Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01189 (beta-endorphin)
 21,003 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of pituitary-dependent Cushing's syndrome are described in which the computed tomography (CT) examination was negative; as the hormone dynamic investigations were directed towards the presence of corticotropin (ACTH) secreting pituitary formations, magnetic resonance imaging (MRI) of the pituitary was performed, which evidenced the presence of such lesions; subsequent neurosurgery confirmed in both cases the location indicated by MRI. In conclusion, the higher sensitivity of MRI compared to CT in the diagnosis of ACTH secreting pituitary adenomas can be noted.
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PMID:Is magnetic resonance imaging more sensitive than computed tomography in the location of corticotropin secreting pituitary adenomas? 256 3

To validate the adequacy of saliva as a biological specimen for the study of glucocorticoid adrenal function, the concentrations of salivary cortisol (SC) and serum total cortisol (TC) were measured by radioimmunoassay (RIA) in several groups of individuals in baseline state and during stimulation tests. The study of diurnal variations of SC in the reference population (n = 29) showed a nyctohemeral rhythm similar to that of TC, with maximal concentrations at 08.00-09.00 h (18 +/- 9 nmol/L) and 61% and 80% decreases at 15.30 and 23.00 h, respectively. After the administration of 1 mg of dexamethasone, SC was reduced in a 95% of its baseline value (n = 18). In all patients with Cushing's syndrome (n = 8) SC was increased whereas TC was normal in 3. All patients with adrenal failure (n = 11) had subnormal SC levels, while TC was normal in 4. The SC response to stimulation with intravenous synthetic adrenocorticotropin (Nuvacthen) (with and without previous suppression with 1 mg dexamethasone), insulin hypoglycemia and glucagon were qualitatively similar to those of TC, although more marked in proportion. These results, together with the practical advantages of saliva as a biological sample (easy obtention of specimen, absence of stress during its collection, and stability of cortisol in it), indicate that SC represents a more reliable measurement than TC as a useful clinical test to detect glucocorticoid dysfunction.
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PMID:[Usefulness of the determination of saliva cortisol in the study of adrenal gland glucocorticoid function]. 260 98

From January 1962 through April 1988, 188 patients with Cushing's syndrome were hospitalized at the Veterans General Hospital. Iatrogenic Cushing's syndrome was found in 123 (65.4%) of the patients. The other 43 patients (22.9%) had excess secretion of adrenocorticotropin (ACTH) and 22 patients (11.7%) had benign or malignant adrenocortical tumors. The clinical features and the functional reserve of the adrenal glands in those patients with iatrogenic Cushing's syndrome were evaluated. A retrospective analysis of the diagnostic rate of the various endocrine evaluations and the radiological examinations was also carried out. Four approaches were used in the management of Cushing's syndrome: (1) pituitary surgery, (2) pituitary irradiation, (3) adrenal surgery, and (4) drug therapy. Both the clinical and hormonal responses to these four management types were evaluated. The endocrinological, radiological, surgical and pathological experiences of 25 patients with Cushing's disease treated with pituitary surgery are presented. Five patients (20%) had an atypical endocrine pattern (20% false-negative). Twenty-four patients received a pituitary CT scan and 16 patients (66.7%) had positive CT findings. Of these 25 patients, 19 had pathology-proven microadenomas (less than 1cm), one had macroadenoma (greater than 1cm) and 5 had no evidence of adenomatous tissue. Among the 22 patients with adrenal Cushing's syndrome, 4 (18%) were suffering from adrenocortical carcinoma and 18 (82%) with benign adrenal tumors. The incidence of left adrenal adenoma was approximately twice that of right adrenal adenoma. The cure rate of adrenocortical adenoma by surgical treatment with unilateral adrenalectomy was excellent, but for adrenocortical carcinoma was poor.
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PMID:Cushing's syndrome: analysis of 188 cases. 262 29

