Predicting Future Renal Function Decline in Patients with Autosomal Dominant Polycystic Kidney Disease Using Mayo Clinic Classification.

INTRODUCTION: Mayo clinic classification (MCC) has been proposed in patients with autosomal dominant polycystic kidney disease (ADPKD) to identify who may experience a rapid decline of renal function. Our aim was to validate this predictive model in a population from southern Spain. METHODS: ADPKD patients with measurements of height-adjusted total kidney volume (HtTKV) and baseline estimated glomerular filtration rate (eGFR) >30 mL/min/1.73 m2 were selected. Last eGFR was estimated with Mayo Clinic (MC) equation and bias and accuracy were studied. We also analyzed predictive capacity of MCC classes using survival analysis and Cox regression models. RESULTS: We included 134 patients with a mean follow-up of 82 months. While baseline eGFR was not different between classes, last eGFR decreased significantly with them. eGFR variation rate was different according to the MCC class with a more rapid decline in 1C, 1D, and 1E classes. Final eGFR predicted was not significantly different from the real one, with an absolute bias of 0.6 ± 17.0 mL/min/1.73 m2. P10 accuracy was low ranging from 37.5 to 59.5% in classes 1C, 1D, and 1E. Using MC equation, the rate of eGFR decline was underestimated in 1C, 1D, and 1E classes. Cox regression analysis showed that MCC class is a predictor of renal survival after adjusting with baseline eGFR, age, sex, and HtTKV, with 1D and 1E classes having the worst prognosis. CONCLUSION: MCC classification is able to identify patients who will undergo a more rapid decline of renal function in a Spanish population. Prediction of future eGFR with MC equation is acceptable as a group, although it shows a loss of accuracy considering individual values. The rate of eGFR decline calculated using MC equation can underestimate the real rate presented by patients of 1C, 1D, and 1E classes.

Comparación de las ecuaciones MDRD y de las antiguas ecuaciones CKD-EPI frente a las nuevas ecuaciones CKD-EPI en pacientes con trasplante renal cuando se emplea 51Cr-EDTA para medir el filtrado glomerular / Comparison of MDRD and the old CKD-EPI equations with the new CKD-EPI equations in kidney transplant patients when glomerular filtration rate is measured with 51Cr-EDTA

INTRODUCCIÓN: Para la estimación del filtrado glomerular renal (FG) en trasplantados renales se emplean las ecuaciones MDRD y CKD-EPI de 2009 que han mostrado diferencias importantes cuando se comparan con el FG medido con técnicas de referencia. OBJETIVO: Analizar el rendimiento de las ecuaciones MDRD, CKD-EPI de 2009 y de 2012 en 270 pacientes trasplantados renales de un año de evolución, comparando con el FG medido con aclaramiento plasmático de 51Cr-EDTA. RESULTADOS: El FG medido fue 43,0 ± 11,4 (18,2-79,4) mL/min/1,73 m2, con niveles de creatinina de 1,42 ± 0,46 (0,60-4,33) mg/dL y de cistatina C de 1,45 ± 0,53 (0,42-3,48) mg/L. El FG medido se correlacionó moderadamente con creatinina (r = -0,61; p < 0,001) y cistatina C (r = - 0,52; p < 0,001). Empleando técnicas de regresión lineal observamos que creatinina, cistatina C, sexo y edad solo explicaban el 52% de la varianza total del FG. Todas las ecuaciones sobrestimaron el FG, con sesgo medio de +11,1 mL/min/1,73 m2 para MDRD, +16,4 mL/min/1,73 m2 para CKD-EPI de 2009, +15 mL/min/1,73 m2 para CKD-EPI con cistatina C y +14,1 mL/min/1,73 m2 para CKD-EPI con creatinina y cistatina C de 2012. Las estimaciones con MDRD y CKD-EPI de 2009 se correlacionaron mejor con 51Cr-EDTA que CKD-EPI con creatinina y/o cistatina C. Las sobrestimaciones se correlacionaron negativamente con los niveles de creatinina y cistatina C, siendo más importantes para CKD-EPI con creatinina y/o cistatina C cuando el FG fue mayor de 60 mL/min/1,73 m2. CONCLUSIONES: Las ecuaciones CKD-EPI de 2012 con creatinina y/o cistatina C sobrestiman el FG de forma muy marcada en estadios 1 y 2 de la enfermedad renal crónica, por lo que en ellos sería recomendable emplear la ecuación MDRD. La técnica de referencia empleada para medir el FG parece tener una influencia muy importante en el sesgo de las ecuaciones

