Background and objectives Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and outcome studies, hampering the development of evidence-based diagnostic and therapeutic concepts. To overcome these limitations, the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome. Design, setting, participants, & measurements Since August of 2009, clinical, biochemical, genetic, and histopathologic information was collected both retrospectively and prospectively from 1655 patients with childhood-onset steroid-resistant nephrotic syndrome, congenital nephrotic syndrome, or persistent subnephrotic proteinuria of likely genetic origin at 67 centers in 21 countries through an online portal. Results Steroid-resistant nephrotic syndrome manifested in the first 5 years of life in 64% of the patients. Congenital nephrotic syndrome accounted for 6% of all patients. Extrarenal abnormalities were reported in 17% of patients. The most common histopathologic diagnoses were FSGS (56%), minimal change nephropathy (21%), and mesangioproliferative GN (12%). Mutation screening was performed in 1174 patients, and a genetic disease cause was identified in 23.6% of the screened patients. Among 14 genes with reported mutations, abnormalities in NPHS2 (n=138), WT1 (n=48), and NPHS1 (n=41) were most commonly identified. The proportion of patients with a genetic disease cause decreased with increasing manifestation age: from 66% in congenital nephrotic syndrome to 15%–16% in schoolchildren and adolescents. Among various intensified immunosuppressive therapy protocols, calcineurin inhibitors and rituximab yielded consistently high response rates, with 40%–45% of patients achieving complete remission. Confirmation of a genetic diagnosis but not the histopathologic disease type was strongly predictive of intensified immunosuppressive therapy responsiveness. Post-transplant disease recurrence was noted in 25.8% of patients without compared with 4.5% (n=4) of patients with a genetic diagnosis. Conclusions The PodoNet cohort may serve as a source of reference for future clinical and genetic research in this rare but significant kidney disease.

Chronic kidney disease (CKD) is an overwhelming illness in children. Considering the importance of CKD in pediatric age group, in addition to the racial and ethnic differences in prevalence and etiology of CKD, epidemiologic studies are recommended. The majority of the results regarding the incidence of CKD have been achieved from studies on hospitalized children. This study evaluates the incidence and etiology of chronic renal failure (CRF) in children in a tertiary care center in Isfahan, the second large province of Iran. A retrospective analysis of medical record data of children less than 19 years old, hospitalized for CKD at St Alzahra hospital, Isfahan, Iran, during the period of November 2001 until December 2011 was made. A total of 268 eligible cases were recruited. Patients were categorized into 5 groups according to glomerular filtration rate (GFR) and KDOQI guideline. Out of 268 patients, 144 patients were male (54%). The mean and median of age was 11.01± 0.39(SD) years and 12.5 years, respectively. Approximately 55% of patients had either systolic and/or diastolic hypertension. The most frequent etiology of CKD was glomerular diseases (34%) followed by reflux nephropathy (16.7%). Most patients (74.8%) had GFR less than 15 ml/min/m², when diagnosed. Anemia was the most prevalent laboratory abnormality (85%). Small-size kidneys were seen in 65.8% of patients. Kidney replacement therapy (either hemo- or peritoneal dialysis) was applied for 72.3% of the patients. The transplantation rate average was 7.2 per million children population. The annual incidences of CKD (mostly advanced stages) were 5.52 per million population (PMP) and 16.8 per million children population, respectively. The all cause of mortality rate was 18.4%. Younger age was the only independent predictor of mortality (p=0.005). We concluded that glomerular diseases are the leading cause of CKD in our patients. In addition, the annual incidence of CKD is considerably high.

