dc.contributor.author
Fasshauer, Maria
dc.contributor.author
Dinges, Sarah
dc.contributor.author
Staudacher, Olga
dc.contributor.author
Völler, Mirjam
dc.contributor.author
Stittrich, Anna
dc.contributor.author
von Bernuth, Horst
dc.contributor.author
Wahn, Volker
dc.contributor.author
Krüger, Renate
dc.date.accessioned
2025-07-30T09:33:05Z
dc.date.available
2025-07-30T09:33:05Z
dc.identifier.uri
https://refubium.fu-berlin.de/handle/fub188/48499
dc.identifier.uri
http://dx.doi.org/10.17169/refubium-48221
dc.description.abstract
In patients with severe and recurrent infections, minimal diagnostic workup to test for Inborn Errors of Immunity (IEI) includes a full blood count, IgG, IgA and IgM. Vaccine antibodies against tetanus toxoid are also frequently measured, whereas testing for anti-polysaccharide IgG antibodies and IgG subclasses is not routinely performed by primary care physicians. This basic approach may cause a significant delay in diagnosing monogenic IEI that can present with an impaired IgG response to polysaccharide antigens with or without IgG subclass deficiency at an early stage. Our article reviews genetically defined IEI, that may initially present with an impaired IgG response to polysaccharide antigens, but normal or only slightly decreased IgG levels and normal responses to protein or conjugate vaccine antigens. We summarize clinical, genetic, and immunological findings characteristic for these IEI. This review may help clinicians to identify patients that require extended immunologic and genetic evaluations despite unremarkable basic immunologic findings. We recommend the inclusion of anti-polysaccharide IgG antibodies as part of the initial routine work-up for possible IEI.
en
dc.rights.uri
https://creativecommons.org/licenses/by/4.0/
dc.subject
inborn errors of immunity
en
dc.subject
primary immunodeficiency
en
dc.subject
polysaccharide
en
dc.subject
pneumococcal infections
en
dc.subject.ddc
600 Technik, Medizin, angewandte Wissenschaften::610 Medizin und Gesundheit::610 Medizin und Gesundheit
dc.title
Monogenic Inborn Errors of Immunity with impaired IgG response to polysaccharide antigens but normal IgG levels and normal IgG response to protein antigens
dc.type
Wissenschaftlicher Artikel
dcterms.bibliographicCitation.articlenumber
1386959
dcterms.bibliographicCitation.doi
10.3389/fped.2024.1386959
dcterms.bibliographicCitation.journaltitle
Frontiers in Pediatrics
dcterms.bibliographicCitation.originalpublishername
Frontiers Media SA
dcterms.bibliographicCitation.volume
12
refubium.affiliation
Charité - Universitätsmedizin Berlin
refubium.resourceType.isindependentpub
no
dcterms.accessRights.openaire
open access
dcterms.bibliographicCitation.pmid
38933494
dcterms.isPartOf.eissn
2296-2360