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Query: UMLS:C0018681 (headache)
56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Pheochromocytomas arise from chromaffin tissue, usually in the adrenal medulla, and are a cause for curable hypertension. Nearly all patients with this tumor are symptomatic, the most common symptoms being headache, palpitations and inappropriate perspiration. Diagnosis is confirmed by finding high levels of plasma catecholamines or increased excretion of catecholamine metabolites (metanephrines, vanillymandelic acid) in the urine. Localization of tumors(s) is important for the surgeon and is accomplished by CT scan, 131I-metaiodobenzylguanidine scintiscans or abdominal aortography. Treatment is surgical extirpation by an experienced team after depleted plasma volume has been replenished. Ten percent of tumors are malignant, 10% are bilateral in the adrenal medullae and 10% are extra-adrenal.
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PMID:Diagnosis and management of pheochromocytoma. 286 7

Fifteen migraine sufferers and a control group were checked for the circadian rhythm of urinary catecholamine and VMA excretion. These data were compared with the typical variations in haemodynamic balance already encountered in migraine sufferers. It is felt that this connection is an essential element in the pathogenesis of headaches.
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PMID:[Desynchronization of noradrenaline and hemodynamic equilibrium in migraine subjects]. 373 82

A patient with intrapericardial pheochromocytoma is presented and the literature on this subject is reviewed. The patient was a 45-year-old housewife who was first referred to Keio University hospital in February 1981 for a 6-year-history of headaches, palpitations and excessive sweating. An episode of hypertensive crisis was observed during surgery for myoma uteri 3 months before her admission. On physical examination, the patient was a normal-appearing, pleasant woman. Supine blood pressure was 130/80 mmHg and standing blood pressure was 124/72 mmHg. Results of routine laboratory studies (including fasting serum glucose and calcium determinations and thyroid function tests) were normal. A 24-hour-urine collection showed VMA level of 10.5 mg and noradrenaline of 809 micrograms. Also repeated measurements of plasma noradrenaline on supine position revealed averages of 2.45 ng/ml. These high levels of catecholamines, clinical manifestations, and provocative tests including a metoclopramide test were strongly suggestive of pheochromocytoma, and further studies for determination of the localization were performed. Results of computed tomography (CT), adrenal scintigraphy using 131I-adosterol and selective venous sampling for catecholamines were equivocal. Then she was followed in an outpatient clinic and treated with alpha- and beta-blocker. In October 1985, she was readmitted to our hospital because of paroxysms which had gradually increased in frequency and severity. Extensive venous sampling in order to determine the localization of the tumor was performed after demonstration of an increased uptake area in the anterior thorax in 131I-metaiodobenzylguanidine scintigraphy. Further, an anterior mediastinal mass was identified in the CT scan of the chest with contrast material. Finally, a thoracic angiogram revealed that the tumor was highly vascular and was fed by the branch of the left internal mammary artery. In January 1986, the patient underwent middle sternotomy with cardiopulmonary bypass. A 4.5 by 3.5 by 3.0 cm soft, dark brown tumor was found on the anterior surface of the heart and excised without difficulty. The tumor seemed to arise from the root of the aorta and main pulmonary artery, and extended inferiorly over the anterior surface of the right ventricle. It was also fed by branches of the right coronary artery. Histologically, the resected tumor was compatible with pheochromocytoma. The tumor content of noradrenaline was 3.34 mg/g wet tissue; however, adrenaline and dopamine were not detected. The postoperative course was uneventful.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Intrapericardial pheochromocytoma: a case report and review of the literature]. 380 33

Phaeochromocytomas are uncommon among patients with hypertension, and sometimes occur in persons without known hypertension, but are important to detect because they are often lethal but commonly curable, and because they are a clue to the presence of associated conditions. Paroxysmal symptoms (especially headache, palpitations, diaphoresis and anxiety), hypertension that is intermittent, unusually labile or resistant to conventional therapy, and conditions known to be associated raise the clinical suspicion of phaeochromocytoma. Biochemical confirmation is commonly achieved by measurement of urinary catecholamines, metanephrines or VMA. Plasma noradrenaline and adrenaline measurements may be superior to measurements of urinary catecholamine metabolites, but strict attention to the details of sample collection, handling and storage, the many sources of possible biological variation and the effects of drugs is critical if diagnostic error is to be avoided. Patients should be evaluated in the drug-free state if at all possible. Anatomical localization, in the abdomen in the vast majority of cases and usually in the adrenal medullae, can generally be accomplished with computed tomographic scans. Bilateral adrenomedullary tumours are the rule in familial phaeochromocytoma. Most phaeochromocytomas are benign and can be excised totally after medical preparation with an alpha-adrenergic antagonist.
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PMID:Phaeochromocytoma. 389 9

