Resumen Los estadios avanzados de la enfermedad renal crónica se asocian a alteraciones en el metabolismo mineral óseo, lo cual aumenta el riesgo de complicaciones graves como la calcifilaxis urémica. A continuación se presenta el caso de un paciente masculino de 65 años, con múltiples comorbilidades, entre ellas enfermedad renal crónica estadio 5 en terapia de hemodiálisis durante largo tiempo, quien presentó hiperparatiroidismo secundario de difícil manejo y se complicó con necrosis peneana secundaria a calcifilaxis urémica. Se considera que este caso puede ser útil para sensibilizar a la comunidad médica sobre la gravedad de la calcifilaxis urémica por lo cual es muy importante la prevención y realización de un diagnóstico temprano antes de que se produzcan complicaciones como la necrosis. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2224).

Abstract The advanced stages of chronic kidney disease are associated with mineral and bone metabolism disorders, which increase the risk of serious complications such as uremic calciphylaxis. Below we present the case of a 65-year-old male patient with multiple comorbidities, including stage 5 chronic kidney disease with long-term hemodialysis treatment, who presented refractory secondary hyperparathyroidism complicated by penile necrosis secondary to uremic calciphylaxis. We believe this case may be useful in sensitizing the medical community on the seriousness of uremic calciphylaxis, emphasizing the importance of prevention and early diagnosis before complications such as necrosis occur. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2224).

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.

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.

Abstract Introduction: kidney transplantation is the standard treatment option for patients with end-stage chronic kidney disease. However, early kidney transplantation is not always possible, and many patients require renal replacement therapy. This study aims to evaluate the short and long-term outcomes after kidney transplantation in patients undergoing dialysis therapy before transplantation. Methods: a retrospective cohort of renal transplant patients older than 18 years of age were evaluated during the years 2005-2018. A descriptive analysis was performed. A proportional Cox-Hazard model was used to evaluate the relationship between patient and transplant survival and pre-transplant dialysis. An analysis of variance was used to compare the glomerular filtration rate during the first two years after transplantation. Results: between 2005-2018, 925 kidney transplants were performed, of which 289 required peritoneal dialysis, 439 haemodialysis and 197 did not require dialysis. Patient and transplant survival rates at 6, 12, 24, 48, 60 months after kidney transplantation were 97.5%, 96.7%, 96.0%, 93.7%, 92.3%, 94.5%, 92.9%, 90.5%, 84.8%, 81.1%. No statistically significant difference was found between peritoneal dialysis patients and hemodialysis patients (p=0.402, p=0.180). Acute rejection occurred in 8.1% of patients in the first year after transplantation and in 13.2% during the entire follow-up. Glomerular filtration rate values were similar in patients with and those without pre-transplant dialysis Conclusions: there were no significant statistical differences in short and long-term outcomes among patients undergoing pre-transplant dialysis vs. no.

Resumen Introducción: el trasplante renal es el tratamiento de elección para los pacientes con enfermedad renal crónica terminal; sin embargo, un trasplante renal anticipado no siempre es posible y muchos pacientes requieren algún tipo de terapia de reemplazo renal previa al trasplante. Objetivo: este estudio tiene como objetivo evaluar los desenlaces a corto y largo plazo, post-trasplante renal, de acuerdo con la modalidad de diálisis previa al trasplante. Métodos: estudio de tipo cohorte retrospectiva. Se incluyeron pacientes mayores de 18 años que recibieron un trasplante renal durante 2005-2018. Inicialmente se realizó estadística descriptiva y posteriormente se realizó análisis multivariado mediante un modelo de riesgos proporcionales de Cox, con el objetivo de evaluar la relación entre la supervivencia del paciente y del injerto y la necesidad de diálisis previa al trasplante. Se evaluó, además, la tasa de filtración glomerular durante los primeros dos años del trasplante renal. Resultados: durante 2005-2018 se realizaron 925 trasplantes renales, 289 estaban en diálisis peritoneal, 439 en hemodiálisis y 197 no estaban en diálisis. La supervivencia del paciente a 6, 12, 24, 48 y 60 meses posterior al trasplante renal fue de 97,5 %, 96,7 %, 96,0 %, 93,7 % y 92,3 %, respectivamente, y del injerto fue de 94,5 %, 92,9 %, 90,5 %, 84,8 %, 81,1 %, también respectivamente, sin encontrar diferencia estadísticamente significativa entre los que tuvieron diálisis peritoneal vs. hemodiálisis (p = 0,402, p = 0,180), tampoco se encontraron diferencias significativas en el análisis multivariado. El 8,1 % de los pacientes presentó rechazo agudo en el primer año post trasplante y el 13,2 % durante todo el seguimiento, sin encontrarse diferencias significativas entre los que habían tenido diálisis peritoneal vs. hemodiálisis. No se encontraron diferencias en la TFG. Conclusiones: no se encontraron diferencias estadísticamente significativas en los desenlaces duros a corto y largo plazo, según la modalidad de diálisis pretrasplante.

