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)

We report a case of pancreatic tumour metastatic to the liver in a patient with insulin-treated diabetes, anaemia, cheilitis, necrolytic migratory erythema, hypokalemia and chronic watery diarrhea, a picture suggesting combined glucagonoma and VIPoma syndromes. Immunocytochemistry of a biopsied hepatic metastatic nodule revealed both glucagon and vasoactive intestinal peptide (VIP) positive cells. Increased plasma glucagon and VIP levels were detected (values of 900 pmol/l and 277 pmol/l respectively). This is the first reported case showing not only immunocytochemical, but also clinical evidence of the combined secretion of these hormones.
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PMID:A combined glucagonoma and VIPoma syndrome. First pathologic and clinical report. 284 62

A case is described that represents the only reported patient with glucagonoma syndrome and morbid obesity. The diagnosis of glucagonoma should be considered in any patient with the classic criteria despite weight gain. The criteria for diagnosis of glucagonoma are 1) the presence of a glucagon-secreting tumor, 2) hyperglucagonemia, and 3) the clinical manifestations of either necrolytic migratory erythema, glucose intolerance, or hypoaminoacidemia.
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PMID:Glucagonoma presenting as morbid obesity. 284 56

A 63-year-old woman with necrolytic migratory erythema associated with a glucagonoma in the pancreas is described. The diagnosis was suggested on the basis of a characteristic lesion in skin biopsy. Infusion of somatostatin (25 micrograms/h) for 48 hours was followed by a rapid and almost complete healing of the skin eruptions. Serum glucagon was depressed during infusion. No significant changes in plasma glucose were detected. As the patient experienced a cramp-like sensation after cessation of somatostatin infusion, a tail-off period in the infusion program is advocated. The pathogenesis of necrolytic erythema in glucagonoma is discussed.
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PMID:Effect of somatostatin in necrolytic migratory erythema of glucagonoma. 286 72

A 46-year-old man had a 7-year history of severe rash, which was then diagnosed as necrolytic migratory erythema. He had a weight loss of 6 kg, abnormal glucose tolerance test findings, anemia, glossitis, hair loss, and hypoproteinemia. Plasma amino acids levels were significantly decreased, and the fasting plasma glucagon (IRG) level was high at 5000 to 8000 pg/ml. Circulating IRG significantly increased after oral glucose loading, meal ingestion, and arginine infusion, and decreased with somatostatin infusion and insulin-induced hypoglycemia. No other gut or pancreatic hormone levels in plasma were elevated. Plasma IRG was eluted by gel-filtration, mainly in the position of true glucagon (MW 3500) by antiserum 30K. The rash was markedly improved after infusion of amino acids. Computerized tomography (CT) scan and celiac angiography revealed a large pancreatic tumor with multiple liver and lymph node metastases. The pancreatic tumor was totally resected, and was identified as glucagonoma by immunohistochemical technique. Since the plasma IRG levels remained high after surgery, the patient received dimethyltriazenoimidazole carboxamide therapy. After several courses of this treatment, plasma IRG levels decreased to 1000 to 2000 pg/ml, and the hepatic metastases were remarkably diminished in size.
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PMID:A functional study of a case of glucagonoma exhibiting typical glucagonoma syndrome. 286 23

A long-acting somatostatin analog, SMS 201-995, is now available to treat the hormonal manifestations of islet cell tumors. We report its use in a patient with a metastatic glucagonoma refractory to conventional therapy. This patient, who was severely disabled by the rash of necrolytic migratory erythema and brittle diabetes mellitus, allowed us to evaluate the therapeutic efficacy of SMS 201-995 and to gain insight into the origin of the rash. SMS 201-995 was administered subcutaneously (.05 mg twice a day). The rash improved markedly within 48 hours and was completely resolved within 1 week of treatment. Insulin requirements decreased from 90 U/day to zero during the first week of treatment. Corresponding to improvement in clinical symptoms circulating glucagon levels showed a marked decrease. There was no substantial change in plasma or urinary levels of zinc or in plasma amino acid levels. When SMS 201-995 was stopped, the rash recurred within 36 hours and it improved within 48 hours of readministration. The rash and diabetes have remained well controlled during 8 months of therapy but no change in tumor size has been seen on CT scan. The rapid changes in the rash related to the administration of SMS 201-995 indicate that the pathogenesis of necrolytic migratory erythema is probably due to circulating hyperglucagonemia or some other hormonal substance produced by the tumor.
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PMID:Use of a somatostatin analog (SMS 201-995) in the glucagonoma syndrome. 287