BACKGROUND: When estimating the glomerular filtration rate (GFR) in kidney transplant patients, significant differences have been found between MDRD and the 2009 CKD-EPI equations, and reference techniques. OBJECTIVE: To analyse and compare the performance of MDRD and the 2009 and 2012 CKD-EPI equations against 51Cr-EDTA plasma clearance in measuring GFR in 270 kidney transplant patients after one year. RESULTS: The mean measured GFR was 43.0 ± 11.4 (18.2-79.4) ml/min/1.73 m2, with creatinine levels of 1.42 ± 0.46 (0.60-4.33) mg/dl and cystatin C levels of 1.45 ± 0.53 (0.42-3.48) mg/l. This correlated moderately with creatinine (r = -0.61, P < .001) and cystatin C (r = -0.52, P < .001). Using linear regression techniques, it was found that creatinine, cystatin C, gender and age only explained 52% of GFR total variance. All equations overestimated GFR, with a mean bias of +11.1 ml/min/1.73 m2 for MDRD, + 16.4 ml/min/1.73 m2 for 2009-CKD-EPI, +15 ml/min/1.73m2 for CKD-EPI with cystatin C, and +14.1 ml/min/1.73 m2 for 2012-CKD-EPI with creatinine and cystatin C. eGFR by MDRD and the 2009 CKD-EPI equation correlated better with 51Cr-EDTA than CKD-EPI with creatinine and/or cystatin C. The overestimations were negatively correlated with creatinine and cystatin C levels, most significantly for CKD-EPI with creatinine and/or cystatin C when GFR was greater than 60 ml/min/1.73 m2. CONCLUSIONS: The 2012 CKD-EPI equations with creatinine and/or cystatin C significantly overestimate GFR in stage 1 and 2 chronic kidney disease. The MDRD equations is therefore recommended in these cases. The reference method used to measure GFR seems to heavily influence the bias of the equations

Comparison of MDRD and the old CKD-EPI equations with the new CKD-EPI equations in kidney transplant patients when glomerular filtration rate is measured with 51Cr-EDTA. / Comparación de las ecuaciones MDRD y de las antiguas ecuaciones CKD-EPI frente a las nuevas ecuaciones CKD-EPI en pacientes con trasplante renal cuando se emplea 51Cr-EDTA para medir el filtrado glomerular.

BACKGROUND: When estimating the glomerular filtration rate (GFR) in kidney transplant patients, significant differences have been found between MDRD and the 2009 CKD-EPI equations, and reference techniques. OBJECTIVE: To analyse and compare the performance of MDRD and the 2009 and 2012 CKD-EPI equations against 51Cr-EDTA plasma clearance in measuring GFR in 270 kidney transplant patients after one year. RESULTS: The mean measured GFR was 43.0±11.4 (18.2-79.4)ml/min/1.73m2, with creatinine levels of 1.42±0.46 (0.60-4.33)mg/dl and cystatin C levels of 1.45±0.53 (0.42-3.48)mg/l. This correlated moderately with creatinine (r=-0.61, P<.001) and cystatin C (r=-0.52, P<.001). Using linear regression techniques, it was found that creatinine, cystatin C, gender and age only explained 52% of GFR total variance. All equations overestimated GFR, with a mean bias of +11.1ml/min/1.73m2 for MDRD, +16.4ml/min/1.73m2 for 2009-CKD-EPI, +15ml/min/1.73m2 for CKD-EPI with cystatin C, and +14.1ml/min/1.73m2 for 2012-CKD-EPI with creatinine and cystatin C. eGFR by MDRD and the 2009 CKD-EPI equation correlated better with 51Cr-EDTA than CKD-EPI with creatinine and/or cystatin C. The overestimations were negatively correlated with creatinine and cystatin C levels, most significantly for CKD-EPI with creatinine and/or cystatin C when GFR was greater than 60ml/min/1.73m2. CONCLUSIONS: The 2012 CKD-EPI equations with creatinine and/or cystatin C significantly overestimate GFR in stage 1 and 2 chronic kidney disease. The MDRD equations is therefore recommended in these cases. The reference method used to measure GFR seems to heavily influence the bias of the equations.

