Nephrotic syndrome in childhood

Česká verzia

Authors: S. Skálová ¹; T. Seeman ²; Š. Štolbová ²; J. Zieg ²
Authors place of work: Dětská klinika FN a LF UK, Hradec Králové ¹; Pediatrická klinika FN Motol a 2. LF UK, Praha ²
Published in the journal: Čes-slov Pediat 2017; 72 (2): 88-98.
Category: Dětská nefrologie - 2.část

Summary

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.

KEY WORDS:
nephrotic syndrome – idiopathic, genetic

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