FURTHER OBSERVATIONS ON THE DEFECTIVE SYNTHESIS OF OVARIAN STEROIDS IN THE STEIN-LEVENTHAL SYNDROME

Abstract

SUMMARY Steroid determinations were carried out in 2·3 ml. cyst fluid obtained from the ovaries of a woman with the Stein—Leventhal syndrome. The following concentrations were found (μg. steroid/100 ml. fluid): pregnenolone, < 2·0; progesterone, < 9·0; 17α-hydroxyprogesterone, 24·0; androstenedione, 139·0; 19-hydroxyandrostenedione, < 4·0; oestrone, < 2·0; oestradiol-17β, < 2·0; 17α-hydroxypregnenolene, < 2·0; dehydroepiandrosterone, 10·0; testosterone, < 4·0. These results therefore confirm and extend those obtained in a previous study (Short & London, 1961). They strongly suggest that in this syndrome there is a defective 19-hydroxylation of androstenedione, rather than either a defective 3β-ol dehydrogenase enzyme system or an increased ovarian production of testosterone as has been suggested by other workers. The possibility that such a defect might be caused by an X chromosome abnormality in some of the theca interna cells of the ovary is discussed.