Lipoprotein glomerulopathy caused by mutation of apolipoprotein E gene in children: a report of two cases / 中华肾脏病杂志

The paper reports two cases of lipoprotein glomerulopathy (LPG) in children. The Sanger sequencing results in 2 cases indicated apolipoprotein E gene mutation[c.127 (exon3) C>T, p.R43C (p.Arg43Cys); c.494 (exon4) G>C, p.R165P (p.Arg165Pro),respectively]. Renal pathological presentation of two children showed that a large number of lipoprotein emboli were formed in the glomerular capillary loop, and the diagnosis of LPG was confirmed. The onset of LPG has no specific clinical manifestation, which is easy to be undiagnosed or misdiagnosed. Renal biopsy is a diagnostic means, glucocorticoid treatment is ineffective, and long-term lipid-lowering treatment may be required for LPG.

A case of podocyte infolding glomerulopathy / 中华肾脏病杂志

Podocyte infolding glomerulopathy (PIG) is a pathologic type of podocyte glomerulopathy reported recently. The characteristic is that the ultrastructure related to podocytes, such as microspheres and microtubules, are folded into the glomerular basement membrane (GBM) under electron microscope. At present, there are few reports about this disease at home and abroad, and most of them are concentrated in Japan. The clinical characteristics and pathogenesis of PIG are still unclear. In this paper, we report a case of clinical manifestations of nephrotic syndrome, renal biopsy indicated PIG, after the treatment of glucocorticoid, hydroxychloroquine and tacrolimus, the patient's clinical symptoms were relieved and urinary protein decreased.

Research progress of renal lipid deposition in obesity related glomerulopathy / 中国医师杂志

Obesity related glomerulopathy (ORG), as a disease with an increasing incidence of metabolic kidney injury, has become a new hot spot in today's society. A variety of factors are involved in the occurrence and development of ORG. Among them, the ectopic deposition of kidney lipids is not only a significant feature of ORG, but also a key link to promote the progress of ORG. This article reviews the related mechanisms of lipid deposition in ORG and the treatment of ORG with lipid deposition as the target.

Clinical analysis of acute post-streptococcal glomerulonephritis and C 3 glomerulopathy in children / 中华实用儿科临床杂志

Objective:To investigate the clinical features of acute post-streptococcal glomerulonephritis (APSGN) and C 3 glomerulopathy (C 3G) in children, and to improve the understanding, diagnosis and treatment of C 3G in children with atypical APSGN. Methods:The clinical data of 100 children whose were clinically diagnosed with APSGN and admitted to the Beijing Children′s Hospital, Capital Medical University from January 2016 to December 2021 were collected and retrospectively analyzed.Seventy-three cases finally diagnosed with APSGN were included in the APSGN group and 27 cases with C 3G were classified into the C 3G group.The clinical manifestations, laboratory results, treatment and prognosis of the 2 groups of children were analyzed and compared by the t-test, Mann-Whitney U test and χ2 test. Results:Both APSGN and C 3G patients had a history of streptococcal infection at the early stage of the disease.There was no significant difference in the onset age and gender between the 2 groups (all P>0.05). The clinical manifestations of APSGN and C 3G at the early stage are sometimes difficult to distinguish.However, the incidence rates of gross hematuria (92.6%) and nephrotic proteinuria (66.7%) in the C 3G group were higher than those in the APSGN group (69.8%, 30.1%) ( χ2=5.583, 10.960; all P<0.05). The laboratory test results suggested that compared with the C 3G group, the APSGN group had higher albumin levels [(36.3±7.4) g/L vs.(28.9±6.8) g/L], but lower triglycerides [(1.2±0.6) mmol/L vs.(1.6±0.7) mmol/L], blood urea [(7.6±5.6) mmol/L vs.(14.7±16.3) mmol/L], blood creatinine [(66.2±45.2) μmol/L vs.(120.1±170.3) μmol/L], and urine protein levels [(43.5±58.5) g/24 h vs.(319.2±994.8) g/24 h] ( t=4.655, 2.738, 2.241, 1.624, 1.448; all P<0.05). As for treatment, the use rates of hormones and other immunosuppressants in the C 3G group were higher than those in the APSGN group (59.3% vs.12.3%, 29.6% vs.1.4%) ( χ2=23.15, 19.22; all P<0.05). The follow-up data revealed that compared with the APSGN group, the C 3G group took a longer time for gross hematuria and microscopic hematuria symptoms to disappear, proteinuria test to turn negative and complement C 3 to recover [51.1(14.3, 90.0) d vs.14.9(6.0, 15.5) d; 218.3(60.0, 277.5) d vs.65.5(27.0, 82.5) d; 127.9(60.0, 180.0) d vs.38.2(13.0, 53.6) d; 129.3(55.5, 225.0) d vs.39.1(24.0, 51.0) d] ( U=2.395, 2.730, 2.890, 3.054; all P<0.05). Conclusions:APSGN children with relatively severe clinical manifestations during the acute stage, especially with unrelieved nephrotic proteinuria, should be highly suspected with C 3G.Such patients should be treated with steroids and undergo renal biopsy and complement investigation if necessary, so as to identify the cause early, adjust the treatment and improve their prognosis.

