Nefropatía por inmunoglobulina M: características histopatológicas y clínicas. Serie de casos / Immunoglobulin M nephropathy: histopathological and clinical characteristics. Case series

La nefropatía por inmunoglobulina M (NIgM) es una glomerulopatía idiopática caracterizada por depósitos mesangiales globales y difusos de IgM. Se realizó un estudio retrospectivo de las características clínicas e histopatológicas de los pacientes con NIgM atendidos en nuestro servicio. De 241 biopsias renales, 21 correspondieron a NIgM (8,7 %). Se incluyeron 18 pacientes (14 de sexo femenino, mediana de edad: 3,08 años). Se excluyó a 1 paciente por enfermedad sistémica asociada y a 2 por seguimiento menor a 1 año. Catorce pacientes se manifestaron con síndrome nefrótico (SN) y 4 con proteinuria aislada o asociada a hematuria. En la microscopia óptica, 13 presentaron hiperplasia mesangial, y 5 esclerosis focal y segmentaria. De los pacientes con SN, 7 fueron corticorresistentes, 4 corticodependientes y 3 presentaban recaídas frecuentes. Todos los pacientes con SN y 1 con proteinuria-hematuria recibieron inmunosupresores; los 18 pacientes recibieron, además, antiproteinúricos. Luego de 5,2 años (2-17,5) de seguimiento, 6 pacientes evolucionaron a enfermedad renal crónica

Immunoglobulin M nephropathy (IgMN) is an idiopathic glomerulopathy characterized by diffuse global mesangial deposits of IgM. We retrospectively studied the clinical and histopathological characteristics of the patients with IgMN seen in our service. Of 241 renal biopsies, 21 corresponded to IgMN (8.7 %). One patient was excluded due to associated systemic disease and 2 due to follow-up less than 1 year, 18 were included (14 girls, median age 3.08 years). Fourteen manifested with nephrotic syndrome (NS) and the remaining with proteinuria (isolated or associated with hematuria). On light microscopy, 13 had hyperplasia with mesangial expansion and 5 had focal and segmental sclerosis. Of the patients with NS, 7 were steroid-resistant, 4 steroid-dependent, and 3 frequent relapsers. All patients with NS and 1 with proteinuria-hematuria received immunosuppressants; the 18 patients also received antiproteinuric drugs. After 5.2 years (2-17.5) of follow-up, 6 patients developed chronic kidney disease.

Mast cells in the kidney biopsies of pediatric patients with lupus nephritis / Mastócitos em biópsias renais de pacientes pediátricos com nefrite lúpica

ABSTRACT Introduction: Mast cells may be involved in inflammation and contribute to the onset of fibrosis in lupus nephritis (LN). Objective: This study aimed to correlate the presence of mast cells in kidney biopsy specimens of pediatric patients with LN with activity (AI) and chronicity (CI) indices and assess how effectively mast cells may be used as a prognostic factor. Method: The study included 40 patients aged 6-18 years diagnosed with LN at the Renal Disease Service of the Federal University of Triângulo Mineiro between 1996 and 2015. Workup and epidemiological data were evaluated vis-à-vis AI, CI, and mast cell counts (MCC). Results: Significant positive correlations were found between mast cell counts (MCC) and AI (p = 0.003; r: 0.66) and MCC and CI (p = 0.048; r: 0.48). The ROC curve showed that mast cells were highly sensitive and specific in the differentiation of patients with an AI > 12 from individuals with an AI ≤ 12. Serum creatinine levels were higher in individuals with class IV LN than in patients with class V disease [1.50 (0.40-20.90) vs. 0.70 (0.62-0.90), p = 0.04]. Blood urea nitrogen had a positive significant correlation with MCC (p = 0.002; r: 0.75). A trend toward a negative correlation was observed between MCC and serum albumin (p = 0.06; r: -0.5459). Kidney biopsies of patients with nephrotic syndrome had higher MCC [2.12 (0.41-5.140) vs. 0.53 (0.0-3.94), p = 0.07]. Conclusion: Inflammatory cell infiltration and morphological differences between cell types in the inflammatory infiltrate are relevant factors in the assessment of the LN. Mast cell analysis and AI/CI assessment may be relevant prognostic indicators for pediatric patients with LN.

