Evidence map›Paper›PMID 33841879›Full record

ArticleClinical & translational immunology2021

Complement factor D haplodeficiency is associated with a reduced complement activation speed and diminished bacterial killing.

Jeroen D Langereis, Renate G van der Molen, Corrie de Kat Angelino, Stefanie S Henriet, Marien I de Jonge, Irma Joosten, Annet Simons, Janneke Hm Schuurs-Hoeijmakers, Marcel van Deuren, Koen van Aerde and 1 more

Open access · goldAbstract read
In one paragraph

Article in Clinical & translational immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed
0.2field-weighted citation impact, top 55% 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

5 citing papers in PubMed, 7 citations in OpenAlex.

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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

11 authors at 1 institution in 1 country.

Jeroen D LangereisDepartment of Laboratory Medicine Laboratory of Medical Immunology Radboud Institute for Molecular Life Sciences Radboudumc Nijmegen The Netherlands.ORCID https://orcid.org/0000-0001-5317-3202
Renate G van der MolenDepartment of Laboratory Medicine Laboratory of Medical Immunology Radboud Institute for Molecular Life Sciences Radboudumc Nijmegen The Netherlands.
Corrie de Kat AngelinoDepartment of Laboratory Medicine Laboratory of Medical Immunology Radboud Institute for Molecular Life Sciences Radboudumc Nijmegen The Netherlands.
Stefanie S HenrietPediatric Infectious Diseases and Immunology Amalia Children's Hospital Nijmegen The Netherlands.
Marien I de JongeDepartment of Laboratory Medicine Laboratory of Medical Immunology Radboud Institute for Molecular Life Sciences Radboudumc Nijmegen The Netherlands.
Irma JoostenDepartment of Laboratory Medicine Laboratory of Medical Immunology Radboud Institute for Molecular Life Sciences Radboudumc Nijmegen The Netherlands.
Annet SimonsDepartment of Human Genetics Radboudumc Nijmegen The Netherlands.
Janneke Hm Schuurs-HoeijmakersDepartment of Human Genetics Radboudumc Nijmegen The Netherlands.
Marcel van DeurenExpertise Center for Immunodeficiency and Autoinflammation (REIA) Radboudumc Nijmegen The Netherlands.
Koen van AerdePediatric Infectious Diseases and Immunology Amalia Children's Hospital Nijmegen The Netherlands.
Michiel van der FlierPediatric Infectious Diseases and Immunology Amalia Children's Hospital Nijmegen The Netherlands.
Radboud University Nijmegen · NL

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectivesComplete deficiency of alternative pathway (AP) complement factors, explained by homozygous mutations, is a well-known risk factor for invasive bacterial infections; however, this is less obvious for heterozygous mutations. We describe two siblings with a heterozygous NM_001928.3(CFD):c.125C>A p.(Ser42*) mutation in the complement factor D (fD) gene having a history of recurrent bacterial infections. We determined the effect of heterozygous fD deficiency on AP complement activity.

methodsWe determined the effect of fD levels on complement activation as measured by AP activity, complement C3 binding to the bacterial surface of

resultsReconstitution of fD-deficient serum with fD increased AP activity in a dose- and time-dependent way. Reconstitution of patient serum with fD to normal levels increased complement C3 binding to Sp, Nm and NTHi, as well as complement-mediated killing of Nm and NTHi. Vaccination increased complement C3 binding and resulted in complete killing of Nm without fD reconstitution.

conclusionWe conclude that low fD serum levels (< 0.5 μg mL

Indexed as

complementfactor Dhaplodeficiencyimmunodeficiencyinfectionvaccination

Identifiers

PMID33841879
PMCPMC8019133
OpenAlexW3151569144

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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.