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Este artículo de investigación se encuentra disponible en la siguiente URL: https://www.revistanefrologia.com/es-pdf-S0211699521001570 En este artículo de investigación también participan: Ramón A. García-Trabanino, Miguel Hueso, Pedro Trinidad, Aquiles Jara, Mónica Furlano, Rosana Gelpi, Ana Vila-Santandreu, César A. Restrepo, Maya Sánchez-Baya, Carolt Arana, Marián Goicoechea, Verónica Coll, Julián Segura, Orlando Gutiérrez, Kamyar Kalantar-Zadeh, Emilio Sánchez y Alejandro Ferreiro.

This study reports a case of a 13-year-old male with a 3-year history of severe and intermittent hypokalemia episodes of unknown origin, requiring admission to the intensive care unit (ICU) for long QT syndrome (LQTS), finally diagnosed of redistributive hypokalemia secondary to the abuse of β-adrenergic agonists in the context of a probable factitious disorder. RESUMO O presente estudo relata o caso de um jovem de 13 anos de idade com histórico, há três anos, de episódios de hipocalemia grave intermitente de origem desconhecida, internado em unidade de terapia intensiva (UTI) por síndrome do QT longo (SQTL). O paciente foi diagnosticado com hipocalemia por redistribuição secundária ao abuso de agonistas β-adrenérgicos, em contexto de provável transtorno factício.

Qualitat de vida; Qüestionari; Insuficiència renal Calidad de vida; Cuestionario; Insuficiencia renal Quality of life; Questionnaire; Kidney Failure Background. Measures of fear of progression or recurrence of illnesses have been criticized for neglecting cross-cultural validity. Therefore, we assessed the psychometric properties of the Spanish version of the Fear of Kidney Failure Questionnaire (FKFQ), to determine whether postdonation fear of kidney failure (FKF) influenced the donors’ psychosocial status, and define variables that characterized donors with high FKFQ scores. Methods. We included 492 participants (211 donors) in a multicenter, 11-year, retrospective, cross-sectional study. Donors were classified with a Latent Class Analysis of the FKFQ-item scores and characterized with a multivariable logistic regression analysis. We calculated the risk ratio based on predicted marginal probabilities. Results. The Spanish version of the FKFQ showed acceptable psychometric properties. FKF was uncommon among donors, but we detected a small subgroup (n = 21, 9.9%) with high FKF (mean FKFQ score = 14.5, 3.1 SD). Compared with other donors, these donors reported higher anxiety and depression (38% and 29% of potential anxiety and depressive disorders), worse quality of life, and less satisfaction with the donation. Donors with high FKFQ scores were characterized by higher neuroticism combined with postdonation physical symptoms that interfered with daily activities. Conclusions. The FKFQ was cross-culturally valid, and thus, it may be used to assess the FKF in Spanish-speaking donors. New interventions that promote positive affectivity and evidence-based treatments for worry could be adapted for treating FKF.

Kidney transplant recipients might be at higher risk for severe coronavirus disease 2019 (COVID‐19). However, risk factors for relevant outcomes remain uncertain in this population. This is a multicentric kidney transplant cohort including 104 hospitalized patients between Mar 4 and Apr 17, 2020. Risk factors for death and acute respiratory distress syndrome (ARDS) were investigated, and clinical and laboratory data was analyzed. The mean age was 60 years. Forty‐seven patients (54.8%) developed ARDS. Obesity was associated to ARDS development (OR 2.63; p=0.04). Significant age differences were not found among patients developing and not developing ARDS (61.3yr vs 57.8yr, p=0.16). Seventy‐six (73%) patients were discharged while 28 (27%) died. Death was more common among the elderly (55yr and 70.8yr, p

Background: In December 2019, the novel SARS-CoV-2 virus triggered a large-scale pandemic of coronavirus disease 2019 (COVID-19) Kidney transplant recipients m

Resumen Introduccion La mayoria de las guias recomiendan realizar una cistografia miccional (CUMS) temprana tras el tratamiento endoscopico del reflujo vesicoureteral (RVU), pero no hay consenso sobre como hacer el seguimiento a largo plazo en este grupo de pacientes. El objetivo de este estudio es analizar si es necesaria la realizacion de una CUMS tardia en aquellos pacientes tratados con exito. Material y metodo Hemos revisado las historias clinicas de aquellos pacientes tratados de RVU con el copolimero de dextranomero/acido hialuronico (Dx/AH) desde 2006 a 2010. Se han seleccionado aquellos pacientes que estaban curados tras el tratamiento con mas de 3 anos de seguimiento y control cistografico tardio. Hemos analizado los hallazgos clinicos y radiologicos a largo plazo. Resultados Ciento sesenta ninos con 228 ureteres refluyentes recibieron tratamiento con Dx/AH con un seguimiento medio de 52,13 meses. A 215 se les realizo CUMS de forma temprana, siendo la tasa de exito del 84,1%. El grupo de estudio fueron 94/215 ureteres refluyentes que tuvieron un seguimiento clinico y cistografico tardio mayor de 3 anos. En el 79,8% la CUMS mostro ausencia de RVU, siendo la tasa de exito tardio clinico del 91,7%. La incidencia de infeccion del tracto urinario febril entre aquellos que estaban curados desde el punto de vista radiologico frente a aquellos en los que recidivo el RVU fue del 8 y 15%, respectivamente. Solo existieron diferencias significativas en la recidiva del RVU entre aquellos ureteres que se habian tratado de forma inicial con una puncion o con 2 punciones de Dx/AH. Conclusion Si el objetivo del tratamiento del RVU es disminuir las infecciones del tracto urinario febril, no es necesario realizar una CUMS tardia tras un tratamiento exitoso inicial con Dx/AH, a pesar de que la tasa de exito radiologico es menor que la del clinico.

