Evidence map›Paper›PMID 41663882›Full record

ArticleEuropean journal of immunology2026

Meningococcal Serogroup Y Meningitis Reveals Inborn Factor B Deficiency.

Camille Bougeard, Eléonore Eskander, Paula Vieira Martins, Joan Bitan, Julia Roquigny, Christèle Kyheng, Tamazoust Guiddir, Véronique Frémeaux-Bacchi, Cécile Gonnin, Carine ElSissy

Abstract readCase Reports
In one paragraph

Article in European journal of immunology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
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

2 citing papers in PubMed.

  1. Article
  2. Article
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

10 authors.

Camille BougeardDepartment of Immunology, Assistance Publique- Hôpitaux de Paris (AP-HP), Georges Pompidou European Hospital, Paris, France.
Eléonore EskanderDepartment of General Pediatrics, Paris-Saclay University, AP-HP, Bicêtre Hospital, Le Kremlin Bicêtre, France.
Paula Vieira MartinsDepartment of Immunology, Assistance Publique- Hôpitaux de Paris (AP-HP), Georges Pompidou European Hospital, Paris, France.
Joan BitanDepartment of Immunology, Assistance Publique- Hôpitaux de Paris (AP-HP), Georges Pompidou European Hospital, Paris, France.ORCID 0000-0002-3923-6972
Julia RoquignyDepartment of Immunology, Assistance Publique- Hôpitaux de Paris (AP-HP), Georges Pompidou European Hospital, Paris, France.
Christèle KyhengDepartment of General Pediatrics, Paris-Saclay University, AP-HP, Bicêtre Hospital, Le Kremlin Bicêtre, France.
Tamazoust GuiddirDepartment of General Pediatrics, Paris-Saclay University, AP-HP, Bicêtre Hospital, Le Kremlin Bicêtre, France.
Véronique Frémeaux-BacchiDepartment of Immunology, Assistance Publique- Hôpitaux de Paris (AP-HP), Georges Pompidou European Hospital, Paris, France.
Cécile GonninDepartment of Immunology, Assistance Publique- Hôpitaux de Paris (AP-HP), Georges Pompidou European Hospital, Paris, France.
Carine ElSissyDepartment of Immunology, Assistance Publique- Hôpitaux de Paris (AP-HP), Georges Pompidou European Hospital, Paris, France.ORCID 0000-0001-7755-6689

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Complement factor B (FB) deficiency is an extremely rare alternative pathway (AP) defect predisposing to invasive infections, with only three families reported to date. We investigated a 14-year-old girl who developed Neisseria meningitidis serogroup Y meningitis complicated by bacteremia and cerebral venous sinus thrombosis. Complement function was assessed by TCA and AP50 hemolytic assay, FB quantification, and functional reconstitution with purified proteins. Genetic analysis of CFB was performed by next-generation sequencing. TCA, C3, and C4 were normal, whereas AP50 was undetectable. Plasma FB concentration was reduced. Reconstitution with purified FB, but not FD, restored AP activity, confirming FB deficiency. Patient plasma failed to support C3b-dependent hemolysis. Sequencing revealed compound heterozygosity for two missense variants in CFB: p.Gly396Arg in the vWFA domain (previously described) and p.Gln713Arg, a novel substitution in the serine protease domain near the catalytic Ser699. Both were classified as likely pathogenic according to ACMG criteria. This report represents the third genetically confirmed case of complete FB deficiency and the first involving a serine protease domain variant. It underscores the diagnostic value of combined quantitative and functional complement assays and highlights infectious risks relevant to patients treated with emerging FB inhibitors.

Indexed as

Complement Factor BMeningitis, MeningococcalNeisseria meningitidis, Serogroup YAdolescentComplement Pathway, AlternativeFemaleHumansMutation, MissenseComplement Factor Balternative pathwaycomplement factor Bfactor B deficiencymeningococcal diseasepediatrics

Identifiers

PMID41663882
PMCPMC12886615

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Registered trials

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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.