Interleukin receptor-associated kinase (IRAK-4) deficiency associated with bacterial infections and failure to sustain antibody responses

J Pediatr. 2004 Apr;144(4):524-6. doi: 10.1016/j.jpeds.2003.11.025.

Abstract

We previously described a girl with recurrent episodes of pneumococcal pneumonia with septicemia and other infections,(1) found to have interleukin-1 receptor-associated kinase 4 deficiency (IRAK-4) deficiency.(2) In this report, we show that our patient is unable to sustain antibody responses either to polysaccharide or protein antigens or to a neoantigen-bacteriophage.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Bacterial / analysis*
  • Bacterial Infections / immunology*
  • Blotting, Northern
  • Blotting, Western
  • Child
  • Consanguinity
  • Female
  • Humans
  • Immunologic Deficiency Syndromes / genetics
  • Interleukin-1 Receptor-Associated Kinases
  • Interleukin-6 / biosynthesis
  • Membrane Glycoproteins / immunology
  • Phosphotransferases (Alcohol Group Acceptor) / deficiency*
  • Phosphotransferases (Alcohol Group Acceptor) / genetics
  • Receptors, Cell Surface / immunology
  • Signal Transduction
  • Toll-Like Receptors

Substances

  • Antibodies, Bacterial
  • Interleukin-6
  • Membrane Glycoproteins
  • Receptors, Cell Surface
  • Toll-Like Receptors
  • Phosphotransferases (Alcohol Group Acceptor)
  • Interleukin-1 Receptor-Associated Kinases