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INTRODUCCION: In nephrotic syndrome, increased podocyturia accompanies pathologic proteinuria. The therapeutic regimen with enalapril, losartan and amiloride could reduce both variables. OBJETIVES: Evaluate the anti-proteinuric effect of 2 non-immunological therapeutic regimens, the quantitative relationship between podocyturia and proteinuria. MATERIAL AND METHODS: We included children aged 4 to 12 years with corticoresistant nephrotic syndrome, using 2 different schemes: group A, enalapril+losartan, and group B, enalapril+losartan+amiloride. RESULTS: In group A, 17 patients completed the study, the initial mean proteinuria was 39mg/m2/h and mean proteinuria at the end was 24mg/m2/h, while in group B 14 patients were treated and the initial average proteinuria was 36mg/m2/h and the end average proteinuria was 13mg/m2/h. The paired T test showed significant differences in the decrease in proteinuria, for patients in group B without variation in podocyturia. The 2 factors associated with an increase in proteinuria were podocyturia and the time elapsed from the diagnosis of cortico-resistant nephrotic syndrome to the start of treatment anti-proteinuric. CONCLUSIONS: The use of amiloride decreased proteinuria, without significantly modifying podocyturia; we did not observe a positive relationship between both variables.

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BACKGROUND: Global cerebral ischemia (GCI) elicits damages to cerebral structures, learning dysfunction, memory impairments, hyperactivity, and anxiety. Circulating levels of galectin-3 (Gal-3) are associated with patient severity and outcome. AIM: To report circulating levels of Gal-3, and cytokines (TNF-α, IL-6, IL-10) in the initial hours (acute) following GCI in a four-vessel occlusion (4-VO) rat model and the effect of melatonin treatment. METHODS: 4-VO model was used to produce GCI using male Sprague-Dawley rats. Groups were: Sham-Veh, Sham-Mel, Isch-Veh and Isch-Mel. Melatonin was administered 30 min after carotid clamp removal. Gal-3 and cytokines levels were measured at 0, 30 min, 6 h and 24 h after the end of cerebral flow interruption using ELISA kits. Motor activity and anxiety were measured using open-field test. RESULTS: Acute GCI (AGCI) followed by reperfusion decreased serum concentrations of TNF-α and increased IL-6 levels 24 h after ischemia, whereas melatonin reduced significantly the concentrations of these cytokines. In all groups IL-10 was higher 30 min and negligible at other times. Circulating levels of Gal-3 were reduced 30 min after ischemia/reperfusion. In the Isch-Mel group the neuroprotective effect generated a reduction in circulating Gal-3 at 6 and 24 h after AGCI, compared with all the groups. Motor activity was increased due to ischemic reperfusion, but acute melatonin treatment reduced locomotion, similar to the control group. Anxiety was reduced in the melatonin group. CONCLUSIONS: Melatonin treatment following AGCI reduces pro-inflammatory factors, Gal-3, motility, and anxiety, therefore it should be considered as supplementary treatment following ischemic stroke.

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INTRODUCCION: In nephrotic syndrome, increased podocyturia accompanies pathologic proteinuria. The therapeutic regimen with enalapril, losartan and amiloride could reduce both variables. OBJETIVES: Evaluate the anti-proteinuric effect of 2 non-immunological therapeutic regimens, the quantitative relationship between podocyturia and proteinuria. MATERIAL AND METHODS: We included children aged 4-12 years with corticoresistant nephrotic syndrome, using 2 different schemes: group A, enalapril + losartan, and group B, enalapril + losartan + amiloride. RESULTS: In group A, 17 patients completed the study, the initial mean proteinuria was 39 mg/m2/h and mean proteinuria at the end was 24 mg/m2/h, while in group B 14 patients were treated and the initial average proteinuria was 36 mg/m2/h and the end average proteinuria was 13 mg/m2/h. The paired T test showed significant differences in the decrease in proteinuria, for patients in group B without variation in podocyturia. The 2 factors associated with an increase in proteinuria were podocyturia and the time elapsed from the diagnosis of cortico-resistant nephrotic syndrome to the start of treatment anti-proteinuric. CONCLUSIONS: The use of amiloride decreased proteinuria, without significantly modifying podocyturia; we did not observe a positive relationship between both variables.