Resumo A síndrome hemolítico-urêmica atípica primária é uma doença rara, caracterizada por anemia hemolítica microangiopática não-imune, trombocitopenia e disfunção renal; está relacionado a alterações na regulação da via alternativa do complemento devido a mutações genéticas. A associação com a síndrome nefrótica é incomum. Apresentamos aqui um paciente pediátrico com diagnóstico de síndrome hemolítico-urêmica atípica primária associada à síndrome nefrótica que respondeu ao tratamento com eculizumab.

Abstract Primary atypical hemolytic-uremic syndrome is a rare disease characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction; it is related to alterations in the regulation of the alternative pathway of complement due to genetic mutations. The association with nephrotic syndrome is unusual. We present here a pediatric patient diagnosed with primary atypical hemolytic-uremic syndrome associated with nephrotic syndrome who responded to eculizumab treatment.

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.

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.

Introducción: La nefropatía inducida por medio de contraste es una de las causas principales de lesión renal aguda, lo cual incrementa la morbilidad y mortalidad intrahospitalaria. La nefroprotección con bicarbonato de sodio ha surgido como una estrategia preventiva, sin embargo su eficacia es controversial cuando se compara con estrategias como la hidratación con solución salina al 0.9%. Objetivo: Comparar la efectividad del bicarbonato de sodio versus la hidratación con solución salina al 0.9% en la prevención de la lesión renal aguda inducida por contraste. Métodos: Se realizó una revisión sistemática de los estudios registrados en COCHRANE, PUBMED, MEDLINE, LILACS, SCIELO y EMBASE. Se incluyeron estudios aleatorizados, controlados donde se evaluó el uso de solución salina al 0.9% versus bicarbonato de sodio para prevenir la nefropatía por medio de contraste. Resultados: Se incluyeron 22 estudios (5,686 pacientes). El bicarbonato de sodio no disminuyó el riesgo de nefropatía inducida por contraste (DR= 0.00 IC 95%= -0.02-0.03; p= 0.83, I2=0%). Tampoco se encontró diferencia significativa en la necesidad de terapia de reemplazo renal (DR= 0.00 IC 95%= -0.01-0-01, I2= 0%, p= 0.99); ni en la mortalidad (DR= -0.00, IC 95%= -0.001-0.001, I2= 0%, p= 0.51). Conclusiones: La administración de bicarbonato de sodio no es superior al suministro de solución salina al 0.9% en la prevención de nefropatía inducida por medio de contraste en pacientes con factores de riesgo. Su uso tampoco es superior en la reducción de mortalidad y el requerimiento de terapia de reemplazo renal.

Introduction: Contrast-induced nephropathy is one of the main causes of acute kidney injury and increased hospital-acquired morbidity and mortality. The use of sodium bicarbonate for nephroprotection has emerged as a preventative strategy; however, its efficacy is controversial compared to other strategies, such as hydration using 0.9% saline solution. Objective: To compare the effectiveness of sodium bicarbonate vs. hydration using 0.9% saline solution to prevent contrast-induced acute kidney injury. Methods: A systematic review of studies registered in the COCHRANE, PUBMED, MEDLINE, LILACS, SCIELO and EMBASE databases was conducted. Randomized controlled studies that evaluated the use of 0.9% saline solution vs. sodium bicarbonate to prevent contrast-induced nephropathy were included. Results: A total of 22 studies (5,686 patients) were included. Sodium bicarbonate did not decrease the risk of contrast-induced nephropathy (RD= 0.00; 95% CI= -0.02 to 0.03; p= 0.83; I²= 0%). No significant differences were found in the demand for renal replacement therapy (RD= 0.00; 95% CI= -0.01 to 0-01; I²= 0%; p= 0.99) or in mortality (RD= -0.00; 95% CI= -0.001 to 0.001; I²= 0%; p= 0.51). Conclusions: Sodium bicarbonate administration is not superior to the use of 0.9% saline solution for preventing contrast-induced nephropathy in patients with risk factors, nor is it better at reducing mortality or the need for renal replacement therapy.