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)

A woman with a multiple-hormone-producing pancreatic islet cell tumor with hepatic metastases and with recurrent hypoglycemic attacks, was treated with streptozotocin. After this treatment, the elevated serum levels of insulin, C-peptide, glucagon and serotonin fell markedly and the low level of fasting blood glucose returned to normal. In accordance with these hormonal changes, scintiscan and CT scan revealed marked regression of the metastatic tumors in the liver. She is alive at this writing, five years after the streptozotocin treatment. Streptozotocin should thus be considered for treatment of malignant islet cell carcinoma with liver metastases and which is not amenable to surgery.
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PMID:Streptozotocin effective for treating multiple-hormone-producing malignant islet cell tumor. 298 54

The glucagonoma syndrome is a rare clinical condition. In this syndrome there is a pancreatic islet cell tumour which secretes glucagon, associated with a distinctive skin eruption--migratory necrolytic erythema. We describe a 79 year old Chinese man who presented with the typical skin features of this condition and on investigation had a pancreatic islet cell tumour. Serum glucagon levels were markedly elevated. He had a partial pancreatectomy and the skin rash improved dramatically. However he had liver metastases and responded poorly to streptozotocin. The skin features recurred and the patient died 9 months after diagnosis. As far as we are aware, this is the first report of a Glucagonoma syndrome in a Chinese.
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PMID:The glucagonoma syndrome in a Chinese man. 299 43

A clinicopathological and immunohistochemical study was carried out on 32 cases of neuroendocrine tumors of the rectum. Typical carcinoids consisted of 27 cases, histologically showing uniform round to columnar cells forming solid alveolar nests and ribbon-like or trabecular arrangement. Neuroendocrine carcinomas consisted of 5 cases in which tumor cells with prominent nuclear atypism were arranged in a ribbon-like or trabecular fashion and formed gland-like structures. There were also small round tumor cells resembling lymphocytes. The prognosis of neuroendocrine carcinomas is very poor with marked tumor invasion of lymphatics and veins resulting in liver metastases and death within one year after operation. Thirty cases out of the 32 showed a positive argyrophil reaction, while immunohistochemistry of 29 cases revealed more than one peptide hormone in 23 cases. The most common hormone was somatostatin being present in 18 of the 23 tumors and glucagon in 16 of the 23 tumors. Gastrin/CCK and calcitonin were proven in 6 of the 23 tumors and in 4 of the 23 tumors, respectively. On the other hand, more than two hormones was present in 15 of the 23 tumors examined. Histologically, neuroendocrine tumors have a very wide spectrum. Histogenetically, typical carcinoids and neuroendocrine carcinomas are considered to be of the same origin with the former showing morphological and functional differentiation to endocrine cells and the latter being more undifferentiated.
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PMID:Histopathological and immunohistochemical study of neuroendocrine tumors of the rectum. 615 May 96

Four patients with metastatic glucagonoma are described. Angiography demonstrated a small avascular primary tumor of the tail of the pancreas in one patient and large hypervascular tumors of the pancreatic head in the other three. Liver metastases, were hypervascular in all four. Including our 4 with 21 cases from the literature, glucagonomas show a 92% incidence of increased tumor vascularity--thus increasing the likelihood of successful angiographic diagnosis. The awareness of clinically subtle or atypical glucagonomas and use of plasma glucagon determination are important factors leading to early diagnosis of these neoplasms. Since angiography can localize the tumor, assess its extent, and detect hepatic metastases, it is essential to the detailed evaluation of glucagonomas.
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PMID:Glucagonoma and its angiographic diagnosis. 630 75