A 41-year-old woman with metastatic glucagonoma and the characteristic disabling rash, necrolytic migratory erythema, was treated with a synthetic somatostatin analog while waiting to undergo curative surgical resection. Plasma glucagon concentration (1,500-3,300 pg/ml, normal less than 200) remained elevated during analog therapy as the rash cleared. Only with surgical resection (partial pancreatectomy and partial hepatectomy) did glucagon levels return to normal. The therapeutic benefit caused by the analog in this syndrome differs from that in other endocrine tumor syndromes such as pancreatic cholera, carcinoid, or gastrinoma where circulating levels of tumor-produced agents are suppressed in conjunction with control of symptoms.
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PMID:Somatostatin analog-induced remission of necrolytic migratory erythema without changes in plasma glucagon concentration. 288 3

A 63 year old man presented with features of the glucagonoma syndrome, that is thromboembolic disease, weight loss, raised sedimentation rate, diabetes mellitus, hypoproteinaemia and reduced plasma amino acid levels, but without necrolytic migratory erythema. The plasma glucagon level was raised and the tumour was demonstrated by abdominal CT scan. Immunofluorescent studies of the resected tumour confirmed the diagnosis. The normal tissue zinc status supports the view that necrolytic migratory erythema is related to zinc deficiency.
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PMID:Glucagonoma without cutaneous manifestations. 299 32

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

The glucagonoma syndrome is a rare clinical entity characterized by a glucagon-producing tumor of the pancreas, necrolytic migratory erythema, weight loss and usually decreased glucose tolerance. Lately there has been increasing interest in the interaction of peptide hormones and the immune system, implicating a regulatory role of the peptide hormones on immune activation and function. We present a patient with metastasizing glucagonoma and highly elevated plasma levels of glucagon and pancreatic polypeptide. Normal numbers of peripheral blood lymphocytes with normal proportions of the T and B populations were seen. Within the T lymphocyte population the percentage of T4+ cells (helper phenotype) was increased with a concomitant decrease in T8+ cells (suppressor/cytotoxic phenotype), resulting in an abnormally high T4/T8 ratio of 6.7 (mean reference value 1.8). Functional tests demonstrated a normal tuberculin reaction and adequate lymphocyte responses in vitro to polyclonal activators. Furthermore we noticed a urine electrophoretic pattern consistent with a proximal tubular kidney defect. It is concluded that studying the immune system in patients with endocrine active pancreatic tumors may give clues on the influence of pancreatic peptide hormones on immune function and regulation.
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PMID:Immunological studies in a patient with the glucagonoma syndrome. 299 60

The correlation between radiography, endoscopy, and histology in the diagnosis of reflux esophagitis, as well as the effect of glucagon on double contrast radiography was studied. The material consisted of 220 out-patients sent to the Oulu University Central Hospital for upper gastrointestinal endoscopy. 109 of these were shown to have reflux esophagitis at endoscopy, the other 111 formed a control group with normal esophageal mucosa. Radiologic examinations were performed after endoscopy on the same day by a radiologist, who knew neither the clinical history of the patients nor the findings at endoscopy. Using endoscopy as a reference, 56% (28/50) of the patients with grade E I reflux esophagitis (erythema, oedema) were diagnosed correctly by double contrast radiography. The corresponding figures concerning grade E II (erosions), grade E III (localized deformity, ulcer), and grade E IV (stricture) reflux esophagitis were 84% (41/49), 100% (4/4), and 100% (6/6). False positive findings were found in 4.5% (5/111). The sensitivity of double contrast radiography as compared to endoscopy in all grades was 73%, its specificity was 96%, and accuracy 84%. The corresponding figures, when only grades E II, E III, and E IV are considered, were 86%, 96%, and 92%. In double contrast radiography, signs sometimes visible in grade E I reflux esophagitis were thick mucosal folds and mucosal granularity. Reliable signs of grade E II reflux esophagitis were streaks and dots of barium against the mucosa either alone or together with thick mucosal folds and mucosal granularity. Specific signs of grade E III and E IV reflux esophagitis were--along with the above--localized deformities, ulcers, and strictures. A hiatus hernia or wide hiatus was detected radiologically in 2/3 of the reflux esophagitis patients, and in 1/3 of the controls. Histologic findings correlated poorly with both endoscopic and radiologic findings. Single contrast radiography was less sensitive than double contrast radiography in detection of superficial mucosal lesions. Glucagon had no advantagous effect on esophageal double contrast radiography. Its use, however, in connection with double contrast radiography of the stomach is unlikely to have any disadvantagous effect on the evaluation of the hiatus and gastroesophageal reflux.
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PMID:Diagnosis of reflux esophagitis. With special reference to double contrast radiography. 403 76


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