[Proteinuria in urinary infection and acute pyelonephritis in paediatric patients: can it replace scintigraphic studies in diagnostic localisation?]. / Estudio de proteinuria en infección urinaria y pielonefritis aguda en Pediatría: ¿puede sustituir en el diagnóstico de localización al estudio gammagráfico?

UNLABELLED: ANTECEDENTS AND OBJECTIVES: The location of the urinary tract infection in children has serious implications both in therapy and prognosis. Affectation in gammagraphic studies is considered the "gold standard" for the diagnosis of acute pyelonephritis. Several studies with biomarkers have been made with controverted results. The objective of this study is to set the utility of beta2-microglobuline, alfa1-microglobuline, Cistatine C, IgG and albumin in the location of the infection, through the analysis of the relation among their urinary excretion, clinical and laboratory parameters and the renal scintigraphy findings. PATIENTS AND METHODS: Prospective observational study made in 40 patients, aged from 1 month to 11 years, after their Hospital admission with suspicion for acute pyelonephritis. Exclusion criteria were: decrease in the glomerular filtration rate, malnutrition, massive albuminuria and history or findings of nephrourologic disease. Clinical and laboratory variables were analyzed, and renal ultrasonography and scintigraphy were performed within the first seven days after admission. Once the fever dropped, the urinary creatinine-protein ratio was analyzed. After the acute process, a renal scintigraphy was performed in order to detect renal scars. The non-parametric Mann-Whitney U test has been used as the statistical hypothesis test, and Chi-square and Fisher s exact tests have been used to compare the qualitative variables. RESULTS: The glomerular filtration rate, as well as the ultrasonography scan, was normal in all patients. 45% of the children (24/80 kidneys) had scintigraphic alterations that were compatible with acute pyelonephritis, and there were not differences in proteinuria between these and those with normal scintigraphy. To greater age greater probability of scintigraphic affectation. The sensitivity and specificity of leukocytes and CRP to predict pyelonephritis were 77-65% and 94-52%, r = 0.70 (CI 95% 0.54-0.87) y 0.75 (CI 95% 0.60-0.90) respectively. An apparent relation between the maximum temperature or duration of the symptoms and the renal affectation does not exist. In all the children, the normality of urinary markers of renal function was confirmed once the acute phase had passed, even in those 3 patients with renal scars (7.5%). CONCLUSIONS: Renal scintigraphy is still being the reference pattern for the diagnosis of acute pyelonephritis in Pediatrics. The combined use of different urinary markers of renal function does not increase its sensitivity. It is necessary to do research in order to confirm the utility of new biomarkers.

Estudio de proteinuria en infección urinaria y pielonefritis aguda en Pediatría: ¿puede sustituir en el diagnóstico de localización al estudio gammagráfico? / No disponible

Antecedentes y objetivos: la localización de la infección urinaria en el niño tiene implicaciones terapéuticas y pronósticas. La afectación gamma gráfica se considera como «patrón oro» en el diagnóstico de pielonefritis aguda. Se han realizado estudios con biomarcadores urinarios con resultados controvertidos. El objetivo de este estudio ha sido determinar la utilidad de β2-microglobulina, α1-microglobulina, cistatina C, IgG y albúmina en el diagnóstico de localización, analizando la relación entre su excreción urinaria, parámetros clínicos y de laboratorio y la afectación renal gammagráfica. Pacientes y métodos: estudio observacional prospectivo realizado en 40 pacientes de un mes a 11 años de edad tras su ingreso hospitalario por sospecha de pielonefritis. Se analizaron variables clínicas y de laboratorio, realizando una ecografía renal y una gammagrafía renal enlos primeros siete días del ingreso. Una vez remitida la fiebre, se analizó el cociente urinario proteína/creatinina para cada variable. Tras el proceso agudo, se realizó una gammagrafía renal en fase tardía para detectar cicatrices renales. Resultados: el filtrado glomerular y la ecografía fueron normales en todos los pacientes. Un 45% de los niños (24/80 riñones) tuvieron alteraciones gammagráficas compatibles con pielonefritis aguda, no existiendo diferencias en la proteinuria entre éstos y aquellos con gammagrafía normal. A mayor edad, mayor probabilidad de(..) (AU)

