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Ovarian steroidogenic responses to gonadotropin-releasing hormone agonist testing with nafarelin in hirsute women with adrenal responses to adrenocorticotropin suggestive of 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency.
J Clin Endocrinol Metab. 1993 Feb; 76(2):450-5.JC

Abstract

Nonclassical 3 beta-hydroxy-delta 5-steroid dehydrogenase (3 beta-HSD) deficiency type of congenital adrenal hyperplasia has been hypothesized to occur in as many as 10-40% of hirsute women, based on the adrenal steroidogenic responses to ACTH. However, diagnostic criteria for this "late-onset" 3 beta-HSD deficiency are not clearly established. Among 40 successive hyperandrogenic women undergoing evaluation of adrenal steroidogenic responses to ACTH, 8 had responses suggestive of 3 beta-HSD deficiency. Since 3 beta-HSD is present in both the ovary and adrenal, we attempted to document the defect in the ovary by stimulating their ovarian function with a gonadotropin-releasing hormone agonist test using nafarelin (6-D-[2-naphthyl]alanine-gonadotropin-releasing hormone). The eight hirsute women had steroid responses to ACTH suggestive of 3 beta-HSD deficiency, namely, the values of the delta 5-steroids, 17-hydroxypregnenolone and dehydroepiandrosterone, 30 and 60 min after ACTH in each hirsute woman were greater than 2 SD above the normal mean. Seven of the eight hirsute women had at least one elevated delta 5/delta 4-steroid ratio; however, only three of the hirsute women had two abnormal ratios. Furthermore, the response of the delta 4-steroid androstenedione and the ratio of androstenedione to cortisol after ACTH were significantly increased in the hirsute women, findings not consistent with 3 beta-HSD deficiency. After nafarelin, five and six hirsute patients had elevated values of the delta 4-steroids androstenedione and 17-hydroxyprogesterone, respectively. No patient had an elevated delta 5/delta 4-steroid ratio after nafarelin. Thus, ovarian steroidogenic responses to nafarelin did not support the diagnosis of 3 beta-HSD deficiency. Rather, they are consistent in most cases with polycystic ovary syndrome due to dysregulation of 17-hydroxylase and 17,20-lyase activities. We propose that increased activity of the enzyme P450c17 alpha in the adrenal cortex is responsible for most of what is often termed late-onset 3 beta-HSD deficiency.

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Authors+Show Affiliations

Barnes RB
Department of Obstetrics/Gynecology, University of Chicago, Pritzker School of Medicine, Illinois 60637.
Ehrmann DA
No affiliation info available
Brigell DF
No affiliation info available
Rosenfield RL
No affiliation info available

MeSH

17-alpha-Hydroxypregnenolone17-alpha-Hydroxyprogesterone3-Hydroxysteroid DehydrogenasesAdolescentAdrenal GlandsAdrenocorticotropic HormoneAdultDehydroepiandrosteroneFemaleHirsutismHumansHydroxyprogesteronesKineticsNafarelinOvarySteroidsTestosterone

Pub Type(s)

