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A Clinicopathological Study on the Biopsy-proven Henoch-Schonlein Purpura Nephritis in Children.

Journal of the Korean Pediatric Society 1986;29(3):265-276.
Published online March 31, 1986.
A Clinicopathological Study on the Biopsy-proven Henoch-Schonlein Purpura Nephritis in Children.
Jong Yoon Kim1, Yong Choi1, Kwang Wook Ko1, Young il Kim2
1Department of Pediatrics, college of medicine, Seoul, National University,Korea.
2Department of pathology, college of medicine, Seoul, National University,Korea.
신생검을 시행한 소아 Henoch-Schonlein 신염에 관한 임상 및 병리학적 고찰
김종윤1, 최 용1, 고광욱1, 김용일2
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 병리학교실
Abstract
40 patients with Henoch-Schonlein nephritis in whom a per cutaneous renal biopsy was performed at department of pediatrics, Seoul National University Hospital during the periods Jan. 1978-Jan. 1985 inclusive were analyzed in view of clinicopathological correlation. The results were as follows: 1) Of the 40 patients reviewed, 24 cases were male and 16 female. The mean age of onset was 9 years 9 months with the range of 4 years and 11 months to 11 years and 4 months. The peak incidence was noted at 9 years of age in 8 cases. 2) Renal symptoms were noted within 3 months after onset of purpura in the majority of cases(27 out of 32 cases). In 8 cases, renal symptoms preceded the occurrence of purpura. The interval was less than 6 months in 6 cases, but in 2 cases, it was 1 year and 7 months and 4 years and 7 months respectively. 3) The renal presentations recorded within 3 months of onset were; nephrotic syndrome and acute nephritic syndrome in 10 cases, nephrotic syndrome and hematuria in 10 cases, acute nephritic syndrome and proteinuria in 1 case, hematuria with proteinuria in 17 cases and hematuria without proteinuria in 2 cases. 4) Preceding URI symptoms were present in 13 cases and initial hypertension in 9 cases. Pyuria was detected in 15 cases. 5) Initial serum IgA was checked in 37 cases and was elevated in 10 cases. The serum complement Cs, C4 and total hemolytic activity CH50 were within normal limit in most cases. The selectivity index was above 0.20 in 18 out of 25 cases. 6) The mean interval between the onset of renal symptoms and renal biopsy was 7 mon-ths. The light microscopic findings according to the ISKDC classification ^ere; grade lb 1 cases Ha 6 cases, Hb 24 cases, IVa 1 case, IVb 5 cases and Vb 3 cases. More extensive histological changes were seemed to be associated with a poor prognosis. But no statistical significance could be found due to the small number of the patients. On immunofluo-rescent microscopy, IgA, IgG, IgM and C3 were deposited more predominantly in mesangium. Electron microscopic findings on 33 cases revealed mesangial electron dense deposit, alone or in combination with subepithelial, subendothelial or intramembranous deposits, in 28 cases. In 5 cases subephithelial and/or suben dothelial electron dense deposits were noted, without mesangial depositss. 7) Among the 40 cases, 24 cases were followed for 1 year or more and their mean duration of follow-up was 3 years and 1 month. The clinical status of each patient was classified according to the scheme originally devised by Meadow et al” and modified by Counahanetal. In this study, state A was not included, because it was not the indication for renalbiopsy. Both deterioration and recovery have occurred since onset. The proportion of patients with normal or minor urinary abnormalities (state B alone) increased from 29% at onset to 63% with follow-up for 1 year or more (state A and B). About 38% of the patients had active renal disease, renal impairment, or had died (state C and D) after 1 year or more,compared with 71% at onset. 8) Treatment consisted of corticosteroids alone, antiplatelet agents alone, corticosteroids and antiplatelet agents or corticosteroids, antiplatelet agents and immunosuppressives. 15 patients received such treatments and 9 none. When patients with any treatment were compared with those with no treatment in respect of the outcome assessed as improved, stationary or aggravated, no significant difference was evident between the two groups (P>0.05).
Key Words: Henoch-Schonlein Nephritis, Percutaneous renal biopsy.


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