C3 glomerulopathy in children: experience at a resource-limited center
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 elect...
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The Korean Pediatric Society
2025-04-01
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| Series: | Clinical and Experimental Pediatrics |
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| Online Access: | http://e-cep.org/upload/pdf/cep-2024-01256.pdf |
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| author | Soumya Reddy Abhishek Ghante Mahesha Vankalakunti Anil Vasudevan |
| author_facet | Soumya Reddy Abhishek Ghante Mahesha Vankalakunti Anil Vasudevan |
| author_sort | Soumya Reddy |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-5b9993f18c944aae8b8d5e629613afd7 |
| institution | OA Journals |
| issn | 2713-4148 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | The Korean Pediatric Society |
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| series | Clinical and Experimental Pediatrics |
| spelling | doaj-art-5b9993f18c944aae8b8d5e629613afd72025-08-20T02:15:30ZengThe Korean Pediatric SocietyClinical and Experimental Pediatrics2713-41482025-04-0168431131810.3345/cep.2024.0125620125555767C3 glomerulopathy in children: experience at a resource-limited centerSoumya Reddy0Abhishek Ghante1Mahesha Vankalakunti2Anil Vasudevan3 Department of Pediatric Nephrology, St. John's Medical College Hospital, St. John's National Academy of Health Sciences, Bengaluru, India Department of Pediatric Nephrology, St. John's Medical College Hospital, St. John's National Academy of Health Sciences, Bengaluru, India Department of Laboratory Medicine, Manipal Hospital, Bengaluru, India Department of Pediatric Nephrology, St. John's Medical College Hospital, St. John's National Academy of Health Sciences, Bengaluru, IndiaBackground 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.http://e-cep.org/upload/pdf/cep-2024-01256.pdfc3 glomerulopathydense deposit diseasealternate complement pathwayglomerulonephritis in india children |
| spellingShingle | Soumya Reddy Abhishek Ghante Mahesha Vankalakunti Anil Vasudevan C3 glomerulopathy in children: experience at a resource-limited center Clinical and Experimental Pediatrics c3 glomerulopathy dense deposit disease alternate complement pathway glomerulonephritis in india children |
| title | C3 glomerulopathy in children: experience at a resource-limited center |
| title_full | C3 glomerulopathy in children: experience at a resource-limited center |
| title_fullStr | C3 glomerulopathy in children: experience at a resource-limited center |
| title_full_unstemmed | C3 glomerulopathy in children: experience at a resource-limited center |
| title_short | C3 glomerulopathy in children: experience at a resource-limited center |
| title_sort | c3 glomerulopathy in children experience at a resource limited center |
| topic | c3 glomerulopathy dense deposit disease alternate complement pathway glomerulonephritis in india children |
| url | http://e-cep.org/upload/pdf/cep-2024-01256.pdf |
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