Streptococcal pneumonia meningitis as an initial presentation of X‐linked agammaglobulinemia: A case report and discussion

Abstract X‐linked agammaglobulinemia (XLA) is a primary immunodeficiency caused by mutations in the gene for Bruton's tyrosine kinase (Btk), with affected males most commonly presenting with recurrent bacterial infections during the first few years of life. Here we present a 17‐month‐old male w...

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Main Authors: Daniel Kadden, Grace Fowler, Elissa Engel, Casey Logan, Kalyani Marathe, Craig Gosdin
Format: Article
Language:English
Published: Elsevier 2021-10-01
Series:Journal of the American College of Emergency Physicians Open
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Online Access:https://doi.org/10.1002/emp2.12553
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author Daniel Kadden
Grace Fowler
Elissa Engel
Casey Logan
Kalyani Marathe
Craig Gosdin
author_facet Daniel Kadden
Grace Fowler
Elissa Engel
Casey Logan
Kalyani Marathe
Craig Gosdin
author_sort Daniel Kadden
collection DOAJ
description Abstract X‐linked agammaglobulinemia (XLA) is a primary immunodeficiency caused by mutations in the gene for Bruton's tyrosine kinase (Btk), with affected males most commonly presenting with recurrent bacterial infections during the first few years of life. Here we present a 17‐month‐old male with a chief complaint of worsening rash and fever, whose history of streptococcal pneumonia meningitis at 5 months of age prompted suspicion for an underlying immunodeficiency and subsequent diagnosis of XLA. Bacterial meningitis is a rare initial presentation of XLA, and therefore physicians may easily overlook any underlying immunodeficiency. Prompt workup for immunodeficiency should be initiated in any vaccinated patient with a history of pneumococcal meningitis outside of the newborn period. Further discussion surrounding the various presentations of XLA, their related clinical manifestations and laboratory findings, and the importance of thorough chart review may encourage earlier diagnosis and initiation of treatment of this disease.
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publishDate 2021-10-01
publisher Elsevier
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series Journal of the American College of Emergency Physicians Open
spelling doaj-art-3e98c22677a64db8a720bfb2d3fe2d4f2025-08-20T02:08:23ZengElsevierJournal of the American College of Emergency Physicians Open2688-11522021-10-0125n/an/a10.1002/emp2.12553Streptococcal pneumonia meningitis as an initial presentation of X‐linked agammaglobulinemia: A case report and discussionDaniel Kadden0Grace Fowler1Elissa Engel2Casey Logan3Kalyani Marathe4Craig Gosdin5UC College of Medicine, Department of Pediatrics Cincinnati Children's Hospital Medical Center Cincinnati Ohio USAUC College of Medicine, Department of Pediatrics Cincinnati Children's Hospital Medical Center Cincinnati Ohio USAUC College of Medicine, Department of Pediatrics Cincinnati Children's Hospital Medical Center Cincinnati Ohio USAUC College of Medicine, Department of Pediatrics Cincinnati Children's Hospital Medical Center Cincinnati Ohio USAUC College of Medicine, Department of Pediatrics Cincinnati Children's Hospital Medical Center Cincinnati Ohio USAUC College of Medicine, Department of Pediatrics Cincinnati Children's Hospital Medical Center Cincinnati Ohio USAAbstract X‐linked agammaglobulinemia (XLA) is a primary immunodeficiency caused by mutations in the gene for Bruton's tyrosine kinase (Btk), with affected males most commonly presenting with recurrent bacterial infections during the first few years of life. Here we present a 17‐month‐old male with a chief complaint of worsening rash and fever, whose history of streptococcal pneumonia meningitis at 5 months of age prompted suspicion for an underlying immunodeficiency and subsequent diagnosis of XLA. Bacterial meningitis is a rare initial presentation of XLA, and therefore physicians may easily overlook any underlying immunodeficiency. Prompt workup for immunodeficiency should be initiated in any vaccinated patient with a history of pneumococcal meningitis outside of the newborn period. Further discussion surrounding the various presentations of XLA, their related clinical manifestations and laboratory findings, and the importance of thorough chart review may encourage earlier diagnosis and initiation of treatment of this disease.https://doi.org/10.1002/emp2.12553absolute neutrophil countbacterial meningitisBruton's tyrosine kinaseecthyma gangrenosumStaphylococcus aureusX‐linked agammaglobulinemia
spellingShingle Daniel Kadden
Grace Fowler
Elissa Engel
Casey Logan
Kalyani Marathe
Craig Gosdin
Streptococcal pneumonia meningitis as an initial presentation of X‐linked agammaglobulinemia: A case report and discussion
Journal of the American College of Emergency Physicians Open
absolute neutrophil count
bacterial meningitis
Bruton's tyrosine kinase
ecthyma gangrenosum
Staphylococcus aureus
X‐linked agammaglobulinemia
title Streptococcal pneumonia meningitis as an initial presentation of X‐linked agammaglobulinemia: A case report and discussion
title_full Streptococcal pneumonia meningitis as an initial presentation of X‐linked agammaglobulinemia: A case report and discussion
title_fullStr Streptococcal pneumonia meningitis as an initial presentation of X‐linked agammaglobulinemia: A case report and discussion
title_full_unstemmed Streptococcal pneumonia meningitis as an initial presentation of X‐linked agammaglobulinemia: A case report and discussion
title_short Streptococcal pneumonia meningitis as an initial presentation of X‐linked agammaglobulinemia: A case report and discussion
title_sort streptococcal pneumonia meningitis as an initial presentation of x linked agammaglobulinemia a case report and discussion
topic absolute neutrophil count
bacterial meningitis
Bruton's tyrosine kinase
ecthyma gangrenosum
Staphylococcus aureus
X‐linked agammaglobulinemia
url https://doi.org/10.1002/emp2.12553
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