RESUMO Introdução: Mastócitos podem participar da inflamação e contribuir para fibrose na nefrite lúpica (NL). Objetivo: Correlacionar mastócitos em biópsias renais (BR) de pacientes pediátricos com NL com índices de atividade (IA) e cronicidade (IC), avaliando sua efetividade como fator prognóstico. Metodologia: Foram estudados 40 pacientes, entre 6 e 18 anos, diagnosticados com NL pelo Serviço de Nefropatologia da UFTM entre 1996 e 2015. Dados laboratoriais e epidemiológicos foram correlacionados com IA, IC e contagem de mastócitos (CM). Resultados: Encontramos correlação positiva e significativa entre contagem de mastócitos (CM) e IA (p = 0,003; r: 0,66) e entre CM e IC (p = 0,048; r: 0,48). Conforme a curva Roc, os mastócitos têm alta sensibilidade e especificidade na diferenciação de pacientes com IA menor ou maior do que 12. A creatinina sérica foi mais elevada na classe IV em relação à classe V [1,50 (0,40 - 20,90) versus 0,70 (0,62 - 0,90), p = 0,04]. Ureia sérica apresentou correlação positiva e significativa com CM (p = 0,002; r: 0,75). Observou-se tendência à correlação negativa entre CM e albumina sérica (p = 0,06; r: -0,5459). BR de pacientes com síndrome nefrótica apresentaram maior CM [2,12 (0,41 - 5,140) versus 0,53 (0,0 - 3,94), p = 0,07]. Conclusão: Não apenas o infiltrado inflamatório como também a diferenciação morfológica dos tipos celulares que o constituem são importantes para a avaliação da NL. Isso indica que a análise dos mastócitos, juntamente com a dos IA e IC, pode ajudar os nefrologistas a definirem o prognóstico de pacientes pediátricos.

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.

Tratamiento estomatológico del paciente con síndrome nefrótico congénito e hipoplasia generalizada del esmalte: informe de un caso / Stomatological treatment of the patient with congenital nephrotic syndrome and generalized enamel hypoplasia: report of a case

El síndrome nefrótico es la glomerulopatía que se presenta con mayor frecuencia a nivel mundial. La historia natural de la enfermedad, laevolución, la histología renal y la respuesta al tratamiento, va desde la remisión hasta el trasplante renal. Los pacientes con nefropatía presentan alteraciones estomatológicas propias de la enfermedad y secundariasal tratamiento. Los niños con enfermedad renal crónica presentan doscondiciones orales importantes: alta incidencia de anomalías dentarias(hipoplasia del esmalte, retraso de erupción, calcificaciones pulpares) ybaja actividad de caries. Objetivo: Describir la técnica de restauración dental a base de ionómero mediante un caso clínico de un paciente con nefropatía e hipoplasia del esmalte. Conclusión: El uso de ionómero devidrio como obturación semipermanente en pacientes con hipoplasia del esmalte es una eficaz alternativa de tratamiento cuando no se pueden explotar opciones como sistemas adhesivos, coronas de acero cromo o coronas para dientes permanentes.

Nephrotic syndrome is the glomerulopathy which occur mostfrequently in the world. The natural history of disease, evolution, renalhistology and response to treatment, ranging from referral to renaltransplantation. Patients with kidney disease have own stomatology alterations and secondary alterations related to treatment. Children with chronic renal failure have two oral conditions of interest: high incidence of dental anomalies (enamel hypoplasia, delayed eruption,pulp calcifications) and low caries activity. Objective: To describe the technique ionomer dental restoration by a clinical case of a patientwith nephropathy and enamel hypoplasia. Conclusion: The use of glass ionomer as semi-shutter in patients with enamel hypoplasia isan effective alternative of treatment when cannot be exploited options such as adhesive systems, steel crowns or crowns for permanent teeth.

Biopsia renal en el segundo trimestre del embarazo: Dos casos clínicos / Renal biopsies during the second trimester of pregnancy: Report of two cases

Kidney biopsies were performed in two women during their 21th and 24th week of pregnancy. The first patient developed an abrupt nephrotic syndrome without hypertension or kidney failure. The pathological study disclosed diffuse podocyte alterations such as those observed in focal and segmental glomerulosclerosis, which had a good response to steroidal treatment. The second patient had a progressive renal failure associated with non-nephrotic proteinuria. The biopsy disclosed a fibrillary glomerulopathy.

