sometimes been difficult to distinguish from the more common 21-hydroxylase deficient CAH because of the 17α-hydroxypregnenolone elevation, or from simple premature adrenarche because of the DHEA elevation. Management Some of the childhood management issues are similar those of 21-hydroxylase deficiency: Replacing mineralocorticoid with fludrocortisone Suppressing DHEA and replacing cortisol with glucocorticoid Providing extra glucocorticoid for stress Close monitoring and perhaps other adjunctive measures to optimize growth Deciding whether surgical repair of virilized female genitalia is warranted However, unlike 21-hydroxylase CAH, children with 3β-HSD CAH may be unable to produce adequate amounts of testosterone (boys) or estradiol (girls) to effect normal pubertal changes. Replacement testosterone or estrogen and progesterone can be initiated at adolescence and continued throughout adult life. Fertility may be impaired by the difficulty of providing appropriate sex hormone levels in the gonads even though the basic anatomy is present. See also Inborn errors of steroid metabolism Adrenal insufficiency Disorders of sexual development Intersexuality, pseudohermaphroditism, and ambiguous genitalia References External links Autosomal recessive disorders Congenital disorders of endocrine system Endocrine-related cutaneous conditions Intersex variations