menotropins and Thalassemia

The pituitary-gonadal function was studied in 18 beta-thalassemic female adolescents, 8 with delayed puberty and 10 with primary amenorrhea,treated with repeated transfusions and long-term iron chelation therapy by subcutaneous infusion. A 100 micrograms gonadotropin-releasing hormone (GnRH) test, a double-bolus GnRH test after estradiol administration in non-responders, a 400 micrograms thyrotropin-releasing hormone (TRH) test and a 'high dose' human menopausal gonadotropin (hMG) test were performed. LH and FSH peak levels were significantly lower in thalassemic patients than in controls, both in the 100 micrograms GnRH test (LH was 4.3 +/- 0.7 mIU/ml vs 40.8 +/- 6.0 mIU/ml and FSH 3.3 +/- 0.5 mIU/ml vs 9.6 +/- 1.1 mIU/ml, respectively) and in the double-bolus GnRH test (LH was 2.3 +/- 0.2 mIU/ml vs 59.0 +/- 4.9 mIU/ml and FSH 1.8 +/- 0.3 mIU/ml vs 14.0 +/- 1.0 mIU/ml). The mean prolactin response to the TRH test was 27.8 +/- 3.2 ng/ml. After the 'high dose' hMG test 12 out of 14 admitted patients showed a poor response, lower than 250 pg/ml. Our data suggest that the hypogonadotropic condition of the thalassemic adolescents is due to pituitary hyporesponsiveness to GnRH and that most of these patients also have an impairment of ovarian function. Both conditions are a consequence of iron deposits in glands. Moreover, there is evidence that pituitary-gonadal function cannot be preserved by long-term iron chelation therapy.

Topics: Adolescent; Chelation Therapy; Child; Deferoxamine; Dose-Response Relationship, Drug; Estradiol; Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Humans; Hypogonadism; Iron; Luteinizing Hormone; Menotropins; Ovary; Pituitary Gland; Prolactin; Thalassemia; Thyrotropin-Releasing Hormone

Endocrine studies were made on 23 female patients aged 13 to 29 years, with delayed puberty or primary amenorrhoea and beta thalassaemia major, and 12 healthy controls, of whom six were prepubertal and six were in Tanner's stage 3-4. Each patient and control received a single intravenous dose of 100 micrograms gonadotrophin releasing hormone (GnRH), and one week later, 10 U/kg body weight of human menopausal gonadotrophin (hMG) to stimulate ovarian function. The patients had decreased gonadotrophin reserves when compared with those of normal controls, only one of 23 patients had an intact luteinising hormone and follicle stimulating hormone response. Most of the thalassaemic patients with delayed puberty showed normal gonad response to human menopausal gonadotrophin (hMG), but three had very low responses, when compared with that of controls. The gonadal failure was even more severe in four of six patients with primary amenorrhoea. It is important to assess hypothalamic-pituitary-gonadal function in young women with beta thalassaemia major, so that those with glandular dysfunction may be started on replacement therapy.

Topics: Adolescent; Adult; Amenorrhea; Estradiol; Female; Follicle Stimulating Hormone; Humans; Luteinizing Hormone; Menotropins; Ovary; Pituitary Hormone-Releasing Hormones; Puberty, Delayed; Stimulation, Chemical; Thalassemia

Eight thalassemic patients, aged 24-35 years, who developed amenorrhea 2-15 years after menarche, were studied. Mean basal serum LH and FSH levels and the peak levels after gonadotropin-releasing hormone were significantly less than corresponding values in normal controls. All patients showed low basal serum levels of estradiol and six had a poor or absent response to human menopausal gonadotropin. One subject had intact pituitary-gonadal function and one patient had an impaired LH and FSH response to gonadotropin-releasing hormone in the presence of a significant increase of estradiol after human menopausal gonadotropin stimulation. The findings regarding pituitary hormones other than gonadotropins suggest that iron overload damages tropic cells unequally and inconsistently. We conclude that both pituitary and gonadal damage may be responsible for the secondary amenorrhea in thalassemic patients.

Topics: Adult; Amenorrhea; Female; Humans; Hypothalamo-Hypophyseal System; Iron; Menotropins; Ovary; Pituitary Function Tests; Pituitary Hormone-Releasing Hormones; Thalassemia; Thyroid Function Tests; Ultrasonography; Uterus

In an attempt to induce or to augment pubertal development and to achieve spermatogenesis, 10 gonadotropin-deficient thalassemic patients 15 to 23 years of age (mean 18.9 years) were treated with exogenous gonadotropins for 1 to 4 years (mean 2.1 years). Seven patients produced sperm during human chorionic gonadotropin (hCG) treatment given for 6 to 14 months. However, full spermatogenesis was achieved only when human menopausal gonadotropin was added to hCG regimen. In one patient, despite cessation of gonadotropin treatment, sexual potency, libido, and spermatogenetic capacity were maintained during the past 2 1/2 years. Our study indicates that it is possible to induce or to restore spermatogenesis in the majority of thalassemic patients and that gonadotrope cells may not be irreversibly damaged by iron deposition.

Topics: Adolescent; Adult; Chorionic Gonadotropin; Humans; Male; Menotropins; Spermatogenesis; Testosterone; Thalassemia