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
- Institución
- Consejo Nacional de Investigaciones Científicas y Técnicas
- OAI Identificador
- oai:ri.conicet.gov.ar:11336/190030
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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 Técnicas2025-09-03T09:55:44Zoai: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:34982025-09-03 09:55:45.036CONICET 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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1842269364226621440 |
score |
13.13397 |