Arrest of follicular development in a patient with 17 alpha-hydroxylase deficiency: folliculogenesis in association with a lack of estrogen synthesis in the ovaries.
The degree of follicular development was examined in a patient with 17 alpha-hydroxylase deficiency that accounted for impairment of estrogen and androgen biosynthesis. The ovarian content of P was markedly higher than those of any other steroids requiring 17 alpha-hydroxylation for synthesis. The morphologic analysis of the ovaries demonstrated that normal follicles could not develop to more than 2.2 mm in diameter, and most follicles with diameters of 1.0 mm or more yielded to atresia. It is known that estrogen and FSH act synergistically on the growth of the follicles. Our data suggest that the follicles can develop up to the size of 2.2 mm in diameter at most with the sole stimulation of gonadotropin.
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital; D004967:Estrogens / Q000096:biosynthesis / Q000502:physiology*; D005260:Female; D006801:Humans; D006080:Ovarian Follicle / Q000503:physiopathology*; D010053:Ovary / Q000378:metabolism*; D013250:Steroid Hydroxylases / Q000172:deficiency*
D004967:Estrogens; D013250:Steroid Hydroxylases