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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.
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