Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0001430 (adenoma)
 21,222 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a 41-year-old hirsute woman, severe hypercalcemia led to the discovery of hyperparathyroidism related to the involvement (hyperplasia/or adenoma) of the 4 parathyroid glands. Plasma and urinary DHA, plasma DHA-sulfate and delta 5 steroid precursors were elevated. Steroid hormone hypersecretion was stimulated by hCG and ACTH, and exhibited a paradoxical rise during dexamethasone administration. Computerized tomography scanning as well as arteriography disclosed bilateral adrenal hyperplasia and left adrenal adenoma. Bilateral adrenal vein catheterization indicated a left/right gradient for delta 5 steroids and delta 5 steroid sulfates. At surgery a left brown adrenal encapsulated adenoma was removed with a hyperplastic adrenal gland. Results of in vitro studies (adrenal steroid content and incubation) together with postadrenalectomy hormonal results suggest that the left brown adrenal adenoma was the main source of excessive androgen production. The infrequent association of an androgen-producing adrenal adenoma with hyperparathyroidism raises the hypothesis of multiple endocrine neoplasia syndrome. However, evidence for this diagnosis is lacking in the absence of other glandular involvement and of family history.
 ...
PMID:Androgen producing adrenal adenoma. Report on a case associated with hyperparathyroidism. 289 90

200 patients with mineralocorticoid hypertension were studied at the Clinical Study Center. The study of 150 patients with primary aldosteronism revealed five distinct subsets based on their responses to the upright posture, after administration of intravenous saline, deoxycorticosterone acetate, and spironolactone. Two new types were identified--aldosterone producing responsive adenoma (AP-RA) and primary adrenal hyperplasia (PAH). Patients with AP-RA maintained normal physiologic responses to the above maneuvers. Patients with PAH had responses similar to patients with an aldosterone producing adenoma (APA) but no tumor was identified. Both types were cured by unilateral adrenalectomy. There has been no change in subtype in up to 20 years of follow-up. The notion of a continuum from low renin hypertension to APA is not supported. Primary deoxycorticosteronism caused by a benign adrenal adenoma, malignancy and hyperplasia is described. Uniquely, overproduction of the 17-deoxysteroids of the zona fasciculata occurs with normal 17-hydroxy function. After the removal of a benign adenoma the contralateral adrenal gland revealed a delay in the 17-deoxysteroid responses to ACTH in the face of normal cortisol increases. This suggests that an independent pituitary regulator of the 17-deoxypathway may exist. Other hypertensive disorders with excessive deoxycorticosterone production are linked with increases of ACTH and cortisol levels. The hallmarks of primary deoxycorticosteronism are hypertension with hypokalemia, suppression of renin and aldosterone, and overproduction of the 17-deoxysteroids.
 ...
PMID:Identification and implications of new types of mineralocorticoid hypertension. 291 10

The practicability and tolerability of trilostane, a competitive inhibitor of 3 beta-hydroxysteroid-delta 5-dehydrogenase, for the therapy of primary aldosteronism was assessed in 1 patient with aldosterone-producing adenoma (APA) and 3 subjects with idiopathic adrenal hyperplasia (IHA). Trilostane afforded reduction of plasma levels of aldosterone, progesterone, deoxycorticosterone, 17-OH progesterone, cortisol, delta 4-androstenedione, and urinary excretion of 17-hydroxycorticosteroid. Conversely, circulating levels of dehydroepiandrosterone, dehydroepiandrosterone sulfate, and urinary excretion of 17-ketosteroids were increased following this drug therapy. Suppression of mineralo- or glucocorticoid biosynthesis was accompanied by an increase in plasma renin activity. One patient with APA or 3 subjects with IHA showed slight or remarkable improvement of hypertension and hypokalemia. Based on these findings, efficacy and tolerability of trilostane appear to aid in the treatment of IHA.
 ...
PMID:Primary aldosteronism treated by trilostane (3 beta-hydroxysteroid dehydrogenase inhibitor). 298 27