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Introducción: La enfermedad de Fabry (EF) es un trastorno hereditario causado por una deficiencia de la actividad de la enzima alfa-galactosidasa A, cuya transmisión está relacionada con el cromosoma X. Objetivos: Los objetivos del estudio fueron: 1. Cuantificar la presencia de podocitos en pacientes pediátricos con EF y compararla con el valor de la podocituria medida en controles sanos. 2. Determinar en pacientes con EF si una mayor podocituria está relacionada con la albuminuria patológica. 3. Determinar los factores de riesgo asociados con la albuminuria patológica. Métodos: Implementamos un estudio analítico observacional de casos y controles, separados en 2 grupos de acuerdo con la ausencia de enfermedad (grupo control) o con la presencia de enfermedad (grupo Fabry). Resultados: Estudiamos a 31 pacientes, 11 con EF y 20 controles, con una media de edad de 11,6 años. La diferencia entre el tiempo medio transcurrido desde el diagnóstico de EF hasta la medición de la podocituria (40 meses) y la aparición de la albuminuria patológica (34 meses) no fue significativa (p: 0,09). Los podocitos se identificaron mediante tinción para sinaptopodina y las diferencias medias cuantitativas entre ambas podociturias fueron estadísticamente significativas (p: 0,001). La albuminuria fue fisiológica en 4 de las pacientes Fabry y el riesgo relativo para desarrollar albuminuria patológica de acuerdo con la podocituria fue en el grupo control 1,1 y en el grupo Fabry 3,9, con un coeficiente de correlación entre la podocituria y la albuminuria en el grupo Fabry de 0,8354. Finalmente los 2 factores de riesgo asociados al desarrollo de albuminuria patológica fueron la podocituria (OR 14) y la edad mayor a 10 años (OR 18). No encontramos riesgo significativo ni en el filtrado glomerular (FG) (OR 0,5), ni en el género (OR 1,3). El FG medio se mantuvo dentro de valores normales. Conclusión: La detección de podocituria en pacientes pediátricos con EF podría utilizarse como un marcador temprano de daño renal previo y relacionado con la albuminuria patológica

Introduction: Fabry disease (FD) is a hereditary disorder caused by a deficiency of α-galactosidase A enzyme activity. The transmission of the disorder is linked to the X chromosome. Objectives: The objectives of the study were: 1. To quantify the presence of podocytes in paediatric patients with FD and compare them with the value of the measured podocyturia in healthy controls. 2. To determine whether a greater podocyturia is related to the onset of pathological albuminuria in patients with FD. 3. To determine the risk factors associated with pathological albuminuria. Methods: We performed an analytical, observational study of Fabry and control subjects, which were separated into 2groups in accordance with the absence of the disease (control group) or the presence of the disease (Fabry group). Results: We studied 31 patients, 11 with FD and 20 controls, with a mean age of 11.6 years. The difference between the mean time elapsed from the diagnosis of FD to the measurement of podocyturia (40 months) and the onset of pathological albuminuria (34 months) was not significant (p = 0.09). Podocytes were identified by staining for the presence of synaptopodin and the mean quantitative differences between both podocyturias were statistically significant (p = 0.001). Albuminuria was physiological in 4 of the patients with FD and the relative risk to develop pathological albuminuria according to podocyturia was 1.1 in the control group and 3.9 in the Fabry group, with a coefficient of correlation between podocyturia and albuminuria in the Fabry group of 0.8354. Finally, the 2 risk factors associated with the development of pathological albuminuria were podocyturia (OR: 14) and being aged over 10 years (OR: 18). We found no significant risk with regard to glomerular filtrate renal (GFR) (OR: 0.5) or gender (OR: 1.3). The mean GFR remained within normal values. Conclusion: The detection of podocyturia in paediatric patients with FD could be used as an early marker of renal damage, preceding and proportional to the occurrence of pathological albuminuria

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INTRODUCTION: Fabry disease (FD) is a hereditary disorder caused by a deficiency of α-galactosidase A enzyme activity. The transmission of the disorder is linked to the X chromosome. OBJECTIVES: The objectives of the study were: 1. To quantify the presence of podocytes in paediatric patients with FD and compare them with the value of the measured podocyturia in healthy controls. 2. To determine whether a greater podocyturia is related to the onset of pathological albuminuria in patients with FD. 3. To determine the risk factors associated with pathological albuminuria. METHODS: We performed an analytical, observational study of Fabry and control subjects, which were separated into 2groups in accordance with the absence of the disease (control group) or the presence of the disease (Fabry group). RESULTS: We studied 31 patients, 11 with FD and 20 controls, with a mean age of 11.6 years. The difference between the mean time elapsed from the diagnosis of FD to the measurement of podocyturia (40 months) and the onset of pathological albuminuria (34 months) was not significant (p=0.09). Podocytes were identified by staining for the presence of synaptopodin and the mean quantitative differences between both podocyturias were statistically significant (p=0.001). Albuminuria was physiological in 4 of the patients with FD and the relative risk to develop pathological albuminuria according to podocyturia was 1.1 in the control group and 3.9 in the Fabry group, with a coefficient of correlation between podocyturia and albuminuria in the Fabry group of 0.8354. Finally, the 2 risk factors associated with the development of pathological albuminuria were podocyturia (OR: 14) and being aged over 10 years (OR: 18). We found no significant risk with regard to glomerular filtrate renal (GFR) (OR: 0.5) or gender (OR: 1.3). The mean GFR remained within normal values. CONCLUSION: The detection of podocyturia in paediatric patients with FD could be used as an early marker of renal damage, preceding and proportional to the occurrence of pathological albuminuria.

