menotropins and Turner-Syndrome

Premature ovarian failure (POF) is a primary ovarian defect characterized by absent menarche or premature depletion of ovarian follicles before the age of 40 years. However, in several instances the distinction between definitive or intermittent POF may be difficult on clinical bases, therefore the more appropriate term Primary Ovarian Insufficiency (POI) has been recently proposed and will be used in this review. POI is a heterogeneous disorder affecting approximately 1% of women <40 years. The most severe forms present with absent pubertal development and primary amenorrhea, whereas forms with post-pubertal onset are characterized by disappearance of menstrual cycles (secondary amenorrhea) associated with a defective folliculogenesis. POI is generally characterized by low levels of gonadal hormones (estrogens and inhibins) and high levels of gonadotropins (LH and FSH) (hypergonadotropic amenorrhea). Heterogeneity of POI is reflected by the variety of possible causes, including autoimmunity, toxics, drugs, as well as genetic defects. Several data indicate that POI has a strong genetic component. In this manuscript we discuss the X chromosome abnormalities that are associated with POI.

Topics: Age of Onset; Amenorrhea; Autoimmunity; Bone Morphogenetic Protein 15; Chromosomes, Human, X; Estrogens; Female; Fragile X Mental Retardation Protein; Humans; Menotropins; Oophoritis; Ovary; Primary Ovarian Insufficiency; Sex Chromosome Aberrations; Turner Syndrome

Turner syndrome (TS) is associated with hypergonadotropic hypogonadism due to gonadal dysgenesis, which results in premature ovarian failure and subsequent infertility. Therefore, counseling and evaluation for fertility preservation are required as early as possible for women with TS.. A 23-year-old unmarried woman with mosaic TS (45, X [4/30] 46, XX [26/30]) presented to the pediatric department of our hospital for fertility counseling; she was accompanied by her mother. She was referred to the reproduction center of our hospital for ovarian reserve assessment and counseling regarding fertility preservation. We decided to retrieve oocytes using DuoStim as the controlled ovarian stimulation protocol. During the first and second oocyte retrievals, a total of 17 (9 and 8, respectively) mature metaphase II oocytes were cryopreserved.. DuoStim may be a useful option for fertility preservation for women with TS and reduced ovarian reserve. This new strategy may obtain the required number of oocytes in the shortest time and preserve the future fertility of women with TS.

Topics: 17 alpha-Hydroxyprogesterone Caproate; Buserelin; Cryopreservation; Dydrogesterone; Female; Fertility Agents, Female; Fertility Preservation; Gonadotropin-Releasing Hormone; Humans; Infertility, Female; Menotropins; Menstruation Disturbances; Mosaicism; Oocyte Retrieval; Ovarian Reserve; Ovulation Induction; Primary Ovarian Insufficiency; Turner Syndrome; Young Adult

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.

Topics: Adolescent; Chorionic Gonadotropin; Estrogens; Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Humans; Karyotyping; Luteinizing Hormone; Menotropins; Menstruation; Puberty; Sex Characteristics; Turner Syndrome

Correlation between ovarian follicular apparatus and hormonal parameters such as serum gonadotropin and urinary estrogen levels was investigated in patients with primary and secondary amenorrhea. Serum gonadotropin levels were elevated in amenorrheic patients without ovarian follicles or with follicles of low developmental stage and pituitary responsiveness to LH-RH in these patients were marked compared with patients with follicles of high developmental stage or normal ovulating women in the follicular phase of the menstrual cycle. The 24-hour urinary excretion of total estrogens was low in patients without follicles or with follicles of low developmental stage and ovarian responsiveness to exogenous gonadotropins was quite low in comparison with patients with highly developed follicles or normal control subjects. Thus, serum gonadotropin and urinary estrogen measurements and LH-RH and gonadotropin loading tests are diagnostic of the presence or absence and the state of development of ovarian follicles in the diagnosis and treatment of primary and secondary amenorrhea.

Topics: Adult; Amenorrhea; Chorionic Gonadotropin; Estrogens; Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Humans; Luteinizing Hormone; Menotropins; Ovarian Follicle; Ovary; Pituitary Gland, Anterior; Turner Syndrome