Progress of C3 glomerulopathy / 国际儿科学杂志

C3 glomerulopathy is a rare disease of glomeruli mediated by abnormal activation of alternative complement pathway secondary to congenital genetic defects and acquired autoantibodies.Renal biopsy is the gold standard for diagnosing C3 glomerulopathy.C3 glomerulopathy encompasses both dense deposit disease and C3 glomerulonephritis.The main glomerular immunofluorescence staining is C3, with few or without immunoglobulins deposition, which is the obvious pathological feature.The clinical manifestations of C3 glomerulopathy are usually various, with limited detection methods and therapies and poor prognosis.This article mainly reviews the progress of C3 glomerulopathy in recent years, in order to improve clinical understanding of C3 glomerulopathy, and choose individualized therapy.

Mechanisms of perirenal adipose tissue inflammation in obesity-related glomerulopathy induced by a high-fat diet in C57BL/6J mice / 中华肾脏病杂志

Objective:To evaluate perirenal adipose tissue inflammation in obesity-related glomerulopathy induced by a high-fat diet in C57BL/6J mice and further explore its probable mechanism.Methods:Twelve 8-10-week-old male C57BL/6J mice were divided into normal diet group (ND, n=6) and high-fat diet group (HFD, n=6) using simple random sampling method. After 14 weeks, the blood and kidney tissue were sampled, and the pathological change in the kidney and perirenal adipose tissue was observed by hematoxylin-eosin, periodic acid-Schiff, and Masson staining. The mRNA expression of tumor necrosis factor-α (TNF-α), M1-type macrophage marker CD11c, interleukin (IL)-1β, monocyte chemotactic protein-1 (MCP-1), IL-10, transforming growth factor-β1, M2-type macrophage marker CD206 and fibronectin 1 in perirenal fat was detected by real-time fluorescence quantitative PCR. The expression of macrophages marker F4/80, CD68 and leukocyte common antigen (LCA) in the kidney and perirenal adipose tissue was detected by immunohistochemistry. Results:After 14 weeks of feeding, compared with mice in the ND group, the weight of mice in the HFD group was significantly higher [(35.83±1.19) g vs (24.06±0.37) g, P<0.05]. In the HFD group, perirenal adipocyte hyperplasia, accompanied by glomerular hyperplasia, mesangial matrix hyperplasia and renal interstitial fibrosis, and other pathological changes was observed (all P<0.05). The level of blood glucose, blood lipid, serum creatinine and blood urea nitrogen was also significantly higher (all P<0.05). The mRNA expression of TNF-α, CD11c, IL-1β and MCP-1 related to M1 macrophages in the perirenal adipose tissue was higher (all P<0.05), and immunohistochemistry showed that the expression of F4/80, CD68 and LCA in the perirenal adipose tissue was higher in HFD group (all P<0.05). The above results showed that the number of macrophages and inflammatory cells in the perirenal adipose tissue was significantly greater in the HFD group than those in the ND group. Pearson linear correlation analysis showed that the average perirenal fat area was positively correlated with macrophages number in perirenal adipose tissue, several morphological indexes such as glomerular cross-sectional area and renal function injury indexes such as blood urea nitrogen (all P<0.05). Conclusion:The C57BL/6J mice model of obesity-related glomerulopathy induced by a high-fat diet is successfully established, and the perirenal adipose tissue shows an obvious inflammatory response, with the macrophages significantly polarized mainly in the pro-inflammatory direction towards the M1-type macrophages.

