Autosomal recessive disorders, such as NF-kappa-B essential modulator (NEMO) or NF-kappa-B alpha inhibitor (IKBA) deficiency, along with IL-1 receptor-associated kinase type 4 (IRAK-4) and myeloid differentiation primary response 88 (MyD88) deficiency, disrupt Toll-like receptor and interleukin-1 receptor signaling pathways, leading to increased susceptibility to severe infections (40). Here, NFKBIA is linked to infection.