gastrins and Parathyroid-Neoplasms

Topics: Adenoma; Carcinoid Tumor; Gastrins; Humans; Male; Middle Aged; Multiple Endocrine Neoplasia Type 1; Neoplasms, Multiple Primary; Parathyroid Glands; Parathyroid Neoplasms; Stomach; Stomach Neoplasms; Zollinger-Ellison Syndrome

Topics: Achlorhydria; Acromegaly; Adenoma; Adenoma, Islet Cell; Adolescent; Adult; Aged; Calcium; Cushing Syndrome; Diarrhea; Female; Gastric Acid; Gastrins; Glucagon; Humans; Hyperinsulinism; Hyperparathyroidism; Hypoglycemia; Hypokalemia; Male; Middle Aged; Neoplasms, Multiple Primary; Pancreatic Polypeptide; Pancreatitis; Parathyroid Glands; Parathyroid Neoplasms; Pituitary Neoplasms; Syndrome; Thyroid Diseases; Zollinger-Ellison Syndrome

Topics: Acute Kidney Injury; Adenoma, Islet Cell; Apudoma; Dehydration; Gastrins; Gastrointestinal Hormones; Gastrointestinal Neoplasms; Humans; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Paraneoplastic Endocrine Syndromes; Parathyroid Neoplasms; Pheochromocytoma; Postgastrectomy Syndromes; Somatostatin; Syndrome; Thyroid Neoplasms; Zollinger-Ellison Syndrome

The familial aggregation of peptic ulcer disease has been well established, as has its association with such clear-cut genetic factors as blood group O and nonsecretor status. However, the genetics of this disorder, or group of disorders, is still in question. Polygenic inheritance is the prevailing hypothesis that has been proposed for peptic ulcer. This hypothesis was based primarily on the exclusion of a simple mode of inheritance for all ulcer disease. Genetic heterogeneity is an alternative hypothesis that can explain both the familial aggregation of peptic ulcer disease and the lack of a simple Mendelian pattern of inheritance. The evidence for genetic heterogeneity in peptic ulcer disease is reviewed, and studies are proposed to test this hypothesis.

Topics: ABO Blood-Group System; Antigens; Chromosome Aberrations; Chromosome Disorders; Duodenal Ulcer; Gastric Juice; Gastrins; Humans; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Parathyroid Neoplasms; Pepsinogens; Peptic Ulcer; Phenotype; Pituitary Neoplasms; Stomach Ulcer; Werner Syndrome

Topics: Adenoma, Islet Cell; Blood Glucose; Calcium; Endocrine System Diseases; Gastrins; Humans; Insulin; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Parathyroid Neoplasms; Pheochromocytoma; Pituitary Neoplasms; Radioimmunoassay; Syndrome; Thyroid Neoplasms; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Blood Glucose; Diagnosis, Differential; Gastrins; Glucose Tolerance Test; Humans; Hyperparathyroidism; Insulin; Pancreatic Neoplasms; Parathyroid Neoplasms; Radioimmunoassay; Tolbutamide; Zollinger-Ellison Syndrome

We describe a patient with multiple endocrine neoplasia type 1 characterized by the simultaneous occurrence of parathyroid cancer, parathyroid adenomas, and pancreatic gastrinoma, who presented with an episode of acute hypercalcemia. The rapid parathyroid hormone assay provided a basis for the diagnosis of parathyroid hyperfunction. Mediastinal metastasis of the parathyroid carcinoma was found at autopsy. However, the staining of pancreatic and gastric tissue for parathyroid hormone-related protein does not make it possible to exclude completely the contribution of this peptide in mediating the hypercalcemia. To our knowledge, this is the first reported case of parathyroid carcinoma as part of the multiple endocrine neoplasia type 1 syndrome.

