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无高雄激素血症的多毛女性存在轻度肾上腺和卵巢类固醇生成异常:特发性多毛症是否存在?

Mild adrenal and ovarian steroidogenic abnormalities in hirsute women without hyperandrogenemia: does idiopathic hirsutism exist?

作者信息

Escobar-Morreale H F, Serrano-Gotarredona J, García-Robles R, Sancho J, Varela C

机构信息

Department of Endocrinology, Hospital Ramón y Cajal, Madrid, Spain.

出版信息

Metabolism. 1997 Aug;46(8):902-7. doi: 10.1016/s0026-0495(97)90077-9.

DOI:10.1016/s0026-0495(97)90077-9
PMID:9258272
Abstract

To study ovarian and adrenal steroid profiles of women with idiopathic hirsutism, we compared sex steroid and basal and corticotropin (ACTH)-stimulated adrenal steroid levels before and after ovarian suppression induced by a long-acting gonadotropin-releasing hormone agonist analog (GnRH-a) in 24 hirsute women without hyperandrogenemia. Twelve healthy women served as controls for basal and ACTH-stimulated adrenal steroid levels. Serum levels of testosterone (T), sex hormone-binding globulin (SHBG), estradiol (E2), basal and ACTH-stimulated 17-hydroxyprogesterone (17OHP), dehydroepiandrosterone (DHEA), DHEA sulfate (DHEAS), delta 4-androstenedione (delta 4-A), 11-deoxycortisol (S) and cortisol (F), and basal and luteinizing hormone-releasing hormone (LHRH)-stimulated gonadotropin levels were measured before and 21 days after 3.75 mg intramuscular triptorelin in hirsute women. Basal T levels and basal and ACTH-stimulated delta 4-A, DHEA, and DHEAS levels were not different in hirsute women with respect to controls. Basal and ACTH-stimulated 17OHP was elevated, and decreased to normal after ovarian suppression with triptorelin. Although basal and ACTH-stimulated delta 4-A levels were normal, the delta delta 4-A/delta F and delta delta 4-A/delta 17OHP ratios were elevated and remained elevated after ovarian suppression, suggesting enhanced adrenal delta 4-17,20-lyase activity. T, F, S, and DHEAS levels were not affected by ovarian suppression. Basal and ACTH-stimulated 17OHP and delta 4-A, and stimulated DHEA concentrations were reduced with ovarian suppression, but their net increment and ratio to the increase of F in response to ACTH remained unchanged, reflecting the ovarian contribution to the secretion of these steroids. We conclude that idiopathic hirsute women with normoandrogenemia show an increase in ovarian secretion of 17OHP and a minimally increased adrenal delta 4-17, 20-lyase activity, suggesting that mild forms of ovarian and adrenal functional hyperandrogenism may be present in these patients with otherwise unexplained hirsutism.

摘要

为研究特发性多毛症女性的卵巢和肾上腺类固醇谱,我们比较了24例无高雄激素血症的多毛症女性在长效促性腺激素释放激素激动剂类似物(GnRH-a)诱导卵巢抑制前后的性类固醇、基础及促肾上腺皮质激素(ACTH)刺激后的肾上腺类固醇水平。12名健康女性作为基础及ACTH刺激后肾上腺类固醇水平的对照。在多毛症女性中,于肌肉注射3.75 mg曲普瑞林前及21天后,测定血清睾酮(T)、性激素结合球蛋白(SHBG)、雌二醇(E2)、基础及ACTH刺激后的17-羟孕酮(17OHP)、脱氢表雄酮(DHEA)、硫酸脱氢表雄酮(DHEAS)、δ4-雄烯二酮(δ4-A)、11-脱氧皮质醇(S)和皮质醇(F),以及基础及促黄体生成素释放激素(LHRH)刺激后的促性腺激素水平。多毛症女性的基础T水平以及基础及ACTH刺激后的δ4-A、DHEA和DHEAS水平与对照组无差异。基础及ACTH刺激后的17OHP升高,经曲普瑞林卵巢抑制后降至正常。尽管基础及ACTH刺激后的δ4-A水平正常,但δ4-A/F和δ4-A/17OHP比值升高,且在卵巢抑制后仍保持升高,提示肾上腺δ4-17,20-裂解酶活性增强。T、F、S和DHEAS水平不受卵巢抑制影响。基础及ACTH刺激后的17OHP和δ4-A以及刺激后的DHEA浓度经卵巢抑制后降低,但其净增量以及对ACTH反应时与F增加的比值保持不变,反映了卵巢对这些类固醇分泌的贡献。我们得出结论,雄激素水平正常的特发性多毛症女性表现为卵巢17OHP分泌增加以及肾上腺δ4-17,20-裂解酶活性轻度增加,提示这些原因不明的多毛症患者可能存在轻度形式的卵巢和肾上腺功能性高雄激素血症。

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