Development and Validation of a New Score to Assess the Risk of Posttransplantation Diabetes Mellitus in Kidney Transplant Recipients.

Background: Posttransplantation diabetes mellitus (PTDM) is a serious complication of solid organ transplantation. It is associated with major adverse cardiovascular events, which are a leading cause of morbidity and mortality in transplant patients. This study aimed to develop and validate a score to predict the risk of PTDM in kidney transplant recipients. Methods: A single-center retrospective cohort study was conducted in a tertiary care hospital in Medellín, Colombia, between 2005 and 2019. Data from 727 kidney transplant recipients were used to develop a risk prediction model. Significant predictors with competing risks were identified using time-dependent Cox proportional hazard regression models. To build the prediction model, the score for each variable was weighted using calculated regression coefficients. External validation was performed using independent data, including 198 kidney transplant recipients from Tübingen, Germany. Results: Among the 727 kidney transplant recipients, 122 developed PTDM. The predictive model was based on 5 predictors (age, gender, body mass index, tacrolimus therapy, and transient posttransplantation hyperglycemia) and exhibited good predictive performance (C-index: 0.7 [95% confidence interval, 0.65-0.76]). The risk score, which included 33 patients with PTDM, was used as a validation data set. The results showed good discrimination (C-index: 0.72 [95% confidence interval, 0.62-0.84]). The Brier score and calibration plot demonstrated an acceptable fit capability in external validation. Conclusions: We proposed and validated a prognostic model to predict the risk of PTDM, which performed well in discrimination and calibration, and is a simple score for use and implementation by means of a nomogram for routine clinical application.

Impacto del lugar de residencia en la supervivencia de los pacientes con trasplante renal. ¿Puede la telemedicina mejorar este desenlace?

Antecedente: el trasplante renal es el tratamiento de elección en la enfermedad crónica terminal. Un adecuado seguimiento en el postrasplante mejora la supervivencia del injerto y del paciente a largo plazo. Objetivo: comparar los desenlaces clínicos de la población trasplantada renal que vive en el área metropolitana de Medellín con los que residen por fuera de este lugar, con el fin de plantear un modelo de atención para el seguimiento por medio de la telemedicina. Métodos: estudio descriptivo, retrospectivo y de un único centro. Se determinó la tasa de supervivencia mediante las curvas de Kaplan-Meier. Resultados: durante el período 2005-2015 se realizaron 754 trasplantes, el 42 % vivía por fuera del área metropolitana. Al agrupar esta cohorte según el lugar de residencia, se observó que la supervivencia de los pacientes residentes en el área metropolitana a 1, 3 y 5 años fue del 96,8 %, 93,7 % y 91,8 %, respectivamente, en contraste con el 94,4 %, 90,3 % y 85,2 % de los del área rural. Esta diferencia fue estadísticamente significativa a favor de los que viven en Medellín (log-rank test p = 0,048; Hazard ratio = 1,68; IC 95 % 0,99-2,84, p = 0,052). Conclusión: la supervivencia fue inferior en los pacientes trasplantados renales que viven por fuera del área Metropolitana. Lo anterior motiva el desarrollo de un modelo de atención para estos pacientes mediado por la telemedicina para facilitar el acceso al seguimiento postrasplante.

Background: Kidney transplantation is the treatment of choice for end-stage renal disease. An adequate post-transplant follow-up improves the graft and patient's long-term survival. Objective: The aim of this study was to compare the outcomes of kidney transplant patients who live in the Medellin metropolitan area with those who live outside this area, to propose a model for follow-up care through telemedicine. Methods: Descriptive, retrospective and one-center study. Kaplan-Meier method was used to determine the survival rate. Results: Between 2005 and 2015, 742 patients were transplanted, 42% of whom lived outside the metropolitan area. The survival rates after 1, 3 and 5 years of treatment in patients in the metropolitan area of Medellín compared to those outside were 96.8%, 93.7% y 91.8% and 94.4%, 90.3% y 85.2% respectively, with statistically significant differences (Log-rank test p=0.048, Hazard ratio 1.68, IC 95% 0.99-2.84, p=0.052). Conclusion: The survival rate was lower in kidney transplant patients living outside the urban area. These findings motivate the development of a telemedicine project to facilitate the follow-up of these patients after a kidney transplantation.