Introduction Improved outcome and longer life expectancy in patients with cystinosis and the intrinsic complexity of the disease, underline the need for a guided transition of patients from pediatric to adult care. The process aims to guarantee the continuum of care and enable the empowerment of patients from guardian to self-care. Methods Bibliography review, expert opinion and anonymous surveys of patients, relatives and patient advocacy groups. Results A new plan to support and coordinate the transition of cystinotic patients providing specific proposals for a variety of medical fields and improved treatment adherence. Nephrologists play a key role in the transition since most cystinotic patients have severe chronic kidney disease and require kidney transplantation before adulthood. Conclusion We present a proposal providing recommendations and a chronogram to aid the transition of adolescents and young adults with cystinosis in our area.

ResumenIntroducciónLa cistinosis es una enfermedad lisosomal minoritaria de expresión sistémica con especial afectación renal y oftalmológica, en la que los pacientes inician terapia renal sustitutiva en la primera década de la vida en ausencia de tratamiento. El pronóstico de la cistinosis depende del diagnóstico precoz, la pronta instauración del tratamiento con cisteamina y el buen cumplimiento terapéutico. La progresión de la enfermedad renal y de las complicaciones extrarrenales y una menor supervivencia, son más acentuadas en pacientes no adherentes.ObjetivoEl objetivo de este trabajo fue la elaboración de unas recomendaciones para la atención integral de la cistinosis y la transición del adolescente a la medicina del adulto, basadas en la experiencia clínica, con el fin de reducir el impacto de la enfermedad y mejorar la calidad de vida y el pronóstico del paciente.MétodoBúsqueda bibliográfica y reuniones de consenso de un equipo multidisciplinar de expertos en la práctica clínica con pacientes afectos de cistinosis (Grupo T-CiS.bcn), procedentes de 5 hospitales localizados en Barcelona.ResultadosEl documento recoge recomendaciones específicas y necesarias para el diagnóstico, tratamiento y seguimiento multidisciplinar de la cistinosis en las siguientes áreas: nefrología, diálisis, trasplante renal, oftalmología, endocrinología, neurología, laboratorio, consejo genético, enfermería y farmacia.ConclusionesDisponer de un documento de referencia para la atención integral de la cistinosis constituye una herramienta de soporte para los profesionales de la salud que asisten a estos pacientes. Los principales pilares en los que se sustenta son: a) el enfoque multidisciplinar, b) la adecuada monitorización de la enfermedad y control de los niveles de cistina intraleucocitarios, c) la importancia de la adherencia al tratamiento con cisteamina y d) la promoción del autocuidado del paciente mediante programas de educación en la enfermedad. Todo ello conducirá, en una segunda fase, a la elaboración de un modelo de transición coordinado entre los servicios de pediatría y de adultos que contemple las necesidades específicas de la cistinosis.AbstractIntroductionCystinosis is a rare lysosomal systemic disease that mainly affects the kidney and the eye. Patients with cystinosis begin renal replacement therapy during the first decade of life in absence of treatment. Prognosis of cystinosis depends on early diagnosis, and prompt starting and good compliance with cysteamine treatment. Kidney disease progression, extra-renal complications and shorter life expectancy are more pronounced in those patients that do not follow treatment.The objective of this work was to elaborate recommendations for the comprehensive care of cystinosis and the facilitation of patient transition from paediatric to adult treatment, based on clinical experience. The goal is to reduce the impact of the disease, and to improve patient quality of life and prognosis.MethodsBibliographic research and consensus meetings among a multidisciplinary professional team of experts in the clinical practice, with cystinotic patients (T-CiS.bcn group) from 5 hospitals located in Barcelona.ResultsThis document gathers specific recommendations for diagnosis, treatment and multidisciplinary follow-up of cystinotic patients in the following areas: nephrology, dialysis, renal transplant, ophthalmology, endocrinology, neurology, laboratory, genetic counselling, nursing and pharmacy.ConclusionsA reference document for the comprehensive care of cystinosis represents a support tool for health professionals who take care of these patients. It is based on the following main pillars: a) a multi-disciplinary approach, b) appropriate disease monitoring and control of intracellular cystine levels in leukocytes, c) the importance of adherence to treatment with cysteamine, and d) the promotion of patient self-care by means of disease education programmes. All these recommendations will lead us, in a second phase, to create a coordinated transition model between paediatric and adult care services which will cover the specific needs of cystinosis.

Introduction Cystinosis is a rare systemic lysosomal storage disease that mainly affects the kidney and the eye. Renal replacement therapy is started in patients with cystinosis during the first decade of life in the absence of treatment. The prognosis of cystinosis depends on early diagnosis and the prompt start of and good compliance with cysteamine treatment. Kidney disease progression, extra-renal complications and shorter life expectancy are more pronounced in patients who do not adhere to treatment. Objective The aim of this work was to establish recommendations for the comprehensive care of cystinosis and facilitate patient transition from paediatric to adult medicine, based on clinical experience. The goal is to reduce the impact of the disease and improve prognosis and patient quality of life. Methods Bibliographic research and consensus meetings with a multidisciplinary professional team of clinical experts in cystinosis (T-CiS.bcn group) from 5 hospitals in Barcelona. Results This consensus document gathers specific recommendations for the diagnosis, treatment and multidisciplinary care of cystinotic patients in the following areas: nephrology, dialysis, kidney transplantation, ophthalmology, endocrinology, neurology, laboratory, genetic counselling, nursing and pharmacy. Conclusions Guidelines for the comprehensive care of cystinosis provide a support tool for health professionals who look after these patients. They are based on the following main pillars: (a) a multidisciplinary approach; (b) appropriate disease monitoring and control of white blood cell (WBC) cystine levels; (c) the importance of adherence to cysteamine treatment; and (d) the promotion of patient self-care by means of disease education programmes. All these recommendations will lead us, in a second phase, to create a coordinated model of transition from paediatric to adult care services which will cover the specific needs of cystinosis.

