The Relationships Between Serum Concentrations of 17OH-Progesterone and Other Serum and Urinary Steroids in Patients with Congenital Adrenal Hyperplasia*

Abstract

Simultaneous determinations of serum concentrations of 17OH-progesterone, testosterone, an-drostenedione, andprogesterone, andof urinary excretion of 17-ketosteroids and pregnanetriolhave been performed at intervals in 31 patients with the C21 hydroxylase form of congenitaladrenalhyperplasia. In prepubertal patients there were highly significant correlations between levels of 17OH-progesterone and those of testosterone, and rostenedione, and progesterone, respectively. Similar correlations were observedin adolescent girls. In adolescent boys rising 17OH-progesterone levels were reflected by increasing levels of androstenedione and progesterone, but there wasno change in serum testosterone concentrations. Levels of serum 17OH-progesterone below 200ng/dl were uniformly associated with normal serum concentrationsof testosterone, androstenedione, and progesterone, and normal urinary 17-ketosteroid and pregnanetriol excretion. In contrast, levels above1000ng/dl were accompanied by increased levels of the other steroids except in adolescent males; in this group the finding of unchanging serum testosterone concentrations in spite of rising 17OH-progesterone levels presumably indicates that testosterone of adrenal origin causes suppression of testicular testosterone production, either through a direct effect upon Leydig cells orviasuppression of LH release.