Nephrotic syndrome

In nephrotic syndrome the glomerular filtration barrier fails to retain protein leading to proteinuria, hypoalbuminaemia and oedema. In childhood, most cases are idiopathic and treatment with steroids leads to complete remission of symptoms. The clinical course of the disease is characterized by relapses. A significant number of patients become dependent on steroids and may require steroid-sparing agents to remain in remission. However the long term prognosis is generally favourable. The group of the patients that do not respond to steroids is invariably a therapeutic challenge for the clinicians and in this group progression to end-stage renal failure is not uncommon.The pathophysiology of nephrotic syndrome is not completely understood. The central role of podocytes and the immunity system have long been speculated. Recent advances in the genetics of nephrotic syndrome have provided a better understanding of the disease.