Síndrome nefrótico como manifestación de microangiopatía trombótica secundaria al uso crónico de sunitinib / Nephrotic syndrome as a manifestation of thrombotic microangiopathy due to long-term use of sunitinib

El síndrome nefrótico en los pacientes con cáncer se puede asociar a su enfermedad de base o al tratamiento quimioterapéutico. El cáncer de órganos sólidos puede producir una glomerulonefritis membranosa que se manifiesta con síndrome nefrótico; otras presentaciones histológicas menos frecuentes son la glomeruloesclerosis focal y segmentaria y la enfermedad de cambios mínimos. Adicionalmente, los tratamientos quimioterapéuticos pueden causar toxicidad renal por afección de los pequeños vasos sanguíneos, los glomérulos, los túbulos y el intersticio. Los inhibidores de la tirosina quinasa como el sunitinib pueden causar daño endotelial y podocitario, produciendo una microangiopatía trombótica limitada a los riñones, que se manifiesta con proteinuria e hipertensión. Se presenta el caso de un hombre anciano con tumor del estroma gastrointestinal (GIST, por sus siglas en inglés) que fue tratado con sunitinib y como complicación presentó una microangiopatía trombótica manifestada con síndrome nefrótico e hipertensión de difícil control, que se controló al suspender este medicamento pero con desenlace fatal por su neoplasia maligna. (AU)

Nephrotic syndrome in patients with cancer may be related to the primary malignancy or chemotherapeutic therapy. Solid organ cancers may cause membranous glomerulonephritis manifesting with nephrotic syndrome; other less common histologic presentations include focal and segmental glomerulosclerosis and minimal change disease. In addition, chemotherapy agents can cause renal toxicity by affecting the small blood vessels, glomeruli, tubules, and interstitium. Tyrosine kinase inhibitors such as sunitinib may cause endothelial and podocyte damage leading to renal limited thrombotic microangiopathy, manifested by proteinuria and hypertension. We report a case of an elderly man with gastrointestinal stromal tumor (GIST) on treatment with sunitinib who had as a complication of a thrombotic microangiopathy manifested with nephrotic syndrome and difficult-to-control hypertension, which was controlled by stopping this drug but with a fatal outcome due to its malignant neoplasm. (AU)

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.

Penile necrosis secondary to uremic calciphylaxis / Necrosis peneana secundaria a calcifilaxis urémica

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).

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).

Chronic gemcitabine-induced thrombotic microangiopathy in the kidneys / Microangiopatía trombótica crónica localizada en riñones asociada al uso de gemcitabina

Abstract Thrombotic microangiopathies (TMAs) are characterized by microvascular occlusion secondary to diffuse endothelial damage which produces inflammation, platelet aggregation and red blood cell destruction, causing ischemic injury to the affected organ. They are clinically characterized by Coombs-negative microangiopathic hemolytic anemia, and multiple organ damage (mainly of the kidneys, central nervous system, cardiovascular apparatus and gastrointestinal tract). They may occur systemically or locally, and they have multiple etiologies. In patients with cancer, determining the cause of thrombotic microangiopathy is a great diagnostic challenge, with the most frequent etiologies being active malignant neoplasms, disseminated intravascular coagulation, infections and antineoplastic drugs. We present the clinical case of a patient with unresectable pancreatic adenocarcinoma on chronic gemcitabine treatment, and highlight the importance of suspecting and distinguishing chemotherapy-induced TMAs from neoplasm-induced TMAs, as their prognosis and treatment are very different. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2249).

