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Pediatric C3 glomerulopathy: a 12-year single-center experience
Zafirah Zahir1,2, Asif Sadiq Wani3,4, Amit Gupta5
1Department of Pathology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Pediatric Nephrology (Berlin, Germany)
|October 1, 2020
Summary
Pediatric complement component 3 glomerulopathy (C3G) differs from adult C3G in presentation and outcomes. Understanding these differences is crucial for diagnosing and managing C3G in children.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Glomerular Diseases
Background:
- Complement component 3 glomerulopathy (C3G) is a rare kidney disease with limited pediatric data.
- This study addresses the scarcity of information on childhood C3G by comparing it with adult cases.
- It also investigates pediatric C3G subgroups and factors predicting poor outcomes.
Purpose of the Study:
- To compare childhood C3G cases with adult C3G.
- To analyze subgroups within pediatric C3G, specifically C3 glomerulonephritis (C3GN) and dense deposit disease (DDD).
- To identify predictors of poor kidney outcomes in pediatric C3G.
Main Methods:
- A 12-year retrospective, single-center cohort observational study.
- Diagnosis of C3G followed the 2013 consensus guidelines.
- Electron microscopy was used to categorize pediatric C3G into C3GN and DDD.
Main Results:
- 162 C3G patients diagnosed (43 pediatric, 119 adult), predominantly male.
- Pediatric DDD cases were younger, presented with more severe disease (crescents), but had less chronicity than pediatric C3GN.
- Adults had lower eGFR and more biopsy chronicity; pediatric C3G showed a better prognosis. Low eGFR and interstitial fibrosis/tubular atrophy predicted kidney failure in children.
Conclusions:
- Pediatric C3G subgroups (C3GN, DDD) show distinct clinical presentations and disease courses but similar long-term outcomes.
- Pediatric C3G is a distinct entity from adult C3G, differing in presentation, lab results, biopsy findings, treatment, and outcomes.
- Childhood C3G requires consideration as a separate condition, not merely a pediatric variant of adult C3G.
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