Background Scant information is available about how transplant centers are managing their use of quantitative molecular testing (QNAT) assays for active cytomegalovirus (CMV) infection monitoring in solid organ transplant (SOT) recipients. The current study was aimed at gathering information on current practices in the management of CMV infection across European centers in the era of molecular testing assays. Methods A questionnaire-based cross-sectional survey study was conducted by the European Study Group of Infections in Immunocompromised Hosts (ESGICH) of the Society of Clinical Microbiology and Infectious Diseases (ESCMID). The invitation and a weekly reminder with a personal link to an internet service provider (h t t p s://es.surveymonkey. com/) was sent to transplant physicians, transplant infectious diseases specialists, and clinical virologists working at 340 European transplant centers. Results Of the 1181 specialists surveyed, a total of 173 responded (14.8%): 73 transplant physicians, 57 transplant infectious diseases specialists, and 43 virologists from 173 institutions located at 23 different countries. The majority of centers used QNAT assays for active CMV infection monitoring. Most centers preferred commercially-available real-time polymerase chain reaction (RT-PCR) assays over laboratory-developed procedures for quantifying CMV DNA load in whole blood or plasma. Use of a wide variety of DNA extraction platforms and RT-PCR assays was reported. All programs used antiviral prophylaxis, preemptive therapy, or both, according to current guidelines. However, the centers used different criteria for starting preemptive antiviral treatment, for monitoring systemic CMV DNA load, and for requesting genotypic assays to detect emerging CMV-resistant variants. Conclusions Significant variation in CMV infection management in SOT recipients still remains across European centers in the era of molecular testing. International multicenter studies are required to achieve commutability of CMV testing and antiviral management procedures. This article is protected by copyright. All rights reserved.

Resumen Introducción: El aumento de la supervivencia de los pacientes con cistinosis y la propia complejidad de la enfermedad explican la necesidad de implementar un proceso de transición guiada desde la medicina pediátrica hasta la del adulto, que permita garantizar el continuum asistencial y posibilite el empoderamiento del paciente desde el cuidado tutelado al autocuidado. Métodos: Revisión bibliográfica, opinión de expertos, encuestas anónimas a pacientes, familiares y asociaciones. Resultados: Elaboración de un documento de transición coordinada, con propuestas concretas por especialidades y de mejora de la adherencia terapéutica y del autocuidado del paciente. El nefrólogo desempeña un papel clave en la transición en la cistinosis debido a la afectación renal que domina la patología y porque la mayoría de los pacientes han recibido un trasplante renal antes de la edad adulta. Conclusión: Se presenta un documento que establece unas recomendaciones y un cronograma para guiar la transición de los adolescentes y adultos jóvenes con cistinosis en nuestro ámbito. Abstract Introduction: Improved outcome and longer life-expectancy in patients with cystinosis, and disease complexity itself, justify planning a guided-transition of affected patients from Pediatrics to adult medicine. The aims of the process are to guarantee the continuum of care and patient empowerment, moving from guardian-care to self-care. Methods: review of articles, expert opinion and anonymous surveys of patients, relatives and patient advocacy groups. Results: elaboration a new document to support and coordinate the transition of patients with cystinosis providing specific proposals in a variety of medical fields, and adherence promotion. Nephrologists play a key role in transition due the fact that most cystinotic patients suffer severe chronic kidney disease, and need kidney transplantation before adulthood. Conclusion: we present a document providing recommendations and suggesting a chronogram to help the process of transition of adolescents and young adults with cystinosis in our area.

Improved outcome and longer life-expectancy in patients with cystinosis, and disease complexity itself, justify planning a guided-transition of affected patients from Pediatrics to adult medicine. The aims of the process are to guarantee the continuum of care and patient empowerment, moving from guardian-care to self-care.review of articles, expert opinion and anonymous surveys of patients, relatives and patient advocacy groups.elaboration a new document to support and coordinate the transition of patients with cystinosis providing specific proposals in a variety of medical fields, and adherence promotion. Nephrologists play a key role in transition due the fact that most cystinotic patients suffer severe chronic kidney disease, and need kidney transplantation before adulthood.we present a document providing recommendations and suggesting a chronogram to help the process of transition of adolescents and young adults with cystinosis in our area.

