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.

Glomerulopatía colapsante en lupus eritematoso sistémico con respuesta positiva a tratamiento con mofetil micofenolato / Collapsing Glomerulopathy in systemic lupus erythematosus with positive response to the treatment with mycophenolate mofetil

La glomerulonefritis colapsante es una enfermedad poco frecuente que puede estar asociada a distintas causas, una de ellas son las enfermedades autoinmunes y dentro de estas el lupus eritematoso sistémico (LES). De manera frecuente se presenta con un cuadro de severas alteraciones renales que tienden a progresar la enfermedad renal terminal, con escasa respuesta a los tratamientos. Se presenta un caso de glomerulonefritis colapsante asociado a lupus eritematoso sistémico que tuvo una respuesta completa al tratamiento de inducción con la combinación de glucocorticoides, antimaláricos y mofetil micofenolato iniciado precozmente por el diagnóstico temprano realizado por biopsia renal(AU)

Collapsing glomerulonefritis is a slightly frequent disease that can be associated to different causes. Autoimmune diseases are part of those, and inside these, the systemic lupus erythematosus (SLE). It frequently appears with manifestations of severe renal alterations that tend to develop the renal terminal disease, with scanty response to the treatments. It is presented a case of collapsing glomerulonefritis associated to systemic lupus erythematosus that had a complete response to the treatment of induction with the combination of glucocorticoids, antimalarials and mycophenolate mofetil used prematurely after the early diagnosis performed by renal biopsy(AU)

A 'Full House' Glomerulopathy in a Patient with Multiple Lentigines Syndrome: A Case Report / Glomerulopatía 'full house' en un paciente con síndrome de lentigos múltiples: un reporte de caso

ABSTRACT Multiple lentigines syndrome (MLS) is an autosomal dominant disease which is usually diagnosed clinically by the presence of characteristic features. The molecular genetic testing is an adjuvant diagnostic tool to identify the mutation of particular genes such as PTPN11 genes, RAF1, BRAF or MAP2K1 genes. This syndrome was formerly known as LEOPARD syndrome or Noonan syndrome with multiple lentigines. 'LEOPARD syndrome ' is an acronym of characteristic features (Lentigines, Electrocardiographic conduction abnormalities, Ocular hypertelorism, Pulmonary stenosis, Abnormalities of the genitalia, Retardation of growth, and Deafness). There was no previous case report about any glomerulonephropathy in association with MLS. We present a case of a patient with MLS with recurrent nephrotic syndrome who was found to have histologic evidence of 'full house ' glomerulopathy.

RESUMEN El síndrome de lentigos múltiples (SLM) es una enfermedad autosómica dominante que de modo general se diagnostica clínicamente por la presencia de rasgos característicos. La prueba genética molecular es una herramienta de diagnóstico auxiliar utilizada para identificar la mutación de genes específicos tales como los genes PTPN11, RAF1, BRAF, o los genes MAP2K1. Este síndrome se conocía anteriormente como síndrome del leopardo o síndrome de Noonan con múltiples lentigos. El síndrome toma su nombre del acrónimo en inglés LEOPARD, que describe sus rasgos característicos (L lentigos; E conducción electrocardiográfica de las anormalidades; O hipertelorismo ocular; P estenosis pulmonar; A anormalidades de los genitales; R retardo del crecimiento; y D deafness, 'sordera ' en inglés), y que fuera introducido por Gorlin et al en 1969. No existía ningún reporte de caso anterior sobre glomerulonefropatía asociada con SLM. Presentamos el caso de un paciente con SLM con síndrome nefrótico recurrente en el que se halló evidencia histológica de glomerulopatía 'full house'.

