1/21. Unilateral macronodular adrenal hyperplasia as an unusual cause of Cushing's syndrome--a case study.To appraise clinicians of the problems that may be encountered in the diagnosis and management of Cushing's syndrome, we present a case report of a 20-year old female, who was admitted with a recently developed central obesity, ammenorrhea hirsuitism, proximal myopathy and depression. She was found to have multiple striae, thin skin, elevated blood pressure glycosuria and hyperglycaemia. Morning and mid-night plasma cortisol concentrations revealed elevated levels, with a loss of diurnal variation. There was a failure of the normal suppressibility of cortisol secretion by low doses of dexamethasone, while a significant suppression of plasma cortisol concentration was observed with high doses of dexamethasone. There were no significant abnormalities observed in the pituitary fossa on skull radiograph and on the cranial computerised tomographic scan. After a period of stabilisation, she had a bilateral adrenalectomy done, with a histopathological finding of a left adrenal macronodular hyperplasia, while the right adrenal gland was small and friable. There was an uneventful post-operative period, with a gradual return to normality of most of the presenting complaints. The hyperglycaemia and hypertension got controlled without medications, while her menstrual cycles resumed within three months of bilateral adrenalectomy. This case report illustrates that an adrenal-dependent Cushing's syndrome may mimic a pituitary-dependent one, especially as regards the suppressibility of plasma cortisol secretion by high doses of dexamethasone.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/21. Induction of endometrial cycles and ovulation in a woman with combined 17alpha-hydroxylase/17,20-lyase deficiency due to compound heterozygous mutations on the p45017alpha gene.OBJECTIVE: To describe the case of a Japanese woman with combined 17alpha-hydroxylase/17,20-lyase deficiency (congenital adrenal hyperplasia type V) and to discuss possible therapeutic procedures in such patients. DESIGN: Case report. SETTING: University hospital. PATIENT(s): A 26-year-old woman with secondary amenorrhea and primary sterility. INTERVENTION(s): Nucleotide sequencing of the P45017alpha gene (CYP17), induction of endometrial maturation with steroid hormone replacement, and ovulation induction with gonadotropin. MAIN OUTCOME MEASURE(s): Nucleotide sequence of CYP17, endometrial thickness and follicle diameter measured by transvaginal ultrasonography, and histologic evaluation of the endometrium. RESULT(s): Two different mutations were detected on CYP17: One was a deletion of the phenylalanine codon (TTC) at either amino acid 53 or 54 in exon 1, and the other was a missense mutation with the substitution of histidine (CAC) by leucine (CTC) at position 373 in exon 6. Repeated histologic evaluations performed during treatment with P consistently revealed an unripe endometrium with glands of the early secretory phase and markedly scanty stroma. Ultrasound examination revealed follicular growth and ovulation after gonadotropin administration, but insufficient thickness of the endometrium. CONCLUSION(s): ovulation induction was possible in this patient with 17alpha-hydroxylase/17,20-lyase deficiency, but the endometrial response to steroid hormone replacement was extremely poor.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
3/21. craniopharyngioma and bardet-biedl syndrome. A case report.BACKGROUND: bardet-biedl syndrome is a rare disorder and associated with a variety of anomalies. CASE: An 18-year-old woman was referred with primary amenorrhea. Following physical, ophthalmologic, psychiatric, hormonal and radiologic examinations, the diagnosis of both craniopharyngioma and bardet-biedl syndrome was established. CONCLUSION: Although the pathogenesis of hypogonadism in a woman with bardet-biedl syndrome remains unclear, cranial structures, especially the hypothalamus and pituitary gland, should be investigated to reveal any possible abnormalities.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
4/21. amenorrhea-galactorrhea syndrome as an uncommon manifestation of isolated neurosarcoidosis.The involvement of the hypothalamic and/or the pituitary gland during granulomatous, infiltrative or autoimmune diseases is a rare condition of acquired hypothalamic dysfunction and non-tumoral-non-vascular hypopituitarism. sarcoidosis is a pathogen-free granulomatous disease which affects both central and peripheral nervous systems in 5-16% of patients. In most cases, nervous involvement by sarcoidosis occurs within a multi-systemic disease, although disease localization limited to the nervous system has also been reported. We observed a 26-year-old Italian woman presenting with low-grade fever, headache, visual disturbances, amenorrhea-galactorrhea syndrome and pituitary failure due to an infiltrative lesion involving the hypothalamus and the pituitary stalk. At first, the diagnosis of "probable" neurosarcoidosis was made according to the clinical picture, magnetic resonance imaging, and the study of cerebrospinal fluid lymphocyte sub-populations. In this case, hyperprolactinemic amenorrhea and galactorrhea combined with blunted responses of gonadotropins to luteinizing hormone releasing hormone and acquired growth hormone failure were to be considered as an infrequent manifestation of neurosarcoidosis limited to the medio-basal brain, without other disease localization. Forty-eight months after disease onset, the occurrence of mediastinal lymph node enlargement and increase of angiotensin converting enzyme in serum allowed us to confirm the diagnosis of sarcoidosis. Neurosarcoidosis is often hard to diagnose, especially when the neurological localization of the disease is not accompanied by other possible systemic signs, and when the lesion is too deep to obtain biopsy confirmation. The study of cerebrospinal fluid and blood lymphocyte sub-populations integrated by magnetic resonance imaging may be helpful for a correct diagnosis.