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)

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

The glucagonoma syndrome is characterized by a necrolytic migratory erythematous rash, angular stomatitis, painful glossitis, a normochromic normocytic anemia, mild diabetes mellitus, weight loss, a tendency to thrombosis, and neuropsychiatric disturbances. The diagnosis is made by finding a high plasma glucagon concentration in the absence of any other cause, such as renal failure or severe stress. A pancreatic alpha-cell tumor can be identified and stained by immunocytochemistry with glucagon antibodies. Optimal treatment is surgical removal, but approximately 50 percent of the tumors have metastasized by the time of diagnosis. Since the tumor is slow-growing, remission can be obtained by hepatic artery embolization to shrink hepatic secondaries or by shrinkage, in about 10 percent of patients, with the combination chemotherapeutic regimen of 5-fluorouracil and streptozotocin. The rash frequently responds to administration of zinc, a high-protein diet, and control of the diabetes with insulin. Alongside the alpha cell in the islets of Langerhans is the D-cell, which produces somatostatin and may well act physiologically as a paracrine inhibitor of glucagon release. A newly developed, long-acting somatostatin analogue, SMS 201-995, which the patient can self-administer as a subcutaneous injection, has proven effective in suppressing glucagon secretion from glucagonomas and, in some cases, causing remission of clinical symptoms.
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PMID:Glucagonoma syndrome. 288 77

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 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 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

Glucagon-producing tumors of the pancreas are among the rarest forms of islet cell tumors. Two patients are described in whom the characteristic dermatitis, glucose intolerance, weight loss, and anemia of the glucagonoma syndrome were due to a metastasizing islet cell carcinoma. In both, removal of the primary tumor with a distal pancreatectomy brought marked relief of all clinical symptoms for 1- and 2-year periods. Because streptozocin, the usual chemotherapeutic agent for these tumors, is quite toxic and frequently unsuccessful dimethyltriazenoimidazole carboximide (DTIC) was used for recurrence after operation. The first patient began taking DTIC when his rash reappeared and his immunoreactive glucagon (IRG) level rose to 6,000 pg/ml (normal, less than 200 pg/ml). Within 3 months, his rash was gone, and IRG level was 75 pg/ml. The second patient developed a neoplastic gastrocolic fistula and was extremely emaciated. With DTIC, the fistula healed, and he gained weight and returned to work. His IRG level has decreased from 2,975 to 200 pg/ml. No side-effects were noted during chemotherapy. Temporary palliation of malignant glucagon-producing neoplasms can be achieved by cytoreductive surgery. When the life-threatening symptoms of this syndrome recur, DTIC chemotherapy seems indicated because of its safety and effectiveness.
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PMID:Operative and chemotherapeutic management of malignant glucagon-producing tumors. 626 42


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