The first case of isolated thyrotropin (TSH) deficiency with Cushing's syndrome was reported. A 46-year-old female was admitted to our hospital because of fatigability, puffy eye-lids, leg edema and petechia. She was treated with thyroid hormone. A low free triiodothyronine continued. Serum TSH was undetectable and serum thyrotropin releasing hormone (TRH) was elevated. No response of serum TSH and normal response of serum prolactin after administration of exogenous TRH suggested the abnormalities of anterior pituitary gland. The secretion of growth hormone and gonadotropin were intact, but the secretion of adrenocorticotropic hormone (ACTH) was impaired. A high level of serum cortisol, no response of serum ACTH by metyrapone test and a tumor mass of abdomen detected by computed tomography led to the diagnosis of Cushing's syndrome. Histological examination of the tumor revealed adrenal adenoma. Twenty two months after the surgery, serum ACTH level was normalized, but the secretion of serum TSH to exogenous TRH was still absent. The results indicated that hypothyroidism in our patient was due to isolated TSH deficiency.
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PMID:A case of isolated thyrotropin deficiency with Cushing's syndrome. 263 42

In humans, the syndrome of cortisol resistance is characterized by the absence of signs and symptoms of Cushing's syndrome, elevated total and unbound plasma cortisol concentrations, and increases in urinary free cortisol excretion and plasma adrenocorticotropic hormone. In one family, a severely affected member had hypertension and hypokalemic alkalosis associated with increased plasma concentrations of corticosterone and deoxycorticosterone. These patients are resistant to suppression of the pituitary-adrenal axis by dexamethasone. Dexamethasone therapy, however, effectively corrected hypertension and hypokalemic alkalosis in the severely affected patient, without causing signs of glucocorticoid excess. The glucocorticoid receptor from these patients has a low affinity for glucocorticoids and is unstable during thermal activation. Both the molecular weight of the glucocorticoid receptor and the size of the corresponding mRNA are similar to those of normal controls. Transformation of B-lymphocytes with Epstein-Barr virus leads to induction of glucocorticoid receptors. Receptor induction, however, is lower in patient cells than those obtained from normal controls. This decreased induction parallels decreased expression of glucocorticoid receptor mRNA. Thus, in this form of glucocorticoid resistance the glucocorticoid receptor is abnormal and leads to diminished target organ responsiveness. Many New World primates exhibit glucocorticoid "resistance," without apparent pathology. These species have markedly elevated plasma cortisol, both total and unbound concentrations, increased urinary free cortisol excretion, and marked increases in plasma adrenocorticotropic hormone and beta-endorphin. The glucocorticoid receptors of these primates have decreased affinity for glucocorticoids, are thermolabile, and are not induced by Epstein-Barr virus transformation as indicated by specific binding and mRNA expression. Both the molecular weight of the glucocorticoid receptor and the size of the corresponding mRNA are similar to those of normal controls. Despite the high plasma cortisol concentrations in these primates, there is no sodium retention and aldosterone levels are actually increased. The kidney aldosterone receptor cross-reacts poorly with cortisol, explaining the absence of sodium retention. New World primates also have progesterone, estrogen, aldosterone, and vitamin D insensitivity, suggesting a common factor linking steroid hormone receptors.
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PMID:Glucocorticoid resistance in humans and nonhuman primates. 264 36

Cushing's syndrome represents a constellation of symptoms of various origins. In most patients, detailed endocrinologic and radiologic testing will differentiate between Cushing's disease, adrenal adenoma, adrenal carcinoma, primary bilateral nodular hyperplasia, and ectopic corticotropin-producing tumors. Although adrenal surgery affords rapid and reliable remission in patients with Cushing's syndrome, it is associated with significant morbidity and mortality. Complications can be minimized by careful perioperative preparation. The indications for adrenal surgery for Cushing's disease have been altered radically by the success and low morbidity of transsphenoidal surgery. Total adrenalectomy is indicated in patients with bilateral nodular hyperplasia and should be considered for adults who have failed selective pituitary adenectomy or hypophysectomy and in whom ectopic corticotropin secretion has been unequivocally ruled out. At the Lahey Clinic, total adrenalectomy is performed through an anterior abdominal incision. Anterior approaches are especially indicated in those patients who require abdominal exploration for other intra-abdominal pathologic conditions that require surgery. Total adrenalectomy is indicated in the very rare patient who has Cushing's syndrome caused by ectopic corticotropin production when the patient is severely ill, a primary tumor is not found, and medical therapy fails or is poorly tolerated. Small adrenal tumors are best approached through a flank incision. Larger potentially malignant tumors should be approached through a thoracoabdominal incision.
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PMID:Surgical management of Cushing's syndrome. 266 80

