A Clinicopathological Study of Rapidly Progressive Glomerulonephritis in Children.
- Author:
Hee Yeon CHO
1
;
Dae Lim CHUNG
;
Ju Hyung KANG
;
Il Soo HA
;
Hae Il CHEONG
;
Yong CHOI
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. cheonghi@plaza.snu.ac.kr
- Publication Type:Multicenter Study ; Original Article
- Keywords:
Rapidly progressive glomerulonephritis;
Children;
Crescent;
Prognostic factor
- MeSH:
Age of Onset;
Anemia;
Basement Membrane;
Biopsy;
Child*;
Classification;
Creatinine;
Diagnosis;
Female;
Follow-Up Studies;
Glomerulonephritis*;
Humans;
Kidney Failure, Chronic;
Male;
Methylprednisolone;
Prognosis;
Proteinuria;
Rare Diseases;
Renal Insufficiency, Chronic
- From:Journal of the Korean Society of Pediatric Nephrology
2004;8(2):176-185
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. METHODS: The clinical manifestations and pathological findings were reviewed retro spectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. RESULTS: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glome rular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. CONCLUSION: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.
