Approximately 10-20% of children and 40% of adults with idiopathic nephrotic syndrome are steroid resistant and progress to end-stage renal disease requiring dialysis or renal transplantation. In these cases, renal histology typically shows focal segmental glomerulosclerosis. Mutations in NPHS1, NPHS2, WT1, CD2AP and ACTN4 genes located on different chromosomes, expressed by glomerular podocyte...

The management of patients with steroid-resistant nephrotic syndrome remains difficult. We repport our experience with Cyclophosphamide therapy, in an attempt to compare the results between an oral protocol and two i.v. protocols. The complete and sustained general remission rate was 43.1%, which confirms the efficacy of the treatment, especially for children with minimal change nephrotic syndr...

There are limited studies on long-term outcomes of childhood onset nephrotic syndrome (NS). A majority of children with NS have steroid-sensitive nephrotic syndrome (SSNS). Steroid-resistant nephrotic syndrome (SRNS) is associated with a high risk of developing end-stage renal disease. Biomarkers and analysis of genetic mutations may provide new information for prognosis in SRNS. Frequently rel...

BACKGROUND The mechanism of steroid resistance in children with the nephrotic syndrome is yet unknown. About 20% of patients demonstrate steroid unresponsiveness and progress to end stage renal disease. Aberrant SOCS3 and SOCS5 expression in steroid resistant and sensitive patients has previously been demonstrated. Here, we investigate genetic and epigenetic mechanisms of regulation of SOCS3 an...

BACKGROUND Nephrotic patients are prone to atherosclerosis in consequence of frequent exposures to hyperlipidemia, hypertension, and immunosuppressive drugs. OBJECTIVES We studied the carotid parameters as early indicators of atherosclerosis in children with nephrotic syndrome. METHODS Between 2008 and 2011, 51 children with history of nephrotic syndrome enrolled in the study. The inclusion...

background: nephrotic syndrome and type 1 diabetes mellitus are two common chronic conditions in children. co-existence of these conditions is an extremely rare finding. case presentation: this report presents a 6-year-old boy who was diagnosed with nephrotic syndrome five years ago and type 1 diabetes mellitus in infancy. renal biopsy showed membranous glomerulonephritis, which is a rare histo...

The term 'minimal change nephrotic syndrome' has become synonymous with the expression 'steroid responsive nephrotic syndrome', the latter term having the merit of focusing on the most important objective characteristic of the condition. Nevertheless, it is somewhat illogical to define this condition by a negative histological feature, which in the majority of cases remains a presumption inferr...

The therapeutic approach to childhood nephrotic syndrome is based on a series of studies that began with an international collaborative effort sponsored by the International Study of Kidney Disease in Children in 1967. The characteristics of children presenting with nephrotic syndrome have changed over recent decades with greater frequency of the challenging condition focal segmental glomerulos...

A chronic, progressive disorder Steroid Resistant Nephrotic Syndrome (SRNS) accounts for 10-20% of all children with Nephrotic Syndrome. It is a heterogeneous disorder comprised of persistent edema, proteinuria, hypoalbuminemia and hyperlipidemia. Treatment for steroid-resistant nephrotic syndrome (SRNS) is challenging and children who suffer from SRNS require aggressive treatment to achieve re...