Familial steroid-sensitive idiopathic nephrotic syndrome: seven cases from three families in China

OBJECTIVES: Familial steroid-sensitive idiopathic nephrotic syndrome is rare, and only approximately 3% of patients have affected siblings. METHODS: Herein, we report seven cases of patients with steroid-sensitive idiopathic nephrotic syndrome from three Chinese families. Mutational screening of the Nphs2 gene was performed in all the patients. RESULTS: All seven of the familial steroid-sensitive idiopathic nephrotic syndrome cases in our sample exhibited minimal change disease, and one case also presented with mesangial proliferative glomerulonephritis, according to the renal pathology. No significant was associations were found between Nphs2 gene mutations and the onset of proteinuria and nephrotic syndrome in these familial cases. CONCLUSIONS: The presence of minimal change disease is important, but it is not an unusual finding in patients with familial steroid-sensitive idiopathic nephrotic syndrome, which appears to be clinically benign and genetically distinct from other types of nephrosis. .

Síndrome nefrótico e injuria renal aguda con microangiopatía trombótica en mujer con enfermedad celíaca: Caso clínico / Nephrotic syndrome and acute kidney injury associated with celiac disease: Report of one case

Celiac disease may be associated with other autoimmune diseases and exceptionally with glomerulopathies and nephrotic syndrome. Associations have been reported with IgA nephropathy, membranoproliferative glomerulonephritis, membranous glomerulopathy and minimal change disease. We report a 63-year-old woman who simultaneously presented with massive nephrotic syndrome (proteinuria 46 g/day) and cachexia due to a malabsorption syndrome secondary to celiac disease. The course of her diseases was complicated with cardiomyopathy due to severe malnutrition, septic shock, acute kidney injury that required dialysis for seven weeks and severe hypertension. A renal biopsy showed a membranoproliferative pattern of injury secondary to a thrombotic microangiopathy and diffusepodocyte damage. Fouryears later, the patient was in good general health, the glomerular filtration rate was 30 ml/min/1.73m² and there was non-nephrotic proteinuria.

Association of proteinuria with various clinical findings and morphologic variables of oxford classification in immunoglobulin a nephropathy patients

Immunoglobulin A nephropathy [IgAN] with nephrotic syndrome is an uncommon form of IgAN. Clinical and morphological characteristics of proteinuria in IgAN, especially when is in nephrotic range have not yet been fully examined. This study was aimed to correlate morphologic variables of the Oxford classification, and various clinical data with proteinuria in IgAN patients. We also aimed to demonstrate the significance of prevention of proteinuria as one of the important factors in progression of this disease. In an observational study conducted on IgAN patients, total of 114 biopsies were entered in the study. IgAN was diagnosed by light and immunofluorescence study. Of 114 patients 70.2% were male. Mean age of patients was 37.7 +/- 13.6 years. The mean of proteinuria was 1742 +/- 1324 mg/day. Also mean of serum creatinine [Cr] was 1.6 +/- 1.5 mg/dL. Of 114 patients, 11[9.6%] had nephrotic range proteinuria. In this study, there was a positive correlation between proteinuria and serum Cr, peri-glomerular fibrosis or interstitial fibrosis. There was a positive association between proteinuria and totally sclerotic glomeruli too. There was also a positive association between the amount of fibrous crescents and the level of proteinuria. Nephrotic proteinuria could just be seen in male patients. Also, nephrotic syndrome had a positive association with the number of crescents. Our findings firstly support the prognostic value of crescent due to its association with proteinuria and secondly imply the importance of treatment of proteinuria to prevent progression of IgAN

Glomerulopatia colapsante / Collapsing glomerulopathy

Background: Collapsing glomerulopathy is a cause of nephrotic syndrome with massive proteinuria secondary to podocyte proliferation and glomerular collapse. It is characterized by an almost inevitable progression to end stage renal failure, poor response to treatment and high post-transplant recurrence. Its frequency has increased in recent years due to its common association with Human Immunodeficiency Virus (HIV) infection and the growing recognition of new etiologic agents such as drugs and parvovirus B19. Therefore, it is a disease of growing interest for clinicians. The aim of this review is to update the clinical presentation, diagnosis, pathogenesis and therapeutic alternatives of this disease.

