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Hyper IgE syndrome (Job syndrome) in Syria: a case report

Hyper IgE syndrome (HIES) is a medical condition that can be sporadic or hereditary. It consists of multiple overlapping primary immunodeficiency conditions and is characterized by a classical triad of high immunoglobulin E (IgE) levels, recurrent pneumonia with pneumatocele and recurrent cold skin...

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Autores principales: Awad, Rama, Kakaje, Ameer
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7685025/
https://www.ncbi.nlm.nih.gov/pubmed/33269088
http://dx.doi.org/10.1093/omcr/omaa106
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author Awad, Rama
Kakaje, Ameer
author_facet Awad, Rama
Kakaje, Ameer
author_sort Awad, Rama
collection PubMed
description Hyper IgE syndrome (HIES) is a medical condition that can be sporadic or hereditary. It consists of multiple overlapping primary immunodeficiency conditions and is characterized by a classical triad of high immunoglobulin E (IgE) levels, recurrent pneumonia with pneumatocele and recurrent cold skin abscesses from staphylococcus infections. Eosinophilia is also common in HIES patients. HIES is often underdiagnosed in Syria as it cannot be confirmed without genetic testing, which is unavailable across Syria for HIES. We present the first case from Syria of a suspected child with HIES that has some additional distinct features. Other cases in a regional country carried atypical novel mutations, which may indicate that these mutations may exist in Syria as well. However, our case had findings that were not reported with other HIES cases. Determining these genes in the case presented was not possible, and future studies need to overcome this hurdle.
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spelling pubmed-76850252020-12-01 Hyper IgE syndrome (Job syndrome) in Syria: a case report Awad, Rama Kakaje, Ameer Oxf Med Case Reports Case Report Hyper IgE syndrome (HIES) is a medical condition that can be sporadic or hereditary. It consists of multiple overlapping primary immunodeficiency conditions and is characterized by a classical triad of high immunoglobulin E (IgE) levels, recurrent pneumonia with pneumatocele and recurrent cold skin abscesses from staphylococcus infections. Eosinophilia is also common in HIES patients. HIES is often underdiagnosed in Syria as it cannot be confirmed without genetic testing, which is unavailable across Syria for HIES. We present the first case from Syria of a suspected child with HIES that has some additional distinct features. Other cases in a regional country carried atypical novel mutations, which may indicate that these mutations may exist in Syria as well. However, our case had findings that were not reported with other HIES cases. Determining these genes in the case presented was not possible, and future studies need to overcome this hurdle. Oxford University Press 2020-11-24 /pmc/articles/PMC7685025/ /pubmed/33269088 http://dx.doi.org/10.1093/omcr/omaa106 Text en © The Author(s) 2020. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com http://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 (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Report
Awad, Rama
Kakaje, Ameer
Hyper IgE syndrome (Job syndrome) in Syria: a case report
title Hyper IgE syndrome (Job syndrome) in Syria: a case report
title_full Hyper IgE syndrome (Job syndrome) in Syria: a case report
title_fullStr Hyper IgE syndrome (Job syndrome) in Syria: a case report
title_full_unstemmed Hyper IgE syndrome (Job syndrome) in Syria: a case report
title_short Hyper IgE syndrome (Job syndrome) in Syria: a case report
title_sort hyper ige syndrome (job syndrome) in syria: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7685025/
https://www.ncbi.nlm.nih.gov/pubmed/33269088
http://dx.doi.org/10.1093/omcr/omaa106
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