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 17-year-old woman manifested fever, abdominal pain, headache, and hypertension caused by a solitary, benign pheochromocytoma. She also had hypercalcemia and elevated plasma immunoreactive calcitonin levels. After removal of the pheochromocytoma, calcium and calcitonin levels returned to normal. Studies of peripheral and tumor venous blood showed no excess or ectopic parathyroid hormone secretion, but the tumor contained and secreted calcitonin. Sporadic pheochromocytoma may secrete calcitonin and cause hypercalcemia by non-parathyroid hormone-mediated mechanisms. The potential is clearly present for confusion with multiple endocrine neoplasia, type 2 (medullary thyroid carcinoma, pheochromocytoma, and primary hyperparathyroidism).
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PMID:Pheochromocytoma associated with hypercalcemia and ectopic secretion of calcitonin. 46 64

In a 20 year period, from 1971 through 1991, 105 chromaffin tumours--excluding cervical ones--were operated by the same surgeon: 50 during the first 15 years and 55 during the last 5 years. Pheochromocytomas are defined as intra-adrenal chromaffin tumours, and paragangliomas--or ectopic pheochromocytomas--as of extra-adrenal location. Among those tumours, 30 were malignant (i.e. metastatic) and 75 benign. Among the 30 malignant tumours, 14 were ectopic, 2 occurred in a MEN II A setting and were bilateral, 2 were associated with liver adenoma and liver hemangioma respectively suggesting Von Hippel-Lindau syndrome, and one case was associated with a seemingly sporadic primary hyperparathyroidism. 9 out of those 30 malignancies were not associated with hypertension. Among 75 benign pheochromocytomas or paragangliomas, 10 were ectopic, 7 occurred in a MEN setting (6 type II, 1 type I). 3 patients without evidence of MEN or other neuroectodermal abnormalities presented bilateral pheochromocytoma, either synchronous (2) or metachronous (1). 7 cases occurred in a Von Hippel-Lindau syndrome (3 bilateral) and 4 in a neurofibromatosis setting (1 bilateral). 3 other cases were familial without evidence of MEN (including a case of triple tumour: bilateral and ectopic and another ectopic case). 2 other cases were associated with seemingly sporadic hyperparathyroidism. As a whole, in 34 of 75 benign pheochromocytomas or paragangliomas, the tumour was not intra-adrenal, unilateral and sporadic. Among those 75 tumours, 22 were not overtly hypertensive, including 10 out of the 41 seemingly intra-adrenal, solitary and sporadic. The pheochromocytoma, benign, intra-adrenal sporadic, hypertensive accounts for no more than 30% of the subphrenic catecholamine-secretin chromaffin tumours.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Can pheochromocytoma be considered a benign unilateral intra-adrenal sporadic hypertensive tumor ? Reflections on a series of 105 surgically treated subdiaphragmatic chromaffin tumors]. 134 86

A variety of vasoactive substances including biogenic amines, neuropeptide Y, somatostatin, enkephalin, ACTH, corticotropin-releasing hormone, growth hormone releasing hormone, vasoactive intestinal peptide, calcitonin, and atrial natriuretic factor have been extracted from intra-adrenal and extra-adrenal pheochromocytomas in men. Some of them appear to play an important role for the development of hypertension or clinical serious symptoms. However, informations on the molecular forms of other substances in pheochromocytomas are still limited, and precise amount of the peptides or hormones in the tumors has not yet been quantitated. Numerous in vitro or in vivo studies of this documented neoplasm over the years have been reviewed in this manuscript. Clinical analyses of early diagnosis, localization diagnosis, treatment of multiple endocrine neoplasia, preoperative and operative treatments are also evaluated in this paper. These informations will probably provide additional evidence for the multi-secretory APUD cells of neural crest origin and will contribute the therapy in patients with pheochromocytoma.
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PMID:[Pheochromocytoma--basic and clinical analyses]. 134 92

The multiple endocrine neoplasia syndromes are an association of tumours of 2 or more endocrine glands. Multiple endocrine neoplasia type 2b (MEN 2b) patients develop medullary thyroid carcinoma and pheochromocytomas as well as unique physical characteristics. Most commonly, MEN2b is inherited with an autosomal dominant pattern although sporadic cases are not uncommon. If untreated the disease may be lethal. The facial, oral and ocular characteristics are reliable markers of the disease. These patients give a history most commonly of slipped capital femoral epiphysis, hypertension and life-long diarrhoea and/or constipation. MEN2b is most commonly characterised by nodules on the anterior aspect of the tongue, thickened lips with nodules, thickened upper eyelids, broadened nasal bridge, thickened corneal nerves and dilated, symmetrical, pedunculated nodules on the cheek mucosa. The patient described has most of these characteristics. Radiographic features of the jaws which have not been previously described are reported. These include a markedly enlarged and bifurcated inferior alveolar canal and shortened roots of the lower incisor teeth. Due to the lethality of the disease, patients who present with the above physical characteristics must be further investigated to exclude MEN2b.
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PMID:Multiple endocrine neoplasia syndrome--type 2b. Case report and review. 135 Oct 93

Pheochromocytoma is an uncommon tumor in childhood; nevertheless, 20% of all pheochromocytomas are found in the pediatric population. Fifteen children have been treated in our institution from 1962 to 1990. One percent of patients referred over the same period for investigation of high blood pressure were found to have a pheochromocytoma. The majority of patients have hypertension. It varies in intensity and is paroxysmal in about half the patients. Many pediatric cases of unilateral, bilateral, extra-adrenal, familial, and recurrent pheochromocytomas have been reported. We reviewed our 28 years experience with this tumor and concentrated on the diagnosis, the preoperative and operative management, and the occurrence of the multiple endocrine neoplasia syndromes.
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PMID:Pheochromocytoma in children: 15 cases. 135 28

