1. Kidney outcomes in children with primary focal segmental glomerulosclerosis from a low- and middle- income country.
- Author
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Priyanka K, Deepthi B, Krishnasamy S, Ganesh RN, Sravani M, and Krishnamurthy S
- Subjects
- Humans, Male, Child, Female, Child, Preschool, Adolescent, Infant, Developing Countries, Kidney pathology, Kidney physiopathology, Renal Insufficiency, Chronic etiology, Renal Insufficiency, Chronic epidemiology, Renal Insufficiency, Chronic diagnosis, Immunosuppressive Agents therapeutic use, Calcineurin Inhibitors therapeutic use, Remission Induction, Follow-Up Studies, Retrospective Studies, Glomerulosclerosis, Focal Segmental diagnosis, Glomerulosclerosis, Focal Segmental epidemiology, Glomerulosclerosis, Focal Segmental pathology, Disease Progression
- Abstract
Background: Limited data exists regarding the clinical course and outcomes of children with primary focal segmental glomerulosclerosis (FSGS) from low- and middle- income countries., Methods: Children aged 1-18 years with biopsy-proven primary FSGS followed from January 2010-June 2023 in a tertiary-care center were enrolled and their clinical profile, histological characteristics, kidney outcomes, and predictors of adverse outcomes were determined., Results: Over 13 years, 73 (54.8% boys) children with median (IQR) age at FSGS diagnosis 6.7 (3,10) years were recruited and followed up for median 4 (2.5,8) years. FSGS-not otherwise specified (NOS) was the most common histological subtype, in 64 (87.6%) children, followed by collapsing variant in 5 (6.8%) children. At last follow-up, 43 (58.9%), 2 (2.7%) and 28 (38.3%) children were in complete remission (CR), partial remission (PR), and no remission (NR) respectively. Calcineurin inhibitors led to CR or PR in 39 (62%) children. Overall, 21 (28.7%) children progressed to chronic kidney disease (CKD) stage 2-5 (19 from NR vs. 2 from PR group; p = 0.03); with 41% of those NR at 12 months progressing to CKD 4-5 by last follow-up. On multivariable analysis, collapsing variant [adjusted HR 2.5 (95%CI 1.5, 4.17), p = 0.001] and segmental sclerosis > 25% [aHR 9.9 (95%CI 2.2, 45.2), p = 0.003] predicted kidney disease progression., Conclusions: In children with FSGS, response to immunosuppression predicts kidney survival as evidenced by nil to lower progression to CKD 2-5 by median follow-up of 4 (2.5,8) years in children with CR and PR, compared to those with no remission at 12 months from diagnosis. Segmental sclerosis > 25% and collapsing variant predicted progression to advanced CKD., (© 2024. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)
- Published
- 2024
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