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 autopsy findings in a 58 year old female with the typical glucagonoma syndrome with heart muscle hypertrophy are described. Despite remission of a skin rash and hyperglucagonemia following removal of metastatic liver tumors, the patient died of massive cerebral hemorrhage caused by uncontrolled hypertension and septic cerebral angiitis. On the basis of surgical and autopsy materials, the tumors in the pancreas and liver were defined as malignant glucagonoma by histological, histochemical, immunohistochemical, and ultrastructural studies. The pathohistological changes in several organs that might be related to the hypersecretion of pancreatic glucagon are discussed.
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PMID:A postmortem study of malignant glucagonoma with heart muscle hypertrophy, including chemical, histochemical, immunohistological and ultrastructural observations. 627 84

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

We have described a 77-year-old woman with a 14 x 15 cm cystic mass arising from the head of pancreas. The serum glucagon level was elevated, and electron microscopy revealed large numbers of alpha granules within the tumor cells. Glucagonoma syndrome should be considered in the differential diagnosis of a large abdominal mass even if skin rash is minimal.
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PMID:Glucagonoma occurring as a large cystic abdominal mass. 632 36

A 39 year old man developed an itchy bullous rash in the perineum and on the extremities. Six years later, after giant intestinal villi had been noted at endoscopy, a diagnosis of the glucagonoma syndrome was made. Investigation revealed a large tumour of the pancreatic body and tail. The molecular species of glucagon secreted by the tumour were characterised using the combined purification procedures of immunoaffinity chromatography followed by gel filtration.
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PMID:Glucagonoma syndrome demonstrating giant duodenal villi. 632 23

A patient is described who presented with a 10 year history of intermittent peptic ulcer symptoms and a 3 year history of an undiagnosed skin rash. Investigations indicated raised plasma levels of gastrin, glucagon and pancreatic polypeptide. A single tumour was localised to the pancreas and resected. The tumour had the typical histology of an apudoma, and contained cells which stained for gastrin, glucagon, pancreatic polypeptide and neurotensin.
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PMID:A unique apudoma producing the glucagonoma and gastrinoma syndromes. 687 3

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

A 55-year-old man presented with an 11-year history of necrolytic migratory erythema and glossitis. After the patient's serum glucagon was demonstrated to be elevated, computed tomography scan revealed a mass involving the head of the pancreas. The patient underwent a Whipple-type pancreatico-duodenectomy and his rash resolved completely 6 days after tumor resection. He received no adjuvant treatment. A discussion of the varying theories regarding the pathogenesis and treatment of glucagon-associated necrolytic migratory erythema is presented.
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PMID:Rapid resolution of necrolytic migratory erythema after glucagonoma resection. 862 4

Glucagonoma is a relatively rare pancreatic islet cell tumor. Historically, these tumors present a typical constellation of symptoms including diabetes, weight loss, anemia, necrolytic migratory erythematous rash, and propensity for thrombosis. This clinical presentation is described as the glucagonoma syndrome. The syndrome can be confirmed with the use of serum measurements of glucagon levels and immunohistochemical assay of the tumor. Variations from the classic syndrome have been described, and serum measurements of glucagon in patients with suspected islet cell tumors can identify subsets of patients with glucagonoma who do not exhibit the classic syndrome. In our case, the unusual presentation of glucagonoma included the previously unreported component of an intravascular venous extension of tumor.
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PMID:Intravascular tumor: a previously unreported finding of glucagonoma. 922 1

Glucagon-producing neuroendocrine tumors typically present with a characteristic constellation of symptoms including necrolytic migratory erythema, non-insulin-dependent diabetes, weight loss, anemia, glossitis, and an increased thrombotic tendency. Most glucagonomas are solid and arise in the body or tail of the pancreas. We report two cases of cystic glucagonoma, one found incidentally in an asymptomatic patient and one in a patient with weight loss and diabetes but no rash. In the first patient, distal pancreatectomy and splenectomy were curative, whereas the second patient continued to exhibit elevated serum glucagon levels and symptoms of glucose intolerance in the absence of demonstrable metastases. Cystic glucagonoma is a unique variant of classic glucagonoma and should be considered in the differential diagnosis of cystic pancreatic neoplasms.
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PMID:Cystic glucagonoma: A rare variant of an uncommon neuroendocrine pancreas tumor. 1045 11

It is clear that cutaneous lesions of metabolic epidermal necrosis in the dog can occur either with a demonstrable glucagon-secreting tumor or with hepatic disease without any detectable glucagonoma. Additional clinical case reports of the disease in cats are needed to better characterize the disease in this species. The lesions of NME-MEN may not represent a specific physiological mechanism of cutaneous disease but instead a pathophysiological process that can be triggered by several systemic metabolic abnormalities. The fact that NME is observed in association with a variety of conditions supports the theory that an overall metabolic derangement results in the rash. The prognosis for canine MEN is poor; however, some affected dogs have been maintained for many months with dietary management. High-quality protein diets such as Hill's Prescription Diet a/d (Hill's Pet Products) or other "recovery" diets may be helpful. Zinc and essential fatty acid supplementation may help some patients. Dietary supplementation with cooked egg yolks may be helpful. It is prudent to avoid corticosteroids in these cases, as development of diabetes mellitus worsens the prognosis. Histopathological examination of the pancreas coupled with determination of plasma glucagon may help define the characteristics of GS versus HS in dogs. It is possible that some dogs diagnosed with MEN-HS may have an undetected pancreatic tumor. Although the hepatic ultrasound findings in dogs with MEN-HS are becoming well characterized, it is possible for dogs with pancreatic neuroendocrine tumors to also have abnormal hepatic ultrasonography. As the presence of MEN and hepatic disease does not necessarily rule out the presence of a pancreatic tumor, prospective studies correlating plasma glucagon levels with pancreatic histopathology in cases of MEN-GS versus MEN-HS seem warranted.
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PMID:Metabolic epidermal necrosis-hepatocutaneous syndrome. 1056 4


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