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)

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

The authors report a case of glucagonoma in a 52 years old man presenting a migratory necrolytic erythema. By conjugated means of arteriography and splenoportography with plasma glucagon assays the tumour was localized in the tail of the pancreas. Surgical excision was easy but hepatic metastases revealed the malignant nature of the tumor. This glucagonoma has been investigated by several approaches including electron microscopy, immunocytochemistry and radioimmunological techniques. The tumor contained scattered glucagon and pancreatic polypeptide immunoreactive cells; insuline, glucagon, somatostatin, pancreatic polypeptide, gastrin and VIP antisera gave negative results. Ultrastructurally, these cells showed atypical secretory granules different from A granules of the normal glucagon cell. Radio immunological determinations carried out after gel permeation chromatography of plasma revealed high molecular weight (4 000, 9 000, 14 000) immunoreactive glucagon peptides. They have been thought to be proglucagon forms which did not react with specific antiglucagon sera used in cytological studies. Reported data are consistent with the classification of this tumor in the category of glucagonoma with the "glucagonoma syndrome".
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PMID:[A human pancreatic glucagonoma, ultrastructural, immunocytochemical and radioimmunological investigations (author's transl)]. 627 65

Diabetic ketoacidosis is an extremely rare manifestation of glucagonoma. We report such a case in a 72-year-old woman known to be diabetic for seven years. The patient was admitted with diabetic ketoacidosis and associated necrolytic migratory erythrema which suggested the diagnosis of glucagonoma. Plasma glucagon levels were increased (569 to 2298 pg/ml). A vascular tumor of the head of the pancreas without obvious hepatic metastases was visualised by angiography. Duodeno-pancreatectomy including the head of the pancreas led to complete recovery of the mucocutaneous lesions and the plasma glucagon level fell (229 pg/ml). The tumor had several histological characteristics suggesting malignancy and a high glucagon content on extraction. Electron microscopy showed multiple A cells and a few isolated B cells. Most of the cells showed immunoreactivity with anti-glucagon and anti-glicentine antibodies. Three months after surgery, the diabetes was again required treatment with insulin. Plasma glucagon level was again increased and chemotherapy with dimethyltriazenimidazolecarboxamide was undertaken.
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PMID:[Glucagonoma with diabetic ketoacidosis; case report]. 629 11

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

Peripancreatic venous abnormalities were demonstrated angiographically in 10 patients with islet cell tumor: six nonfunctioning, two gastrin-producing, one glucagon-producing, and one pancreatic polypeptide-producing. Venous involvement recognized included venous occlusion, venous encasement, and intraportal tumor growth. Seven patients had islet cell carcinoma with hepatic metastases while the other three had benign tumors. Three patients had arteriographic evidence of intraportal tumor growth with the "thread and streaks" sign, similar to that of portal venous extension of hepatocellular carcinoma.
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PMID:Venous involvement in islet cell tumors of the pancreas. 632 Jun 13

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

Alpha-lactalbumin (ALA), a milk protein, was demonstrated by immunohistochemistry with polyclonal antisera from three different sources in primary and metastatic breast carcinomas, and mammary Paget's disease. ALA localization was observed in 67% of mammary carcinomas, and in 62% of their metastases to sites which included lymph nodes, lung, bone, liver, pericardium, skin, and subcutaneous tissue. There was close correlation between primary and metastatic breast carcinomas in ALA positivity, but no correlation between ALA positivity and histologic differentiation. A variety of nonmammary neoplasms were examined for ALA immunoreactivity. In contrast to ALA immunoreactivity of breast tissue, which was removed by preabsorption of antiserum with ALA antigen, a number of skin appendage tumors, salivary gland tumors, and mesotheliomas demonstrated positive staining which was not abolished by preabsorption and was most likely due to the presence of cross-reacting antibodies. One commercial ALA antiserum also reacted with pancreatic islet cells in a distribution similar to glucagon. Our results demonstrate the presence of ALA in breast carcinomas and its potential value to the surgical pathologist in the workup of metastatic carcinomas of unknown primary sites. However, the staining encountered in some nonmammary tumors necessitates caution in its interpretation.
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PMID:Alpha-lactalbumin as an immunohistochemical marker for metastatic breast carcinomas. 632 3

Two cases of glucagonoma, one benign and the other malignant, was presented. Benign glucagonoma in a 29-year-old man with multiple endocrine neoplasia type 1 was composed largely of tumor cells with secretory granules ranging from 139 to 417 nm in diameter identical to A cell granules. There were a few tumor cells which contained no A cell granules but smaller granules of approximately 166 nm diameter similar to those of pancreatic polypeptide containing cells. Radioimmunoassay of the tumor extract showed 319 micrograms/g wet weight of glucagon and 0.72 microgram/g wet weight of pancreatic polypeptide. Malignant glucagonoma in a 34-year-old man was a massive tumor of 7 X 6 X 5 cm replacing the tail and body of the pancreas with multiple metastases. The tumor contained 0.2 microgram/g wet weight of glucagon and 0.065 microgram/g wet weight of vasoactive intestinal peptide. The electron microscopic examination revealed that the tumor cells had variable numbers of atypical secretory granules measuring 110 to 200 nm in diameter different from A cell granules. An analysis of plasma glucagon by the gel filtration technique showed the heterogeneity of glucagon molecules indicating the presence of large glucagon. Atypical secretory granules in malignant glucagonoma were considered to represent immature granules containing the precursor or intermediate of glucagon.
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PMID:Secretory granules in benign and malignant glucagonomas of the pancreas. 632 64

Twenty-four patients who had undergone radical pancreatic resection were evaluated by CT one week to 11 years after surgery. Eighteen patients had had the Whipple procedure; six had had total pancreatectomy. The region between the aorta and superior mesenteric artery, previously occupied by the uncinate process of the pancreas, is an important area to evaluate for tumor recurrence because periampullary tumors tend to metastasize to the lymph nodes in this region. Tumor recurrence here is readily detectable by CT since radical pancreatectomy leaves this area free of soft tissue attenuation material. Administration of glucagon prior to CT scanning helps fill the afferent jejunal loop with oral contrast material, and thus helps define right upper quadrant anatomy. CT demonstrated postoperative complications or tumor recurrence in 16 of the 24 patients and was 100% accurate in patients who had follow-up.
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PMID:Radical pancreatectomy: postoperative evaluation by CT. 647 82

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


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