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Los niños con alteración de su sistema inmunológico son más vulnerables ante las infecciones que el resto de la población. Una de las formas de protegerlos de infecciones graves es a través de la vacunación, deben ser correctamente evaluados al planear los esquemas a fin de establecer los riesgos vs. los beneficios que implican inmunizarlos. El rol del médico pediatra y del médico especialista trabajando en equipo es fundamental, para que puedan beneficiarse con vacunas y esquemas especiales que requieran por su patología de base. Una protección óptima de estos pacientes incluye además la adecuada inmunización de los convivientes y del equipo médico tratante. La inmunización de los huéspedes especiales es una situación clínica compleja que requiere un análisis exhaustivo personalizado en cada caso, debido a las diferentes características de estos pacientes con enfermedades crónicas y/o inmunosuprimidos, los diversos grupos y muchos tipos de terapias inmunosupresoras que se están desarrollando y utilizando en un número cada vez mayor. Es fundamental el trabajo en equipo del médico especialista y el pediatra de cabecera para lograr el mejor control de las enfermedades inmunoprevenibles en estos pacientes de tan alta complejidad

Children with weakened immune systems are more vulnerable to infections than the rest of the population. One of the ways to protect them against serious infections is vaccination; they must be correctly evaluated when planning schedules in order to define the risks versus the benefits involved by their immunization. The role of pediatricians and medical specialists working as a team is fundamental, so that patients can benefit from vaccines and special schedules that they may require due to their underlying pathologies. Optimal protection of these patients also includes the adequate immunization of household members and their treating medical teams. The immunization of special hosts is a complex clinical situation that requires an exhaustive personalized case-by-case analysis, due to the different characteristics of these patients who have chronic diseases and / or are immunosuppressed, the various groups and many types of immunosuppressive therapies that are being developed and increasingly used. The teamwork of specialists and family pediatricians is essential to achieve the best control of immuno-preventable diseases in these highly complex patients

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Resumen: Introducción: En el síndrome nefrótico cortico-sensible (SNCS), la corticoterapia prolongada podría reducir la frecuencia de recaídas. El objetivo de este trabajo fue la comparación de un esquema corticoide prolongado frente al tratamiento habitual del SNCS primario, evaluando los siguientes parámetros: el número de pacientes con recaídas, el número total de recaídas, el tiempo medio transcurrido al iniciar el tratamiento, hasta la remisión y hasta la primera recaída, la dosis acumulada de corticosteroides y la toxicidad esteroide. Métodos: La población se dividió en dos grupos: el grupo A (27 pacientes) recibió 16-β-metilprednisona durante 12 semanas, reduciendo la dosis hasta la semana 24; y el grupo B (29 pacientes) recibió 16-β-metilprednisona durante 12 semanas, y placebo hasta la semana 24. Resultados: La tasa de incidencia acumulada de recaídas (persona/año) fue en el grupo A 36/100, y en el grupo B 66/100 (p = 0.04). El tiempo medio transcurrido (días) hasta la primera recaída fue de 114 en el grupo A y 75 en el grupo B (p = 0.01). Las diferencias de tiempo transcurrido al iniciar tratamiento y hasta la remisión entre ambos grupos no fueron significativas. El total acumulado de recaídas fue de 9 en el grupo A y 17 en el grupo B (p = 0.04), y el total de pacientes con recaídas fue de 3 (grupo A) y 7 (grupo B) (p = 0.17). La dosis media acumulada (mg/m2) por paciente fue de 5,243 en el grupo A y de 4,306 en el grupo B (p = 0.3), y el cortisol sérico (μg/dl) final fue de 14 en el grupo A y 16 en el grupo B (p = 0.4). La toxicidad esteroide fue similar entre ambos grupos. Conclusiones: La duración del tratamiento disminuyó el número de recaídas, sin incrementar la toxicidad esteroide.

Abstract: Background: In the steroid-sensitive nephrotic syndrome (SSNS) the prolonged treatment with steroids could decrease the frequency of relapses. We conducted a comparative study of prolonged steroid scheme and the usual treatment of primary SSNS to assess: the number of patients with relapses, mean time to treatment initiation, to remission and to first relapse, total number of relapses, total cumulative dose of steroids, and the steroid toxicity. Methods: Patients were divided into two groups: group A (27 patients) received 16-β-methylprednisolone for 12 weeks, reducing the steroid until week 24. Group B (29 patients) received 16-β-methylprednisolone for 12 weeks and placebo until week 24. Results: Cumulative incidence rate of relapse (person/years) for group A was of 36/100 and 66/100 for group B (p = 0.04). Average elapsed time to first relapse was of 114 days for group A and of 75 days to for group B (p = 0.01). The difference in time for initial response to treatment and up to achieve remission between both groups was not significant. Total cumulative relapses were 9 for group A and 17 for group B (p = 0.04). Total patients with relapses were 3 for group A and 7 for group B (p = 0.17). Cumulative average dose per patient was 5,243 mg/m2 for group A and 4,306 mg/m2 for group B (p = 0.3), and serum cortisol was 14 μg/dl for group A and 16 μg/dl for group B (p = 0.4). There were no steroid toxicity differences between groups. Conclusions: The duration of the treatment had an impact on the number of relapses without increasing steroid toxicity.