Topics: Acute Disease; Adenoma; Adult; Carcinoma; Fatal Outcome; Gastrinoma; Gastrins; Humans; Hypercalcemia; Hyperparathyroidism; Male; Multiple Endocrine Neoplasia Type 1; Pancreatic Neoplasms; Parathyroid Hormone; Parathyroid Neoplasms

A case of Zollinger-Ellison syndrome of multiple endocrine neoplasia type 1 (MEN 1) origin with hyperparathyroidism and with a rise in serum gastrin due to an unusual parathyroid "gastrinoma" has been investigated. The patient had multiple endocrine tumours (pituitary and parathyroid), but no evidence of pancreatic or duodenal gastrin-producing neoplasm. Radio-immunoassay, immunohistochemistry and electron microscopy showed gastrin in one parathyroid adenoma. These findings, together with a decrease of gastrinaemia after parathyroidectomy suggest that true gastrin was produced by parathyroid tumour cells and that they themselves may be the origin of the hypergastrinaemia. Our ultrastructural investigation extends these observations and the results are discussed.

Topics: Adenoma; Female; Gastrins; Humans; Immunohistochemistry; Microscopy, Electron; Middle Aged; Multiple Endocrine Neoplasia; Parathyroid Glands; Parathyroid Hormone; Parathyroid Neoplasms; Zollinger-Ellison Syndrome

It is well known that primary hyperparathyroidism is often associated with peptic ulcer. The purpose of this study is to confirm the relationship between the gastrin-levels before and after parathyroidectomy in fourteen patients with primary hyperparathyroidism, and to determine the localization of gastrin in the surgically resected parathyroid tumor. The results obtained were as follows: 1) Three patients had peptic ulcer (gastric ulcer and duodenal ulcer), the incidence being 21%. 2) The basal serum gastrin levels were 123.0% +/- 68.1 pg/ml before operation and decreased to 90.2 +/- 44.5 pg/ml after operation. In the 3 patients with slightly elevated gastrin levels, the mean level before operation was 209.1 +/- 61.2 pg/ml. The gastrin level decreased to 116.4 +/- 62.0 pg/ml after operation. 3) Gastrin immunoreactivity was detected in 10 out of 14 tumors and its localization was at the periphery of tumor cells. From these results, we conclude that extragastric gastrin secretion from parathyroid tumors may be one of the cause of peptic ulcer in patients with primary hyperparathyroidism.

Topics: Calcium; Duodenal Ulcer; Gastrins; Humans; Hyperparathyroidism; Parathyroid Glands; Parathyroid Neoplasms; Stomach Ulcer

Topics: Adenoma; Adenoma, Islet Cell; Adrenal Cortex; Female; Gastrins; Glucagon-Like Peptides; Humans; Hydrocortisone; Hyperplasia; Middle Aged; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Parathyroid Neoplasms; Peptides; Thyroid Gland; Thyroid Hormones

The treatment of choice for gastric acid hypersecretion in MEN type I has now changed from total gastrectomy to the long-term administration of H2 receptor blockers or similar agents. However, the importance of parathyroidectomy for the concomitant hypercalcaemia is not fully realized. A case is reported of a subject with MEN type I, who was initially treated with parathyroidectomy and H2 receptor blockade. Following parathyroidectomy, there was a rapid fall of the markedly elevated gastrin levels to the upper limit of the normal range. During follow-up of 2.5 years, there has been no increase in serum gastrin, and the subject's gastric symptoms have resolved, despite the cessation of H2 blockade. Infusion of calcium to pre-operative levels and superimposed secretin stimulation after this period of time closely reproduced the pre-operative hypergastrinaemia. On screening, the subject's only child was found to have hypercalcaemia but normal serum gastrin levels and pituitary function; parathyroidectomy has been carried out. No abnormality of gastrin secretion has been found during follow-up. This case highlights the importance of early parathyroidectomy in this syndrome.

Topics: Adult; Calcium; Female; Follow-Up Studies; Gastrins; Humans; Multiple Endocrine Neoplasia; Parathyroid Glands; Parathyroid Neoplasms

