Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011849 (diabetes)
 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 36 year old woman was admitted because of upper abdominal pain, fullness and weight loss. Pancreatic scintigram revealed abnormal accumulation of the radioisotope in the pancreatic head, and hepatic scintigram showed multiple filling defect in the bilateral lobe. Celiac angiogram demonstrated a tumor stain at the pancreatic head, encasement of the splenic artery and metastasis to the liver. The diagnosis of malignant glucagonoma was substantiated by high serum glucagon level of 1,100 pg/ml. Streptozotocin of 1.5g was administered intravenously once a week, totalling 9g. Thereafter, blood level of glucagon declined to the normal range, accompanied by improvement of diabetes mellitus and weight gain. At laparotomy, there was an over fist-sized mass at the body and tail of the pancreas, infiltrating the pancreatic head and periaortic region, and was found unresectable. On light microscopy, biopsied specimen was seen to be a tumor that contained glucagon. Secretary granules resembling A cell granules were observed by electron microscopy. She died of emaciation 6 years after the onset of the disease. Eighteen cases of glucagonoma reported in Japan, and 64 cases in Europe and the United States were reviewed in terms of the diagnosis and treatment.
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PMID:[Glucagonoma]. 609 16

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

Many of the features of zinc deficiency and of essential fatty acid (EFA) deficiency are similar in both animals and humans. The two agents interact in various ways. EFAs are important in zinc absorption, probably after conversion to prostaglandins (PGs). Zinc seems necessary for at least two stages in EFA metabolism, the conversion of linoleic acid to gamma-linolenic acid, and the mobilisation of dihomogammalinolenic acid (DGLA) for the synthesis of 1 series PGs. Zinc may also be important in the conversion of DGLA to arachidonic acid and in arachidonic acid mobilisation for 2 series PG formation. These interactions shed considerable light on a number of clinical syndromes, including acrodermatitis enteropathica, total parenteral nutrition, diabetes mellitus, the glucagonoma syndrome and sickle cell anaemia. There is substantial evidence to suggest that anorexia nervosa is due to a combined deficiency of zinc and EFAs. Understanding of the roles of zinc and EFAs in these various clinical situations is likely to lead to improved therapy.
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PMID:Interactions between zinc, essential fatty acids and prostaglandins: relevance to acrodermatitis enteropathica, total parenteral nutrition, the glucagonoma syndrome, diabetes, anorexia nervosa and sickle cell anaemia. 625 72

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

A further case of glucagonoma is reported, unusual features being the prolonged duration of cutaneous manifestations (12 years), and the absence of diabetes. Possible explanations for this anomaly: compensatory insulin or somatostatin secretion, production of a non functional glucagon, or low levels of circulating glucagon, are envisaged but without a formal response.
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PMID:[Glucagonoma without diabetes: a case report (author's transl)]. 626 28

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

A 60-year-old patient developed signs and symptoms of glucagonoma syndrome (dermatitis, weight loss, anemia and hypoaminoacidemia). However, diabetes mellitus was absent. Glucagonoma was suspected because of markedly elevated plasma glucagon levels and the tumor was subsequently removed by surgery. Acidethanol extraction of the tumor and immunohistochemistry provided evidence of the presence of all four islet hormones, particularly that of glucagon and pancreatic polypeptide and to a lesser extent of somatostatin and insulin. Immunohistochemistry of the tumor (but not plasma) also showed the presence of alpha-HCG. Plasma glucagon immunoreactivity consisted to a large extent (approx. 90%) of a high molecular form of glucagon, probably proglucagon. In spite of the presence of alpha-HCG - which is assumed to be a marker of malignancy - the patient has been free of recurrence for the 2 1/2 years since surgery. The increasing number of cases reported during the past few years demonstrates that the syndrome is more common than previously suspected. Glucagon secretion and its typical clinical picture may be a valuable marker of a multihormonal pancreatic tumor. In a case of suspected glucagonoma, diagnosis can be established simply by obtaining a plasma glucagon level measurement.
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PMID:[Glucagonoma syndrome in a multihormonal pancreatic tumor]. 628 78

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


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