Systemic inflammation in our patients was evidenced by persistently elevated CRP, ESR and serum amyloid A. This contrasts with the profile seen in patients with classic autosomal recessive IRAK-4 deficiency, who are typically slow to mount an inflammatory response, often with lower inflammatory markers, even in episodes of bacteremia (27). The gene discussed is CRP; the disease is hyperinsulinemic hypoglycemia, familial, 4.