BK virus nephropathy in a heart transplant recipient / Nefropatia pelo vírus BK em um receptor de transplante cardíaco

Abstract BK virus nephropathy in kidney transplantation is widely recognized as an important cause of graft dysfunction and loss. In the case of transplants of organs other than kidney, BK virus nephropathy in native kidneys has been recognized as a cause of chronic kidney disease, which is related with immunosuppression; however, the diagnosis is usually late because the renal dysfunction is attributed to other causes, such as toxicity by anticalcineurinic drugs, interstitial nephritis due to medications, hemodynamic changes, diabetes, hypertension, etc. We report a case of BK virus nephropathy in a patient who underwent heart transplantation due to peripartum cardiomyopathy. The kidney biopsy reported active chronic tubulointerstitial nephritis associated with late stage polyomavirus nephritis and the blood viral load for BK virus was positive (logarithm 4.5). The immunosuppressive treatment was reduced, and after two years of follow-up, the patient had stable renal function with a serum creatinine of 2.5 mg/dL (GFR of 23.4 mL/min/1.73m2). We recommend that the BK virus be considered as a cause of renal dysfunction in heart transplant recipients, with the aim of detecting its replication in time to reduce immunosuppressive therapy before irreversible compromise of renal function may manifest.

Resumo A nefropatia pelo vírus BK no transplante renal é amplamente reconhecida como uma importante causa de disfunção e perda do enxerto. No caso de transplantes de órgãos que não sejam rins, a nefropatia pelo vírus BK em rins nativos tem sido reconhecida como uma causa de doença renal crônica, que está relacionada com imunossupressão; entretanto, o diagnóstico é geralmente tardio porque a disfunção renal é atribuída a outras causas, tais como toxicidade por drogas anticalcineurínicas, nefrite intersticial devido a medicamentos, alterações hemodinâmicas, diabetes, hipertensão, etc. Relatamos um caso de nefropatia pelo vírus BK em um paciente que foi submetido a transplante cardíaco devido à cardiomiopatia periparto. A biópsia renal relatou nefrite túbulo-intersticial crônica ativa associada à nefrite por poliomavírus em estágio avançado e a carga viral sanguínea para o vírus BK foi positiva (logaritmo 4,5). O tratamento imunossupressor foi reduzido, e após dois anos de acompanhamento, o paciente apresentava função renal estável com creatinina sérica de 2,5 mg/dL (TFG de 23,4 mL/min/1,73m2). Recomendamos que o vírus BK seja considerado como uma causa de disfunção renal em receptores de transplante cardíaco, com o objetivo de detectar sua replicação a tempo de reduzir a terapia imunossupressora antes que um comprometimento irreversível da função renal possa se manifestar.

Glomerulonefritis rápidamente progresiva mediada por inmunocomplejos (tipo II). Revisión de la literatura / Immune complex-mediated rapidly progressive glomerulonephritis (type II). Literature review

La glomerulonefritis rápidamente progresiva mediada por complejos inmunes (GMNRP II) es un síndrome clínico caracterizado por el rápido deterioro de la función renal asociado a hematuria, edemas y oliguria. Histológicamente se manifiesta como una glomerulonefritis crescéntica, con la presencia de depósitos granulares en la inmunofluorescencia. Aunque es una enfermedad rara, es grave y puede evolucionar a una enfermedad renal crónica, por lo cual es fundamental su identificación temprana. A continuación, se presenta una revisión sobre este tipo de glomerulonefritis, con énfasis en su etiología y en las opciones terapéuticas existentes en la actualidad

Rapidly progressive immune complex-mediated glomerulonephritis (RPGNMN II) is a clinical syndrome characterized by severe deterioration of renal function associated with hematuria, edema, and oliguria. It is histologically characterized as a crescentic glomerulonephritis, with the presence of granular deposits on immunofluorescence. Although it is a rare condition, it is a potentially serious disease that may progress to chronic renal disease, therefore its early identification is essential. Here we present a review of this form of glomerulonephritis, with emphasis on its etiology and the currently available therapeutic options

Tuberculosis urogenital en un paciente con falla renal, estado del arte / Urogenital tuberculosis in a patient with kidney failure, state of the art

RESUMEN La tuberculosis es una enfermedad infecciosa y frecuente en países en vía de desarrollo. Esta puede causar una amplia variedad de complicaciones y presentaciones atípicas con alta morbimortalidad. De la forma genitourinaria se sospechada muy poco, razón por la cual su diagnóstico se hace, usualmente, de forma tardía o no se realiza. Esto conlleva a consecuencias muy graves en los pacientes, por ejemplo, la enfermedad renal crónica terminal. A continuación, se presenta un reporte de caso de una paciente con la anterior enfermedad, secundaria a una tuberculosis renal bilateral diagnosticada tardíamente y se realiza una revisión de la literatura sobre este tema.

