11-OHD, which is caused by mutations in the CYP11B1 gene, is the second most common cause of CAH with 46,XX DSD in the world, and accounts for about 5% of CAH patients with a European ancestry [126] and for about 15% of CAH patients in the Muslim and Jewish Middle Eastern populations [127]. The gene discussed is CYP11B1; the disease is congenital adrenal hyperplasia.