1/13. adrenocortical carcinoma in two female children.adrenocortical carcinoma is a rare tumor in children. This tumor is more likely to be hormonally active in children than in adults and tends to cause a variety of symptoms, which may mimic other benign endocrinopathies. These tumors are usually diagnosed at advanced stages and portend a dismal prognosis. We describe two cases of adrenocortical carcinoma. One child presented with Cushingoid symptoms secondary to hypercortisolism, including amenorrhea, hirsutism and weight gain. The other child presented with precocious puberty. Both children underwent resection of the tumors. We describe their presenting symptoms, postoperative course, adjuvant therapy and clinical course. Pertinent literature regarding the anatomy of the adrenal gland, pathology of adrenocortical carcinoma, factors influencing outcome, diagnostic modalities and treatment, are discussed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/13. Stress fractures in young athletic women: case reports of unsuspected cortisol-induced osteoporosis.Stress fractures in the female athlete are common events, usually occurring in the lower limb and less often in the pelvic girdle. Two cases are presented of young women athletes who presented with initial lower limb stress fractures, but subsequently fractures of the pelvis and hip thought to be associated with their athletic activity. After careful medical evaluation, they were diagnosed with Cushing's syndrome. One patient had a microadenoma of the pituitary gland secreting excessive amounts of ACTH, and the other had a benign adenoma of the left adrenal gland. Both women had significant decreases in their spinal mineral density. After treatment, partial reversal of these spinal losses occurred. Although stress fractures in the female athlete might be common and thought to be associated with problems of amenorrhea, presentation of unusual anatomical sites for these fractures necessitates a more thorough evaluation for correctable secondary causes.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
3/13. Macronodular adrenocortical hyperplasia in a postmenopausal woman.This case report describes the diagnosis of Cushing's syndrome due to macronodular adrenal hyperplasia in an elderly woman who presented with fatigue, muscle weakness and oedema, and recent excessive bruising. Long-standing disease and comorbidity precluded adrenalectomy. Despite treatment with metyrapone and diuretics, the patient died after two months hospitalisation. Postmortal examination revealed overexpression of luteinising hormone (LH) receptors in the adrenal glands, suggesting that the postmenopausal rise in LH may have a role in adrenal hyperplasia and hypercortisolism.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
4/13. Endocrinopathies of hyperfunction: Cushing's syndrome and aldosteronism.Increased function of the adrenal cortex is a normal response in times of physiologic and psychologic stress. Adrenal cortical secretions (e.g., glucocorticoids, aldosterone) orchestrate a multitude of internal processes aimed at maintaining homeostasis and psychologic integrity. Many patients admitted to a critical care unit will manifest some increase, even minor, in adrenal function. However, excessive secretions of these hormones can have a lethal effect of fluid and electrolyte balance, energy metabolism, and immune function. Cushing's syndrome denotes a disorder characterized by increased circulating levels of glucocorticoids (primarily cortisol). An easily recognizable disorder, it may arise from pathology of the adrenal cortex or the anterior pituitary glands, ectopic secretions from a nonendocrine tumor, or from excessive doses of exogenously administered glucocorticoids. Cushing's syndrome is rarely an admitting diagnosis to critical care but is a disorder that can seriously affect recovery from coexisting illnesses if not treated. Aldosteronism, although rare, will often be diagnosed after admission to a critical care unit for management of troublesome hypertension, hypokalemia, congestive heart failure, and various dysrhythmias. Suspicion of the diagnosis should always arise when these manifestations occur, particularly when hypokalemia is refractory to potassium supplementation. Without timely diagnosis and treatment, these patients will succumb to lethal dysrhythmias.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
5/13. Primary aldosteronism. A study in contrasts.Two patient with primary aldosteronism, one with a solitary adrenal adenoma and the other with bilateral nodular hyperplasia, are described. Both patients showed the classic features of primary aldosteronism in electrolyte and hormone patterns, but there were important differences in the biochemistry of their excised adrenal tissue. In addition, the injection of plasma from the patient with bilateral adrenal hyperplasia into the sheep's transplanted adrenal gland elicited a definite aldosterone secretory response, but there was no aldosterone response to the injection of plasma from the patient with a solitary adrenal adenoma. The findings support the hypothesis that an extra-adrenal stimulus may contribute to the pathogenesis of bilateral adrenal hyperplasia.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
