title: Recurrent Staphylococcus aureus abscess and fatal pneumococcal septicemia due to IRAK-4 deficiency.
abstract: We describe the case of an infant with recurrent episodes of staphylococcal skin abscess and subsequent lethal pneumococcal meningitis/septicemia due to interleukin-1 receptor-associated kinase 4 (IRAK-4) deficiency. In this case, systemic signs of inflammatory response were poor and delayed. Among all other reported cases of IRAK-4 deficiency, none involved severe viral or fungal disease, and the range of infecting bacteria was narrow.
authors.affiliation: Departments of Pediatrics.
authors.forename: Koichi
authors.initials: K
authors.lastname: Shichijo
authors.affiliation: Departments of Pediatrics.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Ogose
authors.affiliation: Departments of Pediatrics.
authors.forename: Mari
authors.initials: M
authors.lastname: Kubota
authors.affiliation: Departments of Pediatrics.
authors.forename: Ayumi
authors.initials: A
authors.lastname: Tomimoto
authors.affiliation: Departments of Pediatrics.
authors.forename: Rieko
authors.initials: R
authors.lastname: Kondo
authors.affiliation: Departments of Pediatrics.
authors.forename: Takako
authors.initials: T
authors.lastname: Taniguchi
authors.affiliation: Departments of Pediatrics.
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Takahashi
authors.affiliation: Departments of Pediatrics.
authors.forename: Tadanori
authors.initials: T
authors.lastname: Nakatsu
authors.affiliation: Dermatology, Tokushima Red Cross Hospital, Tokushima, Japan.
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Urano
authors.affiliation: Departments of Pediatrics.
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Watanabe
mesh_terms: D000038:Abscess / Q000150:complications* / Q000175:diagnosis / Q000382:microbiology; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000175:diagnosis; D007223:Infant; D053592:Interleukin-1 Receptor-Associated Kinases; D008586:Meningitis, Pneumococcal / Q000150:complications* / Q000175:diagnosis / Q000382:microbiology; D000081207:Primary Immunodeficiency Diseases; D018805:Sepsis / Q000175:diagnosis / Q000209:etiology* / Q000382:microbiology; D013203:Staphylococcal Infections / Q000150:complications* / Q000175:diagnosis / Q000382:microbiology; D013211:Staphylococcus aureus / Q000302:isolation & purification*
chemical_list: D053592:Interleukin-1 Receptor-Associated Kinases
keywords: IRAK-4 deficiency; delayed systemic signs of inflammatory response; pneumococcal meningitis/septicemia; staphylococcal skin abscess