%0 Case Reports
%T IRAK-4 deficiency as a cause for familial fatal invasive infection by Streptococcus pneumoniae.
%A Grazioli S
%A Hamilton SJ
%A McKinnon ML
%A Del Bel KL
%A Hoang L
%A Cook VE
%A Hildebrand KJ
%A Junker AK
%A Turvey SE
%J Clin Immunol
%V 163
%N 0
%D Feb 2016
%M 26698383
%F 10.19
%R 10.1016/j.clim.2015.12.007
%X In this Letter to the Editor we report the case of two siblings with fatal pneumococcal meningitis as the initial manifestation of IRAK-4 deficiency caused by previously undescribed mutations in IRAK4. The letter also highlights the importance of invasive pneumococcal infection as a critical 'red flag' warning of the potential for an underlying primary immunodeficiency and identifies some of the challenges in making the clinical diagnosis of IRAK-4 deficiency.