In recent years, cholesterol adrenal scintigraphy has been employed in the morphofunctional study of adrenal hypercorticism. Particularly in Cushing's syndrome, this noninvasive procedure can give a determinant contribution to distinguish ACTH-dependent from ACTH-independent forms. In our Institute, adrenal scintigraphy was performed in 77 patients with Cushing's syndrome diagnosed on clinical grounds confirmed by laboratory investigations and functional tests (17 with cortisol-secreting adenoma, 54 with pituitary ACTH-dependent bilateral adrenal hyperplasia, 2 with ectopic ACTH-dependent bilateral hyperplasia and 4 with bilateral nodular hyperplasia). Three distinct scintigraphic patterns have been identified. The 56 patients with ACTH-dependent Cushing's syndrome showed bilateral symmetric or slightly asymmetric visualization of the adrenal glands; in the 17 patients with ACTH-independent Cushing's syndrome the adrenal scintigraphy only visualized the gland harboring the adenoma; finally, a marked asymmetric visualization of the glands was observed in the 4 patients with adrenal nodular hyperplasia. These data confirm that adrenal scintigraphy is able to distinguish between ACTH-dependent and ACTH-independent Cushing's syndrome and reliably lateralizes adenomas when they are present. Moreover, the morphofunctional information obtained by this procedure, together with the high resolution morphological data by computerized tomography, allows to recognize the presence of bilateral nodular hyperplasia, an uncommon cause of Cushing's syndrome.
 ...
PMID:Adrenal scintigraphy in the morphological and functional evaluation of Cushing's syndrome. 299 68

Mineralocorticoid and glucocorticoid receptors were measured in circulating mononuclear leukocytes in 5 patients affected by Conn's syndrome (3 cases of bilateral adrenal hyperplasia and 2 cases of adenoma plus unilateral hyperplasia). The number of the binding sites per cell resulted significantly lower (189 +/- 114, mean +/- SD), as compared with the normal controls (298 +/- 105). The affinity of aldosterone for the receptor was found to be not different than that of healthy control subjects. The capacity and the affinity of dexamethasone for glucocorticoid receptors ranged in the normal values. These data suggest a possible down-regulation of mineralocorticoid receptors in humans.
 ...
PMID:Mineralocorticoid and glucocorticoid receptors in circulating mononuclear leukocytes of patients with primary hyperaldosteronism. 299 70

The number of mineralocorticoid-binding sites on mononuclear leukocytes and plasma aldosterone (aldo) concentrations were measured in patients with different types of primary hyperaldosteronism. Patients with unilateral adenoma and patients with bilateral adrenal hyperplasia had a significantly lower (P less than 0.001) mean number of binding sites for aldo [144 +/- 36 (+/- SD; n = 6) and 140 +/- 28 sites/cell (n = 4), respectively] compared with normal subjects (292 +/- 110 sites/cell; n = 25). In four patients with dexamethasone-suppressible hyperaldosteronism, mineralocorticoid-binding sites in mononuclear leukocytes were normal (291 +/- 108 sites/cell). In all patients undergoing surgery for unilateral adenoma, the receptors normalized 3 months after the operation. In two patients the reduction in receptors persisted for a short time after surgery even though the plasma aldo level had already normalized. We conclude that mineralocorticoid excess produces down-regulation of mineralocorticoid receptors, which, in turn, might contribute to the genesis of the aldo escape phenomenon.
 ...
PMID:Aldosterone receptors in different types of primary hyperaldosteronism. 303 47

A profile of dexamethasone-suppressible hyperaldosteronism (DSH), a variant of primary aldosteronism, is drawn by reviewing its pathophysiological and clinical aspects. Genetic studies show no HLA linkage and point to an autosomal dominant mode of inheritance, suggesting that the prevalence of this disease has been underestimated in the past. Hypertension, hypokalemia, suppressed renin, and high aldosterone values characterize DSH in the basal state, similar to the other forms of primary aldosteronism, i.e., aldosterone-producing adenoma (APA) or bilateral idiopathic adrenal hyperplasia (IAH). Biochemically DSH and APA can be differentiated from IAH since in both aldosterone does not respond to upright posture, to angiotensin II infusion, and to angiotensin-converting enzyme (ACE) captopril. In contrast, morphologically DSH is similar to IAH, since neither macroscopic nor histologic examinations of the adrenals give evidence of any unilateral abnormality. However, DSH is differentiated from APA and IAH by the hyperresponsiveness of aldosterone to acute ACTH administration as well as by the failure of aldosterone to escape from prolonged ACTH stimulation. The final diagnosis of DSH rests upon the prompt reversal of the features of mineralocorticoid excess by glucocorticoid therapy. In some cases hypertension is unresponsive to dexamethasone and needs alternative treatment. The main pathogenetic hypotheses point to a pituitary and/or an adrenal abnormality, but the intrinsic nature of the disease remains to be elucidated.
 ...
PMID:Dexamethasone-suppressible hyperaldosteronism: pathophysiology, clinical aspects, and new insights into the pathogenesis. 303 79

