Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 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

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

The records of 18 patients with multiple endocrine neoplasia (MEN) type 2A in a family consisting of 87 members in 5 generations, were reviewed with respect to age at onset and length of survival in a study of the natural history of this syndrome. Of these 18 patients, 8 were alive following surgical treatment, while 10 had died without treatment. The clinical onset of medullary thyroid carcinoma was noticed as a nodule in the neck at between 17 and 34 years of age (mean 24 years); the onset of pheochromocytoma was characterized by palpitation, hypertensive attacks or recurrent headache at between 17 and 42 years of age (mean 27 years); and death occurred in 10 patients at between 23 and 67 years of age (mean 40.6 years). This information on the age at onset of individual clinical manifestations, and on the length of survival of gene carriers, may be useful for clinicians who are asked to assess the risk of the development of this syndrome in a genetically predisposed asymptomatic individual, because screening tests are often refused by members of Japanese families with MEN type 2A.
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PMID:The natural history of multiple endocrine neoplasia type 2A--a clinical analysis. 197 66

Omeprazole, a substituted benzimidazole, has been shown to be a potent inhibitor of gastric acid secretion in patients with Zollinger-Ellison syndrome (ZES). We review our experience, as well as the published data on 210 patients with ZES who have required omeprazole for control of gastric acid hypersecretion over the past seven years. The dose of omeprazole required in individual patients ranged from 10 to 180 mg/24 hr with 20-60% requiring a split dosage regimen. Omeprazole was effective in approximately 99% of the patients over a period ranging from 0.5 to 54 months. Twenty-four percent of patients required an increase in omeprazole dose, while 26% required a decrease in dose. Adverse effects attributable to omeprazole were reported in 2% of patients, and in all cases, they were mild (ie, rash, constipation, headache). There was no effect of omeprazole on serum gastrin concentration or on gastric endocrine cells in three studies. Although one patient with multiple endocrine neoplasia, type-I syndrome (MEN-I) in this series developed a gastric carcinoid while taking omeprazole, evidence is presented that suggests the presence of MEN-I per se may be important in determining the development of gastric carcinoid in patients with ZES. It is concluded that omeprazole is safe and effective in patients with ZES, and in these patients, it is the drug of choice for the management of gastric acid hypersecretion. However, yearly assessment is indicated to clearly evaluate the long-term risk of gastric carcinoid as well as therapy directed at the gastrinoma itself.
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PMID:Use of omeprazole in patients with Zollinger-Ellison syndrome. 135 55

Since 1960 we have diagnosed phaeochromocytoma (paraganglioma) in 10 children. The cases include a 15 year old girl who over a three year period presented with multiple paragangliomata and an associated malignant carotid body tumour. All children were hypertensive, eight of 10 presenting with severe headaches. Diagnosis was based on finding a raised urinary vanillylmandelic acid excretion and plasma noradrenaline concentration. In addition six of eight children were hypercalcaemic with raised plasma calcitonin concentrations; plasma parathyroid hormone concentrations were high in two of seven and four out of eight children had raised plasma renin activities on presentation. No child, however, was found to have a multiple endocrine neoplasia syndrome. Despite the introduction of newer techniques for the detection of catecholamine producing tumours we found that selective arteriography and venous catecholamine sampling were superior for tumour localisation compared with ultrasound scanning, computed tomography, and metaiodo-benzyl-guanidine (MIBG) scanning.
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PMID:Phaeochromocytoma--investigation and management of 10 cases. 233 2

A case of multiple endocrine adenopathy (MEA III) presenting as pheochromocytoma complicated the immediate puerperium. The patient presented with paroxysmal changes in blood pressure and episodic headaches with vertigo. Computed tomography identified a left adrenal mass that was diagnosed preoperatively as pheochromocytoma, with the diagnosis subsequently confirmed on histologic sections. Pheochromocytoma complicating pregnancy is rare, and fewer than 150 cases have been reported on. MEA III presenting as pheochromocytoma and complicating the puerperium is described here for the first time.
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PMID:MEA III presenting as pheochromocytoma and complicating pregnancy and the puerperium. A case report. 286 83

