Focal segmental glomerular sclerosis (FSGS) is a pathological term to indicate glomerular lesions associated with distinctive clinical features. In most cases, FSGS is primary in nature and is called idiopathic. Idiopathic FSGS is often associated with a nephrotic syndrome (NS) and may affect both children and adults. While the prognosis of FSGS is relatively good for patients with subnephrotic...

BACKGROUND Primary focal segmental glomerulosclerosis (FSGS) is pathological entity which is characterized by idiopathic steroid-resistant nephrotic syndrome (SRNS) and progression to end-stage renal disease (ESRD) in the majority of affected individuals. Currently, there is no practical noninvasive technique to predict different pathological types of glomerulopathies. In this study, the role o...

INTRODUCTION Some evidence suggest an increase in the prevalence of focal segmental glomerular sclerosis (FSGS) in children. To date, there has been no study of the outcome in children with FSGS and its frequency over several decades in Iran. We aimed to report the changing trend of FSGS incidence and its outcome in a sample of Iranian children. MATERIALS AND METHODS Between 1982 and 2008, al...

FSGS is a podocyte-derived glomerular disease characterized by marked proteinuria, often coupled with steroid resistance, hypertension, and high probability for progression to renal failure. It is a heterogeneous disorder that can present as a primary disorder of unknown etiology. A number of genetic mutations in various proteins have been linked to the development of FSGS. Finally, the glomeru...

Mutations in the inverted formin 2 gene (INF2) have recently been identified as the most common cause of autosomal dominant focal and segmental glomerulosclerosis (FSGS). To quantify the contribution of various genes contributing to FSGS, we sequenced INF2 where all mutations have previously been described (exons 2 to 5) in a total of 215 probands and 281 sporadic individuals with FSGS, along w...

AIM To determine the role of immunoglobulin M (IgM) deposits in clinical manifestations, disease outcome, and treatment response of idiopathic and secondary focal segmental glomerulosclerosis (FSGS). METHODS Kidney biopsy specimens of 171 patients diagnosed with FSGS (primary and secondary) and 50 control patients were retrospectively included in the study. For each patient, clinical and outc...

Circulating permeability factors have been identified in the plasma of patients with focal segmental glomerulosclerosis (FSGS). Post-transplant recurrence of proteinuria, improvement of proteinuria after treatment with plasmapheresis, and induction of proteinuria in experimental animals by plasma fractions each provide evidence for such plasma factors. Advanced proteomic methods have identified...

Heritable and acquired diseases of podocytes can result in focal and segmental glomerulosclerosis (FSGS). We modeled FSGS by passively transferring mouse podocyte-specific sheep Abs into BALB/c mice. BALB/c mice deficient in the key complement regulator, decay-accelerating factor (DAF), but not WT or CD59-deficient BALB/c mice developed histological and ultrastructural features of FSGS, marked ...

Podocyte foot process effacement is characteristic of proteinuric renal diseases. In minimal change nephrotic syndrome (MCNS) foot processes are diffusely effaced whereas the extent of effacement varies in focal segmental glomerulosclerosis (FSGS). Here we measured foot process effacement in FSGS and compared it to that in MCNS and in normal kidneys. A clinical diagnosis was used to differentia...

During the last three decades of the 20th century, a significant development in the field of glomerular diseases was an increased frequency of focal segmental glomerulosclerosis (FSGS) among patients presenting with proteinuria and a primary glomerular disease [1–10]. This increase in FSGS, initially noted in Chicago and other urban areas of the USA [1–7], was subsequently documented in rural a...