Unidad de Nefrología Pediátrica del Hospital Universitario 12 de Octubre / Pediatric Nephrology Unit of the University Hospital 12 de Octubre

La Nefrología Pediátrica es una subespecialidad pediátrica reconocida internacionalmente desde el año 1967 y es una de las áreas de capacitación en vías de acreditación oficial (en el momento actual la acreditación se obtiene a través de la Asociación Española de Nefrología Pediátrica creada en el año 1973). La Unidad de Nefrología Pediátrica del 12 de Octubre está catalogada como nivel II (atiende toda la patología renal pediátrica y la insuficiencia renal terminal mediante diálisis). Cumple todos los requisitos exigidos por la Sociedad de Nefrología Pediátrica para la formación de los especialistas en Pediatría y sus áreas específicas, tanto para la formación general de Pediatría como para la capacitación en Nefrología Pediátrica (subespecialidad pediátrica) contemplando una rotación externa. LaUnidad dispone de consultas externas, planta de hospitalización, hospital de día y sala de intervencionismo compartidas estas dos últimas con otras especialidades pediátricas. En el momento actual hay dos facultativos especialistas de área con capacitación específica en nefrología pediátrica. La cartera de servicios incluye toda la patología renal desde el momento de la concepción hasta los 16 años con excepción del manejo de la enfermedad renal crónica que requiere terapia sustitutiva. Se imparte docencia pregrado a los estudiantes de la Universidad Complutense de Madrid y se colabora activamente en las sesiones y formación de los residentes de la especialidad de Pediatría. La Unidad de Nefrología Pediátrica se mantiene activa con líneas de investigación propias (algunas financiadas) y con colaboración en trabajos, registros y ensayos multicéntricos que producen una actividad cientifica nada despreciable (AU)

Pediatric Nephrology is a pediatric subspecialty that has been internationally known since 1967 and is one of the training areas undergoing official accreditation (at present, accreditation is obtained through the Spanish Association of Pediatric Nephrology created in the year 1973). The Pediatric Nephrology Unit of the 12 de Octubre is listed as level II (it attends to all the pediatric renal conditions and end-stage kidney failure by dialysis). It complies with all the requirements of the Pediatric Nephrology Society for the training of specialists in Pediatrics and their specific areas, both for general Pediatrics training as for training in Pediatric Nephrology (Pediatric subspecialty) contemplating an external rotation. The Unit has external consultations, hospitalization ward, day hospital, and interventionism ward, sharing the latter two with other pediatric specialties. At present, there are two area medical specialists with specific training in pediatric nephrology. The services portfolio includes all the renal conditions from the moment of the conception up to 16 years except for management of chronic kidney disease that requires replacement therapy. Pre-graduate teaching for Complutense University of Madrid students is given and they collaborate actively in the sessions and training of the Pediatric specialty residents. The Pediatric Nephrology Unit remains active with their own research lines (some financed) and with collaboration in multicenter trials, registries, works and that produce a significant scientific activity (AU)

Ascitis urinosa en el periodo neonatal secundaria a válvulas de uretra posterior / Neonatal urinary ascites caused by posterior urethral valves

La causa más frecuente de obstrucción del tracto urinario inferior en los niños son las válvulas de uretra posterior. A pesar de que se relaciona con una importante morbilidad, su pronóstico ha mejorado en los últimos años debido a un diagnóstico de sospecha más precoz, al detectar oligohidramnios, dilatación vesical e hidronefrosis durante el seguimiento ecográfico prenatal. El propósito de esta nota clínica es describir la forma de presentación, el tratamiento proporcionado y la respuesta a éste, en un caso recientemente diagnosticado en nuestro centro (AU)

The most common cause of lower urinary obstruccion in male infants is posterior urethral valves. Although it is related to significant morbidity, prognosis has improved in recent years due to an earlier diagnosis when detecting oligohydramnios, bladder dilatation and hydronephrosis during the prenatal ultrasound evaluation. The aim of this note is to describe the presentation, treatment provided and the clinical course, in a case recently diagnosed in our center (AU)

Síndrome hemolítico-urémico: afectación renal a largo plazo / Hemolytic uremic syndrome: long term renal injury