Antecedents and objectives: the location of the urinary tract infection in children has serious implications both in therapy and prognosis. Affectation in gamma graphic studies is considered the “gold standard” for the diagnosis of acute pyelonephritis. Several studies with biomarkers have been made with controverted results. The objective of this study is to set the utility of beta2-microglobuline, alfa1-microglobuline, Cistatine C, IgG and albumin in the location of the infection, through the analysis of the relation among their urinary excretion, clinical and laboratory parameters and the renal scintigraphy findings. Patients and methods: Prospective observational study made in 40 patients, aged from 1 month to 11 years, after their Hospital admission with suspicion for acute pyelonephritis. Exclusion criteria were: decrease in the glomerular filtration rate, malnutrition, massive albuminuria and history or findings of nephrourologic disease. Clinical and laboratory variables were analyzed, and renal ultrasonography and scintigraphy were performed within the first seven days after admission. Once the fever dropped, the urinary creatinine-protein ratio was analyzed. After the acute process, a renal scintigraphy was performed in order to detect renal scars. The non-parametric Mann-Whitney Utest has been used as the statistical hypothesis test, and Chi-square and Fisher´s exact tests have been used to compare the qualitative variables. Results: The glomerular filtration rate, as well as the ultrasonography scan, was normal in all patients. 45% of the children (24/80kidneys) had scintigraphic alterations that were compatible with acute pyelonephritis, and there were not differencies in proteinuria between these and those with (..) (AU)

Insuficiencia renal aguda tras consumo de setas / No disponible

La recolección y consumo de setas silvestres es una práctica frecuente en nuestro medio y puede dar lugar a intoxicaciones accidentales al ser confundidas con hongos comestibles. Describimos el caso de un niño de 9 años que tras la ingesta de un hongo tóxico comienza horas después con vómitos incoercibles y posterior afectación hepática, hematológica y renal, precisando hemodiálisis. El curso clínico, los hallazgos de laboratorio y la histología renal, donde se evidencia necrosis tubular con membrana basal conservada e infiltrado linfocitario intersticial, confirman el diagnóstico de un síndrome mixto grave. La evolución de la intoxicación fue favorable, recuperándose la función renal y hepática. Ante cualquier caso de insuficiencia renal aguda no filiada en niños, habría que descartarla exposición a tóxicos, ya que podría beneficiarse de un tratamiento precoz con hemoperfusión y así evitar el agravamiento de la función renal y resto de órganos. En nuestro paciente la hemoperfusión no fue realizada por el largo período de latencia que había ocurrido desde la ingesta del tóxico hasta su diagnóstico (AU)

The picking and consumption of wild mushrooms is a frequent practice in our region and may lead to accidental poisoning when confused with edible mushrooms. We describe the case of a 9-year-old boy who, following the ingestion of a poisonous mushroom, presented with uncontrollable vomiting and subsequent hepatic, haematological and renal failure some hours later. The patient required haemodialysis. The clinical course, laboratory findings and renal histology, which showed tubular necrosis with basal membrane preserved and lymphocytic interstitial infiltrate, confirmed the diagnosis of a severe mixed syndrome. The patient evolved favourably after the poisoning, recovering renal and liver function. In any case of acute renal failure of unknown cause in children, it would be necessary to rule out ingestion of mushrooms, since the patient could benefit from early treatment with haemoperfusion and thus prevent the deterioration of the renalfunction and other organs. In our patient, haemoperfusion was not carried out due to the lengthy period of latency since the ingestion of the toxic substance until diagnosis (AU)

[Acute renal failure after intake of mushrooms]. / Insuficiencia renal aguda tras consumo de setas.