Jornadas del Comité de Nefropatía de la Sociedad Argentina de Diabetes. Actualización de cirugía bariátrica en paciente con diabetes y enfermedad renal. Parte 2 / Conference of the Nephropathy Committee of the Argentine Diabetes Society. Bariatric surgery update in a patient with diabetes and kidney disease. Part 2

La prevalencia de obesidad y diabetes mellitus se asocia al desarrollo de enfermedad renal crónica y estadios terminales de la misma. En individuos con obesidad se produce un mecanismo de hiperfiltración, probablemente compensatorio para satisfacer la alta demanda metabólica asociada al aumento del peso corporal, con la presencia de proteinuria en individuos sin enfermedad renal. La histopatología muestra una glomeruloesclerosis focal y segmentaria relacionada con la obesidad en un marco de glomerulomegalia. La cirugía metabólica es el medio más efectivo para obtener una pérdida de peso sustancial y persistente. Se ha demostrado la superioridad de la cirugía sobre el tratamiento médico no solo para lograr un mejor control glucémico, sino también para la reducción de los factores de riesgo cardiovascular. Los mecanismos parecen extenderse más allá de la magnitud de la pérdida de peso e incluyen mejoras tanto en los perfiles de incretinas como en la secreción y la sensibilidad a la insulina. El Comité de Nefropatía de la Sociedad Argentina de Diabetes realizó esta revisión sobre los mecanismos involucrados en la obesidad como causa de enfermedad renal o empeoramiento de la misma por diabetes, y los mecanismos a través de los cuales la cirugía bariátrica beneficiaría a los pacientes con diabetes y enfermedad renal crónica en todos los estadios de la misma, así como los controles pre y posquirúrgicos en este tipo de cirugías.

The prevalence of obesity and diabetes mellitus are associated with the development of chronic kidney disease and its terminal stages. In individuals affected by obesity, a probably compensatory hyperfiltration mechanism occurs to satisfy the high metabolic demand associated with increased body weight; it is also associated with the presence and development of proteinuria in individuals without kidney disease. Histopathology shows obesity-related focal and segmental glomerulosclerosis in a setting of glomerulomegaly. Metabolic surgery is the most effective means of obtaining substantial and lasting weight loss. The superiority of surgery over medical treatment has been demonstrated only to achieve better glycemic control, as well as a reduction in cardiovascular risk factors. The mechanisms appear to extend beyond the magnitude of weight loss and include improvements in incretin profiles, insulin secretion, and insulin sensitivity. The Nephropathy Committee of the Argentine Diabetes Society carried out this review on mechanisms involved in obesity as a cause of kidney disease or worsening of kidney disease due to diabetes, the mechanisms by which bariatric surgery would benefit patients with diabetes and kidney disease chronic and its terminal stages, the pre and post-surgical controls that should be performed by patients undergoing this type of surgery

Jornadas del Comité de Nefropatía de la Sociedad Argentina de Diabetes. Actualización de cirugía bariátrica en paciente con diabetes y enfermedad renal. Parte 1 / Conference of the Nephropathy Committee of the Argentine Society of Diabetes. Bariatric surgery update in a patient with diabetes and kidney disease. Part 1

La prevalencia de obesidad y diabetes mellitus se asocia al desarrollo de enfermedad renal crónica y estadios terminales de la misma. En individuos con obesidad, se produce un mecanismo de hiperfiltración, probablemente compensatorio para satisfacer la alta demanda metabólica asociada al aumento del peso corporal, con la presencia de proteinuria, en individuos sin enfermedad renal. La histopatología muestra una glomeruloesclerosis focal y segmentaria relacionada con la obesidad en un marco de glomerulomegalia. La cirugía metabólica es el medio más efectivo para obtener una pérdida de peso sustancial y persistente. Se ha demostrado la superioridad de la cirugía sobre el tratamiento médico no solo para lograr un mejor control glucémico, sino también para la reducción de los factores de riesgo cardiovascular. Los mecanismos parecen extenderse más allá de la magnitud de la pérdida de peso e incluyen mejoras tanto en los perfiles de incretinas como en la secreción y la sensibilidad a la insulina. El Comité de Nefropatía de la Sociedad Argentina de Diabetes realizó esta revisión sobre los mecanismos involucrados en la obesidad como causa de enfermedad renal o empeoramiento de la misma por diabetes, y los mecanismos a través de los cuales la cirugía bariátrica beneficiaría a los pacientes con diabetes y enfermedad renal crónica en todos los estadios de la misma, así como los controles pre y posquirúrgicos en este tipo de cirugías.

