Predicting kidney outcomes among Latin American patients with lupus nephritis: The prognostic value of interstitial fibrosis and tubular atrophy and tubulointerstitial inflammation.

OBJECTIVE: To assess the effect of tubulointerstitial inflammation (TII) and interstitial fibrosis and tubular atrophy (IFTA) on kidney survival in lupus nephritis (LN). METHODS: Two hundred eighty five patients with biopsy-proven LN were retrospectively studied. Kidney survival was defined as the time from initial biopsy to end-stage kidney disease (ESKD), dialysis, or transplant. Kidney survival analysis was performed by the Kaplan-Meier method and the statistical difference between survival curves compared by the log-rank test. Cumulative incidence functions with competing risk of death for kidney survival were also graphed. Multivariable Cox proportional hazards regression and competing-risk analyses were performed to identify independent predictors of ESKD. RESULTS: Fifty-seven patients (20%) progressed to ESKD during a median time of 4.2 (2.0-55.2) months after biopsy. TII was present in 206 (72.3%) biopsies, while IFTA in 99 (34.7%) biopsies. Patients with moderate-to-severe IFTA had worse kidney survival than those with none or mild IFTA in both the Kaplan-Meier (p = 0.018) and the competing-risk analyses (p = 0.017). Patients with class IV ± V LN had worse kidney survival than those with non-class IV LN by the Kaplan-Meier method (p = 0.050), but not in the competing-risk analysis (p = 0.154). Worse kidney survival was also found among those with fibrous crescents than those without, in both the Kaplan-Meier (p = 0.010) and the competing-risk (p = 0.011) analyses. By multivariable Cox regression analysis, older age (HR 1.04, 95% CI 1.01-1.07) and class IV ± V LN (HR 5.06, 95% CI 1.82-14.09) were associated with higher risk of ESKD after adjusting for sex, ethnicity, TII, and IFTA. By competing-risk analyses, class IV ± V LN (SHR 3.32, 95% CI 1.25-8.83) and no response to immunosuppressive therapy (SHR 4.55, 95% CI 1.54-13.41) were associated with a higher risk of ESKD, while eGFR >90 mL/min/1.73 m2 (SHR 0.98 for each ml/min/1.73 m2, 95% 0.97-0.99) with a lower risk. CONCLUSIONS: Patients with moderate-to-severe IFTA had worse kidney survival than those with none or mild IFTA. Worse kidney survival was also found among those with class IV LN and fibrous crescents versus those without IV LN and fibrous crescents, respectively.

Diagnóstico de amiloidosis renal AA mediante espectrometría de masas en un paciente con proceso inflamatorio crónico grave abdominal

Introducción la amiloidosis es una enfermedad rara, producto del plegamiento y depósito normal de proteínas en tejidos y órganos. Esta enfermedad puede tener un compromiso renal que se manifiesta con síndrome nefrótico y deterioro de la función renal y su etiología puede estar asociada a amiloidosis con compromiso sistémico, siendo la amiloidosis AL y la amiloidosis AA las más frecuentes, esta última está asociada a inflamación crónica grave de origen infecciosa o autoinmune. Para el diagnóstico es fundamental el estudio sistémico multidisciplinario (hematológico, cardiaco, autoinmune, infeccioso y neoplásico), y cuando hay compromiso renal: la biopsia con estudio completo de microscopía de luz, tinciones especiales incluyendo rojo congo, inmunofluorescencia y microscopía electrónica. Cuando no se logra establecer la causa, la espectrometría de masas es una ayuda crucial para el diagnóstico específico. Objetivo se presenta el caso de un paciente con un proceso inflamatorio crónico grave abdominal que evolucionó a síndrome nefrótico por amiloidosis AA, donde la espectrometría de masas ayudó a aclarar el diagnóstico. Presentación del caso se presenta el caso de un paciente con un proceso inflamatorio crónico grave abdominal que evolucionó a síndrome nefrótico por amiloidosis AA, donde la espectrometría de masas ayudó a aclarar el diagnóstico Discusión y conclusiones se considera que la espectrometría de masas es un estudio diagnóstico muy importante para establecer el diagnóstico etiológico de la amiloidosis cuando otros métodos no han logrado establecerlo.

Introduction Amyloidosis is a rare disease, resulting from the accumulation and deposition of insoluble proteins in tissues or organs. This disease may involve the kidney, resulting in nephrotic syndrome and renal failure. The amyloidosis has been associated with systemic involvement, with AL amyloidosis and AA amyloidosis being the most common. The last is associated with various inflammatory disorders as chronic infections and autoimmune diseases. A multidisciplinary approach is required to the diagnosis (hematologic, cardiac, autoimmune, infectious, neoplastic) and in cases of renal involvement, a kidney biopsy with complete study of light microscopy, special stains including congo red, immunofluorescence, electron microscopy is essential for diagnosis. In cases where the cause cannot be stablished, mass spectrometry is practical tool to the identification of the correct type of amyloidosis. Purpose Here, we present a patient with a chronic and severe abdominal inflammatory process that progressed to a nephrotic syndrome due to AA amyloidosis, in which mass spectrometry helped to clarify the diagnosis. Case presentation Here, we present a patient with a chronic and severe abdominal inflammatory process that progressed to a nephrotic syndrome due to AA amyloidosis, in which mass spectrometry helped to clarify the diagnosis Discussion and conclusion Mass spectrometry is considered a useful diagnostic test to confirm the etiology of amyloidosis, especially if other methods are insufficient to establish it.