The picking and consumption of wild mushrooms is a frequent practice in our region and may lead to accidental poisoning when confused with edible mushrooms. We describe the case of a 9-year-old boy who, following the ingestion of a poisonous mushroom, presented with uncontrollable vomiting and subsequent hepatic, haematological and renal failure some hours later. The patient required haemodialysis. The clinical course, laboratory findings and renal histology, which showed tubular necrosis with basal membrane preserved and lymphocytic interstitial infiltrate, confirmed the diagnosis of a severe mixed syndrome. The patient evolved favourably after the poisoning, recovering renal and liver function. In any case of acute renal failure of unknown cause in children, it would be necessary to rule out ingestion of mushrooms, since the patient could benefit from early treatment with haemoperfusion and thus prevent the deterioration of the renal function and other organs. In our patient, haemoperfusion was not carried out due to the lengthy period of latency since the ingestion of the toxic substance until diagnosis.

[Evaluation of therapeutic strategies in congenital nephrotic syndrome of the Finnish type]. / Evaluación de las estrategias terapéuticas en el síndrome nefrótico congénito tipo finlandés.

INTRODUCTION: Congenital nephrotic syndrome of the Finnish type (CNFS) is caused by mutations in the nephrin gene. This disease manifests as massive proteinuria, gross peripheral edema, and ascites during the first weeks of life. In the last few years the prognosis has improved due to new treatment strategies: antiproteinuria drugs, intensive nutrition, nephrectomy, dialysis, and renal transplantation. The aim of this study was to determine the impact of these therapeutic measures. PATIENTS AND METHOD: We performed a descriptive retrospective epidemiological study of 12 patients diagnosed with CNSF between January 1985 and August 2005. We included patients aged less than 14 years old with massive proteinuria and generalized edema during the neonatal period, a large placenta (> 25 % of birth weight), and normal glomerular filtration rate during the first 6 months of life, in whom other causes of congenital nephrotic syndrome were ruled out. RESULTS: The diagnosis was established after a median period of 17 days (range 6-30). The most commonly used treatments were albumin infusions (91.7 %), angiotensin-converting enzyme inhibitors (66.7 %), and indomethacin (58.3 %). Dialysis was started in 58.3 %, at a median age of 3.76 years (2.81-7.6). The main complication was acute peritonitis (85.7 %). Renal transplantation was performed in 58.3 % of the patients; of these, 71.4 % have normal renal function after a median follow-up of 3.73 years (0.8-6.3). The median plasma albumin level during the pretransplant period was 0.17 g/dL (0.12-0.28). Plasma cholesterol and triglyceride levels decreased significantly after renal transplantation (p = 0.043). Fifty percent of the patients achieved adequate height and weight for their age and gender. Mortality was 33.3 %. CONCLUSION: Antiproteinuria drugs and intensive nutritional therapy improve clinical control and delay the start of dialysis and renal transplantation, increasing the probability of success.

Evaluación de las estrategias terapéuticas en el síndrome nefrótico congénito tipo finlandés / Evaluation of therapeutic strategies in congenital nephrotic syndrome of the Finnish type

Introducción El síndrome nefrótico congénito tipo finlandés (SNCF) es una enfermedad causada por diferentes mutaciones en el gen de la nefrina. Se manifiesta con proteinuria masiva, edemas generalizados y ascitis en las primeras semanas de vida. En los últimos años su pronóstico ha mejorado gracias a nuevas estrategias de tratamiento: fármacos antiproteinúricos, nutrición intensa, nefrectomía, diálisis y trasplante renal. El objetivo del estudio es determinar la repercusión de estas medidas terapéuticas. Pacientes y método Estudio epidemiológico descriptivo retrospectivo de 12 pacientes diagnosticados de SNCF entre enero de 1985 y agosto de 2005. Se incluyeron menores de 14 años con proteinuria grave, edema generalizado, peso de la placenta superior al 25 % del peso al nacer, filtrado glomerular normal en los primeros 6 meses, y en los que se descartaron otras causas de síndrome nefrótico congénito. Resultados El diagnóstico se realizó en una mediana de 17 días (rango intercuartílico: 6-30). Los tratamientos más utilizados fueron las infusiones de albúmina (91,7 %), los inhibidores de la enzima conversora de la angiotensina (66,7 %) y la indometacina (58,3 %). El 58,3 % inició diálisis, a una edad mediana de 3,76 años (2,8-7,6). Su principal complicación fue la peritonitis aguda (85,7 %). El 58,3 % recibió un trasplante renal; de ellos, el 71,4 % tiene una función renal normal tras un período de seguimiento mediano de 3,73 años (0,8-6,3). La albuminemia durante el período pretrasplante fue de 0,17 g/dl (0,12-0,28). Las cifras de colesterol y triglicéridos plasmáticos descendieron de forma significativa tras el trasplante renal (p = 0,043). El 50 % de los pacientes alcanzó un peso y talla adecuados para su edad y género. La mortalidad fue del 33,3 %. Conclusión Los fármacos antiproteinúricos y el tratamiento nutricional intensivo permiten un mejor control clínico e iniciar la diálisis y el trasplante renal de forma más tardía y con mayores probabilidades de éxito