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BACKGROUND: In the steroid-sensitive nephrotic syndrome (SSNS) the prolonged treatment with steroids could decrease the frequency of relapses. We conducted a comparative study of prolonged steroid scheme and the usual treatment of primary SSNS to assess: the number of patients with relapses, mean time to treatment initiation, to remission and to first relapse, total number of relapses, total cumulative dose of steroids, and the steroid toxicity. METHODS: Patients were divided into two groups: group A (27 patients) received 16-ß-methylprednisolone for 12 weeks, reducing the steroid until week 24. Group B (29 patients) received 16-ß-methylprednisolone for 12 weeks and placebo until week 24. RESULTS: Cumulative incidence rate of relapse (person/years) for group A was of 36/100 and 66/100 for group B (p=0.04). Average elapsed time to first relapse was of 114 days for group A and of 75 days to for group B (p=0.01). The difference in time for initial response to treatment and up to achieve remission between both groups was not significant. Total cumulative relapses were 9 for group A and 17 for group B (p=0.04). Total patients with relapses were 3 for group A and 7 for group B (p=0.17). Cumulative average dose per patient was 5,243mg/m2 for group A and 4,306mg/m2 for group B (p=0.3), and serum cortisol was 14µg/dl for group A and 16µg/dl for group B (p=0.4). There were no steroid toxicity differences between groups. CONCLUSIONS: The duration of the treatment had an impact on the number of relapses without increasing steroid toxicity.

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Introducción: el tratamiento esteroide del síndrome nefrótico cortico sensible (SNCS) puede causar alteraciones del metabolismo mineral, prevenibles con calcio y vitamina D. Se llevó a cabo un estudio de cohortes de tipo retrospectivo longitudinal a lo largo de 36 meses. Objetivos: 1) evaluar la relación entre la Dosis Acumulativa de corticoides (DAC) con la concentración sérica de 25-OH Vitamina D y con el Contenido Mineral Ëseo (CMO); 2) evaluar la relación entre la DAC y el CMO en la Densitometría Mineral Ësea (DMO). Material y métodos: Incluimos a pacientes entre 2 años y 12 años con síndrome nefrótico primario cortico-sensible (SNCS) (primer episodio o síndrome nefrótico recaedor o síndrome nefrótico cortico-dependiente), normotensos, eutróficos y con FG>90ml/min/1.73m2, los cuales se separaron en 3 grupos: GRUPO A: 3 o 6 años (puntaje Z y CMO), edad ósea, PTHi. Resultados: Evaluamos a 29 pacientes, con una edad media de 4,4 años. La DMO se realizó en 11 pacientes y no hubo diferencias significativas entre los grupos (p=0,08). Tampoco hubo diferencias significativas entre la media de la edad cronológica y la edad ósea media media (p 0,3). La prueba T para evaluar la dosis de 25-OH colecalciferol al aumentar la dosis de Ergocalciferol fue significativa (T:32.4 Q: <0.001). Hubo una correlación significativa entre los tres grupos: entre la dosis de Vitamina D y el dosaje sérico de Vitamina D de 0,9; entre el DAC y la dosis de 25 OH colecalciferol de 0,62 y entre el DAC y el CMO de 0.44. Por último, el aumento promedio en los tres grupos de dosis de vitamina D fue de 1833UI. Conclusiones: Observamos una relación entre la DAC e hipovitaminosis D, corregible al aumentar la dosis de Vitamina D.

Introduction: steroid treatment for corticosteroid-sensitive nephrotic syndrome (CSNS) could cause bone and mineral metabolism alterations, preventable with calcium and Vitamin D. Objectives: We carried out a preliminary retrospective study along 36 months with the following objectives. 1) To evaluate the relationship between Cumulative Corticosteroid Doses (CCD) and 25-0 Vitamin D serum concentration and with Bone Mineral Content (BMC); 2) To evaluate the relationship between CCD and Bone Mineral Densitomety (BMD). Methods: We included patients between 2 and 12 years of age with corticosteroid sensitive primary nephrotic syndrome (CSNS) (first episode, relapsing nephrotic syndrome, corticosteroid dependent nephrotic syndrome) normotensive, eutrophic and FG>:90ml/min/1.73 m2, who were divided into three groups: GROUP A: =3 or 4 relapses/year, GROUP C: CSNS, we measured: a) Quarterly: calcemia, phosphatemia, alkaline phosphatase; b) half-yearly: 25-OH cholecalcipherol levels, CCD; c) annually BMD in children >6 years (score Z and BMC), bone age, PTHi. Results: We evaluated 29 patients, average age: 4.4 years. The BMD was performed on 11 patients and there were no significant differences among the groups (p=0.08). No significant differences were seen between chronologic age and average bone age (p=0.3). Change in 25-OH cholecalcipherol levels due to the increase of ergocalcipherol dose was significant (T:32.4 Q:<0.001). There were significant correlation in the three groups, between Vitamin D dose and Vitamin D serum levels (Pearson correlation R=0.9), between CCD and 25 OH cholecalcipherol dose: (Pearson correlation R=0.62) and between CCD and BMC (Pearson correlation R=0.44). Finally, in these three groups the average increase of vitamin D was: 1833IU. Conclusions: We found a relationship between CCD and hypovitaminosis D, which could be corrected increasing Vitamin D dose.

