Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia

Immunodeficiency; Immunoglobulins; Mortality

Detalles Bibliográficos
Autores: Zielen, Stefan, Duecker, Ruth Pia, Woelke, Sandra, Donath, Helena, Bakhtiar, Sharhzad, Buecker, Aileen, Soler Palacín, Pere
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2021
País:España
Institución:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
Repositorio:Recercat. Dipósit de la Recerca de Catalunya
OAI Identifier:oai:recercat.cat:11351/7788
Acceso en línea:https://hdl.handle.net/11351/7788
http://hdl.handle.net/11351/7788
Access Level:acceso abierto
Palabra clave:Atàxia de Friedreich - Prognosi
Immunoglobulina A
DISEASES::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Cerebellar Diseases::Cerebellar Ataxia::Spinocerebellar Ataxias::Ataxia Telangiectasia
Other subheadings::Other subheadings::Other subheadings::/immunology
ENFERMEDADES::enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::enfermedades cerebelosas::ataxia cerebelosa::ataxias espinocerebelosas::ataxia telangiectasia
Otros calificadores::Otros calificadores::Otros calificadores::/inmunología
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oai_identifier_str oai:recercat.cat:11351/7788
network_acronym_str ES
network_name_str España
repository_id_str
dc.title.none.fl_str_mv Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia
title Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia
spellingShingle Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia
Zielen, Stefan
Atàxia de Friedreich - Prognosi
Immunoglobulina A
DISEASES::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Cerebellar Diseases::Cerebellar Ataxia::Spinocerebellar Ataxias::Ataxia Telangiectasia
Other subheadings::Other subheadings::Other subheadings::/immunology
ENFERMEDADES::enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::enfermedades cerebelosas::ataxia cerebelosa::ataxias espinocerebelosas::ataxia telangiectasia
Otros calificadores::Otros calificadores::Otros calificadores::/inmunología
title_short Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia
title_full Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia
title_fullStr Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia
title_full_unstemmed Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia
title_sort Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia
dc.creator.none.fl_str_mv Zielen, Stefan
Duecker, Ruth Pia
Woelke, Sandra
Donath, Helena
Bakhtiar, Sharhzad
Buecker, Aileen
Soler Palacín, Pere
author Zielen, Stefan
author_facet Zielen, Stefan
Duecker, Ruth Pia
Woelke, Sandra
Donath, Helena
Bakhtiar, Sharhzad
Buecker, Aileen
Soler Palacín, Pere
author_role author
author2 Duecker, Ruth Pia
Woelke, Sandra
Donath, Helena
Bakhtiar, Sharhzad
Buecker, Aileen
Soler Palacín, Pere
author2_role author
author
author
author
author
author
dc.contributor.none.fl_str_mv Institut Català de la Salut
[Zielen S, Duecker RP, Woelke S, Donath H, Buecker A] Division of Allergology, Pulmonology and Cystic Fibrosis, Department for Children and Adolescents, Goethe University, Frankfurt, Germany. [Bakhtiar S] Division for Stem Cell Transplantation, Immunology and Intensive Care Unit, Department for Children and Adolescents, Goethe University, Frankfurt, Germany. [Soler-Palacín P] Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Unitat de Patologia Infecciosa i Immunodeficiències de Pediatria, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain
Vall d'Hebron Barcelona Hospital Campus
dc.subject.none.fl_str_mv Atàxia de Friedreich - Prognosi
Immunoglobulina A
DISEASES::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Cerebellar Diseases::Cerebellar Ataxia::Spinocerebellar Ataxias::Ataxia Telangiectasia
Other subheadings::Other subheadings::Other subheadings::/immunology
ENFERMEDADES::enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::enfermedades cerebelosas::ataxia cerebelosa::ataxias espinocerebelosas::ataxia telangiectasia
Otros calificadores::Otros calificadores::Otros calificadores::/inmunología
topic Atàxia de Friedreich - Prognosi
Immunoglobulina A
DISEASES::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Cerebellar Diseases::Cerebellar Ataxia::Spinocerebellar Ataxias::Ataxia Telangiectasia
Other subheadings::Other subheadings::Other subheadings::/immunology
ENFERMEDADES::enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::enfermedades cerebelosas::ataxia cerebelosa::ataxias espinocerebelosas::ataxia telangiectasia
Otros calificadores::Otros calificadores::Otros calificadores::/inmunología
description Immunodeficiency; Immunoglobulins; Mortality
publishDate 2021
dc.date.none.fl_str_mv 2021
2022
2022
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://hdl.handle.net/11351/7788
http://hdl.handle.net/11351/7788
url https://hdl.handle.net/11351/7788
http://hdl.handle.net/11351/7788
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Journal of Clinical Immunology;41
https://doi.org/10.1007/s10875-021-01090-8
dc.rights.none.fl_str_mv Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Springer
publisher.none.fl_str_mv Springer