Minimal change disease associated with type 1 and type 2 diabetes mellitus / Glomerulopatia de lesões mínimas associada ao diabetes melito do tipo 1 e do tipo 2

A 19-year-old female with type 1 diabetes for four years, and a 73-year-old female with type 2 diabetes for twenty years developed sudden-onset nephrotic syndrome. Examination by light microscopy, immunofluorescence, and electron microscopy (in one case) identified minimal change disease (MCD) in both cases. There was a potential causative drug (meloxicam) for the 73-year-old patient. Both patients were treated with prednisone and responded with complete remission. The patient with type 1 diabetes showed complete remission without relapse, and the patient with type 2 diabetes had two relapses; complete remission was sustained after associated treatment with cyclophosphamide and prednisone. Both patients had two years of follow-up evaluation after remission. We discuss the outcomes of both patients and emphasize the role of kidney biopsy in diabetic patients with an atypical proteinuric clinical course, because patients with MCD clearly respond to corticotherapy alone or in conjunction with other immunosuppressive agents.

Uma paciente de 19 anos de idade com diabetes tipo 1 durante quatro anos e uma paciente de 73 anos de idade com diabetes tipo 2 durante vinte anos desenvolveram quadro súbito de síndrome nefrótica. O exame histológico à microscopia de luz, imunofluorescência e microscopia eletrônica (em um caso) diagnosticou glomerulopatia de lesões mínimas (GLM) em ambos os casos. Na paciente de 73 anos de idade, houve uma associação com o uso de meloxicam. As duas pacientes foram tratadas com corticosteroides e responderam com remissão completa do quadro. A paciente de 19 anos com diabetes tipo 1 apresentou remissão completa sem recidivas, e a paciente de 73 anos com diabetes tipo 2 apresentou dois episódios de recidiva; a remissão completa foi conseguida após associação ao tratamento com ciclofosfamida. As duas pacientes foram seguidas dois anos após a remissão completa. Os casos descritos enfatizam o papel da biópsia renal em pacientes diabéticos com evolução atípica do aparecimento de proteinúria, pois pacientes com GLM respondem bem à corticoterapia como único tratamento ou associado a outro agente imunossupressor.

Maduración ósea y estado nutricional antropométrico en niños, niñas y adolescentes con síndrome nefrótico / Bone maturation and anthropometric nutritional status in children and adolescents with nephrotic syndrome

Los glucocorticoides, comúnmente utilizados en el tratamiento del síndrome nefrótico, pudieran interferir en elcrecimiento y desarrollo del niño. La maduración ósea (MO) por el método Tanner- Whitehouse II (TW I2) constituye un indicador para evaluar el desarrollo somático. Analizar el comportamiento de la MO con relación al estado nutricional antropométrico y a las características clínico – patológicas en niños, niñas y adolescentes con Síndrome Nefrótico. Estudio transversal, exploratorio, descriptivo y correlacional. Se evaluaron 28 pacientes (11 niñas, 17 varones) entre 2 y 16 años con síndrome nefrótico, que acudieronentre agosto y octubre de 2008 a la consulta de nefrología del Hospital Pediátrico Dr. Elías Toro, cuyos padres autorizaron las medicionesde peso y talla y la realización de una radiografía de mano y muñeca izquierdas para edad ósea TW2. Se utilizaron la prueba de ANOVAsimple, t de Student y coeficiente de correlación de Pearson, con nivel de significación estadística de p <0,05. Predominó el género masculino(60,7%). Dos pacientes tuvieron un índice de masa corporal en exceso y ninguno con déficit. Predominó la edadósea normal (85,7%), adelantada en tres niños (10,7%) y retardada en uno. Se observó una relación inversa entre el número de recaídas y el porcentaje de maduración ósea alcanzada en los pacientes masculinos con corticoresistencia (p < 0,05). Este estudio reveló diferencias en el comportamiento de la maduración ósea, según el género y la condición de corticoresistencia

Glucocorticoids, commonly used in the treatment of nephrotic syndrome could interfere in children’s growth and development. Skeletal maturity determined by Tanner-Whitehouse II method (TW2) constitutes an indicator of bone maturation. To analyze the behavior of bone age (BA) related to anthropometric nutritional status and to clinical and pathological characteristics of children and adolescents with nephrotic syndrome. This is a cross-sectional, exploratory, descriptive andcorrelational study. Twenty eight patients (11 girls, 17 boys) diagnosed with nephrotic syndrome that attended the nephrology outpatient clinic of the Pediatric Hospital Dr. Elías Toro, from August to October 2008. Weight and height were measured using international techniques, and a left hand and wrist X-ray was evaluated by the TW2-method. ANOVA, t-Student test and Pearson correlation coefficient were calculated with a significance level of p< 0.05. Results: boys predominated (60.7%). Body mass index was abovenormal in 2 patients and normal in the rest. BA was normal in 24 children (85.7%), advanced in three (10.7%) and delayed in one. An inverse relationship was found between the number of relapses and the percentage of maturity reached in steroid resistant male patients (p < 0.05). This study shows a probable difference in the behavior of BA, according to gender steroid resistance

Proteomic profiling of nephrotic syndrome in serum using magnetic bead based sample fractionation & MALDI-TOF MS.

