Long-term plasmapheresis therapy in the management of focal segmental glomerulosclerosis recurrence after kidney transplantation.

The recurrence of primary focal segmental glomerulosclerosis (FSGS) after kidney transplantation (KT) appears in 30 % of the recipients. Sometimes it can cause the loss of the allograft. Although many treatments for this condition have been reported, 20 %-40 % of the affected patients are refractory or presents frequents relapses. In this paper we describe the evolution of three recipients treated with long-term plasmapheresis therapy after a recurrence of FSGS with a bad or incomplete response to other treatments. Although our findings require confirmation, long-term plasmapheresis could be a therapeutic option for this condition.

High levels of circulating TNFR1 increase the risk of all-cause mortality and progression of renal disease in type 2 diabetic nephropathy.

BACKGROUND: Several studies have demonstrated that levels of circulating inflammatory markers such as tumour necrosis factorα (TNFα), are associated with early progression of diabetic nephropathy (DN). The aim of this study was to investigate whether there is an association between circulating TNFα receptor and disease progression in patients with advanced type 2 DN and severe proteinuria. METHODS: Between 2006 and 2011, we measured levels of circulating soluble TNFα receptor 1 (TNFR1) and soluble TNFα receptor 2 (TNFR2) at baseline and 4 and 12 months in 101 patients included in a multicenter randomized controlled trial to compare the effect of optimal doses of renin-angiotensin system blockers in monotherapy or in combination (dual blockade) to slow progression of established type 2 DN. The primary composite endpoint was a >50% increase in baseline serum creatinine, end-stage renal disease, or death. RESULTS: The median follow-up was 32 months (IQR, 18-48), during which time 28 patients (22.7%) achieved the primary endpoint. The TNFR1 level, but not the TNFR2 level, was correlated with other inflammatory markers. Cox regression analysis showed that the highest TNFR1 levels (HR, 2.60; 95%CI, 1.11-86.34) and baseline proteinuria (HR 1.32; 95%CI 1.15-1.52) were associated with the primary endpoint. The mixed model analysis revealed that TNFR1 and the TNFR2 levels did not change after starting treatment with renin-angiotensin system blockers. CONCLUSIONS: Our results show that the highest levels of TNFR1 are independently associated with progression of renal disease and death in type 2 DN. The renin angiotensin blockers have no effect on these inflammatory markers.

When to perform renal biopsy in patients with type2 diabetes mellitus? Predictive model of non-diabetic renal disease. / ¿Cuándo realizar biopsia renal en pacientes con diabetes mellitus tipo2? Modelo predictivo de enfermedad renal no diabética.

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 type2 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<.001). Patients with NDRD were diagnosed of primary glomerulopathies (52%), nephroangiosclerosis (16%), inmunoallergic interstitial nephritis (15%) and vasculitis (8.5%). In the multivariate analysis, retinopathy (OR26.7; 95%CI: 6.8-104.5), chronic ischaemia of lower limbs (OR4,37; 95%CI: 1.33-14.3), insulin therapy (OR3.05; 95%CI: 1.13-8.25), time course of DM ≥10years (OR2.71; 95%CI: 1.1-6.62) and nephrotic range proteinuria (OR2.91; 95%CI: 1.2-7.1) were independent predictors for DN. Microhaematuria defined as ≥10 red blood cells per high-power field (OR0.032; 95%CI: 0.01-0.11) and overweight (OR0.21; 95%CI: 0.08-0.5) were independent predictors of NDRD. According to the predictive model based on the multivariate analysis, all patients with a score >3 had DN and 94% of cases with a score ≤1 had NDRD (score ranked from -6 to 8points). 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.

The role of complement in IgA nephropathy.

IgA nephropathy (IgAN) is common and often progresses to end stage renal disease. IgAN encompasses a wide range of histology and clinical features. IgAN pathogenesis is incompletely understood; the current multi-hit hypothesis of IgAN pathogenesis does not explain the range of glomerular inflammation and renal injury associated with mesangial IgA deposition. Although associations between IgAN and glomerular and circulating markers of complement activation are established, the mechanism of complement activation and contribution to glomerular inflammation and injury are not defined. Recent identification of specific complement pathways and proteins in severe IgAN cases had advanced our understanding of complement in IgAN pathogenesis. In particular, a growing body of evidence implicates the complement factor H related proteins 1 and 5 and lectin pathway as pathogenic in a subset of patients with severe disease. These data suggest complement deregulation and activity may be dominant drivers of renal injury in IgAN. Thereby, markers of complement activation may identify IgAN patients likely to progress to significant renal impairment and complement inhibition may emerge as an effective method of preventing and reducing glomerular injury in IgAN.

