1/70. The elevated serum alkaline phosphatase--the chase that led to two endocrinopathies and one possible unifying diagnosis.A 39-year-old Chinese man with hypertension being evaluated for elevated serum alkaline phosphatase (SAP) levels was found to have an incidental right adrenal mass. The radiological features were characteristic of a large adrenal myelolipoma. This mass was resected and the diagnosis confirmed pathologically. His blood pressure normalised after removal of the myelolipoma, suggesting that the frequently observed association between myelolipomas and hypertension may not be entirely coincidental. Persistent elevation of the SAP levels and the discovery of hypercalcaemia after surgery led to further investigations which confirmed primary hyperparathyroidism due to a parathyroid adenoma. The patient's serum biochemistry normalised after removal of the adenoma. The association of adrenal myelolipoma with primary hyperparathyroidism has been reported in the literature only once previously. Although unconfirmed by genetic studies this association may possibly represent an unusual variation of the multiple endocrine neoplasia type 1 syndrome.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/70. 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 = pressure (Clic here for more details about this article) |
3/70. Severe non-infectious circulatory shock related to hypopituitarism.The authors report a case of refractory non-infectious circulatory shock with catecholamine and massive fluid loading-resistant features related to hypopituitarism. A 76-year-old man was admitted for shock after suffering from gastroenteritis for 3 days. He was pale and had sparse axillary and pubic hair and small testes. Right catheterization showed shock with low preload pressure and a low oxygen extraction ratio relevant for septic shock. Ultrasound tomography revealed a distended gallbladder due to a stone without peritoneal effusion. A non-inflammatory hydrops of the gallbladder was removed surgically. No microorganism was isolated. Cerebral computed tomography (CT) scan showed a pituitary mass. In the post-surgical period the shock became uncontrollable. Cortisol replacement therapy was instituted and clinical and hemodynamic improvement occurred after 2 h. Hormonal screening on admission before catecholamine administration showed a major decrease in all the hypothalamic-pituitary hormone concentrations. The patient died on day 15 with multiple organ failure. hypopituitarism, probably owing to pituitary adenoma, was the only disease identified in this case. hormone replacement therapy dramatically improved the clinical and hemodynamic status, although the role of an abdominal sepsis could not be eliminated. Arguments that pituitary hormone deficiency might increase the hemodynamic consequences of adrenal deficiency are discussed.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
4/70. aldosterone-producing adenoma without hypertension: a report of two cases.Normotensive primary hyperaldosteronism is exceedingly rare. We report two new cases of this syndrome in two middle-aged women, one of Asian origin. The presenting signs were tetany in one case and an adrenal mass in the other. Neither patient had hypertension, despite repeated measurements with a manual armlet. A typical biological profile of primary hyperaldosteronism was demonstrated in both patients, including hypokalemia with inappropriate kaliuresis, elevated resting plasma aldosterone, and undetectable plasma renin activity. The circadian rhythm of blood pressure was studied by ambulatory monitoring pre- and post-operatively. It confirmed the lack of hypertension, but the circadian rhythm of blood pressure was lost before surgery in one patient. Surgical removal of the histologically typical aldosterone-producing adenomas normalized the kalemia. The main finding in these two patients was spontaneously low blood pressure in the post-operative period. This suggests that excess aldosterone induced relative hypertension in these patients whose blood pressure was spontaneously very low. Genetic screening for dexamethasone-sensitive hyperaldosteronism was negative in both patients.- - - - - - - - - - ranking = 4keywords = pressure (Clic here for more details about this article) |
5/70. Tension pneumocranium, a rare complication of transsphenoidal pituitary surgery: Mayo Clinic experience 1976-1998.We describe four cases of symptomatic pneumocranium, a rare, potentially life-threatening complication of transsphenoidal pituitary surgery. Symptomatic pneumocranium manifested as impaired mental status, headaches, and grand mal seizures, early in the postoperative course after transsphenoidal pituitary surgery. Furthermore, a Cushing response, including systemic hypertension and bradycardia (secondary to intracranial hypertension) was seen, which has not been previously described in association with symptomatic pneumocranium. We describe a previously unreported risk factor for tension pneumocranium, untreated obstructive sleep apnea. Other factors predisposing to tension pneumocranium in our patients included: cerebrospinal fluid leaks, postoperative positive-pressure mask ventilation, large pituitary tumors, and intraoperative lumbar drainage catheters. Surgical drainage of the pneumocranium and repair of any coexistent cerebrospinal fluid leak markedly improved neurologic status. Symptomatic pneumocranium occurring early in the postoperative course after transsphenoidal pituitary surgery is rare, but prompt recognition and treatment of this condition can be life-saving.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
