Abstract
Nephrotic syndrome (NS) is a severe glomerulopathy characterized by severe proteinuria, hypoproteinemia, hypoalbuminemia, hyperlipidemia and widespread edema. There’ve been over sixty genes identified to date that are responsible for the development, structural integrity and functioning of podocytes. Pathogenic nucleotide variants of these genes lead to the podocytopathy in steroid-resistant nephrotic syndrome in 30% of cases. Depending on the protein damaged, the severity of the course, the extrarenal manifestations as well as the therapeutic options may vary. The molecular genetic research has therefore resulted in the fore in recent decades when pediatric patients with steroid-resistant NS are diagnosed.
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