Panorámica de la poliquistosis renal autosómica dominante en una región del sur de España / Overview of autosomal dominant polycystic kidney disease in the south of Spain

Introducción: La poliquistosis renal autosómica dominante es la enfermedad renal hereditaria más frecuente aunque los datos disponibles generalmente son tras el inicio del tratamiento renal sustitutivo. Objetivo: Conocer la situación global de la poliquistosis renal autosómica dominante en el ámbito sanitario de Granada. Material y métodos: Desde enero 2007 hasta diciembre 2016 hemos recogido información clínica, familiar y demográfica de todos los pacientes con poliquistosis renal autosómica dominante, estuvieran o no en tratamiento renal sustitutivo, atendidos en el área de Granada. Se han utilizado los programas informáticos SPSS 15.0 y GenoPro. Resultados: Mil ciento siete pacientes diagnosticados, el 50,6% son varones. Se han estudiado 4-6 generaciones/familia. El 99,1% de raza caucásica. Hay áreas geográficas con mayor concentración. No hay antecedentes familiares en el 2,43%. La edad media de diagnóstico es de 34±17,8 años y en el 57,7% de los casos, el diagnóstico se produce después de tener descendencia. El principal motivo de diagnóstico son los antecedentes familiares (46,4%). La edad media de entrada en técnica es de 54,2±11,05 años. El 96,3% de los fallecidos tenían algún grado de insuficiencia renal en el momento del exitus. La edad media del exitus es de 60,9±14,10 años, siendo desconocida la principal causa de muerte (33,5%) seguida de la cardiovascular (27,8%). Conclusiones: Casos y familias se concentran en algunas áreas geográficas, un número importante de individuos están sin diagnosticar, fallecen antes por causa cardiovascular y se diagnostican tarde respecto al momento reproductivo. Dado que no hay tratamiento curativo, la estrategia de prevención primaria mediante el diagnóstico genético preimplantacional adquiere protagonismo (AU)

Introduction: Although autosomal dominant polycystic kidney disease is the most common hereditary kidney disease, available data tend to be limited to after initiation of renal replacement therapy. Objective: To ascertain an overview of autosomal dominant polycystic kidney disease within the health area of Granada in southern Spain. Material and methods: From January 2007 to December 2016, we collected clinical, family and demographic information about all patients with autosomal dominant polycystic kidney disease, irrespective of whether or not they were treated with RRT, in the Granada health area. The computer software SPSS 15.0 and GenoPro were used. Results: 50.6% of the 1,107 diagnosed patients were men. 99.1% were Caucasian and 4-6 generations/family were studied. The geographical distribution was heterogeneous. There was no family history in 2.43%. The mean age of diagnosis was 34.0±17.80 years and the diagnosis was made after having offspring in 57.7% of cases. The main reason for diagnosis was family history (46.4%). The mean age of initiation of renal replacement therapy was 54.2±11.05 years. 96.3% of the deceased had some degree of renal failure at the time of death. The mean age of death was 60.9±14.10 years, the main cause of death being unknown in 33.5% of cases, followed by cardiovascular (27.8%). Conclusions: Cases and families were concentrated in certain geographical areas and a significant number of individuals were undiagnosed prior to cardiovascular death or diagnosed late after reproduction. Given that there is currently no curative treatment, the primary prevention strategy of preimplantation genetic diagnosis should play a leading role (AU)

Overview of autosomal dominant polycystic kidney disease in the south of Spain. / Panorámica de la poliquistosis renal autosómica dominante en una región del sur de España.

INTRODUCTION: Although autosomal dominant polycystic kidney disease is the most common hereditary kidney disease, available data tend to be limited to after initiation of renal replacement therapy. OBJECTIVE: To ascertain an overview of autosomal dominant polycystic kidney disease within the health area of Granada in southern Spain. MATERIAL AND METHODS: From January 2007 to December 2016, we collected clinical, family and demographic information about all patients with autosomal dominant polycystic kidney disease, irrespective of whether or not they were treated with RRT, in the Granada health area. The computer software SPSS 15.0 and GenoPro were used. RESULTS: 50.6% of the 1,107 diagnosed patients were men. 99.1% were Caucasian and 4-6 generations/family were studied. The geographical distribution was heterogeneous. There was no family history in 2.43%. The mean age of diagnosis was 34.0±17.80 years and the diagnosis was made after having offspring in 57.7% of cases. The main reason for diagnosis was family history (46.4%). The mean age of initiation of renal replacement therapy was 54.2±11.05 years. 96.3% of the deceased had some degree of renal failure at the time of death. The mean age of death was 60.9±14.10 years, the main cause of death being unknown in 33.5% of cases, followed by cardiovascular (27.8%). CONCLUSIONS: Cases and families were concentrated in certain geographical areas and a significant number of individuals were undiagnosed prior to cardiovascular death or diagnosed late after reproduction. Given that there is currently no curative treatment, the primary prevention strategy of preimplantation genetic diagnosis should play a leading role.