SUMMARY Tuberculosis is a common infectious disease in developing countries, which can cause a variety of complications and atypical manifestations with high morbidity and mortality. The urogenital form is rarely suspected, resulting in delayed diagnosis or even no diagnosis, which can have serious consequences for the patients, such as chronic end-stage renal disease. We report on a patient with chronic end-stage renal failure caused by a delayed diagnosis of bilateral renal tuberculosis and a literature review on this topic.

Successful multiple-exchange peritoneal dialysis in a patient with severe hematological toxicity by methotrexate: case report and literature review / Diálise peritoneal com múltiplas trocas bem-sucedida em paciente com grave toxicidade hematológica por metotrexato: relato de caso e revisão de literatura

Abstract Methotrexate is an effective medication to control several diseases; however, it can be very toxic, being myelosuppression one of its main adverse effects, which increases in severity and frequency in patients with renal failure. We present the case of a 68-year-old man with chronic, end-stage renal disease associated with ANCA vasculitis, under treatment with peritoneal dialysis, who received the medication at a low dose, indicated by disease activity, which presented as a complication with severe pancytopenia with mucositis that improved with support measures and multiple-exchange peritoneal dialysis. We reviewed 20 cases published to date of pancytopenia associated with methotrexate in patients on dialysis and found high morbidity and mortality, which is why its use in this type of patient is not recommended. However, when this complication occurs, a therapeutic option could be the use of multiple-exchange peritoneal dialysis in addition to supportive therapy for drug-related toxicity, although it is recognized that studies are required to show the role of multiple-exchange peritoneal dialysis in the removal of this medication.

Resumo Apesar de sua toxicidade, o metotrexato é um medicamento eficaz no controle de várias doenças. A mielossupressão, um de seus principais efeitos adversos, aumenta em gravidade e frequência nos pacientes com insuficiência renal. Apresentamos o caso de um homem de 68 anos de idade com doença renal terminal relacionada à vasculite associada ao ANCA em diálise peritoneal, que recebeu a medicação em dose baixa em função da atividade da doença e que teve como complicação pancitopenia grave com mucosite, tratada com medidas de suporte e diálise peritoneal com múltiplas trocas. Revisamos 20 casos publicados até o presente momento sobre pancitopenia associada a metotrexato em pacientes em diálise. Foi identificada alta morbidade e mortalidade, razão pela qual seu uso nesse tipo de paciente não é recomendado. No entanto, quando esta complicação ocorre, uma opção terapêutica pode ser o uso de diálise peritoneal com múltiplas trocas, além da terapia de suporte para toxicidade medicamentosa. Maiores estudos são necessários para demonstrar o papel da diálise peritoneal com múltiplas trocas na remoção desse medicamento.

Tres casos de mieloma múltiple de novo después de trasplante renal / Three cases of de novo multiple myeloma after kidney transplantation

RESUMEN La enfermedad renal asociada a cadenas ligeras es frecuente en el contexto de las gammapatías monoclonales, afecta los glomérulos o los túbulos renales, y su causa más común es el mieloma múltiple. Puede desarrollarse después de un trasplante renal por recurrencia de un mieloma múltiple ya existente, o puede ser de diagnóstico nuevo y presentarse con deterioro de la función renal y proteinuria. Siempre se requiere una biopsia renal para confirmar el diagnóstico.

ABSTRACT Light chain-associated kidney compromise is frequent in patients with monoclonal gammopathies; it affects the glomeruli or the tubules, and its most common cause is multiple myeloma. It may develop after a kidney transplant due to recurrence of a preexisting multiple myeloma or it can be a de novo disease manifesting as graft dysfunction and proteinuria. A kidney biopsy is always necessary to confirm the diagnosis.

Treatment of post-transplantation lymphoproliferative disorders after kidney transplant with rituximab and conversion to m-TOR inhibitor / Tratamiento de la enfermedad linfoproliferativa post-trasplante renal con Rituximab y conversión a inhibidor m-TOR

Abstract Background: Post-transplantation lymphoproliferative disorders are serious complications of organ transplantation which treatment is not yet standardized. Objective: To describe the clinical response, overall and graft survival of patients in our center with this complication after kidney transplantation, which received rituximab as part of their treatment as well as conversion to m-TOR. Methods: Retrospective study, which included patients, diagnosed with post-transplant lymphoproliferative disorders after kidney transplantation from January 2011 to July 2014. Results: Eight cases were found with a wide spectrum of clinical presentations. Most had monomorphic histology, 85% were associated with Epstein-Barr virus, 25% of patients had tumor involvement of the renal graft, and 12.5% ​​had primary central nervous system lymphoma. All patients were managed with reduction of immunosuppression, conversion to m-TOR (except one who lost the graft at diagnosis) and rituximab-based therapy. The overall response rate was 87.5% (62.5% complete response, 25% partial response). Survival was 87.5% with a median follow-up of 34 months. An additional patient lost the graft, with chronic nephropathy already known. All the remaining patients had stable renal function. Conclusions: There are no standardized treatment regimens for lymphoproliferative disorders after kidney transplantation, but these patients can be managed successfully with reduction of immunosuppression, conversion to m-TOR and rituximab-based schemes.

Resumen Antecedente: La enfermedad linfoproliferativa post-trasplante es una complicación grave del trasplante de órganos cuyo tratamiento aún no se encuentra estandarizado. Objetivo: Describir la respuesta clínica, supervivencia global y del injerto en pacientes con esta complicación post trasplante renal en nuestro centro y que recibieron rituximab como parte de su tratamiento y la conversión a m-TOR. Métodos: Estudio retrospectivo que incluyó pacientes con diagnóstico de enfermedad linfoproliferativa postrasplante renal entre enero de 2011 y julio de 2014. Resultados: Se encontraron ocho casos, con presentaciones clínicas variables. La mayoría correspondieron a histología monomórfica, en 85% se asoció con virus de Epstein-Barr, 25% de los pacientes tenían compromiso tumoral del injerto renal y 12.5% linfoma primario de sistema nervioso central. Todos los pacientes se manejaron con reducción de inmunosupresión, conversión a m-TOR (excepto uno que perdió el injerto al diagnóstico) y tratamiento basado en rituximab. La tasa de respuesta global fue del 87.5% (62.5% respuesta completa, 25% respuesta parcial). La supervivencia fue del 87.5% con una mediana de seguimiento de 34 meses. Un paciente adicional perdió el injerto renal, con nefropatía crónica ya conocida. Los pacientes restantes con función renal estable. Conclusiones: No existen esquemas estandarizados de tratamiento para la enfermedad linfoproliferativa post-trasplante renal, pero estos pacientes pueden ser manejados de forma exitosa con reducción de la inmunosupresión, conversión a m-TOR y esquemas basados en rituximab.

Insuficiencia renal aguda inducida por rabdomiolisis / Insuficiência renal aguda induzida por rabdomiólise / Acute renal failure due to rhabdomyolyisis

La insuficiencia renal aguda (IRA) es una causa frecuente de morbilidad y mortalidad en los servicios de urgencias, hospitalización y cuidado crítico, siendo la rabdomiolisis responsable del 15 % de los casos en los centros hospitalarios de alta complejidad. La rabdomiolisis consiste en daño muscular con necrosis y liberación a la circulación de componentes intracelulares, y puede tener múltiples causas. El daño renal por rabdomiolisis es multifactorial e incluye obstrucción tubular, vasoconstricción y daño oxidativo. El tratamiento principal de la IRA por rabdomiolisis es la hidratación, forzar la diuresis y evitar los fármacos nefrotóxicos. Cuando el daño es lo suficientemente grave para causar uremia, acidosis metabólica, hiperpotasemia y sobrecarga de volumen se debe recurrir a la terapia de reemplazo renal. Aunque la regla es que se recupere la función renal, estos pacientes tienen mayor riesgo futuro de desarrollar enfermedad renal crónica...

