Orphanet: Immunodeficiency due to interleukin 1 receptor associated kinase 4 deficiency

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Immunodeficiency due to interleukin-1 receptor-associated kinase-4 deficiency

Disease definition

Interleukin-1 receptor-associated kinase-4 (IRAK-4) deficiency is an immunodeficiency associated with increased susceptibility to invasive infections caused by pyogenic bacteria.


Classification level: Disorder
  • Synonym(s):
    • IRAK4 deficiency
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Childhood
  • ICD-10: D84.8
  • OMIM: 607676
  • UMLS: C1843256
  • MeSH: -
  • GARD: 10311
  • MedDRA: -
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