CYP11A1 and Congenital adrenal insufficiency with 46, XY sex reversal OR 46,XY disorder of sex development-adrenal insufficiency due to CYP11A1 deficiency: As with CYP11A1/P450scc deficiency, these children all need endocrine follow-up for life to monitor puberty and ovarian function in 46,XX girls and for puberty induction and sex steroid replacement in 46,XY DSD.