Publication at Second Faculty of Medicine, Faculty of Medicine in Hradec Králové |
2017
Abstract
Nephrotic syndrome (NS) includes a group of diseases with increased permeability of glomerular filtration barrier, which causes heavy proteinuria, hypoalbuminemia and edema. Idiopathic NS is the most common type in children.
Most of the children are steroid-sensitive, however, about 20% fail to respond to steroid treatment and are at significant risk for progressive renal failure. Extensive research has revealed the structure of permeability membrane and podocyte function.
More than 40 genes associated with steroid-resistant NS have been discovered. While corticosteroids and other immunosupressives are used in patients with idiopatic NS, immunosupressive therapy should be stopped in patients with proven genetic disease.