Resumen Las microangiopatías trombóticas (MAT) se caracterizan por la oclusión microvascular como consecuencia de una lesión endotelial difusa que produce inflamación, agregación plaquetaria y destrucción de glóbulos rojos, causando daño isquémico del órgano afectado. Se caracterizan clínicamente por anemia hemolítica microangiopática, Coombs negativo, daño multiorgánico (principalmente de riñones, sistema nervioso central, aparato cardiovascular y tracto gastrointestinal). Su presentación puede ser sistémica o localizada y sus etiologías son múltiples. En los pacientes con cáncer es un gran reto diagnóstico establecer la causa de la microangiopatía trombótica, siendo las etiologías más frecuentes la neoplasia maligna activa, la coagulación intravascular diseminada, infecciones y medicamentos antineoplásicos. Se presenta el caso clínico de una paciente con adenocarcinoma cáncer de páncreas irresecable, en manejo crónico con gemcitabina y se resalta la importancia de sospechar y distinguir la MAT inducida por quimioterapia, de la causada por la neoplasia ya que el pronóstico y tratamiento son muy diferentes. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2249).

Trasplante renal en pacientes con infección por el virus de la inmunodeficiencia humana: revisión de la literatura / Renal transplantation in patients with human immunodeficiency virus infection: a literature review

Resumen El trasplante renal es el tratamiento de elección en la enfermedad renal crónica terminal porque mejora la calidad de vida y la supervivencia de los pacientes al compararlo con la diálisis. Sin embargo, para mantener un injerto funcional y evitar el rechazo es necesario el uso de inmunosupresión potente durante toda la vida del injerto, lo cual puede tener como complicaciones una mayor susceptibilidad a presentar infecciones, desarrollo de cáncer, alteraciones metabólicas y problemas cardiovasculares. Los pacientes infectados con el virus de la inmunodeficiencia humana tienen alto riesgo de desarrollar enfermedad renal crónica terminal por múltiples causas. En el siglo pasado, el trasplante renal se consideraba contraindicado para estos pacientes. No obstante, hoy en día el trasplante renal se considera una opción terapéutica para pacientes adecuadamente seleccionados y con protocolos de manejo bien establecidos. Reportándose supervivencia reportadas del injerto y del paciente a tres años de 88,2 % y 82,6 % respectivamente. Este artículo de revisión tiene como objetivo revisar la experiencia mundial existente en el manejo de los pacientes trasplantados renal con infección por VIH.

Summary Kidney transplantation is the recommended treatment for end-stage chronic kidney disease, improving patients' quality of life and survival compared to dialysis. Nevertheless, to keep a functional graft and avoid rejection, strong immunosuppression is required during the graft's lifetime, which can lead to complications such as increased susceptibility to infections, development of cancer, metabolic changes and cardiovascular problems. Patients infected with the human immunodeficiency virus are at high risk of developing end-stage renal disease. Previous this century, kidney transplantation was considered contraindicated for these patients group. However nowadays, kidney transplantation is a therapeutic option for well-selected patients and with well-established treatment protocols. Several studies reported a three-year graft survival rate of 88,2% and patient survival of 82,6%. In this article, we present an overview of the worldwide experience with the treatment of kidney transplant patients with HIV infection.

Enfermedad renal crónica de las comunidades agrícolas, una revisión de la literatura / Chronic Kidney Disease in Agricultural Communities, review of the literature

Resumen La enfermedad renal crónica (ERC) se define como la alteración funcional o estructural progresiva de los riñones que persiste por 3 meses o más. Esta enfermedad afecta el 10 al 15 % de la población mundial, siendo la diabetes mellitus, la hipertensión arterial, las glomerulopatías primarias y las enfermedades genéticas las etiologías más frecuentemente asociadas. Sin embargo, en los países pobres se reportan otras enfermedades causantes de la ERC; entre ellas la nefropatía mesoamericana (NM). La NM se presenta principalmente en la población masculina joven, lo cual lleva a pérdida de la vida laboral productiva. En Colombia hay una alta prevalencia de ERC con etiología no estudiada (mal llamada desconocida) y hay pocas investigaciones de la ERC en las comunidades agrícolas. Teniendo en cuenta que la población campesina de Colombia se expone a noxas climáticas y laborales similares a Mesoamérica, es posible que esta nefropatía también sea una causa de ERC en nuestra población. El presente artículo hace una revisión de la ERC de las comunidades agrícolas, con el fin de sensibilizar el personal de salud en la importancia de la búsqueda de esta enfermedad en la población vulnerable, lo cual podría impactar de una forma positiva en la salud de los agricultores y campesinos.

Summary Chronic kidney disease (CKD) is defined as an abnormality of the kidney structure or function for ≥ 3 months. This disease affects 10% to 15% of the world's population, with diabetes, arterial hypertension, primary glomerulopathies and genetic disorders being the most common etiologies associated with this disease worldwide. Nevertheless, in low-income countries, other diseases causing CKD are also reported; among them, Mesoamerican nephropathy, which is a common cause of CKD in Mesoamerica, especially in the young male population, leading to loss of productive working capacity. In Colombia, the prevalence of CKD with unknown etiology is high and there are few studies on chronic kidney disease in agricultural communities, given that the agricultural population in Colombia is exposed to harmful climatic and occupational agents similar to those in Mesoamerica, it is possible that this nephropathy is also a cause of CKD in our population. This article provides an overview of CKD in agricultural communities to sensitize health workers to the importance of screening for this disease in vulnerable populations, which could have a positive impact on the health of farmers.

Espondiloartropatía destructiva no infecciosa en un paciente en hemodiálisis / Destructive noninfectious spondyloarthropathy in a hemodialysis patient

Resumen La espondiloartropatía destructiva es una patología osteoarticular presente en algunos pacientes con enfermedad crónica que puede afectar varios niveles de la columna vertebral y puede ser asintomática, generar dolor o causar complicaciones que ponen en peligro la integridad de la médula espinal y/o la vida. Presentamos el caso de un hombre de 70 años con enfermedad renal crónica terminal en hemodiálisis quien consultó por dolor dorsal y paraplejia, en quien se diagnosticó espondiloartropatía destructiva no infecciosa por imágenes y estudio histopatológico. Este caso nos muestra la importancia de pensar en esta patología y la necesidad de un enfoque multidisciplinario en el diagnóstico y manejo. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2193).

Abstract Destructive spondyloarthropathy is a bone and joint disease which presents in some patients with chronic illnesses and may affect various levels of the spinal column. It may be asymptomatic, cause pain, or produce spinal cord and/or life-endangering complications. We present the case of a 70-year-old man with end-stage renal disease on hemodialysis who consulted due to back pain and paraplegia. He was diagnosed with destructive noninfectious spondyloarthropathy through imaging and histopathological studies. This case shows us the importance of considering this disease and the need for a multidisciplinary approach in its diagnosis and management. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2193).

Nephrotic syndrome as a manifestation of thrombotic microangiopathy due to long-term use of sunitinib.

Nephrotic syndrome in patients with cancer may be related to the primary malignancy or chemotherapeutic therapy. Solid organ cancers may cause membranous glomerulonephritis which is manifested by nephrotic syndrome; other less common histologic presentations include focal and segmental glomerulosclerosis and minimal change disease. In addition, chemotherapy agents may cause renal toxicity by affecting the small blood vessels, glomeruli, tubules, and interstitium. Tyrosine kinase inhibitors such as sunitinib may cause endothelial and podocyte damage leading to thrombotic microangiopathy affecting only the kidney and manifested by proteinuria and hypertension. We report a case of an elderly man with gastrointestinal stromal tumor (GIST) on treatment with sunitinib who had as a complication a thrombotic microangiopathy manifested with nephrotic syndrome and a hypertension of difficult control, which was finally controlled by stopping this drug but had a fatal outcome due to its malignancy.