Background & objectives: At present, the diagnosis of nephrotic syndrome (NS) requires a renal biopsy which is an invasive procedure. We undertook this pilot study to develop an alternative method and potential new biomarkers for diagnosis, and validated a set of well-integrated tools called ClinProt to investigate serum petidome in NS patients. Methods: The fasting blood samples from 49 patients diagnosed with NS by renal biopsy, including 17 mesangial proliferative glomerulonephritis (MsPGN), 12 minimal change nephrotic syndrome (MCNS), 10 focal segmental glomerulosclerosis (FSGS) and 10 membranous nephropathy (MN), were collected and screened to describe their variability of the serum peptidome. The results in NS group were compared with those in 10 control healthy individuals. Specimens were purified with magnetic beads-based weak cation exchange chromatography and analyzed in a MALDI-TOF MS. Results: The results showed 43, 61, 45 and 19 differential peptide peaks in MsPGN, MCNS, MN and FSGS groups, respectively. A Genetic Algorithm was used to set up the classification models. Cross validation of healthy controls from MsPGN, MCNS, MN and FSGS was 96.18, 100, 98.53 and 94.12 per cent, respectively. The recognition capabilities were 100 per cent. Interpretation & conclusions: Our results showed that proteomic analysis of serum with MALDI-TOF MS is a fast and reproducible approach, which may give an early idea of the pathology of nephrotic syndrome.

Semiología renal y genitourinaria en pediatría: segunda parte / Nephrologoy and urology semiology in children: second part

Entre las patologías renales en niños, se encuentra la injuria renal aguda; que es la pérdida súbita de la función renal; el síndrome nefrótico que es el espectro más grave de proteinuria; el síndrome nefrítico caracterizado por la riada edema, hematuria macro o microscópica e hipertensión arterial.

Among the renal pathology in children is acute kidney injury, which is the sudden loss of kidney function, the nephrotic syndrome is the most severe spectrum of proteinuria, the nephrotic syndrome characterized by the triad of edema, macro or microscopic hematuria and arterial hypertension.

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.

Renal biopsy findings in Jamaican children / Hallazgos en las biopsias renales en niños jamaicanos

OBJECTIVE: To document the histological findings in Jamaican children undergoing renal biopsy in order to determine the relative prevalence of varying types of glomerular disease in the island. METHODS: This study analyses retrospectively the renal histology in all Jamaican children less than age 12 years undergoing their first adequate renal biopsy between January 1985 and December 2008. Clinicopathological data were obtained solely from the histology reports from the University Hospital of the West Indies where all paediatric renal biopsies are processed. RESULTS: Of the 270 children, aged 1 month to 11 years (mean 7.58 years), 147 [58.1%] were males. The commonest indications for renal biopsy were nephrotic syndrome (57.4%) and glomerulonephritis (30%). Most biopsied children (260/270) had glomerular disease. The predominant glomerulonephritides were diffuse proliferative glomerulonephritis (DPGN) (27.7%) and mesangialproliferative glomerulonephritis (MesGN) (25.5%). Glomerular disease was idiopathic in 136/260 (53%) but was infection-associated in 32.3% (84 cases) of which Poststreptococcal glomerulonephritis (PSGN) was the commonest (75%) -predominantly DPGN (74.6%). Hepatitis B followed at 15.5% (13/84) manifested as membranous nephropathy in 83.3% (10/12). In patients with SS disease, DPGN was the commonest histology (47.4%). Systemic lupus erythematosus accounted for 5% ofall renal biopsies. Over time, PSGNoccurred less frequently, with a parallel reduction in DPGN and MesGN. CONCLUSION: In Jamaican children, DPGN is the commonest nephritis. Membranous nephropathy is primarily due to Hepatitis B. The commonest histology in SS disease is DPGN. The role ofinfection in the pathogenesis ofrenal disease in Jamaican children is probably underestimated.

