title: Interleukin receptor-associated kinase (IRAK-4) deficiency associated with bacterial infections and failure to sustain antibody responses.
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.
authors.affiliation: Division of Allergy and Immunology, Department of Pediatrics, University of South Florida All Children's Hospital, Box 7070, 801 6th Street South, St Petersburg, FL 33701, USA. dayn@allkids.org
authors.forename: Noorbibi
authors.initials: N
authors.lastname: Day
authors.forename: Nutthapong
authors.initials: N
authors.lastname: Tangsinmankong
authors.forename: Hans
authors.initials: H
authors.lastname: Ochs
authors.forename: Rajivi
authors.initials: R
authors.lastname: Rucker
authors.forename: Capucine
authors.initials: C
authors.lastname: Picard
authors.forename: Jean-Laurent
authors.initials: JL
authors.lastname: Casanova
authors.forename: Soichi
authors.initials: S
authors.lastname: Haraguchi
authors.forename: Robert
authors.initials: R
authors.lastname: Good
mesh_terms: D000907:Antibodies, Bacterial / Q000032:analysis*; D001424:Bacterial Infections / Q000276:immunology*; D015152:Blotting, Northern; D015153:Blotting, Western; D002648:Child; D003241:Consanguinity; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000235:genetics; D053592:Interleukin-1 Receptor-Associated Kinases; D015850:Interleukin-6 / Q000096:biosynthesis; D008562:Membrane Glycoproteins / Q000276:immunology; D017853:Phosphotransferases (Alcohol Group Acceptor) / Q000172:deficiency* / Q000235:genetics; D011956:Receptors, Cell Surface / Q000276:immunology; D015398:Signal Transduction; D051193:Toll-Like Receptors
chemical_list: D000907:Antibodies, Bacterial; D015850:Interleukin-6; D008562:Membrane Glycoproteins; D011956:Receptors, Cell Surface; D051193:Toll-Like Receptors; D017853:Phosphotransferases (Alcohol Group Acceptor); D053592:Interleukin-1 Receptor-Associated Kinases