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A Child with X-Linked Agammaglobulinemia and Enthesitis-Related Arthritis

X-linked agammaglobulinemia (XLA) is a primary immune deficiency characterized by recurrent bacterial infections and profoundly depressed serum immunoglobulin levels and circulating mature B cells. We describe a 12-year-old boy with XLA and enthesitis-related arthritis (ERA). To date, there has been...

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Autores principales: Sukumaran, Sukesh, Marzan, Katherine, Shaham, Bracha, Church, Joseph A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3134104/
https://www.ncbi.nlm.nih.gov/pubmed/21760802
http://dx.doi.org/10.1155/2011/175973
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author Sukumaran, Sukesh
Marzan, Katherine
Shaham, Bracha
Church, Joseph A.
author_facet Sukumaran, Sukesh
Marzan, Katherine
Shaham, Bracha
Church, Joseph A.
author_sort Sukumaran, Sukesh
collection PubMed
description X-linked agammaglobulinemia (XLA) is a primary immune deficiency characterized by recurrent bacterial infections and profoundly depressed serum immunoglobulin levels and circulating mature B cells. We describe a 12-year-old boy with XLA and enthesitis-related arthritis (ERA). To date, there has been a paucity of reports of noninfectious inflammatory arthritis in children with XLA. This case illustrates that functional B cells and/or immunoglobulin are not required for ERA pathogenesis. In addition, this case suggests a possible link between immune deficiency, immune dysregulation, and rheumatic illness.
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spelling pubmed-31341042011-07-14 A Child with X-Linked Agammaglobulinemia and Enthesitis-Related Arthritis Sukumaran, Sukesh Marzan, Katherine Shaham, Bracha Church, Joseph A. Int J Rheumatol Case Report X-linked agammaglobulinemia (XLA) is a primary immune deficiency characterized by recurrent bacterial infections and profoundly depressed serum immunoglobulin levels and circulating mature B cells. We describe a 12-year-old boy with XLA and enthesitis-related arthritis (ERA). To date, there has been a paucity of reports of noninfectious inflammatory arthritis in children with XLA. This case illustrates that functional B cells and/or immunoglobulin are not required for ERA pathogenesis. In addition, this case suggests a possible link between immune deficiency, immune dysregulation, and rheumatic illness. Hindawi Publishing Corporation 2011 2011-06-01 /pmc/articles/PMC3134104/ /pubmed/21760802 http://dx.doi.org/10.1155/2011/175973 Text en Copyright © 2011 Sukesh Sukumaran et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Sukumaran, Sukesh
Marzan, Katherine
Shaham, Bracha
Church, Joseph A.
A Child with X-Linked Agammaglobulinemia and Enthesitis-Related Arthritis
title A Child with X-Linked Agammaglobulinemia and Enthesitis-Related Arthritis
title_full A Child with X-Linked Agammaglobulinemia and Enthesitis-Related Arthritis
title_fullStr A Child with X-Linked Agammaglobulinemia and Enthesitis-Related Arthritis
title_full_unstemmed A Child with X-Linked Agammaglobulinemia and Enthesitis-Related Arthritis
title_short A Child with X-Linked Agammaglobulinemia and Enthesitis-Related Arthritis
title_sort child with x-linked agammaglobulinemia and enthesitis-related arthritis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3134104/
https://www.ncbi.nlm.nih.gov/pubmed/21760802
http://dx.doi.org/10.1155/2011/175973
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