Feocromocitoma adrenal gigante derecho. Reporte de un caso / Right giant adrenal pheochromocytoma. A case report

Introducción. El feocromocitoma es una neoplasia endocrina productora de catecolaminas, poco común, que generalmente se origina en la medula suprarrenal, y rara vez en el tejido cromafín extraadrenal, dándosele el nombre de paraganglioma. Existe una gran variedad de signos y síntomas secundarios a la secreción excesiva de catecolaminas por lo que su diagnóstico y tratamiento oportunos son fundamentales para evitar complicaciones potencialmente fatales. Caso clínico. Paciente femenina de 54 años, con dolor abdominal intermitente y con aumento progresivo, localizado en el cuadrante superior derecho. Por estudios imagenológicos se diagnosticó una gran masa suprarrenal derecha, con pruebas de laboratorio que encontraron niveles de metanefrinas y catecolaminas en orina normales. Discusión. Debido al tamaño del tumor y al íntimo contacto con las estructuras adyacentes, se realizó la resección por vía abierta, sin complicaciones y con una buena evolución postoperatoria. El informe anatomopatológico confirmó el diagnóstico de feocromocitoma suprarrenal derecho. Conclusión. Aunque poco frecuente, el feocromocitoma es una patología que se debe sospechar ante la presencia de masas suprarrenales y alteraciones relacionadas con la secreción elevada de catecolaminas. Se debe practicar el tratamiento quirúrgico de forma oportuna.

Introduction. Pheochromocytoma is a rare catecholamine-producing endocrine neoplasm that generally originates in the adrenal medulla, and rarely in extra-adrenal chromaffin tissue, giving it the name of paraganglioma. There is a wide variety of signs and symptoms secondary to excessive secretion of catecholamines, so its timely diagnosis and treatment are essential to avoid potentially fatal complications. Clinical case. A 54-year-old female patient with intermittent abdominal pain and progressive increase, located in the right upper quadrant. By imaging studies, a large right adrenal mass was diagnosed, with laboratory tests that found normal levels of metanephrines and catecholamines in urine. Discussion. Due to the size of the tumor and the intimate contact with the adjacent structures, the resection was performed by open approach, without complications and with a good postoperative evolution. The pathology report confirmed the diagnosis of right adrenal pheochromocytoma. Conclusion. Although rare, pheochromocytoma is a pathology that should be suspected in the presence of adrenal masses and changes related to elevated catecholamine secretion. Surgical treatment should be performed in a timely manner.

[Renal involvement in monoclonal gammopathies]. / Compromiso renal en gammapatías monoclonales.

INTRODUCTION: The kidney is one of the organs most frequently affected by disease processes which produce monoclonal immunoglobins, therefore renal morphological and immunopathological alterations should be clearly recognized. OBJECTIVE: To describe the pathological features of renal involvement in monoclonal gammopathies. MATERIAL AND METHODS: A descriptive, retrospective and cross-sectional study of renal biopsies studied in a single center during a period of 14 years was carried out. RESULTS: 102 cases were included, of which 53% were male patients and the median age was 62.5 years (range 34 - 79). 97% of the biopsies were from native kidneys. The most frequent histopathological diagnosis (31.4%) was myeloma kidney, with kappa being the light chain most frequently deposited (65.6% of cases). AL amyloidosis was the second most common (29.4%) where the lambda chain predominated in 86.6%, followed by light chain deposition disease (20.6%) with the predominance of the kappa chain in 66.6%. CONCLUSIONS: The most frequent renal involvement due to monoclonal gammopathies was myeloma kidney with deposition of kappa light chains, followed by AL lambda amyloidosis; these diseases were found more frequently in patients over 50 years of age.

Compromiso renal en gammapatías monoclonales / Renal involvement in monoclonal gammopathies

Introducción: El riñón es uno de los órganos más comprometidos en enfermedades con producción de inmunoglobulinas monoclonales, por lo que es de gran importancia caracterizar muy bien las alteraciones morfológicas e inmunopatológicas en dicho compromiso. Objetivo: Describir las características del compromiso renal en gammapatías monoclonales desde una perspectiva anatomopatológica. Material y métodos: Se realizó un estudio descriptivo, retrospectivo y transversal, recolectándose biopsias renales estudiadas en un único centro durante un período de 14 años. Resultados: Se incluyeron 102 casos provenientes de 102 pacientes, el 53% de sexo masculino, la mediana de la edad fue 62,5 años (rango 34-79). El diagnóstico histopatológico más frecuente fue riñón de mieloma (31,4% de los casos), siendo kappa la cadena ligera más frecuentemente depositada (65,6% de casos), seguido de amiloidosis AL (29,4%), donde predominó la cadena lambda en un 86,6% y la enfermedad por depósitos de cadenas ligeras (20,6%) con predominio de la cadena kappa: 66,6%. El 97% de las biopsias fueron de riñón nativo. Conclusiones: El compromiso renal por gammapatías monoclonales más frecuente fue el riñón de mieloma con depósito de cadenas ligeras kappa, seguida de la amiloidosis AL lambda; estas enfermedades se encontraron con mayor frecuencia en pacientes mayores de 50 años de edad.(AU)

