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C3 glomerulopathy in children: experience at a resource-limited center
Soumya Reddy1, Abhishek Ghante1, Mahesha Vankalakunti2
1Department of Pediatric Nephrology, St. John's Medical College Hospital, St. John's National Academy of Health Sciences, Bengaluru, India.
Clinical and Experimental Pediatrics
|November 28, 2024
Summary
Pediatric C3 glomerulopathy (C3G) presents diversely, often with acute nephritis. Limited diagnostic tools in resource-limited settings hinder comprehensive management, leading to significant kidney morbidity and progression to end-stage kidney disease.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Glomerular Diseases
Background:
- C3 glomerulopathy (C3G) in children exhibits varied clinical and pathological features.
- Diagnostic challenges in resource-limited settings include lack of complement assays and advanced testing.
- This heterogeneity impacts patient outcomes and management strategies.
Purpose of the Study:
- To characterize the clinicopathological spectrum of pediatric C3G in a resource-limited environment.
- To assess the effectiveness of immunosuppressive therapy in these patients.
- To evaluate the renal outcomes and long-term prognosis of children with C3G.
Main Methods:
- Retrospective cohort study of 46 children diagnosed with C3G via kidney biopsy (2013-2021).
- Analysis of clinical, laboratory, treatment, and outcome data at presentation and during follow-up.
- Limited use of electron microscopy; no complement assays or genetic testing performed.
Main Results:
- Common presentations included acute nephritis (58.6%) and rapidly progressive glomerulonephritis (19.5%).
- Focal crescentic glomerulonephritis was the predominant histological pattern (30.4%).
- Only 19.4% achieved complete kidney recovery; 16.1% progressed to end-stage kidney disease, and 4.3% died.
Conclusions:
- Pediatric C3G shows a broad clinicopathological range, with dense deposit disease (DDD) being less frequent.
- Most children experience significant morbidity, underscoring the need for better diagnostic capabilities.
- Absence of genetic and C3Nephritic factor testing impedes precise phenotyping and optimal management in resource-limited settings.
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