A 29-year-old female who had undergone resection of an abdominal paraaortic pheochromocytoma weighing 33 g at the age of 20 had had severe headaches, hypertension and hyperhidrosis 3 years prior to the surgery. Postoperatively, her symptoms completely disappeared and urinary catecholamines were normalized. She was well and had married and had had 2 children. She was admitted to our hospital on August 22, 1982, for further evaluation of hypertension (154/100), which had been diagnosed 2 months previously. Endocrinological studies confirming the presence of a pheochromocytoma were as follows: 1) Plasma noradrenaline level was significantly elevated to 1750 pg/ml. 2) Urinary catecholamine and their metabolites (Metanephrines and VMA) were markedly elevated. Her blood pressure was borderline hypertension and its diurnal rhythm was lost. Her blood pressure decreased to normal values after the oral administration of labetalol (100 mg). Plasma noradrenaline level was still high at 180 minutes after the oral administration of clonidine (150 micrograms). Hypertensive response to insulin-induced hypoglycemia (regular insulin 0.1 u/kg i.v.) was observed, but blood pressure returned to normal after the infusion of glucose alone. Hypertensive response to both metoclopramide (5 mg i.v.) and sulpiride (50 mg per os) was observed accompanying the significant elevation of plasma noradrenaline. Computed tomography and ultrasonography revealed a tumor localized between the aorta and the vena cava inferior. Selective venous sampling also revealed an intrathoracic pheochromocytoma. On October 8, 1982, a 28 g mass was removed from the mediastinum just above the diaphragma. Histologically, it was typical of a pheochromocytoma. Electron microscopy showed large polygonal cells with numerous large secretory granules characteristic of noradrenaline-granules. Postoperative blood pressure was normal, but repeated measurements of plasma and urinary catecholamines were still slightly high. We, therefore, followed her case carefully at our out-patient clinic. In order to clarify the mechanism of catecholamine release by metoclopramide and sulpiride, tissue cultures of removed pheochromocytoma with and without these drugs were carried out. The in vitro studies revealed that metoclopramide released noradrenaline eight-fold and sulpiride 13-fold as compared with noradrenaline in a control medium. We concluded that both drugs stimulated catecholamine secretion directly from the tumor and thus, careless administration of these drugs should be avoided when pheochromocytoma was suspected, large or small. Finally, the rate of tumor growth seemed to be very slow because it took 9 years to ach
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PMID:[Case of intrathoracic pheochromocytoma occurring 9 years after resection of intraabdominal paraaortic pheochromocytoma: effect of metoclopramide and sulpiride on catecholamine secretion in vitro]. 666 41

An Amerindian girl with Friedreich's ataxia presented at the age of 14 years with intermittent bifrontal headaches and abdominal aching, often associated with nausea and recurrent vomiting and an evanescent pink, blotchy rash on the upper trunk. In these attacks she also had hypertension up to 210/160 mm Hg. Renal function studies, including intravenous pyelogram and angiography, were normal. Plasma renin activity (2.5 ng/ml/hr) was also normal. Total body CT scan was negative for phaeochromocytoma, and repeated estimations of 24-hour excretion of urinary VMA were normal or borderline high. Levels of total catecholamines in 24-hour urine were normal twice, but two random specimens during the paroxysmal episodes contained abnormally high levels of norepinephrine and dopamine. Plasma catecholamine concentrations were increased but not as high as with phaeochromocytoma. Blood pressure monitoring demonstrated marked fluctuations with position and temperature. A clonidine suppression test showed a substantial fall of plasma catecholamine levels, consistent with dysautonomia and not with phaeochromocytoma. It is concluded that the patient has dysautonomia of central origin, probably as a manifestation of Friedreich's ataxia. These findings are discussed in relation to the recent demonstration of increased levels of plasma catecholamines in that disease.
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PMID:Friedreich's ataxia with dysautonomia and labile hypertension. 670 98

