Clinicopathologic Correlates Predict the Outcome in Children With Steroid-Resistant Idiopathic Nephrotic Syndrome Treated With Pulse Methylprednisolene Therapy

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dc.contributor.authorKirpekar, Rashmiko
dc.contributor.authorYorgin, Peter D.ko
dc.contributor.authorTune, Bruce M.ko
dc.contributor.authorKim, Mi-Kyungko
dc.contributor.authorSibley, Richard K.ko
dc.date.accessioned2008-07-11T05:09:58Z-
dc.date.available2008-07-11T05:09:58Z-
dc.date.created2012-02-06-
dc.date.created2012-02-06-
dc.date.issued2002-06-
dc.identifier.citationAMERICAN JOURNAL OF KIDNEY DISEASES, v.39, no.6, pp.1143 - 1152-
dc.identifier.issn0272-6386-
dc.identifier.urihttp://hdl.handle.net/10203/5614-
dc.description.abstractAlthough pulse methylprednisolone therapy (PMT) has been used successfully in the management of children with steroid-resistant nephrotic syndrome (SRNS), the relationship between initial presenting findings and renal histological characteristics to the subsequent clinical response to PMT is unknown. A retrospective analysis was conducted in a study cohort of 42 children (30 boys, 12 girls; mean age, 7.4 +/- 4.7 years) with SRNS administered PMT between June 1976 and July 1994 at Stanford University (Stanford, CA). Four diagnostic categories were created: group I, minimal change disease with or without mesangial hypercellularity (n = 10); group II, mesangial proliferation (n = 7); group III, focal segmental glomerulosclerosis (FSGS) with or without mesangial hypercellularity (n = 10); and group IV, FSGS plus mesangial proliferation (n = 15). Primary variables analyzed were remission in response to PMT with or without alkylating agent therapy and end-stage renal disease (ESRD). Remission rates were best in group I (90%) and worst in group IV (46%). With the exception of hematuria, presenting clinical features did not correlate with outcome. Segmental sclerosis, glomerular adhesion to Bowman's capsule, epithelia[ sloughing, corona (segmental scar surrounded by visceral epithelial cells), subepithelial deposits, inflammatory cells, and percentage of interstitium, immunoglobulin M (IgM), IgG, and C3 deposition univariately correlated with ESRD in univariate analysis. In a multivariate logistic regression model, only segmental sclerosis (P = 0.008) correlated with ESRD. Histological analysis is important because it identifies features, including segmental sclerosis, that portend a poor prognosis in children with SRNS. (C) 2002 by the National Kidney Foundation, Inc.-
dc.description.sponsorshipsupported in part by grant no. 5 T32 DK07013-13 from the National Istitute of Diabetes and Digestive and Kidney Diseases (R.K.)en
dc.languageEnglish-
dc.language.isoen_USen
dc.publisherW B Saunders Co-Elsevier Inc-
dc.subjectFOCAL-SEGMENTAL GLOMERULOSCLEROSIS-
dc.subjectINTRAVENOUS METHYLPREDNISOLONE-
dc.subjectCOLLAPSING GLOMERULOPATHY-
dc.subjectALKYLATING-AGENTS-
dc.subjectPODOCYTES-
dc.subjectDISEASE-
dc.subjectPHENOTYPE-
dc.subjectSCLEROSIS-
dc.subjectFEATURES-
dc.subjectDAMAGE-
dc.titleClinicopathologic Correlates Predict the Outcome in Children With Steroid-Resistant Idiopathic Nephrotic Syndrome Treated With Pulse Methylprednisolene Therapy-
dc.typeArticle-
dc.identifier.wosid000176399600003-
dc.identifier.scopusid2-s2.0-0036272277-
dc.type.rimsART-
dc.citation.volume39-
dc.citation.issue6-
dc.citation.beginningpage1143-
dc.citation.endingpage1152-
dc.citation.publicationnameAMERICAN JOURNAL OF KIDNEY DISEASES-
dc.identifier.doi10.1053/ajkd.2002.33382-
dc.embargo.liftdate9999-12-31-
dc.embargo.terms9999-12-31-
dc.contributor.localauthorKim, Mi-Kyung-
dc.contributor.nonIdAuthorKirpekar, Rashmi-
dc.contributor.nonIdAuthorYorgin, Peter D.-
dc.contributor.nonIdAuthorTune, Bruce M.-
dc.contributor.nonIdAuthorSibley, Richard K.-
dc.description.isOpenAccessN-
dc.type.journalArticleArticle-
dc.subject.keywordAuthorfocal segmental glomerulosclerosis (FSGS)-
dc.subject.keywordAuthorminimal change disease (MCD)-
dc.subject.keywordAuthorchlorambucil-
dc.subject.keywordAuthorcyclophosphamide-
dc.subject.keywordAuthorproteinuria-
dc.subject.keywordAuthorglomerular morphometrics-
dc.subject.keywordAuthoroutcome-
dc.subject.keywordAuthorpathology-
dc.subject.keywordPlusFOCAL-SEGMENTAL GLOMERULOSCLEROSIS-
dc.subject.keywordPlusINTRAVENOUS METHYLPREDNISOLONE-
dc.subject.keywordPlusCOLLAPSING GLOMERULOPATHY-
dc.subject.keywordPlusALKYLATING-AGENTS-
dc.subject.keywordPlusPODOCYTES-
dc.subject.keywordPlusDISEASE-
dc.subject.keywordPlusPHENOTYPE-
dc.subject.keywordPlusSCLEROSIS-
dc.subject.keywordPlusFEATURES-
dc.subject.keywordPlusDAMAGE-
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