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

Language

eng

PubMed ID

8381802

Citation

Barnes, R B., et al. "Ovarian Steroidogenic Responses to Gonadotropin-releasing Hormone Agonist Testing With Nafarelin in Hirsute Women With Adrenal Responses to Adrenocorticotropin Suggestive of 3 Beta-hydroxy-delta 5-steroid Dehydrogenase Deficiency." The Journal of Clinical Endocrinology and Metabolism, vol. 76, no. 2, 1993, pp. 450-5.
Barnes RB, Ehrmann DA, Brigell DF, et al. Ovarian steroidogenic responses to gonadotropin-releasing hormone agonist testing with nafarelin in hirsute women with adrenal responses to adrenocorticotropin suggestive of 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency. J Clin Endocrinol Metab. 1993;76(2):450-5.
Barnes, R. B., Ehrmann, D. A., Brigell, D. F., & Rosenfield, R. L. (1993). Ovarian steroidogenic responses to gonadotropin-releasing hormone agonist testing with nafarelin in hirsute women with adrenal responses to adrenocorticotropin suggestive of 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency. The Journal of Clinical Endocrinology and Metabolism, 76(2), 450-5.
Barnes RB, et al. Ovarian Steroidogenic Responses to Gonadotropin-releasing Hormone Agonist Testing With Nafarelin in Hirsute Women With Adrenal Responses to Adrenocorticotropin Suggestive of 3 Beta-hydroxy-delta 5-steroid Dehydrogenase Deficiency. J Clin Endocrinol Metab. 1993;76(2):450-5. PubMed PMID: 8381802.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Ovarian steroidogenic responses to gonadotropin-releasing hormone agonist testing with nafarelin in hirsute women with adrenal responses to adrenocorticotropin suggestive of 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency. AU - Barnes,R B, AU - Ehrmann,D A, AU - Brigell,D F, AU - Rosenfield,R L, PY - 1993/2/1/pubmed PY - 1993/2/1/medline PY - 1993/2/1/entrez SP - 450 EP - 5 JF - The Journal of clinical endocrinology and metabolism JO - J Clin Endocrinol Metab VL - 76 IS - 2 N2 - Nonclassical 3 beta-hydroxy-delta 5-steroid dehydrogenase (3 beta-HSD) deficiency type of congenital adrenal hyperplasia has been hypothesized to occur in as many as 10-40% of hirsute women, based on the adrenal steroidogenic responses to ACTH. However, diagnostic criteria for this "late-onset" 3 beta-HSD deficiency are not clearly established. Among 40 successive hyperandrogenic women undergoing evaluation of adrenal steroidogenic responses to ACTH, 8 had responses suggestive of 3 beta-HSD deficiency. Since 3 beta-HSD is present in both the ovary and adrenal, we attempted to document the defect in the ovary by stimulating their ovarian function with a gonadotropin-releasing hormone agonist test using nafarelin (6-D-[2-naphthyl]alanine-gonadotropin-releasing hormone). The eight hirsute women had steroid responses to ACTH suggestive of 3 beta-HSD deficiency, namely, the values of the delta 5-steroids, 17-hydroxypregnenolone and dehydroepiandrosterone, 30 and 60 min after ACTH in each hirsute woman were greater than 2 SD above the normal mean. Seven of the eight hirsute women had at least one elevated delta 5/delta 4-steroid ratio; however, only three of the hirsute women had two abnormal ratios. Furthermore, the response of the delta 4-steroid androstenedione and the ratio of androstenedione to cortisol after ACTH were significantly increased in the hirsute women, findings not consistent with 3 beta-HSD deficiency. After nafarelin, five and six hirsute patients had elevated values of the delta 4-steroids androstenedione and 17-hydroxyprogesterone, respectively. No patient had an elevated delta 5/delta 4-steroid ratio after nafarelin. Thus, ovarian steroidogenic responses to nafarelin did not support the diagnosis of 3 beta-HSD deficiency. Rather, they are consistent in most cases with polycystic ovary syndrome due to dysregulation of 17-hydroxylase and 17,20-lyase activities. We propose that increased activity of the enzyme P450c17 alpha in the adrenal cortex is responsible for most of what is often termed late-onset 3 beta-HSD deficiency. SN - 0021-972X UR - https://www.unboundmedicine.com/medline/citation/8381802/Ovarian_steroidogenic_responses_to_gonadotropin_releasing_hormone_agonist_testing_with_nafarelin_in_hirsute_women_with_adrenal_responses_to_adrenocorticotropin_suggestive_of_3_beta_hydroxy_delta_5_steroid_dehydrogenase_deficiency_ L2 - https://academic.oup.com/jcem/article-lookup/doi/10.1210/jcem.76.2.8381802 DB - PRIME DP - Unbound Medicine ER -