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Because of their rarity in men, systemic lupus erythematous and lupus nephritis (LN) are poorly understood in men. Our aim was to analyze the clinical presentation and course of histology-proven systemic lupus erythematous and LN in males and to determine the risk factors for progression to end-stage renal disease. Methods: Fifty patients from 2 historical cohorts in Spain (Hospital 12 de Octubre) and Uruguay were retrospectively analyzed and compared with a female cohort matched for age and disease characteristics. Results: The median age at the time of renal biopsy was 27 years (range, 8–79 years). The main forms of presentation were nephrotic syndrome in 26 of 50 patients (52%), and class IV LN in 34 of 50 (68%). After treatment, 21 patients (45.6%) achieved complete renal remission. During follow-up, 12 patients required renal replacement therapy, and 3 patients died of infectious causes. When patients who required renal replacement therapy were compared with those who did not require it, several parameters showed significant differences (P < 0.05) at the time of renal biopsy: estimated glomerular filtration rate < 60 ml/min, hypertension, hypoalbuminemia, and concomitant visceral involvement (neurologic, cardiovascular, and/or pulmonary). In the multivariate analysis, only estimated glomerular filtration rate < 60 ml/min persisted as a risk factor for progression to end-stage renal disease. When compared with a cohort of female patients with LN, there were no significant differences in remission or renal survival. Discussion: LN in males usually presents as nephrotic syndrome, and type IV LN is the most frequent form. An estimated glomerular filtration rate < 60 ml/min at the time of renal biopsy is associated with poor renal outcomes. There were no differences in remission or progression of LN in males when compared with a cohort of female patients with LN.

La amiloidosis renal es una de las complicaciones más graves de la fiebre mediterránea familiar (FMF). La colchicina ha reducido la incidencia de esta complicación, que ahora solo aparece en pacientes no tratados, tratados de manera insuficiente o resistentes al fármaco. No obstante, la colchicina se ha mostrado poco eficaz en pacientes que inician el tratamiento cuando la amiloidosis ya está presente. En este trabajo presentamos el caso de un enfermo con FMF y amiloidosis renal secundaria diagnosticada mediante biopsia renal que desarrolló un síndrome nefrótico completo a pesar del tratamiento con colchicina. Por la mala evolución del cuadro se decidió iniciar tratamiento con anakinra (un inhibidor de la interleucina 1β). En los meses posteriores a la instauración del fármaco el enfermo presentó una mejoría progresiva del síndrome nefrótico, hasta alcanzar la remisión completa. La función renal permaneció estable. Los inhibidores de la interleucina 1β pueden ser un tratamiento efectivo de la FMF en pacientes con amiloidosis renal secundaria.

Renal amyloidosis is one of the most severe complications of familial Mediterranean fever (FMF). Colchicine has reduced the incidence of this complication, which now only appears in untreated, under-treated and resistant patients, but it is usually ineffective in patients with advanced amyloidosis. Here we report a patient with FMF and biopsy-proven amyloidosis who presented with nephrotic syndrome despite colchicine treatment. Anakinra (an interleukin-1β inhibitor) was started and a dramatic complete remission of nephrotic syndrome was observed in the following months. Anakinra can be an effective treatment for FMF patients with severe secondary amyloidosis.

Background: The shortage of organs is a major hurdle in kidney transplantation, and one solution to the problem is to extend the age of the donor. However, organs from older donors are often discarded due to the macroscopic appearance of the parenchyma or major vessels. On the other hand, a large number of elderly patients are potential candidates for kidney transplantation, while many kidneys from elderly deceased donors are discarded due to a lack of age-matched recipients. In addition, a large number are often discarded due to the lack of compatible recipients among elderly patients undergoing chronic dialysis. A possible solution to avoid this wastage of kidneys potentially suitable for transplantation could be the performance of preemptive kidney transplantation (PKT) in carefully selected elderly patients. PKT improves graft and patient survival compared to other renal replacement therapy options. There is no information about PKT in elderly patients receiving kidneys from elderly deceased donors. Methods: From 2007 to 2012, we performed a prospective observational study comparing 26 elderly patients receiving PKT with a control group of 26 elderly patients receiving a first transplant after prior dialysis. Results: Mean age of recipients was 74.3 ± 2.9 years and mean age of donors was 73.8 ± 4.1 years. Induction immunosuppression was similar in both groups. Death-censored graft survival was 96% in the PKT group and 68% in the control group (p = 0.02), at 5 years after transplantation. Immediate and delayed graft function occurred in 92% and 3.8%, respectively, of patients in the PKT group and 53% and 34.6% of patients in the control group (p = 0.005). Acute rejection was significantly more frequent in PKT patients (23.1% vs 3.8%, p = 0.043). At the end of follow-up time 35.5 ± 20.1 months, the glomerular filtration rate was similar in both groups (42.2 ± 11.7 vs 41.7 ± 11.2 ml/min, p-value = 0.72). Patient survival was similar in the two groups. Conclusions: Elderly patients with end stage of renal disease non-dialysis may benefit from PKT elderly deceased donors whose kidneys were to be discarded for there are not patients in the waiting list.

