Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0221002 (primary hyperparathyroidism)
4,921 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We sought an explanation for prior findings of high plasma chromogranin-A (Chr-A) in primary hyperparathyroidism. Chr-A was measured in plasma samples from 55 controls and 73 patients with primary hyperparathyroidism caused by adenoma (n = 14), sporadic or familial hyperplasia (n = 10), or familial multiple endocrine neoplasia type 1 (FMEN1; n = 49). Serum or plasma samples were also tested for calcium, PTH, gastrin, pancreatic polypeptide, CG alpha, and PRL. Plasma Chr-A was 34 +/- 10 in parathyroid adenoma, 55 +/- 33 in parathyroid hyperplasia without FMEN1, 63 +/- 88 in FMEN1, and 25 +/- 8 in controls (mean +/- SD; nanograms per ml; FMEN1 or parathyroid hyperplasia vs. control, P less than 0.05). Plasma Chr-A did not correlate with other hormonal variables in controls. Plasma Chr-A correlated with log serum gastrin (r = 0.43; P = 0.003) and plasma PTH (r = 0.52; P less than 0.05) only in FMEN1. In FMEN1, plasma Chr-A was highest in subjects with Zollinger-Ellison syndrome (ZES, 120 +/- 127; no ZES, 30 +/- 33 (P less than 0.0001). Parathyroidectomy did not decrease plasma Chr-A in patients with parathyroid adenoma or parathyroid hyperplasia. For FMEN1 patients with available pre- and postparathyroidectomy samples, Chr-A decreased postoperatively in four of five patients with ZES compared to none of six patients without ZES (P less than 0.05). Elevated plasma Chr-A is not a general feature of primary hyperparathyroidism. Elevated plasma Chr-A in primary hyperparathyroidism was restricted principally to patients who also had ZES. Primary hyperparathyroidism may influence the level of Chr-A by an effect of hypercalcemia or elevated PTH on Chr-A secretion from pancreatic islet tissue.
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PMID:Plasma chromogranin-A in primary hyperparathyroidism. 257 19

Multiple endocrine neoplastic diseases are genetically determined conditions with particular organ patterns for endocrine tumors. In Type I or Wermer's syndrome the endocrine pancreas, anterior pituitary and parathyroids are involved, insulinoma being the most frequent pancreatic tumor. To facilitate diagnosis, a prolonged oral glucose tolerance test, a fasting test and determination of the glucose-insulin ratio are recommended. Localisation is sought by computer tomography and angiography. A gastrinoma is excluded on the basis of normal gastrin levels in serum and by means of the secretin-provocation-test. Pituitary tumors can be classified more closely with prolactin levels and releasing-hormone tests (LH-RH and TRH). Prolactinoma is the most frequent pituitary tumor and amenable to bromocryptin treatment. If Wermer's syndrome is suspected, primary hyperparathyroidism has to be excluded on the basis of calcium and parathormone levels. Chief cell hyperplasia or multiple adenomas are frequent. Surgical resection is necessary.
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PMID:[Type I multiple endocrine neoplasia--Wermer syndrome]. 257 44

The prevalence of peptic ulcer disease was retrospectively analysed in 35 patients affected by primary hyperparathyroidism consecutively observed from 1977 through 1987. Eight of the examined patients (22.8%) had peptic ulcer (7 duodenal and 1 gastric ulcer), that in five cases (14%) represented the first clinical manifestation of the endocrine disease. A Zollinger-Ellison syndrome was demonstrated in three cases (8.5% of the total series, 37% of the patients with ulcer). There was no difference in calcium, gastrin and parathormone serum level between patients with and without ulcer, excluding patients with Zollinger-Ellison syndrome. These data confirm the high prevalence of peptic ulcer disease in hyperparathyroidism, but the mechanism causing this association remains to be elucidated.
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PMID:[Primary hyperparathyroidism and peptic ulcer]. 262 74

A prospective study involving 7 patients with primary hyperparathyroidism and hypergastrinaemia was conducted to assess the time-dependent change in serum gastrin value before and after parathyroidectomy and to determine at which postoperative stage persistent hypergastrinaemia may be indicative of an associated gastrinoma (Zollinger-Ellison syndrome). Five of the 7 patients had hypergastrinaemia in the early postoperative period. One patient had a strikingly high serum gastrin level pre-operatively (1,500 pg/ml). The mean serum gastrin value declined to within the normal range 6 weeks after parathyroidectomy, except in 1 patient who had a gastrinoma. It is concluded that hypergastrinaemia in patients with primary hyperparathyroidism should only be considered significant if pre-operative gastrin levels are strikingly supranormal and/or levels fail to normalise by the 6th postoperative week.
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PMID:Time-dependent changes in serum gastrin measurements after parathyroidectomy. 272 22

