[Steroidogenesis in patients with various adrenal and gonadal dysfunctions]

The paper deals with the results of investigations of steroidogenesis in patients with ACTH-dependent Icenko-Cushing's disease (CD), congenital adrenal hyperplasia (CAH), and stromal hyperthecosis (SH). Along with the traditional methods of evaluating the activity of steroid-producing glands (diurnal variations of steroid hormones and their precursors in the peripheral blood, their mean daily concentrations), the authors used a simultaneous selective catheterization technique for adrenal and ovarian veins. No pronounced circadian rhythms were revealed in most of the steroids measured in patients with CD. A sharp rise in the mean daily serum 17-hydroxypregnenolone concentrations coupled with the steady-state 17-hydroxyprogesterone level is considered to reflect the activation of steroid biosynthesis via the delta 5-pathway. CD patients were demonstrated to exhibit great differences in the daily pattern of aldosterone secretion depending on the development of essential hypertension. CAH patients had high 17-hydroxypregnenolone and 17-hydroxyprogesterone levels. Like 17-hydroxyprogesterone, 17-hydroxypregnenolone may be used as a diagnostic marker of CAH. Hyperandrogenism in this disorder was largely due to enhanced adrenal production of testosterone and delta 5 androgens DHA and DHA-S. Unlike CAH, SH was not associated with excessive secretion of either DHA or DHA-S, but the ovaries of these patients released greater amounts of testosterone, which led to hyperandrogenism.