Bouallouche A, Brerault J L, Fiet J, Julien R, Vermeulen C, Cathelineau G
Am J Obstet Gynecol. 1983 Sep 1;147(1):57-63. doi: 10.1016/0002-9378(83)90084-4.
Thirty-one cases of idiopathic hirsutism, characterized biochemically in the basal state by increased levels of urinary 3 alpha-androstane-5 alpha, 17 beta-diol and normal levels of the main androgens, were studied. In order to determine a possible etiologic heterogeneity of idiopathic hirsutism, pituitary gonadotropin responses to synthetic luteinizing-releasing hormone (LRH) and adrenal steroid responses to adrenocorticotropic hormone (ACTH) stimulation were evaluated and the results were compared to those in six normal women. On the basis of the results obtained in each hirsute patient after LRH and ACTH tests, two groups were identified. The majority, 23 of 31 hirsute patients (group I), had results similar to those in the control group. In the other eight patients (group II), biologic abnormalities were disclosed and suggested a partial adrenal 11 beta-hydroxylase defect in two patients, an incomplete form of adrenal 3 beta-ol deficiency in one patient, an adrenal hyperreactivity without evident cause in two patients, and polycystic ovary syndrome in association with an adrenal hyperreactivity in three patients. As a group, the eight patients showed ACTH-stimulated increments in testosterone, delta 4-androstenedione, dehydroepiandrosterone, and 17-ketosteroids that were significantly greater (p less than 0.01) than the mean responses in the control group. The conclusion is that some women who previously were designated as having "idiopathic" hirsutism had an adrenal and/or ovarian component to their hyperandrogenism which could be shown only by appropriate dynamic tests.
对31例特发性多毛症患者进行了研究，这些患者在基础状态下的生化特征为尿3α-雄烷-5α,17β-二醇水平升高，主要雄激素水平正常。为了确定特发性多毛症可能的病因异质性，评估了垂体促性腺激素对合成促黄体生成素释放激素（LRH）的反应以及肾上腺类固醇对促肾上腺皮质激素（ACTH）刺激的反应，并将结果与6名正常女性的结果进行了比较。根据LRH和ACTH试验后每个多毛症患者获得的结果，将患者分为两组。大多数患者，即31例多毛症患者中的23例（第一组），其结果与对照组相似。在其他8例患者（第二组）中，发现了生物学异常，提示2例患者存在部分肾上腺11β-羟化酶缺陷，1例患者存在不完全形式的肾上腺3β-醇缺乏，2例患者存在无明显原因的肾上腺高反应性，3例患者存在多囊卵巢综合征合并肾上腺高反应性。作为一个整体，这8例患者在ACTH刺激下睾酮、δ4-雄烯二酮、脱氢表雄酮和17-酮类固醇的增量显著高于（p<0.01）对照组的平均反应。结论是，一些以前被诊断为“特发性”多毛症的女性，其高雄激素血症存在肾上腺和/或卵巢成分，只有通过适当的动态试验才能显示出来。