A family of multiple endocrine neoplasia type I with five confirmed cases in three generations is described. All of them have primary hyperparathyroidism in common. The propositus is 51 year-old male. After a year of symptoms of gastroduodenal ulcer, he was found to have elevated levels of serum gastrin and PTH. The serial imaging studies revealed a tumor in pancreatic head, and Zollinger-Ellison syndrome was diagnosed. The gastrin level was reduced into normal range after extirpation of the tumor, but post surgical elevation of Calcium put the patient under parathyroidectomy, which normalized serum PTH and Calcium levels. His two sisters (I and II), the mother of them, and the daughter of sister I, had neither signs nor symptoms until family study showed hypercalcemia in all. Sister I is a 54 year-old female with enlarged parathyroid. The hyperparathyroidism is of chemical type, but no other endocrinological abnormality is found. The Calcium level decreased after parathyroidectomy. Sister II is a 56 year-old female. The only sign was galactorrhea. Serum PTH and Calcium decreased after parathyroidectomy. The prolactinoma was diagnosed by the increased prolactin levels and enhanced mass lesion in sella turcica. Her serum prolactin levels is now within normal range since she is on bromocryptine. The mother of the above three siblings and the daughter of the sister I are now under further study.
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PMID:[A family with multiple endocrine neoplasia type I presenting prolactinoma, Zollinger-Ellison syndrome and hyperparathyroidism]. 286 39

In patients with hypercalcemia with abdominal symptoms, gastrin concentration is often measured to exclude the Zollinger-Ellison syndrome. We found that interpretation of such measurements is clouded by a contradictory literature. We therefore measured serum gastrin concentrations in 78 patients with primary hyperparathyroidism, 36 with nonparathyroid hypercalcemia, 13 with hypocalcemia, and 33 normocalcemic controls. Gastrin values above normal occurred in 22% of those with primary hyperparathyroidism and 28% of those with nonparathyroid hypercalcemia. Values above 250 pg/mL occurred only in those with hypochlorhydria or multiple endocrine neoplasia, type 1 (MEN 1). After parathyroidectomy, gastrin levels fell significantly, but elevated values tended to recur in those with MEN 1 if hypercalcemia recurred. Thus, chronic hypercalcemia of either parathyroid or nonparathyroid origin may elevate serum gastrin concentrations, but marked elevations suggest either achlorhydria or MEN 1.
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PMID:Serum gastrin level is increased by chronic hypercalcemia of parathyroid or nonparathyroid origin. 286 39

Between 1971 and 1983, 31 males and 13 females were found to have peptic ulceration associated with hypergastrinaemia. An antral G-cell lesion was present in 11 (25 per cent) and a gastrinoma in 14 (32 per cent). There were 11 patients with multiple endocrine adenomatosis (MEA) (25 per cent) and 4 (9 per cent) with primary hyperparathyroidism. Four patients (9 per cent) were unclassified. Length of history and level of gastrin did not differentiate between the groups and an average of 2.5 operations was performed per patient, while the overall mortality was 27.3 per cent. The patients with G-cell lesions were significantly younger than all the other groups (P less than 0.01). Partial gastrectomy adequately treated G-cell hyperplasia. Total gastrectomy was required to treat pancreatic gastrinomata but additional pancreatic resection did not improve the outcome. In MEA, parathyroidectomy did not influence the treatment of a gastrinoma. This is the first recorded experience of surgery for hypergastrinaemia in the United Kingdom and the outcome of such a retrospective study may be a guide to the future management of these conditions.
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PMID:Surgery for peptic ulceration associated with hypergastrinaemia. 287 Jul 56