This Guide for Living Donor Kidney Transplantation (LDKT) has been prepared with the sponsorship of the Spanish Society of Nephrology (SEN), the Spanish Transplant Society (SET), and the Spanish National Transplant Organization (ONT). It updates evidence to offer the best chronic renal failure treatment when a potential living donor is available. The core aim of this Guide is to supply clinicians who evaluate living donors and transplant recipients with the best decision-making tools, to optimise their outcomes.Moreover, the role of living donors in the current KT context should recover the level of importance it had until recently. To this end the new forms of incompatible HLA and/or ABO donation, as well as the paired donation which is possible in several hospitals with experience in LDKT, offer additional ways to treat renal patients with an incompatible donor.Good results in terms of patient and graft survival have expanded the range of circumstances under which living renal donors are accepted. Older donors are now accepted, as are others with factors that affect the decision, such as a borderline clinical history or alterations, which when evaluated may lead to an additional number of transplantations.This Guide does not forget that LDKT may lead to risk for the donor. Pre-donation evaluation has to centre on the problems which may arise over the short or long-term, and these have to be described to the potential donor so that they are able take them into account. Experience over recent years has led to progress in risk analysis, to protect donors’ health. This aspect always has to be taken into account by LDKT programmes when evaluating potential donors.Finally, this Guide has been designed to aid decision-making, with recommendations and suggestions when uncertainties arise in pre-donation studies. Its overarching aim is to ensure that informed consent is based on high quality studies and information supplied to donors and recipients, offering the strongest possible guarantees. Resumen: Esta guía de recomendaciones para el TR de donante vivo (TRDV) es un documento elaborado con el patrocinio de la Sociedad Española de Nefrología, la Sociedad Española de Trasplantes y la Organización Nacional de Trasplantes que actualiza la calidad de la evidencia disponible para ofrecer el mejor tratamiento de la insuficiencia renal crónica cuando se disponga de un donante vivo potencial. El objetivo principal de esta guía es proporcionar a los profesionales con responsabilidad en los estudios previos del donante vivo y del receptor trasplantado, las mejores herramientas para tomar decisiones en beneficio del donante vivo y del receptor del trasplante.Además, en el contexto actual del TR, el donante vivo debe recuperar el protagonismo que alcanzó en un pasado reciente. Para ello, las nuevas modalidades de donación HLA y/o ABO incompatible, así como la donación cruzada disponibles en diversos centros con experiencia en TRDV, son oportunidades adicionales para el tratamiento de enfermos renales que tienen un donante incompatible.Los buenos resultados en supervivencia del paciente y del injerto están ampliando las circunstancias de aceptación de donantes vivos de riñón, incluyendo donantes de mayor edad y otros con algunos condicionantes que incluyen antecedentes o alteraciones límite que, cuando son evaluados con criterios objetivos, pueden aportar un numero adicional de trasplantes.No se ha obviado en esta guía que el TRDV puede representar algún riesgo para el que dona. Estos problemas que pueden aparecer a corto o largo plazo tienen que ser objeto principal de valoración previa a la donación y presentados al potencial donante para que en ejercicio de su autonomía los asuma o rechace. La experiencia acumulada en los últimos años ha permitido avanzar en el análisis de riesgos para preservar la salud de los donantes, aspecto que debe estar siempre presente en los responsables de programas de TRDV cuando se procede al estudio de idoneidad de un potencial donante.Finalmente, esta guía ha sido estructurada para facilitar la toma de decisiones con recomendaciones y sugerencias ante incertidumbres derivadas de los resultados en los exhaustivos estudios predonación. Y todo ello, con el objetivo de que el consentimiento informado que debe certificar la calidad de los estudios y la información proporcionada a donante y receptor, alcancen las mayores garantías posibles.