Resumen: Introducción: Se ha descrito una asociación entre neumonía y glomerulonefritis aguda iniciándose ambos procesos de forma simultánea. Pacientes y métodos: Estudio retrospectivo de 6 pacientes ingresados en nuestro centro entre los años 2001 y 2010 con glomerulonefritis aguda asociada a neumonía concomitante, con frotis y cultivo amigdalar negativos y en ausencia de infección cutánea o episodio de neumonía anterior. Resultados: La media de edad de los pacientes al ingreso fue de 5,9 años sin diferencia de sexos. El diagnóstico de neumonía se realizó en el momento del ingreso, coincidiendo con la sintomatología nefrológica. Los síntomas más frecuentes fueron fiebre y hematuria macroscópica. Todos tuvieron un C3 (fracción C3 del complemento) bajo. En 5 de ellos se encontró una elevación significativa en la cifra de antiestreptolisinas (ASLO). En la mayoría de los casos la afectación renal fue leve a excepción de un caso de insuficiencia renal aguda con creatinina inicial de 2,77 mg/dl y filtrado glomerular de 27 ml/min/1,73 m2 y 2 casos con proteinuria en rango nefrótico. Todos evolucionaron hacia la curación sin tratamiento o con mínimo tratamiento diurético o hipotensor, además de tratamiento antibiótico adecuado para su proceso neumónico, con resolución de la clínica entre 7–10 días y normalización del C3 en un período inferior a los 4 meses. Conclusiones: Aunque descrita con mucho menos frecuencia, existe asociación entre glomerulonefritis aguda y presencia concomitante de neumonía. Las ASLO, en nuestra serie, no son específicas de infección por Streptococcus pyogenes. El pronóstico respiratorio y renal fue favorable en todos los casos. Abstract: Introduction: There is an association between pneumonia and acute glomerulonephritis. Both processes start simultaneously. Patients and methods: A retrospective study of 6 patients admitted to our centre between 2001 and 2010 with acute glomeruolonephritis associated with pneumonia. The result of the smear and tonsil culture was negative and there was an absence of cutaneous infection. Results: The average age of the patients on admission was 5.9 years with no differences in sex. The diagnosis for pneumonia was made at the time of admission, coinciding with the glomerular symptoms. The most frequent symptoms were fever and macrohaematuria. All had low levels of C3. A significant increase in ASLO was found in 5 cases. The majority of the cases had mild symptoms with the exception of one case of acute renal failure with an initial creatinine of 2.77 mg/dL and glomerular filtration rate of 27 ml/min/1.73 m2, and two cases with proteinuria in the nephrotic range. All of them progressed satisfactorily without treatment or with minimum diuretic or hypotensive treatment in addition to the appropriate antibiotic treatment with clinical resolution in 7 to 10 days, and C3 returning to normal within a period of less than 4 months. Conclusions: There is an association between acute glomerulonephritis and pneumonia, although it is very uncommon. The ASLOs in our series are not specific for Streptoccocus. pyogenes infection. The respiratory and renal prognosis was favourable in all cases.

Resumen Introduccion Durante el ejercicio se produce una elevacion fisiologica de la presion arterial. Algunos sujetos previamente normotensos presentan grandes incrementos con el ejercicio, lo que podria ser un factor pronostico adverso. Casos clinicos Dos varones de 15 y 16 anos, sin antecedentes personales de interes, remitidos por perdida de conciencia de minutos de duracion, en el primer caso, con recuperacion espontanea mientras entrenaba al baloncesto y, en el segundo, con cefalea importante desencadenada durante un partido de futbol. Se constataron cifras de presion > P 99 en ambos. La exploracion fisica era normal. Se realizaron determinaciones en condiciones de reposo, que mostraron intervalos de normalidad. Los hallazgos analiticos, funcion renal, metabolica y hormonal fueron normales. No se encontraron anomalias en la eco-Doppler. Se descarto afeccion de organos diana. La monitorizacion ambulatoria de la presion arterial mostro maximos de presion > P 99 coincidiendo con la realizacion de ejercicio; las demas lecturas estaban dentro de la normalidad. Se suprimio el ejercicio fisico intenso sin requerir otro tipo de medidas. Conclusiones Valorar la aparicion de hipertension arterial en adolescentes que practican deporte, introducir la monitorizacion ambulatoria de la presion arterial como cribado en revisiones medico-deportivas y suprimir el esfuerzo como unica medida terapeutica.

Objetivos: Nuestros objetivos han sido determinar aspectos epidemiológicos, formas clínicas y analíticas de los pacientes con síndrome hemolítico-urémico (SHU) tratados en nuestros centros, así como describir las complicaciones renales y extrarrenales, el tipo de tratamiento requerido y relacionar nuestros casos con la bibliografía actual. Métodos: Efectuamos una revisión retrospectiva de la historia clínica, analítica y estudios de diagnóstico por imagen de los pacientes con diagnóstico de SHU, atendidos desde enero de 1974 hasta agosto de 2007, es decir, durante los últimos 33 años. Resultados: Un total de 58 pacientes fueron incluidos en nuestro estudio, con una edad media de 2 años y 11 meses; de ellos, estaban ingresados en verano 34 niños. Destaca la presencia de hipocomplementemia familiar en un caso. Con SHU típico (diarrea positivo [D+]) aparecieron 48 casos en los que se aislaron Salmonella enteritidis y Escherichia coli 0157:H7. Con SHU atípico (diarrea negativo [D–]) se contabilizaron 7 casos, y entre las causas destacaban procesos respiratorios de etiología estreptocócica. El recuento plaquetario fue normal en un caso. De los procedimientos empleados cabe destacar que se realizó biopsia renal en 18 pacientes y diálisis peritoneal en 25 casos. Entre las complicaciones se encontraron: en el grupo D+, un caso de necrosis cortical que requirió trasplante renal y en el grupo D–, un paciente con SHU familiar, hipocomplementemia e hipertensión arterial maligna. Conclusiones: Hemos llegado a las siguientes conclusiones: a) la enfermedad presenta una baja incidencia en nuestro medio; b) se ha detectado un caso con plaquetas normales; c) un paciente presentó SHU familiar recurrente con hipocomplementemia e hipertensión arterial grave, y d) indicadores como la leucocitosis, la oligoanuria o la hipocomplementemia no influyeron en el curso de la enfermedad. : Objectives: Our objectives were to determine epidemiology, clinical and laboratory characteristics of patients with haemolytic-uraemic syndrome (HUS) treated in our centre, to describe renal and extra-renal complications and the treatment required and to relate our findings with the existing bibliography. Methods: We performed a 33-year retrospective review. We included all patients diagnosed with HUS and monitored in our hospital from January 1974 to August 2007. Clinical histories and imaging studies were reviewed. Results: A total of 58 patients were included in our study, with a mean age of 2 years 11 months and most of them were admitted to hospital in summer.Familial hypocomplementaemia was present in one case. A total of 48 patients presented with typical HUS (with diarrhoea D+ HUS). Salmonella enteritidis and Escherichia coli O157:H7 were isolated from those patients.While 7 cases presented with atypical HUS (without diarrhoea, D– HUS), most of them associated with a respiratory tract infection due to Streptococcus. In one case platelet count was normal. Kidney biopsy was performed in 18 patients and 25 cases underwent peritoneal dialysis. As regards complications, one child with D+ HUS experienced renal cortical necrosis and required kidney transplant, while in the D-HUS group, the patient with familial hypocomplementaemia had severe hypertension. Conclusions: a) Incidence of HUS in our environment is low. b) HUS can be present even with a normal platelet count. c) We had one case of HUS in a patient with familial hypocomplementaemia who experienced severe hypertension. d) In our group of patients, the course of the disease was not influenced by the white blood cell counts, decreased diuresis or hypocomplementaemia.