Acute renal failure is a frequent cause of morbidity and mortality in emergency, hospitalization and critical care services. In 15 % of cases it is due to rhabdomyolysis, in which there is breakdown of skeletal muscle with massive necrosis and leakage of muscle cell contents into the circulation. It has many different etiologies. The rhabdomyolysis-induced acute kidney injury results from the combination of several mechanisms, including tubular obstruction, vasoconstriction and oxidative stress. The most important therapeutic measures are: Aggressive repletion of fluids, forced diuresis and avoidance of exposure to nephrotoxic substances. In cases of severe uremia, metabolic acidosis, hiperkalemia or fluid overload it is necessary to start renal replacement therapy. As a rule, kidney function is completely recovered, but these patients have higher risk of future chronic kidney disease...

A insuficiência renal aguda (IRA) é uma causa frequente de morbidade e mortalidade entre os serviços de emergência, internação e cuidados críticos. A rabdomiólise é responsável por 15% dos casos. Consiste de dano muscular com necrose e liberação à circulação de componentes intercelulares, e pode ter varias causas. O dano renal por rabdomiólise é multifatorial e inclui obstrução tubular, vasoconstrição e dano oxidativo. O principal tratamento da IRA por rabdomiólise é a hidratação, forçar a diurese e evitar fármacos nefrotóxicos. Quando o dano é suficientemente grave para causar uremia, acidose metabólica, hiperpotassemia e sobrecarga de volume se deve recorrer à terapia de substituição renal. Embora a regra é que se recupera a função renal, esses pacientes têm um maior risco futuro de desenvolver doença renal crônica...

Clinical outcomes of kidney transplants on patients with end-stage renal disease secondary to lupus nephritis, polycystic kidney disease and diabetic nephropathy / Resultados clínicos de trasplantes de riñón en pacientes con enfermedad renal en etapa terminal secundaria anefritis lupus, enfermedad poliquística renal y nefropatía diabética

Background: Patients with lupus nephritis could progress to endstage renal disease (10-22%); hence, kidney transplants should be considered as the treatment of choice for these patients. Objective: To evaluate the clinical outcomes after kidney transplants in patients with chronic kidney diseases secondary to lupus nephritis, polycystic kidney disease and diabetes nephropathy at Pablo Tobon Uribe Hospital. Methods: A descriptive and retrospective study performed at one kidney transplant center between 2005 and 2013. Results: A total of 136 patients, 27 with lupus nephritis (19.9%), 31 with polycystic kidney disease (22.8%) and 78 with diabetes nephropathy (57.4%), were included in the study. The graft survivals after one, three and five years were 96.3%, 82.5% and 82.5% for lupus nephritis; 90%, 86% and 76.5% for polycystic kidney disease and 91.7%, 80.3% and 67.9% for diabetes nephropathy, respectively, with no significant differences (p= 0.488); the rate of lupus nephritis recurrence was 0.94%/person-year. The etiology of lupus vs diabetes vs polycystic disease was not a risk factor for a decreased time of graft survival (Hazard ratio: 1.43; 95% CI: 0.52-3.93). Conclusion: Kidney transplant patients with end stage renal disease secondary to lupus nephritis has similar graft and patient survival success rates to patients with other kidney diseases. The complication rate and risk of recurrence for lupus nephritis are low. Kidney transplants should be considered as the treatment of choice for patients with end stage renal disease secondary to lupus nephritis.

