Infections in IFNGR-1-deficient children
E Jouanguy1, F Altare, S Lamhamedi-Cherradi
1INSERM U429, Hôpital Necker-Enfants Malades, Paris, France.
Abstract:
Human interferon-gamma 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-Guerin (BCG) vaccine or environmental nontuberculous mycobacteria (NTM). This review compares the infections found in IFNGR-1-deficient children to those in IFN-gamma-deficient or IFNGR-1-deficient mice.
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