The prevalence of obesity and diabetes mellitus are associated with the development of chronic kidney disease and its terminal stages. In individuals affected by obesity, a probably compensatory hyperfiltration mechanism occurs to satisfy the high metabolic demand associated with increased body weight; it is also associated with the presence and development of proteinuria in individuals without kidney disease. Histopathology shows obesity-related focal and segmental glomerulosclerosis in a setting of glomerulomegaly. Metabolic surgery is the most effective means of obtaining substantial and lasting weight loss. The superiority of surgery over medical treatment has been demonstrated only to achieve better glycemic control, as well as a reduction in cardiovascular risk factors. The mechanisms appear to extend beyond the magnitude of weight loss and include improvements in incretin profiles, insulin secretion, and insulin sensitivity. The Nephropathy Committee of the Argentine Diabetes Society carried out this review on mechanisms involved in obesity as a cause of kidney disease or worsening of kidney disease due to diabetes, the mechanisms by which bariatric surgery would benefit patients with diabetes and kidney disease chronic and its terminal stages, the pre and post-surgical controls that should be performed by patients undergoing this type of surgery.

Progress on electron microscopy diagnosis on Banff classification for renal allograft pathology / 器官移植

With the development of organ transplantation in clinical practice, allograft pathology has been constantly developing and advancing. The convening of Banff conference on allograft pathology and the establishment of Banff classification on allograft pathology (Banff classification) are pivotal milestones in the development of international allograft pathology. Since then, Banff classification on pathological diagnosis of various transplant organs have been continually updated and improved. Ultrastructural pathological observation by electron microscope plays an irreplaceable role in the early diagnosis of antibody-mediated rejection, recurrent disease and de novo disease of renal allograft. Early detection and rational treatment help to maintain the long-term survival of renal allograft and reduce the failure of renal allograft. In this article, the basic definition of electron microscope and the ultrastructural pathological diagnosis, the research history and main progress on electron microscope diagnosis on Banff classification for renal allograft pathology were introduced, and typical pathological changes, specific terminology and diagnostic criteria of electron microscope diagnosis on renal allograft biopsy were summarized, aiming to provide reference for clinical and basic research of organ transplantation.

Collagenofibrotic glomerulopathy – A rare disease diagnosed with the aid of transmission electron microscopy

Collagenofibrotic glomerulopathy (CFG) is a rare idiopathic kidney disease characterized by abnormal deposition of atypical Type III collagen fibers in the glomerulus causing subendothelial and mesangial expansion, manifesting as progressive renal dysfunction accompanied by proteinuria. The majority of CFG cases reported in literature are from Japan where this disease entity was initially recognized. There is an increased awareness and diagnosis of this rare renal disease in India with the recent increase in utilization of electron microscopy (EM) in clinical diagnostic settings. We describe a 28-year-old Bangladeshi woman who presented with hypertension and nephrotic range proteinuria not amenable to treatment with steroids and cyclophosphamide, whose renal biopsy demonstrated diagnostic ultrastructural features of CFG. This illustrative case is presented to highlight the role of EM analysis for diagnostic accuracy in renal biopsy evaluation in addition to demonstrating the unusual renal biopsy findings of this rare entity.

Two cases of Type Ⅲ collagen glomerulopathy and literature review / 中南大学学报(医学版)

In this paper, 2 cases of collagen Type Ⅲ glomerulopathy were analyzed. The clinical manifestations mainly included nephrotic syndrome, proteinuria, hypertension and renal dysfunction. One patient showed that the complement factor H-related protein 5 (CFHR5) gene was likely a disease-causing mutation. The pathological examination of renal tissues showed hyperplasia of mesangial matrix, sub-endothelial insertion, and double-track formation. Immunohistochemistry of Type III collagen was positive. Electron microscopy revealed that massive collagen fibers (40-70 nm in diameter) deposited in the mesangial matrix and basement membrane. As for the follow-up results, the normal renal function had kept steady and the proteinuria was moderate in 1 case treated with angiotensin Ⅱ receptor blocker. Due to other system disease, another case developed into acute kidney injury and then received hemodialysis. The clinical manifestations of collagen Type Ⅲ glomerulopathy was atypical, the light microscope pathological features were various, and the disease was mainly diagnosed by electron microscopy and immunohistochemistry.

