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Query: UMLS:C0011849 (diabetes)
 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pancreatic tumors harboring glucagon immunoreactive cells were found in four patients with diabetes mellitus. Alpha-cell (glucagon) granules were present in three tumors; pancreatic polypeptide (PP) immunoreactive cells were detected in two. In two patients the tumors were malignant and one of these had the glucagonoma syndrome; the other was a member of a family with MEN-type I syndrome. These cases illustrate three clinical subtypes of glucagonoma.
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PMID:Glucagonomas. Ultrastructure and immunocytochemistry. 629 22

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

The more common paraneoplastic skin changes associated with internal malignancies (acanthosis nigricans, dermatomyositis, secondary ichthyosis) are discussed. The rare glucagonoma syndrome, consisting of alpha cell tumor of the pancreas, migratory necrolytic erythema, and diabetes mellitus, is described. The importance of recognition of these skin lesions may lead to early detection of the underlying cancer with possible cure.
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PMID:Cutaneous signs of internal malignancies. 684 28

The features of 41 proven or suspected cases of pancreatic glucagonoma and one possible case of renal glucagonoma have been reviewed. Glucagonoma is one form of islet cell neoplasm and involves pancreatic alpha cells. It may occur more frequently in women and is more likely to be malignant than insulinoma. Patients may present with glucose intolerance, an erythematous, eczematous dermatitis, glossitis, stomatitis, vaginitis and unexplained weight loss. Anemia, hypoproteinemia, hypoaminoacidemia and hypolipidemia may also be present. Malignant glucagonoma metastasizes frequently to liver. An evaluation for possible glucagonoma may be considered in a patient with the characteristic eczematous dermatitis, glossitis or stomatitis and glucose intolerance, an unusual or atypical history of diabetes mellitus, or hepatomegaly with other characteristics of glucagonoma. Initial evaluation may include measurement of fasting plasma glucagon concentration, and an oral glucose tolerance test with measurements of plasma glucose and glucagon levels. Extreme fasting hyperglucagonemia, and a paradoxical rise in plasma glucagon concentrations after glucose ingestion should strongly suggest the presence of glucagonoma. Radiographic demonstration of pancreatic glucagonoma is best carried out by celiac arteriography. Surgical excision of the tumor is the treatment of choice. Nonresectable lesions may respond to chemotherapy with streptozotocin. Treatment for the various dermatologic or metabolic complications of glucagonoma which include glucose intolerance, hypoproteinemia, hypocholesterolemia and anemia may not be satisfactory. Glucose intolerance is usually mild and may be adequately treated with dietary or insulin therapy. Rarely, glucagonoma with massive destruction of the pancreas or other factors may induce severe glucose intolerance. In contrast, the anemia, skin rash, and hypoproteinemia do not respond to conservative therapies tested thus far. Glucagonoma is a model for studying the importance of glucagon in causing the hyperglycemia of diabetes mellitus. Study of patients with glucagonoma does suggest that glucagon has some role in the etiology of hyperglycemia in diabetic states; however, as in studies on diabetes, investigations on glucagonoma do not demonstrate that glucagon has a primary role in producing severe glucose intolerance.
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PMID:Clinical and metabolic aspects of glucagonoma. 698 81

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

Glucagonoma is a rare pancreatic tumor, necrolytic migratory erythema is its distinctive feature and it is often associated with diabetes mellitus, weight loss, anemia, hypoaminoacidemia, glossitis and stomatitis. We reported a case of glucagonoma misdiagnosed as "eczema" and "benign hepatic anginoma" for 3 years. His blood glucagon level was 1,758 ng/L. The results of abdominal B-mode ultrasonography and CT scan were negative, but selected arteriogram showed a tumor mass between the pancreatic body and tail. Before operation, treatment with octreotide and supply of amino acids were given with improvement of the skin lesion. After resection of the tumor from pancreas, necrolytic migratory erythema disapeared, but his blood level of glucagon and amino acids did not improve. It is suggested that any diabetic patient with chronic skin damage should be checked for blood glucagon level. In suspected cases, selected arteriogram will be helpful for location of the tumor. Vigorous resection of the pancreatic tumor should be done as soon as possible, even though there is already metastases.
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PMID:[Report of a case of glucagonoma misdiagnosed as "eczema" and "hepatic angioma" for three years and review of literature]. 764 42