Background and aims: Whether cytomegalovirus (CMV) infection increases the risk of cardiovascular complications after kidney transplantation (KT) through different indirect effects remains controversial. Methods: We analyzed the incidence of post-transplant atherosclerotic (PAEs) and thrombotic events (PTEs) in 465 KT recipients according to the previous exposure to any level or high-level (≥1,000 IU/mL) CMV viremia (either asymptomatic or clinical disease) by means of landmark analysis beyond days 30, 180 and 360 after transplantation. Proportional hazards models were constructed with death and graft loss as competing risks. Results: After a median of 722 days, the cumulative incidences of PAE and PTE were 6.0% each. Most PAEs (53.6%) occurred beyond post-transplant day 360, whereas most PTEs (60.7%) were diagnosed between days 30–180.The incidence of PAE beyond day 180 was higher among patients with previous CMV viremia compared to those without (two-year rates: 4.7% versus 0.4%; P-value = 0.035). This difference was more pronounced in recipients developing high-level viremia (6.3% versus 0.7%, respectively; P-value = 0.013). After multivariate adjustment for age, pre-transplant cardiovascular risk, antiplatelet and statin therapy and graft function, however, associations were not maintained either for any-level (hazard ratio [HR]: 1.84; 95% confidence interval [CI]: 0.48–7.05) or high-level CMV viremia (HR: 2.84; 95% CI: 0.78–10.36). No significant differences were found in the remaining landmark analyses (days 30 or 360) or for the outcome of PTE either. Conclusions: Our study does not support that CMV infection independently contributes to the risk of PAE or PTE after KT.

Resumen: Esta guía de recomendaciones para el TR de donante vivo (TRDV) es un documento elaborado con el patrocinio de la Sociedad Española de Nefrología, la Sociedad Española de Trasplantes y la Organización Nacional de Trasplantes que actualiza la calidad de la evidencia disponible para ofrecer el mejor tratamiento de la insuficiencia renal crónica cuando se disponga de un donante vivo potencial. El objetivo principal de esta guía es proporcionar a los profesionales con responsabilidad en los estudios previos del donante vivo y del receptor trasplantado, las mejores herramientas para tomar decisiones en beneficio del donante vivo y del receptor del trasplante.Además, en el contexto actual del TR, el donante vivo debe recuperar el protagonismo que alcanzó en un pasado reciente. Para ello, las nuevas modalidades de donación HLA y/o ABO incompatible, así como la donación cruzada disponibles en diversos centros con experiencia en TRDV, son oportunidades adicionales para el tratamiento de enfermos renales que tienen un donante incompatible.Los buenos resultados en supervivencia del paciente y del injerto están ampliando las circunstancias de aceptación de donantes vivos de riñón, incluyendo donantes de mayor edad y otros con algunos condicionantes que incluyen antecedentes o alteraciones límite que, cuando son evaluados con criterios objetivos, pueden aportar un numero adicional de trasplantes.No se ha obviado en esta guía que el TRDV puede representar algún riesgo para el que dona. Estos problemas que pueden aparecer a corto o largo plazo tienen que ser objeto principal de valoración previa a la donación y presentados al potencial donante para que en ejercicio de su autonomía los asuma o rechace. La experiencia acumulada en los últimos años ha permitido avanzar en el análisis de riesgos para preservar la salud de los donantes, aspecto que debe estar siempre presente en los responsables de programas de TRDV cuando se procede al estudio de idoneidad de un potencial donante.Finalmente, esta guía ha sido estructurada para facilitar la toma de decisiones con recomendaciones y sugerencias ante incertidumbres derivadas de los resultados en los exhaustivos estudios predonación. Y todo ello, con el objetivo de que el consentimiento informado que debe certificar la calidad de los estudios y la información proporcionada a donante y receptor, alcancen las mayores garantías posibles. Abstract: This guide for living donor renal transplantation (LDRT) has been prepared with the sponsorship of the Spanish Society of Nephrology (SEN), the Spanish Transplant Society (SET), and the Spanish National Transplant Organization (ONT). It updates evidence to offer the best chronic renal failure treatment when a potential living donor is available. The core aim of this guide is to supply clinicians who evaluate living donors and transplant recipients with the best decision-making tools, to optimise their outcomes.Moreover, the role of living donors in the current RT context should recover the level of importance it had until recently. To this end the new forms of incompatible HLA and/or ABO donation, as well as the paired donation which is possible in several hospitals with experience in LDRT, offer additional ways to treat renal patients with an incompatible donor.Good results in terms of patient and graft survival have expanded the range of circumstances under which living renal donors are accepted. Older donors are now accepted, as are others with factors that affect the decision, such as a borderline clinical history or alterations, which when evaluated may lead to an additional number of transplantations.This guide does not forget that LDRT may lead to risk for the donor. Pre-donation evaluation has to centre on the problems which may arise over the short or long-term, and these have to be described to the potential donor so that they are able to take them into account. Experience over recent years has led to progress in risk analysis, to protect donors’ health. This aspect always has to be taken into account by LDRT programmes when evaluating potential donors.Finally, this guide has been designed to aid decision-making, with recommendations and suggestions when uncertainties arise in pre-donation studies. Its overarching aim is to ensure that informed consent is based on high quality studies and information supplied to donors and recipients, offering the strongest possible guarantees.

Living donor kidney transplantation (LDKT) is the best treatment option for end stage renal disease in terms of both patient and graft survival. However, figures on LDKT in Spain that had been continuously growing from 2005 to 2014, have experienced a continuous decrease in the last five years.One possible explanation for this decrease is that the significant increase in the number of deceased donors in Spain during the last years, both brain death and controlled circulatory death donors, might have generated the false idea that we have coped with the transplant needs. Moreover, a greater number of deceased donor kidney transplants have caused a heavy workload for the transplant teams.Furthermore, the transplant teams could have moved on to a more conservative approach to the information and assessment of patients and families considering the potential long-term risks for donors in recent papers. However, there is a significant variability in the LDKT rate among transplant centers and regions in Spain independent of their deceased donor rates. This fact and the fact that LDKT is usually a preemptive option for patients with advanced chronic renal failure, as time on dialysis is a negative independent factor for transplant outcomes, lead us to conclude that the decrease in LDKT depends on other factors.Thus, in the kidney transplant annual meeting held at ONT site in 2018, a working group was created to identify other causes for the decrease of LDKT in Spain and its relationship with the different steps of the process. The group was formed by transplant teams, a representative of the transplant group of the Spanish Society of Nephrology (SENTRA), a representative of the Spanish Society of Transplants (SET) and representatives of the Spanish National Transplant Organization (ONT).A self-evaluation survey that contains requests about the phases of the LDKT processes (information, donor work out, informed consent, surgeries, follow-up and human resources) were developed and sent to 33 LDKT teams. All the centers answered the questionnaire. The analysis of the answers has resulted in the creation of a national analysis of strengths, weaknesses, opportunities, threats (SWOT) of the LDKT program in Spain and the development of recommendations targeted to improve every step of the donation process. The work performed, the conclusions and recommendations provided, have been reflected in the following report: Spanish living donor kidney transplant program assessment: recommendations for optimization. This document has also