Patterns of Steroid Excretion in Three Types of Post-Pubertal Hirsutism

Abstract

The steroid excretion of patients with three types of post-pubertal hirsutism has been studied both before and during the administration of corticotrophin. The types of hirsutism distinguished were post-pubertal adrenal virilism (three cases), idiopathic hirsutism (five cases) and the Stein-Leventhal syndrome (seven cases).1. The patients in the adrenal virilism group were found to have a pattern of steroid excretion of the type seen in congenital adrenal hyperplasia. Thus they excreted relatively small amounts of cortisol metabolites in the urine, but the excretions of pregnanetriol, 17-hydroxypregnanolone, 11-oxopregnanetriol and 11-hydroxyandrosterone were high.2. The patients with idiopathic hirsutism showed a tendency to excrete relatively more of those 17-oxosteroids derived from C19 precursors compared with those derived from cortisol. The evidence suggests, however, that this disproportion is due not to an impairment of cortisol synthesis but to a potentiation of the production of adrenal androgens.3. A similar abnormality in the patterns of steroid excretion was also found in some of the patients with the Stein-Leventhal syndrome. Since this abnormality includes the excretion of increased amounts of those 17-oxosteroids thought to be exclusively of adrenal origin, viz. dehydroepiandrosterone and 11-hydroxyandrosterone, this would suggest adrenal involvement in at least some of the cases.

The Plasma Cortisol Response to Acth in “Idiopathic Hirsutism”*

“…Patients with the postpubertal form of congenital adrenal hyperplasia excrete increased quantities of urinary 17-ketosteroids and manifest virilism (3). While neither of these criteria was fulfilled in any of our patients, their urinary pregnanediol and pregnanetriol excretion was not studied..…”

“…These were 10 consecutive women, referred to us with primary complaints of hypertrichosis; their mean age was (1,3), these patients were chosen only if their total 17-ketosteroid and corticoid excretion in a 24-hour urine specimen was within normal range. Adult hirsutism may be a manifestation of ovarian neoplasms and Cushing's syndrome and is associated with the postpubertal analog of congenital adrenal hyperplasia, and the Stein-Leventhal syndrome (3,15). It is most unlikely that the patients studied here represented hirsutism secondary to ovarian neoplasia or Cushing's syndrome, since they were selected from a clinical population in which either of these two disease entities is extremely rare.…”

“…It appears that this disorder is associated with increased production of an adrenal androgen which is reflected by increased excretion of urinary C1902 17-ketosteroids, in particular, androsterone and etiocholanolonel (1)(2)(3)(4)(5)(6)(7). Gallagher and co-workers (1), Kappas, Pearson, West and Gallagher (2) and Brooks and Prunty (3) reported that the urinary 11-oxy-17-ketosteroids may also be increased in these patients, and Bush and Mahesh (4) noted, in particular, increased excretion of 1 1-hydroxyandrosterone. These 11-oxygenated steroids are presumed to be derived principally from cortisol and 11-hydroxyandrostenedione metabolism (8).…”

Urinary 17-Ketosteroid Conjugates in Hirsutism

“…Some of the patients classified as having "idiopathic hirsutism" (7)(8)(9)(10)(11)(12)(13)(14)(15)(16) had menstrual abnormalities, but none of this group had enlarged ovaries. A 24-hour urine specimen served as a control in each patient studied.…”

“…The second abnormality is an increased urinary level of androsterone and etiocholanolone. In the latter instance some authors have suggested that the adrenal is responsible for the precursors of the increased androsterone and etiocholanolone (3)(4)(5)(6)(7), although others have suggested an ovarian origin and still others could not discern the glandular origin of these steroids (8)(9)(10). A significant residual level of urinary 17KS after adrenal suppression following administration of analogs of cortisol (11)(12)(13)(14)(15) favors an ovarian origin of the precursors responsible for the elevation of the androsterone and etiocholanolone values reported.…”