Evidence map›Paper›PMID 28236143›Full record

ArticlePediatric nephrology (Berlin, Germany)2017

C3 glomerulopathy and eculizumab: a report on four paediatric cases.

Célia Lebreton, Justine Bacchetta, Frédérique Dijoud, Lucie Bessenay, Véronique Fremeaux-Bacchi, Anne Laure Sellier-Leclerc

Abstract readCase Reports
PubMed Publisher
In one paragraph

Article in Pediatric nephrology (Berlin, Germany), 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.

0numbers the graph read from it
0cells of the map it votes in
23citing papers in PubMed
3.2field-weighted citation impact, top 8% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

23 citing papers in PubMed, 34 citations in OpenAlex.

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  11. Predictors of poor kidney outcome in children with C3 glomerulopathy.Pediatric nephrology (Berlin, Germany) · 2021
    Article
  12. Case reports in nephrology · 2021
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  16. Recurrent andFrontiers in immunology · 2019
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors at 4 institutions in 1 country.

Célia LebretonService 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.ORCID 0000-0002-0566-2658
Justine BacchettaService 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.
Frédérique DijoudFaculté de Médecine Lyon Est, Université Lyon 1, Lyon, France.
Lucie BessenayService de Pédiatrie, CHU Clermont Ferrand, Clermont Ferrand, France.
Véronique Fremeaux-BacchiLaboratoire d'Immunologie, Hôpital Georges Pompidou, Assistance publique-Hôpitaux de Paris (AP-HP), Paris, France.
Anne Laure Sellier-LeclercService 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. anne-laure.sellier-leclerc@chu-lyon.fr.
Hôpital Femme Mère Enfant · FRUniversité Claude Bernard Lyon 1 · FRAssistance Publique – Hôpitaux de Paris · FRCentre Hospitalier Universitaire de Clermont-Ferrand · FR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundEculizumab 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.

conclusionThese 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.

Indexed as

AdolescentAntibodies, Monoclonal, HumanizedChildComplement C3Complement C3 Nephritic FactorComplement Pathway, AlternativeFemaleGlomerulonephritis, MembranoproliferativeGraft RejectionHumansImmunity, HumoralKidneyKidney Failure, ChronicKidney TransplantationMaleOff-Label UseAntibodies, Monoclonal, HumanizedComplement C3Complement C3 Nephritic FactoreculizumabC3 glomerulonephritisChildrenEculizumabPaediatricsRecurrence post-transplantation

Identifiers

PMID28236143
OpenAlexW2590684495

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.