Background NUP107 is a novel gene associated with autosomal recessive steroid-resistant nephrotic syndrome (SRNS) with focal segmental glomerulosclerosis (FSGS) in children. The frequency of NUP107 mutations in children with SR-FSGS remains unknown. Methods Nine families with two siblings affected by childhood-onset SRNS or proteinuria were recruited. FSGS was confirmed by a kidney biopsy in ...

BACKGROUND The study was undertaken to develop a potential new markers for distinguishing minimal change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS) in children. We hypothesized that matrix metalloproteinase-9/neutrophil gelatinase-associated lipocalin (MMP-9/NGAL) is a better marker of focal sclerosis in the glomerulus then matrix metalloproteinase-9/tissue inhibito...

Previous literature suggests that the recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation is more common in recipients who have received an HLA-identical living-related (LRD) transplant. To address the question if FSGS patients can safely receive a 6-antigen match LRD kidney transplant, we analyzed death-censored renal allograft survival data of FSGS patients fro...

Focal segmental glomerulosclerosis (FSGS) is one of the principal causes of end-stage kidney disease in children worldwide (1-3). During the past decades, different genes mutations have been implicated in pathogenesis of resistant sporadic and hereditary familial forms of FSGS. Nephrin, podocin, alpha-actinin 4 (ACTN4) are some of podocyte structural proteins that their role in pathogenesis of ...

PURPOSE To quantify the hemodynamic impact of a flared renal stent on the performance of fenestrated stent-grafts (FSGs) by analyzing flow patterns and wall shear stress-derived parameters in flared and nonflared FSGs in different physiologic scenarios. METHODS Hypothetical models of FSGs were created with and without flaring of the proximal portion of the renal stent. Flared FSGs with differ...

The clinical course of a young female patient with focal segmental glomerulosclerosis (FSGS) who failed to respond to any of the recommended therapeutic protocols will represent the background of a discussion of currently available alternative treatments for FSGS. Traditionally, FSGS has been believed to have a poor prognosis, with a low response rate to treatment and a progressive course termi...

BACKGROUND The current treatment regimes for patients with nephrotic syndrome due to idiopathic membranous nephropathy (MN) and focal segmental glomerulosclerosis (FSGS) are based on steroids and/or cytotoxic agents. Data on the effect of mycophenolate mofetil (MMF) for these conditions are scarce and confounding. METHODS We compared the efficacy of an MMF-based therapy with standard therapie...

Malone et al. performed next-generation sequencing on 70 families with focal segmental glomerulosclerosis (FSGS) and discovered that 10% had variants in surprising 'old' genes, COL4A3 and COL4A4, which are involved in Alport syndrome and thin basement membrane nephropathy. These data show that a subset of renal manifestations associated with COL4A3 or COL4A4 variants cannot be distinguished fro...