title: Management of a transcranial abscess secondary to interleukin-1 receptor associated kinase 4 deficiency.
abstract: Interleukin-1 receptor associated kinase 4 (IRAK-4) deficiency is a primary immunodeficiency that predisposes to opportunistic pyogenic infections in affected patients. The presentation can be variable, and the microbiological and immunologic management of this condition has been documented; however, the atypical nature of its presentation calls for a different approach in its surgical management. This is the first reported case of transcranial progression of a soft tissue abscess in a patient with IRAK-4 deficiency, with an emphasis on a multidisciplinary approach to treat infection at an extremely vulnerable anatomic site.
authors.affiliation: From the *University Hospital Coventry and Warwickshire National Health Service Trust, Coventry; †Department of Craniofacial Surgery, Birmingham Children's Hospital National Health Service Trust, Birmingham, West Midlands; and ‡Department of Paediatric Immunology, Heartlands Hospital, Birmingham, United Kingdom.
authors.forename: Thomas Edward
authors.initials: TE
authors.lastname: Pidgeon
authors.forename: Fateh
authors.initials: F
authors.lastname: Ahmad
authors.forename: Scott
authors.initials: S
authors.lastname: Hackett
authors.forename: Desiderio
authors.initials: D
authors.lastname: Rodrigues
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nishikawa
mesh_terms: D000038:Abscess / Q000382:microbiology*; D001922:Brain Abscess / Q000382:microbiology*; D002675:Child, Preschool; D004198:Disease Susceptibility; D004388:Dura Mater; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D007223:Infant; D053592:Interleukin-1 Receptor-Associated Kinases; D000081207:Primary Immunodeficiency Diseases; D011552:Pseudomonas Infections / Q000175:diagnosis*; D011550:Pseudomonas aeruginosa / Q000302:isolation & purification; D016896:Treatment Outcome
chemical_list: D053592:Interleukin-1 Receptor-Associated Kinases