Hyperplasia of the parathyroid glands is a central feature of familial multiple endocrine neoplasia type 1. We used cultured bovine parathyroid cells to test for mitogenic activity in plasma from patients with this disorder. Normal plasma stimulated [3H]thymidine incorporation, on the average, to the same extent as it was stimulated in a plasma-free control culture. This contrasted with the results of the tests with plasma from patients with familial multiple endocrine neoplasia type 1, in which parathyroid mitogenic activity increased 2400 percent over the control value (P less than 0.001). Plasma from these patients also stimulated the proliferation of bovine parathyroid cells in culture, whereas plasma from normal subjects inhibited it. Parathyroid mitogenic activity in plasma from the patients with familial multiple endocrine neoplasia type 1 was greater than that in plasma from patients with various other disorders, including sporadic primary hyperparathyroidism (with adenoma, hyperplasia, or cancer of the parathyroid), sporadic primary hypergastrinemia, sporadic pituitary tumor, familial hypocalciuric hypercalcemia, and multiple endocrine neoplasia type 2 (P less than 0.05). Parathyroid mitogenic activity in the plasma of patients with familial multiple endocrine neoplasia type 1 persisted for up to four years after total parathyroidectomy. The plasma also had far more mitogenic activity in cultures of parathyroid cells than did optimal concentrations of known growth factors or of any parathyroid secretagogue. This mitogenic activity had an apparent molecular weight of 50,000 to 55,000. We conclude that primary hyperparathyroidism in familial multiple endocrine neoplasia type 1 may have a humoral cause.

Topics: Adenoma; Adult; Animals; Cattle; Cells, Cultured; Gastrins; Growth Substances; Humans; Hypercalcemia; Hyperparathyroidism; Hyperpituitarism; Hyperplasia; Multiple Endocrine Neoplasia; Parathyroid Glands; Parathyroid Neoplasms; Thymidine; Tritium

To determine if gastrin in hyperparathyroid glands is true gastrin or artifact and to determine the frequency of gastrin in parathyroid glands, 20 parathyroid glands from 11 patients with hyperparathyroidism but without MEA were extracted and analyzed for gastrin. The parathyroid glands from 4 out of 11 patients had measurable gastrin immunoreactivity (10.7 + 6 pg/mg tissue). Column separation chromatography confirmed that this was true gastrin (40% G-34; 50% G-17). Immunohistochemistry with ABC (avidin biotin complex) immunoperoxidase confirmed the presence of gastrin in cytoplasmic vesicles in scattered parathyroid cells. True gastrin does exist in some cells in some patients with hyperparathyroidism.

Topics: Adenoma; APUD Cells; Gastrins; Humans; Hyperparathyroidism; Hyperplasia; Immunoenzyme Techniques; Parathyroid Glands; Parathyroid Neoplasms; Protein Precursors; Radioimmunoassay

The largest-known family tree of a kindred with multiple endocrine neoplasia type I, dating back to 1840, has been constructed in Tasmania. There are over 600 descendants of one English migrant and his spouse living today. Preliminary data suggests that overall, one-quarter of all family members, and one-half of those above the age of 40 manifest one or more endocrine tumours. In the majority of cases, the diagnosis was not suspected until the general practitioner was informed of the family history, as the symptoms are vague, sometimes bizarre, and overlap with those of common disorders.

Topics: Adult; Aged; Child; Female; Gastrins; Humans; Hypercalcemia; Hypertension; Male; Middle Aged; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Parathyroid Neoplasms; Prolactin

Topics: Adult; Gastrins; Humans; Hyperparathyroidism, Secondary; Male; Multiple Endocrine Neoplasia; Parathyroid Hormone; Parathyroid Neoplasms; Pituitary Neoplasms; Prolactin; Zollinger-Ellison Syndrome

In three families with the multiple endocrine adenomatosis type I (MEA I) trait, 51 members were investigated by measurement of circulating peptide hormones as tumor markers. Twenty-five of 51 members (49 percent) were considered to be affected by MEA I disorders. The incidence rose with age (75 percent in generation II). Both sexes were affected equally. Hyperparathyroidism was present in 20 of 25 affected members (80 percent), and pituitary tumors (prolactinomas) were found in four of 25 (16 percent). Endocrine pancreatic tumors were found in nine of 25 affected members (36 percent), but when "probable" tumors (seven) are included the frequency rises to 72 percent. Hyperparathyroidism was found in all except one member with proved lesions in other organs. Among patients with proved and possible endocrine pancreatic tumors, elevated serum levels of gastrin and pancreatic polypeptide were frequently found, 78 percent and 67 percent, respectively, and we suggest that serum gastrin and pancreatic polypeptide levels are the most useful screening markers at present for pancreatic lesions in MEA I.

