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Primary focal segmental glomerular sclerosis in children:clinical course and prognosis

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Authors
Paik, Kyung Hoon; Lee, Bum Hee; Cho, Hee Yeon; Kang, Hee Gyung; Ha, Il Soo; Cheong, Hae Il; Jin, Dong-Kyu; Moon, Kyung Chul; Choi, Yong
Issue Date
2006-10-24
Publisher
Springer Verlag
Citation
Pediatr Nephrol 22:389-395
Keywords
Primary focal segmental glomerular sclerosisFSGSNephrotic syndromeAsymptomatic proteinuriaSteroid responsivenessChronic renal failure
Abstract
To review the clinical course and identify prognostic factors, we retrospectively analyzed 92 children with steroid-resistant primary focal segmental glomerulosclerosis (FSGS). The mean age of onset was 80.4+/-42.4 months. The mean follow-up duration was 98.2+/-63.3 months. Eighty-five patients presented with nephrotic syndrome and seven presented with asymptomatic proteinuria. Thirty-three patients were initial responders to steroid treatment (late non-responders) and 59 were initial nonresponders. At last follow-up, 36 patients (39.1%) were in complete remission, and 29 (31.5%) progressed to chronic renal failure (CRF). Renal survival rates at 5, 10, and 15 years were 84, 64, and 53%, respectively. By morphological classification, there were tip variants (6.1%), collapsing variants (10.6%), cellular variants (1.5%), perihilar variants (9.1%), and NOS (not otherwise specified, 72.7%). Among the variants, there were no significant differences in age of onset, degree of proteinuria, response to treatment, or progression to CRF. Poor prognostic factors for CRF included: asymptomatic proteinuria at presentation, initial renal insufficiency, higher segmental sclerosis (%), severe tubulointerstitial change, initial nonresponse, and absence of remission. In the multivariate analysis, an increase in the initial serum creatinine and resistance to treatment were independent risk factors for CRF. A more prolonged use of corticosteroid therapy and early introduction of cyclosporin A (CsA) may improve the prognosis for primary FSGS in patients with initial steroid nonresponsiveness.
ISSN
0931-041X (print)
1432-198X (online)
Language
English
URI
http://hdl.handle.net/10371/9890
DOI
https://doi.org/10.1007/s00467-006-0301-5
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College of Medicine/School of Medicine (의과대학/대학원)Pathology (병리학전공)Journal Papers (저널논문_병리학전공)
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