Collagenofibrotic glomerulopathy- report of a rare renal disease with serial biopsies

@#Introduction: Collagenofibrotic glomerulopathy or collagen type-III glomerulopathy is a rare glomerular disease characterised by the deposition of type III collagen fibres in the subendothelial space and mesangium of the glomerulus. Case Report: Here, we present a case of collagenofibrotic glomerulopathy in a 49-year-old Indian female, the first to be reported from Singapore. Renal biopsy showed PAS (periodic acid-Schiff), silver and Congo red negative, amorphous extracellular material that expanded mesangial and subendothelial regions. Such materials were strongly positive for anti-collagen III immunofluorescent staining. Under electron microscopy, the mesangial and some subendothelial regions were greatly expanded by abundant collagen fibres which were different from normal collagen III fibres in both appearance and periodicity. Discussion: The availability of past renal biopsies for reference offered insight into disease progression. From the initial diagnosis of focal segmental glomerulosclerosis to eventually collagenofibrotic glomerulopathy over a time span of more than 10 years, this case highlights the gradual accumulation of collagen fibres in the glomeruli before classical features are apparent. It also emphasises the importance of electron microscopy in the diagnosis of this disease.

Primary Membranous Nephropathy With Extremely High Levels Of Ca-125 / Nefropatía Membranosa Primaria Con Niveles Extremadamente Altos De Ca-125

Abstract Membranous nephropathy is a glomerular disease that causes nephrotic syndrome. Absent phospholipase A2 receptor antibodies and absent staining with IgG4 may be linked to malignancy-associated MN. Here we present a case that defies that suggestion. A 42-year-old female presented with anasarca. Kidney biopsy revealed membranous nephropathy, stained positive for IgG but negative for IgG4. Absent phospholipase A2 receptor antibodies was negative. Abdominal tomography revealed a partial thrombosis of the left ovarian vein which raised suspicion for ovarian cancer. Even though her ovaries did not uptake FDG on PET scan, a carbohydrate antigen-125 was ordered. She had extremely high levels of carbohydrate antigen-125 which was unexpected in the course of benign events. Thorax CT, endoscopy, colonoscopy, mammography, and positron emission tomography were clear in terms of malignancy. Samples from both pleural effusion and ascites were consistent with transudate. Tuberculosis tests were negative. Cytology samples were negative for malign cells. Exploratory surgery was planned but rejected by the patient. She was treated as primary disease with cyclosporine and methylprednisolone. Rituximab was off-limits due to insurance rules. She had prompt and excellent response. Steroids were tapered and stopped at sixth month and cyclosporine at twelfth month. In her 36 months of drug-free follow up there has been no disease recurrence or a sign of cancer. Even when all odds are towards malignancy-associated membranous nephropathy, primary disease is still a possibility. We need better markers for malignancy-associated membranous nephropathy.A very high level of CA-125 does not necessarily mean cancer.

