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[Characteristics of pediatric C3 glomerulopathy with decreased factor H in 3 cases]
Rui-juan He1, Hui-jie Xiao, Su-xia Wang
1Department of Pediatrics, First Hospital, Peking University, Beijing 100034, China.
Insights
This study examines three pediatric C3 glomerulopathy cases, revealing varied clinical and pathological features. Complement system dysregulation, indicated by low C3 and Factor H, is crucial in pediatric C3 glomerulopathy.
Area of Science:
- Pediatric Nephrology
- Complement System Biology
- Glomerular Diseases
Context:
- C3 glomerulopathy (C3G) is a rare kidney disease characterized by complement C3 deposition.
- Understanding pediatric C3G is limited due to scarce case reports.
- This study focuses on the clinicopathological characteristics and prognosis of pediatric C3G.
Purpose:
- To investigate the clinicopathological features of three pediatric C3 glomerulopathy cases.
- To analyze the prognosis and treatment responses in these children.
- To enhance the understanding of C3 glomerulopathy in a pediatric population.
Summary:
- Three pediatric cases of C3 glomerulopathy were analyzed for clinical presentation, renal biopsy findings, and treatment outcomes.
- All cases showed C3 deposition in glomeruli, with varied presentations including hematuria and proteinuria.
- Decreased plasma C3 and Factor H (FH) levels were observed, suggesting complement dysregulation.
Impact:
- Highlights the diverse clinical and pathological spectrum of pediatric C3 glomerulopathy.
- Emphasizes the role of complement system abnormalities in the pathogenesis of C3G.
- Provides insights into the management and prognosis of C3G in children.
Objective:
To study the characteristics of clinicopathology and prognosis of 3 pediatric cases diagnosed as C3 glomerulopathy, and to improve the understanding of C3 glomerulopathy in children.
Method:
The medical record, plasma complement C3, Factor H (FH) and its autoantibody, and therapeutic response of the 3 cases were analyzed, and their prognosis were followed up.
Result:
Of the 3 cases, 2 were male and 1 was female, the age of onset was 9 years, 12 years, 5 years 4 months, the duration from onset to renal biopsy was 3 months, 7 months and 20 days, and the follow-up period were 2.6 years, 8 months and 1.5 years respectively.
Clinical Manifestations:
All the 3 cases showed microscopic hematuria, with or without gross hematuria and proteinuria. Two showed persistently decreased plasma complement C3, in the other one C3 was in normal lower limit, all presented with decreased FH concertration, in 1 case anti-FH antibody was positive. Their clinical diagnosis was post-streptococcal glomerulonephritis, nephrotic syndrome (NS) nephritis type, and mesangial proliferative glomerulonephritis respectively.
Pathological Findings:
All showed evident deposition of C3 on glomerular basement membrance (GBM) and mesangial region by immunofluorescence (IF) and electron dense deposit in GBM, mesangial region or para-mesangial region by Electron microscopic (EM) examination Treatment and prognosis: The case with NS showed no response to steroid, so steroid was gradually stopped after renal biopsy and replaced by angiotensin converting enzyme inhibitors (ACEI) and angiotensin receptor antagonist (ARB). The other two cases were treated with ACEI and renal protective treatment. Of the 3 cases, one gradually showed elevated serum creatinine (Scr) and decreased creatinine clearance rate (Ccr), the other two were normal, but slightly increased indications for early kidney injury.
Conclusion:
C3 glomerulopathy is characterized by evident C3 deposition under IF. Its clinical and pathological manifestations vary a lot. The decreased plasma C3 and FH suggest that the abnormal regulation of complement system play an importment role in its pathogenesis.
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