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Journal of the Korean Society of Pediatric Nephrology 2001;5(2): 78-86.
소아 급속 진행성 사구체신염의 임상적 고찰
엄지현, 김미진, 이영목, 김지홍, 이재승, 김병길, 홍순원, 정현주
1연세대학교 의과대학 소아과학교실, 신장질환연구소
2연세대학교 의과대학 소아과학교실, 신장질환연구소
3연세대학교 의과대학 소아과학교실, 신장질환연구소
4연세대학교 의과대학 소아과학교실, 신장질환연구소
5연세대학교 의과대학 소아과학교실, 신장질환연구소
6연세대학교 의과대학 소아과학교실, 신장질환연구소
7연세대학교 의과대학 병리학교실, 신장질환연구소
8연세대학교 의과대학 병리학교실, 신장질환연구소
Analysis of Childhood Rapidly Progressive Glomerulonephritis
Ji Hyun Uhm, Mi Jin Kim, Young-Mock Lee, Ji Hong Kim, Jae Seung Lee, Pyung-Kil Kim, Soon Won Hong, Hyeun Joo Jeung
1Departments of Pediatrics, The Institute of Kidney Disease Yonsei University College of Medicine
2Departments of Pediatrics, The Institute of Kidney Disease Yonsei University College of Medicine
3Departments of Pediatrics, The Institute of Kidney Disease Yonsei University College of Medicine
4Departments of Pediatrics, The Institute of Kidney Disease Yonsei University College of Medicine
5Departments of Pediatrics, The Institute of Kidney Disease Yonsei University College of Medicine
6Departments of Pediatrics, The Institute of Kidney Disease Yonsei University College of Medicine
7Departments of Pathology, The Institute of Kidney Disease Yonsei University College of Medicine
8Departments of Pathology, The Institute of Kidney Disease Yonsei University College of Medicine
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ABSTRACT
Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features
Method : Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{pm}3.8$). 3 had Henoch-$Sch{ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{pm}39.1;mg/dL$ mean serum creatinin, $3.2{pm}1.8;mg/dL$ and mean creatinin clearance, $26.5{pm}13.2;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function.
Conclusion : Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)
Key words: Crescentic glomerulonephritis | Renal failure | BUN | Creatinine | Creatinine clearance | Peritoneal dialysis
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