Selma F. Siegel

ACTH stimulation tests and plasma dehydroepiandrosterone sulfate levels in women with hirsutism

BACKGROUND: Hirsutism in women is a clinical manifestation of excessive production of androgens. The source of the excess androgen may be either the ovaries or the adrenal glands, and distinguishing between these sources may be difficult. METHODS: To determine whether measurements of plasma dehydroepiandrosterone (DHEA) sulfate and ACTH stimulation tests, both widely used in the evaluation of hirsutism in women, provide useful information, we performed both tests in 22 normal women and 31 female patients with hirsutism. The hormones measured in plasma during the ACTH stimulation tests were progesterone, 17-hydroxypregnenolone, 17-hydroxyprogesterone, DHEA, androstenedione, 11-deoxycortisol, and cortisol. RESULTS: The women with hirsutism were divided into four groups based on their individual responses to ACTH stimulation: patients with a possible 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency, those with a possible 21-hydroxylase deficiency, those with a possible 11 beta-hydroxylase deficiency, and those with no apparent defect in steroidogenesis. The results in 19 patients (61 percent) suggested subtle defects in adrenal steroidogenesis. There was no significant correlation between the basal plasma DHEA sulfate levels and the hormonal response to ACTH, nor were the basal levels of hormones predictive of the levels after ACTH stimulation. Eleven patients had significantly elevated basal levels of plasma DHEA sulfate; only 5 of these 11 had responses to ACTH suggestive of compromised steroidogenesis. Thirteen patients who had responses suggestive of defective steroidogenesis had DHEA sulfate levels within the normal range. CONCLUSIONS: A substantial proportion of women with hirsutism have mild defects in adrenal steroidogenesis, revealed by an ACTH stimulation test, that are indicative of late-onset (nonclassic) congenital adrenal hyperplasia. Measurements of basal steroid levels are not helpful in differentiating among the causes of increased androgen production in such patients and may be misleading.

An unusual presentation of periarteritis nodosa

Periarteritis nodosa is characterized by generalized inflammation of medium and small arteries that leads to thrombosis or aneurysmal dilatation. We report the case of a 30-year-old man with no preceding signs or symptoms who presented initially with a chief complaint of mild abdominal pain. He deteriorated clinically during the next six to eight hours, and developed shock secondary to a ruptured aneurysm of the left gastric artery. His postoperative course was complicated by recurrent bleeding and death within 48 hours. Our case represents a protean clinical manifestation of periarteritis nodosa and expands the differential diagnosis of acute abdominal pain.

Detection of Y-chromosome sequences in gonadal tissue of patients with Turner's syndrome (45,X)

To the Editor: Molecular biologic techniques for the detection of Y-chromosome sequences have illustrated the limitations of cytogenetic analysis in the evaluation of patients with Turners syndrom...

Polycystic ovary syndrome

Polycystic ovary syndrome, characterized by hyperandrogenism and menstrual irregularities, is a heterogeneous group of disorders that can disrupt normal hypothalamic-pituitary-ovarian function. Ovarian physiology is reviewed with regard to possible etiologies for excessive androgen secretion. Similar features may be present in errors in steroidogenesis, hyperprolactinemia, androgen-secreting tumors, and Cushings syndrome. Analysis of the interrelationships between the reproductive axis and carbohydrate metabolism suggests that the associated insulin resistance can induce hyperandrogenism. Improved understanding of the various etiologies may expand the therapeutic options for patients.

ACTH Stimulation Tests and Plasma Dehydroepiandrosterone Sulfate Levels in Women with Hirsutism

BACKGROUND Hirsutism in women is a clinical manifestation of excessive production of androgens. The source of the excess androgen may be either the ovaries or the adrenal glands, and distinguishing between these sources may be difficult. METHODS To determine whether measurements of plasma dehydroepiandrosterone (DHEA) sulfate and ACTH stimulation tests, both widely used in the evaluation of hirsutism in women, provide useful information, we performed both tests in 22 normal women and 31 female patients with hirsutism. The hormones measured in plasma during the ACTH stimulation tests were progesterone, 17-hydroxypregnenolone, 17-hydroxyprogesterone, DHEA, androstenedione, 11-deoxycortisol, and cortisol. RESULTS The women with hirsutism were divided into four groups based on their individual responses to ACTH stimulation: patients with a possible 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency, those with a possible 21-hydroxylase deficiency, those with a possible 11 beta-hydroxylase deficiency, and those with no apparent defect in steroidogenesis. The results in 19 patients (61 percent) suggested subtle defects in adrenal steroidogenesis. There was no significant correlation between the basal plasma DHEA sulfate levels and the hormonal response to ACTH, nor were the basal levels of hormones predictive of the levels after ACTH stimulation. Eleven patients had significantly elevated basal levels of plasma DHEA sulfate; only 5 of these 11 had responses to ACTH suggestive of compromised steroidogenesis. Thirteen patients who had responses suggestive of defective steroidogenesis had DHEA sulfate levels within the normal range. CONCLUSIONS A substantial proportion of women with hirsutism have mild defects in adrenal steroidogenesis, revealed by an ACTH stimulation test, that are indicative of late-onset (nonclassic) congenital adrenal hyperplasia. Measurements of basal steroid levels are not helpful in differentiating among the causes of increased androgen production in such patients and may be misleading.