6/13. 17 alpha-Hydroxylase deficiency. A combination of hydroxylation defect and reversible blockade in aldosterone biosynthesis.We have studied the hormonal secretion and excretion patterns in a patient with the XX type of 17 alpha-hydroxylase deficiency. In the untreated state, the patient's urine contained only those steroids which do not require 17-hydroxylation in their biosynthesis. aldosterone was not produced in the patient and the metabolic product of its immediate precursor, 18-hydroxy-11-dehydro-tetrahydrocorticosterone, was excreted in markedly elevated amounts. This apparent complete block in 18 oxidation was reversible upon long-term ACTH suppression within 27 days. Direct in vitro incubation of the patient's adrenal gland removed at operation demonstrated, 1) the complete lack of 17 alpha-hydroxylase activity, 2) the functional block in the ability to oxidize the hydroxyl group at the 18 methyl side chain. The addition of physiological concentrations of angiotensin to the incubation medium further showed, 3) angiotensin mildly stimulated the entire aldosterone biosynthetic pathway, 4) angiotensin directly stimulated the conversion of 18-hydroxycorticosterone to aldosterone. We propose that in this patient, 17-hydroxylase deficiency produced a decreased plasma concentration of cortisol, followed by stimulation of deoxycorticosterone production by ACTH. The resultant increase in extracellular fluid volume suppressed plasma renin activity. This resulted in a low plasma concentration of angiotensin ii which directly suppressed oxidation of 18-hydroxycorticosterone to aldosterone. This defect has been called corticosterone methyl oxidase defect type 2.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/13. Imaging of medullary thyroid carcinoma and hyperfunctioning adrenal medulla using iodine-131 metaiodobenzylguanidine.Scintigraphy with radiolabeled metaiodobenzylguanidine was performed in a patient with MEN Type IIa having a pheochromocytoma of the right adrenal gland, adrenomedullary hyperplasia of the left adrenal gland and a primary medullary thyroid carcinoma. The scintigraphic findings demonstrate visualization of all the above mentioned pathologies.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
8/13. Decreased deoxyribonucleic acid binding of glucocorticoid-receptor complex in cultured skin fibroblasts from a patient with the glucocorticoid resistance syndrome.A patient with the syndrome of glucocorticoid resistance was studied. A 27-yr-old woman initially was diagnosed as having Cushing's disease, based on the findings of high plasma ACTH and serum cortisol levels, increased urinary cortisol secretion, resistance to adrenal suppression with dexamethasone, and bilateral adrenal hyperplasia by computed tomography and scintigraphy of the adrenal glands. However, she had no signs or symptoms of Cushing's syndrome. During a 5-yr follow-up, no clinical abnormalities developed, although hypercortisolism persisted. End-organ resistance to cortisol was suspected. To explain the end-organ resistance to cortisol, the glucocorticoid receptors (GR) in peripheral mononuclear leukocytes and cultured skin fibroblasts from a forearm skin biopsy were characterized and compared with the results of similar studies in normal subjects. The patient's GR in whole cell assays had an increased dissociation constant (Kd). In the cytosol of cultured skin fibroblasts from the patient, there was also decreased binding capacity. The thermal stability and the sedimentation coefficient in a sucrose density gradient of the receptors in the cytosol of cultured skin fibroblasts from the patient and normal subjects were similar. GR complex activation, analyzed by deae-cellulose chromatography, was decreased in the patient. dna binding of the GR complex after temperature-induced activation was lower in the patient than in normal subjects. Nuclear translocation of GR complexes from the patient was also slightly decreased. These results suggest that the patient's glucocorticoid resistance was due to a decrease in the affinity of the receptor for glucocorticoids and a decrease in the binding of the GR complex to dna.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
9/13. Primary aldosteronism due to unilateral adrenal hyperplasia.A 45-yr-old man with hypertension, hypokalemia, low plasma renin, and hyperaldosteronism was studied. plasma and urine aldosterone were consistently above normal, remaining abnormally high even on a 300-meq sodium intake. plasma aldosterone had a marked circadian rhythm, which was correlated with plasma cortisol. aldosterone secretion was temporarily suppressed after dexamethasone administration and was stimulated by exogenous ACTH. The effect of posture was variable in the eight studies performed, possible due to episodic secretion of aldosterone observed near the sampling times of 0800 and 1200 h. Blood from the right adrenal vein contained 50--100 times more aldosterone than the left adrenal venous samples. The right adrenal gland was excised and found to contain many microscopic subcapsular nests of clear cells. plasma aldosterone, renin, and potassium returned to normal after surgery, and blood pressure fell to 120/75 over the next 8 months. Three years later, the patient is normotensive without drugs.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
10/13. Cushing's syndrome with bilateral multinodular adrenal hyperplasia. Ultrastructural, histochemical, and immunohistochemical study.An unusual case of Cushing's syndrome of a 59-year-old man with bilateral multinodular adrenal hyperplasia and microadenoma of the pituitary gland is presented. Failure to suppress plasma cortisol with large doses of dexamethasone may suggest autonomous growth of hyperplastic nodules of the adrenals, which were at first induced by prolonged stimuli of ACTH from the microadenoma of the pituitary gland. ACTH could not be detected in the microadenoma cells on paraffin sections, while Crooke's cells were strongly positive for ACTH. The interrelation between bilateral multinodular adrenal hyperplasia and pituitary microadenoma is discussed.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
| | Next -> |