Antecedentes: Pacientes con nefritis lúpica pueden progresar a enfermedad renal crónica terminal (10-22%); en estos pacientes el trasplante renal debe ser considerado como la terapia de elección. Objetivo: Evaluar los desenlaces clínicos de un grupo de pacientes con enfermedad renal crónica terminal por nefropatía lúpica, enfermedad renal poliquística y nefropatía diabética que fueron sometidos a trasplante renal en el Hospital Pablo Tobón Uribe. Métodos: Estudio retrospectivo, descriptivo, realizado en un solo centro de trasplante renal, durante el período 2005-2013. Resultados: Se evaluaron 136 pacientes: 27 con nefritis lúpica (19.9%), 31 con enfermedad renal poliquística (22.8%) y 78 con nefropatía diabética (57.4%). La supervivencia del injerto a uno, tres y cinco años fue de de 96.3%, 82.5% y 82.5% en nefropatía lúpica, 90%, 86% y 76.5% en enfermedad renal poliquística y 91.7%, 80.3% y 67.9% en nefropatía diabética respectivamente, sin diferencias estadísticas significativas (Long Rank test= 0.488). La tasa de recurrencia de nefritis lúpica posterior al trasplante renal fue de 0.94%/persona-año. Tener lupus vs diabetes o enfermedad renal poliquística no fue un factor de riesgo para disminución del tiempo de supervivencia del injerto (Hazard ratio= 1.43; 95% IC= 0.52-3.93). Conclusiones: Los pacientes enfermedad renal crónica terminal secundaria a nefritis lúpica, que son llevados a trasplante renal tienen tasas de éxito similar en cuanto a supervivencia del injerto y del paciente, al compararlos con otras enfermedades renales. La tasa de complicaciones y el riesgo de recurrencia de la nefropatía lúpica son bajos. El trasplante renal debe ser considerado como la terapia de elección para los pacientes con enfermedad renal crónica estadio terminal secundaria a nefritis lúpica.

Enfermedad renal crónica en pacientes trasplantados de hígado en el Hospital Pablo Tobón Uribe 2005-2013 / Chronic Kidney Disease in Liver Transplant Patients in the Hospital Pablo Tobón Uribe 2005-2013

Avances en la terapia inmunosupresora han revolucionado los resultados a largo plazo del trasplante de hígado, sin embargo esto ha incrementado la prevalencia de enfermedad renal crónica (ERC); existen algunos factores de riesgo asociados como el uso de inhibidores de calcineurínicos, la presencia de diabetes e hipertensión. El objetivo de este estudio es determinar la incidencia de ERC en los pacientes trasplantados de hígado del hospital Pablo Tobón Uribe durante los años 2005-2013 y evaluar las complicaciones asociadas. Metodología: cohorte retrospectiva. Resultados: se evaluaron 215 pacientes trasplantados de hígado, la edad mediana fue de 50,37 años (DE ± 12,6), el 42,8% mujeres; 3,3% de los pacientes necesitaron terapia de reemplazo renal al primer mes del trasplante; la terapia inmunosupresora más utilizada fue ciclosporina en el 90,7%. Durante el seguimiento la tasa de filtración disminuye con el tiempo, con una mediana de 86,2 mL/min/1,73 (DE ± 25,9) al momento del trasplante y llegando a 74,2 mL/min/1,73 (DE ± 24,5) a 3 años de seguimiento. La velocidad de deterioro de la función renal por medio del modelo de ecuaciones generalizadas estimadas fue de 3,5 mL/año (IC: 95% 2,44-4,74, p= 0,000). Al momento del trasplante de hígado el 16,3% de los pacientes presentaban una TFG menor a 60 mL/min; a tres años de seguimiento fue de 29,6%; al agrupar las complicaciones encontradas, según presencia o no de disfunción renal al final del seguimiento, encontramos que a excepción de la muerte, la presencia de enfermedad cerebrovascular y enfermedad coronaria fue similar en ambos grupos. Conclusión: la ERC es una complicación frecuente en los pacientes trasplantados de hígado, nuestra recomendación es el control frecuente de los marcadores de daño renal.

Advances in immunosuppressive therapy have revolutionized long-term results of liver transplantation, but this has increased the prevalence of chronic kidney disease (CKD). Some risk factors including diabetes and hypertension are associated with the use of calcineurin inhibitors. The objective of this study is to determine the incidence of CKD in liver transplant patients at the Hospital Pablo Tobon Uribe from 2005 to 2013 and then assess associated complications. Methods: This is a retrospective cohort study. Results: This study evaluated 215 patients with liver transplants. Average patient age at transplant was 50.37 years (SD +/- 12.6), and 42.8% of the patients were women. Kidney replacements were required by 3.3% of the patients within the first month after liver transplantation. The most frequently used immunosuppressive therapy was cyclosporine which was used for 90.7% of the patients. During follow-up, the filtration rate decreased over time with a median of 86.2ml/min/1.73 (SD +/- 25.9) at transplant. This reached 74.2ml/min/1.73 (SD +/- 24.5) at 3 years follow-up. The rate of deterioration of renal function determined by generalized estimated equations was 3.5ml/year (95% CI 2.44 to 4.74, p = 0.000). At the time of liver transplantation 16.3% of patients had glomerular filtration rates of less than 60ml/min. After three years of follow-up, 29.6% of patients had glomerular filtration rates of less than 60ml/min. By grouping complications such as the presence or absence of renal dysfunction at follow-up, we found that, except for death, the presence of cerebrovascular disease and coronary heart disease was similar in both groups. Conclusion: CKD is a frequent complication in liver transplant patients. Our recommendation is frequent monitoring of kidney damage markers.