Topics: Adenoma; Adolescent; Adult; Age Factors; Aged; Female; Gastrins; Humans; Hyperparathyroidism; Insulinoma; Liver Neoplasms; Male; Middle Aged; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Pancreatic Polypeptide; Parathyroid Neoplasms; Pedigree; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

In 36 patients with verified hyperparathyroidism (HPT), serum gastrin and its response to secretin was studied before and after parathyroidectomy. In most of the patients gastric secretion-basal acid output (BAO) and maximal acid output (MAO)--was also studied. Seventeen patients had increased serum gastrin values preoperatively and/or postoperatively. Most of the serum gastrin increases were moderate and all but one of the patients with values above 250 pmol/l had hypochlorhydria. Nine patients had a positive secretin test according to conventional criteria preoperatively or postoperatively, but showed no other signs suggestive of a gastrin-producing tumour. Most of the patients with a serum gastrin increase after injection of secretin had hypochlorhydria or achlorhydria. The BAO/MAO ratio was less than 0.6 in all patients. The results of this study do not support the view that primary HPT is often associated with a gastrin-producing tumour as part of a multiple endocrine adenomatosis (MEA type I), but indicate that the observed hypergastrinemia in HPT is almost exclusively related to hypo- or achlorhydria. The findings also give reason to doubt the value of the secretin test.

Topics: Adenoma; Female; Gastric Acid; Gastrins; Humans; Hyperparathyroidism; Male; Middle Aged; Parathyroid Neoplasms; Peptic Ulcer; Secretin

The high incidence of gastroduodenal ulcers in patients with hyperparathyroidism has been ascribed to gastric hyperacidity induced by hypercalcaemia. In a clinical study comprising 40 patients with solitary parathyroid adenoma, a negative correlation between serum calcium and spontaneous gastric acid secretion was found. At the same time a positive correlation was found between serum calcium and gastrin. It is concluded that gastric hyperacidity is not part of the syndrome provoked by parathyroid adenomas, and that a mechanism inhibitory to gastrin is active in these patients. It is suggested that the inhibitor could be calcitonin.

Topics: Adenoma; Adult; Aged; Calcitonin; Calcium; Female; Gastric Acid; Gastrins; Humans; Male; Middle Aged; Parathyroid Hormone; Parathyroid Neoplasms

The authors present a brief report on a familial case of Wermer's syndrome, and review the principal characteristics of this "multiple endocrine neoplasm" which usually affects the parathyroids, pancreas, and anterior pituitary.

Topics: Adult; Female; Gastrins; Glucagon; Humans; Insulin; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Parathyroid Neoplasms; Pituitary Neoplasms; Syndrome; Vasoactive Intestinal Peptide

A father and son each presented with severe watery diarrhea. The son was found to have a pancreatic islet-cell tumor associated with the pancreatic cholera syndrome, as well as a parathyroid adenoma. The father was found to have multiple islet-cell adenomas and the Zollinger-Ellison syndrome. Pancreatic tumor tissue from each patient contained detectable gastrin and vasoactive intestinal peptide; however, a much higher gastrin concentration was found in the tumor tissue from the father and a much higher vasoactive intestinal peptide content in the tumor tissue from the son. Thus, watery diarrhea may be mediated by different hormones in families having multiple endocrine neoplasia; the precise cause of the diarrheal syndrome should be defined to ensure the proper therapy.

Topics: Adenoma; Adenoma, Islet Cell; Adult; Diarrhea; Endocrine System Diseases; Gastrins; Humans; Male; Middle Aged; Neoplasms, Multiple Primary; Pancreatic Neoplasms; Parathyroid Neoplasms; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

A rare case of the Zollinger-Ellison syndrome associated with hyperparathyroidism and ectopic gastric tissue in the lower esophageal mucosa is reported. Preoperatively the patient, a 53-year-old woman, had hyperchlorhydria and her fasting serum gastrin concentration was mildly elevated. There was a considerable increase in the gastric acid output and concentration of serum calcium after secretin infusion. At operation the patient had a gastric ulcer 10 cm in diameter, an islet cell tumour of the pancreas 14 cm in diameter, and ectopic gastric mucosa in the distal third of the esophagus. A gastrectomy was perfomed, the pancreatic tumour excised and part of the distal esophagus removed through a left thoracotomy. Four months after the operation the gastrin concentration had returned to low normal, but the serum calcium values remained high. One month later two parathyroid adenomas were removed which effectively cured the hypercalcemia.