Glomerulonefritis con semilunas / Glomerulonephritis with crescents

La glomerulonefritis rápidamente progresiva (GNRP) es una entidad caracterizada por una brusca y progresiva declinación de la función renal y por la presencia en la biopsia renal de proliferación celular extra capilar (semilunas) que ocupan el espacio de Bowmans. Nosotros analizamos en forma retrospectiva 37 niños con diagnóstico de GNRP (50% o más de los glomérulos con semilunas) asistidos en esta institución durante los últimos 20 años. El propósito fue evaluar la presentación clínica e histopatológica, etiología, evolución y factores de mal pronóstico. La edad media al diagnóstico fue de 11 ± 3,5 años. Los síntomas de presentación fueron: hematuria 100% de los casos (hematuria macroscópica 56%); hipertensión arterial 92%; proteinuria 88%; síndrome nefrótico 57%. Fue necesaria diálisis al ingreso en el 64,1% de los casos. Las biopsias renales fueron realizadas a 38 ± 26 días desde el comienzo de los síntomas. El porcentaje de glomérulos que presentaron semilunas fue del 81,4%; las mismas fueron epiteliales en el 28,3% de los casos; fibroepiteliales en el 21,8% y fibrosas en el 31,3%. En el 75,8% de las biopsias se encontró fibrosis intersticial y atrofia tubular moderada y/o severa. La inmunofluorescencia no mostro depósitos de complejos inmunes (GN pauci-inmune) en el 40,6% de las biopsias, mostró depósitos granulares de complejos inmunes en el 48,6% y depósitos lineales de anticuerpos anti membrana basal glomerular (Goodpasture´s) en el 10,8%. El tratamiento fue iniciado a 36 ± 32 días desde el comienzo de los síntomas. Todos los pacientes recibieron tratamiento de sostén; en 29 de ellos se indicaron además esteroides y ciclofosfamida, y en 5 solo esteroides. El tiempo medio de seguimiento fue de 4,6 ± 3,9 años. La sobrevida de los pacientes al final del seguimiento fue del 87% (IC95% 55-97%) y la sobrevida del órgano fue del 17% (IC95% 7-38%). Por análisis multivariado encontramos que la fibrosis intersticial y atrofia tubular moderada y/o severa fue el único factor que se relacionó con pérdida del órgano (OR: 14,6 IC95%2,6-80) p= 0,001. Nuestros resultados muestran que la GNRP en niños es una entidad con pobre pronóstico en relación a la función renal. El factor de peor pronóstico que puede llevar a la pérdida del órgano es el compromiso túbulo-intersticial (AU)

Rapidly progressive glomerulonephritis (RPGN) is characterized by a sudden and progressive decrease of kidney function and extra-capillary cell proliferation (crescents) occupying the Bowman's space on the biopsy. We retrospectively analysed 37 children with RPGN (50% or more of glomeruli with crescents) seen at our institution over the past 20 years. The purpose of the study was to evaluate clinical and histopathological presentation, etiology, outcome, and factors of poor prognosis. Mean age at diagnosis was 11 ± 3.5 years. Presenting symptoms were: hematuria in 100% of the cases (macroscopic hematuria 56%); arterial hypertension in 92%; proteinuria in 88%; and nephrotic syndrome in 57%. Dialysis was necessary on admission in 64.1% of the cases. Kidney biopsies were performed at 38 ± 26 days after symptom onset. The percentage of glomeruli that presented crescents was 81.4%; they were epithelial in 28.3% of the cases, fibroepithelial in 21.8%, and fibrous in 31.3%. In 75.8% of the biopsies interstitial fibrosis and moderate and/or severe tubular atrophy was found. Immunofluorescence techniques did not show immune complex deposits (pauci-immune GN) in 40.6% of the biopsies. Granular deposits of immune complexes were found in 48.6% and linear anti-glomerular basement membrane deposits (Goodpasture´s) in 10.8%. Treatment was started 36 ± 32 days after symptom onset. All patients received support treatment; in 29 steroids and cyclophosphamide were also indicated, and in 5 steroids only. Mean time of follow-up was 4.6 ± 3.9 years. Patient survival at the end of follow-up was 87% (95%CI 55-97%) and organ survival was 17% (95%CI 7-38%). On multivariate analysis we found that interstitial fibrosis and moderate and/or severe tubular atrophy was the only factor related to organ loss (OR: 14.6; 95%CI 2.6-80) p= 0.001). Our results show that RPGN in children has a poor prognosis regarding kidney function. Tubulo-interstitial involvement is the factor of poor prognosis that may lead to organ loss (AU)

