Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01275 (glucagon)
 26,492 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Immunocytochemical stains for various pancreatic hormones were performed on 77 pancreatic endocrine tumors from 59 patients [17 with hypoglycemia, three with glucagonoma syndrome, 18 were Zollinger-Ellison syndrome, six with WDHA (watery, diarrhea, hypokalemia, and achlorhydria) syndrome and 15 without endocrine symptoms]. In all tumors that caused either hypoglycemia or glucagonoma syndrome, insulin and glucagon were respectively identified. On the other hand, only 10 tumors from 18 patients with Zollinger-Ellison syndrome were positive for gastrin, and only four of six patients with WDHA syndrome had a vasoactive intestinal peptides-positive tumor. Ten of 15 clinically silent tumors contained hormone-producing cells but without a consistent pattern. Ten neoplasms were negative for all hormones tested. Twenty-six tumors showed positively for more than one hormone and usually one cell type predominated. Four patients had multiple tumors which showed variation in the architecture and cellular composition. The tumors were classified into three major histopathologic groups: solid, gyriform, and glandular. The correlation between the pattern of growth and the hormonal production was generally poor. However, a pure gyriform pattern was often associated with insulin production, and glandular differentiation was commonly seen in tumors associated with Zollinger-Ellison syndrome. This study demonstrates the reliability of the immunocytochemical method for the specific identification of cell types in pancreatic endocrine tumors.
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PMID:Retrospective study of 77 pancreatic endocrine tumors using the immunoperoxidase method. 612 37

A case is reported of a patient who presented with symptomatic hypoglycaemia and who had three pancreatic tumours resected over the ensuing eight years. Immunocytochemistry demonstrated two of these to be insulinomas and the third to be a glucagonoma. In addition metastatic spread of cells positive for glucagon had occurred to a lymph node and multiple nodules staining positively for glucagon were present in the remainder of the pancreas.
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PMID:Recurrent insulinoma syndrome with metastatic glucagonoma. 613 29

We treated a hypertensive patient with recurrent pheochromocytoma (paraganglioma) associated with glucagonoma. No clinical symptom of glucagonoma was found and it could be detected only by a slight elevation of plasma immunoreactive glucagon (IRG) while the extirpated pancreatic tumor contained much IRG. This case could not be classified as either multiple endocrine neoplasia (MEN) type I or type II.
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PMID:Recurrent pheochromocytoma associated with glucagonoma. A case report. 613 77

Plasma responses of the major immunoreactive glucagon (IRG) components have been investigated in a case of glucagonoma syndrome. Fasting plasma IRG was 4155 pg/ml. Gel chromatography of plasma revealed that 66% of immunoreactivity was present as IRG9000, while IRG3500 accounted for an additional 26%. The appearance in peripheral plasma of these two glucagon fractions was examined after administration of a number of compounds. IRG levels were clearly elevated after arginine and tolbutamide. Both calcium and calcitonin induced a biphasic rise of IRG, the increase being slower after calcium administration. Somatostatin suppressed plasma IRG levels. All tests induced changes in both IRG3500 and IRG9000. In general, relative changes were more pronounced in IRG3500 than in IRG9000, while absolute changes were greater in IRG9000. The shape of the response curves of IRG3500 and IRG9000 was quite similar after arginine, calcium and somatostatin. After tolbutamide the IRG9000 response was delayed as compared to the IRG3500 component. During the latter part of the calcitonin infusion, IRG9000 remained elevated while IRG3500 was back at its starting level.
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PMID:The glucagonoma syndrome: stimulus-induced plasma responses of circulating glucagon components IRG9000 and IRG3500. 614 91

To evaluate the relationship between glucagon antibody antigenic determinants and selective reactivity with plasma void volume (Vo) and lower molecular weight immunoreactive glucagon (IRG) components, we studied plasma IRG levels and molecular profiles in normal subjects and patients with disturbances in plasma glucagon levels using three glucagon antibodies, 30K and P7 raised against the whole peptide and antibody X4 raised against the C-terminal tryptic fragment of glucagon. In normal subjects and pancreatectomized patients, plasma IRG levels were 2- to 3-fold higher with the C-terminus-directed antibody X4 than with either 30K or P7, but in glucagonoma and uremic patients, this discrepancy was smaller. Gel filtration analysis revealed that these antibodies reacted identically with 3500 mol wt IRG and 9000 mol wt IRG in normal, glucagonoma, pancreatectomized, and uremic plasma. Relative immunoreactivity of Vo IRG was approximately 5:2:1 with antibodies X4, 30K, and P7, respectively. In two subjects with unexplained hyperglucagonemia, recovery of IRG was entirely in the Vo, with antiserum X4 reacting one third as well as 30K and P7 not reacting at all. Furthermore, this material did not dilute out in parallel to glucagon standard. These data indicate differential immunoreactivity of the high molecular weight circulating IRG component, with a series of three glucagon antibodies reacting similarly with all other plasma IRG fractions, and suggest that Vo IRG material in plasma is predominantly the result of an immunologically cross-reacting peptide sequence in a plasma protein. The selective immunoreactivity of this component with different antibodies has important implications for the glucagon RIA and may have some bearing on other immunoassays as well.
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PMID:Differential immunoreactivity of plasma glucagon components in man: studies with different glucagon antibodies. 618 27

