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Pediatr Infect Dis J. 2014 Jul;33(7):767-9. doi: 10.1097/INF.0000000000000277.

Liver abscess complicated by diaphragm perforation and pleural empyema leads to the discovery of interleukin-1 receptor-associated kinase 4 deficiency.

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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.


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.

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