IRAK4 and hyperinsulinemic hypoglycemia, familial, 4: Specific defects of molecules in the TLR signaling pathway including interleukin-1-receptor associated kinase-4 deficiency (IRAK-4), myeloid differentiation factor 88 (MYD88) and nuclear factor-κB essential modulator deficiency (NEMO) [63,75,76,77,78] have recently been defined.