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.

The aim of this prospective, multicenter study was to define the etiology and clinical features of acute kidney injury (AKI) in a pediatric patient cohort and to determine prognostic factors. Pediatric-modified RIFLE (pRIFLE) criteria were used to classify AKI. The patient cohort comprised 472 pediatric patients (264 males, 208 females), of whom 32.6% were newborns (median age 3 days, range 1-24 days), and 67.4% were children aged >1 month (median 2.99 years, range 1 month-18 years). The most common medical conditions were prematurity (42.2%) and congenital heart disease (CHD, 11.7%) in newborns, and malignancy (12.9%) and CHD (12.3%) in children aged >1 month. Hypoxic/ischemic injury and sepsis were the leading causes of AKI in both age groups. Dialysis was performed in 30.3% of newborns and 33.6% of children aged >1 month. Mortality was higher in the newborns (42.6 vs. 27.9%; p < 0.005). Stepwise multiple regression analysis revealed the major independent risk factors to be mechanical ventilation [relative risk (RR) 17.31, 95% confidence interval (95% CI) 4.88-61.42], hypervolemia (RR 12.90, 95% CI 1.97-84.37), CHD (RR 9.85, 95% CI 2.08-46.60), and metabolic acidosis (RR 7.64, 95% CI 2.90-20.15) in newborns and mechanical ventilation (RR 8.73, 95% CI 3.95-19.29), hypoxia (RR 5.35, 95% CI 2.26-12.67), and intrinsic AKI (RR 4.91, 95% CI 2.04-11.78) in children aged >1 month.

Herein the results of a multicenter study from the Turkish Pediatric Kidney Transplantation Study Group are reported. The aims of this study were to compare the quality of life (QoL) scores of Turkish children who are dialysis patients (DP), renal transplant recipients (TR), and age-matched healthy controls and to compare child-self and parent-proxy scores. The Turkish versions of the Kinder Lebensqualität Fragebogen (KINDL(R)) questionnaires were used as a QoL measure. The study group consisted of 211 children and adolescents with chronic kidney disease (CKD) (139 TR and 72 DP aged between 4-18 years; 13.7 +/- 3.5 years) from 11 university hospitals, 129 parents of these patients, 232 age-matched healthy children and adolescents (aged between 4-18 years; 13.1+/-3.5 years) and 156 of their parents. Patients with CKD had lower scores in all subscales except for physical well-being than those in the control group. TR had higher scores in physical well-being, self-esteem, friends' subscales, and total scores than DP. Child-self scores were lower than parent-proxy scores, especially in CKD, DP, and control groups. Concordance between parent-proxy and child-self reports in the TR, DP, CKD, and control groups was only moderate for the majority of subscales (r = 0.41-0.61). It was concluded that parent-proxy scores on the QoL were not equivalent to child-self scores and that evaluating both children's and parents' perspectives were important. Additionally, psychosocial counseling is crucial not only for patients with CKD but also for their parents.

Smaller instrument size to 20Fr did not significantly increase the operative time, and resulted in the same success rates as the adult sized devices. However, low blood transfusion rates were only reached in the minimal access percutaneous nephrolithotomy group.

Genetic screening paradigms for congenital and infantile nephrotic syndrome are well established; however, screening in adolescents has received only minor attention. To help rectify this, we analyzed an unselected adolescent cohort of the international PodoNet registry to develop a rational screening approach based on 227 patients with nonsyndromic steroid-resistant nephrotic syndrome aged 10-20 years. Of these, 21% had a positive family history. Autosomal dominant cases were screened for WT1, TRPC6, ACTN4, and INF2 mutations. All other patients had the NPHS2 gene screened, and WT1 was tested in sporadic cases. In addition, 40 sporadic cases had the entire coding region of INF2 tested. Of the autosomal recessive and the sporadic cases, 13 and 6%, respectively, were found to have podocin-associated nephrotic syndrome, and 56% of them were compound heterozygous for the nonneutral p.R229Q polymorphism. Four percent of the sporadic and 10% of the autosomal dominant cases had a mutation in WT1. Pathogenic INF2 mutations were found in 20% of the dominant but none of the sporadic cases. In a large cohort of adolescents including both familial and sporadic disease, NPHS2 mutations explained about 7% and WT1 4% of cases, whereas INF2 proved relevant only in autosomal dominant familial disease. Thus, screening of the entire coding sequence of NPHS2 and exons 8-9 of WT1 appears to be the most rational and cost-effective screening approach in sporadic juvenile steroid-resistant nephrotic syndrome.

Percutaneous nephrolithotomy in children is safe, feasible and effective. Stone burden and operative time are independent risk factors for complications. The modified Clavien system provides a straightforward and validated method to classify postoperative complications.

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This is the largest multicenter study defining the demographic data for childhood vasculitides. The distribution of childhood vasculitides was different in our population where KD is much less frequent, whereas HSP constitutes an overwhelming majority. C-PAN was more frequent as well.