[OSTEOPOROSIS AND BODY MASS INDEX IN RENAL TRANSPLANT RECIPIENTS]. / OSTEOPOROSIS E ÍNDICE DE MASA CORPORAL EN EL TRASPLANTADO RENAL.

INTRODUCTION: frequently after kidney transplantation there is an increase in weight with a resulting high percent of obesity in these recipients. This combined with a rapid loss of bone mass, a higher prevalence of osteoporosis and fractures is evident than in normal populations. OBJECTIVES: to explore the relationship between body mass index (BMI) and prevalence of osteoporosis in a population of renal transplant recipients. METHODS: prospective longitudinal study design. The study was conducted on 306 kidney transplant recipients. The relationship between weigh and body mass index with femoral and lumbar osteopenia and osteoporosis prevalence at the moment of transplant and at 12 months post was explored. RESULTS: there was a high prevalence of overweight (35.6%) and obese (14.1%) recipients after renal transplant and 1 year after (42.2% and 24.2% respectively). Significant differences were found(p = 0.049) between the weight at the time of transplant and the presence of osteopenia or osteoporosis at the lumbar level one year after, the highest weights were in recipients with osteoporosis. The mean BMI was higher (p = 0.028) in osteoporotic patients (26.59 kg/m2) than in patients with osteopenia (24.23 kg/m2). CONCLUSION: results seem to be consistent with recent studies in the general population showing excessive weight as a possible factor detrimental to the bone health.

Introducción y objetivos: tras el trasplante renal es frecuente un aumento de peso, así como un elevado porcentaje de obesidad en estos pacientes. Por otro lado, tras el trasplante se produce una pérdida de la masa ósea, siendo la prevalencia de osteoporosis y fracturas óseas mayor que en la población general. Objetivos: explorar la relación entre el índice de masa corporal y la prevalencia de osteopenia y osteoporosis en una población de trasplantados renales. Material y método: estudio longitudinal prospectivo sobre una muestra de 306 trasplantados renales. Se exploraron las relaciones entre el peso y el índice de masa corporal con la prevalencia de osteopenia y osteoporosis a nivel femoral y lumbar en el momento del trasplante y a los 12 meses del mismo. Resultados: se halló una alta prevalencia de sobrepeso (35,6%) y obesidad (14,1%) tras el trasplante renal y al año del mismo (42,2% y 24,2%, respectivamente). Se hallaron diferencias estadísticamente significativas (p = 0,049) entre el peso en el momento del trasplante y la presencia de osteopenia u osteoporosis al año del mismo a nivel lumbar, siendo el peso medio más elevado entre los pacientes con osteoporosis. La media del IMC fue más elevada (p = 0,028) en los pacientes osteoporóticos (26,59 kg/m2) que en los pacientes con osteopenia (24,23 kg/m2). Conclusiones: nuestros resultados parecen estar en concordancia con recientes estudios realizados en la población general, que muestran el sobrepeso como un posible factor perjudicial para el hueso.

Effect of paricalcitol on mineral bone metabolism in kidney transplant recipients with secondary hyperparathyroidism.