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La poliquistosis renal autosómica dominante (PQRAD) se caracteriza por la aparición de quistes renales. Objetivos: Evaluar el crecimiento del volumen renal y de los quistes en los pacientes tratados con sirolimus, en comparación con aquellos que reciben el tratamiento habitual para la PQRAD; evaluar la aparición de efectos adversos relacionados con el uso de sirolimus; evaluar los cambios en la presión arterial, la proteinuria y el filtrado glomerular (FG) estimado. Materiales y métodos: Durante 24 meses se asignaron al azar 12 pacientes con PQRAD a un grupo de tratamiento con sirolimus (6 pacientes recibieron sirolimus 2 a 3 mg/m2/día, con un máximo de 5 mg/día) o a un grupo control (bajo tratamiento habitual). Resultados: De los 12 pacientes, los 6 pertenecientes al grupo de tratamiento con sirolimus tuvieron, al final del estudio, un aumento del volumen renal total y del volumen quístico del 13% y el 32%, respectivamente. En el grupo control, los 6 pacientes restantes tuvieron aumentos del 11% y el 23%, respectivamente. El FG fue normal para ambos grupos. La proteinuria para los grupos de sirolimus y control fue inicialmente 7.3 mg/m2/h y 6 mg/m2/h, respectivamente. Al finalizar el estudio era normal para ambos grupos. Tres pacientes tenían hipertensión arterial, pero a los 24 meses la presión arterial fue normal. Los efectos adversos observados fueron: anemia, diarrea y úlceras bucales. Conclusiones: El sirolimus no disminuyó el volumen renal ni el quístico. No hubo un aumento significativo en la proteinuria o una disminución en el FG. La media de la presión arterial se mantuvo normal.

Autosomal Dominant Polycystic Kidney Disease (ADPKD) is characterized by the develop-ment of kidney cysts. Objectives: To assess the growth of kidney and cystic volume in patients treated with rapamycin compared with patients receiving the usual treatment for ADPKD; assess the occurrence of adverse effects associated with the use of sirolimus; evaluate changes in blood pressure, proteinuria and estimated glomerular filtration rate (eGFR). Materials and methods: For a 24-month period, 12 patients with ADPKD were randomly allocated to a sirolimus group (6 patients, received rapamycin, 2-3 mg/m2/day, max. 5 mg/day) and the other 6 patients were allocated to a control group (receiving conventional treatment). Results: Out of the 12 patients, the 6 patients in the sirolimus group had a total increase in kidney and cystic volume of 13% and 32%, respectively, by the end of the study. In the control group, the other 6 patients had increases of 11% and 23%, respectively. eGFR was normal in both groups. Baseline proteinuria was 7.3 mg/m2/hour and 6 mg/m2/hour in the sirolimus- and control groups, respectively. By the end of the study, it was normal in both groups. Three patients had high blood pressure at baseline, but it was normalized at 24 months. Adverse effects were: anemia, diarr-hea, and mouth sores. Conclusions: Sirolimus did not reduce kidney and cystic volume. No significant increase in proteinuria or decrease in eGFR were observed. Mean blood pressure remained normal.

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CONTEXT: Two Argentinean siblings (a boy and a girl) from a nonconsanguineous family presented with hypercalcemia, hypercalciuria, hypophosphatemia, low parathyroid hormone (PTH), and nephrocalcinosis. OBJECTIVE: The goal of this study was to identify genetic causes of the clinical findings in the two siblings. DESIGN: Whole exome sequencing was performed to identify disease-causing mutations in the youngest sibling, and a candidate variant was screened in other family members by Sanger sequencing. In vitro experiments were conducted to determine the effects of the mutation that was identified. PATIENTS AND OTHER PARTICIPANTS: Affected siblings (2 y.o. female and 10 y.o male) and their parents were included in the study. Informed consent was obtained for genetic studies. RESULTS: A novel homozygous mutation in the gene encoding the renal sodium-dependent phosphate transporter SLC34A1 was identified in both siblings (c.1484G>A, p.Arg495His). In vitro studies showed that the p.Arg495His mutation resulted in decreased phosphate uptake when compared to wild-type SLC34A1. CONCLUSIONS: The homozygous G>A transition that results in the substitution of histidine for arginine at position 495 of the renal sodium-dependent phosphate transporter, SLC34A1, is involved in disease pathogenesis in these patients. Our report of the second family with two mutated SLC34A1 alleles expands the known phenotype of this rare condition.

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UNLABELLED: Idiopathic hypercalciuria may be associated with urinary tract infection, hematuria, nephrolithiasis and osteopenia. In order to describe the occurrence of these concurrent conditions related to the variation in urinary calcium and hypercalciuria response to sequential therapy, with a normal protein and low sodium diet, potassium citrate and hydrochlorothiazide; 46 patients older than 4 years, with no urinary tract diseases, sphincter control and normal blood creatinine values were followed-up during 43 months. Hypercalciuria was seen to be associated with kidney stones (EAR 47%; RR 3.3), hematuria (EAR 71%; RR 2.5), urinary tract infections (EAR 57%; RR 3), and osteopenia (EAR 33%; RR 3). A normal value of urinary calcium was achieved with sequential therapy in 43 patients, but during follow-up 32 patients discontinued treatment and hypercalciuria recurred in 44% of them, in association with hematuria and urinary tract infection. CONCLUSIONS: Sequential therapy reduced hypercalciuria and the incidence of associated diseases.

