Renal Amyloidosis Secondary to ANCA-Associated Vasculitis: A Case Report / 中国医学科学杂志(英文版)

Renal amyloidosis secondary to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare. Here, we reported a 77-year-old woman with ANCA-associated vasculitis. Renal biopsy with Masson trichrome staining showed pauci-immune crescentic glomerulonephritis, and electron microscopy showed amyloid deposition in the mesangial area. Immunofluorescence revealed kappa light chain and lambda light chain negative. Bone marrow biopsy revealed no clonal plasma cell. Finally, she was diagnosed as ANCA-associated vasculitis with secondary renal amyloid A amyloidosis.

Nefropatía por C1q: una rara variedad de síndrome nefrótico: caso clínico / C1q nephropathy: report of one case

C1q nephropathy is a rare glomerulopathy characterized by mesangial deposition of the complement component C1q. These deposits can be isolated or associated with immunoglobulins or complement fractions, which are observed by immunofluorescence or immunohistochemical microscopy. In ultramicroscopy, dense mesangial deposits and alterations of the podocyte are observed. Clinically it presents as a nephrotic syndrome (NS) or by alterations of the urinalysis such as proteinuria and/or hematuria in children and young adults. In light microscopy, it is expressed with a morphological pattern of minimal change disease (MCD), mesangial proliferative glomerulonephritis or focal segmental glomerulosclerosis (FSGS). The NS during its evolution usually evolve in steroid resistance or steroid dependency, often requiring the association of immunosuppressants to obtain remission. We report a 14 years old male with a history of NS and its evolution under various treatments during a 12-year follow-up.

Glomerulonefritis rápidamente progresiva de etiología posinfecciosa. Caso pediátrico / Postinfectious rapidly progressive glomerulonephritis in a pediatric patient

La glomerulonefritis rápidamente progresiva de etiología posinfecciosa es rara en la infancia, con una prevalencia estimada del 1-3 %. La mayoría debuta como insuficiencia renal aguda y su tratamiento se basa en el uso de corticoides y ciclofosfamida. Si se realiza diagnóstico precoz, el 70 % presenta una recuperación temprana de la función renal. En los últimos años, se han descrito "glomerulopatías por C3", que presentan características que se superponen. Son útiles, en el diagnóstico diferencial, la inmunofluorescencia y la determinación del factor nefrítico. Se presenta un varón de 4 años que acude por fiebre y cuadro respiratorio. Se observa microhematuria, proteinuria, descenso de filtrado glomerular y descenso de C3, y se sospecha glomerulonefritis aguda. Se realiza una biopsia, cuya microscopía óptica muestra la presencia de semilunas epiteliales, y la electrónica, depósitos subepiteliales en forma de joroba, por lo que se diagnostica glomerulonefritis rápidamente progresiva de etiología posinfecciosa.

Postinfectious glomerulonephritis is rarely presented as rapidly progressive glomerulonephritis in children; the prevalence is approximately 1-3 %. Most children have acute onset of renal failure; initial treatment involves corticosteroids and immunosuppressive therapy. Early diagnosis improves prognosis. In recent years, an entity known as "C3 glomerulopathies" has been described, presenting characteristics that overlap. In the differential diagnosis, the immunofluorescence and the determination of the nephritic factor are useful. We report a 4-year-old boy with fever, respiratory symptoms and hyporexia. Microhematuria, proteinuria, decline in glomerular filtration and depressed C3 were found. Acute glomerulonephritis was suspected. Renal biopsy showed crescent formation, immunofluorescence staining for C3 and subepithelial humps. Therefore, postinfectious glomerulonephritis with crescent formations was diagnosed.

Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement / Glomerulonefrite rapidamente progressiva associada a ANCA em uma criança com acometimento renal isolado

ABSTRACT Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis.

