Deficiency of interleukin-1 receptor-associated kinase 4 presenting as fatal Pseudomonas aeruginosa bacteremia in two siblings

Theodouli Stergiopoulou, Thomas J. Walsh, Marie Christine Seghaye, Mihai G. Netea, Jean Laurent Casanova, Michel Moutschen, Capucine Picard

Research output: Contribution to journalArticle


Interleukin-1 receptor-associated kinase 4 (IRAK-4) deficiency is a primary immunodeficiency of innate immunity. This is the case of a previous healthy toddler and his sibling, who both died of fulminant sepsis due to Pseudomonas aeruginosa. Subsequent genetic analysis demonstrated IRAK-4 deficiency with compound heterozygous splice mutations. Fulminant fatal P. aeruginosa sepsis may be the first manifestation of IRAK-4 deficiency.

Original languageEnglish (US)
Pages (from-to)299-300
Number of pages2
JournalPediatric Infectious Disease Journal
Issue number3
StatePublished - Mar 4 2015



  • Pseudomonas sepsis
  • delayed umbilical cord separation
  • ecthyma gangrenosum
  • ecthyma gangrenosum
  • interleukin-1 receptor-associated kinase 4
  • primary immunodeficiency

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Microbiology (medical)
  • Infectious Diseases

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