Does renal function improve after parathyroidectomy in primary hyperparathyroidism? / ¿Mejora la función renal tras la paratiroidectomía en el hiperparatirodismo primario?

INTRODUCTION: Primary hyperparathyroidism (PHPT) is a common endocrine disorder characterised by hypercalcaemia and parathormone increase. Decreased glomerular filtration rate (<60ml/min) continues to be a parathyroidectomy (PTX) criterion in asymptomatic PHPT. The influence of PTX on renal function evolution is the subject of debate. OBJECTIVE: To analyse the clinical, laboratory and histological characteristics of patients undergoing PHPT, as well as renal function evolution after PTX. MATERIAL AND METHODS: Retrospective study of 297 patients diagnosed with PHPT and referred to surgery in a single centre between 1998 and 2016. Laboratory parameters were determined at baseline, one week and one year after PTX. RESULTS: The Incidence of PTX was 38 cases/million/year. Mean age was 60±14 years and 80.5% of the patients were female. Approximately 65.3% were asymptomatic. Nephrolithiasis was the most common clinical finding (33%), followed by bone involvement (29.5%). PTX indications were: clinical symptoms (34.7%), hypercalcaemia>11.2mg/dl (27%), nephrolithiasis (13%), low bone mass (12%), age<50 years (11%) and decreased glomerular filtration rate<60ml/min (2.3%). For diagnostic localisation, spect-MIBI had a sensitivity of 92% and cervical ultrasound of 70%. A total of 94.3% of PHPT cases were due to a parathyroid adenoma. After PTX, normalisation of PHPT-related parameters was observed. We found a significant increase in serum creatinine levels (0.81 vs 0.85mg/dl, P<.001) from the first week post-PTX until the end of the first year. The renal function was only found to be significant in patients with glomerular filtration rate>60ml/min (baseline serum creatinine levels 0.77mg/dl vs serum creatinine levels after one year 0.81mg/dl, P<.001). CONCLUSIONS: PHPT was asymptomatic in most patients who underwent surgery. Hypercalcaemia and nephrolithiasis were the most common indications of parathyroidectomy in asymptomatic patients. MIBI scan was the most useful localisation method. Surgical treatment of PHPT is followed by renal function impairment, which persists after the first week post-PTX.

C3 glomerulopathies. A new perspective on glomerular diseases.

Membranoproliferative glomerulonephritis denotes a general pattern of glomerular injury that is easily recognised by light microscopy. With additional studies, MPGN subgrouping is possible. For example, electron microscopy resolves differences in electron-dense deposition location, while immunofluorescence typically detects the composition of electron-dense deposits. A C3 glomerulopathy (C3G) is a recently described entity, a proliferative glomerulonephritis (usually but not always), with a MPGN pattern on light microscopy, with C3 staining alone on immunofluorescence, implicating hyperactivity of the alternative complement pathway. The evaluation of C3G in a patient should focus on the complement cascade, as deregulation of the alternative pathway and terminal complement cascade underlies pathogenesis. Although there are no specific treatments currently available for C3G, a better understanding of their pathogenesis would set the stage for the possible use of anti-complement drugs, as eculizumab. In this review, we summarise the pathogenesis of the C3 glomerulopathies, focusing on the role of complement, the patient cohorts recently reported and options of treatment up to the current moment.

Cholesterol atheroembolism and combined treatment with steroids and iloprost.

Cholesterol atheroembolism (CAE) is a systemic disorder whose incidence has increased in recent decades and that presents high morbidity and mortality. Although several therapeutic alternatives have been reported, there is no consensus about the best treatment for this disease. In this paper we report the case of a patient with CAE with skin, bowel and kidney involvement who presented a good response to combined therapy with steroids and prostaglandin analogues. Although there are no conclusive studies, we recommend this therapeutic alternative in the management of CAE with organic failure.