Different glucagon-like peptide-1 (GLP-1) molecular forms are produced in the pancreas and the small intestine by differential processing of proglucagon. In this report, molecular forms of GLP-1 in two human pancreatic endocrine tumors were studied and compared with those in the pancreas and small intestine. A predominant GLP-1 immunoreactive form in the pancreas was eluted at the position of GLP-1(1-36) amide, whereas a predominant immunoreactive form in the ileal mucosa was eluted at the position of GLP-1(7-36) amide. In a glucagonoma, GLP-1 immunoreactive forms corresponding to GLP-1(7-36) amide and GLP-1(7-37) were predominant and immunoreactive forms at the position of GLP-1(1-36) amide and GLP-1(1-37) were minor. In another tumor, an insulinoma, immunoreactive forms were detected at the positions of GLP-1(7-36) amide, GLP-1(7-37), GLP-1(1-36) amide and GLP-1(1-37). Thus, the pattern of GLP-1 molecules in pancreatic tumors was not a pancreatic pattern and mimicked that found in the small intestine or consisted of both the patterns found in the small intestine and the pancreas. These data suggest that neoplastic transformation of the islet cells is associated with a switching in processing phenotype from islet (A) cells to intestinal (L) cells.
Diabetes Res Clin Pract 1994 Aug
PMID:Glucagon-like peptide-1 (GLP-1) molecular forms in human pancreatic endocrine tumors resemble those in intestine rather than pancreas. 783 11

alpha-Cells secrete glucagon in a fuel-dependent fashion. We tested the hypothesis that alpha-cells contain sulfonylurea- and ATP-sensitive K+ channels. We studied two clonal lines of alpha-TC cells (simian virus 40 T-antigen induced glucagonoma cells) and for reference purposes, similarly transformed beta-TC insulinoma cells. alpha-TC cells each contained approximately 3000 high-affinity binding sites for the sulfonylurea [3H]glyburide. Whole-cell ATP- and tolbutamide-sensitive K+ currents of alpha-TC and beta-TC cells, relative to cell surface area, were comparable. In cell-attached membrane patches of alpha-TC cells, two types of K+ channels were observed. They had slope conductances of approximately 63 and 33 pS when the electrode contained 151 mM K+. Tolbutamide and diazoxide decreased and enhanced, respectively, the open probability of these channels. The membrane of alpha-TC cells depolarized periodically. This electrical activity was inhibited by diazoxide. A physiological mixture of amino acids enhanced glucagon release, and high glucose partially inhibited this release. Tolbutamide also enhanced glucagon release, whereas diazoxide inhibited it. Thus, alpha-TC glucagonoma cells contain ATP-sensitive K+ channels that regulate glucagon release, yet allow inhibition of hormone release by glucose.
Diabetes 1993 Dec
PMID:Sulfonylurea-binding sites and ATP-sensitive K+ channels in alpha-TC glucagonoma and beta-TC insulinoma cells. 824 22

A 67-year-old man with diabetes, weight loss and anaemia initially presented with a widespread scaling erythematous rash; a skin biopsy demonstrated marked suprabasal acantholysis. A subsequent biopsy showed localized upper epidermal necrolysis and the diagnosis of glucagonoma syndrome was later confirmed. Glucagonoma syndrome should be considered in patients with diabetes, weight loss and anaemia who present with a scaling rash, the histology of which shows suprabasal acantholysis. The extent of any upper epidermal necrolysis may be be very limited.
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PMID:Suprabasal acantholysis--an unusual feature of necrolytic migratory erythema. 825 73

A polymerase chain reaction-based subtractive hybridization procedure was applied to cDNAs prepared from mouse insulinoma (beta TC3) and glucagonoma (alpha TC2) cell lines to construct a library of cDNAs that are highly expressed in pancreatic beta-cells. An analysis of 555 randomly chosen clones in the library showed that 80 were derived from abundant mRNAs and were accounted for by 29 distinct sequences. Of these, 17 were identical or homologous to known mammalian cDNAs or expressed sequence tags. Genes known to be highly expressed in beta-cells were represented at a high frequency, namely insulin (15 of 80 clones), islet amyloid polypeptide (8 of 80 clones), proinsulin convertase 1 (6 of 80 clones), and neuropeptide Y (2 of 80 clones). Many of the novel cDNA sequences that were highly represented in the library showed a relative specificity to beta-cells compared with other tissues, including glucagonoma, liver, kidney, brain, 3T3 fibroblasts, and AtT20 corticotrophs, and warrant further investigation. When combined with functional or immunological screening procedures, the approach will be useful for the isolation of beta-cell-specific molecules for immunological and genetic investigations of beta-cell function and pathology.
Diabetes 1996 Feb
PMID:A subtractive cloning approach to the identification of mRNAs specifically expressed in pancreatic beta-cells. 854 54


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