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

Abstract

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.

DOI: 10.1097/INF.0000000000000277

Cite this paper

@article{Schndorf2014LiverAC, title={Liver abscess complicated by diaphragm perforation and pleural empyema leads to the discovery of interleukin-1 receptor-associated kinase 4 deficiency.}, author={Dominik Sch{\"{o}ndorf and Horst von Bernuth and Arne Simon and G K Schneider and Uwe Koelsch and Klaus Schwarz and Clemens-Magnus Meier and J{\"{o}rg Groe-Onnebrink and L Gortner and Tilman Robert Rohrer}, journal={The Pediatric infectious disease journal}, year={2014}, volume={33 7}, pages={767-9} }