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Published on: August 19, 2020
[Clinical and pathological characteristics of focal segmental glomerulosclerosis in children]
Jian-ping Huang1, Jing-jing Zhang, Jing-cheng Liu
1Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.
Insights
Focal segmental glomerulosclerosis (FSGS) is common in school-aged children, often presenting as nephrotic syndrome. Combination therapies like pulse methylprednisolone and cyclosporin A show promising remission rates for pediatric FSGS.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
- Renal Pathology
Context:
- Focal segmental glomerulosclerosis (FSGS) is a significant cause of kidney disease in children.
- Understanding the clinical and pathological spectrum of pediatric FSGS is crucial for effective management.
- This study retrospectively analyzed 38 pediatric FSGS cases.
Purpose:
- To investigate the clinical and pathological characteristics of primary focal segmental glomerulosclerosis (FSGS) in children.
- To evaluate treatment responses and identify factors influencing outcomes in pediatric FSGS.
Summary:
- The study included 38 children (25 boys, 13 girls) aged 1.5–15 years with primary FSGS.
- Nephrotic syndrome was the predominant presentation (34 cases), with hematuria common (63%).
- Tip variant FSGS was associated with simple nephrotic syndrome, while diffuse mesangial hypercellularity correlated with hematuria.
- Pulse methylprednisolone and pulse cyclophosphamide or cyclosporin A showed good response rates (83% remission).
- Proteinuria levels post-treatment correlated with tubulointerstitial lesions and renal function.
Impact:
- Highlights the commonality of FSGS in school-aged children and its varied presentations.
- Suggests that specific FSGS variants have distinct clinical features.
- Indicates the efficacy of intensive immunosuppressive therapies in achieving remission in pediatric FSGS.
- Emphasizes the correlation between post-treatment proteinuria and long-term renal health.
Objective:
To investigate the clinical and pathological characteristics of focal segmental glomerulosclerosis (FSGS) in children.
Methods:
The data of 38 children,aged from one and half to 15 years, 25 boys and 13 girls, with primary FSGS were studied retrospectively.
Results:
Majority of the cases in this study were school-aged children. The average age of initial onset was 8.9 +/- 3.68 years. The ratio of boys to girls was 1.92. The clinical manifestation included isolated proteinuria in 3 cases, proteinuria and hematuria in 1 and nephrotic syndrome in 34 (simple type in 16 and nephritic type in 18). Of 38 cases, 24 (63%) presented with hematuria, 11 (29%) with hypertension and 7 (18%) with decreased creatinine clearance. The pathologic classification included perihilar variant in 17 cases, peripheral variant in 14 and tip variant in 7. The predominant clinical feature of children with tip variant was simple type of nephrotic syndrome (86%). Microscopic hematuria was not common (29%). Blood pressure and renal function were normal. The children with diffuse mesangial hypercellularity superimposed on changes of FSGS (in 21 of 38 cases) were more likely to have hematuria (76%) and less simple nephrotic syndrome (30%). The initial treatment response to prednisone in 34 cases with nephrotic syndrome showed sensitive in 12 cases, resistant in 21 and unknown in 1. Transition from sensitive to resistant occurred in six of 12 children. Three of 4 cases with non-nephrotic syndrome showed no response and the remaining one had unknown response. It was found that 44% of children who received cyclophosphamide and 83% of children who received pulse methylprednisolone and pulse cyclophosphamide or cyclosporin A in addition to oral steroids had complete or partial remission. Correlation analysis showed that the level of proteinuria after treatment was correlated directly with renal tubulointerstitial lesion and renal function (Pr = 0.48, P < 0.05; Pr = 0.45, P < 0.05).
Conclusion:
FSGS was common in school-aged children. The predominant presenting feature was nephrotic syndrome. Hematuria was common. Hypertension and renal insufficiency were less frequently seen. The renal biopsy showed multiple variants. Pulse methylprednisolone and pulse cyclophosphamide or cyclosporin A treatments showed relatively good response.
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