Glomerulonefritis difusa aguda postestreptocócica-psoriasis guttata. A propósito de un caso clínico / A case of acute diffuse poststreptococcal glomerulonephritis: guttate psoriasis

La glomerulonefritis asociada a la infección por estreptococo beta hemolítico del grupo A es la más conocida y es la causa más común de síndrome nefrítico en la edad pediátrica. La psoriasis es una enfermedad cutánea hereditaria eritematodescamativa poco frecuente, representa el 4,1% de las dermatosis que ocurren en niños menores de 16 años. Se presenta el caso de un adolescente de 12 años donde la infección por estreptococo beta hemolítico del grupo A de las vías respiratorias altas ocasionó glomerulonefritis difusa aguda, con expresión concomitante de psoriasis guttata. Se revisan los mecanismos inmunes en ambas patologías.

Glomerulonephritis associated with group A beta-hemolytic streptococcal infection is the best known and most common cause of nephritic syndrome in children. Psoriasis is often an erythematous hereditary skin disease. It represents 4.1% of the dermatoses children under 16 years. The study presents the case of a 12 year old patient where Group A beta-hemolytic streptococcal acute upper respiratory infection caused diffuse glomerulonephritis, with concomitant expression of guttate psoriasis. A review of immune mechanisms of both diseases is added.

Chronic renal disease and reversible posterior leukoencephalopathy syndrome after liver transplantation / Enfermedad renal crónica y síndrome de leucoencefalopatía posterior reversible después del trasplante del hígado

This is a case report of a patient who developed chronic renal dysfunction and neurologic emergency with multiple cranial lesions after liver transplantation. Immune-complex glomerulonephritis was confirmed on the basis of histopathologic evaluation of the renal biopsy. According to clinical features and brain magnetic resonance imaging follow-up, neuroradiographic atypical reversible posterior leukoencephalopathy syndrome (RPLS) was finally diagnosed.

Este es un reporte de caso de un paciente que desarrolló una disfunción renal crónica y requirió emergencia neurológica con múltiples lesiones craneales luego de un trasplante del hígado. La evaluación histopatológica de la biopsia renal permitió confirmar una glomerulonefritis por complejos inmunes. De acuerdo con las características clínicas y el seguimiento mediante tomografía por resonancia magnética del cerebro (de la resonancia magnética cerebral, finalmente se diagnóstico un síndrome de leucoencefalopatía posterior reversible atípico neuroradiográfico (SLPR).

Pulmonary renal syndrome in a case of wegener´s granulomatosis.

We report a case of a 42-year-old patient who presented with Wegener’s granulomatosis complicated by pulmonary renal syndrome, i.e., diffuse alveolar haemorrhage and rapidly progressive crescentic glomerulonephritis. The patient was treated with plasmapheresis and immunosuppressive drugs — intravenous cyclophosphamide and methyl prednisolone. The clinical, haematological and biochemical parameters improved substantially and remission is achieved.

Meningitis granulomatosa, glomerulonefritis rápidamente progresiva y vasculitis / Granulomatous meningitis, crescentic glomerulonephritis and vasculitis

El compromiso meníngeo es una manifestación infrecuente de la granulomatosis de Wegener. Puede manifestarse como cefalea con hiperproteinorraquia y engrosamiento de la duramadre con aspecto granulomatoso, que se observa en la resonancia magnética. Presentamos un varón de 57 años con granulomatosis de Wegener que debutó con compromiso de vías aéreas superiores, oídos, órbitas y meningitis granulomatosa asintomática y que posteriormente evolucionó con mononeuritis múltiple y glomerulonefritis crescéntica ANCA positiva. La presencia de ANCA y el compromiso sistémico (vías aéreas superiores, oído, órbitas, nervios periféricos, duramadre y glomerulonefritis rápidamente progresiva) permitieron en este caso llegar a un diagnóstico de certeza e iniciar el tratamiento inmunosupresor combinado (corticoides y ciclofosfamida). Evolucionó con remisión clínica y serológica (negativización de ANCA), pero persistiendo leve deterioro secuelar auditivo y de la función renal, sin recidiva de la enfermedad de base.

