Pediatric Nephrology

, Volume 20, Issue 9, pp 1273–1278

Clinicopathologic features, outcome, and therapeutic interventions in four children with isolated C3 mesangial proliferative glomerulonephritis

Authors

  • Kazuro Yagi
    • Department of PediatricsKinki University School of Medicine
  • Hidehiko Yanagida
    • Department of PediatricsKinki University School of Medicine
  • Keisuke Sugimoto
    • Department of PediatricsKinki University School of Medicine
  • Hiroaki Kuwajima
    • Department of PediatricsKinki University School of Medicine
  • Nobutada Tabata
    • Department of PediatricsKinki University School of Medicine
  • Kosuke Morita
    • Department of PediatricsKinki University School of Medicine
  • Mitsuru Okada
    • Department of PediatricsKinki University School of Medicine
    • Department of PediatricsKinki University School of Medicine
Original Article

DOI: 10.1007/s00467-005-1919-4

Cite this article as:
Yagi, K., Yanagida, H., Sugimoto, K. et al. Pediatr Nephrol (2005) 20: 1273. doi:10.1007/s00467-005-1919-4

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.

Keywords

C3 depositionGlomerulonephritisPediatric patientTreatmentClinicopathologic featuresPrognosis

Copyright information

© IPNA 2005