Introducción: El síndrome hemolítico urémico es la causa más frecuente de insuficiencia renal aguda no prerrenal en edad pediátrica y suele ser secundario a diarrea aguda infecciosa, generalmente por Escherichia coli productor de verotoxina. Se caracteriza por fracaso renal agudo, anemia hemolítica y trombocitopenia. Puede producir secuelas renales a largo plazo y llevar a insuficiencia renal terminal. Material y métodos: El objetivo de este estudio retrospectivo es describir las secuelas renales y analizar los factores pronósticos de las mismas en los pacientes pediátricos atendidos en un hospital terciario español en los últimos 28 años. Resultados: Han sido atendidos 43 niños con esta patología, 38 de ellos con antecedentes de diarrea. Entre los seguidos más de 3 meses, en el momento de la última revisión (media 6,6 años, DE 5 años de seguimiento), un 21,6% (8/37) presentaba deterioro del filtrado glomerular. Incluyendo proteinuria e hipertensión arterial, encontramos hasta un 35,1% (13/37) de enfermedad renal crónica. Un 8,1% (3/37) entró en insuficiencia renal terminal. Encontramos como factores pronósticos de lesión renal a largo plazo en regresión logística univariante: hipertensión durante el ingreso, mayor leucocitosis, mayor duración del ingreso, mayor duración de la anuria y menor filtrado glomerular al alta. El análisis multivariante muestra asociación con la duración de la anuria. Conclusiones: Aproximadamente, una tercera parte de nuestros casos de síndrome hemolítico-urémico desarrollaron a largo plazo afectación renal en grado variable. Una mayor y más prolongada afectación de la función renal inicial se asocia en nuestro estudio con una mayor probabilidad de deterioro renal en su evolución posterior (AU)

Introduction: Hemolytic uremic syndrome (HUS) is the most frequent cause of non-pre-renal acute renal failure in pediatrics and it is usually secondary to acute infectious diarrhea, generally due to Shiga-toxin producing E. coli. It is characterized by acute renal failure, hemolytic anemia and thrombocytopenia. It can lead to renal sequels in the long term and to end-stage renal disease. Methods: The aim of this retrospective study is to describe and analyze renal sequels and their prognostic factors in pediatric patients suffering from HUS in a Spanish tertiary hospital during the last 28 years. Results: 43 children with this condition were admitted, with 38 of them having presented with diarrhea previously. Among those with a follow-up longer than 3 months, 21.6% (8/37) had a reduction in glomerular filtration rate (GFR) in the last visit. Including proteinuria and hypertension, we found up to 35.1% (13/37) prevalence of chronic kidney disease, with 8.1% (3/37) of the patients with end-stage renal disease. We found the following to be prognostic factors for renal injury in the long term in a univariate logistic regression: hypertension, a longer stay in the hospital, more prolonged anuria, more severe leukocytosis and lower GFR at discharge. Multivariate logistic regression showed an association with time in anuria. Conclusions: Approximately one third of our cases of hemolytic uremic syndrome developed some degree of chronic kidney disease in the long term. A deeper and longer initial renal disfunction is associated with a higher probability of subsequent renal problems (AU)

Evolución a largo plazo de la nefritis lúpica de inicio en la edad pediátrica / Long-term outcome of paediatric lupus nephritis

