Hemizygous variants with loss of IKBKG/NEMO function are lethal in males, while IKBKG/NEMO variants with partial suppression of protein function are observed in heterozygous females with IP (3), as well as in males with the mosaic form of the disease or XXY genotype (Klinefelter syndrome) (4). This evidence concerns the gene IKBKG and Klinefelter syndrome.