Kidney transplantation in children and adolescents with C3 glomerulopathy or immune complex membranoproliferative glomerulonephritis: a real-world study within the CERTAIN research network

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dc.authorscopusidOzan Özkaya / 7003365098
dc.authorwosidOzan Özkaya / DKK-1373-2022
dc.contributor.authorPatry, Christian
dc.contributor.authorWebb, Nicholas J. A.
dc.contributor.authorFeisst, Manuel
dc.contributor.authorKrupka, Kai
dc.contributor.authorBecker, Jan
dc.contributor.authorBald, Martin
dc.contributor.authorAntoniello, Benedetta
dc.contributor.authorBilge, Ilmay
dc.contributor.authorGülhan, Bora
dc.contributor.authorHogan, Julien
dc.contributor.authorKanzelmeyer, Nele
dc.contributor.authorÖzkaya, Ozan
dc.contributor.authorBuescher, Anja
dc.contributor.authorSellier-Leclerc, Anne-Laure
dc.contributor.authorShenoy, Mohan
dc.contributor.authorWeber, Lutz T.
dc.contributor.authorFichtner, Alexander
dc.contributor.authorHoecker, Britta
dc.contributor.authorMeier, Matthias
dc.date.accessioned2025-04-18T10:30:18Z
dc.date.available2025-04-18T10:30:18Z
dc.date.issued2024
dc.departmentİstinye Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü
dc.description.abstractBackgroundComplement 3 glomerulopathy (C3G) and immune complex membranoproliferative glomerulonephritis (IC-MPGN) are ultra-rare chronic kidney diseases with an overall poor prognosis, with approximately 40-50% of patients progressing to kidney failure within 10 years of diagnosis. C3G is characterized by a high rate of disease recurrence in the transplanted kidney. However, there is a lack of published data on clinical outcomes in the pediatric population following transplantation.MethodsIn this multicenter longitudinal cohort study of the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN) registry, we compared the post-transplant outcomes of pediatric patients with C3G (n = 17) or IC-MPGN (n = 3) with a matched case-control group (n = 20).ResultsEleven of 20 children (55%) with C3G or IC-MPGN experienced a recurrence within 5 years post-transplant. Patients with C3G or IC-MPGN had a 5-year graft survival of 61.4%, which was significantly (P = 0.029) lower than the 5-year graft survival of 90% in controls; five patients with C3G or IC-MPGN lost their graft due to recurrence during this observation period. Both the 1-year (20%) and the 5-year (42%) rates of biopsy-proven acute rejection episodes were comparable between patients and controls. Complement-targeted therapy with eculizumab, either as prophylaxis or treatment, did not appear to be effective.ConclusionsThese data in pediatric patients with C3G or IC-MPGN show a high risk of post-transplant disease recurrence (55%) and a significantly lower 5-year graft survival compared to matched controls with other primary kidney diseases. These data underscore the need for post-transplant patients for effective and specific therapies that target the underlying disease mechanism.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information
dc.description.sponsorshipNovartis Pharma AG
dc.identifier.citationPatry, C., Webb, N. J., Feißt, M., Krupka, K., Becker, J., Bald, M., ... & Tönshoff, B. (2024). Kidney transplantation in children and adolescents with C3 glomerulopathy or immune complex membranoproliferative glomerulonephritis: a real-world study within the CERTAIN research network. Pediatric Nephrology, 39(12), 3569-3580.
dc.identifier.doi10.1007/s00467-024-06476-5
dc.identifier.endpage3580
dc.identifier.issn0931-041X
dc.identifier.issn1432-198X
dc.identifier.issue12
dc.identifier.pmid39110227
dc.identifier.scopus2-s2.0-85200566955
dc.identifier.scopusqualityQ1
dc.identifier.startpage3569
dc.identifier.urihttp://dx.doi.org/10.1007/s00467-024-06476-5
dc.identifier.urihttps://hdl.handle.net/20.500.12713/7094
dc.identifier.volume39
dc.identifier.wosWOS:001285469700001
dc.identifier.wosqualityQ1
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.institutionauthorÖzkaya, Ozan
dc.institutionauthoridOzan Özkaya / 0000-0002-0198-1221
dc.language.isoen
dc.publisherSpringer
dc.relation.ispartofPediatric nephrology
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectComplement 3 Glomerulopathy
dc.subjectImmune Complex Membranoproliferative Glomerulonephritis
dc.subjectRecurrence
dc.subjectPediatric Kidney Transplantation
dc.subjectGraft Survival
dc.titleKidney transplantation in children and adolescents with C3 glomerulopathy or immune complex membranoproliferative glomerulonephritis: a real-world study within the CERTAIN research network
dc.typeArticle

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