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Liver Abscess Complicated by Diaphragm Perforation and Pleural Empyema Leads to the Discovery of Interleukin-1 Receptor-associated Kinase 4 Deficiency

Schöndorf, Dominik MD*; von Bernuth, Horst MD, PhD†‡; Simon, Arne MD§; Schneider, Günther MD; Kölsch, Uwe MD; Schwarz, Klaus MD‖**; Meier, Clemens-Magnus MD††; Groe-Onnebrink, Jörg MD; Gortner, Ludwig MD*; Rohrer, Tilman R. MD*

Pediatric Infectious Disease Journal:
doi: 10.1097/INF.0000000000000277
Pathogenesis and Host Response

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.

Author Information

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:

© 2014 by Lippincott Williams & Wilkins, Inc.