Introduction Congenital nephrotic syndrome of the Finnish type (CNFS) is caused by mutations in the nephrin gene. This disease manifests as massive proteinuria, gross peripheral edema, and ascites during the first weeks of life. In the last few years the prognosis has improved due to new treatment strategies: antiproteinuria drugs, intensive nutrition, nephrectomy, dialysis, and renal transplantation. The aim of this study was to determine the impact of these therapeutic measures. Patients and method We performed a descriptive retrospective epidemiological study of 12 patients diagnosed with CNSF between January 1985 and August 2005. We included patients aged less than 14 years old with massive proteinuria and generalized edema during the neonatal period, a large placenta (> 25 % of birth weight), and normal glomerular filtration rate during the first 6 months of life, in whom other causes of congenital nephrotic syndrome were ruled out. Results The diagnosis was established after a median period of 17 days (range 6-30). The most commonly used treatments were albumin infusions (91.7 %), angiotensin-converting enzyme inhibitors (66.7 %), and indomethacin (58.3 %). Dialysis was started in 58.3 %, at a median age of 3.76 years (2.81-7.6). The main complication was acute peritonitis (85.7 %). Renal transplantation was performed in 58.3 % of the patients; of these, 71.4 % have normal renal function after a median follow-up of 3.73 years (0.8-6.3). The median plasma albumin level during the pretransplant period was 0.17 g/dL (0.12-0.28). Plasma cholesterol and triglyceride levels decreased significantly after renal transplantation (p = 0.043). Fifty percent of the patients achieved adequate height and weight for their age and gender. Mortality was 33.3 %. Conclusion Antiproteinuria drugs and intensive nutritional therapy improve clinical control and delay the start of dialysis and renal transplantation, increasing the probability of success

[Guidelines of the Spanish Society of Nephrology. Clinical practice guidelines for peritoneal dialysis]. / Guías Sociedad Española de Nefrología. Guías de práctica clínica en diálisis peritoneal.

In Spain and in each of its autonomous communities, the dialysis treatment of chronic renal disease stage 5 is totally covered by public health. Peritoneal dialysis, in any of its modalities, is established as the preferred home dialysis technique and is chosen by high percentage of patients as their choice in dialysis treatment. The Spanish Society of Nephrology has promoted a project of creation of performance guides in the field of peritoneal dialysis, entrusting a work group composed of members of the Spanish Society of Nephrology a with the development of these guides. The information offered is based on levels of evidence, opinion and clinical experience of the most relevant publications of the topic. In these guides, after defining the concept of << peritoneal dialysis>>, the obligations and responsibilities of the sanitation team of the peritoneal dialysis unit are determined, and protocols and performance procedures that try to include all the aspects that concern the patient with chronic renal disease in substitute treatment with this technique are developed. They propose prescription objectives based on available clinical evidence and, lacking this, on the consensus of the experts' opinions. The final aim is to improve the care and quality of the of the patient in peritoneal dialysis, optimizing in this way the survival of the patient and of the technique. In Spain, as in other neighbouring countries, peritoneal dialysis has an incidence and prevalence that is much lower than that of hemodialysis, ranging in the last evaluation by the Spanish Society of Nephrology between 5 and 24% in the different autonomous communities. The great majority of peritoneal dialysis units form part of the public network of the Spanish state, with special representation as a Satellite Unit or Concerted Center related to the public hospital of reference, on which it must depend.

Immunoprophylaxis with Simulect (basiliximab) in kidney transplantation: results from routine clinical practice at 18 kidney transplant units.