Some guidelines recommend an early voiding cystourethrography (VCUG) after endoscopic treatment of vesicoureteral reflux (VUR), but there's no consensus if it's necessary a long-term follow-up in these patients. The aim of our study is analyze if it's necessary a delayed VCUG after initial successful treatment with Dx/HA.We have reviewed all medical charts of patients that underwent Dx/HA treatment from 2006 to 2010. We have selected patients with initial successful treatment and more than 3 years of radiological and clinical follow-up. We have analyzed late clinical and radiological outcomes.One hundred and sixty children with 228 refluxing ureters underwent Dx/HA endoscopic treatment with a mean follow-up of 52.13 months. Early VCUG was performed in 215 ureters with an initial successful rate of 84.1%. The group of study was 94/215 ureters with more than 3 years of follow-up with a delayed VCUG. VUR was still resolved in 79,8% of the ureters. Clinical success rate was 91.7%. The incidence of febrile urinary tract infection in those patients with cured VUR and those with a relapsed VUR was 8 and 15%, respectively; but there were no significant differences. We have not found any variable related with relapsed VUR except those ureters that initially received 2 injections (P.05).If our objective in the treatment of VUR is to reduce the incidence of febrile urinary tract infection it is not necessary to perform a delayed VCUG even though the long-term radiological outcomes is worse than clinical outcome.

La púrpura de Schönlein-Henoch es una vasculitis sistémica por hipersensibilidad mediada por inmunoglobulina A (IgA) que afecta casi exclusivamente a la edad pediátrica. La afectación renal que puede presentarse desde su inició o bien posteriormente es la que condiciona la mayoría de las veces el pronóstico a largo plazo.Con el objetivo de evaluar la afectación renal se procedió al estudio retrospectivo de 764 pacientes afectados de púrpura de Schönlein-Henoch. De ellos, 153 (20%) presentaron afectación renal siendo la forma de presentación más frecuente la hematuria/proteinuria no nefrótica (67 pacientes) seguida de la hematuria aislada (41 pacientes).Se efectuó biopsia renal en un total de 39 pacientes siendo el hallazgo anatomopatológico más frecuente la proliferación mesangial difusa con depósitos de IgA y menos de un 50 % de semilunas.Los tratamientos efectuados no fueron determinantes por lo que respecta a la evolución final. Tres pacientes (2 %) evolucionaron a insuficiencia renal terminal precisando trasplante renal. Un paciente falleció. Hay que destacar la reaparición de la enfermedad en dos de los pacientes trasplantados.Se remarca la evolución generalmente benigna de la nefropatía y la necesidad de un seguimiento a largo plazo de la misma. : Schönlein-Henoch purpura is a systemic vasculitis due to IgA-mediated hypersensitivity, almost exclusively affecting the pediatric age group. Long-term prognosis is mainly conditioned by renal involvement, which can appear at onset or during the course of the disease.To evaluate renal involvement, 764 patients with Schönlein-Henoch purpura were retrospectively reviewed. Of these, 153 (20 %) had renal involvement, the most frequent form of presentation being non-nephrotic hematuria/proteinuria (67 patients) followed by isolated hematuria (41 patients).Renal biopsy was performed in 39 patients, and the most frequent pathological findings were diffuse mesangial proliferation with IgA deposits and less than 50 % of crescentic glomeruli.Treatment was not curative. Three patients (2 %) progressed to end-stage renal failure and required renal transplantation. One patient died. Notably, two of the patients who underwent renal transplantation had disease recurrence.We stress that nephropathy is usually benign and that it requires long-term follow-up.

