C3 glomerulopathy and eculizumab: a report on four paediatric cases.
Background: Eculizumab may be used to treat C3-glomerulopathy (C3G), a rare but severe glomerular disease. Diagnosis and Treatment: Patients 1, 2 and 3 were diagnosed with nephritic syndrome with alternative complement pathway activation (low C3, C3Nef-positive) and C3G at the age of 9, 13 and 12 ye...
| Published in: | Pediatric Nephrology Vol. 32; no. 6; pp. 1023 - 1029 |
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| Main Authors: | , , , , , |
| Format: | case study research tables/charts Journal Article |
| Published: |
Springer Nature
Jun2017
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| Online Access: | View this record in EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=122598295&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 122598295 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 0931041X EF1 jtl: Pediatric Nephrology issn: 0931041X maglogo: N pubinfo: dt: Jun2017 vid: 32 iid: 6 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 122598295 122598295 144169826 122598295 10.1007/s00467-017-3619-2 122598295 ppf: 1023 ppct: 6 formats: fmt: @attributes: type: P tig: atl: C3 glomerulopathy and eculizumab: a report on four paediatric cases. aug: au: Lebreton, Célia Bacchetta, Justine Dijoud, Frédérique Bessenay, Lucie Fremeaux-Bacchi, Véronique Sellier-Leclerc, Anne affil: Service de Néphrologie Rhumatologie Dermatologie Pédiatriques, Centre de Référence des Maladies Rénales Rares Hôpital Femme Mère Enfant , Hospices Civils de Lyon , 59 boulevard Pinel 69677 Bron France sug: subj: Kidney Glomerulus Pathology Glomerulonephritis Drug Therapy Antibodies, Monoclonal Therapeutic Use Glomerulonephritis Pathology Glomerulonephritis Symptoms Proteinuria Urine Disease Progression Kidney Failure, Chronic Surgery Kidney Transplantation Recurrence Graft Rejection Glomerulonephritis Classification Antibodies, Monoclonal Pharmacodynamics Case Studies Male Female Child Adolescence Human Treatment Outcomes Child: 6-12 years Adolescent: 13-18 years Male Female ab: Background: Eculizumab may be used to treat C3-glomerulopathy (C3G), a rare but severe glomerular disease. Diagnosis and Treatment: Patients 1, 2 and 3 were diagnosed with nephritic syndrome with alternative complement pathway activation (low C3, C3Nef-positive) and C3G at the age of 9, 13 and 12 years, respectively. Treatment with eculizumab normalized proteinuria within 1, 2 and 7 months, respectively. Proteinuria relapsed when eculizumab was withdrawn, but the re-introduction of eculizumab normalized proteinuria. Patient 4 was diagnosed with C3G at 9 years of age, with progression to end-stage renal disease within 2 years, followed by a first renal transplantation (R-Tx) with early disease recurrence and graft loss within 39 months. After a second R-Tx, she rapidly presented with biological and histological recurrence: therapy with eculizumab was started, with no effect on proteinuria after 5 months, in a complex clinical setting (i.e. association of C3G recurrence, humoral rejection and BK nephritis). Eculizumab was withdrawn due to multiple viral reactivations, but the re-introduction of the drug a few months later enabled a moderate decrease in proteinuria. Conclusion: These cases illustrate the efficacy of eculizumab, at least on native kidneys, in paediatric C3G. However, larger international studies are warranted to confirm the benefit and safety of eculizumab therapy. pubtype: Academic Journal doctype: case study research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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