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Article: Fatal SARS in X-Linked Lymphoproliferative Disease Type 1: A Case Report

TitleFatal SARS in X-Linked Lymphoproliferative Disease Type 1: A Case Report
Authors
Keywordsagammaglobulinemia
COVID-19
inborn error of immunity
severe acute respiratory syndrome (SARS)
X-linked lymphoproliferative disease type 1 (XLP1)
Issue Date14-Apr-2022
PublisherFrontiers Media
Citation
Frontiers in Pediatrics, 2022, v. 10 How to Cite?
Abstract

X-linked lymphoproliferative disease (XLP1) is an inborn error of immunity (IEI) with severe immune dysregulation caused by a mutation in the SH2D1A gene resulting in the absence or dysfunction of signaling lymphocytic activation molecule (SLAM)-associated protein (SAP). The severe acute respiratory syndrome (SARS) caused by SARS-coronavirus (CoV), a highly pathogenic CoV, has been shown to only cause mild diseases in Asian children. We report on a 5-year-old Nepalese boy with agammaglobulinemia and probable SARS who died of diffuse alveolar damage 22 days after admission amid the SARS outbreak. The index patient and his younger brother were genetically confirmed to have XLP1. In the current coronavirus disease 2019 (COVID-19) pandemic, most children also had mild disease only. Children with severe COVID-19 would warrant investigations for underlying IEI, particularly along the pathways leading to immune dysregulation.


Persistent Identifierhttp://hdl.handle.net/10722/331414
ISSN
2023 Impact Factor: 2.1
2023 SCImago Journal Rankings: 0.715
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorChung, Ming Hin-
dc.contributor.authorChua, Gilbert-
dc.contributor.authorLeung, Daniel-
dc.contributor.authorChan, Koon Wing-
dc.contributor.authorNicholls, John-
dc.contributor.authorLau, Yu Lung-
dc.date.accessioned2023-09-21T06:55:29Z-
dc.date.available2023-09-21T06:55:29Z-
dc.date.issued2022-04-14-
dc.identifier.citationFrontiers in Pediatrics, 2022, v. 10-
dc.identifier.issn2296-2360-
dc.identifier.urihttp://hdl.handle.net/10722/331414-
dc.description.abstract<p>X-linked lymphoproliferative disease (XLP1) is an inborn error of immunity (IEI) with severe immune dysregulation caused by a mutation in the <em>SH2D1A</em> gene resulting in the absence or dysfunction of signaling lymphocytic activation molecule (SLAM)-associated protein (SAP). The severe acute respiratory syndrome (SARS) caused by SARS-coronavirus (CoV), a highly pathogenic CoV, has been shown to only cause mild diseases in Asian children. We report on a 5-year-old Nepalese boy with agammaglobulinemia and probable SARS who died of diffuse alveolar damage 22 days after admission amid the SARS outbreak. The index patient and his younger brother were genetically confirmed to have XLP1. In the current coronavirus disease 2019 (COVID-19) pandemic, most children also had mild disease only. Children with severe COVID-19 would warrant investigations for underlying IEI, particularly along the pathways leading to immune dysregulation.</p>-
dc.languageeng-
dc.publisherFrontiers Media-
dc.relation.ispartofFrontiers in Pediatrics-
dc.rightsThis work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.-
dc.subjectagammaglobulinemia-
dc.subjectCOVID-19-
dc.subjectinborn error of immunity-
dc.subjectsevere acute respiratory syndrome (SARS)-
dc.subjectX-linked lymphoproliferative disease type 1 (XLP1)-
dc.titleFatal SARS in X-Linked Lymphoproliferative Disease Type 1: A Case Report-
dc.typeArticle-
dc.identifier.doi10.3389/fped.2022.794110-
dc.identifier.scopuseid_2-s2.0-85128910660-
dc.identifier.volume10-
dc.identifier.eissn2296-2360-
dc.identifier.isiWOS:000795469500001-
dc.identifier.issnl2296-2360-

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