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

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dc.contributor.authorPaik, Kyung Hoon-
dc.contributor.authorLee, Bum Hee-
dc.contributor.authorCho, Hee Yeon-
dc.contributor.authorKang, Hee Gyung-
dc.contributor.authorHa, Il Soo-
dc.contributor.authorCheong, Hae Il-
dc.contributor.authorJin, Dong-Kyu-
dc.contributor.authorMoon, Kyung Chul-
dc.contributor.authorChoi, Yong-
dc.date.accessioned2009-09-26T05:47:06Z-
dc.date.available2009-09-26T05:47:06Z-
dc.date.issued2006-10-24-
dc.identifier.citationPediatr Nephrol 22:389-395en
dc.identifier.issn0931-041X (print)-
dc.identifier.issn1432-198X (online)-
dc.identifier.urihttps://hdl.handle.net/10371/9890-
dc.description.abstractTo 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.isoen-
dc.publisherSpringer Verlagen
dc.subjectPrimary focal segmental glomerular sclerosisen
dc.subjectFSGSen
dc.subjectNephrotic syndromeen
dc.subjectAsymptomatic proteinuriaen
dc.subjectSteroid responsivenessen
dc.subjectChronic renal failureen
dc.titlePrimary focal segmental glomerular sclerosis in children:clinical course and prognosisen
dc.typeArticleen
dc.contributor.AlternativeAuthor박경훈-
dc.contributor.AlternativeAuthor이범희-
dc.contributor.AlternativeAuthor조희연-
dc.contributor.AlternativeAuthor강희경-
dc.contributor.AlternativeAuthor하일수-
dc.contributor.AlternativeAuthor정해일-
dc.contributor.AlternativeAuthor진동규-
dc.contributor.AlternativeAuthor문경철-
dc.contributor.AlternativeAuthor최용-
dc.identifier.doi10.1007/s00467-006-0301-5-
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