A Clinicopathological Study of Rapidly Progressive Glomerulonephritis in Children
Journal of the Korean Society of Pediatric Nephrology
;
: 176-185, 2004.
Article
in Korean
| WPRIM
| ID: wpr-46798
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Prognosis
/
Proteinuria
/
Basement Membrane
/
Biopsy
/
Methylprednisolone
/
Follow-Up Studies
/
Classification
/
Age of Onset
/
Creatinine
/
Rare Diseases
Type of study:
Controlled clinical trial
/
Diagnostic study
/
Observational study
/
Prognostic study
Limits:
Child
/
Female
/
Humans
/
Male
Language:
Korean
Journal:
Journal of the Korean Society of Pediatric Nephrology
Year:
2004
Type:
Article
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