Resumen La nefropatía membranosa es una enfermedad glomerular que causa el síndrome nefrótico. La ausencia de anticuerpos contra el receptor de fosfolipasa A2 y de tinción para IgG4 puede deberse a una nefropatía membranosa asociada a cáncer. A continuación, se presenta un caso que desafía esta sugerencia. Una paciente de 42 años realizó una consulta por anasarca. A partir de la biopsia de riñón, se diagnosticó nefropatía membranosa con tinción positiva para IgG, pero negativa para IgG4. No se detectó la presencia de anticuerpos contra el receptor de fosfolipasa A2. La tomografía abdominal reveló una trombosis parcial en la vena ovárica izquierda, lo cual generó sospecha de cáncer de ovario. Si bien los ovarios no mostraron absorción de FDG en la tomografía por emisión de positrones, se solicitó una prueba de antígeno carbohidrato 125. Se le detectaron niveles elevados del antígeno carbohidrato 125, lo cual no es esperable en casos de eventos benignos. La tomografía computarizada de tórax, endoscopía, colonoscopía, mamografía y tomografía por emisión de positrones no mostraron tumores. Las muestras de derrame pleural y de ascitis fueron indicativas de trasudado. Las pruebas de tuberculosisarrojaron resultados negativos. El examen citológico fue negativo para células malignas. Se sugirió una cirugía exploradora, pero la paciente no aceptó. Se la trató con ciclosporina y metilprednisolona por enfermedad primaria. No se utilizó rituximab por reglas de su cobertura médica. La paciente tuvo una excelente respuesta al tratamiento de forma rápida. Los esteroides se disminuyeron de forma progresiva y se suspendieron a los seis meses, y la ciclosporina, a los doce meses. Durante los 36 meses de seguimiento sin medicación no ha habido recidiva ni signos de cáncer. Incluso cuando existen grandes probabilidades de que se trate de una nefropatía membranosa asociada a cáncer, aún es posible que se trate de una enfermedad primaria. Es necesario contar con mejores marcadores de nefropatía membranosa asociada a cáncer. Un nivel elevado de CA-125 no necesariamente es indicador de cáncer.

Glomerulopatía membranosa con glomerulonefritis necrosante. Una asociación infrecuente / Membranous glomerulopathy with necrotizing glomerulonephritis. An infrequent association

Resumen Antecedentes: la asociación entre glomerulopatía membranosa y glomerulonefritis necrosante crescéntica es infrecuente: 0.4%, confiriendo un escenario de peor pronóstico. Se requieren estudios que precisen el esquema de tratamiento óptimo, sin embargo, el rápido inicio de terapia inmunosupresora impacta en la preservación de la función renal. Objetivo: exponer un caso en el cual se presenta esta asociación y tiene adecuada respuesta con el tratamiento realizado. Métodos: en este artículo se presenta un caso de una mujer con síndrome nefrótico a quien se le documenta glomerulopatía membranosa con proliferación extracapilar confirmada con biopsia renal, sin evidencia de autoinmunidad, neoplasia ni proceso infeccioso. Tras el diagnóstico se inició manejo con metilprednisolona y ciclofosfamida con adecuada evolución, sin deterioro de la función renal y con mejoría de proteinuria. Conclusión: la presencia de proliferación extracapilar da peor pronóstico en los pacientes con glomerulopatía membranosa, el inicio oportuno del tratamiento es fundamental. (Acta Med Colomb 2019; 44: 39-42).

Abstract Background: the association between membranous glomerulopathy and crescentic necrotizing glomerulonephritis is infrequent: 0.4%, conferring a worst prognosis scenario. Studies that require the optimal treatment scheme are required; however, it has been described that the rapid onset of immunosuppressive therapy impacts on the preservation of renal function. Objective: to present a case in which this association is presented and has an adequate response to the performed treatment. Methods: in this article, a case of a woman with nephrotic syndrome with a documented membranous glomerulopathy with extracapillary proliferation confirmed by renal biopsy, without evidence of autoimmunity, neoplasia or infectious process is presented. After the diagnosis, treatment with methylprednisolone and cyclophosphamide was started with adequate evolution, without deterioration of renal function and with improvement of proteinuria. Conclusion: the presence of extracapillary proliferation gives worse prognosis in patients with membranous glomerulopathy. The timely initiation of treatment is essential. (Acta Med Colomb 2019; 44: 39-42).

Obesidad y riñón / Obesity and the kidney

RESUMEN Introducción: La obesidad es un problema de salud mundial y su frecuencia se está incrementando tanto en adultos como en niños. Una de sus complicaciones es la glomerulopatía asociada a la obesidad. Objetivo: Informar acerca de esta enfermedad y la actitud del pediatra para tratar de evitarla. Métodos: Revisión de la literatura médica más reciente sobre la enfermedad y el incremento de la obesidad en la edad pediátrica en las bases de datos PubMed, SciELO y LILACS. Se utilizaron las palabras clave: glomerulopatía relacionada con la obesidad, obesidad y sobrepeso en el niño, tratamiento de la obesidad. Resultados: La obesidad en el niño es en alto porcentaje de causa nutricional y en esta condición los factores ambientales y socioculturales juegan importante papel. La predisposición a padecer la glomerulopatía de la obesidad comienza desde la niñez. El tratamiento de los pediatras en estos casos estará dirigido a la prevención de la enfermedad puesto que se puede desarrollar en la adultez. Cuando fracasan las medidas preventivas, queda la posibilidad de la cirugía bariátrica con poca experiencia en la edad pediátrica y retos éticos importantes y a pesar de que pudiera ser una alternativa de tratamiento, no es aceptada hasta el presente en forma amplia. Conclusiones: La glomerulopatía de la obesidad, por lo general, no aparece hasta la adultez, pero es necesario prevenirla desde la edad pediátrica y para su prevención los pediatras deben estar atentos a los factores de riesgo que pueden aparecer desde las primeras etapas de la vida(AU)

ABSTRACT Introduction: Obesity is a global health problem and its frequency is increasing as much as in adults than in children. One of its complications is glomerulopathy associated to obesity. Objective: To inform on this disease and the attitude of pediatricians towards this trying to avoid it. Methods: Reviewing of the most recent medical literature on this disease and the increase of obesity in the pediatric age in PubMed, SCIELO and LILACS databases. The keywords used for the search were: glomerulopathy related to obesity, obesity and overweight in children, and obesity treatment. Results: Obesity in children is in a high percentage due to nutritional causes and in this disease environmental and sociocultural factors play an important role. The predisposition to suffer from glomerulopathy by obesity starts in childhood. The treatment prepared by the pediatricians in these cases will be directed to the prevention of the disease because it can be developed in adulthood. When preventive measures fail, there is the possibility of performing a bariatric surgery, having in this regard few experiences in the pediatric ages and important ethical challenges; and instead of being an alternative treatment, it is not widely accepted. Conclusions: Generally, glomerulopathy of obesity doesn't appear until adulthood, but it is necessary to prevent it since the pediatric age; and for its prevention pediatricians must be attentive to the risk factors that can appear from the earliest stages of life(AU)

Methods for glomerular quantification in dogs: a comparative study / Metodologias para quantificação glomerular em cães: um estudo comparativo

ABSTRACT: It was evaluated the different methodologies for glomerular enumeration described in the literature in a quantitative, double-blind study on the number of glomeruli present in kidney biopsy specimens obtained using a Tru-cut-type biopsy needle. Eighteen samples were taken (n=18) from various regions of canine kidneys harvested immediately after euthanasia. Sample collection was carried out using Tru-cut-type, semi-automated, 16 gauge needles. Reading and evaluation of the specimens were performed independently by four researchers by means of eye loupe inspection, light microscopy, light microscopy with a reduced condenser aperture, and dissection microscopy. Samples were also subjected to histopathological evaluation for comparison purposes. There was no inter-researcher variation for any of the tested methods. The glomerular counts obtained using light microscopy with reduced condenser aperture were similar to those reported in the histopathological evaluation (P=0.44);whereas, the other glomerular enumeration methods yielded significantly lower counts when compared to the histopathological analysis (eye loupe inspection: P<0.01; conventional light microscopy: P=0.02; dissection microscopy: P<0.01).Light microscope with lowering of the condenser lens delivers better results than conventional light microscopy and eye loupe inspection. Results obtained using dissection microscopy and eye loupe inspection did not correlate well with those provided by histopathological evaluation; consequently, these two methods should be avoided.

RESUMO: Avaliou-se as diferentes metodologias de quantificação glomerular descritas na literatura em um estudo quantitativo, duplo-cego, sobre o número de glomérulos presentes em amostras de biópsias renais obtidas com agulha de biópsia tipo Tru-cut. Dezoito amostras foram coletadas (n=18) de várias regiões de rins caninos colhidos imediatamente após a eutanásia. A coleta das amostras foi realizada com agulhas semi-automáticas de calibre 16, tipo Tru-cut. A leitura e avaliação das amostras foram realizadas de forma independente por quatro pesquisadores por meio de inspeção visual, microscopia de luz, microscopia de luz com redução da lente condensadora e microscopia de dissecção. As amostras também foram submetidas à avaliação histopatológica para fins de comparação e variação do pesquisador para qualquer um dos métodos testados. As contagens glomerulares obtidas por microscopia de luz com redução da lente condensadora foram semelhantes àquelas encontradas na avaliação histopatológica (P=0.44), enquanto que os outros métodos de quantificação glomerular produziram contagens significativamente mais baixas a análise histopatológica (inspeção da lupa dos olhos: P<0.01; microscopia de luz convencional: P=0.02; microscopia dissecação: P<0.01). Microscópio de luz com abaixamento da lente condensadora fornece melhores resultados do que microscopia de luz convencional e inspeção da lupa. As contagens obtidas usando microscopia de dissecção e inspeção de lupa de olho não se correlacionaram bem com aqueles fornecidos pela avaliação histopatológica; consequentemente, estes dois métodos devem ser evitados.

