[某些形式多毛症中21-羟化酶部分缺乏]

[Partial deficiency in 21-hydroxylase in certain forms of hirsutism].

作者信息

Lucisano A, Satta M A, Tripodi R, Mobili L, De Cicco F, Monaco F, Dell'Acqua S, Roche J

机构信息

Istituto di Clinica Ostetrica e Ginecologia U. C. S. C., Roma.

出版信息

C R Seances Soc Biol Fil. 1989;183(2):117-21.

PMID:2555033

Abstract

The ACTH test is important when hirsutism occurs in women with a slight 21-hydroxylase deficiency, and normal basal 17-OH Progesterone (17-OH-P/plasma levels). Extensive hormonal assays: LH, FSH, Prolactin, 17 beta-estradiol (E2), Estrone, 17OH-P, Androstenedione, Testosterone, Cortisol (C), Dehydroepiandrosterone-S (DEA-S) were carried out in 36 hirsute women. 13 of these presented hormone levels as found in polycystic ovary syndrome (PCOS), 6 women presented a slight 21-hydroxylase deficiency (increased plasma 17-OH-P and decreased C after ACTH test with significant, p less than 0.01, increase of 17-OH-P/C and 17 women presented idiopathic hirsutism (IH). The hormonal pattern, in the basal condition, is not different in IH or in slight 21-hydroxylase deficiency. The ACTH test is able to differentiate between IH and adrenal hirsutism.

摘要

当患有轻度21-羟化酶缺乏且基础17-羟孕酮（17-OH-P/血浆水平）正常的女性出现多毛症时，促肾上腺皮质激素（ACTH）试验很重要。对36名多毛女性进行了广泛的激素检测：促黄体生成素（LH）、促卵泡生成素（FSH）、催乳素、17β-雌二醇（E2）、雌酮、17-OH-P、雄烯二酮、睾酮、皮质醇（C）、硫酸脱氢表雄酮（DEA-S）。其中13名患者的激素水平符合多囊卵巢综合征（PCOS）的表现，6名女性存在轻度21-羟化酶缺乏（ACTH试验后血浆17-OH-P升高，C降低，17-OH-P/C显著升高，p<0.01），17名女性表现为特发性多毛症（IH）。在基础状态下，IH或轻度21-羟化酶缺乏患者的激素模式并无差异。ACTH试验能够区分IH和肾上腺性多毛症。