INTRODUCTION: Secondary hyperparathyroidism is highly prevalent in kidney transplant recipients, and commonly results in hypercalcaemia; an association to osteopenia and bone fractures has also been observed. Paricalcitol has proved effective to control secondary hyperparathyroidism in chronic kidney disease in both dialysed and non-dialysed patients, with a low hypercalcaemia incidence. Currently available experience on paricalcitol use in kidney transplant recipients is scarce. Our main aim was to show the effect of paricalcitol on mineral bone metabolism in kidney transplant recipients with secondary hyperparathyroidism. MATERIAL AND METHODS: A retrospective multicentre study in kidney transplant recipients aged>18 years with a 12-month or longer post-transplantation course, stable renal function, having received paricalcitol for more than 12 months, with available clinical follow-up for a 24-month period. RESULTS: A total of 69 patients with a 120 ± 92-month post-transplantation course were included. Baseline creatinine was 2.2 ± 0.9 mg/dl y GFR-MDRD was 36 ± 20 ml/min/1.73 m(2). Paricalcitol doses were gradually increased during the study: baseline 3.8 ± 1.9 µg/week, 12 months 5.2 ± 2.4 µg/week; 24 months 6.0 ± 2.9 µg/week (P<.001). Serum PTH levels showed a significant fast decline: baseline 288 ± 152 pg/ml; 6 months 226 ± 184 pg/ml; 12 months 207 ± 120; 24 months 193 ± 119 pg/ml (P<.001). Reduction from baseline PTH was ≥30% in 42.4% of patients at 12 months y in 65.2% of patients at 24 months. Alkaline phosphatase showed a significant decrease in first 6 months followed by a plateau: baseline 92 ± 50 IU/l; 6 months 85 ± 36 IU/l, 12 months 81 ± 39 IU/l (P<.001). Overall, no changes were observed in serum calcium and phosphorus, and in urine calcium excretion. PTH decline was larger in patients with higher baseline levels. Patients with lower baseline calcium levels showed significantly increased levels (mean increase was 0.5-0.6 mg/dl) but still within normal range, whereas patients with baseline calcium>10mg/dl showed gradually decreasing levels. Fifteen (21.7%) patients had received prior calcitriol therapy. When shifted to paricalcitol, such patients required paricalcitol doses significantly larger than those not having received calcitriol. Paricalcitol was used concomitantly to cinacalcet in 11 patients with significant PTH reductions being achieved; clinical course was similar to other patients and paricalcitol doses were also similar. CONCLUSIONS: Paricalcitol is an effective therapy for secondary hyperparathyroidism in kidney transplant recipients. Overall, no significant changes were observed in calcium and phosphorus levels or urinary excretion. Patients having previously received calcitriol required higher paricalcitol doses. When used in patients receiving cinacalcet, paricalcitol results in a significant PTH fall, with paricalcitol doses being similar to those used in patients not receiving cinacalcet.

Osteoporosis e índice de masa corporal en el trasplantado renal / Osteoporosis and body mass index in renal transplant recipients

Introducción y objetivos: tras el trasplante renal es frecuente un aumento de peso, así como un elevado porcentaje de obesidad en estos pacientes. Por otro lado, tras el trasplante se produce una pérdida de la masa ósea, siendo la prevalencia de osteoporosis y fracturas óseas mayor que en la población general. Objetivos: explorar la relación entre el índice de masa corporal y la prevalencia de osteopenia y osteoporosis en una población de trasplantados renales. Material y método: estudio longitudinal prospectivo sobre una muestra de 306 trasplantados renales. Se exploraron las relaciones entre el peso y el índice de masa corporal con la prevalencia de osteopenia y osteoporosis a nivel femoral y lumbar en el momento del trasplante y a los 12 meses del mismo. Resultados: se halló una alta prevalencia de sobrepeso (35,6%) y obesidad (14,1%) tras el trasplante renal y al año del mismo (42,2% y 24,2%, respectivamente). Se hallaron diferencias estadísticamente significativas (p=0,049) entre el peso en el momento del trasplante y la presencia de osteopenia u osteoporosis al año del mismo a nivel lumbar, siendo el peso medio más elevado entre los pacientes con osteoporosis. La media del IMC fue más elevada (p=0,028) en los pacientes osteoporóticos (26,59 kg/m2) que en los pacientes con osteopenia (24,23 kg/m2). Conclusiones: nuestros resultados parecen estar en concordancia con recientes estudios realizados en la población general, que muestran el sobrepeso como un posible factor perjudicial para el hueso (AU)

Introduction: frequently after kidney transplantation there is an increase in weight with a resulting high percent of obesity in these recipients. This combined with a rapid loss of bone mass, a higher prevalence of osteoporosis and fractures is evident than in normal populations. Objectives: to explore the relationship between body mass index (BMI) and prevalence of osteoporosis in a population of renal transplant recipients. Methods: prospective longitudinal study design. The study was conducted on 306 kidney transplant recipients. The relationship between weigh and body mass index with femoral and lumbar osteopenia and osteoporosis prevalence at the moment of transplant and at 12 months post was explored. Results: there was a high prevalence of overweight (35.6%) and obese (14.1%) recipients after renal transplant and 1 year after (42.2% and 24.2% respectively). Significant differences were found(p=0.049) between the weight at the time of transplant and the presence of osteopenia or osteoporosis at the lumbar level one year after, the highest weights were in recipients with osteoporosis. The mean BMI was higher (p=0.028) in osteoporotic patients (26.59 kg/m2) than in patients with osteopenia (24.23 kg/m2). Conclusion: results seem to be consistent with recent studies in the general population showing excessive weight as a possible factor detrimental to the bone health (AU)

