1/11. Diffuse stromal Leydig cell hyperplasia: a unique cause of postmenopausal hyperandrogenism and virilization.A 60-year-old woman presented with diffuse scalp alopecia, hirsutism, and clitorimegaly, and the mean serum testosterone levels were greater than 200 ng/dL. Findings on computed tomography of both adrenal glands were normal. After bilateral oophorectomy, a unique histological picture consisting of diffuse stromal Leydig cell hyperplasia was found. Reinke crystals were present, but neither hilus cell hyperplasia nor stromal hyperthecosis was noted. Sequencing of the 11 exons of the gene for the luteinizing hormone receptor revealed no abnormality. Relevant data suggest that treatment of the postmenopausal woman with hyperandrogenism and virilization is bilateral laparoscopic oophorectomy if she has no pronounced ovarian enlargement or adrenal tumor on imaging. In this setting, an intensive endocrine evaluation or a search for metastatic disease seems to be unnecessary.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/11. Valproate, hyperandrogenism, and polycystic ovaries: a report of 3 cases.BACKGROUND: Reproductive endocrine disorders characterized by menstrual disorders, polycystic ovaries, and hyperandrogenism seem to be common among women treated with sodium valproate for epilepsy. OBJECTIVE: To describe the development of valproate-related reproductive endocrine disorders in women with epilepsy. DESIGN: Case report. patients: Three patients developed a reproductive endocrine disorder during treatment with valproate. It was characterized by hyperandrogenism and polycystic ovaries in all cases, and it was associated with weight gain and menstrual disorders in 2 of the 3 women. RESULTS: Replacing valproate with lamotrigine resulted in a decrease in serum testosterone concentrations in all 3 women. The polycystic changes disappeared from the ovaries in 2 of the women after valproate therapy was discontinued, and the 2 women who had gained weight and developed amenorrhea while being treated with valproate lost weight and resumed menstruating after the change in medication. CONCLUSIONS: The 3 cases presented here illustrate the development of reproductive endocrine disorders after the initiation of valproate therapy in women with epilepsy. The disorders were characterized by hyperandrogenism and polycystic ovaries in all cases, and were associated with weight gain and menstrual disorders in 2 of the 3 women. An evaluation of ovarian structure and function should be considered in women of reproductive age being treated with valproate for epilepsy, especially if they develop menstrual cycle disturbances during treatment.- - - - - - - - - - ranking = 4keywords = endocrine (Clic here for more details about this article) |
3/11. Long-term remission of ovarian hyperandrogenism after short-term treatment with a gonadotropin-releasing hormone agonist.OBJECTIVE: To assess the long-term effects of GnRH agonist (GnRH-a) therapy in a patient with benign ovarian hyperandrogenism. DESIGN: Case report. SETTING: University Hospital endocrine outpatient's clinic. PATIENT(S): A 55-year-old postmenopausal woman with hirsutism and virilization of ovarian origin. INTERVENTION(S): Treatment with a course of GnRH-a (triptorelin 3.75 mg IM every 28 days for 4 months). Follow-up for 3 years. MAIN OUTCOME MEASURE(S): serum gonadotropin and androgen levels, clinical assessment using the Ferriman-Gallwey score, and assessment of ovarian morphology by ultrasonography. RESULT(S): Administration of triptorelin resulted in suppression of serum testosterone and gonadotropin values and relief of the hyperandrogenic symptoms. Upon discontinuation of treatment, the patient's serum gonadotropin levels returned to the postmenopausal range, but the testosterone levels remained normal and the patient was asymptomatic for an observation period of 3 years. CONCLUSION(S): This case is the first example of long-term remission of ovarian hyperandrogenism in a postmenopausal woman, after short-term treatment with GnRH-a. This supports the view that GnRH-a therapy could be used, even in short courses, for the long-term suppression of benign ovarian hyperandrogenism.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
4/11. Postmenopausal hyperthecosis: functional dysregulation of androgenesis in climacteric ovary.BACKGROUND: hyperandrogenism of ovarian origin is rare in postmenopausal women. However, there is evidence that the ovaries of postmenopausal women are active endocrine glands, secreting mainly androgens. CASE: A postmenopausal woman sought treatment for progressive hirsutism. Endocrine evaluation revealed androgen excess. Transvaginal ultrasound revealed enlarged ovaries. hysterectomy and bilateral oophorectomy were recommended. However, surgery had to be withheld for 6 months while the patient recovered from an acute myocardial infarction. In the interim, the patient's hyperandrogenemia was successfully treated with monthly injections of the gonadotropin-releasing hormone agonist (GnRH), leuprolide acetate. CONCLUSION: This report illustrates the potential for postmenopausal ovaries to become active androgen-secreting endocrine organs. It also demonstrates the efficacy of pharmacologic intervention for postmenopausal ovarian hyperthecosis when the patient is a poor surgical candidate.- - - - - - - - - - ranking = 2keywords = endocrine (Clic here for more details about this article) |