RESUMO A glomerulonefrite (GN) pauci-imune é mais comum em idosos em comparação com crianças, e a etiologia ainda não é completamente compreendida. A positividade do anticorpo citoplasmático antineutrófilo (ANCA) ocorre em 80% dos pacientes. Relatamos o caso de uma menina de 7 anos de idade que apresentou mal-estar e creatinina discretamente elevada, diagnosticada como glomerulonefrite rapidamente progressiva pauci-imune associada a ANCA com crescentes em 20 dos 25 glomérulos (80%). Destes 20 crescentes, 12 eram celulares, 4 fibrocelulares e 4 globalmente escleróticos. Ela não apresentava púrpura, artrite ou sintomas sistêmicos e respondeu bem ao tratamento imunossupressor inicial, apesar da histopatologia relativamente grave. A paciente recebeu três pulsos de metilprednisolona intravenosa (30 mg/kg em dias alternados) inicialmente e continuou com ciclofosfamida (2 mg/kg por dia) por via oral durante 3 meses com prednisona (1 mg/kg por dia). Em um mês, a remissão foi alcançada com creatinina sérica normal e a prednisona foi gradualmente reduzida. O caso desta criança com uma doença pediátrica relativamente rara enfatiza a importância do tratamento imunossupressor precoce e agressivo em pacientes com GN rapidamente progressiva renal associada à ANCA, mesmo com uma apresentação clínica leve. Como em nossa paciente, os achados clínicos e laboratoriais podem nem sempre refletir exatamente a gravidade da histopatologia renal e, assim, a biópsia renal é obrigatória nessas crianças para orientar a conduta clínica e auxiliar no prognóstico.

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.

Síndrome pulmón-riñón: serie de 12 casos / Pulmonary renal syndrome: series of 12 cases

El síndrome pulmón-riñón es una entidad infrecuente, que comprende un gran espectro de patologías, como las vasculitis asociadas a ANCA y la enfermedad por anticuerpos antimembrana basal glomerular entre otras. Se describen en esta serie 12 casos donde las entidades más prevalentes fueron las antes mencionadas, observándose además un caso de lupus y uno de granulomatosis con poliangeítis, que se encuentran dentro de las causas menos frecuentes. La forma de presentación clínica inicial fue simultánea renal y pulmonar en 5/12 pacientes y renal en 7/12 de los mismos. El diagnóstico temprano de dichas patologías basándose en criterios clínicos, radiológicos, de laboratorio e histológicos, permite instaurar terapéuticas tempranas como la inmunosupresión y plasmaféresis, pudiendo prevenir complicaciones tales como las infecciones y la insuficiencia renal crónica terminal, siendo las primeras la principal causa de muerte (AU)

Pulmonary-renal syndrome is an infrequent condition. It includes a wide variety of conditions such as ANCA (antineutro-phil cytoplasmic autoantibody) associated with systemic vasculitis and anti-GBM (anti-glomerular basement membrane) disease among others. In this series we describe twelve cases, in which the most prevalent diseases were the above mentioned as well as one case of lupus and one of granulomatosis with polyangiitis (these being less frequent causes). The clinical presentation was both renal and pulmonary simultaneously in five of twelve patients and renal in seven of twelve patients. Early diagnosis of this condition on the basis of clinical, radiological, histological and analytic criteria allows early treatments such as immunosuppression and plasma exchange, thus avoiding complications such as infections (the main cause of death) and terminal chronic renal failure (AU)

Encefalopatía posterior reversible en el curso de una emergencia hipertensiva, serie de cuatro niños hospitalizados en el Centro Hospitalario Pereira Rossell / Posterior reversible encephalopathy during a hypertensive emergency in 4 children hospitalized at the Pereira Rossell Pediatric Hospital / Encefalopatia reversível durante uma emergência hipertensiva em 4 crianças hospitalizadas no Centro Hospitalar Pereira Rossell