Efecto de paricalcitol sobre el metabolismo mineralóseo en pacientes trasplantados renales con hiperparatiroidismo secundario / Effect of paricalcitol on mineral bone metabolism in kidney transplant recipients with secondary hyperparathyroidism

Introducción: El hiperparatiroidismo secundario es muy prevalente en pacientes trasplantados renales. Cursa con frecuencia con hipercalcemia y se ha asociado al desarrollo de osteopenia y fracturas óseas. El paricalcitol ha mostrado su eficacia en el control del hiperparatiroidismo secundario en la enfermedad renal crónica con y sin diálisis, con una baja incidencia de hipercalcemia. La experiencia con paricalcitol en trasplantados renales es muy escasa. El objetivo de este trabajo fue mostrar el efecto sobre el metabolismo mineralóseo del paricalcitol en trasplantados renales con hiperparatiroidismo secundario. Material y métodos: Estudio retrospectivo multicéntrico con trasplantados renales de más de 18 años de edad y más de 12 meses de evolución postrasplante, con función renal estable, que hayan sido tratados con paricalcitol durante más de 12 meses, con seguimiento clínico hasta los 24 meses de tratamiento. Resultados: Se incluyó a 69 pacientes, con 120±92 meses postrasplante, con creatinina inicial de 2,2±0,9mg/dl y FG-MDRD 36±20ml/min/1,73 m2. La dosis de paricalcitol se incrementó progresivamente durante el estudio: basal 3,8±1,9μg/semana, 12 meses 5,2±2,4μg/semana; 24 meses 6,0±2,9μg/semana (p<0,001). Los niveles séricos de PTH descendieron de forma rápida y significativa: basal 288±152 pg/ml; 6 meses 226±184 pg/ml; 12 meses 207±120; 24 meses 193±119 pg/ml (p<0,001). Observamos una reducción sobre PTH basal ≥30% en el 42,4% de los pacientes a los 12 meses y en el 65,2% de los pacientes a los 24 meses. La fosfatasa alcalina descendió también significativamente en los 6 primeros meses para luego estabilizarse: basal 92±50 UI/l; 6 meses 85±36 UI/l, 12 meses 81±39 UI/l (p<0,001). Globalmente no hubo modificaciones en el calcio o fósforo séricos ni en la excreción urinaria de calcio. La reducción de PTH fue más importante en trasplantados con niveles séricos más elevados de partida. Observamos que los pacientes con calcio basal más bajo mostraron un incremento significativo de sus cifras de 0,5-0,6 mg/dl en promedio aunque manteniéndose en rango de normalidad, mientras que pacientes con calcio basal > 10 mg/dl mostraron una reducción progresiva de sus cifras. Quince (21,7%) pacientes seguían tratamiento previo con calcitriol y al cambiarlos a paricalcitol precisaron dosis significativamente mayores que los pacientes que no habían recibido calcitriol. El paricalcitol fue asociado a cinacalcet en 11 pacientes, con reducciones significativas de PTH, con evolución similar al resto de la población y con dosis de paricalcitol también similares. Conclusiones: Paricalcitol es eficaz en el tratamiento del hiperparatiroidismo secundario de trasplantados renales. Globalmente no observamos modificaciones significativas de los niveles de calcio ni de fósforo, ni en su excreción urinaria. Los pacientes en tratamiento previo con calcitriol precisaron dosis mayores de paricalcitol. Cuando el paricalcitol se administra a pacientes tratados con cinacalcet, se observa un descenso significativo de la PTH con dosis de paricalcitol similar a pacientes sin cinacalcet (AU)

