1/19. Ovarian hyperthecosis in the adolescent patient.The endocrine findings in two adolescents with hyperthecosis are compared to those in a patient with an androgenic ovarian tumor. In patients with hyperthecosis, luteinizing hormone values were elevated or in the upper normal range, and plasma testosterone and androstenedione values were increased. Following dexamethasone suppression, testosterone and androstenedione values remained elevated, but after administration of human chorionic gonadotropin, they increased further in only one patient. Baseline 17-ketosteroid values were normal, suppressed with dexamethasone, and stimulated to baseline levels following HCG. The patient with a lipoid cell ovarian tumor had low baseline LH levels, and elevated testosterone, androstenedione, and 17-ketosteroid values. dexamethasone produced little change in urinary or plasma values, but the 17-ketosteroids increased markedly after administration of HCG. The finding of low serum LH values in patients with hirsutism and elevated androgen secretion should alert the clinician to the possibility of a tumor.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
2/19. food-dependent androgen and cortisol secretion by a gastric inhibitory polypeptide-receptor expressive adrenocortical adenoma leading to hirsutism and subclinical Cushing's syndrome: in vivo and in vitro studies.Aberrant gastric inhibitory polypeptide (GIP) receptor expression in bilaterally hyperplastic adrenals or unilateral adrenal adenomas is a rare form of adrenal hyperfunction. So far, only few cases have been described. In all these cases, cortisol was the predominant steroid released in a food-dependent manner, leading to the development of non-ACTH-dependent Cushing's syndrome. In the present study, we describe a novel case of a GIP receptor-expressive adrenocortical adenomatous nodule, detected incidentally by computed tomography scanning in a 41-yr-old lady with hirsutism but no clinical signs of Cushing's syndrome, on physical examination. Hormonal investigations in morning fasting samples showed slightly elevated androgen levels, low-normal baseline cortisol, normal suppression of cortisol after dexamethasone administration, and ACTH levels that were not suppressed and did stimulate after CRH administration. The elevated urinary free cortisol excretion, in conjunction with an atypical cortisol diurnal rhythm, raised the possibility of an aberrant stimulation of cortisol production by the adrenal tumor. Further studies demonstrated food-dependent secretion of cortisol, which was abolished by prior octreotide administration. Notably, substantial amounts of adrenal androgens were also secreted after food consumption. Removal of the tumor resulted in undetectable cortisol and androgen levels that did not respond to food consumption. Histological examination of the excised tumor revealed an adrenocortical adenomatous nodule originating from the inner zona reticularis, consisting mainly of compact cells. A steroidogenic secretory pattern, indicating the concomitant release of adrenal androgens and cortisol, was also observed in vitro from tumor cells cultured in the presence of GIP. The in vitro secretory response to GIP was higher for the adrenal androgen DHEA, compared with cortisol. The expression of the GIP receptor in tumor cells, but not in the adjacent normal adrenal, was demonstrated by RT-PCR), using specific oligonucleotide probes for this receptor. In summary, we describe a patient with a GIP-expressive cortisol and androgen oversecreting adrenocortical nodule with the unusual presentation of hirsutism and not the typical clinical signs of Cushing's syndrome. It is of note that food intake in this patient provoked a substantial increase in both adrenal androgen and cortisol levels that, together with the histological appearance of this nodule, was compatible with a zona reticularis-derived tumor. Thus, aberrant expression of the GIP receptor does not exclusively involve cells of a zona fasciculata phenotype, as previously reported, but may also occur in other types of differentiated adrenocortical cells.- - - - - - - - - - ranking = 1.5keywords = administration (Clic here for more details about this article) |
3/19. Testosterone-secreting gonadotropin-responsive adrenal adenoma and its treatment with the antiandrogen flutamide.A 55-year-old woman with virilization had an appreciably elevated testosterone level, which was not suppressed by dexamethasone, but was increased by stimulation with human chorionic gonadotropin (hCG). ultrasonography and computed tomography revealed an adenoma 2.5-3.0 cm in diameter in the right adrenal gland. The patient was treated with the antiandrogen flutamide in a daily dose of 500 mg for 4 months. A substantial regression of her hirsutism was observed during flutamide administration, but the serum testosterone level remained high. Right adrenalectomy was performed. Histologically, the tumor proved to be an adrenocortical adenoma of zona reticularis type. The adenoma tissue contained specific hCG receptors (187 fmol/g). The steroid concentration in the tumor tissue was examined by means of high pressure liquid chromatography-radioimmunoassay (HPLC-RIA). A significantly increased testosterone content was detected, and the levels of its precursors, androstenedione and dehydroepiandrosterone, were also elevated. Following adrenalectomy, serum testosterone concentration decreased to the normal level. The mechanism of the inappropriate regulation in the testosterone production of the adrenal tumor has not been fully elucidated.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