Epidemiological study of 7316 patients on haemodialysis treated in FME clinics in Spain, using data from the EuCliD® database: results from years 2009-2010.

Observational study of patients on hemodialysis (HD) in FMC® Spain clinics over the years 2009 and 2010. The data were collected from the EuClid® database, implemented in the clinics of FMC®, which complies with the following feature: record online, compulsory, conducted in patients incidents and that it covers the entire population on HD in these clinics. Its aim is to understand the characteristics of patients and treatment patterns, comparing them with other studies described in the literature and in order to improve their prognosis and quality of life. Include 2637 incidents patients and 4679 prevalent, which makes a total of 7316 patients. In prevalent patients: 24.4% were diabetic; 76.3% had cardio-vascular disease (CVD) and 13.4% cancer. Among the incidents, these percentages were: 33.5% diabetic; 80.6% had CVD and 12.6% cancer. The prevalent patients had such as vascular access: FAV 68.5%, prosthesis 5.6%, permanent catheter 23.7% and 2.3% temporary catheter. The average of the duration of the sessions of HD was 230 minutes. 23.2% of the prevalent patients were on on-line hemodiafiltration. These patients hospitalization rates were 0.46 hospitalizations per incident patient per year and 0.52 per prevalent patient per year. The annual gross mortality rate was 12%. The mortality of the patients in this study HD is smaller than these of the Spanish Registry of Dialysis and Transplant (GRER). The result of morbidity and mortality of the FMC clinics of Spain can, therefore, be as good compared with these of the GRER and other international series. That does not mean that there are not areas of improvement as the increase in the time of dialysis, the percentage of patients on on-line hemodiafiltration convective techniques and the percentage of FAV.

¿Cuándo realizar biopsia renal en pacientes con diabetes mellitus tipo2? Modelo predictivo de enfermedad renal no diabética / When to perform renal biopsy in patients with type 2 diabetes mellitus? Predictive model of non-diabetic renal disease

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 < 0,001). Histología encontrada en la ERND: glomerulopatías primarias (52%), nefroangioesclerosis (16%), nefritis intersticial inmunoalérgica (15%) y vasculitis (8,5%). En el análisis multivariable, la retinopatía (OR 26,7; IC 95%: 6,8-104,5), la isquemia crónica de miembros inferiores (OR 4,37; IC 95%: 1,33-14,3), la insulinoterapia (OR 3,05; IC 95%: 1,13-8,25), una evolución de la DM ≥ 10 años (OR 2,71; IC 95%: 1,1-6,62) y la proteinuria nefrótica (OR 2,91; IC 95%: 1,2-7,1) fueron predictores independientes de ND. La microhematuria, definida como ≥ 10 hematíes/campo (OR 0,032; IC 95%: 0,01-0,11) y el sobrepeso (OR 0,21; IC 95%: 0,08-0,55) lo fueron de ERND. Según el modelo predictivo resultante del estudio multivariable para ND, el rango de puntuación varió de -6 a 8 puntos. Todos los pacientes con un score > 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

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 < .001). Patients with NDRD were diagnosed of primary glomerulopathies (52%), nephroangiosclerosis (16%), inmunoallergic interstitial nephritis (15%) and vasculitis (8.5%). In the multivariate analysis, retinopathy (OR 26.7; 95% CI: 6.8-104.5), chronic ischaemia of lower limbs (OR 4,37; 95% CI: 1.33-14.3), insulin therapy (OR 3.05; 95% CI: 1.13-8.25), time course of DM ≥ 10 years (OR 2.71; 95% CI: 1.1-6.62) and nephrotic range proteinuria (OR 2.91; 95% CI: 1.2-7.1) were independent predictors for DN. Microhaematuria defined as ≥ 10 red blood cells per high-power field (OR 0.032; 95% CI: 0.01-0.11) and overweight (OR 0.21; 95% CI: 0.08-0.5) were independent predictors of NDRD. According to the predictive model based on the multivariate analysis, all patients with a score > 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

Nefritis intersticial tuberculosa, un diagnóstico difícil que precisa de una alta sospecha / Tuberculous interstitial nephritis: A difficult diagnosis that requires a high clinical suspicion

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¿Mejora la función renal tras la paratiroidectomía en el hiperparatirodismo primario? / Does renal function improve after parathyroidectomy in primary hyperparathyroidism?