AIMS OF THE STUDY: To evaluate the outcome of kidney transplantation in children with low weight. METHODS: Retrospective review of the medical records of patients weighing 11 kg or less that received kidney transplantation between 2001 and 2013 were retrospectively reviewed. RESULTS: Fifty-nine kidney transplantations were performed in pediatric patients in our center, 12 of them were performed in patients weighing 11 kg or less (20%). The mean age of the recipient at the time of transplantation was 2 years (1-3.5); the mean weight was 9.4 ± 1.1 kg (8.3-11). The etiology of kidney failure was malformative in 42% of patients, inherited in 33%, glomerular in 8% and oiler etiologies in 17% of the patients. Two patients did not receive replacement therapy before transplantation (16.7%), nine received peritoneal dialysis (75%) and one of them hemodialysis (8.3%). Eleven of the grafts were from cadaveric donor (91.7%) and one of them from a living donor (8.3%). The mean donor age was 10 years (0.5-29). There was one case of acute graft thrombosis (8.3%) and one case of eventration requiring reoperation; there were no other major complications. Mean follow-up was 59 months (4-130). Overall survival (OS) was 100% at 1 year and 91.7% at 5 years. There was one death in a patient with mitochondrial disease with a functioning graft. Graft survival (GS) was 92% at 1 year and 75% at 5 years. CONCLUSION: Kidney transplantation is the treatment of choice for end-stage kidney failure in the young child. It provides good results in terms of patient and graft survival.

Objetivo: Estudio retrospectivo observacional multicéntrico de los pacientes trasplantados renales pediátricos, para conocer la situación actual frente al citomegalovirus (CMV), antes de participar en un ensayo clínico internacional de profilaxis durante 6 meses. Material y métodos: Se incluyen 239 pacientes menores de 19 años, procedentes de 5 centros entre 2005-2009, con seguimiento de 1 año. Resultados: La serología frente al CMV era negativa en 54 % de los receptores y 34,7 % de los donantes. Sesenta pacientes (25,1 %) fueron considerados de alto riesgo [Donante (D)+/Receptor (R)-] para infección por CMV. El 80,8 % realizó algún tipo de profilaxis, incluyendo todos los pacientes de alto riesgo, un tiempo medio de 65,5 días. La incidencia de positivización de CMV fue del 24,26 % (58 pacientes de los 239 trasplantados), con una incidencia de enfermedad del 6,7 %. La infección por CMV se asociaba con el estatus serológico (D/R) (p < 0,001), con la seropositividad del donante (p < 0,001) y con un tiempo de profilaxis < 20 días (p < 0,05). No hubo ningún caso de éxitus o pérdida del injerto secundaria a la infección, ni de resistencia al tratamiento. Conclusiones: La principal estrategia preventiva frente al CMV en el trasplante renal pediátrico en nuestro país es la quimioprofilaxis (81 %), con una incidencia de CMV del 24 % y de enfermedad del 6,7%, sin graves efectos directos ni indirectos en el primer año postrasplante. Su incidencia está relacionada, fundamentalmente, con el estatus serológico D/R y con la seropositividad del donante. Objective: An observational retrospective multicentre study of kidney transplants in paediatric patients was performed to evaluate the current situation of cytomegalovirus (CMV) in this population, before our participation in an international clinical trial of prophylaxis for 6 months. Material and method: Our study included 239 patients aged

An observational retrospective multicentre study of kidney transplants in paediatric patients was performed to evaluate the current situation of cytomegalovirus (CMV) in this population, before our participation in an international clinical trial of prophylaxis for 6 months.Our study included 239 patients aged19 years, from 5 Spanish centres between 2005-2009, with 1 year of follow-up.Pretransplant CMV serology was negative in 54% of recipients and 34.7% of donors. Sixty patients (25.1%) were considered at high risk (D+/R-) for CMV infection. Prophylaxis was used in 80.8% of recipients, including all high-risk patients, for an average time of 65.5 days. CMV viraemia occurred in 24.26% (58 cases among 239 patients), and disease in 6.7%. CMV infection was associated with serological status (D/R) (P.001), positive serology of the donor (P.001) and duration of prophylaxis20 days (P.05). There were no cases of patient or graft loss secondary to infection, nor resistance to treatment.The main preventative strategy against CMV in paediatric renal transplantation in our country is chemical prophylaxis (81%), with an incidence of infection and disease of 24% and 6.7%, respectively. There were no serious direct or indirect effects in the first year post-transplant. The incidence is mainly linked with serological D/R and positive donor status.

There is an association between pneumonia and acute glomerulonephritis. Both processes start simultaneously.A retrospective study of 6 patients admitted to our centre between 2001 and 2010 with acute glomeruolonephritis associated with pneumonia. The result of the smear and tonsil culture was negative and there was an absence of cutaneous infection.The average age of the patients on admission was 5.9 years with no differences in sex. The diagnosis for pneumonia was made at the time of admission, coinciding with the glomerular symptoms. The most frequent symptoms were fever and macrohaematuria. All had low levels of C3. A significant increase in ASLO was found in 5 cases. The majority of the cases had mild symptoms with the exception of one case of acute renal failure with an initial creatinine of 2.77mg/dL and glomerular filtration rate of 27ml/min/1.73m(2), and two cases with proteinuria in the nephrotic range. All of them progressed satisfactorily without treatment or with minimum diuretic or hypotensive treatment in addition to the appropriate antibiotic treatment with clinical resolution in 7 to 10 days, and C3 returning to normal within a period of less than 4 months.There is an association between acute glomerulonephritis and pneumonia, although it is very uncommon. The ASLOs in our series are not specific for Streptoccocus. pyogenes infection. The respiratory and renal prognosis was favourable in all cases.

