Pidgeon Thomas Edward, Ahmad Fateh, Hackett Scott, Rodrigues Desiderio, Nishikawa Hiroshi
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.
J Craniofac Surg. 2015 Jan;26(1):e2-3. doi: 10.1097/SCS.0000000000001184.
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.
白细胞介素-1受体相关激酶4(IRAK-4)缺陷是一种原发性免疫缺陷病,使受影响的患者易发生机会性化脓性感染。其临床表现可能多样,这种疾病的微生物学和免疫学管理已有文献记载;然而,因其表现的非典型性,在手术管理方面需要采取不同的方法。这是首例报道的IRAK-4缺陷患者软组织脓肿经颅进展的病例,重点是采用多学科方法治疗一个极其脆弱解剖部位的感染。