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Idiopathic hypercalciuria may be associated with urinary tract infection, hematuria, nephrolithiasis and osteopenia. In order to describe the occurrence of these concurrent conditions related to the variation in urinary calcium and hypercalciuria response to sequential therapy, with a normal protein and low sodium diet, potassium citrate and hydrochlorothiazide; 46 patients older than 4 years, with no urinary tract diseases, sphincter control and normal blood creatinine values were followed-up during 43 months. Hypercalciuria was seen to be associated with kidney stones (EAR 47%; RR 3.3), hematuria (EAR 71%; RR 2.5), urinary tract infections (EAR 57%; RR 3), and osteopenia (EAR 33%; RR 3). A normal value of urinary calcium was achieved with sequential therapy in 43 patients, but during follow-up 32 patients discontinued treatment and hypercalciuria recurred in 44% of them, in association with hematuria and urinary tract infection. Conclusions. Sequential therapy reduced hypercalciuria and the incidence of associated diseases.

La hipercalciuria idiopática puede asociarse con infección urinaria, hematuria, nefrolitiasis y osteopenia; con los objetivos de describir la aparición de estas patologías concurrentes, relacionadas con la variación del calcio urinario y la respuesta al tratamiento secuencial de la hipercalciuria, con dieta normoproteica-hiposódica, citrato de potasio e hidroclorotiazida, controlamos durante 43 meses a 46 pacientes mayores de 4 años, sin uropatías, con control esfinteriano y creatininemias normales. Observamos que la hipercalciuria se asoció con litiasis renal (RAE 47%; RR 3,3); hematuria (RAE 71%; RR 2,5); infecciones urinarias (RAE 57%; RR 3) y osteopenia (RAE 33%; RR 3). La terapéutica secuencial normalizó el calcio urinario en 43 pacientes, pero durante el seguimiento, 32 interrumpieron el tratamiento y la hipercalciuria reapareció en 44% de ellos, asociada a hematuria e infección urinaria. Conclusiones. El tratamiento secuencial redujo la hipercalciuria y disminuyó la incidencia de patologías asociadas.

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Idiopathic hypercalciuria may be associated with urinary tract infection, hematuria, nephrolithiasis and osteopenia. In order to describe the occurrence of these concurrent conditions related to the variation in urinary calcium and hypercalciuria response to sequential therapy, with a normal protein and low sodium diet, potassium citrate and hydrochlorothiazide; 46 patients older than 4 years, with no urinary tract diseases, sphincter control and normal blood creatinine values were followed-up during 43 months. Hypercalciuria was seen to be associated with kidney stones (EAR 47%; RR 3.3), hematuria (EAR 71%; RR 2.5), urinary tract infections (EAR 57%; RR 3), and osteopenia (EAR 33%; RR 3). A normal value of urinary calcium was achieved with sequential therapy in 43 patients, but during follow-up 32 patients discontinued treatment and hypercalciuria recurred in 44% of them, in association with hematuria and urinary tract infection. Conclusions. Sequential therapy reduced hypercalciuria and the incidence of associated diseases.(AU)

La hipercalciuria idiopática puede asociarse con infección urinaria, hematuria, nefrolitiasis y osteopenia; con los objetivos de describir la aparición de estas patologías concurrentes, relacionadas con la variación del calcio urinario y la respuesta al tratamiento secuencial de la hipercalciuria, con dieta normoproteica-hiposódica, citrato de potasio e hidroclorotiazida, controlamos durante 43 meses a 46 pacientes mayores de 4 años, sin uropatías, con control esfinteriano y creatininemias normales. Observamos que la hipercalciuria se asoció con litiasis renal (RAE 47%; RR 3,3); hematuria (RAE 71%; RR 2,5); infecciones urinarias (RAE 57%; RR 3) y osteopenia (RAE 33%; RR 3). La terapéutica secuencial normalizó el calcio urinario en 43 pacientes, pero durante el seguimiento, 32 interrumpieron el tratamiento y la hipercalciuria reapareció en 44% de ellos, asociada a hematuria e infección urinaria. Conclusiones. El tratamiento secuencial redujo la hipercalciuria y disminuyó la incidencia de patologías asociadas.(AU)

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UNLABELLED: Idiopathic hypercalciuria may be associated with urinary tract infection, hematuria, nephrolithiasis and osteopenia. In order to describe the occurrence of these concurrent conditions related to the variation in urinary calcium and hypercalciuria response to sequential therapy, with a normal protein and low sodium diet, potassium citrate and hydrochlorothiazide; 46 patients older than 4 years, with no urinary tract diseases, sphincter control and normal blood creatinine values were followed-up during 43 months. Hypercalciuria was seen to be associated with kidney stones (EAR 47

; RR 3.3), hematuria (EAR 71

; RR 2.5), urinary tract infections (EAR 57

; RR 3), and osteopenia (EAR 33

; RR 3). A normal value of urinary calcium was achieved with sequential therapy in 43 patients, but during follow-up 32 patients discontinued treatment and hypercalciuria recurred in 44

of them, in association with hematuria and urinary tract infection. CONCLUSIONS: Sequential therapy reduced hypercalciuria and the incidence of associated diseases.

