SÍNDROME NEFRÓTICO RESISTENTE A CORTICOIDES EN PEDIATRÍA, ACTUALIZACIÓN

The nephrotic Syndrome is the most common cause of glomerulonephropathy in pediatrics. Clinical findings are derived from hypoalbuminemia. The main therapy remains to be corticosteroids. According to the response to the corticosteroids therapy, there are two categories: Steroid-sensitive nephrotic syndrome, and the ones that do not respond to steroids: steroid-resistant nephrotic syndrome. Most of the patients that have a corticoid-resistant nephrotic syndrome have a focal segmental glomerulosclerosis, which is associated with a 50% risk of terminal renal disease, so renal biopsy is recommended in these patients. Another important factor is genetic testing, this is because some mutations are associated with a failure in the response to steroids, an example is the NPHS2 gene mutation (podocin) which is the most common described cause of NS. Calcineurin inhibitors are recommended as the best initial therapy in these cases, additionally recent researches include high dose or pulses of corticosteroids, cyclophosphamide, mycophenolate mofetil, and rituximab, alone or in combination as alternate option obtaining variable and imprecise results due to small sample sizes in the studies. With this article we introduce an updated review of the national and international literature about CRNS in Pediatrics, looking forward to achieve new alternatives for its diagnosis and more efficient treatments.