Introduction: Secondary hyperparathyroidism is highly prevalent in kidney transplant recipients, and commonly results in hypercalcaemia; an association to osteopenia and bone fractures has also been observed. Paricalcitol has proved effective to control secondary hyperparathyroidism in chronic kidney disease in both dialysed and non-dialysed patients, with a low hypercalcaemia incidence. Currently available experience on paricalcitol use in kidney transplant recipients is scarce. Our main aim was to show the effect of paricalcitol on mineral bone metabolism in kidney transplant recipients with secondary hyperparathyroidism. Material and methods: A retrospective multicentre study in kidney transplant recipients aged > 18 years with a 12-month or longer post-transplantation course, stable renal function, having received paricalcitol for more than 12 months, with available clinical follow-up for a 24-month period. Results: A total of 69 patients with a 120 ± 92-month post-transplantation course were included. Baseline creatinine was 2.2 ± 0.9 mg/dl y GFR-MDRD was 36 ± 20 ml/min/1.73m2. Paricalcitol doses were gradually increased during the study: baseline 3.8 ± 1.9 g/week, 12 months 5.2 ± 2.4 g/week; 24 months 6.0 ± 2.9 g/week (P<.001). Serum PTH levels showed a significant fast decline: baseline 288 ± 152 pg/ml; 6 months 226 ± 184 pg/ml; 12 months 207 ± 120; 24 months 193 ± 119 pg/ml (P<.001). Reduction from baseline PTH was ≥30% in 42.4% of patients at 12 months y in 65.2% of patients at 24 months. Alkaline phosphatase showed a significant decrease in first 6 months followed by a plateau: baseline 92 ± 50 IU/l; 6 months 85 ± 36 IU/l, 12 months 81 ± 39 IU/l (P<.001). Overall, no changes were observed in serum calcium and phosphorus, and in urine calcium excretion. PTH decline was larger in patients with higher baseline levels. Patients with lower baseline calcium levels showed significantly increased levels (mean increase was 0.5-0.6 mg/dl) but still within normal range, whereas patients with baseline calcium > 10 mg/dl showed gradually decreasing levels. Fifteen (21.7%) patients had received prior calcitriol therapy. When shifted to paricalcitol, such patients required paricalcitol doses significantly larger than those not having received calcitriol. Paricalcitol was used concomitantly to cinacalcet in 11 patients with significant PTH reductions being achieved; clinical course was similar to other patients and paricalcitol doses were also similar. Conclusions: Paricalcitol is an effective therapy for secondary hyperparathyroidism in kidney transplant recipients. Overall, no significant changes were observed in calcium and phosphorus levels or urinary excretion. Patients having previously received calcitriol required higher paricalcitol doses. When used in patients receiving cinacalcet, paricalcitol results in a significant PTH fall, with paricalcitol doses being similar to those used in patients not receiving cinacalcet (AU)

Estudio del índice de masa corporal y su relación con la densidad mineral ósea en pacientes con enfermedad renal crónica y tratamiento en hemodiálisis / Study of body mass index and its relationship with bone mineral density in patients with chronic kidney disease and hemodialysis treatment

Fundamento: Las alteraciones en el metabolismo mineral óseo representan una causa importante de morbilidad y mortalidad en los pacientes con insuficiencia renal crónica en hemodiálisis (IRC). La disminución de masa ósea y el riesgo de fracturas son un hallazgo frecuente. Diferentes factores explican esta disminución de masa ósea; entre ellos los relacionados con sus valores antropométricos. El objetivo de este trabajo es conocer el comportamiento de la densidad mineral ósea frente al peso y talla utilizando el índice de masa corporal (IMC) de pacientes con Enfermedad Renal Crónica (ERC) y tratamiento en hemodiálisis. Material y métodos: Se estudió la densidad mineral ósea (DMO), mediciones T-score y Z-score en cuello de fémur, trocánter, intertrocánter, 1/3 proximal fémur, triangulo de Ward´s, L2, L3 y L4, usando densitometría DXA y la composición corporal en 73 pacientes (40 hombres y 33 mujeres) en hemodiálisis. Resultados: El tiempo total en diálisis de estos pacientes fue para las mujeres 9,7 ± 5,54 años y para los varones 10,18 ± 7,16 años. Como grupo los pacientes mostraron una correlación positiva muy significativa entre el DMO y el peso, la altura y el IMC, las fracturas, el tiempo en diálisis y la PTH intacta. Conclusiones: Los pacientes con ERC en programa de hemodiálisis periódica, muestran una reducción significativa de la DMO, que afecta tanto a columna lumbar como fémur. El peso y la talla influyen sobre la DMO y sobre el recambio óseo, factores muy importantes de predicción de riesgo de fractura. El IMC es el principal determinante de DMO (AU)