Primary aldosteronism, Cushing's syndrome and pheochromocytoma are the most frequent endocrine hypertensive disorders. Following biochemical confirmation of the clinical diagnosis, mainly non-invasive imaging techniques are necessary to determine the cause of the hormone excess. Diagnosis of primary aldosteronism is confirmed by analysis of aldosterone and renin in peripheral venous blood. Differentiation between adenoma and idiopathic adrenal hyperplasia usually is achieved by computed tomography and isotope scan. A reliable test for the biochemical confirmation of Cushing's syndrome is the determination of urine-free cortisol. In patients with equivocal results the dexamethasone suppression test is performed. Differential diagnosis between ACTH-dependent Cushing's syndrome (pituitary or ectopic) and primary adrenal disorders can be made by determination of plasma-ACTH and -cortisol. Non-invasive localization procedures include computed tomography of the abdomen, computed tomography or magnetic resonance imaging of the pituitary gland, sonography and isotope scan. Diagnosis of pheochromocytoma is based on elevated catecholamine levels in urine or plasma. The tumor is localized by ultrasound, computed tomography or isotope scan.
 ...
PMID:[Rational diagnosis of endocrine forms of hypertension]. 307 51

After diagnosis of primary aldosteronism on the basis of biochemical evidence, the detection of the tumour is of crucial importance in the management of the disease. We reviewed the efficacy of CT-Scan, Iodo-Cholesterol Scintigraphy, digitalized phlebography, adrenal vein sampling for steroid measurements (AVS), and Nuclear Magnetic Resonance (NMR) in 160 hypertensive patients with primary aldosteronism. Diagnosis of Conn's adenoma (n = 96) or Adrenal Hyperplasia (n = 40) was confirmed by surgery or at least two concordant tumour localization tests. Scintigraphy gave a correct diagnosis in 53% of the 51 exams, CT-Scan in 82% of the 85 exams, and phlebography in 79% of 61 exams. Plasma Aldosterone/Cortisol ratio was 5 times higher on the side of adenoma in 55% of the 47 cases but this ratio was also present in 23% of 22 patients with adrenal hyperplasia. Each procedure exhibited few false positive and false negative cases. NMR performed in 15 patients with Conn's adenoma identified all the cases. But tumours displayed a signal close to the liver signal and identical to the normal adrenal. These results and the risk of invasive procedure (failure of catheterization of the right adrenal vein (n = 6) and adrenal haematoma (n = 2) lead us to propose a schema of exploration of patients with primary aldosteronism. The CT-Scan could be performed at the first step once the biological diagnosis confirmed. Phlebography and AVS will be performed only if tumour was less than 1 cm at the CT-Scan despite important biological abnormalities. This schema requires to be validated by a prospective evaluation.
 ...
PMID:[Localization of primary hyperaldosteronism]. 320 1

A study was made of 9 patients with primary aldosteronism due to aldosterone-producing adenoma (APA) and 8 subjects with idiopathic adrenal hyperplasia (IHA) to clarify the pathogenesis of sustained hypertension after surgical or non-surgical treatment. Following each treatment, a complete improvement of hypertension was obtained in 12 patients (6 APA, 6 IHA), while 5 (3 APA, 2 IHA) showed still hypertensive status. Renal or renovascular lesions were prominent only in the hypertension-unchanged group. Under regular sodium diet, the ratio of urinary excretion of sodium to creatinine of this hypertensive group was significantly lower than that of the hypertension-improved group. However, the results of other renal function tests were similar in both groups. After respective treatments, suppressed plasma renin activity and elevated plasma aldosterone concentration were improved in all patients. In addition, patients of both groups showed normal response of the renin-aldosterone system following diuretic and dietary induced sodium and volume depletion. Based on these findings, renal or renovascular lesions appear to play an important role in the pathogenesis of maintenance of hypertension in this disorder after respective treatments.
 ...
PMID:Therapeutic results of primary aldosteronism with special reference to renal or renovascular lesions. 328 72


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>