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迟发性3β-羟类固醇脱氢酶缺乏症:患病率、临床表现及内分泌模式

Late-onset 3 beta-ol-hydroxysteroid-dehydrogenase deficiency: prevalence, clinical presentation and endocrine pattern.

作者信息

Carmina E, Rosato F, Caputo A, Janní A

出版信息

Acta Eur Fertil. 1985 Nov-Dec;16(6):427-30.

PMID:3008481
Abstract

3 patients with late-onset 3 beta-HSD deficiency are described. They presented increased DHEAs/delta 4 ratio either in basal conditions or after ACTH stimulation. All patients were hirsute but only one had increased serum values of delta 4 and total testosterone. However, all patients presented increased serum levels of free testosterone. Two patients had normal menstrual cycles and in one case a secondary PCO was found.

摘要

本文描述了3例迟发性3β-羟类固醇脱氢酶(3β-HSD)缺乏症患者。他们在基础状态或促肾上腺皮质激素(ACTH)刺激后,脱氢表雄酮硫酸盐(DHEAs)/Δ4比值升高。所有患者均多毛,但只有1例患者的血清Δ4和总睾酮值升高。然而,所有患者的血清游离睾酮水平均升高。2例患者月经周期正常,1例发现继发性多囊卵巢。

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1
Late-onset 3 beta-ol-hydroxysteroid-dehydrogenase deficiency: prevalence, clinical presentation and endocrine pattern.迟发性3β-羟类固醇脱氢酶缺乏症:患病率、临床表现及内分泌模式
Acta Eur Fertil. 1985 Nov-Dec;16(6):427-30.
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Evidence for reduced 3 beta-ol-hydroxysteroid dehydrogenase activity in some hirsute women thought to have polycystic ovary syndrome.在一些被认为患有多囊卵巢综合征的多毛女性中,存在3β-羟类固醇脱氢酶活性降低的证据。
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