Introduction: The kidney is one of the organs most frequently affected by disease processes which produce monoclonal immunoglobins, therefore renal morphological and immunopathological alterations should be clearly recognized. Objective: To describe the pathological features of renal involvement in monoclonal gammopathies. Material and methods: A descriptive, retrospective and cross-sectional study of renal biopsies studied in a single center during a period of 14 years was carried out. Results: 102 cases were included, of which 53% were male patients and the median age was 62.5 years (range 34 - 79). 97% of the biopsies were from native kidneys. The most frequent histopathological diagnosis (31.4%) was myeloma kidney, with kappa being the light chain most frequently deposited (65.6% of cases). AL amyloidosis was the second most common (29.4%) where the lambda chain predominated in 86.6%, followed by light chain deposition disease (20.6%) with the predominance of the kappa chain in 66.6%. Conclusions: The most frequent renal involvement due to monoclonal gammopathies was myeloma kidney with deposition of kappa light chains, followed by AL lambda amyloidosis; these diseases were found more frequently in patients over 50 years of age.(AU)

Hallazgos histopatológicos en biopsia renal de pacientes con COVID-19 y compromiso renal / Histopathological findings in renal biopsy of patients with COVID-19 and renal involvement

Introducción. La lesión renal aguda (LRA) en el paciente con COVID-19 ocurre más frecuentemente en presencia de enfermedades crónicas como diabetes, obesidad, hipertensión arterial y enfermedad renal crónica previa, considerándose un fuerte predictor de resultados desfavorables y mortalidad. El propósito de este estudio fue describir las características histopatológicas en biopsias renales de pacientes hospitalizados por COVID-19, que experimentaron algún grado de daño renal durante su hospitalización. Metodología. Se incluyeron 30 pacientes mayores de 18 años, hospitalizados en diferentes centros de atención en Medellín, Colombia, con diagnóstico confirmado de COVID-19, sin antecedente de terapia de reemplazo renal, que durante la infección desarrollaron algún grado de daño renal, y que tuvieran estudio histopatológico de biopsia renal. Se analizaron las características demográficas, formas clínicas de presentación y hallazgos histopatológicos a nivel renal. Resultados. La mayoría de los pacientes eran de sexo masculino (70%). Los antecedentes patológicos más frecuentes fueron la enfermedad renal crónica previa (16,7%), diabetes mellitus (16,7%), trasplante renal (13,3%) y VIH (10%). El 35,7% de los pacientes no tenían ninguna comorbilidad subyacente. La manifestación clínica inicial más frecuente fue la LRA (56,7%). Algunos pacientes tuvieron más de una manifestación clínica inicial. El 100% de los pacientes evaluados tuvieron hallazgos histopatológicos renales, siendo la nefritis tubulointersticial aguda (40%) el más frecuente. Conclusión. Nuestro estudio no descarta una posible asociación del sexo masculino con peores desenlaces en la enfermedad COVID-19. La LRA fue el hallazgo clínico inicial más frecuente. Es posible que los hallazgos histopatológicos del presente estudio puedan ser consecuencia del daño directo a nivel tubulointersticial renal y la mala perfusión renal, dado el estado de choque por la tormenta inflamatoria, el empeoramiento de enfermedades preexistentes, o la superposición clínica con otras entidades. Sin embargo, son necesarios más estudios para dilucidar los mecanismos por los cuales se generan estas lesiones

Acute kidney injury (AKI) in patients with COVID-19 occurs more frequently in the presence of chronic diseases such as diabetes, obesity, hypertension, and previous chronic kidney disease, and is considered a strong predictor of unfavorable outcomes and mortality. The purpose of this study was to describe the histopathological characteristics in kidney biopsies from patients hospitalized for COVID-19, who experienced some degree of kidney damage during their hospitalization. Methodology. We included 30 patients over 18 years of age, hospitalized in different care centers in Medellín, Colombia, with a confirmed diagnosis of COVID-19, without a history of renal replacement therapy, who developed some degree of kidney disease during the infection, and had histopathological study of renal biopsy. Demographic characteristics, clinical manifestations and histopathological findings were analyzed. Results. Most of the patients were male (70%). The most frequent previous pathological findings were chronic kidney disease (16.7%), diabetes mellitus (16.7%), kidney transplant (13.3%) and HIV (10%). 35.7% of the patients did not have any underlying comorbidity. The most frequent initial clinical manifestation was AKI (56.7%). Some patients had more than one initial clinical manifestation. 100% of the patients had renal histopathological findings, with acute tubulointerstitial nephritis (40%) being the most frequent. Conclusion. Our study does not rule out a possible association of the male gender with worse outcomes in COVID-19 disease. AKI was the most common initial clinical finding. It is possible that the histopathological findings of this study may be a consequence of direct damage at the renal tubulointerstitial level and poor renal perfusion due to the inflammatory storm, worsening of pre-existing diseases, or clinical overlap with other entities. However, more studies are needed to elucidate the mechanisms by which these lesions are generated

Hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli in a renal transplant recipient case report / Síndrome hemolítico-urêmica causada por Escherichia coli produtora de toxina Shiga em um relato de caso de receptor de transplante renal

Abstract Thrombotic microangiopathies are disorders characterized by nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and multi-systemic failure. They are classified as thrombotic thrombocytopenic purpura, atypical hemolytic-uremic syndrome, and typical hemolytic uremic syndrome. The latter is associated with intestinal infections by Shiga toxin-producing bacteria. Typical hemolytic uremic syndrome in adults is an extremely rare condition, characterized by high morbidity and mortality. It has been seldom described in solid organ transplant recipients. Here is presented the case of a kidney transplant recipient who had typical hemolytic uremic syndrome with multisystem commitment, refractory to management and with a fatal outcome.

