1/73. hypertension and virilization caused by a unique desoxycorticosterone- and androgen-secreting adrenal adenoma.We describe a rare androgen and desoxycorticosterone (DOC)-secreting adrenal tumor in a non-Cushingoid 14 year-old Haitian girl with secondary amenorrhea, hypertension and virilization. Her steroid pattern simulated an 11 beta-hydroxylation defect with notable elevation of adrenal androgens, 11-desoxycortisol (S), DOC, 17 alpha-hydroxyprogesterone and pregnenelone. Exogenous ACTH stimulated steroidogenesis. A CAT scan unfortunately failed to delineate an adrenal mass. dexamethasone (DEX) was administered, therefore, which partially suppressed androgen levels, reduced DOC and S by 80% and 82% respectively, and normalized blood pressure. Nevertheless, the response to glucocorticoid was incomplete and an MRI was obtained, which revealed a right adrenal tumor. Post surgery, the patient promptly resumed menses and became normotensive. This case illustrates that ACTH and DEX cannot reliably differentiate tumor from hyperplasia, whereas the simultaneous increase of delta 4 and delta 5 steroids, present here, may favor a tumor. This case also allows speculation that the hypersecretion of DOC may result from inhibition of 11 beta-hydroxylase activity by excess androgens. The importance of appropriate imaging for diagnosis is underscored.- - - - - - - - - - ranking = 1keywords = amenorrhea (Clic here for more details about this article) |
2/73. Long-term treatment with bromocriptine of a plurihormonal pituitary adenoma secreting thyrotropin, growth hormone and prolactin.A 48-year-old female presented with acromegaly, amenorrhea and hyperthyroidism associated with high serum free T4 levels and measurable TSH concentrations. The administration of GHRH induced significant increases in GH, PRL and TSH. Conversely, intravenous infusion of dopamine or oral administration of bromocriptine effectively inhibited GH, PRL and TSH secretion. serum alpha-subunit levels were neither affected by GHRH, dopamine nor bromocriptine. Transsphenoidal surgery was performed and immunostaining of the tissue showed that the adenoma cells were positive for GH, PRL or TSH. The patient was treated with bromocriptine at a daily oral dose of 10 mg after surgery. serum TSH were initially suppressed but returned within reference intervals with persistent normalized free T4 levels. serum PRL became undetectable and GH levels were stable around 6 ng/ml except the periods of poor drug compliance, when serum TSH, GH and PRL levels rose considerably. The patient was followed-up for 10 years without any change in the residual adenoma tissues as detected by magnetic resonance imaging. These findings suggest that long-term bromocriptine therapy is effective in treating the hypersecretory state of a plurihormonal adenoma secreting TSH, GH and PRL.- - - - - - - - - - ranking = 1keywords = amenorrhea (Clic here for more details about this article) |
3/73. Nonfunctioning pituitary macroadenoma presenting with mild hyperprolactinemia and amenorrhea.OBJECTIVE: To describe a patient with a clinically nonfunctioning pituitary macroadenoma who presented with mild hyperprolactinemia and amenorrhea. DESIGN: Case report. SETTING: Tertiary care medical facility. PATIENT(S): A 44-year-old woman with a 6-month history of amenorrhea. INTERVENTION(S): Pituitary testing, magnetic resonance imaging of the sella turcica, and transsphenoidal surgery. MAIN OUTCOME MEASURE(S): Pituitary function testing, magnetic resonance imaging, and return of menstrual cycles. RESULT(S): Baseline laboratory data revealed a serum prolactin level of 34 ng/mL (normal range, 3-20 ng/mL), normal thyroid function test results, and an FSH level of 6.7 mIU/mL. A second fasting prolactin level was 48 ng/mL. magnetic resonance imaging of the sella turcica revealed a pituitary macroadenoma measuring 1.4 x 3.2 cm. Further testing of baseline pituitary function revealed normal findings. The patient underwent an uncomplicated transsphenoidal resection of the pituitary tumor and maintained normal pituitary function. Pathologic evaluation revealed a pituitary adenoma that stained positive for FSH and focally for the alpha subunit. The adenoma stained negative for GH, prolactin, ACTH, LH, and TSH. CONCLUSION(S): This patient had a nonsecreting gonadotroph macroadenoma that resulted in hypogonadotropic hypogonadism along with mild hyperprolactinemia, presumably secondary to interruption of normal transport down the pituitary stalk.- - - - - - - - - - ranking = 6keywords = amenorrhea (Clic here for more details about this article) |
