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  Innate Immune Defects




Sepsis with gram positive organisms is a feature of innate immune deficiencies with defective toll-like receptor signaling such as IRAK4, MyD88, and NEMO deficiency. Infections with gram negative organisms have also been described (some patients with MyD88 deficiency have recurrent Pseudomonas infections as a primary phenotype). Patients with IRAK4 and MyD88 deficiency are characterized by poor febrile response and low inflammatory marker elevation despite the presence of invasive infections such as sepsis or meningitis.

IRAK4 Deficiency

MyD88 Deficiency

NEMO Deficiency

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