Topics: Adenoma; Calcium; Choristoma; Esophageal Neoplasms; Esophagus; Female; Gastric Juice; Gastric Mucosa; Gastrins; Hormones, Ectopic; Humans; Hyperparathyroidism; Middle Aged; Paraneoplastic Endocrine Syndromes; Parathyroid Hormone; Parathyroid Neoplasms; Stomach; Zollinger-Ellison Syndrome

The relatives of 25 index patients with primary parathyroid hyperplasia were tested for hypercalcemia. At least 13 of these patients had one or more first degree relatives with hypercalcemia. Two familial syndromes each with autosomal dominant transmission were recognized. Two index patients were part of large kindreds categorized as having familial hypocalciuric hypercalcemia (FHH). Manifestations of multiple endocrine neoplasia type I were present in the kindreds of at least four other index patients (FMEN I). In seven other kindreds there were too few affected members to allow definitive classification. Differences between manifestations of FHH and FMEN I were described. Among offspring of affected persons in kindreds with FHH, as distinct from FMEN I, the prevalence of hypercalcemia approached the theoretic maximum of 50 per cent during the first two decades. In FHH, nephrolithiasis and peptic disease were unusual; moderate hypercalcemia occurred without hypercalciuria; and subtotal parathyroidectomy did not abolish hypercalcemia. Concentrations of peptide hormones other than parathyroid hormone (PTH) were normal in those with FHH; in FMEN I high concentrations of glucagon in plasma were found in five of six patients tested, and high concentrations of gastrin were found in three of 12 patients. Hypergastrinemia generally accompanied obvious peptic disease. Distinction of the two conditions is important since patients with FHH may not benefit from subtotal parathyroidectomy, but they generally have a better clinical prognosis than do patients with FMEN I.

Topics: Adolescent; Adult; Aged; Calcium; Child; Female; Gastrins; Glucagon; Humans; Hypercalcemia; Hyperplasia; Male; Middle Aged; Neoplasms, Multiple Primary; Parathyroid Glands; Parathyroid Hormone; Parathyroid Neoplasms; Pedigree; Prolactin

The relationship between the serum gastrin and calcium concentrations has been examined in patients with multiple endocrine neoplasia type I. Variations in gastrin concentrations were induced by metiamide and secretin; calcium concentrations were altered by parathyroidectomy and by calcium infusion. Changes in the serum gastrin concentrations were not accompanied by changes in the serum calcium concentration. However, alteration of the serum calcium was accompanied by significant parallel changes in the serum gastrin. It is concluded that acute changes in the serum calcium may induce changes in the serum gastrin. Parathyroidectomy in these patients produced a fall in the serum gastrin, but the ability to produce large quantities of gastrin remains. It is postulated that the thyro-parathyroid hormones may modulate the relationship between calcium and gastrin.

Topics: Adult; Aged; Calcium; Female; Gastrins; Humans; Male; Metiamide; Middle Aged; Multiple Endocrine Neoplasia; Neoplasms, Multiple Primary; Parathyroid Neoplasms; Secretin; Zollinger-Ellison Syndrome

