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Primary focal segmental glomerular sclerosis in children:clinical course and prognosis
DC Field | Value | Language |
---|---|---|
dc.contributor.author | Paik, Kyung Hoon | - |
dc.contributor.author | Lee, Bum Hee | - |
dc.contributor.author | Cho, Hee Yeon | - |
dc.contributor.author | Kang, Hee Gyung | - |
dc.contributor.author | Ha, Il Soo | - |
dc.contributor.author | Cheong, Hae Il | - |
dc.contributor.author | Jin, Dong-Kyu | - |
dc.contributor.author | Moon, Kyung Chul | - |
dc.contributor.author | Choi, Yong | - |
dc.date.accessioned | 2009-09-26T05:47:06Z | - |
dc.date.available | 2009-09-26T05:47:06Z | - |
dc.date.issued | 2006-10-24 | - |
dc.identifier.citation | Pediatr Nephrol 22:389-395 | en |
dc.identifier.issn | 0931-041X (print) | - |
dc.identifier.issn | 1432-198X (online) | - |
dc.identifier.uri | https://hdl.handle.net/10371/9890 | - |
dc.description.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. | en |
dc.language.iso | en | - |
dc.publisher | Springer Verlag | en |
dc.subject | Primary focal segmental glomerular sclerosis | en |
dc.subject | FSGS | en |
dc.subject | Nephrotic syndrome | en |
dc.subject | Asymptomatic proteinuria | en |
dc.subject | Steroid responsiveness | en |
dc.subject | Chronic renal failure | en |
dc.title | Primary focal segmental glomerular sclerosis in children:clinical course and prognosis | en |
dc.type | Article | en |
dc.contributor.AlternativeAuthor | 박경훈 | - |
dc.contributor.AlternativeAuthor | 이범희 | - |
dc.contributor.AlternativeAuthor | 조희연 | - |
dc.contributor.AlternativeAuthor | 강희경 | - |
dc.contributor.AlternativeAuthor | 하일수 | - |
dc.contributor.AlternativeAuthor | 정해일 | - |
dc.contributor.AlternativeAuthor | 진동규 | - |
dc.contributor.AlternativeAuthor | 문경철 | - |
dc.contributor.AlternativeAuthor | 최용 | - |
dc.identifier.doi | 10.1007/s00467-006-0301-5 | - |
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