Introducción: El hiperparatiroidismo primario (HPTP) es un trastorno endocrino frecuente, caracterizado por hipercalcemia y elevación de la parathormona. La disminución del filtrado glomerular ( < 60ml/min) se mantiene en la guías como un criterio para la realización de la paratiroidectomía (PTX) en el HPTP asintomático. La influencia que tiene la PTX sobre la evolución de la función renal es controvertida. Objetivos: Analizar las características clínicas, analíticas e histológicas de los pacientes intervenidos por HPTP, así como la evolución de la función renal tras la PTX. Material y métodos: Estudio retrospectivo de 297 pacientes con HPTP remitidos a cirugía en un único centro entre 1998 y 2016. Los parámetros analíticos se determinaron en situación basal, a la semana y al año de la PTX. Resultados: La incidencia de PTX fue de 38 casos/millón/año. La edad media fue 60 ± 14 años y el 80,5% de los pacientes eran mujeres. El 65,3% estaban asintomáticos. La nefrolitiasis fue el hallazgo clínico más frecuente (33%) seguido de la afectación ósea (29,5%). Las indicaciones de PTX fueron: síntomas clínicos (34,7%), hipercalcemia > 11,2 mg/dl (27%), litiasis renal (13%), baja masa ósea (12%), edad < 50 años (11%) y disminución del filtrado < 60 ml/min (2,3%). En el diagnóstico de localización el spect-MIBI presentó una sensibilidad del 92% y la ecografía cervical del 70%. El 94,3% de los casos de HPTP eran debidos a un adenoma paratiroideo. Tras la PTX se objetivó normalización de los parámetros relacionados con el HPTP. Objetivamos un incremento significativo de la creatinina sérica (0,81 vs. 0,85 mg/dl, p < 0,001) desde la primera semana del postoperatorio y que se mantiene al año. Cuando comparamos los pacientes según el filtrado glomerular basal, encontramos que el deterioro de la función renal solamente fue significativo en pacientes con filtrado glomerular > 60 ml/min (creatinina sérica basal 0,77 mg/dl vs. creatinina sérica al año 0,81 mg/dl, p < 0,001). Conclusiones: El HPTP cursó asintomático en la mayoría de los pacientes intervenidos. La hipercalcemia y la nefrolitiasis fueron las indicaciones más frecuentes de paratiroidectomía en los pacientes asintomáticos. El scan-MIBI fue el método de localización más útil. La curación quirúrgica del HPTP se sigue de un deterioro de la función renal, que se mantiene desde la primera semana de la cirugía

Introduction: Primary hyperparathyroidism (PHPT) is a common endocrine disorder characterised by hypercalcaemia and parathormone increase. Decreased glomerular filtration rate ( < 60 ml/min) continues to be a parathyroidectomy (PTX) criterion in asymptomatic PHPT. The influence of PTX on renal function evolution is the subject of debate. Objective: To analyse the clinical, laboratory and histological characteristics of patients undergoing PHPT, as well as renal function evolution after PTX. Material and methods: Retrospective study of 297 patients diagnosed with PHPT and referred to surgery in a single centre between 1998 and 2016. Laboratory parameters were determined at baseline, one week and one year after PTX. Results: The Incidence of PTX was 38 cases/million/year. Mean age was 60 ± 14 years and 80.5% of the patients were female. Approximately 65.3% were asymptomatic. Nephrolithiasis was the most common clinical finding (33%), followed by bone involvement (29.5%). PTX indications were: clinical symptoms (34.7%), hypercalcaemia > 11.2 mg/dl (27%), nephrolithiasis (13%), low bone mass (12%), age < 50 years (11%) and decreased glomerular filtration rate < 60 ml/min (2.3%). For diagnostic localisation, spect-MIBI had a sensitivity of 92% and cervical ultrasound of 70%. A total of 94.3% of PHPT cases were due to a parathyroid adenoma. After PTX, normalisation of PHPT-related parameters was observed. We found a significant increase in serum creatinine levels (0.81 vs 0.85 mg/dl, P < .001) from the first week post-PTX until the end of the first year. The renal function was only found to be significant in patients with glomerular filtration rate>60ml/min (baseline serum creatinine levels 0.77 mg/dl vs serum creatinine levels after one year 0.81 mg/dl, P < .001). Conclusions: PHPT was asymptomatic in most patients who underwent surgery. Hypercalcaemia and nephrolithiasis were the most common indications of parathyroidectomy in asymptomatic patients. MIBI scan was the most useful localisation method. Surgical treatment of PHPT is followed by renal function impairment, which persists after the first week post-PTX

