How common are polycystic ovaries and the polycystic ovarian syndrome in women with Cushing's syndrome?
How common are polycystic ovaries and the polycystic ovarian syndrome in women with Cushing's syndrome?
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DOI：
10.1046/j.1365-2265.2000.01117.x
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发表时间：
2000-10-01
影响因子：
3.2
通讯作者：
Grossman, AB
中科院分区：
文献类型：
--
作者：
Kaltsas, GA;Korbonits, M;Grossman, AB
目的库欣综合征(CS)患者可出现月经不规律和高雄激素血症的症状/体征，其表型与多囊卵巢综合征(PCOS)相似，但目前尚无多囊卵巢(PCO)和/或PCOS在CS患者中的患病率数据。本研究旨在通过分析育龄妇女CS的临床、内分泌学和超声特征，探讨CS在育龄妇女中的存在情况。设计一项前瞻性研究，对1994年8月至2000年1月所有患有CS的育龄妇女(年龄18~40岁)进行研究。CS的诊断是基于适当的临床特征和血清午夜皮质醇升高，在正式的小剂量地塞米松抑制试验(LDDST)后，900h血清皮质醇未能降至低于50nmol/L。所有女性都有与可能的高雄激素血症、月经紊乱和不孕症相关的临床特征，并测量了循环中的促性腺激素、雌二醇、雄激素和SHBG水平；在她们的初步评估中进行了卵巢超声检查。结果11例患者均为ACTH依赖型CS，其中9例为库欣病(CD)，1例为支气管类癌引起的异位ACTH综合征，1例为周期性CS(来源不明)，2例为ACTH非依赖性CS(肾上腺腺瘤)。所有患有CS的女性至少有一种高雄激素血症的症状/体征(13例多毛症，7例痤疮，5例男性型脱发)。9名女性(70%)有月经紊乱(4名月经过少，4名闭经，1名多经)，而4名女性(30%)月经正常。作为一个整体，该组的血清雌二醇水平与正常月经妇女早期卵泡期的水平相似；然而，7名妇女的雌二醇、黄体生成素和卵泡刺激素水平较低，提示性腺激素减退症。血清雄激素水平(睾酮、雄二酮和DHEAS)，即使在出现高雄激素症状/体征的情况下，也在正常参考范围内，但即使在月经周期正常的妇女中，SHBG水平也一致下降。尿游离皮质醇与血清雌二醇、睾酮和SHBG水平呈负相关(r=-0.8，r=-0.86和r=-0.66，P<；0.02，P<；0.01和P<；0.05)，而与血清雌二醇、睾酮和SHBG水平呈负相关(r=-0.8，r=-0.86和r=-0.66，P<；0.01和P<；0.05)，而与黄体生成素和卵泡刺激素水平无关。尽管这13例患者中有7例缺乏正常促性腺激素刺激，但双侧卵巢的体积相对较小：右侧7.3ml，范围2.8-12.8ml，左侧5.3ml，范围2.3-13ml。雌激素充足(E&2>；140pmol/L)的女性血清雄烯二酮水平高于雌激素缺乏者(E-2&Lt；140pmol/L)，尿游离皮质醇水平低于雌激素缺乏者(E-2&Lt；140pmol/L)。13名妇女中有6名(46%)的卵巢形态提示为多囊卵巢，6名雌激素充足的妇女中有4名，7名雌激素缺乏的妇女中有2名。结论PCO和PCOS在库欣综合征患者中很常见；库欣综合征和皮质醇分泌中度升高的患者在雌二醇水平正常的情况下维持促性腺激素对卵巢的刺激，而库欣综合征和皮质醇分泌较高的患者发生性腺激素减退症。然而，即使在后一组中，卵巢容量仍保持较高水平，一些卵巢形态显示为后囊性卵巢阻塞。
OBJECTIVE Women with Cushing's syndrome (CS) may present with menstrual irregularity and symptoms/signs of hyperandrogenism, a phenotype similar to that of the polycystic ovarian syndrome (PCOS); however, currently there are no data on the prevalence of either polycystic ovaries (PCO) and/or PCOS in patients with CS. The aim of this study was to investigate their presence among women of reproductive age presenting with CS by analysing clinical, endocrinological and ultrasonographic features.DESIGN Prospective study of all women within the reproductive age (range 18-40 years) who presented with CS between August 1994 and January 2000.SUBJECTS AND METHODS Thirteen women (median age 32 years, range 18-39 years) with CS were evaluated. The diagnosis of CS was based on the presence of appropriate clinical features and an elevated serum midnight cortisol with failure to suppress 0900 hours serum cortisol to less than 50 nmol/l following a formal low-dose dexamethasone suppression test (LDDST). All women had their clinical features relevant to possible hyperandrogenism, menstrual disorder and infertility recorded, and circulating gonadotrophins, oestradiol, androgens and SHBG levels measured; ovarian ultrasonography was performed during their initial assessment. Relevant MR/CT imaging of the pituitary and/or adrenal glands was performed.RESULTS Eleven women had ACTH-dependent CS [nine Cushing's disease (CD), one ectopic ACTH syndrome due to a bronchial carcinoid, one periodic CS of unknown origin); two patients had ACTH-independent CS (adrenal adenomas). All women with CS had at least one symptom/sign of hyperandrogenism (13 hirsutism, seven acne, five male-pattern alopecia). Nine women (70%) had menstrual disturbances (four oligomenorrhoea, four amenorrhoea, one polymenorrhoea) while four women (30%) had a normal menstrual pattern. Serum oestradiol levels for the group as a whole were similar to those observed in the early follicular phase of normally menstruating women; however, seven women had low oestradiol, LH and FSH levels suggestive of hypogonadotrophic hypogonadism. Serum androgen levels (testosterone, androstendione and DHEAS), even in the presence of symptoms/signs of hyperandrogenism, were within the normal reference range but SHBG levels were uniformly decreased even in women with normal menstrual cycles. There was a negative correlation between urinary free cortisol, but not mean serum cortisol, and serum oestradiol, testosterone and SHBG levels (r = - 0.8, r = - 0.86 and r = - 0.66, P < 0.02, P < 0.01 and P < 0.05, respectively), but not LH or FSH levels. Despite the fact that seven of these 13 patients lacked normal gonadotrophin stimulation, ovarian volumes of both ovaries were relatively preserved: right 7.3 ml, range 2.8-12.8 ml, and left 5.3 ml, range 2.3-13 ml. Women who were defined as oestrogen sufficient (E-2 > 140 pmol/l) had higher serum androstenedione, and lower urinary free cortisol levels, than women who were oestrogen deficient (E-2 < 140 pmol/l). Six of the 13 women (46%) had ovarian morphology suggestive of PCO, four of six oestrogen sufficient women and two of seven oestrogen deficient women. The results did not differ according to the underlying cause of CS.CONCLUSIONS PCO and PCOS are common in women with Cushing's syndrome; women with Cushing's syndrome and only moderately elevated cortisol secretion maintain gonadotrophin stimulation to the ovary with normal oestradiol levels, in contrast to women with Cushing's syndrome and higher cortisol secretion who develop hypogonadotrophic hypogonadism. However, even in the latter group, high ovarian volumes were maintained and some had ovarian morphology suggestive of PCO.