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- Title
Primary focal segmental glomerular sclerosis in children: clinical course and prognosis.
- Authors
Kyung Hoon Paik; Bum Hee Lee; Hee Yeon Cho; Hee Gyung Kang; Il Soo Ha; Hae Il Cheong; Dong-Kyu Jin; Kyung Chul Moon; Yong Choi
- 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.
- Subjects
KIDNEY disease diagnosis; CHRONIC kidney failure; NEPHROTIC syndrome; CHRONIC diseases; SERUM; PROTEINURIA; PEDIATRIC nephrology diagnosis
- Publication
Pediatric Nephrology, 2007, Vol 22, Issue 3, p389
- ISSN
0931-041X
- Publication type
Article
- DOI
10.1007/s00467-006-0301-5