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 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

Within four years a 44-year-old man developed a glucagonorma syndrome with insulin dependent diabetes mellitus, weight loss, diarrhea, anemia and a marked superinfected eczema. He developed an organo-cerebral psychosyndrome with cognitive retardation and syncoptic disturbance of consciousness, followed by a tetraspasticity with tetraparesis, micturition difficulties and fecal incontinence. There were a general cerebral atrophy as verified by means of MRT and signs of a demyelinating cerebral disease. The plasma concentration of glucagon was 48 fold elevated to 8,536 ng/l. By means of ultrasonography, CT, ERCP, and angiography a tumorous mass of the corpus and tail of the pancreas, 61 x 32 mm in size, was found with signs of infiltration into the region of the aorta and the splenic vein. Furthermore the liver showed diffuse partially cystic metastases. The diagnosis was certified by fine needle biopsy and histologic examination with Grimelius straining. A thrombosis of the femoral vein was detected by CT. The patient was treated by a debulking resection of the corpus and cauda of the pancreas combined with splenectomy and a drug therapy using octreotide. All paraneoplastic symptoms could be widely reduced. Plasma glucagon concentration decreased from 2,200 ng/l to 600 ng/l. Because of a liver enlargement due to the growth of metastases he was successfully treated with dacarbazine 250 mg/m2 per day during six monthly cycles for five days and interferon-alpha 3 x 3 millions units per week for six months followed by a normalization of the liver volumen.
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PMID:[Paraneoplastic spastic tetraparesis in glucagonoma syndrome. Successful therapy with octreotide, dacarbazine and interferon-alpha]. 892 39