Investigations have attempted to modify the outcome of tubular injury by either ameliorating renal tubular damage or promoting tubular regeneration in the case of acute tubular necrosis. We investigated the protective effect of Eprex an erythropoietin analogue on tubular injury induced by gentamicin (GM). Forty male Wistar rats were randomly divided into four groups. In group 1,rats were served as a sham group. In group 2, rats were injected intraperitoneally with 100 mg/kg of GM for 10 consecutive days (positive control group) and then were sacrificed. In group 3, rats received GM for 10 days then Eprex 100U/kg was injected intraperitoneally for the next 10 days and then they were sacrificed at the day 20th. In group 4 rats were injected a combination of GM (80 mg/kg) and Eprex 100U/kg intraperitoneally for 10 days and then were sacrificed. The results indicated that, Eprex prevented the increase in serum creatinine (Cr) and blood urea nitrogen (BUN). The effect of Eprex on damage score, showed that co-administration of GM and Eprex (group 3 and 4) reduced the kidney tissue damage compared to positive control group (P<0.05). This result indicat that Eprex potentially can reduce or prevent the kidney tissue damage. Ameliorative effect of Eprex when the drug was given in combination with GM and also when the drug was applied after GM-induced tubular damage, revealed the renoprotective potency of Eprex. Eprex is a promising drug to prevent or attenuate tubular damage induced by GM or other nephrotoxic agents which act through the same mechanisms as gentamicin.

The etiology of acute kidney injury (AKI) varies in different countries. In addition, the etiology of AKI in hospitalized children is multifactorial. The importance of diagnosing AKI is not only because of short-term high morbidity and mortality rate, but also for its effect on developing chronic kidney disease. we studied retrospectively AKIs of children who were hospitalized over 10 years in a University hospital. A retrospective analysis of the medical recorded data of 180 children less than 18 years treated for AKI in Alzahra Hospital, Isfahan, Iran, were performed during the period of March 2001 to February 2011. For each patient, demographic and anthropometric data, laboratory data, electrocardiographic findings, ultrasound results, etiology of AKI and short-term outcomes were recorded. The male to female ratio was 1.57 to 1. Mean age was 5.28 ± 6.3 (SD) years and the median was 1.8 years. The more frequent age group was children less than 2 years. The mortality rate was 22.2% (40 patients). The mortality was not correlated with age (p= 0.74). Renal replacement therapy was recommended for 62 patients (34.4%). Mean of the first and last glomerular filtration rate (GFR) were 18.33± 1.12 ml/min/1.73 m² and 52.53 ± 2.98 ml/min/1.73 m², respectively. The most common urinary sediment finding in approximately 70% of the patients was either renal epithelial cell or renal cell cast. Increased kidney echogenicity was the most common ultrasound finding (48%). Using ANOVA regression analysis, the etiology of disease was the only predictor of mortality (p=0.0001). We concluded that the mortality is still high in AKI. Furthermore, the poor outcome (defined as low GFR) are higher among patients with low levels of first GFR and higher RIFLE score.

Background: Chronic renal failure (CRF) causes a gradual decline in kidney function to the extent that CRF patients need long-term clinical care, which affects the patients’ family function and quality of life (QoL). Objectives: The present study was conducted to study the effects of the family-centered empowerment model on QoL in children with CRF during 2012-2013. Patients and Methods: In this quasi-experimental study, 68 children with CRF and their parents were randomly assigned to two groups, intervention and control, via a random numbers table. An empowerment program was then conducted over the course of seven 45-minute sessions, and a questionnaire to ascertain demographic characteristics and the core pediatric QoL Inventory (version 4) were administered to both groups before the sessions and one month after the last training session. The data were analyzed using SPSS 20. Results: The mean age of the children was 10.2 and 10.5 years in the intervention and control groups, respectively. The duration of the disease was five years in both groups. Furthermore, a significant difference was seen in the mean score of the children’s QoL from their own perspectives in the physical and psychosocial domains and the total QoL score in the intervention group before and after the training (P < 0.05). Conclusions: Since family-centered empowerment interventions can determine the training- and treatment-related needs of patients and are low cost and effective, they may help parents promote their children’s self-efficacy and QoL. Keywords: Quality of Life; Chronic Renal Failure; Child; Family Empowerment