| Full text | |
| Author(s): |
Guaragna, Mara S.
;
Casimiro, Fernanda M. S.
;
Varela, Patricia
;
de S. Feltran, Luciana
;
Watanabe, Andreia
;
Neves, Precil D. M. M.
;
Pesquero, Joao B.
;
Belangero, Vera M. S.
;
Nogueira, Paulo C. K.
;
Onuchic, Luiz F.
Total Authors: 10
|
| Document type: | Journal article |
| Source: | Pediatric Nephrology; v. N/A, p. 17-pg., 2025-01-30. |
| Abstract | |
Predicting the risks of progression to chronic kidney disease (CKD) stage 5 in idiopathic nephrotic syndrome (NS) and recurrence of the disease (rNS) following kidney transplantation (KT) is a key assessment to provide essential management information. NS has been categorized etiologically as genetic and immune-based. A genetic cause can be identified in similar to 30% of children with steroid-resistant NS (SRNS), a finding associated with a very low risk of rNS following KT. In immune-based NS, clinical overlap is observed among steroid-sensitive NS, secondary-resistant NS, and SRNS not associated with disease-causing genetic variants (non-monogenic SRNS). While similar to 50% of SRNS patients with no identified monogenic disease respond to intensified immunosuppressive treatments, the ones that do not respond to this therapy have a high risk of progression to CKD stage 5 and post-KT rNS. Secondary-resistant patients who progress to CKD stage 5 display the highest risk of post-KT rNS. The proposed shared underlying mechanism of the immune-based NS associated with post-KT rNS is based on a systemic circulating factor (CF) that affects glomerular permeability by inducing foot process effacement and focal segmental glomerulosclerosis. However, identifying patients without a detected genetic form who will recur post-KT is a major challenge. Extensive efforts, therefore, have been made to identify CFs and biomarkers potentially capable of predicting the risk of progression to CKD stage 5 and post-KT rNS. This review discusses the in vitro and in vivo approaches employed to date to identify and characterize potential CFs and CF-induced biomarkers of recurrent NS and offers an assessment of their potential to improve outcomes of KT in this patient population. (AU) | |
| FAPESP's process: | 20/02988-7 - Decoding the impact of microenvironment and signaling pathways in health and disease in brain, adrenal gland and kidney |
| Grantee: | Suely Kazue Nagahashi Marie |
| Support Opportunities: | Research Projects - Thematic Grants |
| FAPESP's process: | 20/15800-6 - Epidemiology of pediatric nephrotic syndrome in Brazil: incidence, prevalence, response to steroids and construction of the basis for molecular genetic analyses |
| Grantee: | Paulo Cesar Koch Nogueira |
| Support Opportunities: | Regular Research Grants |
| FAPESP's process: | 20/14635-1 - Modeling of monogenic diseases for physiopathological studies and pharmacological tests using specialized cells derived from iPSCs |
| Grantee: | João Bosco Pesquero |
| Support Opportunities: | Research Projects - Thematic Grants |