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 author reviews a personal and other reported cases of necrolytic migratory erythema, a useful marker of glucagon-secreting islet cell tumours of the pancreas. The lesions extend and migrate as the centres form either a bulla or a parakeratotic crust. The eruption involves particularly the body folds, feet and thighs; evolves in waves. The tongue may be smooth and firey red. Histologically, the striking feature is an eosinophilic degeneration and death of the upper part of the epidermis. Ablation of the tumour is followed by cure of the rash but in many cases the presence of metastases makes cure impossible. The exact cause of the eruption remains unknown.
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PMID:[Necrolytic migratory erythema: glucagonoma syndrome. Clinical and histological aspects]. 609 68

A 66 year old patient with diabetes had a necrolytic migratory erythema, weight loss and anaemia. Plasma immunoreactive glucagon (IRG) of 2465 pmoles/l (normal 35 +/- 5 SEM pmoles/l) suggested the existence of a glucagonoma which was confirmed by arteriography and subsequently removed by surgery. Although plasma IRG returned to normal, glucose tolerance and insulin secretion remained pathological. Plasma amino acid levels had been reduced but were corrected by surgery. Pancreatic polypeptide, however, 298 pmoles/l before was still 206 pmoles/l after the operation (normal 12-48 pmoles per litre). Column chromatography of plasma and tumor extracts showed quantitatively important IRG fractions with molecular weights above 9000 daltons, possibly precursors of glucagon. Beside a 50-fold IRG excess, the tumour concentrations of insulin and somatostatin were 4 to 150 times increased. By contrast, pancreatic polypeptide was present in normal amounts. Electron microscopic examination showed atypical A-cell granula and unusual abundance of mitochondria.
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PMID:In vitro and in vivo studies on glucagonoma tissue. 610 27

A 73-year-old man had diabetes mellitus, diarrhea, weight loss, and a rash of several years' duration. The rash, termed necrolytic migratory erythema, was the most characteristic feature and eventually suggested the diagnosis of a glucagon-secreting tumor of the pancreas. Diabetic ketoacidosis also developed in our patient, a previously unrecognized occurrence with this syndrome.
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PMID:The glucagonoma syndrome. Report of a case. 624 59

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

Four patients with metastatic glucagonoma and one patient with metastatic diarrheogenic islet cell carcinoma of the pancreas were treated with dimethyltriazenoimidazole carboxamide (DTIC), 250 mg/M2 daily for five days repeated every four weeks. All patients responded clinically and chemically in one or more ways by a reduction in plasma glucagon levels, improved glucose tolerance, decreased measureable tumor, weight gain, and resolution of necrolytic migratory erythema and diarrhea. This experience and other cases from the literature call for the investigation of DTIC as the initial therapy in metastatic islet cell carcinoma of the pancreas and as being of possible benefit in other tumors of neuroendocrine origin.
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PMID:Dimethyltriazenoimidazole carboxamide therapy of islet cell carcinoma of the pancreas. 626 76

Review of the 55 reported cases of glucagon-producing tumors reveals that a distinctive clinical syndrome consisting of diabetes, a peculiar dermatitis termed necrolytic migratory erythema, weight loss and an increased tendency for thrombosis is associated with these neoplasms. Normochromic normocytic anemia, hypocholesterolemia, hypoproteinemia and generalized hypoaminoacidemia are frequent laboratory findings. Definitive diagnosis of a glucagonoma requires elevation of the fasting serum glucagon level. Selective arteriography of the pancreas has been the best method for localizing these neoplasms preoperatively, but the noninvasive technics of ultrasound and CAT scanning can also be helpful. When the tumor is benign, complete surgical excision can completely reverse all the clinical manifestations of the glucagonoma syndrome and result in lasting cure. Since, however, approximately three-fourths of these tumors are malignant, palliative therapy is frequently required. Cytoreductive surgery can decrease the amount of hormone-producing tissue and can improve or even temporarily reverse the clinical symptomatology. For disseminated disease, chemotherapy is necessary. The best results have been obtained with DTIC although streptozotocin has also been used.
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PMID:Clinical aspects of glucagon-producing islet cell tumors. 627 69

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

A diabetic patient developed necrolytic migratory erythema with extensive angioplasia and high molecular weight glucagon-like polypeptide. There was no associated neoplasm such as glucagonoma. Lesions in the skin were studied by standard optical microscopy and by radioautography after incorporation of tritiated thymidine. Alterations in the skin begin as focal necrosis in the epidermis and in epithelial structures of adnexa, followed by marked angioplasia and a superficial and deep perivascular dermatitis.
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PMID:Angioplastic necrolytic migratory erythema. Unique association of necrolytic migratory erythema, extensive angioplasia, and high molecular weight glucagon-like polypeptide. 689 98

Glucagonoma Syndrome is a rare syndrome comprising hyperglucagonemia, diabetes mellitus, necrolytic migratory erythema and hypoaminoacidemia in the setting of a glucagon producing, alpha cell tumour of the pancreas. We report a case of Glucagonoma Syndrome palliatively treated successfully with octreotide. In addition to classical clinical and biochemical findings, this patient also had a Glomus Jugulare tumour, and Empty Sella Syndrome and demonstrated an unusual pattern of plasma lactate dehydrogenase isoenzymes, features not previously reported in this syndrome.
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PMID:Glucagonoma syndrome with increased lactate dehydrogenase isoenzymes: octreotide treatment. 752 1


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