[Clinical and pathological characteristics of focal segmental glomerulosclerosis in children].
OBJECTIVE
To investigate the clinical and pathological characteristics of focal segmental glomerulosclerosis (FSGS) in children.


METHODS
The data of 38 children,aged from one and half to 15 years, 25 boys and 13 girls, with primary FSGS were studied retrospectively.


RESULTS
Majority of the cases in this study were school-aged children. The average age of initial onset was 8.9 +/- 3.68 years. The ratio of boys to girls was 1.92. The clinical manifestation included isolated proteinuria in 3 cases, proteinuria and hematuria in 1 and nephrotic syndrome in 34 (simple type in 16 and nephritic type in 18). Of 38 cases, 24 (63%) presented with hematuria, 11 (29%) with hypertension and 7 (18%) with decreased creatinine clearance. The pathologic classification included perihilar variant in 17 cases, peripheral variant in 14 and tip variant in 7. The predominant clinical feature of children with tip variant was simple type of nephrotic syndrome (86%). Microscopic hematuria was not common (29%). Blood pressure and renal function were normal. The children with diffuse mesangial hypercellularity superimposed on changes of FSGS (in 21 of 38 cases) were more likely to have hematuria (76%) and less simple nephrotic syndrome (30%). The initial treatment response to prednisone in 34 cases with nephrotic syndrome showed sensitive in 12 cases, resistant in 21 and unknown in 1. Transition from sensitive to resistant occurred in six of 12 children. Three of 4 cases with non-nephrotic syndrome showed no response and the remaining one had unknown response. It was found that 44% of children who received cyclophosphamide and 83% of children who received pulse methylprednisolone and pulse cyclophosphamide or cyclosporin A in addition to oral steroids had complete or partial remission. Correlation analysis showed that the level of proteinuria after treatment was correlated directly with renal tubulointerstitial lesion and renal function (Pr = 0.48, P < 0.05; Pr = 0.45, P < 0.05).


CONCLUSIONS
FSGS was common in school-aged children. The predominant presenting feature was nephrotic syndrome. Hematuria was common. Hypertension and renal insufficiency were less frequently seen. The renal biopsy showed multiple variants. Pulse methylprednisolone and pulse cyclophosphamide or cyclosporin A treatments showed relatively good response.
Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.
Jian-ping
JP
Huang
Jing-jing
JJ
Zhang
Jing-cheng
JC
Liu
Ying-nan
YN
Chen
Yong
Y
Yao
Hui-jie
HJ
Xiao
Ji-yun
JY
Yang
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000097:blood; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D005938:Glucocorticoids / Q000627:therapeutic use; D006417:Hematuria / Q000209:etiology; D006801:Humans; D006973:Hypertension / Q000209:etiology; D007223:Infant; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D011379:Prognosis; D011507:Proteinuria / Q000209:etiology; D012189:Retrospective Studies; D016896:Treatment Outcome
D005938:Glucocorticoids; D003404:Creatinine; D008775:Methylprednisolone