Pathological and Evolutive Correlations in Steroid Resistant Nephrotic Syndrome in Children

Abstract

Background Nephrotic syndrome (NS) is the term used for the association of edema and massive proteinuria. From a therapeutic point of view, it is important to distinguish between primitive and secondary kidney damage. The clinical evolution, prognosis and therapeutic response in the NS in children are directly determined by the anatomopathological aspect. Steroid resistant nephrotic syndrome was diagnosed in patients with idiopathic NS based on lack of complete remission despite treatment with steroids. Purpose To analyse the anatomopathological aspects of steroid resistant nephrotic syndrome (SRNS) and their correlation with evolution. Materials and Methods We made a retrospective study with the aim to analyze the anatomo-pathological aspects and their correlations with evolution in 68 cases of steroid resistant nephrotic syndrome (SRNS) hospitalized in the Pediatric Nephrology Department in Iaşi, Romania. We defined SRNS in all cases without response to corticosteroids after the first month of therapy. For all the cases selected, the period of follow-up was the minimal 6 months. Results and Discussions A 36% case of nephrotic syndrome was corticoresistant, with the mean age at onset of patients with SRNS being 9.18 years, compared to KDIGO studies in which the corticosteroid resistance is 10–20%. Renal biopsy was performed in 80.88% children with SRNS and was allowed the evaluation of the activity and chronicity index. Total remission was obtained in 44.01% children with SRNS. The correlation of the anatomopathological aspects with the evolution is not statistically significant (p = 0.76), observing different therapeutic responses to all the analyzed histological types. Conclusion Almost half of NS in children are cortico resistant. Remission was obtained in 44% of cases of SRNS. Predicting the response to long-term treatment in SRNS is difficult using only renal biopsy; it is necessary to introduce genetic molecular analyses to establish a judicious therapeutic attitude.

A rare cause of steroid-resistant nephrotic syndrome – a case report

“…The incidence of steroid resistance in children with nephrotic syndrome is between 35% and 92% according to the statistics [1,2]. Mutation of a gene responsible for podocyte building was detected in one out of three patients.…”

“…firmly established, Fsgs -focal segmental glomerulosclerosis, McD -minimal change disease, DMs -diffuse mesangial sclerosis, it -interstitial fibrosis, ta -tubular atrophy, csa -cyclosporin a, rtX -rituximab, ace-i -angiotensin-converting enzyme (ace) inhibitors ckD -chronic kidney disease, tx -kidney transplantation, eskD -end stage kidney diseaseCentral European Journal of Immunology 2023; 48(2) …”