4/73. Pituitary adenoma showing intermittent secretion of high molecular weight adrenocorticotropin without evidence of Cushing's disease.A 29-year-old woman was admitted in March 1998 due to high plasma ACTH levels, amenorrhea and uncontrolled diabetes mellitus (DM) which had persisted since 1991. plasma ACTH levels showed a wide range of changes: they were usually high (59-240 pg/ml), intermittently very high (336-942 pg/ml), and sometimes normal or low. plasma cortisol levels were usually normal but were sometimes high when the ACTH levels were very high. However, even when the plasma ACTH levels were very high, she did not show any cushingoid features. DM was diagnosed as non-insulin-dependent DM. plasma ACTH showed an excessive response to CRH, while cortisol showed a delayed response. plasma cortisol showed a poor response to ACTH-(1-24). ACTH receptor gene analysis revealed no mutations in the ACTH receptor-coding region. MRI showed a nonenhancing mass on the left side of the pituitary. cavernous sinus sampling showed a very high plasma ACTH level in the left cavernous sinus compared with the levels in the right cavernous sinus and peripheral blood. Sephadex G-75 gel filtration of plasma ACTH immunoreactivity in plasma obtained by cavernous sinus sampling showed mainly high molecular forms of ACTH, probably proopiomelanocortin and ACTH-beta-lipotropin. This case is a very rare form of pituitary adenoma showing intermittent secretion of high molecular ACTH unaccompanied by cushingoid features.- - - - - - - - - - ranking = 1keywords = amenorrhea (Clic here for more details about this article) |
5/73. Delayed puberty associated with hyperprolactinemia caused by pituitary microadenoma.Primary amenorrhea caused by the hyperprolactinemia is a rare condition characterized by the onset of thelarche and pubarche at appropriate ages but arrest of pubertal development before menarche. hyperprolactinemia might be found in a few women with primary amenorrhea, yet relevant experience has apparently not been reported. We report a 16-year-old patient with hyperprolactinemia caused by a pituitary microadenoma. Her only symptom was delayed puberty without galactorrhea. bromocriptine therapy was useful in order to induce the ovulation and cause the menarche.- - - - - - - - - - ranking = 2keywords = amenorrhea (Clic here for more details about this article) |
6/73. Cortisol producing adrenal adenoma--a new manifestation of Gardner's syndrome.INTRODUCTION: Familial adenomatous polyposis (FAP) is an autosomal dominant disorder which typically presents with colorectal cancer in early adult life secondary to extensive adenomatous polyps of the colon. Gardner's syndrome is a variant of FAP in which desmoid tumors, osteomas and pigmented retinal lesions occur together with intestinal manifestations. The APC gene (adenomatous polyposis coli) at 5q21 is a tumor suppressor gene which is mutant in FAP. PATIENT: A 36 year old woman presented with a history of polyposis ventriculi, ovarian desmoid cysts, and disseminated desmoid tumors. Her familial history was unremarkable. On admission she complained weight gain, secondary amenorrhea, and episodes of hypertension followed by paroxysmal headache. RESULTS: Elevated urinary free cortisol (878 microg/24h), suppressed basal ACTH (< 5 pg/ml) and insuppressible serum cortisol after low dose dexamethasone (189 ng/ml) revealed adrenal Cushing's syndrome. Abdominal NMR showed an adrenal mass two centimeter in diameter with inhomogeneous contrast enhancement. Unilateral adrenalectomy was performed and an adrenal adenoma was diagnosed by histological criteria. For mutational detection dna from peripheral blood leucocytes was extracted. A protein truncation test was performed, which revealed a termination mutation between codon 1099 and 1623 of the APC gene. Direct sequencing showed a point mutation in exon 15 of the APC gene at position 1542 (CAG --> TAG). This region is known to be altered in patients with extraintestinal manifestation of FAP. CONCLUSION: In patients with Gardner's syndrome adrenal tumors leading to hormonal excess should be considered. Whether mutations in the APC gene have implications in sporadic adrenal tumorigenesis needs to be proven.- - - - - - - - - - ranking = 1keywords = amenorrhea (Clic here for more details about this article) |
7/73. diagnosis of acromegaly in a patient with type 1 diabetes mellitus.OBJECTIVE: To describe a case of acromegaly in a young patient with poorly controlled diabetes mellitus and suggest guidelines to distinguish acromegaly and high growth hormone (GH) levels previously reported in poorly controlled type 1 diabetes. methods: We present a detailed case report, including clinical findings and serial laboratory results in a patient with type 1 diabetes and a GH-secreting pituitary tumor. RESULTS: A 28-year-old woman with type 1 diabetes underwent assessment for secondary amenorrhea and worsening glycemic control. A low estradiol level and an inappropriately low level of follicle-stimulating hormone prompted magnetic resonance imaging of the head, which demonstrated a pituitary adenoma. Subsequent endocrine investigation revealed a high insulin-like growth factor i (IGF-I) level (849 mg/L; normal range, 122 to 400). The concentration of insulin-like growth factor-binding protein-3 (IGFBP-3) was also elevated (5.5 mg/L; normal range, 2.0 to 4.2). GH levels measured during episodes of