Related Experiment Video
Updated: Aug 17, 2026
An Efficient Sieving Method to Isolate Intact Glomeruli from Adult Rat Kidney
Published on: November 1, 2018
Clinicopathologic features, outcome, and therapeutic interventions in four children with isolated C3 mesangial
Kazuro Yagi1, Hidehiko Yanagida, Keisuke Sugimoto
1Department of Pediatrics, Kinki University School of Medicine, 377-2 Ohno-higashi, 589-8511 Osaka-Sayama, Japan.
Insights
Isolated C3 glomerulonephritis (i-C3-GN) prognosis varies in children. Aggressive treatment improved outcomes for severe cases, highlighting individualized pediatric care for this rare kidney disease.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Glomerular Diseases
Background:
- Isolated C3 glomerulonephritis (i-C3-GN) is a rare chronic kidney disease.
- Prognosis and treatment for pediatric i-C3-GN are not well-defined.
Purpose of the Study:
- To report clinical features, outcomes, and interventions for pediatric i-C3-GN.
- To assess the impact of treatment on disease progression.
Main Methods:
- Retrospective case series of 4 pediatric patients (6-18 years) with i-C3-GN.
- Analysis of clinical presentation, renal biopsy findings, and treatment responses.
Main Results:
- Patients presented with hematuria and/or proteinuria.
- Mild histologic changes correlated with benign outcomes over 10 years.
- Moderate histologic changes showed initial renal function loss and proteinuria, which improved with prednisolone, cyclophosphamide, warfarin, and ACE inhibitors.
Conclusions:
- Pediatric i-C3-GN exhibits variable clinical courses.
- Combined immunosuppressive therapy may improve proteinuria and renal function in severe cases.
Abstract:
Since isolated C3 mesangial proliferative glomerulonephritis in the absence of systemic disease (i-C3-GN) is an uncommon chronic glomerular disease, long-term prognosis and optimal therapeutic intervention for it are not yet fully defined, especially in children. We report clinical features, outcome, and interventions in 4 patients, ranging from 6 to 18 years old, with i-C3-GN. Microscopic or macroscopic hematuria with or without proteinuria was first noted between 3 and 8 years. When present, proteinuria ranged from 0.2 to 1.0 g/24 h. Persistent hypocomplementemia and circulating immune complexes were found in 1 patient. None of the patients had nephrotic syndrome or hypertension. Percutaneous renal biopsy specimens showed varying degrees of mesangial proliferative glomerulonephritis; 2 patients showed mild mesangial proliferation, while others exhibited moderate histologic severity. In 1 patient with a mild mesangial increase, tubulointerstitial changes were associated. Both patients exhibiting mild mesangial changes followed a benign clinical course with normal renal function over 10 years of follow-up. Patients with moderately severe mesangial alteration manifested slight renal function loss and moderate proteinuria at the time of biopsy, but these largely resolved after a six-month course of prednisolone combined with cyclophosphamide, warfarin, and an angiotensin-converting enzyme inhibitor. Thus, clinical manifestations and the need for aggressive treatment appear to vary among pediatric patients with i-C3-GN. Therapy combining prednisolone with immunosuppression seemed to reduce proteinuria and improve glomerular function in patients with moderately severe mesangial proliferation.
