Ibáñez L, Potau N, Zampolli M, Prat N, Gussinyé M, Saenger P, Vicens-Calvet E, Carrascosa A
Endocrinology Unit, Hospital Materno-Infantil Vall d'Hebron, Autonomous University, Barcelona, Spain.
J Clin Endocrinol Metab. 1994 Dec;79(6):1778-84. doi: 10.1210/jcem.79.6.7989484.
Functional ovarian hyperandrogenism (FOH) is characterized by an abnormal ovarian response to challenge with the GnRH analogs nafarelin and leuprolide acetate, similar to that observed in women with well defined polycystic ovary syndrome, regardless of whether elevated LH levels or polycystic ovaries are present. We studied an unselected group of 42 hyperandrogenic adolescents (age range, 14-22 yr; mean, 18.1 +/- 2.5 yr) 1) to determine FOH incidence through the assessment of ovarian-steroidogenic response to a single dose of leuprolide acetate, 2) to assess the clinical characteristics of patients according to their responses to GnRH analog stimulation, and 3) to evaluate adrenal steroidogenic function and its relation to ovarian hyperandrogenism in patients with either normal or abnormal responses to leuprolide acetate challenge. All patients underwent leuprolide acetate and ACTH testing, dexamethasone and ovarian suppression tests, and pelvic ultrasonography. Twenty-four (58%) patients had supranormal plasma 17-hydroxyprogesterone (17-OHP) responses to leuprolide acetate characteristic of FOH, and in 18, the 17-OHP response was similar to that of controls (n = 24; age, 17.1 +/- 2.3 yr). Seven patients (5 with FOH and 2 with normal responses to leuprolide acetate) had an abnormal response to ACTH, but only 1 had conclusive evidence of 21-hydroxylase deficiency. In 16 patients, the response to both stimulation tests was normal. Only 13 (54%) of the 24 FOH patients had polycystic ovaries on ultrasonography, and in 11 (46%), basal plasma LH levels were elevated. In FOH patients, reduction in testosterone and androstenedione plasma levels was significantly greater after ovarian suppression than after dexamethasone challenge (P < 0.0005 and P < 0.02, respectively). Peak plasma 17-OHP levels postleoprolide acetate simulation correlated with dexamethasone-suppressed plasma testosterone concentrations, dexamethasone-suppressed plasma androstenedione levels, and the free androgen index postdexamethasone treatment (r = 0.4, P = 0.01; r+ 0.4, P < 0.05; and r = 0.41, P = 0.007, respectively), Plasma sex hormone-binding globulin levels after dexamethasone administration correlated negatively with the baseline free androgen index (r = -.0.67; P < 0.0001). Considering our diagnostic criteria, 26 (62%) of our collective of 42 patients had abnormal responses to one or both stimulation tests, whereas 16 (37%) had normal response. FOH is the most common cause in (58%) of androgen excess in adolescence. Short term leuprolide acetate stimulation is a reliable tool fro identification of the ovary as the source of their hyperandrogenism.
功能性卵巢雄激素过多症（FOH）的特征是卵巢对促性腺激素释放激素（GnRH）类似物那法瑞林和醋酸亮丙瑞林激发试验反应异常，这与多囊卵巢综合征明确的女性所观察到的情况相似，无论是否存在促黄体生成素（LH）水平升高或多囊卵巢。我们研究了一组未经挑选的42名高雄激素血症青少年（年龄范围14 - 22岁；平均18.1±2.5岁），1）通过评估单剂量醋酸亮丙瑞林激发试验的卵巢类固醇生成反应来确定FOH发病率，2）根据患者对GnRH类似物刺激的反应评估临床特征，3）评估醋酸亮丙瑞林激发试验反应正常或异常患者的肾上腺类固醇生成功能及其与卵巢雄激素过多症的关系。所有患者均接受了醋酸亮丙瑞林和促肾上腺皮质激素（ACTH）试验、地塞米松和卵巢抑制试验以及盆腔超声检查。24名（58%）患者对醋酸亮丙瑞林激发试验出现血浆17 - 羟孕酮（17 - OHP）超正常反应，具有FOH特征，18名患者的17 - OHP反应与对照组（n = 24；年龄17.1±2.3岁）相似。7名患者（5名FOH患者和2名对醋酸亮丙瑞林反应正常的患者）对ACTH试验反应异常，但仅1名有21 - 羟化酶缺乏的确凿证据。16名患者对两种激发试验反应均正常。24名FOH患者中仅13名（54%）超声检查有多囊卵巢，11名（46%）基础血浆LH水平升高。在FOH患者中，卵巢抑制后血浆睾酮和雄烯二酮水平的降低显著大于地塞米松激发试验后（分别为P < 0.0005和P < 0.02）。醋酸亮丙瑞林激发试验后血浆17 - OHP峰值水平与地塞米松抑制后的血浆睾酮浓度、地塞米松抑制后的血浆雄烯二酮水平以及地塞米松治疗后的游离雄激素指数相关（分别为r = 0.4，P = 0.01；r = 0.4，P < 0.05；r = 0.41，P = 0.007），地塞米松给药后血浆性激素结合球蛋白水平与基线游离雄激素指数呈负相关（r = - 0.67；P < 我们的42名患者中有26名（62%）对一种或两种激发试验反应异常，而16名（37%）反应正常。FOH是青春期雄激素过多症（58%）的最常见原因。短期醋酸亮丙瑞林激发试验是确定卵巢为高雄激素血症来源的可靠工具。
