Abstract
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 language | English (US) |
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Pages (from-to) | 299-300 |
Number of pages | 2 |
Journal | Pediatric Infectious Disease Journal |
Volume | 34 |
Issue number | 3 |
DOIs | |
State | Published - Mar 4 2015 |
Keywords
- 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