Introducción: El riñón es uno de los órganos más frecuentemente afectados por el lupus eritematoso sistémico. Recientemente se ha indicado que el pronóstico renal en esta enfermedad ha mejorado con un diagnóstico y tratamiento específico más precoz e intenso. A menudo, los estudios en edad pediátrica carecen de un tiempo de seguimiento suficiente para conocer la evolución a largo plazo. Pacientes y métodos: Estudio descriptivo retrospectivo desde 1986 hasta 2009 basado en la revisión de historias clínicas de pacientes diagnosticados de nefritis lúpica en la sección de Nefrología Infantil de un hospital terciario. Se incluye a pacientes menores de 18 años de edad al diagnóstico y se excluye a pacientes con seguimiento menor de 3 meses o datos incompletos. Recogida y descripción evolutiva de variables clínicas, analíticas de función renal y actividad de la enfermedad así como anatomía patológica y tratamientos empleados. Análisis de variables de riesgo para existencia de afectación renal en el último control. Resultados: Recuperamos datos de 16 pacientes (3 varones y 13 mujeres) con una edad al inicio del lupus eritematoso sistémico de 10,6±2,9 años y al diagnóstico de nefritis lúpica de 12,6±3,5 años. Se obtuvo biopsia renal en 14 de ellos, en 11 de estos (78,6%) 3 meses o menos tras el diagnóstico. En 9 casos (64,3%) se encontró una clase IV de la OMS (3 casos clase III y 2 casos clase II). En el último control (10,7±6,7 años de evolución), 7/16 (43,8%) presentaban enfermedad renal crónica: 5 casos con proteinuria (uno en rango nefrótico), un caso en estadio 2 y un caso en hemodiálisis tras fracaso de injerto renal. Entre los seguidos más de 10 años, 4/9 (44,4%) tenían alteraciones: 3 pacientes presentaban proteinuria más el paciente en hemodiálisis. La mayoría (15/16) recibieron tratamiento con bolos de ciclofosfamida intravenosa en número variable tras el diagnóstico. Solamente se encontró asociación de la afectación renal con una mayor proteinuria a los 3 meses, al año y a los 3 años de evolución. Conclusiones: Consideramos la evolución de nuestros pacientes a largo plazo como muy satisfactoria. Aunque un 44,4% de aquellos con más de 10 años de seguimiento presentaba alguna alteración renal, solamente uno (11,1%) ha evolucionado a insuficiencia renal terminal. Estos pacientes presentaban una mayor proteinuria desde los primeros controles (AU)

Introduction: Kidney is frequently affected in patients with systemic lupus erythematosus (SLE). It has been recently suggested that the renal outcome in this disease has improved with an earlier diagnosis and intensive specific treatment. Quite often, articles with paediatric patients lack a prolonged follow-up which could help to predict long term outcome. Methods: This is a retrospective descriptive study based on the review of clinical records from patients with a diagnosis of lupus nephritis in a Paediatric Nephrology unit of a tertiary care centre, between 1987 and 2009. Patients were included with an age up to 18 at diagnosis. They were excluded if follow-up period was shorter than 3 months or if data lacked about it. It includes descriptive evolution of clinical parameters, kidney function, lupus activity as well as pathology and treatment. Several variables were tested for association with chronic kidney disease (CKD) in the last checkpoint. Results: Data were obtained from 16 patients, 3 men and 13 women. Age at the beginning of SLE was 10.6±2.9 years and 12.6±3.5 years at debut of lupus nephritis. Biopsy was obtained in 14 of them, in 11 cases (78.6 %), 3 or less months following diagnosis. In 9 cases (64.3 per cent), biopsy showed WHO class IV, 3 cases, class III and 2 cases, class II. In the last control (10.7±6.7 years of follow-up), 7/16 (43.8 %) had developed CKD: 5 cases with proteinuria (one in nephrotic range), one in stage 2 and one patient in haemodialysis after renal graft loss. Among those with a follow-up longer than 10 years, 4/9 (44.4 %) of them showed some sign of renal disease: 3/9 with proteinuria, and the one needing haemodialysis. Most of the patients (15/16) received treatment with intravenous cyclophosphamide boluses in a variable number after diagnosis. Only a more intense proteinuria at 3 months, 1 year and 3 years of evolution was related to CKD in the last visit. Conclusions: We consider the long term evolution of our patients to have been very satisfactory. Although 44.4 % of them developed renal anomalies after 10 or more years, only one (11.1%) reached an end-stage renal disease. These patients presented a higher grade of proteinuria from the first visits (AU)

[Long-term outcome of paediatric lupus nephritis]. / Evolución a largo plazo de la nefritis lúpica de inicio en la edad pediátrica.