The differential diagnosis of hypercalcemia has expanded to over 25 separate disease states, with primary hyperparathyroidism and malignancy accounting for 80-90% of all hypercalcemic patients. Primary hyperparathyroidism comprises the majority of hypercalcemic patients among the ambulatory population, but malignancy accounts for up to 65% of such patients in the hospital. Factors favoring primary hyperparathyroidism include a family history of hyperparathyroidism or multiple endocrine neoplasia, a history of childhood radiation to the head and neck, the postmenopausal state, a history of renal calculi or peptic ulcer, hypertension, the induction of hypercalcemia by thiazides, or an asymptomatic patient with a prolonged, stable mild hypercalcemia. The usefulness of the serum calcium, parathyroid hormone, chloride, phosphorus, serum 25-OHD, and 1,25-(OH)2D, and urinary calcium in the differential diagnosis of hypercalcemia is discussed. The pitfalls of an excessive reliance on the serum PTH in diagnosing hyperparathyroidism are stressed. The discriminant values of the serum calcium, chloride, phosphorus, and parathyroid hormone are explored, with the serum parathyroid hormone, chloride, and calcium proving most useful in separating primary hyperparathyroidism from other forms of hypercalcemia. Multivariate discriminant analysis using the serum calcium, phosphorus, and chloride and the hematocrit achieves an accuracy of 95-98% and is the most economical method of identifying hyperparathyroidism. The addition of the amino-terminal or intact PTH assay increases the accuracy to 99% and is essential in the presence of renal insufficiency.
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PMID:Differential diagnosis of hypercalcemia. 176 70

During the past 20 years (1970-90), we had 24 patients with pheochromocytoma: 19 diagnosed clinically and 5 post-mortem. Their ages ranged from 17 to 74 (mean, 43.2 years). Males (n = 14) outnumbered females (n = 10), a 1.41:1 M:F ratio. A majority were symptomatic (95%), with a typical triad of headaches, palpitations and diaphoresis. Most frequent finding was hypertension (95%). It was sustained in 60% and paroxysmal in 35%. In 6 patients (25%) pheochromocytomas were bilateral, all familial. Fifteen were solitary adrenal tumors (63%); 3 (12.5%) were extra-adrenal: 2 intra-abdominal, and 1 cardiac paraganglioma of right atrium. Of 6 familial cases, 4 were associated to Von Hippel-Lindau (VHL) disease, while 2 were multiple endocrine neoplasia (MEN-II) patients. All familial cases were bilateral and in the adrenals. There were no malignancies. Among the 19 clinical cases pre-operative Dx was made by positive urine VMA or catecholamines urine levels: (95 and 100% sensitivity respectively). Preoperative visualization by CT or MRI was done in 62% of the most recent patients. In 5 earlier cases the diagnosis was made post mortem: 3 died of cerebral hemorrhage, 1 with a pons infarct and 1 with congestive heart failure (CHF). There were 2 post-operative deaths and another died 13 years later from thyroid medullary carcinoma. Of the 19 operated, 13 (68%) were cured. Thus pheochromocytomas retain considerable morbidity and some mortality. These rare tumors constitute a clinical diagnostic challenge yet a rewarding therapeutic experience for the alert physician.
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PMID:Pheochromocytoma: a twenty year experience at the University Hospital. 177 16

We present a case of unilateral adrenal medullary hyperplasia in a 63-year-old woman with clinical signs and symptoms of pheochromocytoma unassociated with multiple endocrine neoplasia. The surgically removed adrenal gland revealed diffuse medullary hyperplasia with multiple micronodules measuring up to 2 mm. The micronodules were composed of enlarged chromaffin cells with atypia, histologically similar to those of pheochromocytoma, forming small solid alveolar patterns separated by a fibrovascular stroma. Removal of the hyperplastic adrenal gland resulted in disappearance of paroxysmal nocturnal hypertension and palpitation. These results suggest that diffuse and nodular medullary hyperplasia is the precursor of pheochromocytoma.
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PMID:Adrenal medullary hyperplasia. Hyperplasia-pheochromocytoma sequence. 226 Apr 75

We describe a patient who presented with constitutional symptoms, severe hypertension, and purpuric lesions over the knees, thighs, and penis. The patient was eventually diagnosed as having multiple endocrine neoplasia type II, with cutaneous leukocytoclastic vasculitis. The cutaneous vasculitis persisted despite treatment with high-dose systemic corticosteroids, but rapidly resolved after the removal of bilateral pheochromocytomas. This case demonstrates cutaneous leukocytoclastic vasculitis in association with pheochromocytoma.
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PMID:Cutaneous vasculitis associated with pheochromocytoma. 226 Oct 6

Pheochromocytoma exists in black African countries with the same frequency observed in other places. The characteristics of our series of nine patients are the followings: an average age of 19, 6 years old, a sex-ratio of 1, 25, a paroxysmal high blood pressure for six patients, no MEN neither malignant tumors, the measures of V.M.A. done positively eight times, a retropneumoperitoneum scan, seven arteriographies, three echographies which were consistently positive, one death post surgery by collapsus consecutive to tumor excision, only one contralateral recurrence and positive followings in all other cases.
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PMID:[Pheochromocytoma in black Africa. Report of 9 cases]. 227 50


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