We measured multiple components of serum or plasma in 221 members of a kindred with familial multiple endocrine neoplasia type 1 (FMEN1). The kindred showed typical features of FMEN1; the FMEN1 gene could be traced through 7 generations with 74 members identifiable as gene carriers. Between family screening in 1981 and completion of our study in 1985, we identified 16 previously unscreened members as carriers of the FMEN1 gene. The earliest age at diagnosis of FMEN1 was 17. The tests with the greatest yield of abnormal results among carriers of the FMEN1 gene were albumin-adjusted calcium, PTH, gastrin, and (in females) prolactin. The following tests provided little or no use in identifying carriers: prolactin (in males), pancreatic polypeptide, glucagon, glicentin, insulin, growth hormone, motilin, and somatostatin. Primary hyperparathyroidism was the commonest expression of the FMEN1 gene; the gene penetrance for this trait increased from near 0% before age 15 to near 100% after age 40. It appeared prior to development of serious morbidity from hypergastrinemia or hyperprolactinemia. All 42 co-operating members who were alive and expressing the FMEN1 gene in 1984 showed active or treated primary hyperparathyroidism. Primary hypergastrinemia had a prevalence below half of that for primary hyperparathyroidism at all ages and was not diagnosed in the absence of primary hyperparathyroidism. Primary hyperprolactinemia was still less prevalent than primary hypergastrinemia. It was limited almost exclusively to females.
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PMID:Multiple endocrine neoplasia type I: assessment of laboratory tests to screen for the gene in a large kindred. 287 98

Forty-nine members of 6 families with multiple endocrine neoplasia type 1 (MEN 1) were investigated with a standardized meal stimulation test to detect the presence of pancreatic endocrine tumors. Fifteen age-matched subjects and 4 patients with primary hyperparathyroidism also were studied. Serum pancreatic polypeptide (PP), gastrin, and insulin as well as plasma glucagon and somatostatin concentrations were determined before and during the test meal. Patients with demonstrable pancreatic endocrine tumors had significantly increased mean basal and peak serum PP (P less than 0.001) and gastrin (P less than 0.001) responses to the meal compared with healthy family members and normal subjects. Seven of 12 MEN 1 patients with parathyroid and pituitary disease but no demonstrable pancreatic endocrine tumors had exaggerated PP and/or gastrin responses to the meal; 4 of them developed pancreatic endocrine tumors, detected by abdominal computerized tomography, 0.5-4 yr later. None of the healthy members of the MEN 1 families or the patients with primary hyperparathyroidism had responses different from those of the normal subjects. Our experience with the meal stimulation test indicates that an elevated basal or exaggerated serum PP and/or gastrin response is an earlier sign of pancreatic involvement in the MEN 1 trait than is abdominal computerized tomography.
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PMID:A standardized meal stimulation test of the endocrine pancreas for early detection of pancreatic endocrine tumors in multiple endocrine neoplasia type 1 syndrome: five years experience. 288 95

This study evaluates prospectively the effect of parathyroidectomy on basal acid output (BAO), maximal acid output (MAO), fasting serum gastrin, secretin-stimulated serum gastrin, and sensitivity to antisecretory medication in 10 consecutive patients with primary hyperparathyroidism (PHP), Zollinger-Ellison syndrome (ZES), and multiple endocrine neoplasia type I (MEN-I). After parathyroidectomy, 9 of 10 patients remained normocalcemic, and each had a lower BAO; 6 of 9 no longer had gastric acid hypersecretion (less than 15 mEq/hr). Seven of 9 normocalcemic patients had a lower MAO, and a decrease in fasting serum gastrin. Two patients showed no evidence of ZES, a normal BAO, normal fasting serum gastrin concentration, and a negative secretin response after parathyroidectomy. Parathyroidectomy also reduced the dose of histamine H2-receptor antagonist required to control gastric acid secretion in 60% of patients. After successful parathyroidectomy three patients were studied for drug sensitivity, and each had greater acid inhibition with a given dose of histamine H2-receptor antagonist than preoperatively. One patient remained hypercalcemic after surgery and had no change in BAO, MAO, or gastrin. All patients with postoperative normocalcemia will have a lower BAO, 80% a lower MAO, 80% a decreased fasting serum gastrin, and 33% a negative secretin test. Antisecretory medication dose can be reduced because patients have reduced BAO and increased sensitivity to histamine H2-receptor antagonist. The study supports parathyroidectomy as the initial surgical procedure of choice in patients with PHP, ZES, and MEN-I.
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PMID:Effect of parathyroidectomy in patients with hyperparathyroidism, Zollinger-Ellison syndrome, and multiple endocrine neoplasia type I: a prospective study. 289 Dec 1


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