Introducción: la encefalopatía posterior reversible (PRES) es un complejo sindromático caracterizado por cefaleas, crisis epilépticas, trastornos visuales y alteración del sensorio asociado a cambios en la sustancia blanca de lóbulos occipitales y parietales en la resonancia nuclear magnética. Se relaciona con diversas causas que provocan edema cerebral, entre las que se destaca la hipertensión arterial (HTA) y los tratamientos inmunosupresores. Descripción de casos: se incluyeron cuatro niños hospitalizados entre el 1 de enero de 2005 y el 31 de diciembre de 2015 en el Centro Hospitalario Pereira Rossell. La mediana de edad fue 11,5 años. La manifestación clínica inicial en tres de los cuatro pacientes fue disnea y edemas generalizados. Todos presentaron hipertensión endocraneana y convulsiones generalizadas. La tomografía computada fue la primera imagen solicitada en todos los pacientes, evidenciado lesiones hipodensas córtico-subcorticales occipitales, parietales y frontales. Se realizó resonancia magnética en tres casos con hallazgos característicos. Todos presentaron síndrome nefrítico con cifras de tensión arterial mayores al P99, insuficiencia renal reversible, disminución de C3 y aumento del antiestreptolisina O (AELO). El control de HTA requirió politerapia en unidad de cuidado crítico. Ninguno presentó secuelas neurológicas. Conclusiones: la PRES es una asociación o manifestación poco habitual de la emergencia hipertensiva. Es necesaria la sospecha e identificación por parte del pediatra con el fin de realizar un abordaje terapéutico adecuado y precoz.

Introduction: posterior reversible encephalopathy (PRES) is a complex syndrome characterized by headaches, epileptic seizures, visual disturbances and sensorial alterations associated to changes of white matter in parieto-occipital regions shown in magnetic nuclear resonance. It is linked to various causes that provoke cerebral edema including arterial hypertension (AH) and immunosuppressive treatments. Clinical cases: 4 children admitted between January 1st, 2005 and December 31st, 2015 at the Pereira Rossell Pediatric Hospital. Median age: 11.5 years. The initial clinical manifestation in 3 of 4 patients was dyspnea and generalized edema. All of them presented endocranial hypertension and generalized convulsions. CT was the first requested image to all patients, and it was clear that all of them had experienced occipital cortical-subcortical, parietal and frontal hypodense lesions. In three of the cases we performed a Magnetic Resonance that showed typical findings. All of them presented nephritic syndrome with arterial hypertension over P99, reversible renal failure, a decrease of C3 and an increase of Antistreptolysin O (AELO). HTA control required polytherapy at the critical care unit. None of them presented neurological sequelae. Conclusions: PRES is an association or unusual manifestation of hypertensive emergency. Early detection by pediatricians seems essential to provide an appropriate therapeutic approach.

Introdução: a encefalopatia posterior reversível (PRES) é uma síndrome complexa caracterizada por dores de cabeça, convulsões epilépticas, distúrbios visuais e alterações sensoriais associadas à mudança da substância branca nas regiões parieto-occipitais observadas na ressonância magnética nuclear. Está ligada a várias causas que provocam o edema cerebral, incluindo hipertensão arterial (HA) e tratamentos imunossupressores. Casos clínicos: 4 crianças internadas entre 1º de janeiro de 2005 e 31 de dezembro de 2015 no Hospital Pediátrico Pereira Rossell. Idade mediana: 11,5 anos. A manifestação clínica inicial em 3 dos 4 pacientes foi dispneia e edema generalizado. Todos apresentaram hipertensão endocraniana e convulsões generalizadas. A TC foi a primeira imagem solicitada e todos os pacientes mostraram lesões occipitais corticais-subcorticais, parietais e hipodensas frontais evidentes. Em três dos casos, a Ressonância Magnética mostrou achados típicos. Todos apresentaram síndrome nefrítica com hipertensão arterial acima de P99, insuficiência renal reversível, diminuição de C3 e aumento de antiestreptolisina O (AELO). O controle de HTA requereu politerapia na unidade de terapia intensiva. Nenhum dos pacientes apresentou sequelas neurológicas. Conclusões: A PRES é uma associação ou manifestação de emergência hipertensiva pouco comum. A detecção precoce do pediatra é essencial para fornecer uma abordagem terapêutica adequada.