Modalidad previa de diálisis y su influencia en los resultados del trasplante renal / Impact of predialysis therapy on kidney transplant outcomes

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.

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.

It is time to adapt the definition of chronic kidney disease according to age

Abstract Chronic kidney disease is a condition with high morbidity, mortality and healthcare costs which affects all population groups, having a significant impact on their quality of life. Its classification has been modified over time and there is still no universal consensus to differentiate a physiological change in kidney clearance from a pathological change. Below, we will discuss the importance of reconsidering the definition and classification in the general population according to age, including children and adults. (Acta Med Colomb 2021; 46. DOI:https://doi.org/10.36104/amc.2021.2080).

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.

Nephrotic syndrome as a manifestation of thrombotic microangiopathy due to long-term use of sunitinib. / Síndrome nefrótico como manifestación de microangiopatía trombótica secundaria al uso crónico de sunitinib.

Nephrotic syndrome in patients with cancer may be related to the primary malignancy or chemotherapeutic therapy. Solid organ cancers may cause membranous glomerulonephritis manifesting with nephrotic syndrome; other less common histologic presentations include focal and segmental glomerulosclerosis and minimal change disease. In addition, chemotherapy agents can cause renal toxicity by affecting the small blood vessels, glomeruli, tubules, and interstitium. Tyrosine kinase inhibitors such as sunitinib may cause endothelial and podocyte damage leading to renal limited thrombotic microangiopathy, manifested by proteinuria and hypertension. We report a case of an elderly man with gastrointestinal stromal tumor (GIST) on treatment with sunitinib who had as a complication of a thrombotic microangiopathy manifested with nephrotic syndrome and difficult-to-control hypertension, which was controlled by stopping this drug but with a fatal outcome due to its malignant neoplasm.

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.

Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome / Síndrome nefrótica associada à síndrome hemolítico-urêmica atípica primária

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 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.

Hemolysis in Hemodialysis, Secondary to Severe Vena Cava Stenosis.

Complications in hemodialysis patients are increasingly rare thanks to advances in technology, including more compatible membranes, more flexible lines, safety in water treatments, alarms in the circuit, and standardization in dialysate fluids plus exhaustive chemical and microbiological tests. In addition, it is highly unusual having hemolysis on hemodialysis; however, it is a life-threatening complication, so the cause must be identified and early managed. The etiology can be chemical or mechanical; however, so far, there are no reports in the literature of an association with severe stenosis of the vena cava, as it is described in the case reported here, where a patient presented hemolysis in two hemodialysis sessions, without initially being possible to find the cause; the only identifiable factor was that he had a dysfunctional tunneled jugular catheter, with a history of difficult vascular access. The patient underwent interventional radiology, finding 99% stenosis of the vena cava, which prevented the passage of the contrast agent to the atrium. Angioplasty and catheter replacement were performed, with a resolution of the complication; the subsequent dialysis therapies were satisfactory.

Multifocal skeletal tuberculosis with mycobacteremia after kidney transplantation: A case report.

Solid organ transplant recipients have a higher risk of active Mycobacterium tuberculosis infection (TB) compared to the general population. Recognized risk factors are immunosuppressant use, graft dysfunction, diabetes mellitus, liver disease caused by the hepatitis C virus, and co-infections by other opportunists. Most of the active TB cases reported in solid organ transplant recipients occur in kidney transplant patients, especially if they come from M tuberculosis-endemic areas. Extrapulmonary and disseminated TB are among the wide spectrum of clinical presentations found, but the lungs are the most common organ affected. Disseminated disease occurs in up to a third of the affected population, however, multifocal osteoarticular TB with mycobacteremia is unusual. We report the case of a kidney transplant patient with disseminated M tuberculosis infection, who presented with multifocal skeletal TB.