Nonclassical congenital adrenal hyperplasia and the polycystic ovarian syndrome.
Nonclassical congenital adrenal hyperplasia and the polycystic ovarian syndrome.
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非经典先天性肾上腺增生症和多囊卵巢综合征。
DOI：
10.1111/j.1749-6632.1993.tb43866.x
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发表时间：
1993
影响因子：
5.2
通讯作者：
New,MI
中科院分区：
文献类型：
--
作者：
New,MI
PCOS具有包括卵巢类固醇生成明显功能障碍的激素模式，尽管内分泌变化的序列（或序列），可能具有可建立PCOS的固有旁分泌异常，仅被假定。在雄激素过度产生（或即使在雄激素水平正常化后下丘脑-垂体-肾上腺轴持续紊乱）的情况下发生PCOS，表明肾上腺酶缺陷，但性腺和肾上腺皮质通过血清类固醇的确切相互作用仍然是开放的。已经提出了几个有趣的PCOS发展模式，但这个问题仍然存在争议。卵巢超声异常在PCOS和肾上腺酶缺陷的妇女中可能相似，根据垂体促性腺激素对LHRH的反应将PCOS与非经典21-羟化酶缺陷鉴别也是非特异性的。17-OHP对ACTH测试的反应是必要的，以作出诊断（见列线图）。PCOS和非经典21-羟化酶缺乏症妇女亚组的初步数据表明，（1）超声检查卵巢囊性改变的大小和（2）LH：FSH比值可能通过地塞米松治疗得到改善。通过ACTH检测确定肾上腺酶紊乱对雄激素过多症状的患者具有成本效益，为夫妇生育能力的改善提供了良好的预后，并且在家族遗传学研究中也提供了信息。
PCOS has a hormonal pattern that includes an apparent dysfunction in ovarian steroidogenesis, although the sequence (or sequences) of endocrine changes, possibly with inherent paracrine abnormalities that can establish PCOS, are only postulated. Development of PCOS in the setting of androgen overproduction (or persistent disturbances of the hypothalamic-pituitary-adrenal axis even after normalization of androgen levels) points to adrenal enzyme defects, but the precise interaction of gonad and adrenal cortex via serum steroids remains open. Several interesting schemas for the development of PCOS have been proposed, but the subject remains controversial. Sonographic abnormalities of the ovaries may be similar in women with PCOS and women with adrenal enzyme defects, and differentiation of PCOS from nonclassical 21-hydroxylase deficiency on the basis of pituitary gonadotropin response to LHRH is also nonspecific. The 17-OHP response to ACTH testing is necessary to make the diagnosis (see nomogram). The preliminary data on the subgroup of women with PCOS and nonclassical 21-hydroxylase deficiency indicate that (1) the size of cystic changes of ovaries on sonography and (2) LH: FSH ratios may improve with dexamethasone treatment. Identification of an adrenal enzymatic disorder by ACTH testing is cost-effective in patients with symptoms of androgen excess, offers a good prognosis for improved fertility in couples, and as well is informative in family genetic studies.