Cases reported "Hyperandrogenism"

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1/6. Adrenal adenocarcinoma and empty sella syndrome in a 37-year-old woman.

    The case of a 37-year-old woman with secondary amenorrhea and clear signs of hyperandrogenism is reported. The patient underwent hormonal evaluation including circadian rhythm of cortisol, gonadotropin-releasing hormone/thyroid-stimulating hormone (GnRH/TRH) test, corticotropic-releasing hormone (CRH) test and dexamethasone suppression test. She also underwent pelvic and adrenal ultrasound examination, adrenal computed axial tomography (CAT) scan and cranial nuclear magnetic resonance (NMR). A mass about 10 cm in size was detected in the left adrenal region. The sella was empty and the pituitary displaced downward. Suspected adrenal adenocarcinoma was confirmed by histological examination after surgical removal of the mass. This case is of interest for physicians because of the mixed androgen and cortisol secretion of the adenocarcinoma in a hyperprolactinemic patient with empty sella. Moreover, it suggests the need to investigate the adrenal gland in patients with hyperprolactinemia and hirsutism.
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keywords = amenorrhea
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2/6. 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.
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keywords = amenorrhea
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3/6. biliary atresia with hyperandrogenic amenorrhea.

    The etiology of hyperandrogenic amenorrhea in a patient with biliary atresia successfully treated by a Kasai operation was unclear. Delayed puberty and menarche were evident at 16 years of age. Investigations showed no luteinizing hormone (LH)-follicle-stimulating hormone surge. A LH-releasing hormone provocative test showed a normal response. Peripheral aromatization of androgens appeared to function normally. Free testosterone (T) was normal, however, plasma levels of sex-hormone-binding globulin and total T were high. After menarche at 18 years of age, anovulatory menstrual cycles continued. A combination of estrogen and progesterone therapy was effective. A possible explanation may be that metabolic clearance of T is reduced in the presence of liver cirrhosis and a portosystemic shunt.
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keywords = amenorrhea
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4/6. 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.
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keywords = amenorrhea
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5/6. Intraoperative venous blood sampling to localize a small androgen-producing ovarian tumor.

    BACKGROUND: Among other causes of virilization, ovarian tumors can be the cause of excessive androgen production. We report the case of a leydig cell tumor of the ovary where diagnostic attempts to localize the source of hyperandrogenism preoperatively failed owing to relatively small tumor size. CASE: A 36-year-old woman presented with clinical signs of severe virilization including progressive balding, increased hirsutism, secondary amenorrhea and enlargement of the clitoris. Extensive work-up included endocrinological tests, pelvic ultrasound, magnetic resonance imaging, chromosomal analysis, norcholesterol scintigraphy and selective venous sampling, without direct localization of the source of hyperandrogenism. Persistently high plasma testosterone prompted an explorative laparotomy. Intraoperative selective blood sampling of the ovarian veins and palpation gave evidence of a right ovarian tumor, which was then removed. Histological examination revealed the presence of a pure leydig cell tumor. CONCLUSION: Exploratory laparotomy with intraoperative selective blood sampling of the ovarian veins might be a useful approach in patients without accurate preoperative localization of androgen-producing tumors of the ovaries.
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keywords = amenorrhea
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6/6. 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.
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keywords = amenorrhea
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