Resumo Microangiopatias trombóticas são distúrbios caracterizados por anemia hemolítica microangiopática não imune, trombocitopenia e insuficiência multissistêmica. Elas são classificadas como púrpura trombocitopênica trombótica, síndrome hemolítico-urêmica atípica e síndrome urêmica hemolítica típica. Essa última está associada a infecções intestinais por bactérias produtoras da toxina Shiga. A síndrome hemolítica urêmica típica em adultos é uma condição extremamente rara, caracterizada por alta morbimortalidade. Esta é raramente descrita em receptores de transplantes de órgãos sólidos. Apresentamos aqui o caso de um receptor de transplante renal que apresentava síndrome hemolítico-urêmica típica com comprometimento multissistêmico, refratário ao tratamento, e com desfecho fatal.

[The relevance of electron microscopy in kidney biopsies to 21st century pathology]. / Microscopía electrónica en biopsias renales: una evaluación de su utilidad en el siglo XXI.

INTRODUCTION: Electron microscopy (EM) has been used in the study of renal biopsies for more than 5 decades; however, it is expensive and the possibility of restricting it to selected cases has been considered. This study aims to reevaluate the necessity for EM in the diagnosis of renal biopsies today. MATERIAL AND METHODS: All renal biopsies taken between 2016 and 2019 with adequate light microscopy (LM), immunofluorescence (IF) and EM studies were included. The initial diagnosis (without EM) and the final diagnosis (with EM) was recorded. EM was considered necessary in cases in which the initial and final diagnoses did not concur, when diagnosis could not be made with LM and IF only or if the EM study revealed further clinically relevant findings. RESULTS: A total of 621 biopsies were included, 498 (80.2%) of native kidneys and 123 (19.8%) of transplanted kidneys. In 115 cases (18.5%) EM had been deemed necessary for diagnosis; it was required more frequently in hereditary diseases (96.8%) and isolated hematuria (88.9%) but less often in nephrotic syndrome (6.7%) and renal transplant biopsy (5.7%) (p < 0.001). CONCLUSIONS: EM was required in less than a fifth of renal biopsies, being more necessary in isolated hematuria and hereditary diseases and less so in nephrotic syndrome and in renal graft biopsies. These findings may prove useful as a guide to case selection protocols in which EM could be considered as a non-mandatory technique.

Kidney transplantation outcomes in lupus nephritis: A 37-year single-center experience from Latin America.

OBJECTIVE: We assessed patient and graft outcomes and prognostic factors in kidney transplantation in patients with end-stage kidney disease (ESKD) secondary to lupus nephritis (LN) undergoing kidney transplantation from August 1977 to December 2014 in a Latin American single center. METHODS: The primary endpoint was patient survival, and the secondary endpoints were death-censored graft survival for the first renal transplant and the rate of recurrent LN (RLN). Kaplan-Meier method was used for survival analysis. Factors predicting patient and death-censored graft survivals were examined by Cox proportional-hazards regression analyses. RESULTS: 185 patients were retrospectively evaluated. Patient survival rates were 88% at one year, 82% at three years, 78% at five years, and 67% at ten years. Death-censored graft survival for the first renal transplant was 93% at one year, 89% at three years, 87% at five years, and 80% at ten years. RLN was diagnosed in 2 patients (1.08%), but no graft was lost because of RLN. Thirty-nine (21.1%) patients died, and 65 (35.1%) patients experienced graft loss during the follow-up. By multivariable analyses, older recipient age and 1-month posttransplantation eGFR <45 ml/min/1.73m2 were associated with lower patient survival and an increased risk of graft loss, while induction immunosuppressive therapy exerted a protective effect on patients' survival. In the subgroup of patients in whom disease activity was measured at the time of transplantation, a higher SLEDAI score was also associated with lower patient survival and an increased risk of graft loss. CONCLUSION: In a mostly Mestizo population, kidney transplantation is an excellent therapeutic alternative in LN patients with ESKD. Older recipient age, an eGFR <45 ml/min/1.73m2 at one month posttransplantation, and disease activity at the time of transplantation are predictive of a lower patient and death-censored graft survival, while induction immunosuppressive therapy has a protective effect on patient survival. RLN is rare and does not influence the risk of graft loss.

Cultural adaptation of two school-based smoking prevention programs in Bogotá, Colombia.

Smoking prevention among adolescents is a public health challenge that is even more significant in low- and middle-income countries where local evidence is limited and smoking rates remain high. Evidence-based interventions could be transferred to low- and middle-income country settings but only after appropriate cultural adaptation. This paper aims to describe the process of the cultural adaptation of two school-based smoking prevention interventions, A Stop Smoking in Schools Trial and Dead Cool, to be implemented in Bogotá, Colombia. A recognized heuristic framework guided the cultural adaptation through five stages. We conducted a concurrent nested mixed-methods study consisting of a qualitative descriptive case study and a quantitative pre- and post quasi-experiment without a control. Contextual, content, training, and implementation modifications were made to the programs to address cultural factors, to maintain the fidelity of implementation, and to increase the pupils' engagement with the programs. Modifications incorporated the suggestions of stakeholders, the original developers, and local community members, whilst considering the feasibility of delivering the programs. Involving stakeholders, original program developers, and community members in the cultural adaptation of evidence-based interventions is essential to properly adapt them to the local context, and to maintain the fidelity of program implementation.

Hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli in a renal transplant recipient case report.

Thrombotic microangiopathies are disorders characterized by nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and multi-systemic failure. They are classified as thrombotic thrombocytopenic purpura, atypical hemolytic-uremic syndrome, and typical hemolytic uremic syndrome. The latter is associated with intestinal infections by Shiga toxin-producing bacteria. Typical hemolytic uremic syndrome in adults is an extremely rare condition, characterized by high morbidity and mortality. It has been seldom described in solid organ transplant recipients. Here is presented the case of a kidney transplant recipient who had typical hemolytic uremic syndrome with multisystem commitment, refractory to management and with a fatal outcome.

Reporte de un caso de Malacoplaquia renal: una enfermedad inusual de difícil diagnóstico / Renal malokaplakia: An unusual disease of difficult diagnosis

RESUMEN La malacoplaquia es una enfermedad granulomatosa crónica inicialmente descrita en las vías urinarias, pero posteriormente encontrada en múltiples órganos. La presentación clínica depende del sitio comprometido; en el tracto urinario produce disuria, dolor pélvico o abdominal, fiebre, malestar general y, a veces, sensación de masa. Su etiopatogenia aún no ha sido completamente entendida, pero se cree que es debida a un trastorno en la función lisosomal de los macrófagos que afecta la destrucción de las bacterias. Histológicamente se caracteriza por presentar macrófagos llamados "células de von Hansemann" e inclusiones de Michaelis-Gutmann. Describimos el caso de una paciente con diabetes mellitus de larga evolución y malacoplaquia renal que le produjo síntomas debilitantes crónicos.

SUMMARY Malakoplakia is a chronic granulomatous disease, firstly described in the urinary tract but then it has been reported in many organs. The clinical presentation depends on the affected site, when it affects the urinary tract the symptoms are dysuria, abdominal or pelvic pain, fever, discomfort, and, something mass sensation. The etiopathogenesis is not yet clear, but is believed it is due to lysosomal dysfunction of the macrophages that affects the destruction of bacteria. Histologically it is characterized for the presence of von Hansemann cells and Michaelis-Gutmann bodies. We describe the case of a patient with long-term diabetes mellitus and renal malakoplakia that caused chronic debilitating symptoms.

Glomerulonefritis C3: una nueva categoría de glomerulonefritis con implicaciones etiopatogénicas / C3 glomerulonephritis: a new category of glomerulonephritis with etiopathogenic implications / Glomerulonefrite C3: uma nova categoria de glomerulonefrite com envolvimentos etiopatogênicas

Introducción: las glomerulonefritis con depósitos exclusivos de la fracción C3 del complemento (GN-C3) pueden implicar alteración en la vía alterna de este. Objetivos: describir retrospectivamente una serie de casos de GN-C3 y determinar la frecuencia con que los pacientes continúan con alteraciones renales y/o hipocomplementemia. Métodos: se evaluaron las características histológicas y clínicas y la evolución de los 22 casos de GN-C3 diagnosticados entre 2004 y 2012 en el Departamento de Patología (Facultad de Medicina, Universidad de Antioquia). Resultados: 14 de los pacientes fueron niños y 12 fueron hombres; la mediana de edad fue de 13 años (rango: 3-65). Diez se presentaron como síndrome nefrítico, siete como GN rápidamente progresiva, tres como insuficiencia renal aguda, uno como insuficiencia renal crónica y uno como síndrome nefrótico-nefrítico; 21 tenían hipocomplementemia C3. Todas las biopsias mostraron GN proliferativa. Ocho pacientes tuvieron remisión completa; cuatro, alteraciones persistentes del uroanálisis; seis desarrollaron enfermedad renal crónica, en cinco de ellos terminal; en cuatro no hubo seguimiento. En nueve pacientes hubo seguimiento de los niveles séricos de C3 y en todos ellos se normalizaron entre 1 y 3 meses después de la biopsia. Conclusiones: las GN-C3 pueden producir alteraciones renales persistentes o recurrentes y evolucionar a la insuficiencia renal terminal. Es recomendable el seguimiento clínico a largo plazo, con mediciones repetidas de los niveles de C3.