Uso de rituximab en pacientes pediátricos con síndrome nefrótico córtico-resistente. Experiencia de un centro / Use of rituximab in pediatric patients with steroidresistant nephrotic syndrome. A single center study / Uso de rituximab em pacientes pediátricos com síndrome nefrótica córtico-resistente. Experiência de um centro

Introducción: los esteroides son el tratamiento de elección del síndrome nefrótico con tasas de éxito hasta del 90%. En pacientes que no responden adecuadamente a ellos, se han usado diferentes esquemas de inmunosupresión. Objetivo y métodos: describir la respuesta terapéutica en un grupo de siete niños con síndrome nefrótico córtico-dependiente (SNCD) o córtico-resistente (SNCR) que recibieron tratamiento con rituximab y micofenolato mofetil en un hospital universitario de la ciudad de Medellín durante los años 2010-2012. Resultados: dos pacientes tenían SNCD y cinco, SNCR; la mediana de edad en el momento del diagnóstico fue de 2 años (p25-75: 1-5); seis meses después de la aplicación del rituximab se encontró disminución de la proteinuria en el 93% de los pacientes; los esteroides se lograron suspender en el 100%; además, disminuyó el número de recaídas; sin embargo, la proteinuria reapareció un año después de dicho tratamiento. Conclusión: con el rituximab disminuyen la proteinuria y la dosis de esteroides, pero la enfermedad recurre 12 meses después de usarlo. Se sugiere hacer otro estudio evaluando el efecto de una segunda dosis de rituximab al año de la primera.

Introduction: Steroids are the cornerstone of therapy for nephrotic syndrome (NS) with a remission rate as high as 90%. In patients who do not respond to them or are steroid dependent, other immunosuppressive drugs have been used. Although rituximab use in NS is off-label, many authors have published their experience with it. Objective and methods: To describe retrospectively a group of seven children with nephrotic syndrome, either steroid-dependent (SDNS) or steroid- resistant (SRNS), treated with rituximab and mycophenolate, at Pablo Tobón Uribe Hospital, in Medellín, Colombia. Results: Two patients with SDNS and five with SRNS were evaluated; median age at diagnosis was 2 years (p25-75: 1-5); six months after treatment with rituximab there was reduction in proteinuria (93%), in the steroid dose (100%) and in the relapse episodes. However, proteinuria reappeared 12 months after treatment. Conclusion: During the first year after rituximab treatment of NS there is reduction in proteinuria and in the steroid dose, but thereafter there is relapse. It is suggested to carry out another study using a second dose of rituximab one year after the first one.

Introdução: os esteroides são o tratamento de eleição da síndrome nefrótica com taxas de sucesso até da 90%. Em pacientes que não respondem adequadamente a eles, usaram-se diferentes esquemas de imunossupressão. Não está aprovado o uso do rituximab em pacientes com síndrome nefrótica, mas alguns grupos publicaram sua experiência com ele nesta doença. Objetivo e métodos: descrever a resposta terapêutica num grupo de sete meninos com síndrome nefrótica córtico-dependente (SNCD) ou córtico -resistente (SNCR) que receberam tratamento com rituximab e micofenolato mofetil num hospital universitário da cidade de Medellín durante os anos 2010-2012. Resultados: dois pacientes tinham SNCD e cinco, SNCR; a idade no momento do diagnóstico foi de 2 anos (p25-75: 1-5); seis meses depois da aplicação do rituximab se encontrou diminuição de 93% da proteinúria e de 100% na dose de prednisolona; ademais, diminuiu o número de recaídas; no entanto, a proteinúria reapareceu um ano depois de dito tratamento. Conclusão: com o rituximab diminuem a proteinúria e a dose de esteroides, mas a doença recorre 12 meses depois de usá-lo. Sugere-se fazer outro estudo avaliando o efeito de uma segunda dose de rituximab no ano seguinte da primeira dose.

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.