5/11. Coincidental diagnosis of an occult hilar steroid cell tumor of the ovary and a cortisol-secreting adrenal adenoma in a 49-year-old woman with severe hyperandrogenism.OBJECTIVE: To report an exceptional association between an occult ovarian steroid cell tumor and a cortisol-secreting adrenal adenoma. DESIGN: Case report. SETTING: endocrinology and nutrition unit at a general hospital in spain. PATIENT(S): A 49-year-old woman who presented with persistence of severe hyperandrogenism after removal of a left adrenal adenoma. INTERVENTION(S): An endocrine study evaluating serum androgens, adrenal function, and tumor markers was ordered. Transvaginal sonography was done to rule out an ovarian tumor. Finally, a selective catheterization of ovarian veins allowed the correct diagnosis. MAIN OUTCOME MEASURE(S): Clinical and endocrine description of the patient and preoperative localization of the source of T secretion. RESULT(S): After adrenal surgery, urinary free cortisol levels decreased to normal values, but serum T remained within the tumoral range (3.04 ng/mL). Selective catheterization of ovarian veins revealed a gradient of T concentration in the right ovary. After bilateral annexectomy, a microscopic steroid cell tumor of hilar type was found in the right ovary. serum T fell within the normal range, and hirsutism progressively improved. CONCLUSION(S): This unusual association between an occult-virilizing ovarian steroid cell tumor and a cortisol-secreting adrenal adenoma illustrates the value of an accurate preoperative workup in women with severe hyperandrogenism.- - - - - - - - - - ranking = 2keywords = endocrine (Clic here for more details about this article) |
6/11. Seckel-like syndrome: a patient with precocious puberty associated with nonclassical congenital adrenal hyperplasia.Seckel syndrome is a rare, recessively inherited disorder of severe growth retardation with low birth weight and distinct craniofacial, orodental and skeletal anomalies. In addition to these characteristics, some cardiovascular, hematopoietic, endocrine and central nervous system abnormalities have also been described. We report a patient with Seckel-like syndrome who has precocious puberty associated with non-classical congenital adrenal hyperplasia (NCCAH). She was admitted to our clinic three times. She was diagnosed as having Seckel-like syndrome and premature thelarche at the age of 8.9 years. At 10.9 years old she was admitted to our clinic with pubic hair and cliteromegaly. Hormonal findings revealed NCCAH and hydrocortisone therapy was offered but the patient was non-compliant. At 13.6 years she had acanthosis nigricans as an additional clinical finding and her pubertal stage was 4. She had irregular menses. On hormonal evaluation she had euglycemic hyperinsulinism accompanying mild hypertriglyceridemia and functional ovarian hyperandrogenism. Premature pubarche, hyper-insulinism, dyslipidemia, and hyperandrogenism, and some combinations of these, can be associated with reduced fetal growth. This is the first report of hyperinsulinism, and probably NCCAH, in association with Seckel syndrome.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
7/11. hyperandrogenism in a postmenopausal woman presenting with a metastatic ileum endocrine tumor.OBJECTIVE: To elucidate the mechanism of the hyperandrogenism found in a postmenopausal woman presenting an ileum endocrine tumor with ovarian metastases. DESIGN: Case report. SETTING: University hospital. PATIENT(S): A postmenopausal woman was referred for hirsutism. Basal plasma testosterone was high, 6.6 nM/L (normal,- - - - - - - - - - ranking = 7keywords = endocrine (Clic here for more details about this article) |
8/11. Mild cutaneous manifestation in two young women with extraordinary hyperandrogenemia.hyperandrogenism with hyperandrogenemia should be considered in those with severe acne of sudden onset or conspicuous male-pattern baldness with hairline recession, although the majority of female patients with acne or androgenetic alopecia possess no endocrine disorder. Herein we describe on the contrary 2 young women with primary amenorrhea displaying prominent hyperandrogenemia but subtle cutaneous manifestation. The first one presenting vertical alopecia had an elevated level of serum dehydroepiandrosterone sulfate (>800 microg/dl) and was suspected to be a case of late-onset, non-classical adrenal hyperplasia. The second case with mild acne had a soaring serum level of total testosterone >9,000 ng/dl derived from an androgen-secreting adrenal adenoma overexpressing steroidogenic acute regulatory protein, P450 side-chain cleavage enzyme and aromatase. A careful patient history and a complete physical examination are mandatory in each individual female case with acne or alopecia. The possibility of adrenal tumor should be explored in patients with escalated circulating testosterone.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
9/11. hyperandrogenism, insulin resistance, and acanthosis nigricans (hair-AN) syndrome: spontaneous remission in a 15-year-old girl.acanthosis nigricans is associated with many diseases, including internal malignancies, genetic disorders, and endocrine abnormalities. insulin resistance frequently accompanies endocrine-associated acanthosis nigricans. We describe a patient with acanthosis nigricans associated with hyperandrogenism and extreme insulin resistance that spontaneously resolved after a marked decrease in insulin receptor antibodies.- - - - - - - - - - ranking = 2keywords = endocrine (Clic here for more details about this article) |
10/11. A case of severe hyperandrogenism, acanthosis nigricans and over diabetes: the use of non-invasive methods for diagnosis, pathogenesis and management.hyperandrogenism is characterized clinically by hirsutism, acne, androgens-dependent alopecia and elevated serum concentrations of androgens (testosterone and androstenedione). polycystic ovary syndrome is the most frequent cause of hyperandrogenism. Nevertheless, the differential diagnosis includes androgen-secreting tumors of the ovary or adrenal gland. Although rare, it is important to consider this diagnosis in patients with serum testosterone concentrations greater than 7 nmol/l. A 35-year-old woman who presented with hirsutism, amenorrhea and acanthosis nigricans is described. The endocrine abnormalities included a serum testosterone concentration of 9 nmol/l and overt type II diabetes mellitus. Imaging studies, including magnetic resonance imaging and Doppler ultrasonography, did not disclose a secreting tumor. After cyproterone acetate was prescribed the serum testosterone concentration returned to normal. The recent application of modern, high-resolution diagnostic ultrasonography and magnetic resonance imaging enabled a morphologically based diagnosis in a case of severe hyperandrogenism, with no need for invasive procedures. The therapeutic response to antiandrogens is reassuring.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
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