Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody d...

Autores
Bezrodnik, Liliana; Gaillard, Maria Isabel; Carelli, Daniela
Año de publicación
2011
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
We present the clinical and B cell immunophenotypical characterization of 94 patients with Common Variable immunodeficiency (CVID), selective IgA deficiency (SIgAD) and polysaccharide antibody deficiency syndrome (SAD). Study design: We retrospectively investigated clinical findings and B cell compartment in 31 patients with CVID, 35 with SIgAD and 28 with SAD. Regardless of underlying disease, a delay was observed between age at diagnosis and onset of first symptoms. The predominant clinical findings were upper and lower respiratory tract infections. Allergic symptoms were more frequent in SAD and SIgAD patients, hematological and autoimmune manifestations in CVID and celiac disease in SIgAD. B-cell Immunophenotype abnormalities were observed in SAD and CVID patients: both had reduced memory B cells (CD19{+} CD27{+}), and increased transitional B cells (CD24{ + + } CD38{+ +}) was found in SAD. We did not find any statistically significant abnormalities in any of differentiation stages of B cells in SIgAD. Defects of the B cell compartment were associated with bronchiectasis, splenomegaly, autoimmunity and/or malignancy in CVID and SAD patients.We conclude that flow cytometric evaluation of the B cell compartment could be a useful tool for the diagnosis and follow up of these patients.
Fil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Área de Inmunología; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Gaillard, Maria Isabel. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Área de Inmunología; Argentina
Fil: Carelli, Daniela. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Área de Inmunología; Argentina
Materia
COMMON VARIABLE IMMUNODEFICIENCY
IGA DEFICIENCY
POLYSACCHARIDE ANTIBODY DEFICIENCY SYNDROME
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/190030
Acceder
id CONICETDig_0712dd3e7104fe91117f2af69856b940
oai_identifier_str oai:ri.conicet.gov.ar:11336/190030
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndromeBezrodnik, LilianaGaillard, Maria IsabelCarelli, DanielaCOMMON VARIABLE IMMUNODEFICIENCYIGA DEFICIENCYPOLYSACCHARIDE ANTIBODY DEFICIENCY SYNDROMEhttps://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3We present the clinical and B cell immunophenotypical characterization of 94 patients with Common Variable immunodeficiency (CVID), selective IgA deficiency (SIgAD) and polysaccharide antibody deficiency syndrome (SAD). Study design: We retrospectively investigated clinical findings and B cell compartment in 31 patients with CVID, 35 with SIgAD and 28 with SAD. Regardless of underlying disease, a delay was observed between age at diagnosis and onset of first symptoms. The predominant clinical findings were upper and lower respiratory tract infections. Allergic symptoms were more frequent in SAD and SIgAD patients, hematological and autoimmune manifestations in CVID and celiac disease in SIgAD. B-cell Immunophenotype abnormalities were observed in SAD and CVID patients: both had reduced memory B cells (CD19{+} CD27{+}), and increased transitional B cells (CD24{ + + } CD38{+ +}) was found in SAD. We did not find any statistically significant abnormalities in any of differentiation stages of B cells in SIgAD. Defects of the B cell compartment were associated with bronchiectasis, splenomegaly, autoimmunity and/or malignancy in CVID and SAD patients.We conclude that flow cytometric evaluation of the B cell compartment could be a useful tool for the diagnosis and follow up of these patients.Fil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Área de Inmunología; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Gaillard, Maria Isabel. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Área de Inmunología; ArgentinaFil: Carelli, Daniela. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Área de Inmunología; ArgentinaIOS Press2011-06info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/190030Bezrodnik, Liliana; Gaillard, Maria Isabel; Carelli, Daniela; Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndrome; IOS Press; Pediatric Infectious Disease Journal; 6; 3; 6-2011; 159-1661532-0987CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://content.iospress.com/articles/journal-of-pediatric-infectious-diseases/jpi00320info:eu-repo/semantics/altIdentifier/doi/10.3233/JPI-2011-0320info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-29T09:58:54Zoai:ri.conicet.gov.ar:11336/190030instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-29 09:58:54.585CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndrome
title Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndrome
spellingShingle Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndrome
Bezrodnik, Liliana
COMMON VARIABLE IMMUNODEFICIENCY
IGA DEFICIENCY
POLYSACCHARIDE ANTIBODY DEFICIENCY SYNDROME
title_short Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndrome
title_full Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndrome
title_fullStr Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndrome
title_full_unstemmed Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndrome
title_sort Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndrome
dc.creator.none.fl_str_mv Bezrodnik, Liliana
Gaillard, Maria Isabel
Carelli, Daniela
author Bezrodnik, Liliana
author_facet Bezrodnik, Liliana
Gaillard, Maria Isabel
Carelli, Daniela
author_role author
author2 Gaillard, Maria Isabel
Carelli, Daniela
author2_role author
author
dc.subject.none.fl_str_mv COMMON VARIABLE IMMUNODEFICIENCY
IGA DEFICIENCY
POLYSACCHARIDE ANTIBODY DEFICIENCY SYNDROME
topic COMMON VARIABLE IMMUNODEFICIENCY
IGA DEFICIENCY
POLYSACCHARIDE ANTIBODY DEFICIENCY SYNDROME
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.2
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv We present the clinical and B cell immunophenotypical characterization of 94 patients with Common Variable immunodeficiency (CVID), selective IgA deficiency (SIgAD) and polysaccharide antibody deficiency syndrome (SAD). Study design: We retrospectively investigated clinical findings and B cell compartment in 31 patients with CVID, 35 with SIgAD and 28 with SAD. Regardless of underlying disease, a delay was observed between age at diagnosis and onset of first symptoms. The predominant clinical findings were upper and lower respiratory tract infections. Allergic symptoms were more frequent in SAD and SIgAD patients, hematological and autoimmune manifestations in CVID and celiac disease in SIgAD. B-cell Immunophenotype abnormalities were observed in SAD and CVID patients: both had reduced memory B cells (CD19{+} CD27{+}), and increased transitional B cells (CD24{ + + } CD38{+ +}) was found in SAD. We did not find any statistically significant abnormalities in any of differentiation stages of B cells in SIgAD. Defects of the B cell compartment were associated with bronchiectasis, splenomegaly, autoimmunity and/or malignancy in CVID and SAD patients.We conclude that flow cytometric evaluation of the B cell compartment could be a useful tool for the diagnosis and follow up of these patients.
Fil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Área de Inmunología; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Gaillard, Maria Isabel. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Área de Inmunología; Argentina
Fil: Carelli, Daniela. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Área de Inmunología; Argentina
description We present the clinical and B cell immunophenotypical characterization of 94 patients with Common Variable immunodeficiency (CVID), selective IgA deficiency (SIgAD) and polysaccharide antibody deficiency syndrome (SAD). Study design: We retrospectively investigated clinical findings and B cell compartment in 31 patients with CVID, 35 with SIgAD and 28 with SAD. Regardless of underlying disease, a delay was observed between age at diagnosis and onset of first symptoms. The predominant clinical findings were upper and lower respiratory tract infections. Allergic symptoms were more frequent in SAD and SIgAD patients, hematological and autoimmune manifestations in CVID and celiac disease in SIgAD. B-cell Immunophenotype abnormalities were observed in SAD and CVID patients: both had reduced memory B cells (CD19{+} CD27{+}), and increased transitional B cells (CD24{ + + } CD38{+ +}) was found in SAD. We did not find any statistically significant abnormalities in any of differentiation stages of B cells in SIgAD. Defects of the B cell compartment were associated with bronchiectasis, splenomegaly, autoimmunity and/or malignancy in CVID and SAD patients.We conclude that flow cytometric evaluation of the B cell compartment could be a useful tool for the diagnosis and follow up of these patients.
publishDate 2011
dc.date.none.fl_str_mv 2011-06
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/190030
Bezrodnik, Liliana; Gaillard, Maria Isabel; Carelli, Daniela; Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndrome; IOS Press; Pediatric Infectious Disease Journal; 6; 3; 6-2011; 159-166
1532-0987
CONICET Digital
CONICET
url http://hdl.handle.net/11336/190030
identifier_str_mv Bezrodnik, Liliana; Gaillard, Maria Isabel; Carelli, Daniela; Clinical and immunological assessment of 94 patients with primary humoral immunodeficiency: Common variable immunodeficiency, selective IgA deficiency and polysaccharide antibody deficiency syndrome; IOS Press; Pediatric Infectious Disease Journal; 6; 3; 6-2011; 159-166
1532-0987
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/https://content.iospress.com/articles/journal-of-pediatric-infectious-diseases/jpi00320
info:eu-repo/semantics/altIdentifier/doi/10.3233/JPI-2011-0320
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv IOS Press
publisher.none.fl_str_mv IOS Press
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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score 13.070432

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