menotropins and Disorders-of-Sex-Development

Despite the efforts of a large number of investigators, the role of GnRH in clinical gynecology is uncertain. At present, its greatest utility is in research directed toward the understanding of hypothalamic-pituitary interrelationships. However, a clear understanding of the hypothalamic control of gonadotropin secretion awaits the actual measurement of the secretion of GnRH by the hypothalamus. In addition, a better understanding of the ability of the pituitary to secrete gonadotropins in various disorders of menstruation and maturation will probably be achieved through the determination of the capacity of the pituitary to synthesize as well as release gonadotropins in response to GnRH. Such determinations will probably utilize repeated or continuous infusions of GnRH rather than the currently more popular single injection technique. Finally, GnRH may be useful in the induction of ovulation. A definition of its role in ovulation induction awaits the results of additional clinical studies. Understanding of the nature of hypothalamic control of the pituitary is as yet incomplete. The availability of hypothalamic releasing factors will make it possible to study in greater detail the mechanisms by which the fine regulation of the endocrine system is achieved.

Topics: Adenoma; Amenorrhea; Clomiphene; Cushing Syndrome; Diabetes Mellitus; Disorders of Sex Development; Female; Galactorrhea; Humans; Hypogonadism; Hypothalamo-Hypophyseal System; Menotropins; Menstruation; Myotonic Dystrophy; Ovulation; Pituitary Diseases; Pituitary Neoplasms; Polycystic Ovary Syndrome; Postpartum Period; Pregnancy

In true hermaphroditism diverse phenotypes and karyotypes are found; there are no distinctive laboratory features that can distinguish it from other intersex disorders, thus the diagnosis is made by the histological findings. Existence of Leydig cells is demonstrated by testosterone levels above the female range; however, presence of ovarian tissue cannot be ascertained because of the absence of a reliable functional test. Unless appropriate biopsies are performed or the whole gonad is removed, there is a risk of not diagnosing true hermaphroditism. To find a reliable test that can differentiate patients with true hermaphroditism from those with other intersex disorders, we investigated the estradiol (E2) response to human menopausal gonadotropins (hMG) in infants with genital ambiguity. These results were correlated with the histological findings. Eleven infants with genital ambiguity and four with a high scrotal testis were stimulated every 12 h with 2 IU/kg hMG. If E2 rose above 80 pg/mL (cut-off point), the test was discontinued; if after 7 days E2 remained below 80 pg/mL, the hMG dose was doubled and stimulation extended for 7 additional days. In five patients in whom true hermaphroditism was later histologically demonstrated, E2 rose above 80 pg/mL. In two of them, ovarian tissue was removed and hMG stimulation repeated; no response above our cut-off point was observed during the second test. The maximal E2 response to hMG in the remaining 10 individuals was 43 pg/mL; after laparotomy or gonadal biopsies no ovarian tissue was found. The hMG stimulation test can be considered a reliable and safe dynamic procedure for demonstrating the presence or absence of ovarian tissue in infants with genital ambiguity.

Topics: Adolescent; Child; Child, Preschool; Disorders of Sex Development; Estradiol; Female; Follicle Stimulating Hormone; Humans; Infant; Karyotyping; Luteinizing Hormone; Male; Menotropins; Osmolar Concentration; Ovary; Testis

Three previously unreported cases of true hermaphroditism are described and discussed with regard to their gonadal function and the value of endoscopic examination. All three patients showed a good response of serum testosterone to stimulation with human chorionic gonadotropin before gonadal biopsy. The third patient showed significant elevation of serum oestradiol level on stimulation with human menopausal gonadotropin at two years of age before operation. Endoscopic examination to search for cervix uteri at an early stage of diagnosis, and pre- and postoperative functional evaluation of the gonads by hormonal stimulation are emphasized.

Topics: Cervix Uteri; Chorionic Gonadotropin; Disorders of Sex Development; Estradiol; Female; Gonads; Humans; Infant; Menotropins; Testosterone