Introduction: Glomerulonephritis with only deposits of C3 (GN-C3) could involve alteration on the complement alternative pathway. Objective: To describe retrospectively a series of GNC3 cases and to determine the frequency with which patients continue with renal alterations and/or hypocomplementemia. Methods: The 22 cases of GN-C3 diagnosed between 2004 and 2012 at the Department of Pathology, Faculty of Medicine, University of Antioquia (Medellin, Colombia) were included. Their histological and clinical characteristics and their outcome were evaluated. Results: 14 patients were children and 12 were males. Mean age was 13 years (range: 3-65). Ten presented as a nephritic syndrome, seven as a rapidly progressive GN, three as acute renal failure, one as chronic renal failure, and one as a nephrotic-nephritic syndrome. The C3 fraction of complement was low in 21 cases. All biopsies showed proliferative GN. There was complete remission in eight patients, persistent urinalysis alterations in four, chronic renal failure in six, five of them end-stage. No follow-up was done in four. In nine patients follow-up determination of C3 serum levels was done; in all of them they normalized between 1 to 3 months after biopsy. Conclusions: GN-C3 can produce persistent or recurrent kidney alterations and end-stage renal disease. Long-term follow-up with repeated determinations of C3 is advisable.

Introdução: as glomerulonefrite com depósitos exclusivos da fração C3 do complemento (GN-C3) podem implicar alteração na via alterna deste. Objetivos: descrever retrospectivamente uma série de casos de GN-C3 e determinar a frequência com que os pacientes continuam com alterações renais e/ ou hipocomplementemia. Métodos: avaliaram-se as características histológicas e clínicas e a evolução dos 22 casos de GN-C3 diagnosticados entre 2004 e 2012 no Departamento de Patologia (Faculdade de Medicina, Universidade de Antioquia). Resultados: 14 dos pacientes foram meninos e 12 foram homens; a média de idade foi de 13 anos (casta: 3-65). Dez se apresentaram como síndrome nefrítico, sete como GN rapidamente progressiva, três como insuficiência renal aguda, um como insuficiência renal crônica e um como síndrome nefrótico-nefrítico; 21 tinham hipocomplementemia C3. Todas as biopsias mostraram GN proliferativa. Oito pacientes tiveram remissão completa; quatro, alterações persistentes do uroanálise; seis desenvolveram doença renal crônica, em cinco deles terminal; em quatro não teve seguimento. Em nove pacientes teve seguimento dos níveis séricos de C3 e em todos eles se normalizaram entre 1 e 3 meses depois da biopsia. Conclusões: as GN-C3 podem produzir alterações renais persistentes.

Conferencia clínico-patológica (CPC): paciente con malformación cardíaca congénita y endocarditis infecciosa / Clinico-pathological conference (CPC): patient with congenital heart malformation and infective endocarditis / Conferência clínico patológica (CPC): paciente com malformação cardíaca congênita e endocardites infecciosa

Se presenta el caso de un hombre de 17 años, sin antecedentes de importancia, quien consultó por deterioro de su clase funcional, fatigabilidad y disnea, de un mes de evolución, además de fiebre y síndrome de respuesta inflamatoria sistémica (SIRS), en quien se documentaron una malformación congénita de novo tipo situs inversus con levocardia y una comunicación interventricular (CIV), asociadas a transposición de grandes vasos; durante la hospitalización recibió varios tratamientos con antibióticos sin aislamiento microbiológico ni identificación de la causa de su descompensación hemodinámica. En el examen posmortem se identificó endocarditis por Aspergillus spp., con embolia a diferentes órganos.

We present the case of a 17 year-old-man, with no remarkable past medical history, who had a one month history of worsening functional class, fatigability and dyspnea, in addition to fever and systemic inflammatory response syndrome (SIRS); a de-novo congenital heart malformation, situs inversus type, with levocardia and a ventricular septal defect (VSD) associated with transposition of great vessels were documented. During hospitalization he received several antibiotic treatments without microbiological isolation or identification of the cause of hemodynamic decompensation. An Aspergillus endocarditis with emboli to different organs was identified in the post-mortem examination.

Se apresenta o caso de um homem de 17 anos, sem antecedentes de importância, quem consultou por um mês de evolução de deterioração de sua classe funcional, fatigabilidade e dispneia, além de febre e síndrome de resposta inflamatória sistémica (SIRS) em quem se documentaram uma malformação congênita de novo tipo situs inversus com levocardia e uma comunicação interventricular (CIV), associada a transposição de grandes copos; durante a hospitalização recebeu vários tratamentos com antibióticos sem isolamento microbiológico nem identificação da causa de sua descompensação hemodinâmica. No exame post-mortem se identificou endocardites por Aspergillus spp., com embolia a diferentes órgãos.

Granulomatosis con poliangeítis, doble positividad de anticuerpos contra el citoplasma de neutrófilos y granuloma renal / Granulomatosis with polyangiitis with antibodies to neutrophil cytoplasm, anti-proteinase 3, and anti-myeloperoxidase, plus an unusual finding of a renal granuloma

Las vasculitis sistémicas idiopáticas se caracterizan por inflamación y necrosis de lasparedes de los vasos de origen desconocido1; se han propuesto medicamentos e infeccionescomo posibles disparadores de estas enfermedades2. A continuación se presenta el caso deun paciente con diagnóstico de granulomatosis con poliangeítis, con anticuerpos contrael citoplasma de neutrófilos tanto antiproteinasa 3  como antimieloperoxidasa y con elhallazgo poco usual de granuloma renal...

