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Journal of the Korean Society of Pediatric Nephrology 2004;8(2): 176-185.
소아 급속 진행성 사구체 신염의 임상-병리학적 고찰
조희연, 정대림, 강주영, 하일수, 최용, 정해일
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 소아과학교실
3서울대학교 의과대학 소아과학교실
4서울대학교 의과대학 소아과학교실
5서울대학교 의과대학 소아과학교실
6서울대학교 의과대학 소아과학교실
A Clinicopathological Study of Rapidly Progressive Glomerulonephritis in Children
Hee-Yeon Cho, Dae-Lim Chung, Ju-Hyung Kang, Il-Soo Ha, Yong Choi, Hae-Il Cheong
1Department of Pediatrics, Seoul National University College of Medicine
2Department of Pediatrics, Seoul National University College of Medicine
3Department of Pediatrics, Seoul National University College of Medicine
4Department of Pediatrics, Seoul National University College of Medicine
5Department of Pediatrics, Seoul National University College of Medicine
6Department of Pediatrics, Seoul National University College of Medicine
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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 retrospectively 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-glomerular 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.
Key words: Rapidly progressive glomeruloneuhritis | Children | Crescent | Prognostic factor
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