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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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2011
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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. |
format | Online Article Text |
id | pubmed-3134104 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
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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