¿Puede ser donante de órganos un paciente con enfermedad de Pompe? / Can a Pompe disease patient be an organ donor?

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Nefropatía por cilindros biliares asociada a disfunción hepática severa causada por esteroides anabolizantes / Bile cast nephropathy associated with severe liver dysfunction caused by anabolic steroids

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Glomerulopatías C3. Una nueva perspectiva en enfermedades glomerulares / C3 Glomerulopathies. A new perspective on glomerular diseases

El término glomerulonefritis membranoproliferativa denota un patrón general de daño glomerular fácilmente reconocido por microscopía óptica. Con estudios adiciones de microscopía electrónica e inmunofluorescencia, la clasificación en subgrupos es posible. El estudio por microscopía electrónica resuelve las diferencias según la localización de los depósitos electrodensos, mientras que la inmunofluorescencia detecta la composición de los depósitos electrodensos. La glomerulopatía C3 es una entidad descrita de forma reciente, una glomerulonefritis proliferativa (normalmente, pero no siempre), con un patrón de glomerulonefritis membranoproliferativa en la microscopía óptica y con depósitos de C3 aislados en el estudio de inmunofluorescencia, implicando una hiperactividad de la vía alternativa del complemento. La evaluación de un paciente con glomerulopatía C3 debe centrarse en la cascada del complemento, en la desregulación de la vía alternativa del complemento y en la cascada terminal del complemento. Aunque no hay actualmente tratamientos específicos para las glomerulopatías C3, una mejor comprensión de la patogénesis sentaría las bases para el posible uso de drogas anticomplemento como terapia de elección, como el eculizumab. En la presente revisión, se resume la patogenia de las glomerulopatías C3, centrándonos en el papel del complemento, las series de casos recientemente publicados y las opciones terapéuticas hasta el momento actual (AU)

Membranoproliferative glomerulonephritis (MPGN) denotes a general pattern of glomerular injury that is easily recognized by light microscopy. With additional studies, MPGN subgrouping is possible. For example, electron microscopy resolves differences in electron-dense deposition location, while immunofluorescence typically detects the composition of electron-dense deposits. A C3 glomerulopathy (C3G) is a recently described entity, a proliferative glomerulonephritis (usually but not always), with a MPGN pattern on light microscopy, with C3 staining alone on inmunoflouresencie, implicating hyperactivity of the alternative complement pathway. The evaluation of C3G should focus on the complement cascade, as dysregulation of the alternative pathway and terminal complement cascade underlies pathogenesis. Although there are no specific treatments currently available for C3G, a better understanding of their pathogenesis would set the stage for the possible use of anti-complement drugs, as eculizumab. In this review, we summarise the pathogenesis of the C3 glomerulopathies, focusing on the role of complement, the patient cohorts recently reported and options of treatment up to the current moment (AU)

Acute renal failure due to interstitial nephritis after intravesical instillation of BCG.

Intravesical chemotherapy with bacilli Calmette-Guerin (BCG) has been an established therapy for preventing recurrence of, and for treatment of, superficial transitional cell carcinoma of the bladder, but it is not without side effects. A variety of renal complications have been reported and attributed to mycobacterial infection. Although renal complications are uncommon, several cases of interstitial nephritis (with or without granulomas) and mesangial glomerulonephritis have been reported. We report a 76-year-old male patient who developed acute renal failure due to interstitial nephritis after intravesical instillation of BCG. Corticosteroids may serve the recovery of renal function without concomitant use of anti-tubercular therapy, provided systemic signs and mycobacterial infection are absent. Serum creatinine should be checked in at-risk patients in order to detect this complication early.