Infectious and inflammatory events in 2 siblings with IRF9 deficiency under immunoglobulin replacement therapy

Deficiency of interferon regulatory issue 9 (IRF9) is a particularly uncommon inborn error of immunity characterised by impaired kind I interferon signaling. It was first described in 2018 in a toddler with recurrent viral infections who developed life-threatening influenza.1 In 2019, we reported a household with 2 siblings affected by full IRF9 deficiency. The eldest youngster had skilled extreme viral infections, whereas the youngest was recognized shortly after delivery.2 To our information, no extra instances of IRF9 deficiency have been reported thus far.

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