OBJECTIVE: The objective of this study is to assess a Simulect (basiliximab) regimen in routine clinical practice in the Spanish kidney transplantation units to evaluate efficacy and safety. METHODS: In this prospective, observational study, data on demographics, parameters of efficacy, and safety in patients who under with kidney transplantation treated with Simulect (basiliximab) were collected through an on-line collection system. RESULTS: One hundred sixty three patients at 18 kidney transplant units included 12 months follow-up. The patient mean age was 52 years (DS 13,67) including 96 (58.90%) men and 67 (41.10%) women. Cold ischemia time was 19 hours (DS 6,79). Only 2 patients presented with PRA >50%. For prophylactic immunosuppression, 67.13% of patients received triple therapy with CNI (cyclosporine 49.65% or tacrolimus 17.48%), MMF (66.43%) or AZA (10.49%), and steroids. Incidence of acute rejection (AR) at 12 months was 12.27% (1.84% steroid-resistant). In subgroup analysis, AR was 13.5% in nondiabetics and 4.5% in diabetics, including 3 steroid-resistant episodes (1.84%) in nondiabetics and none in diabetics. In relation to donor age, AR was incidence 10.3% in patients with kidneys from donors aged 50 years or younger and 10.6% when donors were older than 50 years, including 1 (1.73%) and 2 (1.93%) steroid-resistant episodes, respectively. The graft and patient survival rates at 12 months were 90% and 98%, respectively. CONCLUSIONS: Simulect (basiliximab) used in routine clinical practice provided good prophylaxis against acute rejection in several kidney transplant patient populations, similar to that observed in randomized clinical studies with excellent tolerability and safety.

Trasplante renal y crisis epilépticas en la infancia / Renal transplantation and epileptic episode in childhood

La semiología clínica neurológica que puede presentarse en el contexto de un trasplante renal, comprende un amplio y variado abanico de enfermedades neurológicas. Y una gran parte de las complicaciones neurológicas del trasplante renal pueden desarrollar crisis convulsivas. Objetivo: Se pretende analizar la incidencia de las crisis convulsivas en niños que tras el diagnóstico de insuficiencia renal terminal han recibido un trasplante renal, estudiando las características semiológicas de los episodios convulsivos, su posterior evolución y la presencia de posibles secuelas. Se han relacionado los distintos factores desencadenantes de las crisis, con su evolución y con su tratamiento. Material y métodos: Durante un período de 20 años, se revisan de forma retrospectiva 42 pacientes que ingresados en el hospital han sido sometidos a un trasplante renal. El estudio informático de las 18 variables médicas recogidas se realiza con el programa SPSS versión 9.0.1. Resultados: Del total de enfermos, 13 habían sufrido algún tipo de crisis convulsiva (30,95 por ciento), con una edad media de aparición de la primera crisis de 69 meses. Como factor desencadenante, hubo un desequilibrio hidroelectrolítico en 8 casos, hipertensión en 2, y otras causas no bien determinadas en los restantes. sólo en 3 casos que correspondían a epilepsias previas al fallo renal, se mantenían las manifestaciones críticas. Comentarios: Las crisis epilépticas del trasplante renal en la infancia pueden ser consideradas como crisis ocasionales, que no suelen cronificarse ni dejar secuelas deficitarias. El manejo de las crisis en cualquier fallo renal debe ser divulgado entre los profesionales que atienden enfermedades sistémicas (AU)

Recurrencia de hialinosis segmentaria y focal en el trasplante renal / Recurrence of focal glomerulosclerosis after renal transplantation

No disponible

Insuficiencia renal como factor desencadenante de crisis epilépticas / Renal failure as a factor leading to epileptic seizures