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
5/21. 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 = 2keywords = gland (Clic here for more details about this article) |
6/21. 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 = 1keywords = gland (Clic here for more details about this article) |
7/21. Pituitary sarcoidosis causing secondary amenorrhea. A case report.A 32-year-old woman with hypogonadotropic amenorrhea and sarcoidosis had an enlarged pituitary stalk and gland on computed tomography. Concentrations of luteinizing and growth hormone did not increase after the administration of gonadotropin releasing hormone and insulin-induced hypoglycemia, respectively. The absence of response possibly was the consequence of the patient's estrogen deficiency. The response of thyroid stimulating hormone, prolactin and cortisol was normal after provocative pituitary stimulation. central nervous system sarcoidosis occurs in 3.5-5.0% of patients with systemic sarcoidosis. Hypothalamic or pituitary sarcoidosis occurs in approximately 0.5% of patients. diabetes insipidus and hyperprolactinemia are the most frequent manifestations of hypothalamic-pituitary sarcoidosis, occurring in one-half and one-third, respectively, of such patients. Menstrual disturbances, including amenorrhea, are reported infrequently. Computed tomography and provocative pituitary-hypothalamic testing are useful in detecting central nervous system sarcoidosis and in delineating the site of involvement.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
8/21. Asherman's syndrome: a review of the literature, and a husband and wife's 20-year world-wide experience.Asherman's syndrome is reviewed, and 27 cases treated by us in iran, england, new zealand and australia over a 20-year period are analysed. Aetiological factors and treatment are discussed. In view of the high incidence of complications in subsequent pregnancies, the need for prevention is stressed. Although more common in some countries, it is, nevertheless, of world-wide distribution and, unless looked for, will be missed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
9/21. ACTH deficiency, hyperprolactinemia and benign intracranial hypertension. A case report.A 26-year-old female with ACTH deficiency, hyperprolactinemia and benign intracranial hypertension is reported. Her symptoms of adrenocortical insufficiency and persistent amenorrhea appeared after her last child birth one year previously. During an infectious disease she became critically ill with hypotension and was treated with iv penicillin. A bacterial infection was, however, not diagnosed. After 4 days she developed symptoms and signs of intracranial hypertension. She improved gradually within 10 days without specific therapy against the intracranial pressure. Endocrine investigation disclosed a secondary adrenocortical failure. The lesion appeared to be located in the pituitary gland since plasma ACTH and cortisol did not respond to CRH. A moderately elevated serum PRL was found, whereas the pituitary reserves of TSH, GH, LH and FSH were normal, as was a computed tomographic scan of the pituitary gland. The patient was given cortisone substitution therapy and recovered immediately. Within the following year she regained normal menstruations and became pregnant. A possible autoimmune etiology of her isolated ACTH deficiency precipitated in the puerperium is discussed.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
10/21. carcinoid tumor of the pancreas causing the diarrheogenic syndrome: report of a case combined with multiple endocrine neoplasia, type I.A case of carcinoid tumor of the pancreas with the watery diarrhea, hypokalemia, and hypochlorhydria syndrome in association with hyperparathyroidism and the amenorrhea-galactorrhea syndrome is presented. Resection of the three grossly enlarged, hyperplastic parathyroid glands restored eucalcemia in this patient. A subsequent excision of the 370 gm pancreatic carcinoid tumor resulted in a cure of the watery diarrhea and a return of the gastric acid secretion to normal. Immunocytochemical studies of the pancreatic tumor demonstrated a positive stain only for serotonin, and negative results for vasoactive intestinal polypeptide, pancreatic polypeptide, glucagon, insulin, cholecystokinin, gastrin, and calcitonin were obtained. These studies suggest that in this patient, serotonin was a causative agent of the watery diarrhea syndrome.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
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