[Rokitansky-Kuster-Hauser syndrome: presentation of 14 cases].
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Biomedical subjects
Publications and source records attributed to L Falsetti.
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The Authors propose a new direct method for the determination of free Testosterone (F.Te). Our study was made following this method: 1) on a control group composed of 12 healthy men and 21 healthy women, and 2) on a group of 29 patients suffering from Polycystic Ovary Syndrome (PCOS) with clinical signs of hyperandrogenism. This pathological group presented acne and hirsutism in 95% of the cases. The Authors demonstrate how the determination of F.Te permits a 93.1% correct endocrinological diagnosis of hyperandrogenism.
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In this study the Authors have obtained an ovulatory response using intravenous Gn-RH treatment in four patients with hypothalamic hypogonadotropic amenorrhea. The Gn-RH was administered in a pulsatile manner with a small portable autoinfusion pump (Autosyringe, Inc., Hooksett). The ovarian follicular maturation, as it appears from the pelvic echography, was perfectly similar to that observed in a spontaneous ovulatory cycle. Endocrine picture by serial determination of gonadotropin; prolactin, estradiol and progesterone concentrations showed hormone values to be in the normal range. During the therapy no complications were observed; at the present time no pregnancy has occurred.
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Twenty-five women presenting moderate-severe PCOS-dependant hirsuitism were treated for 6 months with GnRH-A (Group A) or pill-combined and GnRH (Group B). Both therapeutic regimens significantly decreased androgen plasma levels. Hair diameter reduced in Group A by 22-34% and in Group B by 25-35%, while hirsuitism score respectively decreased by 22 and 24%. Clinical results, overlapping in the two groups, prove that GnRH-A are active in hirsuitism. Side effects, however, especially a decreased bone density (3.9%) in Group A make it necessary an association with oral contraceptive.
A hundred and twenty healthy women who did not desire pregnancy were selected to check the effects of triphasic oral contraceptives on endocrine parameters and on plasma levels of steroid-binding proteins. Three groups of 40 women each were treated with 3 different pills containing different doses of ethynilestradiol in combination with noretistherone or levonorgestrel or gestodene, for a 6-month period. Serum concentrations of pituitary, ovarian and adrenal hormones, sex-hormone binding globulin and corticosteroid binding globulin were measured, basally and after the 6th cycle. Triiodothyronine and thyroxine were also tested. After 6 cycles the three oral contraceptives determined the inhibition of gonadotropins, ovarian steroids and Dehydroepiandrosterone Sulphate as well as an increase in Cortisol, triiodothyronine, thyroxine, sex hormone binding globulin and corticosteroid binding globulin. Prolactin levels did not vary. In the groups that receive the pills containing noretistherone, levonorgestrel and gestodene, sex hormone binding globulin increased by 138-136 and 156 per cent respectively, while corticosteroid binding globulin increased by 89-75 and 82 per cent respectively. The higher increase in sex hormone binding globulin, caused by gestodene-containing pill, testifies to the selectivity of this progestogen towards a lower androgenicity than norethisterone and levonorgestrel.
We present a case of microprolactinoma (3 mm) grown into macroprolactinoma (21 mm) during pregnancy. This patient was admitted in our hospital for left hemianopsia 13 days after delivery. Through Magnetic Resonance Imaging (MRI) a macroprolactinoma (21 mm in diameter) was diagnosed. The Prolactin (PRL) serum levels were 152 ng/ml. The patient started Cabergoline treatment with good results: normalization of PRL levels after 5 days, of the visual field after 58 days and complete disappearance of prolactinoma within 180 days from the beginning of treatment.
The article reports on a case of functioning adrenal carcinoma in a woman of 40 with concomitant in situ carcinoma of the portio (CIN 3). She had had amenorrhoea for 14 months and early menopause was suspected. Hirsutism and other signs of virilization such as clitoromegaly and voice changes were detected upon hospitalization.. The characteristic of the clinical case is given by hormone production involving not only androgens of prevalently adrenal genesis (dehydroepiandrosterone, dehydroepiandrosterone sulphate and androstenedione), but also testosterone, more suggestive of a gonadal neoplasm. The adrenal carcinoma only produced androgens, as plasma cortisol and aldosterone concentrations were normal. Diagnosis of adrenal carcinoma was first made with hormone assays and then with the adrenal block test using dexamethasone, with adrenal ecography and abdomino-pelvic computed tomography (CT). When amenorrhoea is present and hirsutism and other signs of virilization occur ex-novo in a short period, in a woman of fertile age, a thorough endocrine study must be carried out. According to the hormone profile, the next diagnostic step should be aimed at detecting a prospective ovarian or adrenal neoplasm.