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Table 2 Clinical and management features of different forms of HIES

From: Case report of a young child with disseminated histoplasmosis and review of hyper immunoglobulin e syndrome (HIES)

Mutation Inheritance Cases reported Clinical distinctive characteristics Management
STAT3 AD 300-400 (in U.S.); 0.5-1 per million Skeletal and dental abnormalities Non-immunologic evaluation and treatment, prophylactic antibiotics +/- antifungals
TYK2 AR 1-2 (none in U.S.) No pneumatoceles, recurrent viral infections, mycobacterial infection Prophylactic antibiotics +/- antifungals +/- antivirals
DOC8 AR 50-60 Recurrent viral infections, food allergies/rhinitis, lymphopenia, increased risk of malignancy, CNS vasculitis Prophylactic antibiotics and antivirals +/- antifungals, IVIG if antibody deficiency, allogeneic hematopoietic stem cell transplantation