Abstract
We describe the case of an infant with recurrent episodes of staphylococcal skin abscess and subsequent lethal pneumococcal meningitis/septicemia due to interleukin-1 receptor-associated kinase 4 (IRAK-4) deficiency. In this case, systemic signs of inflammatory response were poor and delayed. Among all other reported cases of IRAK-4 deficiency, none involved severe viral or fungal disease, and the range of infecting bacteria was narrow.
Keywords:
IRAK-4 deficiency; delayed systemic signs of inflammatory response; pneumococcal meningitis/septicemia; staphylococcal skin abscess.
© 2015 Japan Pediatric Society.
MeSH terms
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Abscess / complications*
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Abscess / diagnosis
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Abscess / microbiology
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Fatal Outcome
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Female
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Follow-Up Studies
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Humans
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Immunologic Deficiency Syndromes / complications*
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Immunologic Deficiency Syndromes / diagnosis
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Infant
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Interleukin-1 Receptor-Associated Kinases
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Meningitis, Pneumococcal / complications*
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Meningitis, Pneumococcal / diagnosis
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Meningitis, Pneumococcal / microbiology
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Primary Immunodeficiency Diseases
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Sepsis / diagnosis
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Sepsis / etiology*
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Sepsis / microbiology
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Staphylococcal Infections / complications*
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Staphylococcal Infections / diagnosis
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Staphylococcal Infections / microbiology
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Staphylococcus aureus / isolation & purification*
Substances
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Interleukin-1 Receptor-Associated Kinases