4/19. Case report: acromegaly and Cushing's disease in a patient with synchronous pituitary adenomas.A 40-year-old white woman presented with hirsutism, amenorrhea, generalized fatigue, diffuse weight gain, acral changes, and coarsened facial features. physical examination revealed mild diastolic hypertension, acromegalic features, hirsutism, and seborrhea. The growth hormone concentration was elevated and did not suppress after glucose administration. Urinary free cortisol excretion was increased and was not suppressed during a 2 mg low-dose dexamethasone suppression test. magnetic resonance imaging of the sella demonstrated a 1.3 x 1.2 x 0.8 cm pituitary adenoma. Trans-sphenoidal resection was performed, and portions of the resected tumor were analyzed by routine pathologic methods. Histopathologic and immunohistochemical findings indicated discrete growth hormone- and adrenocorticotropic hormone-producing pituitary adenomas. Coexisting acromegaly and Cushing's syndrome due to pituitary neoplasia was previously reported in two patients. However, to the authors' knowledge, this represents the first description of a patient with acromegaly and Cushing's disease resulting from discrete synchronous adenomas of the pituitary gland as defined by modern histopathologic techniques.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
5/19. Testosterone/epitestosterone ratio in urine: a possible diagnostic tool in the disclosure of exogenous testosterone administration.A 37-year-old woman presented with a history of secondary amenorrhoea and hirsutism for 4 years. She had elevated serum levels of testosterone and dihydrotestosterone, and decreased serum levels of sex hormone binding globulin and oestradiol. Almost daily use of a testosterone-containing ointment in the vulvar region for 6 years was disclosed as the cause of the hyperandrogenism. serum testosterone, testosterone excretion rate in urine and testosterone/epitestosterone ratio in urine were determined at fixed intervals 24 h before and 48 h after application of the testosterone-containing ointment. There was a rapid increase in serum testosterone, with a peak level after 4-6 h. The testosterone excretion rate and the testosterone/epitestosterone ratio in urine peaked after 2-4 h. After 48 h the serum testosterone level was still about twice the basal value. The testosterone/epitestosterone level was over the 'doping limit' of 6 for 28 h. We conclude that determination of the testosterone/epitestosterone ratio in urine would have disclosed exogenous testosterone administration in this patient. We recommend this test for patients in whom exogenous testosterone administration is suspected.- - - - - - - - - - ranking = 3keywords = administration (Clic here for more details about this article) |
6/19. Clinical course of genetic diseases of the insulin receptor (type A and Rabson-Mendenhall syndromes): a 30-year prospective.The interaction of insulin with its cell surface receptor is the first step in insulin action and the first identified target of insulin resistance. The insulin resistance in several syndromic forms of extreme insulin resistance has been shown to be caused by mutations in the receptor gene. We studied 8 female patients with the type A form of extreme insulin resistance and 3 patients (2 male and 1 female) with the Rabson-Mendenhall syndrome and followed the natural history of these patients for up to 30 years. The 11 patients ranged in age from 7 to 32 years at presentation. All 11 patients had extreme insulin resistance, acanthosis nigricans, and hyperandrogenism in the female patients, and all but 1 were of normal body weight. This phenotype strongly predicts mutations in the insulin receptor: of the 8 patients studied, 7 were found to have mutations. Similar results from the literature are found in other patients with type A and Rabson-Mendenhall syndromes and leprechaunism. The hyperandrogenic state resulting from hyperinsulinemia and insulin resistance in these patients was extreme: 6 of 8 patients had ovarian surgery to correct the polycystic ovarian syndrome and elevation of serum testosterone. By contrast, a larger group of insulin-resistant patients who were obese with hyperandrogenism, insulin resistance, and acanthosis nigricans (hair-AN syndrome) did not have a high probability of mutations in the insulin receptor. The morbidity and mortality of these patients were high: 3 of 11 died, 9 of 11 were diabetic and 1 had impaired glucose tolerance, and 7 of 9 patients had 1 or more severe complication of diabetes. Our literature review revealed that the mortality of leprechaunism is so high that the term leprechaunism should be restricted to infants or young children under 2 years of age. Analogous to patients with the common forms of type 2 diabetes, these patients had a heterogeneous course. In 2 patients who were able to maintain extremely high endogenous insulin production, the fasting blood glucose remained normal even though post-glucose-challenge levels were elevated. Most patients, however, required large doses of exogenous insulin to ameliorate the severe hyperglycemia. Preliminary results of a recent study suggest that recombinant leptin administration may benefit these patients with severe insulin resistance.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
