C3 glomerulopathy in children: experience at a resource-limited center
- Author:
Soumya REDDY
1
;
Abhishek GHANTE
;
Mahesha VANKALAKUNTI
;
Anil VASUDEVAN
Author Information
- Publication Type:Original Article
- From: Clinical and Experimental Pediatrics 2025;68(4):311-318
- CountryRepublic of Korea
- Language:English
-
Abstract:
Background:In children, C3 glomerulopathy (C3G) is a heterogeneous disease characterized by diverse clinicopathological profiles and kidney outcomes. However, diagnostic work-up in resource-limited settings is challenging because of the unavailability of complement assays and limited access to electron microscopy or genetic testing.Purpose: This study aimed to describe the clinicopathological features and response to immunosuppression and evaluate renal outcomes among children with C3G in a resource-limited setting.
Methods:This retrospective cohort study involved a review of the hospital records of 46 children (2013–2021) diagnosed with C3G on kidney biopsy. Their clinical, laboratory, treatment, and outcome details at onset and follow-up were noted.
Results:The mean (standard deviation) age was 9 (4) years. The common presentation was acute nephritis (27 [58.6%]), while 1 in 5 (19.5%) presented with rapidly progressive glomerulonephritis. Focal crescentic glomerulonephritis (14 [30.4%]) was the common histological pattern. Electron microscopy was performed in 22 (47.8%), of which 17 were C3 glomerulonephritis and 4 were dense deposit disease (DDD). None of the patients underwent complement assay or genetic testing. Almost two-thirds (63%) received empirical immunosuppressive therapy, most commonly steroids. Of the 31/46 who completed follow-up (median [interquartile range] duration, 11.5 [6–24] months), 6 (19.4%) demonstrated complete kidney recovery, while the other 25 (80.7%) had kidney sequelae; of them, 5 (16.1%) progressed to end-stage kidney disease and 2 (4.3%) died by the last follow-up.
Conclusion:Pediatric C3G has a variable clinicopathological spectrum, while DDD is less common. Most patients present with glomerulonephritis and significant morbidities. The lack of genetic and C3Nephritic factor testing is a barrier to the comprehensive phenotyping and management of C3G in resource-limited settings.
