Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 12-year-old boy with a norepinephrine-secreting pheochromocytoma that caused hypertension resistant to oral alpha adrenergic blockade is reported. Resistance to alpha adrenergic blocking agents developed when the patient's daily propranolol dosage was lowered from 10 to 1 mg/kg. Subsequently, alpha methyl tyrosine, an inhibitor of tyrosine hydroxylase, the rate-limiting enzyme in catecholamine biosynthesis, controlled the patient's blood pressure and was associated with reduction in total urinary catecholamine excretion. Norepinephrine content of the tumor and uninvolved adrenal gland removal at surgery was reduced. These findings confirm that alpha methyl tyrosine inhibited in vivo synthesis of catecholamines.
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PMID:Childhood pheochromocytoma: treatment with alpha methyl tyrosine for resistant hypertension. 1 59

Fifteen patients with the diagnosis of multiple endocrine adenomatosis, type II, syndrome (MEA II) were reported from a single center to discuss the dilemmas of early detection and treatment of the adrenal medullary, thyroid, and parathyroid gland diseases. Ten patients came from three families. Three of the patients died, none in hypertensive crisis. Bilateral adrenal medullary disease was present in six patients. Five patients with proved pheochromocytoma had hypertension. All had diagnostic urinary catecholamine values. Nine normotensive patients without proved pheochromocytoma but in a high-risk category for adrenal medullary disease, have multiple suspicious urinary cathecholamines suggestive of adrenal medullary hyperplasia. Bilateral adrenalectomy is recommended for proved adrenal medullary disease in the MEA II syndrome. Medullary carcinoma of the thyroid gland was found in 13 patients and is believed to be present in two others. Five of the proved cases were occult, being discovered by elevation of pentagastrin-stimulated serum calcitonin levels, justifying total thyroidectomy. Parathyroid hyperplasia was found in three patients with preoperative hypercalcemia and in four others with preoperative normocalcemia. Conservative treatment of parathyroid gland hyperplasia in the MEA II syndrome is substantiated. Metachronous phenotypic expression of the syndrome components was significant.
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PMID:Dilemmas in the early diagnosis and treatment of multiple endocrine adenomatosis, type II. 1 7

Pheochromocytoma is an infrequent cause of hypertension. In spite of its rarity, pheochromocytoma has assumed notorious importance because or a wide variety of clinical features associated with this syndrome. Hypertension remains the most important clinical lead. The presence of other features, such as severe headache, perspiration, palpitations or orthostatic hypotension, makes the diagnosis of pheochromocytoma likely. The diagnosis of this condition can be made with greater certainty than that of any other form of secondary hypertension. Pharmacologic tests are no longer used for evaluation purposes. Biochemical tests are the most important aids to diagnosis, provided highly specific methods are used to determine the levels of urinary catecholamines or their metabolites. Interference by various drugs should be avoided. Most of the pheochromocytomas are found in the abdomen, predominantly in the adrenals. Successful outcome of surgery depends critically on adequate preoperative preparation of the patient with adrenergic blocking drugs and proper intraoperative care. Surgery should only be performed in an institution with experience in treating these tumors. Invasive localizing procedures could be dangerous in patients with pheochromocytoma and are best avoided. Medical therapy proves to be quite successful for those patients who are unable or unwilling to undergo surgery and for those with demonstrated malignant tumor. The postoperative course in most instances is uneventful. The physician should be aware of familial forms of pheochromocytomas and screen all the hypertensive members of the patient's family for the presence of this condition. Pheochromocytoma, with its multiple facets, presents a challenge to the clinician; however, with prompt diagnosis and proper treatment, pheochromocytoma can often be cured. The improved management of patients with this potentially lethal condition is the result of better knowledge of biochemical pharmacology, improved preoperative, surgical and postoperative care.
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PMID:Pheochromocytoma--recognition and management. 4 12

The frequency of latent disorders of glucose regulation during pheochromocytoma, is evaluated at 75% of cases. Detailed analysis of 83 cases with a diabetic state, gave the following results: insulin dependent diabetes, 37 cases. Non-insulin dependent, 14 cases. Latent diabetes, 32 cases. The characteristics of the insulin-dependent diabetes were not always suggestive. Insulin dependency was, however, unusual above a certain age. We noted loss of weight in spite of good control of the diabetes, the absence of acidosis and ketosis contrasting with rapid loss of weight. In fact, it is above all the hypertension which should lead to diagnosis. Surgical operation, cures or improves considerably the diabetic state, thus proving the symptomatic nature of this diabetes.
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PMID:[Diabetes mellitus in pheochromocytoma]. 18 6