spontaneous hyperglycemia (>180 mg/dL) were in the range of 3 to 5 ng/mL and failed to suppress to below 2 ng/mL after a bromocriptine suppression test. The patient underwent transsphenoidal resection of a pituitary tumor, which stained positively for GH by immunohistochemistry. Postoperatively, glycemic control improved, with decreased fluctuations of hypoglycemia and hyperglycemia, despite a decrease in insulin requirements. CONCLUSION: This report highlights the difficulty in interpreting GH and IGF-I levels in patients with type 1 diabetes. In addition, a detailed review of the literature suggests that IGFBP-3 measurements may be helpful in confirming the diagnosis of concurrent acromegaly and distinguishing it from high GH levels attributable to poor control of diabetes.- - - - - - - - - - ranking = 1keywords = amenorrhea (Clic here for more details about this article) |
8/73. Primary amenorrhea accompanied by adrenal adenoma: start of menarche soon after tumor resection.We report a patient with primary amenorrhea accompanied by adrenal adenoma. A 21-year-old woman was admitted to our hospital because of primary amenorrhea and hyperprogesteronemia without virilization. Venous sampling showed progesterone overproduction at the left adrenal gland. Computed tomography revealed a 7 cm mass in left adrenal gland. serum pregnenorone, 17-hydroxypregnenorone, dehydroepiandrosterone, 17-hydroxyprogesterone, deoxycorticosterone, and 11-deoxycortisol were elevated. The diagnosis of steroid hormone producing tumor was made. Following tumor resection, serum progesterone normalized 4 days later, and menarche occurred 51 days after operation. The present case indicated that adrenal functioning tumor should not be overlooked in patients with primary amenorrhea.- - - - - - - - - - ranking = 7keywords = amenorrhea (Clic here for more details about this article) |
9/73. Cyclic estrogen-progestin hormone therapy as a new therapeutic approach in the treatment of functional alterations of the hypothalamus-pituitary-ovary axis: case reports.amenorrhea is a clinical condition characterized by failure of menarche or by the absence of menstruation for six months in a woman with previous periodic menses. We report a first case of a 30 year-old woman affected by polycystic ovarian disease (PCOD) whose amenorrhea ceased after a 6-month combination treatment with cyclic estradiol-norethisterone acetate. After the withdrawal of the hormone therapy, a stable recovery of periodic menses was observed. We describe a second case of a 23 year-old woman whose amenorrhea was caused by a hypogonadotropic hypogonadism due to a non-functioning pituitary adenoma. After the administration of the previously described therapy both a disappearance of the adenoma and a recover of periodic menses were observed. We hypothesized that the outcomes in our cases could be the consequence of a balancing action induced by the exogenous hormone administration. The exogenous hormones may have reset the feedback between the hypothalamus and pituitary gland through mimicking the physiological hormones pattern of the 28-day cycle.- - - - - - - - - - ranking = 2keywords = amenorrhea (Clic here for more details about this article) |
10/73. Isolated familial somatotropinomas: clinical features and analysis of the MEN1 gene.Isolated familial somatotropinomas (IFS) rarely occurs in the absence of multiple endocrine neoplasia type I (MEN1) or the carney complex. In the present study we report two Italian siblings affected by GH-secreting adenomas. There was no history of parental consanguinity. The sister presented at 18 years of age with secondary amenorrhea and acromegalic features and one of her two brothers presented with gigantism at the same age. Endocrinological investigations confirmed GH hypersecretion in both cases. Although a pituitary microadenoma was detected in both patients, transsphenoidal surgery was not successful. The sister received conventional radiotherapy and acromegaly is now considered controlled; the brother is being treated with octreotide LAR 30 mg monthly and the disease is considered clinically active. patients, their parents and the unaffected brother underwent extensive evaluation, and no features of MEN1 or carney complex were found. Analysis of polymorphic microsatellite markers from chromosome 11q13 (D11S599, D11S4945, D11S4939, D11S4938 and D11S987) showed that the acromegalic siblings had inherited different maternal chromosomes and shared the paternal chromosome. No pathogenic MEN1 sequence changes were detected by sequencing or dideoxy fingerprinting of the coding sequence (exons 2-10) and exon/intron junctions. Although mutations in the promoter, introns or untranslated regions of the MEN1 gene cannot be excluded, germline mutations within the coding region of this gene do not appear responsible for IFS in this family.- - - - - - - - - - ranking = 1keywords = amenorrhea (Clic here for more details about this article) |
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