Analysis of Childhood Rapidly Progressive Glomerulonephritis

Uhm, Ji Hyun; Kim, Mi Jin; Lee, Young-Mock; Kim, Ji Hong; Lee, Jae Seung; Kim, Pyung-Kil; Hong, Soon Won; Jeung, Hyeun Joo

J Korean Soc Pediatr Nephrol > Volume 5(2); 2001 > Article

J Korean Soc Pediatr Nephrol 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

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)