In 1966, during cholecystectomy for cholecystolithiasis, a 56-year-old man was found to have islet-cell carcinoma metastatic to the liver; his fasting serum glucose level was normal. In 1971, he developed peptic ulcer disease and symptoms of fasting hypoglycemia; inappropriate secretion of insulin was shown. His primary pancreatic tumor was removed in 1973. During the next 9 years, his liver metastases continued to grow and his fasting serum glucose level was maintained at 35 to 116 mg/dL with diazoxide and hydrochlorothiazide therapy. In 1982, he developed clinical evidence of the glucagonoma syndrome, with glucagon levels between 4000 and 11 000 pg/mL. Since then, his fasting serum glucose level has been maintained at 58 to 119 mg/dL without medication. This patient has survived 17 years with a malignant insulinoma and without islet-cell chemotherapy. His course shows that malignant insulinomas may secrete other peptide hormones that can induce various clinical syndromes.
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PMID:Metastatic insulinoma with long survival and glucagonoma syndrome. 631 34

Hypoaminoacidemia and skin rash are features of the glucagonoma syndrome. A glucagonoma patient with earlier insulin treated, noninsulin-dependent diabetes, and functional liver metastases, 3 yr postresection of pancreatic tumor, was treated with a high protein diet for 2 wk, then switched to a high carbohydrate diet for 3 wk followed by 3 wk on high protein diet, which continued for 3 months with additional carbohydrate. While on the high protein diet urine nitrogen indicated frank retention and total plasma amino acid levels normalized each time. Plasma amino acid decreased again after 1 wk on the high carbohydrate diet. Skin rash varied irrespective of amino acid levels, but cleared 4 days after resection of metastases. However, total amino acid did not reach normal levels on a conventional diet 3 wk postsurgery, but were normal 6 wk later. A high protein diet can normalize plasma amino acids and allow nitrogen retention in glucagonoma, apparently overriding the gluconeogenic drive of the high circulating glucagon levels. The skin rash may not be only attributed to hypoaminoacidemia.
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PMID:Disappearance of glucagonoma rash after surgical resection, but not during dietary normalization of serum amino acids. 632 Jun 31

A case of a 65-year-old woman with a pancreatic tumor secreting insulin, glucagon, and associated with high PTH levels and hypercalcemia is reported. The patient underwent two Streptozotocin (STZ) treatments (1 g iv/week for 10 weeks) after liver metastases were found. Hormonal and metabolic parameters were monitorized . Before the first STZ treatment insulin levels ranged between 78 and 132 microU/ml. After STZ administration insulin decreased and then remained lower (8-48 microU/ml) until the death of the patient. Pre-treatment glucagon levels ranged between 1.3 and 3.9 ng/ml. STZ induced a decrease of glucagon to 0.5 ng/ml. Glucagon chromatography revealed the prevalence of high molecular weight (greater than 6,000 mol wt) immunoreactive glucagon (0.9 ng/ml) drastically reduced by STZ treatment (0.15 ng/ml). Hypoaminoacidemia was observed before STZ administration, but at the end of the therapy plasma amino acid concentrations were normal. Hypercalcemia too was sensitive to STZ, but not PTH value, which remained high. The second STZ treatment performed a year later was less effective and so a chemotherapeutic protocol was started. Our findings suggest a cytolitic effect of STZ on malignant A-cell, with reduction of glucagon levels and restoration of amino acid metabolism. This effect would be useful for medical treatment of non-operable glucagon secreting tumors.
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PMID:Effect of streptozotocin in a case of glucagon-secreting malignant islets-cell tumor. 632 96

A series of 156 patients with gastrointestinal carcinoid tumors is presented. The tumors were grouped as foregut, midgut and hindgut carcinoids. The appendiceal tumors were grouped separately. Lack of specific symptoms resulted in only 26% correct presumptive diagnoses. This also reflected the patient's delay greater than 2 years in 10% and doctor's delay greater than 2 years in 29%. In the larger group (midgut), 41% were multiple primary tumors. Liver metastases were found in 46% and tumors bigger than 1 cm metastasized to the liver in 58%. The five-year survival of patients with liver metastases was 43% while the figure for those without metastases was 80%. In 47 patients with appendiceal carcinoids, 68% had tumors less than 1 cm. No metastases to the regional lymph nodes, viscera or skeleton were found. Thirty-three patients were traced and none had died of the tumor. Two foregut and 4 hindgut tumors were re-examined using immunocytochemistry. In one of the foregut tumors, cells containing serotonin were found while the other one contained gastrin cells. In 3 hindgut tumors PP-cells were seen and one of them also harbored glucagon/glicentin cells. In one hindgut tumor no peptides were found.
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PMID:Carcinoid tumors in the gastrointestinal tract--an analysis of 156 cases. 665 73