6/70. Primary aldosteronism in pregnancy.Aldosteronism is a rare complication of pregnancy. We report a case of a 26-year-old woman who became pregnant soon after a diagnosis of primary aldosteronism due to left adrenal adenoma was made. Only oral potassium supplementation was required in addition to routine prenatal care until 36 weeks' gestation. Subsequently, antihypertensive medication was needed to control elevated blood pressure. A healthy male infant was delivered by cesarean section because of abruptio placentae. The postoperative course was uneventful. Left adrenalectomy was conducted eight months after delivery under laparoscopic visualization. In this case report, we discuss management of aldosteronism in pregnancy and review the literature.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
7/70. Concurrent secretion of aldosterone and cortisol from an adrenal adenoma - value of MRI in diagnosis.A 43-year-old female with a 24-years history of hypertension presented for further investigation and management of primary hyperaldosternoism. Postural studies were not conclusive and magnetic resonance (MR) imaging demonstrated a 27 x 18 mm lesion of the right adrenal gland which showed no signal loss during in and out of phase imaging. Although these appearances were considered to be atypical of those seen on MR in patients with aldosterone producing adrenal adenomas the patient underwent an adrenalectomy with removal of a 3 x 3 x 2 cm right adrenal mass. Post-operatively she became hypotensive and a 0900 hours serum cortisol was undetectable (< 50 nmol/l), consistent with adrenal insufficiency. Following the administration of hydrocortisone there was normalization of the blood pressure and subsequent adrenal stimulation tests confirmed the presence of functioning adrenal tissue albeit with an inadequate response. Cortisol measurement from preoperative samples revealed loss of normal diurnal rhythm whereas DHEAS levels both pre and postoperatively were undetectable, consistent with ACTH supression resulting from autonomous cortisol secretion in addition to aldosterone. Concurrent secretion of cortisol should always be considered in Conn's adenomas particularly when atypical radiological features are present.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
8/70. Non-secreting atypical parathyroid adenoma.Parathyroid tumors can be divided in adenomas and carcinomas, usually detected by hypercalcemia. We report a case of parathyroid adenoma in a young man, who complained of a pressure in the left neck region. physical examination revealed a firm mass in the neck, without lymphnodes. Although Ca (9.7 mg/dl), phosphorus (3.3 mg/dl) and intact-PTH (49 pg/ml) were normal, imaging techniques (computed tomography scan and sestamibi substraction scan) suggested that the mass could arise from the parathyroid gland. histology and immune staining for chromogranin and parathyroid hormone confirmed the parathyroid nature of the mass. Histological criteria defined the lesion as an atypical parathyroid adenoma. We review the pathology, diagnosis and treatment of parathyroid adenomas in its non-secreting atypical form.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
9/70. Severe hypertension and massive osteoporosis as presenting symptoms of Cushing's syndrome.Though Cushing's syndrome is a well-known clinical problem in terms of side effects of steroid therapy, endogenous Cushing's syndrome is a relatively rare diagnosis. We treated a 27-year-old patient who presented with severe hypertension and massive osteoporosis. We could diagnose a central cushing syndrome by endocrinological function tests which, in retrospect, existed undiagnosed for more than 5 years. However, magnetic resonance imaging did not display an adenoma neither of the hypophysis nor of the adrenal glands. During explorative surgery, a cylindric microadenoma of the pituary gland was found and excised. After surgery, the blood pressure returned to normal, making further antihypertensive treatment unnecessary.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
10/70. aldosterone-producing adenoma with prolonged amelioration by dexamethasone.Secretion of aldosterone from aldosterone-producing adenoma (APA) is to some degree under the control of ACTH and the suppressible effect of glucocorticoid on plasma aldosterone concentration (PAC) and blood pressure has been reported to be transient. We report a rare case of aldosteronism due to APA in which PAC and blood pressure were well controlled with small dose dexamethasone for over one year. No chimeric gene of glucocorticoid-remediable aldosteronism (GRA) was found in dna of APA and leukocytes from peripheral blood and 17alpha-hydroxylase deficiency (17-OH-D) was ruled out by endocrinological examinations, this case indicates the possibility of an unknown mechanism of ACTH-dependent APA.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
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