been reviewed by a panel of experts, representatives of the scientific societies (Spanish Society of Urology (AEU), Spanish Society of Nephrology Nursery (SEDEN), Spanish Society of Immunology (SEI/GETH)) and the patient association ALCER. Finally, the report has been submitted to public consultation, reaching ample consensus. In addition, the transplant competent authorities of the different regions in Spainhave adopted the report at institutional level.The work done and the recommendations to optimize LDKT are summarized in the present manuscript, organized by the different phases of the donation process. Resumen: El trasplante renal de donante vivo (TRDV) es la opción terapéutica con las mejores expectativas de supervivencia para el injerto y para el paciente con insuficiencia renal terminal; sin embargo, este tipo de trasplantes ha experimentado un descenso progresivo en los últimos años en España.Entre las posibles explicaciones del descenso de actividad se encuentra la coincidencia en el tiempo con un aumento en el número de donantes renales fallecidos, tanto por muerte encefálica como por asistolia controlada, que podría haber generado una falsa impresión de ausencia de necesidad del TRDV. Además, la disponibilidad de un mayor número de riñones para trasplante habría supuesto un incremento en la carga de trabajo de los profesionales que pudiera enlentecer los procesos de donación en vida. Otro posible argumento radica en un posible cambio de actitud hacia posturas más conservadoras a la hora de informar a pacientes y a familiares acerca de esta opción terapéutica, a raíz de los artículos publicados respecto al riesgo de la donación a largo plazo. Sin embargo, existe una importantísima variabilidad en la actividad entre centros y comunidades autónomas, no explicada por el volumen de trasplante procedente de otros tipos de donante. Este dato, unido a que la indicación de donación renal en vida se realiza de manera mayoritaria en situación de enfermedad renal crónica avanzada (ERCA) y que el tiempo en diálisis es un factor pronóstico negativo respecto a la supervivencia postrasplante, permite concluir que el descenso depende además de otros factores.Por este motivo, en la reunión anual de equipos de trasplante renal, celebrada en la sede de la Organización Nacional de Trasplantes (ONT) en 2018, se constituyó un grupo de trabajo formado por equipos de trasplante renal, el grupo de trasplantes de la Sociedad Española de Nefrología (SEN) (SENTRA), la Sociedad Española de Trasplantes (SET) y la ONT, con el objetivo de identificar otras causas que condicionaron el descenso de la actividad de este tipo de trasplantes en España y su posible relación con la gestión del proceso de donación de vivo.El grupo de trabajo diseñó un cuestionario de autoevaluación, que fue cumplimentado por las 33 unidades de trasplante renal de donante vivo activas en España. El cuestionario contiene preguntas sobre las diferentes fases del proceso de donación de vivo: información inicial, estudio del donante vivo e información de los riesgos, consentimiento, recursos humanos (RRHH), nefrectomía, trasplante y seguimiento posterior.El análisis de las respuestas ha dado como resultado la creación de un análisis de debilidades, amenazas, fortalezas y oportunidades (DAFO) del programa a nivel nacional y ha permitido elaborar recomendaciones específicas dirigidas a mejorar cada una de las fases del proceso de donación en vida. El documento, denominado Análisis de situación del trasplante renal de donante vivo y hoja de ruta ha sido también revisado por un panel de expertos en TRDV, representantes de varias sociedades científicas implicadas (Asociación Espa˜nola de Urología [AEU], Sociedad Espa˜nola de Enfermería Nefrológica [SEDEN], Sociedad Espa˜nola de Inmunología [SEI/GETH]), el Grupo de Trabajo Enfermedad Renal Crónica Avanzada (ACERCA), la Asociación de Pacientes para la Lucha Contra la Enfermedad Renal (ALCER) y sometido posteriormente a consulta pública. Tras incluir las mejoras sugeridas, el documento final ha sido adoptado institucionalmente en el Consejo Interterritorial de Trasplantes (CIT) del Sistema Nacional de Salud.El trabajo realizado y las recomendaciones para optimizar el TRVD se describen a lo largo del presente artículo, organizados por los diferentes apartados del proceso de donación.

