Focal Segmental Glomerulosclerosis and Chronic Kidney Disease in Pediatric Patients

Focal segmental glomerulosclerosis (FSGS) is one of the most common forms of acquired glomerular disease leading to end-stage kidney disease. Its incidence is rising around the world. There is no proven therapy for those patients who do not respond to corticosteroids and it can recur in 20% to 25% of patients who receive a kidney transplant. The disease can be primary, or it can be secondary to various conditions including vesicoureteral reflux, obesity, medications, and infections. Recent advances have demonstrated the important role of genetic mutations in podocyte proteins as a cause of FSGS. There is an urgent need for randomized clinical trials to develop safe and effective therapy for FSGS that occurs in the native or transplanted kidney.