Spontaneous feminization and menstrual function developing during puberty in Turner's syndrome.
An 18-year-old patient with Turner's syndrome presented with cyclical vaginal bleeding and spontaneous development of secondary sexual characteristics. She demonstrated classic features of Turner's phenotype, and a culture of blood lymphocytes revealed a 45,XO karyotype. The patient's plasma and urinary estrogen concentrations were similar to those in normal adult women in the late proliferative phase. In contrast, luteinizing hormone (LH) and follicle-stimulating hormone (FSH) levels were elevated to values seen in postmenopausal women. Dynamic testing revealed no estrogen response to human menopausal gonadotropin (/MG) and a paradoxical fall in estrogen after administration of human chorionic gonadotropin (hCG). Administration of luteinizing hormone-releasing hormone (LH-RH) resulted in an exaggerated response of both gonadotropins. Dilatation and curettage revealed endometrial hyperplasia, and a laparotomy revealed the presence of 2 gonadlike structures. The histologic diagnosis was lutein cyst. Karyotyping of lymphocytes, skin from the abdominal incision, and tissue from both gonadal structures revealed a 45,XO karyotype without evidence of mosaicism.
D000293:Adolescent; D006063:Chorionic Gonadotropin / Q000494:pharmacology; D004967:Estrogens / Q000096:biosynthesis / Q000378:metabolism; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D007987:Gonadotropin-Releasing Hormone / Q000494:pharmacology; D006801:Humans; D007621:Karyotyping; D007986:Luteinizing Hormone / Q000097:blood; D008596:Menotropins / Q000494:pharmacology; D008598:Menstruation; D011627:Puberty; D012727:Sex Characteristics; D014424:Turner Syndrome / Q000378:metabolism / Q000503:physiopathology* / Q000601:surgery
D006063:Chorionic Gonadotropin; D004967:Estrogens; D007987:Gonadotropin-Releasing Hormone; D008596:Menotropins; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone