical records of patients with the disease and one clinical assessment of all living patients. Results: Between 1973 and 2000, 703 kidney biopsies were done to patients with systemic lupus erythematosus. Of these, 40 were membranous glomerulonephritis and in 33 patients (28 women, age range 6-71 years), data on the evolution and survival was obtained. Nineteen had type Va and the rest type Vb nephritis. Two presented with renal failure and 11 with proteinuria over 3.5 g/24h. The median follow-up since the renal biopsy was 63 months (range 1-316). At the end of follow-up, four had a creatinine clearance of less then 15 ml/h and four a clearance between 15 and 29 ml/h (one of these received a renal allograft). Eleven (33percent) patients had died, mostly due to infections. Life expectancy at five years with a creatinine clearance over 15 ml/h was 75percent. Bad prognostic factors were an elevated creatinine clearance over 15 ml/h was 75percent. Bad prognostic factors were an elevated creatinine and high blood pressure at the moment of the biopsy. Conclusions: The clinical outcome of these patients was bad. Twelve percent reached a stage of terminal renal failure. This is in contrast with the 3percent progression to a similar stage of proliferative glomerulonephritis treated with i.v. cyclophosphamide. New therapies for this condition must be sought.

The role of electron microscopy for the diagnosis of glomerulopathies

CONTEXTO: A microscopia eletrônica tem sido usada há mais de três décadas para o diagnóstico morfológico das doenças glomerulares e seu valor tem sido amplamente enfatizado. Entretanto, relatos recentes têm analisado criticamente o uso rotineiro da microscopia eletrônica. O seu uso em outras áreas de diagnóstico como doenças tumorais tem declinado consideravelmente. Além disso, em virtude da inevitável pressão financeira para redução dos custos da investigação na rotina diagnóstica, a seleção dos casos para microscopia eletrônica tem sido rigorosa. OBJETIVO: Com o intuito de se identificarem as doenças glomerulares que dependem da microscopia eletrônica para o diagnóstico final, foram revisadas biópsias renais recebidas no período de 12 anos. TIPO DE ESTUDO: Prospectivo LOCAL: Hospital Ana Costa, Hospital Guilherme Alvaro e Serviço de Anatomia Patológica de Santos, São Paulo, Brasil. PARTICIPANTES: 200 biópsias renais consecutivas, obtidas de hospital privado e hospital-escola de 1979 a 1991. PRINCIPAIS VARIAVEIS: Todos os casos foram analisados por microscopia óptica, imunofluorescência e microscopia eletrônica. O diagnóstico foi inicialmente feito por microscopia óptica e de imunofluorescência, e posteriormente pela microscopia eletrônica. RESULTADOS: A microscopia eletrônica foi diagnóstica ou essencial para o diagnóstico em 10,0% dos casos, correspondendo a 3,4% de glomerulopatias primárias e 100% das glomerulopatias hereditárias. A microscopia eletrônica foi contributiva para o diagnóstico em 5,5% dos casos, confirmando os diagnósticos formulados com base em dados clínicos e laboratoriais, e achados de microscopia óptica e de imunofluorescência. Obtivemos 7,5% de imunofluorescências discordantes, assim consideradas quando os achados de imunofluorescência não foram confirmados pela microscopia eletrônica. Em 77,0% dos casos, o diagnóstico final pôde ser estabelecido exclusivamente com base nos achados de microscopia óptica e de imunofluorescência. CONCLUSÕES: Foi possível diagnosticar com exatidão grande porcentagem (82,5 - 90,0%) dos casos com base nos achados isolados de microscopia óptica e de imunofluorescência. A microscopia eletrônica foi essencial para o diagnóstico das nefropatias hereditárias.

Membranous Nephropathy after Allogeneic Hematopoietic Stem Cell Transplantation in a Patient with Aplastic Anemia: A Case Report

Nephrotic syndrome has been described as one of the clinical forms of chronic graft-versus-host disease (cGVHD), but a limited number of cases have been described. We experienced a young female patient with nephrotic syndrome developed 22 months after allogeneic hematopoietic stem cell transplantation (HSCT) for severe aplastic anemia. She had been well after successful management for gut-limited cGVHD until she developed a clinical nephrotic syndrome with hypoalbuminemia of 2.0 g/dL and 24-hr urine protein of 6.88 g/dL. On physical examination and laboratory findings, there was no other evidence of cGVHD. Clinical and renal biopsy findings were consistent with cGVHD-related membranous nephropathy, and immunosuppressive agents with cyclosporine and prednisone were prescribed. After 3 month of treatment, the proteinuria decreased to normal range; and the patient from nephrotic syndrome nearly recovered. We recommend cGVHD-related glomerulonephritis should be considered in all patients with hypoalbuminemia following allogeneic HSCT, even if there is no other evidence of clinical GVHD.

Covert bacteriuria in nephrotic syndrome.

Prevalence of covert bacteriuria in patients of nephrotic syndrome admitted for kidney biopsy was studied in 205 patients. Age ranged from 10 years to 65 years. 148 patients were male and 57 were female. Prevalence of covert bacteriuria was found in 38 patients (18.53%). In bacteriuric patients 30 were male, 8 were female. Escherichia coli was the commonest organism grown in bacteriuric patients (30 or 78.9%). Serum albumin was low and 24-hour urinary protein excretion was high in bacteriuric patients in comparison to abacteriuric patients. In bacteriuric patients membranous nephropathy was the commonest histopathological finding present in 15 patients (39.47%).

Glycosuria in glomerular diseases: histopathology and clinical correlations

There are doubts about the presence of glycosuria and the progress of glomerular disease. Some reports suggest that glycosuria could be an index of a more severe tubulointerstitial lesion. We investigated the presence of glycosuria in 60 patients with primary glomerular diseases: 17 patients (28 percent) and glycosuria and 43 patients (72 percent) were glycosuria free. The two groups were similar in age, arterial pressure and sex. Serum creatinine was higher in patients with glycosuria (2.0 + 1.7 vs 1.3 + 0.9 mg/dl, P<0.05). The protein excretion rate was 7.5 + 3.7 vs 5.3 + 4.2g/day (P>0.05) in patients with and without glycosuria, respectively, while serum albumin was lower in patients with glycosuria (1.7 + 0.6 vs 2.7 + 1.0 g/dl, P<0.05). Several histological forms were present in the group with glycosuria, with membranous glomerulonephritis being the most frequent. Histological evidence of tubular atrophy and interstitial fibrosis prevailed in patients with glycosuria, suggesting a poor prognosis for these patients. We may conclude that the presence of glycosuria in patients with glomerular disease is associated with more pronounced tubular atrophy and interstitial fibrosis and therefore imply a poorer prognosis.