A 20-yr-old black woman presented in 1969 with headache, amenorrhea, hyperprolactinemia, hypogonadotropism, hypogonadism, and hypercalcemia due to a chromophobe adenoma. She received 5000 rads to the sella. One year later she was found to have hyperparathyroidism due to parathyroid adenoma and three and a half glands were removed. Thirteen years later she presented with 3 months of profuse watery diarrhea, hypokalemia, hypercalcemia, hyperchloremic metabolic acidosis, and a normal anion gap. A vasoactive intestinal polypeptide-producing tumor of the pancreas was found and successfully removed, after which hypercalcemia resolved. This is an unusual case of the multiple endocrine neoplasia syndrome, type 1, being associated with a vasoactive intestinal polypeptide-oma and pancreatic cholera.
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PMID:Multiple endocrine neoplasia, type 1, with pancreatic cholera. 288 44

Thirty-two histopathologically confirmed pheochromocytomas were diagnosed at Henry Ford Hospital, Detroit, between 1951 and 1982. Eleven (34%) of these cases were clinically unsuspected exhibiting none of the typical symptoms of palpitation, diaphoresis, or headache, and only five were hypertensive. These pheochromocytomas may be discovered during computed tomography of the abdomen while evaluating multiple endocrine neoplasia, abdominal pain, and abdominal masses, or they may present at autopsy, at surgery, or as a mass lesion without paroxysmal symptoms or hypertension. Before 1962, 53% of these tumors were undiagnosed before surgery or autopsy; however, since 1962 only 18% of pheochromocytomas have remained clinically unsuspected. By maintaining a higher index of suspicion and using newer biochemical and imaging techniques, the incidence of clinically unsuspected pheochromocytomas should be reduced.
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PMID:Clinically unsuspected pheochromocytomas. Experience at Henry Ford Hospital and a review of the literature. 394 65

Pheochromocytoma is surgically curable, and lethal complications often ensue when the diagnosis is not made. We review 54 autopsy-proven cases of pheochromocytoma seen at the Mayo Clinic over the 50-year period 1928-1977. There were 31 females and 23 males, and the patients' ages ranged from 40 hours to 92 years (mean, 53 years). Pheochromocytoma was multiple in 10 patients (19%), extra-adrenal in 5 (9%), and malignant in 6 (11%). In 13 (24%) pheochromocytoma had been correctly diagnosed in life--after the investigation of hypertension in 8 patients, incidentally at laparotomy for unrelated conditions in 4 patients, and in association with the multiple endocrine neoplasia syndrome in 1 patient. In the group of 41 patients (76%) in whom pheochromocytoma had not been suspected clinically, hypertension also was a common finding (22 patients, 54%). Symptoms such as headache, sweating, and palpitations were nonspecific, but all occurred more often among the group of patients in whom the diagnosis had been made. For both groups, hypertensive or hypotensive or hypotensive crisis precipitated by surgery for unrelated conditions was a common cause of death. Prevention of such deaths requires a high degree of clinical alertness and biochemical screening tests for pheochromocytoma in patients with labile or accelerated hypertension.
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PMID:Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series. 645 59

A large pituitary prolactinoma was found in a mentally deficient 45 year old woman presenting with amenorrhoea, galactorrhoea, headache, anaemia and hypertension, and removed surgically. She was subsequently found to have multiple adrenal aldosterone-producing adenomas, a gastric schwannoma and colonic polyadenomas. All these tumors were also removed surgically. Despite the absence of parathyroid and islet-cell hyperplasia, this case seems to be a variant of multiple endocrine neoplasia (MEN) type I.
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PMID:Pituitary prolactinoma, adrenal aldosterone-producing adenomas, gastric schwannoma and colonic polyadenomas: a possible variant of multiple endocrine neoplasia (MEN) type I. 711 90


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