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La persistencia de la proteinuria nefrótica favorece la progresión hacia la insuficiencia renal. Hemos diseñado un protocolo terapéutico con sirolimus para ese grupo de pacientes implementando un estudio clínico prospectivo, intervencionista y no aleatorizado, sobre una cohorte de 13 pacientes con una edad media de 10 años; todos con síndrome nefrótico córtico-resistente primario y glomerulosclerosis focal y segmentaria; resistentes también a la ciclofosfamida, a los inhibidores de la calcineurina y al empleo de enalapril y losartán. La dosis media empleada de sirolimus fue de 3,6 mg/m2/día y el tratamiento duró 12 meses. Evaluamos la eficacia terapéutica acorde a la reducción de la proteinuria (respuesta total, parcial y ausente), y también fueron evaluadas la severidad del daño histológico pretratamiento y el tiempo previo transcurrido hasta recibir el sirolimus. Nueve de los trece pacientes tuvieron remisión parcial o total del síndrome nefrótico, y el tiempo medio previo transcurrido y la severidad del daño histológico influyeron en el tipo de respuesta. Consideramos que el sirolimus es una opción válida de tratamiento para los pacientes con síndrome nefrótico córtico-resistente, aunque probablemente sea necesario un inicio terapéutico más precoz (AU)

Persistent nephrotic syndrome that does not respond to treatment may cause progression to kidney failure. We designed a therapeutic protocol with sirolimus for this group of patients. We conducted a prospective, interventional, time series, cohort study lasting 20 months. Thirteen patients were enrolled, with a mean age of 10 years (range: 8-18 years old) with steroid-resistant primary nephrotic syndrome and a histological diagnosis of focal and segmental glomerulosclerosis. We administered sirolimus 3.6mg/m2/day. The duration of this regimen was 12 months in responsive patients. The protocol's efficacy was assessed according to reduction of proteinuria (3 response levels: total, partial, or no response). Severity of histological renal damage and mean time from clinical diagnosis to protocol initiation were also assessed. Nine of 13 patients responded to the treatment with sirolimus, and mean progression time and the severity of histological renal damage influenced response to therapy. We believe that sirolimus is a valid treatment option in patients with steroid-resistant nephrotic syndrome, even though this regimen probably requires an earlier treatment (AU)

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Persistent nephrotic syndrome that does not respond to treatment may cause progression to kidney failure. We designed a therapeutic protocol with sirolimus for this group of patients. We conducted a prospective, interventional, time series, cohort study lasting 20 months. Thirteen patients were enrolled, with a mean age of 10 years (range: 8-18 years old) with steroid-resistant primary nephrotic syndrome and a histological diagnosis of focal and segmental glomerulosclerosis. We administered sirolimus 3.6mg/m2/day. The duration of this regimen was 12 months in responsive patients. The protocol's efficacy was assessed according to reduction of proteinuria (3 response levels: total, partial, or no response). Severity of histological renal damage and mean time from clinical diagnosis to protocol initiation were also assessed. Nine of 13 patients responded to the treatment with sirolimus, and mean progression time and the severity of histological renal damage influenced response to therapy. We believe that sirolimus is a valid treatment option in patients with steroid-resistant nephrotic syndrome, even though this regimen probably requires an earlier treatment.

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Introducción: Los pacientes adolescentes representan un desafío para el nefrólogo. Aunque la patología renal del mismo se asemeja más a la del adulto, tiene una epidemiología única a considerar. Métodos: Estudio descriptivo, retrospectivo. Se relevaron 43 historias clínicas de pacientes entre 10 Y 18 anos ingresados por primera vez en nefrología entre enero de 2009 y mayo de 2011, analizando datos demográficos, motivo de consulta, lugar de referencia, tiempo entre inicio de síntomas y la referencia al servicio, días de internación, diagnóstico de enfermedad renal y causas de ingreso a diálisis. Resultados: Se internaron 394 pacientes, 136 fueron adolescentes. 43 internaciones de primera vez, y de estos, 24 mujeres. Edad promedio 13,5 años ± 2,37. 27 pacientes de provincia de Bs. As., 11 CABA, y 5 resto del País. 27 derivados desde nuestro propio hospital y 16 de otros centros médicos. Los síntomas y signos más frecuentes fueron hematuria, edemas, proteinuria y disminución del filtrado glornerular. La media entre inido de síntomas y la consulta fue 15 días (rango 1-30). Promedio de días de internación 13,7 (rango 2-95). Las patologías prevalentes fueron las glomerulopatias y la nefropatía lúpica la enfermedad renal más frecuente. 11 pacientes requirieron diálisis, y 9 permanentemente. Conclusiones: Los adolescentes presentan prevalencia patológica diferente a la de pediatría. La presencia de IRCT sin diagnóstico previo fue significativa.

