No abstract

The sexual development and behavior of 37 women suffering from the gonadal dysgenesis syndrome were investigated. It was found that their sexual development was significantly retarded compared with a control group of 50 fertile patients of the same age. This retardation was greater in a subgroup of 19 women with the Turner syndrome than in a subgroup of 18 women with pure gonadal dysgenesis. The levels of gonadotrophins LH and FSH, estradiol, progesterone and testosterone in blood were similar in these two subgroups. In two-thirds of the gonadal dysgenesis syndrome patients, the estradiol level was mostly at the low end of normal. The assessment of their sexual life using the Sexual Activity of Women (SAW) and Sexual Function of Women (SFW) questionnaires indicated a lower sexual desire, a reduced orgastic capacity, and a weaker sexual activity in the pathological group. However, 24 patients living in a permanent and stable partner relationship at the time of the examination, did not significantly differ from the control group in their responses to the SFW questionnaire. The patients with gonadal dysgenesis syndrome were surprisingly well adapted to their shortcomings. The occurrence and intensity of most neurotic symptoms assessed by the N5 Questionnaire were scored lower by the patients with gonadal dysgenesis syndrome than by the control group.

SUMMARYDuring all developmental stages of female puberty (P i -P j ) plasma LH and FSH concentration determined by radioimmunoassay decreased after a single i.m. injection of 50 ygfkg estradiol-dipropionate in oil. This decrease coincidenced with the peaking level of circulating estradiol (measured by radioimmunoassay).Only in the group of girls in an advanced stage of puberty (premenarche P 4 ) this initial nadir was followed by a secondary increase in plasma LH concentration but not in that of FSH. This secondary increase occurred during the period of decreasing level of circulating estradiol. However, only in two of the five girls at this advanced stage of puberty (P 4 ) the maximum of plasma LH approached the value of the preovulatory plasma LH peak in adult women. These results suggest that the potential for a stimulatory (positive) estrogen feedback response appears after midpuberty at the stage P 4 .It has been demonstrated in rats that the mechanism of the stimulatory (positive) feedback action of estrogen on gonadotropin release becomes operative at a low level of sensitivity long before estrarche. The concentration of circulating endogenous estrogen up to that time seems insufhcient to trigger the release (PnESi, ! al., 1968, i97z ; CALIGARIS et al., 1 972 ). In contrast, in female rhesus monkeys estrogen induced gonadotropin surges were not demonstrable until some months after menarche (DIERSCHKE et al., 1974 ). In the present study, serial determinations of plasma LH and FSH levels have been made in healthy prepubertal and pubertal girls to discover the stage of female sexual development at which the stimulatory estrogen feedback mechanism attains a level of sensitivity at which an acute increase in circulating estrogen concentration induced by a single administration of steroid is able to cause the characteristic gonadotropin release.

The sexual development and sex life of 12 women with the Küstner-Rokitansky syndrome, in whom the developmental anomaly (vaginal agenesis) was corrected through surgery, were examined through structured interviews and by means of questionnaires. The comparison group was 22 women who had regular menses and a functional sex life. All differences between the groups were statistically nonsignificant. Only in one questionnaire was there a slight trend toward retarded heterosexual development in the patient sample.

No abstract