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.

Glomérulonefritis en infección por Streptococcus pneumoniae en pediatría, análisis de caso clínico / Glomerulonephritis in Streptococcus pneumoniae infection in pediatrics, clinical case analysis

Introducción: la glomerulonefritis postestreptocócica (GNPE) es causada, en general, por una infección previa por Streptococcus pyogenes. Se produce por enfermedad glomerular por complejos inmunes inducida por la infección. La presentación clínica de la GNPE es variable, llegando a síndrome nefrítico. El diagnóstico se basa en la clínica de nefritis, asociada a la confirmación de la infección por laboratorio. No existe un tratamiento específico, por lo que el manejo consta de medidas de soporte y tratamiento sintomático, especialmente de la hipertensión arterial y edema. La resolución del cuadro es, en general, rápida y el pronóstico es bueno. Caso clínico: En el presente trabajo se expone un caso clínico pediátrico, de un paciente de 4 años, con sepsis de origen faringoamigdaliano por Streptococcus pneumoniae, asociada a pansinusitis con celulitis preseptal y a glomerulonefritis con síndrome nefrótico impuro. Discusión: En el caso clínico expuesto, el paciente manifiesta una presentación atípica de la glomerulonefritis post-estreptocócica, que además fue producida por Streptococcus pneumoniae, a diferencia de la mayoría de los casos de esta enfermedad. Destacamos la importancia de la sospecha diagnóstica, los análisis de laboratorio dirigidos a detectar la causa y el manejo de estos casos de manera multidisciplinaria.

Introduction: poststreptococcal glomerulonephritis (PSGN) is frecuently caused by a prior infection with Streptococcus pyogenes. It is generated by a inmune complex glomerular disease induced by the streptococcal infection. The clinical presentation of PSGN varies from asymptomatic, to microscopic hematuria to nephritic syndrome. The diagnosis is based on clinical findings of nephritis, associated to confirmation of infection with laboratory tests. There is no specific treatment for PSGN, management is based on support care and symptomatic treatment, with emphasis on controling hypertension and edema. The resolution is, in general terms, fast and with favorable prognosis. Case report: On the present work we expose a pediatric clinical case, of a four year old patient, who had sepsis of tonsil-pharyngeal origin caused by Streptococcus pneumoniae, associated with pansinusitis with presepta lcellulitis and atypical neprhotic syndrome due to glomerulonephritis. Discussion: The case shows an atypicalpresentation of poststreptococcal glomerulonephritis, produced by Streptococcus pneumoniae, in contrast to the most frecuent cases of this disorder. We would like to emphasize the importance of the suspecting the diagnosis, directed laboratory tests to detect the cause, and multidisciplinary management.

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.

Malignant Hypertension with an Unusual Presentation Mimicking the Immune Mediated Pulmonary Renal Syndrome

A 27-year-old man presented at the emergency room with hemoptysis. His blood pressure was 180/100 mm Hg, and he had no history of hypertension. Chest radiographs showed bilateral infiltration, suggestive of alveolar hemorrhage. His laboratory data were consistent with acute kidney injury. His serum creatinine level increased abruptly; therefore, renal biopsy was performed. Steroid pulse therapy was administered because of a strong suspicion of immune-mediated pulmonary renal syndrome. Renal biopsy showed proliferative endarteritis, fibrinoid necrosis, and intraluminal thrombi in the vessels without crescent formation or necrotizing lesions. Steroid pulse therapy rapidly tapered and stopped. His serum creatinine level gradually decreased with strict blood pressure control. Ten months after discharge, his blood pressure was approximately 120/80 mm Hg with a serum creatinine level of 1.98 mg/dL. Pulmonary renal syndrome is generally caused by an immune-mediated mechanism. However, malignant hypertension accompanying renal insufficiency and heart dysfunction causing end-organ damage can create a pulmonary hemorrhage, similar to pulmonary renal syndrome caused by an immune-mediated mechanism. The present case shows that hypertension, a common disease, can possibly cause pulmonary renal syndrome, a rare condition.

