Ovarian fibromata in four successive generations.
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Adolescent↗
Biomedical subjects
H S Safaii
Publications and source records attributed to H S Safaii.
Mixed gonadal dysgenesis without virilization.
A 19-year-old phenotypic female with primary amenorrhea and 45X,0/46X,Y chromosomal mosaicism was found to have asymmetrical gonadal dysgenesis. Her lack of virilization precludes her precise categorization in the current nomenclature of gonadal dysgenesis and she is considered a unique variant of the syndrome of mixed gonadal dysgenesis.
Adult↗
Testicular response to exogenous gonadotropins in the syndrome of feminizing testes.
The steroid response of testes to exogenous gonadotropin administration was studied before castration in a patient with the complete form of the testicular feminization syndrome. Urinary steroid determination were made along with measurements of plasma testosterone during adrenal suppression with dexamethasone and after stimulation with human menopausal gonadotropin (HMG) and HMG plus human chorionic gonadotropin (HCG). Our data provide evidence that the gonads in the testicular feminization syndrome synthesize steroids normally. Estrogens and testosterone are secreted by the testes, and the Leydig cells of the testes are capable of responding to exogenous HMG and HCG stimulation. These findins are consistent with the hypothesis that there is no abnormal testicular steroidogenesis in these genotypic males. Additional evidence is provided that these gonads secrete testosterone in amounts comparable to those of normally functioning testes. Failure of virilization in the presence of androgen secretion as indicated by previous authors is probably the result of a deficiency of androgenic action at the end organs. The presence of sparse pubic and axillary hair in the mother and the finding of the same type of karyotype (46,XY) in a sibling suggest that hereditary factors play a role in this disorder.
17-Ketosteroids↗
Immediate effect of prostaglandin F2alpha during the luteal phase of the menstrual cycle.
Serum luteinizing hormone, estradiol, 17-hydroxyprogesterone, and progesterone determinations were performed in five normally ovulating women prior to, during, and after prostaglandin F2alpha (PGF2alpha was infusion. A total of 75 mg of PGF2alpha was infused over 48 hours, beginning on the 4th day of the luteal phase. Endometrial biopsies were obtained at the completion of the infusion. In all patients, transient decreases in serum progesterone and 17-hydroxyprogesterone levels were observed during PGF2alpha infusion. Subsequent rises in the serum progesterone and 17-hydroxyprogesterone levels were observed 24 hours after completion of the infusion. There was no significant change in serum luteinizing hormone levels. Premature menstrual bleeding occurred with 24 to 72 hours after the termination of the infusion, while progesterone and 17-hydroxyprogesterone levels were still elevated. Endometrial histology revealed focal stromal necrosis in the presence of the normal secretory endometrium. It is concluded that, in the human, PGF2alpha at this dose and time schedule is not luteolytic.
Adult↗