Glomerulopatía colapsante con insuficiencia renal terminal de rápida progresión / Collapsing glomerulopathy with rapid progression to terminal renal failure

La glomerulopatía colapsante (GC) constituye una variedad de la glomeruloesclerosis focal y segmentaria. Afecta tanto a la población adulta (40%) como a la infantil (20%); presentándose con mayor frecuencia en hombres jóvenes y afrodescendientes. Clínicamente se presenta como un síndrome nefrótico, con niveles elevados de úrea y creatinina. Se presenta el caso de paciente femenino de 22 años, quien acude por presentar fiebre, edema matutino en miembros inferiores, e intolerancia oral de 9 días de evolución. Al examen físico: Hipertensión arterial y ascitis. La GC es una entidad poco diagnosticada, que progresa rápidamente a insuficiencia renal terminal a pesar de recibir cualquier tratamiento sistémico descrito hasta la actualidad, por lo que amerita mayor investigación en el ámbito terapéutico(AU)

Collapsing glomerulopathy (GC) is a variety of focal segmental glomerulosclerosis. It affects both adult population (40%) and children (20%); it occurs most often in young people, male and of African descent. Clinically it is presented as a nephrotic syndrome, with high levels of urea and creatinine serum. There is insufficient evidence regarding the treatment of this entity, so that steroids and immunosuppressants are used at high doses. We present the case of a 22-year old female, who presented fever, edema in the lower limbs and oral intolerance of 9 days of evolution. Physical examination showed: high blood pressure and ascitis. This nephropathy is an underdiagnosed entity rapidly progressing to kidney failure despite receiving any systemic treatment described until now, so it merits further research in the therapeutic field(AU)

Pathology of C3 Glomerulopathy

C3 glomerulopathy is a renal disorder involving dysregulation of alternative pathway complement activation. In most instances, a membranoproliferative pattern of glomerular injury with a prevalence of C3 deposition is observed by immunofluorescence microscopy. Dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are subclasses of C3 glomerulopathy that are distinguishable by electron microscopy. Highly electron-dense transformation of glomerular basement membrane is characteristic of DDD. C3GN should be differentiated from post-infectious glomerulonephritis and other immune complex-mediated glomerulonephritides showing C3 deposits.

Renal and Urinary Manifestations of Inflammatory Bowel Disease / 대한소화기학회지

The incidence of inflammatory bowel disease (IBD) is increasing rapidly and extra-intestinal manifestations in IBD are also increasing. The prevalence of renal and urinary involvement in IBD ranges from 4–23%. Nephrolithiasis is the most common urinary complication in IBD patients. Parenchymal renal disease is rare but has been well documented and presents most commonly as glomerulonephritis or tubulointerstitial nephritis. The overall morbidity of IBD-related renal manifestations is significant. Therefore, a high index of clinical suspicion and optimal monitoring of the renal function are needed for the early diagnosis and prevention of IBD-related renal manifestations and complications.

Renal and Urinary Manifestations of Inflammatory Bowel Disease / 대한소화기병학회잡지

The incidence of inflammatory bowel disease (IBD) is increasing rapidly and extra-intestinal manifestations in IBD are also increasing. The prevalence of renal and urinary involvement in IBD ranges from 4–23%. Nephrolithiasis is the most common urinary complication in IBD patients. Parenchymal renal disease is rare but has been well documented and presents most commonly as glomerulonephritis or tubulointerstitial nephritis. The overall morbidity of IBD-related renal manifestations is significant. Therefore, a high index of clinical suspicion and optimal monitoring of the renal function are needed for the early diagnosis and prevention of IBD-related renal manifestations and complications.