dc.source.none.fl_str_mv Scientia
reponame:Recercat. Dipósit de la Recerca de Catalunya
instname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
instname_str Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
reponame_str Recercat. Dipósit de la Recerca de Catalunya
collection Recercat. Dipósit de la Recerca de Catalunya
repository.name.fl_str_mv
repository.mail.fl_str_mv
_version_ 1869403087185117184
spelling Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-TelangiectasiaZielen, StefanDuecker, Ruth PiaWoelke, SandraDonath, HelenaBakhtiar, SharhzadBuecker, AileenSoler Palacín, PereAtàxia de Friedreich - PrognosiImmunoglobulina ADISEASES::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Cerebellar Diseases::Cerebellar Ataxia::Spinocerebellar Ataxias::Ataxia TelangiectasiaOther subheadings::Other subheadings::Other subheadings::/immunologyENFERMEDADES::enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::enfermedades cerebelosas::ataxia cerebelosa::ataxias espinocerebelosas::ataxia telangiectasiaOtros calificadores::Otros calificadores::Otros calificadores::/inmunologíaImmunodeficiency; Immunoglobulins; MortalityInmunodeficiencia; Inmunoglobulinas; MortalidadImmunodeficiència; Immunoglobulines; MortalitatPatients with ataxia-telangiectasia (A-T) suffer from progressive cerebellar ataxia, immunodeficiency, respiratory failure, and cancer susceptibility. From a clinical point of view, A-T patients with IgA deficiency show more symptoms and may have a poorer prognosis. In this study, we analyzed mortality and immunity data of 659 A-T patients with regard to IgA deficiency collected from the European Society for Immunodeficiencies (ESID) registry and from 66 patients with classical A-T who attended at the Frankfurt Goethe-University between 2012 and 2018. We studied peripheral B- and T-cell subsets and T-cell repertoire of the Frankfurt cohort and survival rates of all A-T patients in the ESID registry. Patients with A-T have significant alterations in their lymphocyte phenotypes. All subsets (CD3, CD4, CD8, CD19, CD4/CD45RA, and CD8/CD45RA) were significantly diminished compared to standard values. Patients with IgA deficiency (n = 35) had significantly lower lymphocyte counts compared to A-T patients without IgA deficiency (n = 31) due to a further decrease of naïve CD4 T-cells, central memory CD4 cells, and regulatory T-cells. Although both patient groups showed affected TCR-ß repertoires compared to controls, no differences could be detected between patients with and without IgA deficiency. Overall survival of patients with IgA deficiency was significantly diminished. For the first time, our data show that patients with IgA deficiency have significantly lower lymphocyte counts and subsets, which are accompanied with reduced survival, compared to A-T patients without IgA deficiency. IgA, a simple surrogate marker, is indicating the poorest prognosis for classical A-T patients. Both non-interventional clinical trials were registered at clinicaltrials.gov 2012 (Susceptibility to infections in ataxia-telangiectasia; NCT02345135) and 2017 (Susceptibility to Infections, tumor risk and liver disease in patients with ataxia-telangiectasia; NCT03357978)Open Access funding enabled and organized by Projekt DEAL. The ESID Registry was supported by the German Federal Ministry of Education and Research (BMBF 01GM0896, 01GM1111B, 01GM1517C, 01EO1303 and 01ZZ1801B) EU grant no. HEALTH-F2-2008–201549 (EURO-PADnet), the pharmaceutical companies Novartis, GlaxoSmithKline, LFB, and UCB UK, the Plasma Protein Therapeutics Association (PPTA), the Care-for-Rare Foundation, PROimmune e.V, LFB, and the Deutsche Forschungsgemeinschaft (DFG, German Research Foundation) under Germany’s Excellence Strategy—EXC 2155 RESIST—Project ID 39087428. EGD is supported by the UK National Institute of Health Research and the Great Ormond Street Hospital Biomedical Research Centre.SpringerInstitut Català de la Salut[Zielen S, Duecker RP, Woelke S, Donath H, Buecker A] Division of Allergology, Pulmonology and Cystic Fibrosis, Department for Children and Adolescents, Goethe University, Frankfurt, Germany. [Bakhtiar S] Division for Stem Cell Transplantation, Immunology and Intensive Care Unit, Department for Children and Adolescents, Goethe University, Frankfurt, Germany. [Soler-Palacín P] Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Unitat de Patologia Infecciosa i Immunodeficiències de Pediatria, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, SpainVall d'Hebron Barcelona Hospital Campus202220222021info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://hdl.handle.net/11351/7788http://hdl.handle.net/11351/7788Scientiareponame:Recercat. Dipósit de la Recerca de Catalunyainstname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)InglésJournal of Clinical Immunology;41https://doi.org/10.1007/s10875-021-01090-8Attribution 4.0 Internationalhttp://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:recercat.cat:11351/77882026-05-29T05:05:01Z
score 15,811543

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