Predictors of poor kidney outcome in children with C3 glomerulopathy

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dc.authoridCanpolat, Nur/0000-0002-3420-9756
dc.authoridTaşdemir, Mehmet/0000-0002-5579-6339
dc.authoridSaygili, Seha Kamil/0000-0002-2424-6959
dc.authoridSoylu, Alper/0000-0002-5901-7398
dc.authoridOzcelik, Gul/0000-0001-9394-2977
dc.authoridÇELAKIL, MEHTAP/0000-0002-5354-1455
dc.authoridTekcan Karalı, Demet/0000-0001-7013-1354
dc.authorwosidCanpolat, Nur/AHE-2082-2022
dc.authorwosidTaşdemir, Mehmet/V-7413-2017
dc.authorwosidSaygili, Seha Kamil/GLU-6742-2022
dc.authorwosidBakkaloglu, Sevcan/AAE-9884-2021
dc.authorwosidSoylu, Alper/AAR-7694-2020
dc.authorwosidYuruk Yildirim, Zeynep Nagehan/AAE-1511-2020
dc.authorwosidOzcelik, Gul/GQQ-3364-2022
dc.contributor.authorPinarbasi, Ayse Seda
dc.contributor.authorDursun, Ismail
dc.contributor.authorGokce, Ibrahim
dc.contributor.authorcomak, Elif
dc.contributor.authorSaygili, Seha
dc.contributor.authorBayram, Meral Torun
dc.contributor.authorDonmez, Osman
dc.date.accessioned2024-08-04T20:49:04Z
dc.date.available2024-08-04T20:49:04Z
dc.date.issued2021
dc.departmentİnönü Üniversitesien_US
dc.description.abstractBackground C3 glomerulopathy (C3G) is characterized by heterogeneous clinical presentation, outcome, and predominant C3 accumulation in glomeruli without significant IgG. There is scarce outcome data regarding childhood C3G. We describe clinical and pathological features, treatment and outcomes, and risk factors for progression to chronic kidney disease stage 5 (CKD5) in the largest pediatric series with biopsy-proven C3G. Methods Sixty pediatric patients with C3G from 21 referral centers in Turkey were included in this retrospective study. Patients were categorized according to CKD stage at last visit as CKD5 or non-CKD5. Demographic data, clinicopathologic findings, treatment, and outcome data were compared and possible risk factors for CKD5 progression determined using Cox proportional hazards model. Results Mean age at diagnosis was 10.6 +/- 3.0 years and follow-up time 48.3 +/- 36.3 months. Almost half the patients had gross hematuria and hypertension at diagnosis. Nephritic-nephrotic syndrome was the commonest presenting feature (41.6%) and 1/5 of patients presented with nephrotic syndrome. Membranoproliferative glomerulonephritis was the leading injury pattern, while 40 patients had only C3 staining. Patients with DDD had significantly lower baseline serum albumin compared with C3GN. Eighteen patients received eculizumab. Clinical remission was achieved in 68.3%. At last follow-up, 10 patients (16.6%) developed CKD5: they had lower baseline eGFR and albumin and higher frequency of nephrotic syndrome and dialysis requirement than non-CKD5 patients. Lower serum albumin and eGFR at diagnosis were independent predictors for CKD5 development. Conclusions Children with C3G who have impaired kidney function and hypoalbuminemia at diagnosis should be carefully monitored for risk of progression to CKD5.en_US
dc.identifier.doi10.1007/s00467-020-04799-7
dc.identifier.endpage1205en_US
dc.identifier.issn0931-041X
dc.identifier.issn1432-198X
dc.identifier.issue5en_US
dc.identifier.pmid33130981en_US
dc.identifier.scopus2-s2.0-85094862751en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage1195en_US
dc.identifier.urihttps://doi.org/10.1007/s00467-020-04799-7
dc.identifier.urihttps://hdl.handle.net/11616/99608
dc.identifier.volume36en_US
dc.identifier.wosWOS:000583120600001en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherSpringeren_US
dc.relation.ispartofPediatric Nephrologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectComplementen_US
dc.subjectCKD stage 5en_US
dc.subjectChildrenen_US
dc.subjectC3 glomerulopathyen_US
dc.subjectPredictorsen_US
dc.titlePredictors of poor kidney outcome in children with C3 glomerulopathyen_US
dc.typeArticleen_US

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