Hyperparathyroidism has been associated with an increased incidence of duodenal ulcer, increased acid secretion, and increased plasma gastrin levels. A relationship between these changes, increased serum calcium levels, and the increased incidence of peptic ulceration has been suggested, especially since increased plasma gastrin levels, serum calcium levels, and gastric acid secretion decrease after parathyroidectomy. We have previously suggested that the decrease in plasma gastrin levels after parathyroidectomy may suggest an extragastric source of gastrin, whereas others using immunofluorescent studies have suggested that the parathyroid adenomas themselves might be the source of this gastrin. We prospectively studied in fifteen patients with primary hyperparathyroidism, plasma gastrin and serum calcium levels before and after parathyroidectomy, as well as the gastrin content of parathyroid tumor tissue. The mean basal plasma gastrin level before operation was significantly greater than that of a control group and decreased insignificantly after operation, in contrast to serum calcium levels. No positive correlation could be found between plasma gastrin and serum calcium levels before and after operation. Parathyroid tumor tissue was assayed for gastrin content by radioimmunoassay and no detectable amounts of gastrin could be recovered from any tumor. The results do not support the concept that the extragastric source of gastrin in patients with hyperparathyroidism is the parathyroid adenoma itself.

Topics: Adenoma; Adult; Aged; Duodenal Ulcer; Female; Gastrins; Hormones, Ectopic; Humans; Hyperparathyroidism; Male; Middle Aged; Parathyroid Neoplasms

Topics: Adenoma; Adult; Aged; Carcinoid Tumor; Diagnosis, Differential; Duodenal Neoplasms; Endocrine Glands; Female; Gastrins; Glucagon; Humans; Hyperparathyroidism; Male; Middle Aged; Pancreas; Pancreatic Neoplasms; Parathyroid Neoplasms; Radiography; Stomach Neoplasms; Syndrome; Zollinger-Ellison Syndrome

Topics: Adenoma; Adult; Aged; Blood Glucose; Diazoxide; Duodenal Ulcer; Female; Gastrectomy; Gastrins; Glucagon; Hormones, Ectopic; Humans; Hyperparathyroidism; Hypoglycemia; Insulin; Insulin Secretion; Lymphatic Metastasis; Male; Middle Aged; Multiple Endocrine Neoplasia; Pancreatectomy; Pancreatic Neoplasms; Parathyroid Neoplasms; Peptic Ulcer Perforation; Splenectomy; Zollinger-Ellison Syndrome

Topics: Adult; Blood Glucose; Calcitonin; Calcium; Circadian Rhythm; Duodenal Ulcer; Female; Follicle Stimulating Hormone; Gastrectomy; Gastrins; Glucagon; Growth Hormone; Hormones; Humans; Hydrocortisone; Insulin; Luteinizing Hormone; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Parathyroid Neoplasms; Pedigree; Phosphorus; Prolactin; Thyrotropin

Topics: Adenoma; Adult; Aged; Calcium; Female; Gastric Acidity Determination; Gastric Juice; Gastrins; Histamine; Humans; Hyperparathyroidism; Intestinal Mucosa; Male; Middle Aged; Parathyroid Neoplasms; Pepsin A; Phosphorus; Stimulation, Chemical; Stomach

Topics: Adenoma; Calcium; Gastric Juice; Gastrins; Gastrointestinal Hormones; Humans; Hypercalcemia; Parathyroid Glands; Parathyroid Neoplasms; Pentagastrin; Peptides; Stomach

Topics: Adenoma; Adolescent; Adrenal Gland Neoplasms; Adult; Calcium; Diabetes Complications; Female; Gastrins; Humans; Hyperparathyroidism, Secondary; Male; Parathyroid Diseases; Parathyroid Neoplasms; Pituitary Neoplasms

Topics: Adenoma; Adult; Aged; Biological Assay; Calcium; Female; Gastric Juice; Gastrins; Histamine; Humans; Hyperparathyroidism; Male; Middle Aged; Parathyroid Neoplasms; Radioimmunoassay

Topics: Adenoma; Animals; Biopsy; Cell Count; Fluorescent Antibody Technique; Gastric Mucosa; Gastrins; Histocytochemistry; Humans; Hyperparathyroidism; Hyperplasia; Microscopy; Microscopy, Electron; Parathyroid Neoplasms; Peptides; Pylorus; Rabbits; Television; Thyroid Diseases

Topics: Adrenal Gland Neoplasms; Adult; Child; Gastrins; Glucagon; Glucose Tolerance Test; Humans; Insulin; Insulin Secretion; Islets of Langerhans; Multiple Endocrine Neoplasia; Pancreatic Diseases; Pancreatic Neoplasms; Parathyroid Neoplasms; Tolbutamide