Our objectives were to determine epidemiology, clinical and laboratory characteristics of patients with haemolytic-uraemic syndrome (HUS) treated in our centre, to describe renal and extra-renal complications and the treatment required and to relate our findings with the existing bibliography.We performed a 33-year retrospective review. We included all patients diagnosed with HUS and monitored in our hospital from January 1974 to August 2007. Clinical histories and imaging studies were reviewed.A total of 58 patients were included in our study, with a mean age of 2 years 11 months and most of them were admitted to hospital in summer. Familial hypocomplementaemia was present in one case. A total of 48 patients presented with typical HUS (with diarrhoea D+ HUS). Salmonella enteritidis and Escherichia coli O157:H7 were isolated from those patients. While 7 cases presented with atypical HUS (without diarrhoea, D- HUS), most of them associated with a respiratory tract infection due to Streptococcus. In one case platelet count was normal. Kidney biopsy was performed in 18 patients and 25 cases underwent peritoneal dialysis. As regards complications, one child with D+ HUS experienced renal cortical necrosis and required kidney transplant, while in the D-HUS group, the patient with familial hypocomplementaemia had severe hypertension.a) Incidence of HUS in our environment is low. b) HUS can be present even with a normal platelet count. c) We had one case of HUS in a patient with familial hypocomplementaemia who experienced severe hypertension. d) In our group of patients, the course of the disease was not influenced by the white blood cell counts, decreased diuresis or hypocomplementaemia.

Objetivos Revision de los pacientes con enfermedad de Berger, su forma de presentacion, el tratamiento realizado, los hallazgos anatomopatologicos con respecto al tratamiento y la evolucion clinica a largo plazo. Pacientes y metodos Pacientes con clinica sugestiva de enfermedad de Berger y confirmacion anatomopatologica. La clasificacion histologica se realizo mediante los criterios de Haas. Resultados Se revisaron retrospectivamente 35 pacientes, 14 ninas y 21 ninos, diagnosticados de enfermedad de Berger mediante biopsia renal. La edad media fue de 9,4 anos (5-16). La clinica de inicio mas frecuente fue la hematuria macroscopica seguida de hematuria macroscopica y proteinuria de rango no nefrotico. Presentaron al inicio insuficiencia renal 9 pacientes, 7 recuperaron la funcion. Las cifras de IgA fue elevada en 9 pacientes. Presentaron proteinuria 22 casos (10 en rango nefrotico). El tiempo de realizacion de la biopsia desde el inicio fue variable: 17 durante el primer ano de evolucion y 18 despues del ano. Anatomopatologicamente los resultados fueron los siguientes: 8 de clase 1, 18 de clase 3 y 4 clase 4; no se pudieron revisar las biopsias de 5 pacientes. 27 pacientes han sido dados de alta por edad (18 anos): 13 asintomaticos, 5 con microhematuria (mH) persistente, 2 con mH y brotes de hematuria macroscopica, 5 con mH y proteinuria, 1 proteinuria aislada, 1 insuficiencia renal y mH/proteinuria. Siguen control 8 pacientes, 7 con funcion renal normal aunque 3 de ellos, con diversos grados de proteinuria (2 de rango nefrotico). Solo 1 paciente ha presentado insuficiencia renal cronica terminal (IRCT) y se ha trasplantado en edad pediatrica. Conclusiones 1 . La enfermedad de Berger es una causa frecuente de hematuria macroscopica en la infancia y es infradiagnosticada al requerir para su diagnostico definitivo la confirmacion anatomopatologica. 2 . A pesar de su pronostico favorable durante la infancia, puede existir evolucion a IRCT (en nuestra serie 1 caso); se desconoce la verdadera evolucion de estos pacientes durante la edad adulta.

Schönlein-Henoch purpura is a systemic vasculitis due to IgA-mediated hypersensitivity, almost exclusively affecting the pediatric age group. Long-term prognosis is mainly conditioned by renal involvement, which can appear at onset or during the course of the disease. To evaluate renal involvement, 764 patients with Schönlein-Henoch purpura were retrospectively reviewed. Of these, 153 (20 %) had renal involvement, the most frequent form of presentation being non-nephrotic hematuria/proteinuria (67 patients) followed by isolated hematuria (41 patients). Renal biopsy was performed in 39 patients, and the most frequent pathological findings were diffuse mesangial proliferation with IgA deposits and less than 50 % of crescentic glomeruli. Treatment was not curative. Three patients (2 %) progressed to end-stage renal failure and required renal transplantation. One patient died. Notably, two of the patients who underwent renal transplantation had disease recurrence. We stress that nephropathy is usually benign and that it requires long-term follow-up.

Objetivos: 1. Describir la epidemiología, formas clínicas y analíticas de los pacientes. 2. Describir las complicaciones tanto renales como extrarrenales y el tipo de tratamiento requerido. 3. Comparar y relacionar nuestros datos con la bibliografía actual. Material y métodos: Estudio observacional, longitudinal y descriptivo en todos los pacientes afectados por el síndrome hemolítico-urémico (SHU), 55, desde enero de 1974 hasta junio de 2006. Resultados: La edad media fue de 2 años y 11 meses. Destaca la presencia de hipocomplementemia familiar en un caso. Ingresados en verano, 34 niños. SHU típico (positivo a diarrea) 48 casos, aislándose salmonella enteritidis y E. coli 0157:H7.SHU atípico (negativo a diarrea) 7 casos, destacando 2 procesos respiratorios de etiología estreptocócica. Recuento de plaquetas normales en un caso. Procedimientos: Biopsia renal: 18 pacientes. Diálisis peritoneal: 25 casos. Complicaciones: en el grupo D positivo un caso de necrosis cortical que requirió trasplante renal, otro niño ingresó cadáver y la necropsia confirmó SHU. En el grupo D negativo un paciente con SHU familiar, hipocomplementemia e hipertensión arterial maligna. Conclusiones: 1. Baja incidencia en nuestro medio. 2. Un paciente con plaquetas normales. 3. Un paciente afectado de SHU familiar, recurrente y con hipocomplementemia. 4. Indicadores como la leucocitosis, oligoanuria, hipocomplementemia no influyeron en el curso de la enfermedad. 5. Mortalidad en un paciente.