Idiopathic systemic vasculitis is characterized by inflammation and necrosis of the vessel walls of unknown origin. Medications and infections have been proposed as potential triggers of these diseases. The case is presented on a patient diagnosed with granulomatosis with polyangiitis with antibodies to neutrophil cytoplasm, as well as anti-proteinase 3 and anti-myeloperoxidase, plus the unusual finding of renal granuloma...

The collapsing variant of focal segmental glomerulosclerosis in children / Variante colapsante de la glomeruloesclerosis focal y segmentaria en niños

The collapsing variant of focal segmental glomerulosclerosis (FSGS) is a renal injury that may be idiopathic or associated with various factors; it is characterized by glomerular collapse, which leads to steroid-resistant nephrotic syndrome (NS) and progressive chronic renal failure. FSGS has not been well studied in children, in which most of the cases are idiopathic. We report six cases of the collapsing variant of FSGS in HIV-negative children who were resistant to immunosuppressive treatment. Three of the children died.

La variedad colapsante de glomeruloesclerosis focal y segmentaria (GEFS) es una lesión renal que puede ser idiopática o estar asociada a diferentes factores; se caracteriza por colapso glomerular que lleva a un síndrome nefrótico corticorresistente y a falla renal crónica progresiva. Ha sido poco estudiada en niños y en ellos la mayoría de los casos son idiopáticos. Presentamos seis casos de esta variedad de GEFS en niños negativos para VIH, resistentes al tratamiento inmunosupresor; tres de ellos murieron.

Conferencia clínico-patológica (CPC): trasplantado renal con nódulos pulmonares / Clinicopathological conference (CPC): renal transplant recipient with pulmonary nodules

Se presenta el caso de un hombre de 41 años de edad, trasplantado renal en 2005, quien ingresó al hospital por 10 días de evolución de fiebre, diarrea y síntomas respiratorios. Durante su evolución se documentó la presencia de un nódulo violáceo en la piel, varios nódulos pulmonares y úlceras en el colon; con una fuerte sospecha de infección micótica debido a sus antecedentes, se inició terapia antifúngica, pero finalmente falleció por insuficiencia respiratoria.

We report the case of a 41 year-old man, who had a renal transplantation in 2005. He was admitted to the hospital because of 10 days of fever, diarrhea and respiratory symptoms. During his stay, a nodular lesion in the skin, several pulmonary nodules and colon ulcers were documented; due to his past medical history he was highly suspicious of having a fungal infection; antifungal therapy was started, but he died due to respiratory insufficiency.

Glomerular diseases in a Hispanic population: review of a regional renal biopsy database.

CONTEXT AND OBJECTIVE: Epidemiological data provide useful information for clinical practice and investigations. This study aimed to determine glomerular disease frequencies in a region of Colombia and it represents the basis for future studies. DESIGN AND SETTING: Single-center retrospective analysis at the University of Antioquia, Colombia. METHODS: All native renal biopsies (July 1998 to December 2007) were reviewed, but only glomerular diseases were analyzed. The diagnosis of each case was based on histological, immunopathological and clinical features. RESULTS: A total of 1,040 biopsies were included. In 302 cases (29.0%), the patient's age was

Glomerular diseases in a Hispanic population: review of a regional renal biopsy database / Enfermedades glomerulares en una población hispánica: resultados de un registro regional de biopsias renales

CONTEXT AND OBJECTIVE: Epidemiological data provide useful information for clinical practice and investigations. This study aimed to determine glomerular disease frequencies in a region of Colombia and it represents the basis for future studies. DESIGN AND SETTING: Single-center retrospective analysis at the University of Antioquia, Colombia. METHODS: All native renal biopsies (July 1998 to December 2007) were reviewed, but only glomerular diseases were analyzed. The diagnosis of each case was based on histological, immunopathological and clinical features. RESULTS: A total of 1,040 biopsies were included. In 302 cases (29.0 percent), the patient's age was < 15 years. Primary glomerular diseases were diagnosed in 828 biopsies (79.6 percent) and secondary in 212 (20.4 percent). The most common primary diseases were focal and segmental glomerulosclerosis (FSGS) (34.8 percent), immunoglobulin A (IgA) nephropathy (IgAN) (11.8 percent), membranous glomerulonephritis (MGN) (10.6 percent), minimal change disease (MCD) (10.6 percent), crescentic glomerulonephritis (GN) (5.6 percent), and non-IgA mesangial proliferative GN (5.6 percent). Postinfectious GN represented 10.7 percent of the diagnoses if included as primary GN. Lupus nephritis corresponded to 17.8 percent of the entire series. In adults, the order of the most frequent primary diseases was: FSGS, IgAN, MGN, crescentic GN and MCD. In children (< 15 years), the most frequent were: FSGS, postinfectious GN, MCD, non-IgA mesangial proliferative GN, endocapillary diffuse GN and IgAN. CONCLUSIONS: As among Afro-Americans, FSGS is the most frequent type of glomerulopathy in our population, but in our group, there are more cases of IgAN. The reasons for these findings are unclear. This information is an important contribution towards understanding the prevalence of renal diseases in Latin America.