Eight cases of pheochromocytoma in children were managed between 1958 and 1978. Headache, visual blurring, sweating, and hypertension were the most common findings. One patient presented with a hypertensive crisis during appendectomy. Three other children had a family history of pheochromocytoma and Hippel-Landau disease, thyroid carcinoma, and renal stones. The diagnostic approach was based on a high suspicion of the disease from the clinical picture. The most reliable laboratory investigations were urinary VMA, metanephrine, and catecholamines. I.V.P. and angiography were successful in localizing the tumor. In 4 cases, CT scan localized the tumor, although a second tumor was not seen in 1 case. Preoperative control of hypertension was achieved with phenoxybenzamine, propanolol, apresoline and reserpine in seven cases. The main anesthetic drugs used were pentothal, methoxyflurane, nitrous oxide, Innovar, and pancuronium. Tumors were located in the right adrenal in four, the left adrenal in two, both adrenals in one, and bilateral para-aortic sites in one. In two cases, hypertensive episodes during manipulation of the tumor were controlled with phentolamine. Ligation of the venous drainage from a tumor was associated with a sudden sustained fall in systemic blood pressure in six cases. In two, blood pressure remained elevated until a second tumor was found and removed. Hypotension was then corrected by the rapid infusion of fluid and blood, and vasopressors were not used. There were no postoperative complications and all children remained normotensive during the follow-up. The tumors of seven of the patients were benign. One tumor showed a low grade malignancy.
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PMID:Pheochromocytoma in children--an update. 741 63

Between 1981 and 1993, 14 patients with tumoral adrenal disease were diagnosed and treated in the Urology Services of both Hospitals. Nature of the adrenal disease was functional in 8 patients (two carcinomas and six pheochromocytomas) and non-functional in the rest (three carcinomas, two adenomas and one myelolipoma). Reference is made to the clinical manifestations and laboratory tests related to hormonal activity, depending on whether the tumour is functional or non-functional, chromaffin or non-chromaffin. Patients with functional carcinomas had Cushing's syndrome, with very clear virtilization signs. Urine 17-hydroxycorticosteroid, 17-cetosteroides and cortisol were all increased, same as plasma cortisol. Patients with pheochromocytomas had hypertension and headaches (six patients), sweating (five patients), anxiety (four patients) and loss of weight (two patients). All of them had increased urine vainillylmandelic acid and catecholamines. Clinical signs and symptoms of non-functional tumours were related to bulk growth and size (in the three carcinomas), and sometimes was highly anodyne, or even absent (in the two adenomas and the myelolipoma), the cause of discovery being accidental during an ultrasound examination. An analysis is made of the different imaging diagnostic procedures performed, such as IVU (performed in 13 patients) with a 38.4% resolutory power; ultrasound (performed in 11 patients) with an 81.8% resolution; CAT (performed in 6 patients) with a 100% resolution capacity and arteriography (performed in 3 patients) with a 100% resolution power, although patients had previously undergone ultrasound and CAT.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Clinical features, diagnosis, and treatment of tumor lesions arising in the adrenal gland]. 777 Dec 35

Cluster headache is a rare, very severe disorder that is clinically well characterized with a relatively poorly understood pathophysiology. Alterations of the hypothalamic-pituitary axis due to chronobiological changes, such as typical temporal pattern of both cluster periods and attacks, point to a central etiopathogenesis. Multiple local and systemic autonomic symptoms are compatible with an altered balance of the sympathetic and parasympathetic nervous system. In this connection, too, a central etiology is postulated. To evaluate the activation of the sympathetic nervous system, in 12 cluster headache patients we investigated the plasma catecholamines norepinephrine and epinephrine four times a day (7.00, 12.00, 17.00, 23.00) in the cluster period. In the cerebrospinal fluid we determined the transmitters norepinephrine, epinephrine, dopamine and the metabolites homovanillic acid (HVA), vanillymandelic acid (VMA) and 5-hydroxyindoleacetic acid (5-HIAA). Values of plasma norepinephrine in the morning (p < 0.01), in the evening (p < 0.01) and the daily mean value (223.8 = 58.3 nmol/ml) were significantly decreased in the cluster headache group in comparison to the control group (328.8 = 53.0 nmol/ml, p < 0.01). The plasma epinephrine showed no significant changes. In the CSF of cluster headache patients norepinephrine (p < 0.05), HVA (p < 0.01), and 5-HIAA (p < 0.01) were significantly decreased. Plasma norepinephrine was correlated with CSF values of HVA and 5-HIAA. The longer the duration of the disease, the lower the values of HVA and 5-HIAA in the CSF of cluster headache patients. Moreover, plasma norepinephrine showed a significant correlation with the duration, the intensity and the frequency of the attacks. The results of this study implicate decreased activity of the sympathetic nervous system with alteration of circadian rhythmicity during the cluster period. The decreased CSF transmitter values may support the hypothesis of a central etiopathogenesis of cluster headache. Moreover, plasma norepinephrine seems to be involved in triggering and continuing the attacks. The anatomical region in which this interface of sympathetic and neurogenic inflammatory processes might be located is the trigemino-vascular system.
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PMID:[Reduced sympathetic nervous system activity during the cluster period of cluster-headache]. 870 Dec 43


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