7/19. diagnosis and resection of an oral contraceptive-suppressible sertoli-leydig cell tumor with preservation of fertility and a 7-year follow-up.A 21-year-old white woman presented with virilization, hirsutism, and acne of 1.5 years' duration. Endocrine testing demonstrated complete suppression of serum testosterone, from 5.3 to 0.6 ng/mL, and serum androstenedione, from 4.7 to 1.7 ng/mL, after oral administration of 50 micrograms of mestranol and 1 mg of norethindrone for 21 days. No suppression of either steroid was produced by dexamethasone, whereas serum dehydroepiandrosterone sulfate was suppressed from 5.2 to 1.9 micrograms/mL. A left salpingo-oophorectomy was performed for a 3 x 4-cm sertoli-leydig cell tumor of intermediate differentiation. Intraoperative studies demonstrated that the tumor secreted testosterone, androstenedione, 17 alpha-hydroxyprogesterone, and estradiol, but not dehydroepiandrosterone sulfate. These findings support the thesis that hormonal manipulation tests cannot differentiate between adrenal and ovarian virilizing tumors. Nor does the oral contraceptive suppression of testosterone secretion exclude an ovarian malignancy. The patient remains free of recurrence after 7 years.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
8/19. Virilizing brenner tumor of the ovary: case report.A case of ovarian brenner tumor displaying androgenic activity and clinical masculinization is reported in a 65-year-old woman. plasma androstenedione, dehydroepiandrosterone sulfate (DHEAS), testosterone, dihydrotestosterone, cortisol, estradiol, FSH, LH, and prolactin were measured before and after suppression with dexamethasone and stimulation with hCG, and again after removal of the tumor. The plasma androgenic compounds were measured in both ovarian and peripheral veins. Basal levels of androstenedione and of total testosterone were significantly elevated, decreasing to normal ranges after operation and remaining after 3 months. There was a sharp increase of total testosterone, DHEAS, and dihydrotestosterone levels after administration of hCG. The levels of androstenedione, DHEAS, testosterone, and dihydrotestosterone were higher in the ovarian vein than in the peripheral vein. These findings indicate that the tumor was the main source of these androgenic hormones.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
9/19. Normal ovarian function in a mild form of late-onset 3 beta-hydroxysteroid dehydrogenase deficiency.Late-onset 3 beta-hydroxysteroid dehydrogenase (HSD) deficiency was diagnosed in a 30-year-old woman with hirsutism and normal menstrual cycles. No genital abnormalities were present. Elevated basal serum levels of delta 5-3 beta-hydroxysteroids were demonstrated. serum pregnenolone (P5) was 3.0 ng/ml, and dehydroepiandrosterone sulphate (DHEA-S) 3245 ng/ml. Basal serum levels of delta 4 steroids were low or within normal limits. serum progesterone (P) was 0.5 ng/ml, 17 alpha-hydroxyprogesterone (17-OHP) 0.2 ng/ml, androstenedione (delta 4A) 0.4 ng/ml, and testosterone (T) 0.1 ng/ml. All delta 5/delta 4 steroid ratios were elevated. dexamethasone (DEX) administration normalized the elevated levels of delta 5-3 beta-hydroxysteroids, whereas delta 4-3-ketometabolites exhibited only minor modifications. The DHEA-S/delta 4A ratio increased more than five times over the basal ratio, and P5/P and 17 alpha-hydroxypregnenolone (17-OHP5)/17-OHP ratios did not increase after adrenocorticotrophic hormone ACTH stimulation. Studies of basal ovarian function revealed 17 beta-estradiol (E2) and gonadotropins within normal limits according to the menstrual cycle. In the follicular phase, follicle-stimulating hormone (FSH) was 101.3 ng/ml, luteinizing hormone (LH) 46.0 ng/ml, and E2 49.7 pg/ml; in the luteal phase, FSH was 180.0 ng/ml, LH 69.3 ng/ml, and E2 50.1 pg/ml. The presence of ovulatory cycles was documented on the basis of the biphasic pattern of the basal body temperature cycles and the increment in P levels. This case demonstrates the existence of normal ovulatory function in a woman with late-onset of a mild form of HSD.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
10/19. Mild adrenal 3 beta-hydroxysteroid dehydrogenase deficiency with hyperaldosteronism.The patient was admitted to our hospital at 19 and again at 22-yr of age for hirsutism and hypertension. Her baseline and ACTH-stimulated plasma 17-hydroxy pregnenolone, dehydroepiandrosterone and dehydroepiandrosterone sulfate were increased whereas plasma 17-hydroxy progesterone and androstenedione were normal and responded poorly to ACTH. plasma deoxycorticosterone, corticosterone and cortisol baseline levels were normal, and they responded normally to ACTH. The plasma aldosterone concentration (PAC) was always high and responded well to ACTH, angiotensin iii and furosemide-upright stimulation. However, plasma renin activity (PRA) was normal or slightly high, and responded normally to furosemide-upright stimulation and fluorohydrocortisone suppression. dexamethasone (2 mg/day) for 1-2 weeks suppressed the androgens, cortisol and corticosterone levels. PRA and PAC were suppressed temporally, but PRA returned to normal and PAC to be a high level after 2 weeks of dexamethasone administration. blood pressure was also reduced temporally but returned to a high level after 2 weeks of dexamethasone. These results indicate that primary aldosteronism and dexamethasone-suppressible hyperaldosteronism were not likely to be present, and unknown aldosterone stimulating factors which potentiated the action of endogenous angiotensin II or ACTH might be responsible for the hyperaldosteronism in this patient. We conclude that this patient had a mild and non-salt losing 3 beta-HSD deficiency in the zona reticularis with normal fasciculata and high glomerulosa function.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
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