The effectiveness of ketoconazole for the therapy of Cushing's syndrome, both of adrenal and pituitary origin, was retrospectively evaluated. To this end, the clinical and hormonal results in 5 patients were assessed. There were 4 females and one male, with ages ranging between 17 and 64 years. Three of them, who had Cushing's disease, received sustained treatment with ketoconazole when other therapeutic measures had failed. The two remaining patients (one with Cushing's disease and the other with adrenal adenoma) were treated with this drug in the preoperative period to alleviate the deterioration in the clinical condition caused by hypercortisolism. The clinical evolution of the patients is described, as well as the plasma adrenocorticotropin (ACTH) and the free cortisol in 24 hour urine in the subsequent controls. In view of the study results, we feel that ketoconazole is an effective treatment for the long term control of Cushing's syndrome. In addition, it may be also useful as preoperative therapy in those patients severely deteriorated by the sustained hypercortisolism.
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PMID:[Evaluation of the treatment of Cushing's syndrome using ketoconazole]. 269 99

A 49-year old woman with a 5-year history of Cushing's syndrome was evaluated. Biochemical measurements revealed high cortisol and adrenocorticotropic hormone (ACTH) levels consistent with the ACTH-dependent type of Cushing's syndrome. However, the source of ACTH seemed to be autonomous as she demonstrated abnormal feedback control, with lack of response to metyrapone and high coses of dexamethasone. A search for an ectopic ACTH-secreting nonpituitary neoplasm was unsuccessful. Transsphenoidal pituitary exploration revealed a normal pituitary gland, but an ectopic pituitary adenoma was found incidentally in the mucosa of the sphenoid sinus. This adenoma stained strongly positive for ACTH on immunocytochemical studies. Resection of this lesion led to remission of the Cushing's syndrome. This variant of ACTH-dependent Cushing's syndrome can mimic the ectopic ACTH-dependent type and should be looked for in patients who fail to respond to pituitary operation.
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PMID:Cushing's syndrome caused by an ectopic pituitary adenoma. 282 48

ACTH concentration was measured in simultaneously drawn blood samples from the left and right inferior petrosal sinuses before and after administration of corticotropin-releasing hormone (CRH). Such samples were successfully obtained in 20 of 21 patients with ACTH-dependent Cushing's syndrome on whom it was attempted. In 11 of the 20 patients there was no concentration difference between petrosal sinus and peripheral blood. But 13 of 19 patients had a unilateral central to peripheral concentration gradient greater than 1.4 after CRH administration. In the other six patients no ipsi- to contralateral gradient was demonstrable. Two of these patients had a proven ectopic ACTH-producing tumor; no adenoma was found at operation in three; on patient is awaiting operation. In 10 of 13 patients with unilateral gradient a microadenoma was found on the same side at operation. One patient had a hemi-hypophysectomy on the side of the higher gradient: hypocortisolemia developed in her postoperatively. Two other patients are awaiting operation. The results indicate that simultaneous bilateral catheterization of the inferior petrosal sinus with CRH stimulation is a highly informative examination with few side-effects and will contribute to better diagnosis and treatment of Cushing's syndrome.
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PMID:[Simultaneous bilateral catheterization of the inferior petrosal sinus in Cushing's syndrome. ACTH determination for the diagnosis and location of the side of a hypophyseal microadenoma before and after administration of corticotropin-releasing hormone]. 282 62

Computed tomographic (CT) scans of the adrenal glands were obtained in nine patients with Cushing disease as a result of an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma and macronodular hyperplasia of the adrenal glands. The findings were compared retrospectively with those in six patients with Cushing syndrome as a result of an autonomous adrenal adenoma and 16 with Cushing syndrome as a result of ectopic ACTH production. Seven of nine patients with macronodular adrenal hyperplasia had hyperplastic adrenal glands in addition to single or multiple focal adrenal nodules. The six patients with hypercortisolism caused by an autonomous adrenal adenoma showed atrophic (four patients) or normal (two patients) ipsilateral and contralateral adrenal glands. Fourteen of 16 patients with the ectopic ACTH syndrome had hyperplasia of the adrenal glands without nodularity, and only two had focal nodules.
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PMID:Macronodular adrenal hyperplasia in Cushing disease. 282 31


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