Nephrotic Syndrome In Children – Present State And Future Perspectives

Matjaž Kopač

Abstract


The nephrotic syndrome is characterized by increased permeability across the glomerular filtration barrier. Several mechanisms of glomerular injury are responsible for pathogenesis, such as circulating factor, circulating immune factors in immune-mediated disorders and mutations in podocyte or slit diaphragm proteins. Children with nephrotic syndrome are classified as primary, secondary and congenital / infantile nephrotic syndrome. Children with idiopathic nephrotic syndrome, the most common form, can be divided into those with steroid-resistant nephrotic syndrome, who are at increased risk for developing end-stage renal disease, and those with steroid-responsive nephrotic syndrome, representing the majority of cases. They often have serious side effects due to corticosteroids and treatment with other agents is recommended in order to maintain remission while reducing corticosteroid dosing. The purpose of treatment of steroid-resistant cases is to decrease proteinuria and to preserve kidney function. Treatment strategies include immunosuppressive and non-immunosuppressive measures. A kidney biopsy should be done in these children in order to reveal the underlying histology. In patients with a strong suspicion for a genetic cause genetic testing should be done as well. Congenital nephrotic syndrome is a nephrotic syndrome that presents at birth or during the first three months of life while infantile nephrotic syndrome presents between three and twelve months of age. In majority of these children there is a genetic basis for the disease and poor outcome with no indication for immunosuppressive treatment. A new way of possible treatment of this type of nephrotic syndrome in the future is presented.

Keywords


Nephrotic syndrome; Children; Steroid-resistance; Steroid-responsiveness; Genetics; Treatment

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