Objetivo: Se pretende analizar la incidencia de las crisis convulsivas en la insuficiencia renal, estudiando las características semiológicas de los episodios convulsivos, su posterior evolución y la presencia de posibles secuelas. Se han relacionado los distintos factores desencadenantes de las crisis, con su evolución y con su tratamiento. Material y métodos: Estudio retrospectivo por revisión de historias clínicas. Durante un período de 20 años se revisan de forma retrospectiva 108 pacientes ingresados en el hospital con el diagnóstico de insuficiencia renal: 55 con prediálisis, 42 con trasplante renal, 7 con diálisis peritoneal, y 3, hemodiálisis. Un caso se desechó por no cumplir los criterios de selección. Se analizaron 18 variables cuantitativas y cualitativas, utilizando las medidas de tendencia central o de localización en las variables cuantitativas, y los porcentajes en las cualitativas. El test de chi cuadrado ( 2) se usó al comparar dos proporciones. Resultados: Del total de enfermos, 16 habían sufrido algún tipo de crisis convulsiva (14,95%), y de ellos ninguno se encontraba en el grupo de prediálisis. Como factor desencadenante, hubo un desequilibrio hidroelectrolítico en 8 casos, hipertensión en cuatro, y otras causas no bien determinadas en los restantes. Sólo en 3 casos que correspondían a epilepsias previas a la insuficiencia renal, se mantenían las manifestaciones críticas. Conclusiones: Las crisis epilépticas de la insuficiencia renal en la infancia pueden considerarse crisis ocasionales, que no suelen cronificarse ni dejar secuelas deficitarias. El tratamiento de las crisis en la insuficiencia renal debe ser divulgado entre los profesionales que atienden enfermedades sistémicas (AU)

[Renal failure as a factor leading to epileptic seizures]. / Insuficiencia renal como factor desencadenante de crisis epilépticas.

OBJECTIVE: To analyze the incidence of convulsive seizures in renal failure by studying the semiological characteristics of the convulsive episodes, their subsequent evolution and the presence of possible sequelae. The relationship between the different potential causes of the seizures and their evolution and treatment was also analyzed. MATERIAL AND METHODS: We retrospectively reviewed the clinical histories of 108 patients admitted to the hospital over a 20-year period with a diagnosis of renal failure: 55 were undergoing predialysis, 42 renal transplantation, 7 peritoneal dialysis and 3 hemodialysis. One patient was excluded for not fulfilling the selection criteria. Computer study of the 18 quantitative and qualitative variables was carried out with the SPSS 9.0.1 program. RESULTS: Of the 107 patients, 16 (14.95 %) had suffered some type of convulsive seizure. None was in the predialysis group. As triggering factors, hydroelectrolyte imbalance was found in eight patients and hypertension was found in four. In the remaining patients the causes were not well-defined. Only three patients with epilepsy prior to renal failure presented sequelae. CONCLUSIONS: Epileptic seizures in renal failure in childhood can be considered as occasional seizures that do not usually become chronic or produce sequelae. Information on the management of seizures in renal failure should be disseminated among professionals treating systemic diseases.

Towards guidelines for dialysis in children with end-stage renal disease.

There are few data describing the current practices of treatment selection for children with end-stage renal disease (ESRD). In an effort to establish a consensus among Spanish pediatric nephrologists for inclusion and exclusion criteria for renal replacement therapy in children with ESRD, in 1995 we surveyed members of the Spanish Pediatric Nephrology Association. Although only 43% of members responded, pediatric nephrologists and bioethicists studied the results and compiled a list of ten guidelines for treatment of children with ESRD. The proposed guidelines are meant to be a starting point for further discussion. An emphasis on flexibility, individual case assessment, and consideration of the best interests of the patient must remain central to any treatment plan. Decision making should ideally be shared by parents, professionals, the child, when appropriate, and ethics committees, as necessary.

[Renal malformations in the authors' community. General considerations]. / Estudio sobre las malformaciones renales en nuestra área. Consideraciones generales.

The aim of this work was to study kidney malformations in infants. For this purpose, a study team was formed with two principal goals: to study the anatomic and histologic aspects, and then to correlate them to familial and clinical characteristics as well as to radiologic and echographic studies. We studied 129 cases of kidney malformations found during a study of 4,800 necropsies that had been carried out on infants, as well as of nephrectomized kidneys and biopsy samples. As a result of this study, we have compiled a mixed criteria (anatomic and clinical) classification of kidney malformations, complete with a glossary of equivalent terms to denominate different types of kidney malformations which have been called by a wide variety of nomenclatures in the bibliography.