Selective catheterization of hepatic, intestinal and adrenal veins with blood sampling for serotonin and catecholamine determination was evaluated regarding its use in the diagnosis, location and characterization of carcinoids and pheochromocytomas. Catheterization of intestinal veins via the transhepatic route and of the adrenal veins via the femoral and caval veins was performed in 49 patients without major complications. High pressure liquid chromatography with electrochemical detection was used to quantitate norepinephrine and epinephrine in plasma and serotonin in plasma and whole blood. Serotonin in plasma was also determined by an enzymatic procedure. In 30 patients with suspected or verified carcinoid tumors concentration of serotonin in tumor-draining veins was clearly elevated in all patients but one. In this patient, who previously had been treated with temporary liver dearterialization, the serotonin concentration in the hepatic vein was within the normal range in spite of the existence of liver metastases. Hyperserotoninemia was registered in one patient without detectable carcinoid tumor cells. In three patients determination of norepinephrine and epinephrine in adrenal venous blood diagnosed a hyperplasia and tumors in the adrenal medulla. In these cases angiography and computed tomography were negative. Microscopic analyses revealed serotonin in all carcinoids and substance P-like immunoreactivity in a large percentage of these tumors. PP-like and glucagon-like immunoreactivity were observed in two endocrine pancreatic tumors. In normal adrenal medulla and in adrenal medullary tumor tissue catecholamine fluorescence and enkephalin-like immunoreactivity were demonstrated. In the two pheochromocytomas ACTH-like, somatostatin-like and calcitonin-like immunoreactivities were identified. The technique with determinations of plasma serotonin and catecholamines in combination with selective catheterization is a useful investigation for the diagnosis, location and follow-up of patients with carcinoids and pheochromocytomas.
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PMID:Localization of carcinoids and pheochromocytomas with vein catheterization and amine determination. 717 48

The pathogenetic mechanism underlying glucose intolerance in pancreatic cancer is still unclear. We studied the pattern of three glucose regulating hormones (C-peptide, glucagon and GH) in pancreatic cancer patients with (N = 34) and without (N = 8) hyperglycemia, and compared the findings made with those from subjects with other hyperglycemic conditions of well-known origin [type I diabetes mellitus (8 cases) and diabetes mellitus secondary to chronic pancreatitis (13 cases) or liver cirrhosis (4 cases)]. In hyperglycemic pancreatic cancer patients, C-peptide was absent in 26% of the cases, reduced in 24%, elevated in 29% and within the normal range in the remaining 21%. In normoglycemic pancreatic cancer this hormone was reduced in two cases (25%) and within the normal range in all the others. GH was within the normal range in all cases: glucagon was below the normal range in some hyperglycemic pancreatic cancer patients (41%) or within the normal range in all the remaining patients. No correlations were found between the three hormones when findings from subjects were considered all together. However, in pancreatic cancer C-peptide and glucagon presented consensual variations. C-peptide, glucagon and GH levels were not related to tumor volume; glucagon was found to be associated with liver metastases. C-peptide was correlated with serum ALT and ALP. We may conclude that hyperglycemia associated with pancreatic cancer may be caused by different mechanisms. In some cases a reduced secretion of both insulin and glucagon was observed, as occurs in chronic pancreatitis. In the majority of patients, beta cell function appears normal, and the hyperglycemic state may depend on an altered peripheral sensitivity to insulin due to the pancreatic pathology itself or to consensual liver involvement.
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PMID:C-peptide pattern in patients with pancreatic cancer. 813 97


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