Title:Idiopathic Nephrotic Syndrome in Pediatrics: An Up-to-date
Volume: 18
Issue: 4
Author(s): Roberta da Silva Filha, Kassia Burini, Laura Gregório Pires, Sérgio Veloso Brant Pinheiro and Ana Cristina Simões e Silva*
Affiliation:
- Faculty of Medicine, Interdisciplinary Laboratory of Medical Investigation, Federal University of Minas Gerais (UFMG), Belo Horizonte, MG, Brazil
- Department of Pediatrics, Unit of Pediatric Nephrology, Faculty of Medicine, UFMG, Belo Horizonte, MG, Brazil
Keywords:
Idiopathic nephrotic syndrome, children, proteinuria, podocyte, steroid therapy, focal segmental glomerulosclerosis.
Abstract:
Background: Idiopathic or Primary Nephrotic Syndrome (INS) is a common glomerular
disease in pediatric population, characterized by proteinuria, edema and hypoalbuminemia with variable
findings in renal histopathology.
Objective: This review aims to summarize current data on the etiopathogenesis diagnosis, protocols
of treatment and potential therapeutic advances in INS.
Methods: This narrative review searched for articles on histopathology, physiopathology, genetic
causes, diagnosis and treatment of INS in pediatric patients. The databases evaluated were PubMed
and Scopus.
Results: INS is caused by an alteration in the permeability of the glomerular filtration barrier with
unknown etiology. There are several gaps in the etiopathogenesis, response to treatment and clinical
course of INS that justify further investigation. Novel advances include the recent understanding of
the role of podocytes in INS and the identification of genes associated with the disease. The role of
immune system cells and molecules has also been investigated. The diagnosis relies on clinical findings,
laboratory exams and renal histology for selected cases. The treatment is primarily based on
steroids administration. In case of failure, other medications should be tried. Recent studies have also
searched for novel biomarkers for diagnosis and alternative therapeutic approaches.
Conclusion: The therapeutic response to corticosteroids still remains the main predictive factor for
the prognosis of the disease. Genetic and pharmacogenomics tools may allow the identification of
cases not responsive to immunosuppressive medications.
