Interleukin-1 receptor-associated kinase 4 (IRAK-4) deficiency predisposes to severe invasive bacterial infections in infancy and early childhood, often with a fatal course caused by a defect in Toll-like receptor and interleukin-1 receptor signaling. Despite severe invasive infections, acute phase responses are often diminished. We report the successful treatment of a child with multiple liver abscesses, diaphragm perforation and pleural empyema, accompanied by strong acute phase responses as a unique presentation of IRAK-4 deficiency.
From the *Department of General Pediatrics and Neonatology, Saarland University Medical Center, Homburg/Saar; †Department of Pediatric Pulmonology and Immunology, Charité Hospital, Humboldt University; ‡Department of Immunology, Laboratory Berlin Charité Vivantes, Berlin; §Departments of Pediatric Oncology and Hematology, and ¶Diagnostic and Interventional Radiology, Saarland University Medical Center, Homburg/Saar; ‖Institute of Transfusion Medicine, University of Ulm; **Institute of Clinical Transfusion Medicine and Immunogenetics, German Red-Cross Blood Service Baden-Württemberg-Hessen, Ulm; and ††Department of Pediatric Surgery, Saarland University Medical Center, Homburg/Saar, Germany.
Accepted for publication January 7, 2014.
The authors have no funding or conflicts of interest to disclose.
Address for correspondence: Dominik Schöndorf, MD, Department of General Pediatrics and Neonatology, Saarland University Medical Center, Kirrberger Str., Building 9 66421 Homburg/Saar, Germany. E-mail: email@example.com.