Resumen: Objetivo: Identificar, en la literatura científica internacional, los obstáculos y factores potencialmente favorecedores para el avance durante su carrera profesional de las mujeres académicas e investigadoras en ciencias biomédicas. Método: Se realizó una búsqueda sistemática en PubMed, Scopus, CinahlPlus, Cochrane Database of Systematic Reviews, PsycInfo y Sociological Abstracts de artículos publicados en inglés y español entre enero de 2006 y diciembre de 2016 sobre el fenómeno del techo de cristal en mujeres académicas e investigadoras en ciencias biomédicas. El cribado se llevó a cabo por revisoras independientes. Resultados: Se encontraron 2254 estudios, de los cuales se incluyeron 23 en la revisión. Los obstáculos identificados para la promoción de las mujeres académicas o investigadoras en ciencias biomédicas son los sesgos de género en la evaluación de la investigación, el individualismo y la falta de colaboración, la falta de influencia de las mujeres, las desigualdades de género en la contratación y la promoción, la percepción de sexismo y discriminación en el clima laboral, y las dificultades de conciliación. Los elementos que favorecen son los ejemplos de mujeres en puestos de liderazgo, la mentorización, facilitar la conciliación, la transparencia en la contratación, la participación en la toma de decisiones, realizar auditorías de género en la evaluación de la investigación, la conciencia de las desigualdades de género, promover la colaboración y la equidad salarial. Conclusiones: Potenciar los elementos que favorecen la promoción de las mujeres académicas en ciencias biomédicas contribuiría a reducir el fenómeno del techo de cristal en esta área, al aumentar su participación, representación y liderazgo. Se requiere un cambio de valores organizacional e institucional. Abstract: Objective: To identify in the international scientific literature the obstacles and potential promoters for the advancement of women academics and researchers in biomedical sciences during their professional careers. Method: PubMed, Scopus, CinahlPlus, Cochrane Database of Systematic Reviews, PsycInfo and Sociological Abstracts were systematically searched for articles published in English and Spanish between January 2006 and December 2016 on the phenomenon of the glass ceiling in women academics and researchers in biomedical sciences. The screening was carried out by independent reviewers. Results: A total of 2254 studies were found, of which 23 were included in the review. The obstacles identified for the promotion of women academics and/or researchers in biomedical sciences are: gender bias in the evaluation of research results, individualism and lack of collaboration, women's lack of influence, the existence of gender inequalities in access to employment. The perception of sexism and discrimination in the work environment, and the difficulties in reconciling work and family life. The promoting elements are: examples of women in leadership positions, mentoring, facilitating conciliation, transparency in recruitment, participation in decision-making, gender assessment of research, awareness of gender inequalities in institutions, promoting collaboration, and pay equity. Conclusions: By enhancing the elements favouring the promotion of academic women in biomedical sciences would help to reduce the glass ceiling in the career paths of women academics and health science researchers by increasing their participation, leadership and representation. A change of organizational and institutional values is required to achieve this.

Introduction: Diabetic nephropathy (DN) is one of the most frequent complications in patients with diabetes mellitus (DM) and its diagnosis is usually established on clinical grounds. However, kidney involvement in some diabetic patients can be due to other causes, and renal biopsy might be needed to exclude them. The aim of our study was to establish the clinical and analytical data that predict DN and no-diabetic renal disease (NDRD), and to develop a predictive model (score) to confirm or dismiss DN. Material and methods: We conducted a transversal, observational and retrospective study, including renal biopsies performed in type 2 DM patients, between 2000 and 2018. Results: Two hundred seven DM patients were included in our study. The mean age was 64.5 ± 10.6 years and 74% were male. DN was found in 126 (61%) of the biopsies and NDRD in 81 (39%). Diabetic retinopathy was presented in 58% of DN patients, but only in 6% of NDRD patients (p 3 had DN and 94% of cases with a score ≤1 had NDRD (score ranked from −6 to 8 points). Conclusions: NDRD is common in DM patients (39%), being primary glomerulonephritis the most frequent ethology. The absence of retinopathy and the presence of microhematuria are highly suggestive of NDRD. The use of our predictive model could facilitate the indication of performing a renal biopsy in DM patients. Resumen: Introducción: La nefropatía diabética (ND) es una complicación frecuente de la diabetes mellitus (DM), y su diagnóstico suele ser clínico. Sin embargo, en numerosas ocasiones la enfermedad renal que presentan los pacientes diabéticos es debida a otras causas cuyo diagnóstico es histológico. El objetivo del estudio fue determinar los datos clínicos y analíticos predictores de ND y enfermedad renal no diabética (ERND), y elaborar un modelo predictivo (score) para confirmar o descartar ND. Material y métodos: Estudio observacional, transversal y retrospectivo de biopsias renales realizadas en pacientes diabéticos tipo 2 entre 2000 y 2018. Resultados: Se incluyeron 207 pacientes diabéticos con una edad media de 64.5 ± 10,6 años; el 74% eran varones. La biopsia mostró ND en 126 (61%) y en 81 ERND (39%). La retinopatía diabética estaba presente en el 58% de los pacientes con ND y en el 6% del grupo con ERND (p 3 era tenían ND, y el 94% de los casos con score ≤ 1 punto fueron ERND. Conclusiones: La ERND es frecuente en pacientes con DM (39%). La etiología más frecuente son las glomerulonefritis primarias. La ausencia de retinopatía y la presencia de microhematuria son altamente sugestivas de ERND. La utilización de un sistema de puntuación facilita la indicación de biopsia renal en pacientes diabéticos.