CONTEXTO Y OBJETIVO: Los datos epidemiológicos dan información útil en clínica e investigación. Nuestro objetivo fue determinar frecuencias de enfermedad glomerular en una región de Colombia y representa la base para trabajos futuros. DISEÑO Y UBICACIÓN: Análisis retrospectivo en un único centro: Universidad de Antioquia, Colombia. MÉTODOS: Todas las biopsias de riñón nativo fueron revisadas (1998 - 2007), pero solo analizamos enfermedades glomerulares. El diagnóstico en cada caso estuvo basado en histología, inmunopatología y características clínicas. RESULTS: 1.040 biopsias fueron incluidas. En 302 casos (29,0 por ciento) la edad del paciente fue < 15 años. El diagnóstico fue enfermedad primaria en 828 biopsias (79,6 por ciento) y secundaria en 212 (20,4 por ciento). Las enfermedades primarias más frecuentes fueron glomeruloesclerosis focal y segmentaria (GEFyS) (34,8 por ciento), nefropatía IgA (NIgA) (11,8 por ciento), glomerulonefritis membranosa (GNM) (10,6 por ciento), enfermedad de cambios mínimos (ECM) (10,6 por ciento), glomerulonefritis (GN) extracapilar (5,6 por ciento) y GN proliferativa mesangial no-IgA (5,6 por ciento). La GN postinfecciosa representa el 10,7 por ciento de glomerulopatías primarias. La nefritis lúpica corresponde al 17,8 por ciento de todos los casos. En adultos el orden de frecuencia de enfermedades primarias es: GEFyS, NIgA, GNM, GN extracapilar y ECM. En niños (< 15 años) las más frecuentes fueron: GEFyS, GN postinfecciosa, ECM, GN mesangial no-IgA, GN endocapilar difusa y NIgA. CONCLUSIONES: Al igual que en afro-americanos, en nuestra población la GEFyS es la glomerulopatía más frecuente, pero en nuestro grupo hay más NIgA. Las razones para estos hallazgos no se conocen. La información presentada aquí es una contribución importante para el entendimiento de la prevalencia de enfermedades renales en Latinoamérica.

Evaluación de la produccion de beta - amiloide por la mutación E280A en el gen de la presenilina 1 / Evaluation of amyloid - beta by the E280A mutation in presenilin gene

Introducción. La mutación E280A en el gen de presenilina 1 se encuentra asociada al grupo familiar más grande del mundo con enfermedad de Alzheimer. La presenilina 1 es un componente esencial del complejo g-secretasa, responsable de la producción del péptido beta-amiloide, considerado clave en la fisiopatogenia de la enfermedad. Objetivo. Determinar si la mutación E280A en presenilina 1 incrementa la producción de beta-amiloide, como reflejo de la ganancia de función g-secretasa. Materiales y métodos. Se evaluaron, por la tinción con rojo congo e inmunohistoquímica, depósitos sistémicos de beta-amiloide en tejidos de cadáveres afectados, portadores de la mutación E280A en la presenilina 1 y cadáveres de no afectados. Se cuantificó por la técnica de ELISA el beta-amiloide de 40 y 42 aminoácidos en cultivos de células CHO que expresan la proteína precursora de amiloide, transfectadas con los cADN de presenilina 1 portando las mutaciones M146L, E280A, DE9 y L392V. Resultados. Se encontraron depósitos proteicos en todos los tejidos estudiados y escasos depósitos de beta-amiloide. No se encontró diferencia en la cantidad de depósitos, ni en su localización. No se observó incremento en la producción de beta-amiloide en las células transfectadas con los cADN de presenilina 1 con las mutaciones comparadas con los controles. Conclusiones. La mutación E280A en presenilina 1 no aumentó la producción de beta-amiloide en tejidos periféricos no neuronales o en el modelo in vitro, contrario a lo que sucede en una ganancia de función de g-secretasa.

Introduction. The E280A mutation of the presenilin 1 gene has been found to be the most common associate in Alzheimer’s patients with a family history of this disease. Presenilin 1 is a critical component of the g-secretase complex and plays an essential role in the production of amyloid-b peptide. This peptide has been strongly associated with the physiopathology of the disease. Objective. The E280A mutation in the presenilin 1 was investigated for increased production of amyloid-b, as a response to gain in g-secretase function. Materials and methods. Levels of systemic amyloid-b were measured with congo red staining and immuno-histochemistry of the tissues of affected cadavers, compared with non-affected cadavers. The 40 and 42 amino acid amyloid-b levels were quantified by ELISA assay in CHO cell cultures. The amyloid precursor protein expressed by the cultures was detected by transfection with the cDNAs of presenilin 1 carrying the M146L, E280A, DE9 y L392V mutations. Results. Protein deposits were found in all tissues investigatged, but only a few with b-amyloid deposition. No differences were observed in the amount or location of amyloid-b between affected and unaffected cadavers. Not increase was noted in the production of amyloid-b from the CHO cells transfected with cDNA from any of the mutations of presenilin 1. Conclusions. The E280A mutation in the presenilin 1 gene was not associated with the increased production of amyloid-b in non-neuronal peripheral tissues, or in the in vitro model. This is in contrast to the expectation in a g-secretase gain of function.