Waldenstrom Macroglobulinemia with CD5+ Expression Presented as Cryoglobulinemic Glomerulonephropathy: A Case Report

Waldenstrom macroglobulinemia (WM) is a B-cell lymphoproliferative disorder associated with bone marrow involvement of lymphoplasmacytic lymphoma (LPL) and an IgM monoclonal gammopathy. Generally B-lymphocytes in LPL do not express CD5 that is important for differential diagnosis of B-cell lymphoproliferative disorders. In WM, various renal diseases and type I cryoglobulinemia are well described separately, but cryoglobulinemic glomerulonephropathy is very rarely reported. A 61-yr-old woman complained of generalized edema, cyanosis of the extremities in cold weather, visual disturbance, and pancytopenia. Bone marrow and renal biopsy showed CD5+ expressing B-cells and cryoglobulinemic glomerulonephropathy. With the diagnosis of WM, she received cyclophosphamide, doxorubicin, vincristine and prednisolone chemotherapy and got complete remission. Here, we report a rare case of WM associated with unusual expression of CD5+ B-lymphocytes and cryoglobulinemic glomerulonephropathy, and emphasize the importance of the clinical features in differentiating CD5+ B-cell lymphoproliferative disorders.

Glomerulonephritis in schistosomiasis mansoni: a time to reappraise / Glomerulonefrite na esquistossomose mansônica: um tempo para reavaliar

INTRODUCTION: The current prevalence of glomerulonephritis in patients with hepatosplenic schistosomiasis mansoni in Brazil was evaluated. METHODS: Sixty three patients (mean age 45.5±11 years) attending the outpatient infectious disease clinic of a University Hospital in Belo Horizonte, Brazil, from 2007 to 2009, were consecutively examined and enrolled in the present investigation. Diagnosis of hepatosplenic schistosomiasis was based on epidemiological, clinical and parasitological data and imaging techniques. Eight patients, who presented >30mg/day albuminuria, were submitted to percutaneous ultrasound guided renal biopsy. Kidney tissue fragments were examined under light, direct immunofluorescence and electron microscopy. RESULTS: All patients showed mesangial enlargement. In five, mesangial hypercellularity was observed and four presented duplication of the glomerular basement membrane. Areas of glomerular sclerosis were diagnosed in four. Deposits of immunoglobulin M and C3 were present in six samples; deposits of IgG in four, IgA in three and C1q in two samples. In all patients, immunoglobulin A was reported in the lumen of renal tubules. Deposits of kappa and lambda were observed in six samples. Electron microscopy revealed dense deposits in the glomerular tissue of three patients. Arterial hypertension, small esophageal varices, slight increases in serum creatinine and decreases in serum albumin were associated with glomerular disease. CONCLUSIONS: Renal disease associated with hepatosplenic schistosomiasis was verified in 12.7 percent of patients and type I membranoproliferative glomerulonephritis was observed in 50 percent of them. Schistosomal glomerulopathy still is an important problem in patients with hepatosplenic schistosomiasis in Brazil.