INTRODUCTION: Kidney is frequently affected in patients with systemic lupus erythematosus (SLE). It has been recently suggested that the renal outcome in this disease has improved with an earlier diagnosis and intensive specific treatment. Quite often, articles with paediatric patients lack a prolonged follow-up which could help to predict long term outcome. METHODS: This is a retrospective descriptive study based on the review of clinical records from patients with a diagnosis of lupus nephritis in a Paediatric Nephrology unit of a tertiary care centre, between 1987 and 2009. Patients were included with an age up to 18 at diagnosis. They were excluded if follow-up period was shorter than 3 months or if data lacked about it. It includes descriptive evolution of clinical parameters, kidney function, lupus activity as well as pathology and treatment. Several variables were tested for association with chronic kidney disease (CKD) in the last checkpoint. RESULTS: Data were obtained from 16 patients, 3 men and 13 women. Age at the beginning of SLE was 10.6 + or - 2.9 years and 12.6 + or - 3.5 years at debut of lupus nephritis. Biopsy was obtained in 14 of them, in 11 cases (78.6 %), 3 or less months following diagnosis. In 9 cases (64.3 per cent), biopsy showed WHO class IV, 3 cases, class III and 2 cases, class II. In the last control (10.7 + or - 6.7 years of follow-up), 7/16 (43.8 %) had developed CKD: 5 cases with proteinuria (one in nephrotic range), one in stage 2 and one patient in haemodialysis after renal graft loss. Among those with a follow-up longer than 10 years, 4/9 (44.4 %) of them showed some sign of renal disease: 3/9 with proteinuria, and the one needing haemodialysis. Most of the patients (15/16) received treatment with intravenous cyclophosphamide boluses in a variable number after diagnosis. Only a more intense proteinuria at 3 months, 1 year and 3 years of evolution was related to CKD in the last visit. CONCLUSIONS: We consider the long term evolution of our patients to have been very satisfactory. Although 44.4 % of them developed renal anomalies after 10 or more years, only one (11.1%) reached an end-stage renal disease. These patients presented a higher grade of proteinuria from the first visits.

[Hemolytic uremic syndrome: long term renal injury]. / Síndrome hemolítico-urémico: afectación renal a largo plazo.

INTRODUCTION: Hemolytic uremic syndrome (HUS) is the most frequent cause of non-pre-renal acute renal failure in pediatrics and it is usually secondary to acute infectious diarrhea, generally due to Shiga-toxin producing E. coli. It is characterized by acute renal failure, hemolytic anemia and thrombocytopenia. It can lead to renal sequels in the long term and to end-stage renal disease. METHODS: The aim of this retrospective study is to describe and analyze renal sequels and their prognostic factors in pediatric patients suffering from HUS in a Spanish tertiary hospital during the last 28 years. RESULTS: 43 children with this condition were admitted, with 38 of them having presented with diarrhea previously. Among those with a follow-up longer than 3 months, 21.6% (8/37) had a reduction in glomerular filtration rate (GFR) in the last visit. Including proteinuria and hypertension, we found up to 35.1% (13/37) prevalence of chronic kidney disease, with 8.1% (3/37) of the patients with end-stage renal disease. We found the following to be prognostic factors for renal injury in the long term in a univariate logistic regression: hypertension, a longer stay in the hospital, more prolonged anuria, more severe leukocytosis and lower GFR at discharge. Multivariate logistic regression showed an association with time in anuria. CONCLUSIONS: Approximately one third of our cases of hemolytic uremic syndrome developed some degree of chronic kidney disease in the long term. A deeper and longer initial renal disfunction is associated with a higher probability of subsequent renal problems.

[Urolithiasis in childhood]. / Urolitiasis en la infancia.

We report a retrospective study of 51 children who presented urolithiasis between 1980 and 1989 in our Hospital. Mean age was 7 years and the male:female ratio was 2. 1:1. A positive family history was found in 60% of cases. It was done metabolic evaluation in every case: hypercalciuria was found in 34% of cases. In 6% of cases there were hyperuricosuria. None of our patients presented hyperoxaluria, cystinuria or hypocitraturia. Abdominal echography was the most sensible an specific imaging technique of diagnosis. In 16 cases it was necessary a surgical procedure although most cases received only medical management. Four patients were treated with extracorporeal shock-wave lithotripsy. We emphasize the importance of metabolic evaluation. We report our own protocol of study and results.