Accessible online at: www.karger.com/journals/nef Dear Sir, The presence of circulating antigliadine antibodies is frequent in IgA nephropathy [1–5]. However, any sort of relationship between celiac disease and nephrotic syndrome has not been described in the medical literature until now. It is this association that we bring forth. Five patients (3 boys/2 girls) out of a total of 251 patients with nephrotic syndrome have been followed in our Department. All 5 presented both nephrotic syndrome and celiac disease and were diagnosed during the last 5 years. The mean age of onset was 4.5 years. Celiac disease was first diagnosed in 4 patients previous to the nephrotic syndrome. Celiac markers were looked for in the fifth patient after the first episode of nephrotic syndrome. One of the 5 patients presented both nephrotic syndrome and diabetes mellitus. The diagnosis of nephrotic syndrome was made according to clinical and analytical findings, whereas the diagnosis of celiac disease involved clinical and laboratory findings (chronic diarrhea, positive antibodies) and pathological confirmation after bowel biopsy. Kidney biopsies were done in 2 patients: 1 showed minimal change lesions while the other presented IgM infiltrates. Regarding clinical evolution, 1 of the patients resulted as corticosensitive and 3 of them were corticoid dependent, requiring cyclophosphamide and one of them levamisole. The fifth patient was lost during follow-up. After a review of the literature, no reference has been found of the association between celiac disease and nephrotic syndrome. Mignot et al. [6] looked for a relationship between HLA DQA1 and HLA DQB1 in three groups of patients with, respectively, nephrotic syndrome, diabetes mellitus and celiac disease. No genetic relationship was found. On the other hand, the prevalence of celiac disease in the general population is approximately 3 per thousand, whereas nephrotic syndrome in the pediatric population is around 2–7 per 100,000. In our series, the incidence of celiac disease in patients diagnosed with nephrotic syndrome amounts to 2%, without any explanation of the cause. With this note, we wish to underline the presence of celiac disease in nephrotic syndrome and recommend the inclusion of celiac disease screening in these children. References

ResumenIntroducciónEl aumento de la supervivencia de los pacientes con cistinosis y la propia complejidad de la enfermedad explican la necesidad de implementar un proceso de transición guiada desde la medicina pediátrica hasta la del adulto, que permita garantizar el continuum asistencial y posibilite el empoderamiento del paciente desde el cuidado tutelado al autocuidado.MétodosRevisión bibliográfica, opinión de expertos, encuestas anónimas a pacientes, familiares y asociaciones.ResultadosElaboración de un documento de transición coordinada, con propuestas concretas por especialidades y de mejora de la adherencia terapéutica y del autocuidado del paciente. El nefrólogo desempeña un papel clave en la transición en la cistinosis debido a la afectación renal que domina la patología y porque la mayoría de los pacientes han recibido un trasplante renal antes de la edad adulta.ConclusiónSe presenta un documento que establece unas recomendaciones y un cronograma para guiar la transición de los adolescentes y adultos jóvenes con cistinosis en nuestro ámbito.AbstractIntroductionImproved outcome and longer life-expectancy in patients with cystinosis, and disease complexity itself, justify planning a guided-transition of affected patients from Pediatrics to adult medicine. The aims of the process are to guarantee the continuum of care and patient empowerment, moving from guardian-care to self-care.Methodsreview of articles, expert opinion and anonymous surveys of patients, relatives and patient advocacy groups.Resultselaboration a new document to support and coordinate the transition of patients with cystinosis providing specific proposals in a variety of medical fields, and adherence promotion. Nephrologists play a key role in transition due the fact that most cystinotic patients suffer severe chronic kidney disease, and need kidney transplantation before adulthood.Conclusionwe present a document providing recommendations and suggesting a chronogram to help the process of transition of adolescents and young adults with cystinosis in our area.

To evaluate the outcome of kidney transplantation in children with low weight.Retrospective review of the medical records of patients weighing 11 kg or less that received kidney transplantation between 2001 and 2013 were retrospectively reviewed.Fifty-nine kidney transplantations were performed in pediatric patients in our center, 12 of them were performed in patients weighing 11 kg or less (20%). The mean age of the recipient at the time of transplantation was 2 years (1-3.5); the mean weight was 9.4 ± 1.1 kg (8.3-11). The etiology of kidney failure was malformative in 42% of patients, inherited in 33%, glomerular in 8% and oiler etiologies in 17% of the patients. Two patients did not receive replacement therapy before transplantation (16.7%), nine received peritoneal dialysis (75%) and one of them hemodialysis (8.3%). Eleven of the grafts were from cadaveric donor (91.7%) and one of them from a living donor (8.3%). The mean donor age was 10 years (0.5-29). There was one case of acute graft thrombosis (8.3%) and one case of eventration requiring reoperation; there were no other major complications. Mean follow-up was 59 months (4-130). Overall survival (OS) was 100% at 1 year and 91.7% at 5 years. There was one death in a patient with mitochondrial disease with a functioning graft. Graft survival (GS) was 92% at 1 year and 75% at 5 years.Kidney transplantation is the treatment of choice for end-stage kidney failure in the young child. It provides good results in terms of patient and graft survival.