Myocardial scinitgrams, using 99mTc-stannous pyrophosphate, showed an acute posterior infarction and an abnormally placed left kidney in a 24-year-old hypertensive man; Further study revealed that the kidney was displaced by a mass later proven to be a pheochromocytoma. The latter was the cause of his hypertension and probably instigated the acute myocardial infarction.
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PMID:Renal displacement visualized on myocardial scintigram: case report. 19 80

Hypertension in children has been reported with increasing frequency because of increased awareness of its occurrence by clinicians. Renovascular lesions have been stressed in the past. However, in recent years, a number of nonrenovascular renal lesions have received attention and form the basis for this report. Unilateral chronic atrophic pyelonphritis, segmental unilateral pyelonephritis, the Ask-Upmark kidney, and unilateral renal hypoplasia have been associated with curable hypertension. The subject of juxtaglomerular cell hyperplasia, which has variably been reported in these cases, is reviewed. Ureteral obstruction, in the form of uretero-pelvic or ureterovesical junction obstruction, solitary renal cysts, the unilateral multicystic kidney, renal trauma, and renal tumors (Wilms' tumor and juxtaglomerular cell tumors) may also be associated with hypertension in children. Pheochromocytoma must be ruled out in all cases. Because of renewed interest, these nonrenovascular renal causes of hypertension are now likely to be diagnosed with increased frequency.
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PMID:Nonrenovascular renal hypertension in children. 20 75

The authors discuss the radiological signs in 18 patients with an adrenal tumour. 7 of them had a Cushing's syndrome, 10 others a phaechromocytoma and the last had primary hyperaldosteronism. The diagnosis was made from the history, the clinical picture, hormone estimations and pharmacodynamic tests, whilts in the majority of cases the tumour was localised by radiodiagnosis. In all cases, there was hypertension, permanent in tumours of the adrenal cortex, paroxysmal or permanent in the cases of pheochromocytomas. We emphasise the importance of retro-pneumoperitoneum, as the radiological investigation of choice, in the localisation of adrenal tumours, especially pheochromocytomas, and in Cushing's syndrome. In cases of pheochromocytoma, one should follow carefully the blood pressure, during special radiological investigations, in view of the danger of a sudden rise or fall in blood pressure, the first is treated with phentolamine, the second with noradrenaline solution. Finally, a scan using I 131 19-iodocholesterol may be valuable in diagnosis and localisation of adrenal tumours; it has in particular given very encouraging results in the differential diagnosis of adrenal tumours with the clinical presentation of Cushing's disease.
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PMID:[Radiological signs of tumours of the adrenal glands (author's transl)]. 21 64

A 51-year-old female was diagnosed preoperatively to have a pheochromocytoma producing ACTH. This diagnosis was based upon her paroxysmal hypertension, hyperpigmentation, and hypokalemia. Elevated levels of serum and urine corticosteroids, plasma ACTH, urinary VMA, and catecholamines fell after a right adrenal pheochromocytoma was removed. Subsequently this tumor was found to have a high content of ACTH. Review of the literature indicates a mortality rate of 57% for this syndrome. Proper preoperative recognition and management can result in total cure.
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PMID:Ectopic ACTH syndrome due to pheochromocytoma: case report and review of the literature. 22 80

Neurohumoral mechanisms operating via the catecholamines are discussed in their relationship to such hypertensive diseases as pheochromocytoma and labile and established essential hypertension. 2. In pheochromocytoma, diagnosis depends almost entirely on identification of increased amounts of catecholamine metabolites in the urine. Because of the danger, manipulative or invasive procedures both for diagnosis and during surgery should be kept at a minimum. 3. In established essential hypertension, reactivity to norepinephrine and plasma norepinephrine are increased, whereas norepinephrine uptake and apparent secretion rate are decreased. 4. In labile essential hypertension, reactivity to epinephrine and probably plasma epinephrine are increased and uptake of epinephrine decreased. 5. Labile hypertension with all its characteristics may or may not coexist with established essential hypertension with all its features. 6. The sympathetic nervous system is also involved in other types of hypertensive disease. Many patients with renovascular hypertension as well as with primary and secondary hyperaldosteronism also have essential hypertension. Angiotensin II affects the sympathetic nervous system and the juxtaglomerular apparatus appears to be beta adrenergic receptor activated, at least in part.
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PMID:Neurohumoral mechanisms in hypertension. 23 64

A 29-year-old man with a marfanoid habitus, peculiar mucosal neuromas of the lips and tongue, high arched palate, hyperplastic corneal nerves, and hypertension was found at operation to have medullary carcinoma of the thyroid, parathyroid hyperplasia, and pheochromocytoma. These symptoms and findings are characteristic of multiple endocrine adenomatosis (MEA IIb) syndromes.
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PMID:Multiple endocrine adenomatosis type IIb. Diagnosis and treatment. 24 66


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