Meningeal involvement is an infrequent manifestation of Wegener's granulomatosis. Clinical manifestations can be headache with high protein level in the cerebrospinal fluid and an enhanced MRI signal of granulomatous thickening of the duramater in the brain. We report a 57 year-old male with Wegener granulomatosis with onset manifestations of asymptomatic granulomatous meningitis, upper respiratory tract, ears and orbits involvement. He progressively developed ANCA positive multiple mononeuritis and crescentic glomerulonephritis. The diagnostic confirmation of Wegener's granulomatosis based on a positive ANCA test and on the evidence of systemic disease (crescentic glomerulonephritis and involvement of the upper respiratory tract, ears, orbits, peripheral nerves and duramater) allowed a prompt initiation of aggressive immunosuppressive treatment with systemic cyclophosphamide and high - dosis corticosteroids. The patient entered into a sustained clinical remission with mild residual neurosensorial hearing loss and renal failure.

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.

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...

Medical causes and histological pattern of glomerulonephritis.

Glomerulonephritis (GN) is one of the commonest causes of chronic renal failure (CRF) and end stage renal failure (ESRF). The outcome largely depends on the underlying medical cause, clinical findings and histological appearance. 1188 patients were taken in this study. Of these, 980 had primary and 208 secondary GN. Commonest histological types of primary GN were mesangial proliferative glomerulonephritis (31%) and membranous nephropathy (21%). Among patients with secondary GN, predominant cause was lupus nephritis (76%) followed by poststreptococcal GN (18%). Clinically, nephrotic syndrome was present in 54%, hypertension in 39% and renal failure in 28%. But clinical pictures were variable among different histological types of GN.

Acute glomerulonephritis in southern Iran

Acute post streptococcal glomerulonephritis [APSGN] is the most common type of in-patient glomerulonephritis [GN] in childhood. It has not been studied well in this region yet. Here, we report our experience with APSGN in a tertiary referral center during a five-year period. Hospital records of all 137 children who had been admitted to Nemazee hospital, between 2001 and 2006, with diagnosis of acute glomerulonephritis [AGN] were reviewed. All demographic, clinical, paraclinical data and consumed medications were obtained. Among 137 children diagnosed as AGN, 122 [89%] had APSGN. Other 15 [11%] children had membranoproliferative glomerulonephritis [n=4], mesangioproliferative glomerulonephritis [n=4], IgA nephropathy [n=2], lupus nephritis [n=2], rapidly progressive glomerulonephritis [n=2], and focal segmental glomerulosclerosis [n=1]. Mean [SD] age in children with APSGN was 8.5 [3.5] [range, 3.5-13] years, 117 [96%] children developed APSGN following a sore throat and 5 [4%] following an impetigo, with 95 [78%] during the cold seasons of the year. Periorbital edema was found in 97.5%, hypertension 75%, gross hematuria 72%, oliguria 37%, generalized edema 19%, azotemia [BUN > 20] 80%, and nephrotic-range proteinuria 24.5%. A high anti streptolysin-O [ASO] titer and a low C3 level was detected in 84% and 86%, respectively. There was dilutional anemia in 51.5%, hyponatremia in 27%, and hyperkalemia 14%. With regard to medications, 19 patients received only furosemide, 73 cases furosemide and nifidipine, and 10 patients furosemide, nifidipine, and another antihypertensive medication. Hypertensive encephalopathy occurred in 3 cases, but no mortality was reported during the study period. APSGN is the most common type of glomerulonephritis in this region. It follows sore throat in the majority of cases. It usually has an uneventful course

Relationship between Brucella immunocomplex and glomerulopathies

Brucellosis is a zoonotic disease with a very wide spectrum of clinical findings. Brucellosis is about 10 times more prevalent in patients with renal failure [dialysis patients] compared to population background. Precipitation of immunocomplexes produced by brucellosis is important in causing glomerulonephritis. Because the hallmark of glomerular diseases is abnormal protein loss in the urine we have decided to study proteinuria in brucellosis immunocomplex. The aim of this study was to evaluate probable relationship between brucellosis and glomerulonephritis. This cross sectional study, performed on 200 patients with a history of the disease for about 1 year, diagnosed as having chronic brucellosis. The diagnosis was confirmed in 150 patients and by applying Brucella Standard Agglutination Test [SAT] and 2-mercaptoethanol test [2-ME]. Titers of IgG/IgM and IgG/IgA in two groups of "with proteinuria" and "without proteinuria" was measured. Both SAT and 2-ME tests test demonstrated that proteinuria increases with rising antibodies titers. In SAT, titer 1/160 was the most frequent, observed in 44% of the patients. In 2-ME test 1/40 titer was observed in 44% of the patients and was the most frequent. Our results clearly demonstrated that in both chronic and acute patients, proteinuria increases with rising IgG/IgA and IgM/IgG titers. Therefore brucellosis can cause nephropathy but chronic or untreated brucellosis is more important because it can permanently damage kidney

