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Journal of the Korean Society of Pediatric Nephrology 2003;7(2): 133-141.
소아에서 일차성 막성 신병증의 임상-병리학적 고찰
이범희, 조희연, 강주형, 강희경, 하일수, 정해일, 이현순, 최용
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 소아과학교실
3서울대학교 의과대학 소아과학교실
4서울대학교 의과대학 소아과학교실
5서울대학교 의과대학 소아과학교실
6서울대학교 의과대학 소아과학교실
7서울대학교 의과대학 해부병리학교실
8서울대학교 의과대학 소아과학교실
A Clinicopathological Study of Idiopathic Membranous Nephropathy in Children
Bum-Hee Lee, Hee-Yeon Cho, Ju-Hyung Kang, Hee-Gyung Kang, Il-Soo Ha, Hae-Il Cheong, Hyun-Soon Lee, Yong Choi
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
7Department of Pathology, Seoul National University College of Medicine
8Department of Pediatrics, Seoul National University College of Medicine
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ABSTRACT
Purpose : Idiopathic Membranous Nephropathy(IMN) is a rare renal disease in children. To help better understanding of its clinical course and treatment strategies, we reviewed the clinical manifestations and pathological findings of children with IMN.
Methods : Among 58 cases with MN, from 1977 to 2003, 42(72.4%) were hepatitis B virus (HBV) associated and 16(27.6%), 6 males and 10 females, were idiopathic. All cases diagnosed aster 2000 were IMN. Several clinicopathological findings(sex, onset age, proteinuria, serum albumin, cholesterol, creatinine clearance, tubulointerstitial changes, glomerular sclerosis, hypertension, renal vein thrombosis, the use of ACE inhibitor, and immunosuppressive therapy) were compared between the remission and the non-remission group of the patients with IMN.
Results : The median onset age was 13.4 years. Clinical manifestations were nephrotic syn-drome(7 cases, 43.8%), gross hematuria(5 cases, 31.3%) and microscopic hematuria with proteinuria(3 cases, 18.8%). Hypertension, hypocalcemic tetany and renal vein thrombosis were accompanied in 2, 1 and 2 cases, respectively. In addition to the typical findings of MN, the kidney biopsies showed segmental sclerosis(5 cases, 31.3%) or global sclerosis(6 cases, 37.5 %), diffuse crescents(1 case), and mild(11 cases, 68.7%) or moderate tubulointerstitial changes(3 cases, 18.8%). Thirteen cases(86.7%) received oral steroid. Among them 2 cases received cyclophophamide and 1 received cyclosporin as well. Ten cases(62.5%) received ACE inhibitors. In the patients followed up, 7 cases(46.7%) became free from proteinuria (remission group) while 8(53.3%) presented continous proteinuria (non-remission group), two (13.3%) of which progressed to renal failure. Clinicopathological findings showed no significant differences between the two groups.
Conclusion : With HBV vaccination, HBV associated MN decreased markedly and IMN has taken up most of MN in children. For better understanding of this rare disease, a prospective multicenter study of the clinical course and treatment strategies should be done.
Key words: Idiopathic membranous nephropathy | Children | Treatment
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