Dynamics of adrenal steroidogenesis in childhood: changes in the excretion of 16-oxygenated- and 11-oxygenated-steroids by 21-hydroxylase deficient children at various ages.

Abstract

The close relationship between C19O2 steroid excretion and the ratio of C19O2/C21O5 steroids indicates that it is the elevation of C17,20-lyase activity, which represents the decisive factor in the cessation of androgen-corticoid disproportion during the puberty of healthy children. In 3-6 year-old C21-hydroxylase deficient children treated with corticoids in doses only partially suppressing endogenous ACTH secretion, the excretion of total C19 steroids increased continuously parallel with a well defined elevation of 16 alpha-oxygenated-C21 steroid excretion. The patients did not show the physical signs of adrenarche before six years. This can be attributed to three factors: a) substitutional corticoid therapy; b) intraglandular control of delta 5-pathway by 16 alpha-hydroxylation of C21 steroids; c) neutralisation by 11 beta-hydroxylation of the androgen effect of C19O2 steroids.