OBJETIVO: Documentar los hallazgos histológicos en niños jamaicanos a los que se les ha realizado biopsias renales para determinar la prevalencia relativa de los diversos tipos de enfermedad glomerular en la isla. MÉTODOS: Este estudio analiza retrospectivamente la histología renal en todos los niños jamaicanos menores de 12 años sometidos a su primera biopsia renal adecuada entre enero de 1985 y diciembre de 2008. Los datos clinicopatológicos fueron obtenidos exclusivamente de los reportes de histología del Hospital Universitario de West Indies, dónde se procesan todas las biopsias renales. RESULTADOS: De 270 niños, cuyas edades fluctuaban de 1 mes a 11 años (media 7.58 años), 147 [58.1%] eran varones. Las indicaciones más comunes para la biopsia renal fueron el síndrome nefrótico (57.4%) y la glomerulonefritis (30%). La mayoría de los niños sometidos a biopsia (260/270) tenían la enfermedad del glomerular. Las glomerulonefritis predominantes fueron la glomerulonefritis proliferativa difusa (GNPD) (27.7%) y glomerulonefritis proliferativa mesangial (GNMes) (25.5%). La enfermedad glomerular fue idiomática en 136/260 (53%) pero estuvo asociada con infecciones en 32.3% (84 casos) en los cuales la glomerulonefritis poststreptocóccica (GNPS) fue la más común (75%) - predominantemente GNDP (74.6%). La hepatitis B siguió con 15.5% (13/84), manifestada como nefropatía membranosa en 83.3% (10/12). En los pacientes con la enfermedad de la hemoglobina SS, la GNDP fue la histología más común (47.4%). El lupus eritematoso sistémico representó el 5% de todas las biopsias renales. Al pasar el tiempo, la GNPS ocurrió menos frecuentemente, con una reducción paralela en GNPD y GNMes. CONCLUSIÓN: En los niños jamaicanos, la GNPD es la nefritis más común. La nefropatía membranosa se debe principalmente a la Hepatitis B. La histología más común en el caso de la enfermedad de hemoglobina SS es la GNPD. Probablemente se subestima el papel que las infecciones desempeñan en la patogénesis de la enfermedad renal en los niños jamaicanos.

Estudo das doenças glomerulares em pacientes internados no Hospital Geral César Cals (Fortaleza-CE) / Study of glomerular diseases in patients admitted to the Hospital Geral César Cals (Fortaleza-CE)

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

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

Glomerulosclerosis; lipids and lipoproteins, a risk factor

Objective is to determine the changes in lipids and lipoproteins in patients with glomerulosclerosis and healthy controls. Department of Biochemistry Frontier Medical College, Abbottabad with the collaboration of Nephrology Unit, Ayoub Medical Complex, Abbottabad. April, 2006 February 2007. Study includes 50 subjects out of which 25 adult patients [18 males, 7 females; age range 20 50 years] with glomerulosclerosis were selected from Nephrology Unit of Ayoub Medical Complex, Abbottabad. Apparently, 25 clinically healthy volunteers of similar age, sex, body mass index [BMI], and socio economic status as that of patients were selected from various areas of Abbottabad as controls Serum triacylglycerols serum total cholesterol, and HDL cholesterol levels were measured by kit method LDL cholesterol was calculated according to the Friedewald formula[17]. Serum VLDL-cholesterol as calculated according to the formula proposed by Wilson cited by Delongi[18]. The results were expressed as mean +/- standard error of mean Statistical analysis was done using student 's test. Distribution of 25 patients and 25 controls according to age is made. Male subjects with glomerulosclerosis were greater in number than female with male to female ratio of 1.85:1 Comparison of serum TGs, TC, HDL-c, LDL-c, VLDL-c, and HDL-c, LDL-c ratio. The mean values of TAGs, TC, HDL-c, LDL-c, VLDL-c, and ratio of HDL-c, vs. LDL-c of patients were observed significantly high [P<0.001], when compared to controls. In addition significant low values [P<0.001] of HDL-c in patients were also observed when compared to healthy controls. It is concluded that the results of this study provide evidence for raised lipids and lipoproteins levels, which is invariable feature of nephritic syndrome with glomerulosclerosis. It is suggested that the patients with nephritic glomerulosclerosis should be assessed and managed to avoid potential of accelerating the development of coronary artery disease and increasing risk of renal failure. Hopefully earlier intervention might decrease higher morbidity and mortality