title: [Steroidogenesis in patients with various adrenal and gonadal dysfunctions].
abstract: 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.
authors.forename: N P
authors.initials: NP
authors.lastname: Goncharov
authors.forename: G S
authors.initials: GS
authors.lastname: Kolesnikova
authors.forename: V I
authors.initials: VI
authors.lastname: Vorontsov
authors.forename: L Ia
authors.initials: LIa
authors.lastname: Rozhinskaia
authors.forename: E I
authors.initials: EI
authors.lastname: Marova
mesh_terms: D006907:17-alpha-Hydroxypregnenolone / Q000097:blood; D019326:17-alpha-Hydroxyprogesterone; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood / Q000503:physiopathology*; D000328:Adult; D000728:Androgens / Q000097:blood; D002940:Circadian Rhythm; D003480:Cushing Syndrome / Q000097:blood / Q000503:physiopathology*; D005260:Female; D006801:Humans; D006908:Hydroxyprogesterones / Q000097:blood; D017588:Hyperandrogenism / Q000097:blood / Q000503:physiopathology*; D008297:Male; D010049:Ovarian Diseases / Q000097:blood / Q000503:physiopathology*; D013256:Steroids / Q000096:biosynthesis*; D013739:Testosterone / Q000097:blood; D013799:Theca Cells
chemical_list: D000728:Androgens; D006908:Hydroxyprogesterones; D013256:Steroids; D006907:17-alpha-Hydroxypregnenolone; D013739:Testosterone; D019326:17-alpha-Hydroxyprogesterone
vernacular_title: Steroidogenez u bol'nykh s razlichnymi disfunktsiiami nadpochechnikovykh i polovykh zhelez.