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IL-1

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By Faye Kehler Family Physician and GP Anesthetist since 1987 interested in all aspects of Medicine
https://www.readbyqxmd.com/read/25232776/deficiency-of-interleukin-1-receptor-associated-kinase-4-presenting-as-fatal-pseudomonas-aeruginosa-bacteremia-in-two-siblings
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Theodouli Stergiopoulou, Thomas J Walsh, Marie-Christine Seghaye, Mihai G Netea, Jean-Laurent Casanova, Michel Moutschen, Capucine Picard
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.
March 2015: Pediatric Infectious Disease Journal
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