Infections in IFNGR-1-Deficient Children

Abstract
Human interferon-γ receptor 1 (IFNGR-1) deficiency is a newly identified autosomal recessive inherited immune disorder. Children with IFNGR-1 deficiency exhibit a severe, profound and selective susceptibility to weakly virulent mycobacteria, such as bacillus Calmette-Guérin (BCG) vaccine or environmental nontubcrculous mycobacteria (NTM). This review compares the infections found in IFNGR-1-deficient children to those in IFN-γ-deficient or IFNGR-1-deficient mice.