INTRODUÇÃO: Avaliou-se a frequência de glomerulonefrite em pacientes com esquistossomose hepatosplênica no Brasil. MÉTODOS: Selecionou-se para o estudo, 63 pacientes (idade média de 45,5±11 anos) avaliados consecutivamente no ambulatório de doenças infecciosas de um hospital universitário de Belo Horizonte, Brasil, no período de 2007 a 2009. O diagnóstico da esquistossomose foi baseado em dados epidemiológicos, clínicos, parasitológicos e de imagem. Os oito pacientes que apresentaram albuminúria acima de 30mg em 24 horas submeteram-se a biópsia renal percutânea dirigida por ultrassonografia. As amostras de tecido renal foram analisadas à microscopia óptica, eletrônica e de fluorescência direta. RESULTADOS: Havia expansão do mesângio em todos. Em cinco, houve proliferação de células mesangiais e em quatro observou-se duplicação da membrana basal glomerular. Áreas de esclerose glomerular foram diagnosticadas em quatro. Depósitos de imunglobulinas M e C3 foram patentes em seis amostras; IgG em quatro, IgA em três e C1q em duas. Em todos os pacientes relatou-se fluorescência para IgA dentro dos túbulos renais. Depósitos de kappa e lambda foram vistos em seis amostras. A microscopia eletrônica demonstrou depósitos eletrondensos em tecido glomerular. A presença de hipertensão arterial, varizes do esôfago de pequeno calibre, pequenos aumentos de creatinina e diminuição de albumina sérica associaram-se à ocorrência de dano renal. CONCLUSÕES: A frequência de lesão renal foi de 12,7 por cento, no presente estudo, e a glomerulonefrite membranoproliferativa do tipo I foi encontrada em 50 por cento. A lesão renal associada à esquistossomose permanece um problema importante no Brasil.

Acute glomerulonephritis after upper airway or skin infection: descriptive analysis of 82 cases between 14 and 64 years-old / Glomerulonefrite aguda após infecção de vias aéreas superiores ou pele: análise descritiva de 82 pacientes entre 14 e 64 anos de idade

INTRODUÇÃO: A glomerulonefrite aguda (GNA) após infecção de vias aéreas superiores ou pele é uma doença renal causada geralmente por cepas estreptocócicas nefritogênicas, podendo cursar com quadro súbito de hematúria macroscópica, hipertensão arterial, edema e, ocasionalmente, insuficiência renal aguda, sendo comum na infância e pouco incidente em adultos e indivíduos mais jovens. OBJETIVO: Analisar, de forma descritiva, os dados da apresentação inicial da GNA após infecção de vias aéreas superiores ou pele em pacientes com mais de 14 anos de idade, com ênfase em seus aspectos epidemiológicos e clínicos. PACIENTES E MÉTODOS: Foram analisados os dados clínicos de 82 pacientes, atendidos em nosso Serviço no período de 1972 a 2001, distribuídos em três grupos etários: grupo 1, com indivíduos entre 14 e 20 anos (n = 52); grupo 2, entre 21 e 30 anos (n = 19); e grupo 3, com idade > 31 anos (n = 11). RESULTADOS: Houve um predomínio do quadro entre pacientes mais jovens (grupo 1), do sexo masculino e da cor branca, precedido, principalmente, por infecção de pele, manifestando-se mais comumente por edema de membros inferiores e/ou face. Em alguns casos, até com síndrome nefrótica, e hipertensão arterial, sobretudo nos adultos com mais de 30 anos (grupo 3), sendo menos frequente o achado de hematúria macroscópica e, raramente, de insuficiência renal aguda. CONCLUSÃO: Nossos achados ressaltam a importância de se estudar a GNA após infecção de vias aéreas superiores ou pele em indivíduos mais jovens e adultos, procurando melhor caracterizar seus aspectos clínicos, sobretudo por se tratar de um grupo de pacientes no qual a doença é menos incidente.

