Evidence map›Paper›PMID 42318533›Full record

ArticleKidney international reports2026

Atypical Hemolytic Uremic Syndrome in Children and Adults With the Hot Spot C3 Gene Variant p.Arg161Trp.

Lieke Ter Steeg, Romy N Bouwmeester, Mendy Ter Avest, Frederike J Bemelman, Antonia H M Bouts, Obbo W Bredewold, Eiske Dorresteijn, Mark Eijgelsheim, Flore A P T Engels, Valentina Gracchi and 14 more

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In one paragraph

Article in Kidney international reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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1 · What the graph read from it

What it found

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2 · The registry

The trial behind it

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3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

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5 · Who and what money

Authors and funding

24 authors.

Lieke Ter SteegDepartment of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
Romy N BouwmeesterDepartment of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
Mendy Ter AvestDepartment of Pharmacy, Pharmacology & Toxicology, Radboud University Medical Center, Nijmegen, The Netherlands.
Frederike J BemelmanDepartment of Nephrology, Amsterdam University Medical Center, Amsterdam, The Netherlands.
Antonia H M BoutsDepartment of Pediatric Nephrology, Emma Children's Hospital, Amsterdam University Medical Center, Amsterdam, The Netherlands.
Obbo W BredewoldDepartment of Nephrology, Leiden University Medical Center, Leiden, The Netherlands.
Eiske DorresteijnDepartment of Pediatric Nephrology, Sophia Children's Hospital, Erasmus Medical Center, Rotterdam, The Netherlands.
Mark EijgelsheimDepartment of Internal Medicine, Division of Nephrology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Flore A P T EngelsDepartment of Pediatric Nephrology, MosaKids Children's Hospital, Maastricht University Medical Center, Maastricht, The Netherlands.
Valentina GracchiDepartment of Pediatric Nephrology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Rob Ter HeineDepartment of Pharmacy, Pharmacology & Toxicology, Radboud University Medical Center, Nijmegen, The Netherlands.
Mandy G Keijzer-VeenDepartment of Pediatric Nephrology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
Anne-Els van de LogtDepartment of Nephrology, Radboud University Medical Center, Nijmegen, The Netherlands.
Wilbert A G van der MeijdenDepartment of Nephrology, Radboud University Medical Center, Nijmegen, The Netherlands.
Roos W G van RooijDepartment of Pediatric Nephrology, Willem-Alexander Children's Hospital, Leiden University Medical Center, Leiden, The Netherlands.
David SeversDepartment of Internal Medicine, Division of Nephrology and Transplantation, Erasmus Medical Center, University Medical Center Rotterdam, Rotterdam, The Netherlands.
Sjoerd A M E G TimmermansDepartment of Internal Medicine, Section Nephrology and Immunology, Maastricht University Medical Center, Maastricht, The Netherlands.
Arjan D van ZuilenDepartment of Nephrology and Hypertension, University Medical Center Utrecht, Utrecht, The Netherlands.
Marloes A H M MichelsDepartment of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
Kioa L WijnsmaDepartment of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
Lambertus P W J van den HeuvelDepartment of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
Jack F M WetzelsDepartment of Nephrology, Radboud University Medical Center, Nijmegen, The Netherlands.
Caroline DuineveldDepartment of Nephrology, Radboud University Medical Center, Nijmegen, The Netherlands.
Nicole C A J van de KarDepartment of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Introduction: The gain-of-function variant C3 p.Arg161Trp is found in a quarter of Dutch patients with complement-mediated atypical hemolytic uremic syndrome (CaHUS). In this study, we describe the clinical phenotype of C3 p.Arg161Trp-associated CaHUS. Methods: Dutch patients with CaHUS with the C3 p.Arg161Trp variant, identified before October 2023, were included in this retrospective, observational study. Results: A total of 37 patients (11 children and 26 adults) were included, with onset before and after availability of eculizumab. Presentation at onset differed between children and adults, specifically in platelet count (21 vs. 79 x 10 Conclusion: The clinical phenotype of C3 p.Arg161Trp-associated CaHUS has significant heterogeneity, with differences in presentation and outcomes between children and adults. Children present with more profound hemolysis but milder acute kidney injury (AKI). Furthermore, C3 p.Arg161Trp is linked to a high risk of relapse.

Indexed as

atypical hemolytic uremic syndromeC3 p.Arg161Trpclinical phenotypecomplementeculizumabthrombotic microangiopathy

Identifiers

PMID42318533
PMCPMC13273819

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