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尽管卵泡内17β-雌二醇水平极低，但17α-羟化酶缺乏症患者仍可能进行体外受精和初级胚胎分裂。

In vitro fertilization and primary embryonic cleavage are possible in 17 alpha-hydroxylase deficiency despite extremely low intrafollicular 17 beta-estradiol.

作者信息

Rabinovici J, Blankstein J, Goldman B, Rudak E, Dor Y, Pariente C, Geier A, Lunenfeld B, Mashiach S

机构信息

Department of Obstetrics and Gynecology, Chaim Sheba Medical Center, Tel-Hashomer, Tel-Aviv, Israel.

出版信息

J Clin Endocrinol Metab. 1989 Mar;68(3):693-7. doi: 10.1210/jcem-68-3-693.

DOI:10.1210/jcem-68-3-693
PMID:2493041
Abstract

Congenital adrenal hyperplasia due to 17 alpha-hydroxylase deficiency in genotypic females is characterized by primary amenorrhea and the absence of sexual maturation due to inadequate biosynthesis of ovarian androgens and estrogens. We induced ovarian follicular development in a woman with this syndrome. Ovum pick-up, in vitro fertilization, and primary embryonic development were achieved despite undetectable plasma estradiol and extremely low ovarian androgen concentrations and minute concentrations of these hormones in the ovarian follicular fluid.

摘要

基因型女性因17α-羟化酶缺乏所致的先天性肾上腺皮质增生症，其特征为原发性闭经，且由于卵巢雄激素和雌激素生物合成不足而缺乏性成熟。我们诱导了一名患有该综合征的女性的卵泡发育。尽管血浆雌二醇检测不到，卵巢雄激素浓度极低，且卵泡液中这些激素的浓度微乎其微，但仍实现了取卵、体外受精和早期胚胎发育。

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