Background: Alterations in bone mineral metabolism represent an important cause of morbidity and mortality in patients with chronic renal failure on haemodialysis, the decrease in bone mass and fracture risk are a common finding, there are several factors that explain this decrease in bone mass, including those associated with anthropometric values. The aim of this study was to analyze bone mineral density versus height and weight using the body mass index (BMI) of patients with chronic kidney disease (CKD) and haemodialysis treatment. Material and methods: We studied bone mineral density (BMD) measurements T-score and Z-score at femoral neck, trochanter, intertrochanter, 1 / 3 proximal femur, Ward?s triangle, L2, L3 and L4, using DXA densitometry and body composition in 73 patients (40 men and 33 women) on haemodialysis. Results: duration on dialysis was 9.7 ± 5.54 years for women and 10.18 ± 7.16 years for men. As a group the patients showed a significant positive correlation between BMD and weight, height, and BMI, fractures, time on dialysis and intact PTH. Conclusions: Patients with CKD on haemodialysis program show a significant reduction in BMD, which affects both lumbar spine and femur. The weight and size influence on BMD and bone turnover, important factors for predicting fracture risk. BMI is the main determinant of BMD (AU)

[Cystinosis: diagnosis through the measurement of the leukocyte cystine content by HPLC]. / Cistinosis: diagnóstico mediante la determinación del contenido de cistina intraleucocitaria por cromatografía líquida de alta resolución.

BACKGROUND AND OBJECTIVE: Cystinosis is an autosomal recessive disorder characterized by an accumulation of intralysosomal cystine. Three disease forms exist, infantile, juvenile or late-onset, and ocular nonnephropathic cystinosis, delineated on the basis of severity of symptoms and age of onset. The knowledge of early clinic manifestations and the onset of the appropriate therapy delay the evolution of the disease and improve the general conditions. Therefore, it is necessary to develop a sensible diagnostic method for early detection and treatment of the disease. CLINICAL CASE AND METHODS: The leukocyte cystine content was determined by HPLC in a 42 years old female patient after renal transplantation, and with the clinical characteristic complications of the intermediate cystinosis. Equally, the molecular characterization of the structural defects of the cystinosin (CTNS) gene was made in the patient and in all family members. RESULTS: By measuring of the leukocyte cystine content in the patient and family members, we have determined 5 family members as heterozygous. This result was confirmed by molecular analysis that showed the approximately 65 kb deletion in the 5 family members. The patient was heterozygous for the approximately 65 kb deletion, and the second alteration was not determined. CONCLUSIONS: We presented a useful diagnostic method, based in the determination of cystine content of polymorphonuclear leukocytes, which permits to detect the heterozygous individuals.

Cistinosis: diagnóstico mediante la determinación del contenido de cistina intraleucocitaria por cromatografía líquida de alta resolución / Cystinosis: diagnostic through the measurement of the leukocyte cystine content by HPLC

FUNDAMENTO Y OBJETIVO: La cistinosis es una enfermedad autosómica recesiva caracterizada por una acumulación de cistina en el interior de los lisosomas. Existen 3 formas de la enfermedad, la infantil, la juvenil y la ocular no nefropática, clasificadas según la gravedad de los síntomas y la edad de inicio. El conocimiento de las manifestaciones clínicas tempranas y el inicio de un tratamiento adecuado retrasan la evolución de la enfermedad y mejoran las condiciones generales. Por ello es necesario desarrollar un método diagnóstico sensible para la detección temprana y el tratamiento de la enfermedad. CASO CLÍNICO Y MÉTODOS: Se determinó el contenido de cistina intraleucocitaria mediante cromatografía líquida de alta resolución (HPLC), en una paciente de 42 años de edad que había recibido un trasplante renal y presentaba las características clínicas de la cistinosis intermedia. Se realizó asimismo el estudio molecular de posibles alteraciones estructurales en el gen de la cistinosina (CTNS), tanto en la paciente como en los miembros de la familia. RESULTADOS: Mediante la determinación del contenido de cistina intraleucocitaria en la paciente y los miembros familiares, se encontró a 5 miembros de la familia heterocigotos para la enfermedad. Este resultado se confirmó posteriormente con el análisis molecular, que mostró la presencia de la deleción de aproximadamente 65 kb en todos ellos. La paciente fue heterocigota para dicha deleción, pero la segunda alteración no se determinó. CONCLUSIONES: La determinación del contenido de cistina de leucocitos polimorfonucleares, que es un método útil de diagnóstico de cistinosis, permite detectar a los individuos heterocigotos (AU)