Introduction: Teenager patients represent a challenge for Nephrologists. Although renal pathology of such patients is similar to adults, it has a unique epidemiology which must be considered. Methods: Retrospective, descriptive study. 43 clinical histories of patients between 10 and 18 years old admitted for the first time in Nephrology division between January 2009 and May 2011 were reported. We analyzed demographic data, consultation cause, reference place, time between the beginning of symptoms and the reference to the division, days of hospitalization, renal disease diagnosis and dialysis admission causes. Results: 394 patients were admitted, 136 were teenagers, 43 admissions for the first time and from these 24 women. Average age 13,5 years old +-2,37.27 patients from Buenos Aires province, 11 Capital City of Buenos Aires and 5 from the rest of the country. 27 referred from our own hospital and 16 from other medical centers. The 139 most frequent symptoms and signs were hematuria, edema, proteinuria and decrease of glomerular filtration. The average between the beginning of symptoms and consultation was 15 days (range1-30). Admission day average 13,7(range 2-95). Prevalent pathologies were glomerulopathies and lupic nephropathy the most frequent renal disease. 11 patients required dialysis. Conclusions: Teenagers show different pathological evidence in comparison to pediatrics. The presence of IRCT without previous diagnosis was significant.

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Introducción: Los pacientes adolescentes representan un desafío para el nefrólogo. Aunque la patología renal del mismo se asemeja más a la del adulto, tiene una epidemiología única a considerar. Métodos: Estudio descriptivo, retrospectivo. Se relevaron 43 historias clínicas de pacientes entre 10 Y 18 anos ingresados por primera vez en nefrología entre enero de 2009 y mayo de 2011, analizando datos demográficos, motivo de consulta, lugar de referencia, tiempo entre inicio de síntomas y la referencia al servicio, días de internación, diagnóstico de enfermedad renal y causas de ingreso a diálisis. Resultados: Se internaron 394 pacientes, 136 fueron adolescentes. 43 internaciones de primera vez, y de estos, 24 mujeres. Edad promedio 13,5 años ± 2,37. 27 pacientes de provincia de Bs. As., 11 CABA, y 5 resto del País. 27 derivados desde nuestro propio hospital y 16 de otros centros médicos. Los síntomas y signos más frecuentes fueron hematuria, edemas, proteinuria y disminución del filtrado glornerular. La media entre inido de síntomas y la consulta fue 15 días (rango 1-30). Promedio de días de internación 13,7 (rango 2-95). Las patologías prevalentes fueron las glomerulopatias y la nefropatía lúpica la enfermedad renal más frecuente. 11 pacientes requirieron diálisis, y 9 permanentemente. Conclusiones: Los adolescentes presentan prevalencia patológica diferente a la de pediatría. La presencia de IRCT sin diagnóstico previo fue significativa.(AU)

Introduction: Teenager patients represent a challenge for Nephrologists. Although renal pathology of such patients is similar to adults, it has a unique epidemiology which must be considered. Methods: Retrospective, descriptive study. 43 clinical histories of patients between 10 and 18 years old admitted for the first time in Nephrology division between January 2009 and May 2011 were reported. We analyzed demographic data, consultation cause, reference place, time between the beginning of symptoms and the reference to the division, days of hospitalization, renal disease diagnosis and dialysis admission causes. Results: 394 patients were admitted, 136 were teenagers, 43 admissions for the first time and from these 24 women. Average age 13,5 years old +-2,37.27 patients from Buenos Aires province, 11 Capital City of Buenos Aires and 5 from the rest of the country. 27 referred from our own hospital and 16 from other medical centers. The 139 most frequent symptoms and signs were hematuria, edema, proteinuria and decrease of glomerular filtration. The average between the beginning of symptoms and consultation was 15 days (range1-30). Admission day average 13,7(range 2-95). Prevalent pathologies were glomerulopathies and lupic nephropathy the most frequent renal disease. 11 patients required dialysis. Conclusions: Teenagers show different pathological evidence in comparison to pediatrics. The presence of IRCT without previous diagnosis was significant.(AU)

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BACKGROUND: Haemolytic uraemic syndrome (HUS) is characterized by haemolytic anaemia, thrombocytopaenia and acute renal failure. The aim of this study was to investigate the levels of oxidative stress (OS) during the acute phase of HUS. METHODS: This prospective study included 18 patients diagnosed with D + HUS, 6 age-matched healthy controls and 29 children with end-stage renal disease (ESRD) not caused by HUS under regular haemodialysis. Plasma lipid peroxidation and non-enzymatic antioxidant defences were measured as thiobarbituric acid-reactive substances (TBARs) and total reactive antioxidant potential (TRAP), respectively, during hospitalization and in control individuals. RESULTS: TBARs were significantly higher in both oliguric and non-oliguric patients at admission (1.8 ± 0.1; 1.7 ± 0.2 µM) and discharge (1.5 ± 0.1; 1.0 ± 0.1 µM) vs controls (0.5 ± 0.1 µM, P < 0.01) following disease progression. Maximal TBARs values differed significantly between oliguric and non-oliguric groups (4.5 ± 0.9 vs 2.4 ± 0.3 µM, P < 0.01) and were significantly higher (P < 0.05) than those found in ESRD patients (1.63 ± 0.1). TRAP values were significantly higher at admission and when the disease was fully established (measured here as highest TBARs record) vs controls (675 ± 51, 657 ± 60 and 317 ± 30 µM Trolox, P < 0.01), and were similar to control values at discharge (325 ± 33 µM Trolox). CONCLUSIONS: We demonstrate here increased levels of OS during the acute phase of HUS, with peak plasma lipid peroxidation values well above those registered in ESRD individuals, and suggest a connection between OS and the clinical course of HUS.

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