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Late-onset congenital adrenal hyperplasia in women with hirsutism.
Eur J Clin Invest. 1992 Oct; 22(10):651-8.EJ

Abstract

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, or increased peripheral conversion of weak androgenic hormones to more potent androgens. Late-onset (non-classic) congenital adrenal hyperplasia is a cause of hirsutism in adult women, but its frequency and the patterns of abnormalities in adrenal hormone secretion are not well understood. The frequency of non-classical adrenal hyperplasia due to deficiencies of 3 beta-hydroxy-delta 5-steroid dehydrogenase, 21-hydroxylase, and 11 beta-hydroxylase among 65 women with hirsutism were determined. All enzyme defects were identified by comparing the patients' hormonal responses to 0.25 mg intravenous bolus of alpha 1-24-ACTH with those of age-matched normal women. The hormones measured in plasma during the ACTH stimulation tests were progesterone, 17-hydroxypregnenolone, 17-hydroxyprogesterone, DHEA-sulfate, androstenedione, testosterone, 11-deoxycortisol, and cortisol. Similarly these hormones were measured after overnight 1 mg oral dexamethasone. Twelve women (18.5%) had 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency, 24 (37%) 21-hydroxylase deficiency, and 14 (21.5%) 11 beta-hydroxylase deficiency. Women with 21-hydroxylase deficiency also had evidence of a partial deficiency in 11 beta-hydroxylase activity (12 of the 24 patients). Similarly, most (11 of the 14) of the women with 11 beta-hydroxylase deficiency also had evidence of a deficiency in 3 beta-hydroxy-delta 5-steroid dehydrogenase activity. Among the 15 patients with no adrenal biosynthetic defect, eight had high plasma androgen concentrations, and seven had normal concentrations.(ABSTRACT TRUNCATED AT 250 WORDS)

Authors+Show Affiliations

Department of Medicine, School of Medicine, University of Jordan, Amman.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

1333959

Citation

Arnaout, M A.. "Late-onset Congenital Adrenal Hyperplasia in Women With Hirsutism." European Journal of Clinical Investigation, vol. 22, no. 10, 1992, pp. 651-8.
Arnaout MA. Late-onset congenital adrenal hyperplasia in women with hirsutism. Eur J Clin Invest. 1992;22(10):651-8.
Arnaout, M. A. (1992). Late-onset congenital adrenal hyperplasia in women with hirsutism. European Journal of Clinical Investigation, 22(10), 651-8.
Arnaout MA. Late-onset Congenital Adrenal Hyperplasia in Women With Hirsutism. Eur J Clin Invest. 1992;22(10):651-8. PubMed PMID: 1333959.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Late-onset congenital adrenal hyperplasia in women with hirsutism. A1 - Arnaout,M A, PY - 1992/10/1/pubmed PY - 1992/10/1/medline PY - 1992/10/1/entrez SP - 651 EP - 8 JF - European journal of clinical investigation JO - Eur J Clin Invest VL - 22 IS - 10 N2 - 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, or increased peripheral conversion of weak androgenic hormones to more potent androgens. Late-onset (non-classic) congenital adrenal hyperplasia is a cause of hirsutism in adult women, but its frequency and the patterns of abnormalities in adrenal hormone secretion are not well understood. The frequency of non-classical adrenal hyperplasia due to deficiencies of 3 beta-hydroxy-delta 5-steroid dehydrogenase, 21-hydroxylase, and 11 beta-hydroxylase among 65 women with hirsutism were determined. All enzyme defects were identified by comparing the patients' hormonal responses to 0.25 mg intravenous bolus of alpha 1-24-ACTH with those of age-matched normal women. The hormones measured in plasma during the ACTH stimulation tests were progesterone, 17-hydroxypregnenolone, 17-hydroxyprogesterone, DHEA-sulfate, androstenedione, testosterone, 11-deoxycortisol, and cortisol. Similarly these hormones were measured after overnight 1 mg oral dexamethasone. Twelve women (18.5%) had 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency, 24 (37%) 21-hydroxylase deficiency, and 14 (21.5%) 11 beta-hydroxylase deficiency. Women with 21-hydroxylase deficiency also had evidence of a partial deficiency in 11 beta-hydroxylase activity (12 of the 24 patients). Similarly, most (11 of the 14) of the women with 11 beta-hydroxylase deficiency also had evidence of a deficiency in 3 beta-hydroxy-delta 5-steroid dehydrogenase activity. Among the 15 patients with no adrenal biosynthetic defect, eight had high plasma androgen concentrations, and seven had normal concentrations.(ABSTRACT TRUNCATED AT 250 WORDS) SN - 0014-2972 UR - https://www.unboundmedicine.com/medline/citation/1333959/Late_onset_congenital_adrenal_hyperplasia_in_women_with_hirsutism_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0014-2972&date=1992&volume=22&issue=10&spage=651 DB - PRIME DP - Unbound Medicine ER -