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Idiopathic IgA Nepropathy in Children.

Journal of the Korean Pediatric Society 1986;29(6):633-643.
Published online June 30, 1986.
Idiopathic IgA Nepropathy in Children.
Hae Il Cheong1, Han Wook Yoo2, Kwang Wook Ko2
1Department of Pediatrics, Seoul Red Cross Hospital
2Department of Pediatrics, College of Medicine, Seoul National University
소아에 있어서의 특발성 IgA 신병증
정해일1, 유한욱2, 고광욱2
1서울적십자병원 소아과
2서울대학교 의과대학 소아과학교실
We analyzed clinical findings, pathological findings and clinico-pathological correlations in 55 children with idiopathic IgA nephropathy confirmed immunopathologically, and the results were as follows; 1) The mean age at onset was 9.65±2.45 years with relatively even distribution in school ages and males outnumbered females by 1.75 : 1. 2) The main mode of presentation was typical recurrent gross hematuria in 29(52.7%), and the next was nephrotic syndrome in 10(18.2%). 3) Nephrotic range of proteinuria and elevated serum IgA levels were observed in 34.5% and 25.5% of cases, respectively. 4) Light microscopically, 30 cases(54.5%) showed mesangial proliferation with less than 50% crescents/segmental lesions, 16 cases(29.1%) did pure mesangial proliferation and cases (14.5%) did minor change. 5) Immunofluorescent microscopically, IgA+C3 or IgA+IgG+C3 combination of mesangial deposits was the most common and in 7 cases (12.7%) capillary staining of IgA was accompanied. 6) Electron microscopically, 52.9% showed only mesangial electron dense deposits and 41.2% did mesangial deposits with variable combinations of subendothelial, subepithelial and intramembranous deposits. In 5.9% of cases, no deposits were found. 7) U/A findings between gross hematuric episodes showed no microscopic hematuria and HQ proteinuria in 30.9%, microscopic hematuria without proteinuria in 40.0% and proteinuria with/without microscopic hematuria in 29.1% of cases. 8) During follow-up, 2 cases expired due to ARF and CRF, each, and another 2 became the state of mild renal insufficiency. 9) More severe form of mode of presentation, heavy proteinuria or accompanying tubulointerstitial changes was noted more frequently in those with more severe glomerular changes. And each of such findings as initial hypertension, initial azotemia, accompanying loin pain, presence of proteinuria between gross hematuric episodes and accompanying capillary stain-ing of IgA in immunofluorescent study was revealed as a poor prognostic indicator so far as the maintenance of normal renal function was concerned.
Key Words: IgA nephropathy, Children, Clinico-pathological correlation, Prognostic indicator

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