Twenty pancreatic endocrine tumors and one duodenal somatostatinoma were stained with polyclonal antisera against eight hormones, neuron-specific (NSE), and with a monoclonal antibody against the endocrine granule protein chromogranin. Silver staining was performed on all tumors with the Grimelius method. All the tumors and all cells in the normal pancreatic islet showed NSE immunoreactivity. Chromogranin immunoreactivity was found predominantly in the glucagon cells of normal islets, in one glucagonoma, four gastrinomas and in 10 tumors associated with multiple hormonal production, but not in five insulinomas or in one somatostatinoma. Grimelius reactivity was identical to chromogranin immunoreactivity in all cases except that more cells in the tumors and in the normal islets were positive for chromogranin. Both chromogranin and NSE were detected in a metastasized pancreatic endocrine tumor which had metastasized to a peripancreatic lymph node. The metastatic tumor failed to stain with antisera to all eight hormones. These results indicate that chromogranin and NSE are excellent general markers for pancreatic endocrine tumors and that the Grimelius stain and other related argyrophilic reactions may involve binding to chromogranin.
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PMID:Immunohistochemical detection of chromogranin and neuron-specific enolase in pancreatic endocrine neoplasms. 620 1

A 58-year-old white woman with known metastatic glucagonoma had widespread necrolytic migratory erythema characteristic of the glucagonoma syndrome. She did not respond to conventional chemotherapy with streptozocin. After one course of dacarbazine therapy, she had remission of the glucagonoma clinically with regression of tumor metastases as defined by liver scanning. After 10 months and additional courses of dacarbazine therapy, she remains in clinical remission. Plasma glucagon levels have decreased although they remain at two to four times the upper limit of normal. On several occasions there was resolution of this patient's rash after intravenous glucose in the absence of supplemental amino acids. We conclude that dacarbazine is an effective mode of chemotherapy for malignant glucagonoma.
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PMID:Malignant glucagonoma syndrome: response to chemotherapy. 625 16

Although the features of the glucagonoma syndrome had been described in isolated reports since 1942, this potentially curable condition has only recently been adequately defined. In 1974, MALLINSON collected nine cases and described the association of a peculiar skin rash with glucagon-secreting tumors of the pancreas. The typical skin rash, necrolytic migratory erythema, is accompanied by other disturbances including weight loss, anaemia, glossitis, cheilitis angularis, psychiatric symptoms. Diabetes is not a prominent feature, and the impairment of glucose tolerance can indeed be very slight. Up to 1979, about 50 cases have been reported. More than half of these patients had malignant tumors. The authors report on a 67-year-old man in whom a glucagonoma in the tail of the pancreas has been resected with apparent cure. The almost complete disappearance of the skin rash within three weeks of extirpation of the tumor has been most impressive. In June 1979 a similar tumor has been operated by one of the authors in a 60-year-old man. In this instance the tumor was located in the pancreatic corpus and could be radically resected by left-sided pancreatectomy.
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PMID:[The glucagon syndrome]. 625 12

Gel fractionation of portal, arterial and peripheral plasma glucagon levels was performed before and after the successful removal of a glucagonoma. A 47 year old woman had symptoms of dermatitis, weight loss, anemia and diabetes mellitus over a 16 year period. Removal of the alpha-cell tumor corrected all of her symptoms. Gel filtration of portal, arterial and peripheral blood showed two peaks of glucagon radioimmunoassay activity, a higher molecular weight glucagon with a molecular weight of 9,000 and a 3,500 dalton glucagon. Five minutes after tumor removal, the higher molecular weight glucagon had disappeared completely from the arterial and peripheral blood but not from the portal vein.
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PMID:Pattern of immunoreactive glucagon in portal, arterial and peripheral plasma before and after removal of glucagonoma. 625 27

The diagnosis of glucagonoma was made in a 51 year-old woman who suffered from a polymorphous dermatitis and an insulin-dependent diabetes mellitus. Denutrition was present and there was a previous history of thrombo-embolism. Immunoreactive plasma glucagon was constantly higher than 1 000 pg/ml (N less than 175). Plasma aminoacids were low. After angiographic confirmation, the tumour and part of its hepatic metastases were resected. The dermatitis disappeared soon after. Its recurrence required chemotherapy (successively mithramycin, streptozotocin, DTIC) and good clinical results were obtained. On histological examination, the cutaneous lesions consisted of an epidermal edema, and a bullous intra-epidermic detachment. The pancreatic tumour was of the trabecular type with a very important sclerosis. On electron microscopy, the tumoral cells, some with a syncitial aspect, contained granules of the D1 type. These granules are different from the typical glucagon granules. The clinical and biological features in this case are compared with those of the 41 cases of glucagonoma previously published.
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PMID:[Clinical, biological, histological, ultrastructural and therapeutic studies in one case (author's transl)]. 625 30


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