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

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...

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Bibliographic Details
Published inThe Pediatric infectious disease journal Vol. 33; no. 7; p. 767
Main Authors Schöndorf, Dominik, von Bernuth, Horst, Simon, Arne, Schneider, Günther, Kölsch, Uwe, Schwarz, Klaus, Meier, Clemens-Magnus, Groe-Onnebrink, Jörg, Gortner, Ludwig, Rohrer, Tilman R
Format Journal Article
LanguageEnglish
Published United States 01.07.2014
Subjects
Anti-Bacterial Agents - therapeutic use
Diaphragm - pathology
Empyema, Pleural - etiology
Empyema, Pleural - pathology
Female
Humans
Infant
Infant, Newborn
Interleukin-1 Receptor-Associated Kinases - deficiency
Interleukin-6 - blood
Liver Abscess - complications
Liver Abscess - etiology
Liver Abscess - pathology
Magnetic Resonance Imaging
Radiography, Abdominal
Radiography, Thoracic
Severe Combined Immunodeficiency - complications
Treatment Outcome
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Summary: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.
ISSN:1532-0987
DOI:10.1097/INF.0000000000000277