Hypogonadism and Mineralocorticoid Excess

Abstract

VARIOUS inherited defects in adrenal steroidogenesis have been described and carefully studied. The best known examples involve deficiencies in 21 and 11β-hydroxylation, which are necessary steps in cortisol biosynthesis.^{1 2 3} The initial biosynthetic pathways in both the adrenal gland and the gonad require similar enzyme-controlled alterations of the precursor steroid molecule.⁴ A defect in 17α-hydroxylation should theoretically diminish the synthesis of cortisol, estrogens and androgens. The presence of alternate pathways, however, might be associated with increased synthesis and secretion of various biologically active precursors, which could have clinical effects quite different from the virilization seen in other types of congenital adrenal . . .