Comment on: A Case of Post-streptococcal Glomerulonephritis with Diffuse Alveolar Hemorrhage

No abstract available.

Paquimeningitis hipertrófica, glomerulonefritis y vasculitis de pequeños vasos asociada a ANCA / Hypertrophic pachymeningitis, glomerulonephritis and P-ANCA associated small vessel vasculitis

La paquimeningitis hipertrófica es una enfermedad poco frecuente caracterizada por engrosamiento de la duramadre. Presentamos una paciente con esta enfermedad que se manifestó con cefalea crónica y en la que concomitantemente se evidenció una glomerulonefritis necrotizante extracapilar pauciinmune asociada a anticuerpos anticitoplasma de neutrófilos de patrón perinuclear (ANCA-P). El diagnóstico se estableció por resonancia nuclear magnética. Recibió tratamiento inmunosupresor con prednisona y ciclofosfamida con evolución favorable.

Hypertrophic pachymeningitis is a very unusual disease, the main characteristic of which is thickening of the dura mater. We describe a patient who started this illness showing chronic headache and pauci-immune necrotizing extracapillary perinuclear antineutrophil cytoplasmic antibody (P-ANCA) associated glomerulonephritis. The diagnosis was made by brain magnetic resonance image. She received immunosuppressant therapy with prednisonel and cyclophosphamide with clinical improvement.

A Case of Post-Streptococcal Glomerulonephritis with Diffuse Alveolar Hemorrhage

Acute post-streptococcal glomerulonephritis (PSGN) is characterized by an abrupt onset of edema, hypertension, and hematuria. Life-threatening diffuse alveolar hemorrhage (DAH) is rarely associated with acute PSGN. There have been only two reported cases worldwide, and no case has been reported previously in Korea. Here, we present a patient who clinically presented with pulmonary-renal syndrome; the renal histology revealed post-infectious glomerulonephritis of immune complex origin. A 59-yr-old woman was admitted with oliguria and hemoptysis two weeks after pharyngitis. Renal insufficiency rapidly progressed, and respiratory distress developed. Chest radiography showed acute progressive bilateral pulmonary infiltrates. The clinical presentation suggested DAH with PSGN. Three days after treatment with high-dose steroids, the respiratory distress and pulmonary infiltrates resolved. Electron microscopy of a renal biopsy specimen sample revealed diffuse proliferative glomerulonephritis with characteristic subendothelial deposits of immune complex ("hump''). The renal function of the patient was restored, and the serum creatinine level was normalized after treatment.

Síndrome nefrítico (Proceso de enfermeria) / Nephritic syndrome (Infirmary Process)

Presenta estudio de procesos de enfermería con el fin de brindar atención integral a la niñez para evitar complicaciones del sindrome nefritico por medios de los planes de cuidados de enfermería que solucionen las necesidades del paciente, familia y comunidad. Durante este estudio se mostro que tanto la teoría como la práctica tienen mucha relación ya que los pacientes presentaban los mismo signos y síntomas que nos ofrecen las bibliografía consultadas. La conclusión del estudio fue lograr sensiblizar al paciente y a su familia con las acciones de enfermería brindadas en visitas domiciliares realizadas. Ademas enriquecer los conocimientos sobre el diagnóstico de síndrome nefrítico y poder diferenciar cuales son las principales causas que pueden ocasionar un síndrome nefrítico, lo cual es una experiencia para los estudiantes de enfermería, teniendo como resultado la recuperación de salud y disminuyendo las diferentes complicaciones del paciente.