Resumen: Introducción: La nefropatía diabética (ND) es una complicación frecuente de la diabetes mellitus (DM), y su diagnóstico suele ser clínico. Sin embargo, en numerosas ocasiones la enfermedad renal que presentan los pacientes diabéticos es debida a otras causas cuyo diagnóstico es histológico. El objetivo del estudio fue determinar los datos clínicos y analíticos predictores de ND y enfermedad renal no diabética (ERND), y elaborar un modelo predictivo (score) para confirmar o descartar ND. Material y métodos: Estudio observacional, transversal y retrospectivo de biopsias renales realizadas en pacientes diabéticos tipo 2 entre 2000 y 2018. Resultados: Se incluyeron 207 pacientes diabéticos con una edad media de 64,5 ± 10,6 años; el 74% eran varones. La biopsia mostró ND en 126 (61%) y en 81 ERND (39%). La retinopatía diabética estaba presente en el 58% de los pacientes con ND y en el 6% del grupo con ERND (p 3 era tenían ND, y el 94% de los casos con score ≤ 1 punto fueron ERND. Conclusiones: La ERND es frecuente en pacientes con DM (39%). La etiología más frecuente son las glomerulonefritis primarias. La ausencia de retinopatía y la presencia de microhematuria son altamente sugestivas de ERND. La utilización de un sistema de puntuación facilita la indicación de biopsia renal en pacientes diabéticos. Abstract: Introduction: Diabetic nephropathy (DN) is one of the most frequent complications in patients with diabetes mellitus (DM) and its diagnosis is usually established on clinical grounds. However, kidney involvement in some diabetic patients can be due to other causes, and renal biopsy might be needed to exclude them. The aim of our study was to establish the clinical and analytical data that predict DN and no-diabetic renal disease (NDRD), and to develop a predictive model (score) to confirm or dismiss DN. Material and methods: We conducted a transversal, observational and retrospective study, including renal biopsies performed in type 2 DM patients, between 2000 and 2018. Results: Two hundred seven DM patients were included in our study. The mean age was 64.5 ± 10.6 years and 74% were male. DN was found in 126 (61%) of the biopsies and NDRD in 81 (39%). Diabetic retinopathy was presented in 58% of DN patients, but only in 6% of NDRD patients (P 3 had DN and 94% of cases with a score ≤ 1 had NDRD (score ranked from −6 to 8 points). Conclusions: NDRD is common in DM patients (39%), being primary glomerulonephritis the most frequent ethology. The absence of retinopathy and the presence of microhematuria are highly suggestive of NDRD. The use of our predictive model could facilitate the indication of performing a renal biopsy in DM patients. Palabras clave: Diabetes mellitus, Biopsia renal, Nefropatía diabética, Enfermedad renal no diabética, Modelo predictivo, Keywords: Diabetes mellitus, Renal biopsy, Diabetic nephropathy, Non-diabetic renal disease, Predictive model

Immunoglobulin light-chain amyloidosis (AL) and multiple myeloma (MM) are 2 distinct monoclonal gammopathies that involve the same cellular compartment: clonal plasma cells (PCs). Despite the fact that knowledge about MM PC biology has significantly increased in the last decade, the same does not apply for AL. Here, we used an integrative phenotypic, molecular, and genomic approach to study clonal PCs from 24 newly diagnosed patients with AL. Through principal-component-analysis, we demonstrated highly overlapping phenotypic profiles between AL and both monoclonal gammopathy of undetermined significance and MM PCs. However, in contrast to MM, highly purified fluorescence-activated cell-sorted clonal PCs from AL (n = 9) showed almost normal transcriptome, with only 38 deregulated genes vs normal PCs; these included a few tumor-suppressor (CDH1, RCAN) and proapoptotic (GLIPR1, FAS) genes. Notwithstanding, clonal PCs in AL (n = 11) were genomically unstable, with a median of 9 copy number alterations (CNAs) per case, many of such CNAs being similar to those found in MM. Whole-exome sequencing (WES) performed in 5 AL patients revealed a median of 15 nonrecurrent mutations per case. Altogether, our results show that in the absence of a unifying mutation by WES, clonal PCs in AL display phenotypic and CNA profiles similar to MM, but their transcriptome is remarkably similar to that of normal PCs., (© 2016 by The American Society of Hematology.)