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-Hydroxylase Deficiency Despite Extremely Low Intrafollicular 17β-Estradiol
Departments of Obstetrics and Gynecology, Endocrinology, and Genetics, Chaim Sheba Medical Center TelHashomer
The Sackler School of Medicine, Tel-Aviv University Tel-Aviv, Israel
Address all correspondence and requests for reprints to: Jaron Rabinovici, M.D., Reproductive Endocrinology Center, Department of Obstetrics, Gynecology, and Reproductive Sciences, University of California San Francisco, HSE 1630, Box 0556, 505 Parnassus Avenue, San Francisco, California 94143.
Congenital adrenal hyperplasia due to 17
-hy-droxylase 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.
* Current address: Department of Obstetrics and Gynecology, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44195.
Received April 4, 1988.
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