Vokaer R
Clinique Obstétricale et Gynécologique, Université Libre, Bruxelles, Belgique.
Rev Fr Gynecol Obstet. 1973 Apr;68(4):221-9.
The individual features of the Stein-Leventhal syndrome has been submitted to a step-by-step review by the author. After a critical study of its clinical manifestations, the author insisted on the variously hypothetized pathways of biosynthesis of ovarian steroids, commenting each one of them and drawing diagrams of special clarity. The alterations that can possibly occur in Stein-Leventhal syndrome: aromatization deficiency, partial enzymatic inhibition due to 3 beta ol dehydrogenase deficiency, have been investigated. To follow, the author discussed: the mechanism of adrenocortical interference, the part played by the pituitary gland and hypothalamus. In the author's opinion, four etiological types of the "micropolycystic ovarian syndrome" can be distinguished, according as the hypothalamic origin is a primary one or secondary to hereditary hypercorticism, and according as the biosynthesis inhibition takes place at the aromatization level or at the levels at which 3 beta ol dehydrogenase activity exerts itself. Interesting therapeutical and prognostic conclusions can be drawn from these suggestions.
作者已对斯坦因-莱文塔尔综合征的个体特征进行了逐步回顾。在对其临床表现进行批判性研究之后，作者着重探讨了卵巢甾体生物合成的各种假设途径，对每条途径进行了评论并绘制了特别清晰的示意图。研究了斯坦因-莱文塔尔综合征可能出现的改变：芳香化酶缺乏、因3β-醇脱氢酶缺乏导致的部分酶抑制。接下来，作者讨论了：肾上腺皮质干扰的机制、垂体和下丘脑所起的作用。作者认为，根据下丘脑起源是原发性的还是继发于遗传性皮质醇增多症，以及生物合成抑制是发生在芳香化水平还是3β-醇脱氢酶发挥作用的水平，可以区分出“微多囊卵巢综合征”的四种病因类型。从这些观点中可以得出有趣的治疗和预后结论。
