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A case of granulomatous lung disease in a patient with Good's syndrome
Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome...
Autores principales: | , , , , , , |
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Formato: | Texto |
Lenguaje: | English |
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The Korean Association of Internal Medicine
2008
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2687676/ https://www.ncbi.nlm.nih.gov/pubmed/19119261 http://dx.doi.org/10.3904/kjim.2008.23.4.219 |
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author | Lee, Seung Heon Lee, Sang-Min Yang, Seok-Chul Yoo, Chul-Gyu Kim, Young Whan Shim, Young-Soo Han, Sung Koo |
author_facet | Lee, Seung Heon Lee, Sang-Min Yang, Seok-Chul Yoo, Chul-Gyu Kim, Young Whan Shim, Young-Soo Han, Sung Koo |
author_sort | Lee, Seung Heon |
collection | PubMed |
description | Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome are very susceptible to infections; common respiratory and opportunistic infections can be life-threatening. There are no reports of granulomatous lung disease in patients with Good's syndrome, although it has been observed in patients with common variable immunodeficiency, of which Good's syndrome is a subset. We describe a 53-year-old male thymoma patient who presented with respiratory symptoms caused by granulomatous lung disease and an opportunistic infection. He died of uncontrolled fungal infection despite repeated intravenous immunoglobulin and supportive care. Clinicians should look for evidence of immunologic dysfunction in thymoma patients presenting with severe recurrent infections, especially opportunistic infections. |
format | Text |
id | pubmed-2687676 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2008 |
publisher | The Korean Association of Internal Medicine |
record_format | MEDLINE/PubMed |
spelling | pubmed-26876762009-06-15 A case of granulomatous lung disease in a patient with Good's syndrome Lee, Seung Heon Lee, Sang-Min Yang, Seok-Chul Yoo, Chul-Gyu Kim, Young Whan Shim, Young-Soo Han, Sung Koo Korean J Intern Med Case Report Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome are very susceptible to infections; common respiratory and opportunistic infections can be life-threatening. There are no reports of granulomatous lung disease in patients with Good's syndrome, although it has been observed in patients with common variable immunodeficiency, of which Good's syndrome is a subset. We describe a 53-year-old male thymoma patient who presented with respiratory symptoms caused by granulomatous lung disease and an opportunistic infection. He died of uncontrolled fungal infection despite repeated intravenous immunoglobulin and supportive care. Clinicians should look for evidence of immunologic dysfunction in thymoma patients presenting with severe recurrent infections, especially opportunistic infections. The Korean Association of Internal Medicine 2008-12 2008-12-30 /pmc/articles/PMC2687676/ /pubmed/19119261 http://dx.doi.org/10.3904/kjim.2008.23.4.219 Text en Copyright © 2008 The Korean Association of Internal Medicine https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/by-nc/4.0 (https://creativecommons.org/licenses/by-nc/4.0/) ) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Lee, Seung Heon Lee, Sang-Min Yang, Seok-Chul Yoo, Chul-Gyu Kim, Young Whan Shim, Young-Soo Han, Sung Koo A case of granulomatous lung disease in a patient with Good's syndrome |
title | A case of granulomatous lung disease in a patient with Good's syndrome |
title_full | A case of granulomatous lung disease in a patient with Good's syndrome |
title_fullStr | A case of granulomatous lung disease in a patient with Good's syndrome |
title_full_unstemmed | A case of granulomatous lung disease in a patient with Good's syndrome |
title_short | A case of granulomatous lung disease in a patient with Good's syndrome |
title_sort | case of granulomatous lung disease in a patient with good's syndrome |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2687676/ https://www.ncbi.nlm.nih.gov/pubmed/19119261 http://dx.doi.org/10.3904/kjim.2008.23.4.219 |
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