INTRODUCTION: Acute glomerulonephritis (AGN) after infection of the upper airways or skin is a kidney disease usually caused by streptococcal nephritogenic strains and may present with sudden onset of gross hematuria, hypertension, edema and, occasionally, acute renal failure, is common in childhood and little incident in adults and younger individuals. OBJECTIVE: To analyze, in a descriptive way, data from the initial presentation of GNA after infection of the upper airways or skin in patients over 14 years of age, with emphasis on its epidemiological and clinical aspects. PATIENTS AND METHODS: We reviewed the clinical data of 82 patients treated at our department during the period 1972-2001, divided into three groups: group 1, with individuals between 14 and 20 years (n = 52), group 2, between 21 and 30 years (n = 19) and group 3, aged >; 31 years (n = 11). RESULTS: There was a predominance of the table among younger patients (group 1), male and white, mostly preceded by infection of the skin, appearing most commonly on lower extremity edema and/or face. In some cases, even with nephrotic syndrome, and hypertension, especially in adults over 30 years (group 3), being the least frequent finding of gross hematuria, and rarely, acute renal failure. CONCLUSION: Our findings underscore the importance of studying the AGN after infection of the upper airways or skin in younger individuals and adults, seeking to better characterize its clinical, mainly because it is a group of patients where the disease is less incident.

Alpha1-microglobulin: a marker for early detection of tubular disorders in diabetic nephropathy

Tubular damage as suggested by tubular proteinuria is a recognised feature of glomerulonephritis. The objectives of the study were to compare the level of alpha-microglobulin in normal and diabetic patients, and also to find out whether the level of alpha-1 microglobulin could become a laboratory marker for tubulo-interstitial damage in diabetic nephropathy. Twenty-nine registered Type II diabetic patients of either sex were studied. The patients' age ranged from 41-50 years who were admitted in the medical ward, and those who visited the outdoor department of Sir Ganga Ram Hospital, Lahore were included in the study. The duration of study was one year from June 2006 to June 2007. Ten normal subjects with no history of diabetes were taken as controls. Blood samples and 24 hour urine samples of patients of all groups were collected. The levels of urinary protein and blood sugar were estimated by auto analyser. Proteinuria positive urinary samples were analysed by SDS-PAGE electrophoresis. The level of alpha-1 urinary protein was significantly increased in the group of diabetic patients as compared to the urinary protein level of normal subjects. Blood sugar level was also significantly increased in patients as compared to controls. Level of low molecular weight protein alpha-1 microglobulin showed an electrophoresis band of 28 Kda with an average volume of 6741.88 in the urine sample of patients. On the other hand, a very light, hardly recognizable band was observed in normal subjects. Urinary alpha-1 microglobulin provides a noninvasive and inexpensive diagnostic alternative for the diagnosis and monitoring of urinary tract disorders, i.e., early detection of tubular disorders of diabetic nephropathy. We propose that SDSPAGE electrophoresis is a comparatively inexpensive diagnostic approach to detect this marker in the urine sample

Spectrum of glomerular diseases at King Hussein medical center

To determine the histopathological patterns of glomerulonephritis according to the clinical presentation. This is a retrospective analysis of light microscopy results of native kidney biopsies done during the period of January 1[st], 2005 until December 31[st], 2008. There were 273 native kidney biopsies performed during this period. Data were collected from the computer data base of Princess Iman Research and Laboratory Center, King Hussein Medical Center, Amman, Jordan. All biopsies were examined by our renal histopathologist. The most common indication was nephrotic syndrome and the most common cause of nephrotic syndrome in our patients was membranous glomerulonephritis. The main cause of subnephrotic proteinuria was minimal change disease and focal and segmental glomerulosclerosis. Membranoproliferative glomerulonephritis was the most frequent finding in patients presenting with microscopic hematuria. In acute nephritis the most common lesions were crescentic, diffuse proliferative and necrotizing glomerulonephritis. Acute tubular necrosis was the most common cause of acute kidney injury. Changes of end stage kidney disease were the most frequent findings in patient with chronic kidney disease. In patients with systemic lupus erythematosus with renal involvement, the most common lesion was class IV lupus nephritis. Kidney biopsy is an extremely helpful investigation and it should be performed once indicated. There is a need for a national registry of kidney biopsies. The histopathological findings are similar to other studies done in Jordan and in the neighboring countries