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)

Most, if not all, of the glucagon-producing tumours of the pancreas are malignant. For this reason an early diagnosis is essential. The glucagonoma syndrome is associated with a skin rash, stomatitis, anaemia, glucose-intolerance, hypoaminoacidaemia, weight loss, elevated sedimentation rate and hyperglucagonaemia. The more important and constant findings are the skin lesion, the low level of aminoacids in the blood and the increased glucagon concentrations. The skin lesion is not pathognomonic, but any therapy-resistant bullous dermatosis which microscopically is characterized by epidermal changes should alert the clinician to suspect a glucagonoma. The syndrome can be proved by demonstration of hyperglucagonaemia and a pancreatic tumour.
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PMID:Possible entries to the diagnosis of a glucagon-producing tumour. 22 89

Four patients with glucagon-producing tumours of the pancreas were investigated. Fasting plasma glucagon concentrations ranged from 209--625 pmol/l. Plasma insulin concentrations were normal except in one patient, where the tumour also produced insulin (558 pmol/l). Intravenous glucose (25 g/m2) depressed the glucagon concentration in two patients, while no change was noted in the others. Intravenous arginine stimulated glucagon secretion in three patients, but not in the fourth. Intravenous somatostatin suppressed glucagon secretion in all three patients investigated. All patients had abnormally low plasma levels of individual amino acids; glucogenic and branched-chain amino acids were equally depressed. Surgical removal of the tumours led to complete recovery from dermatosis and the glucagon levels were normalized. Postoperative tests were performed in three patients. The alpha-cell responsiveness to iv glucose was restored. Glucose tolerance (Kg-value) was improved in one patient (0.73 to 1.65), persistently low in one patient (0.75 to 0.72) and impaired in the third patient (1.35 to 1.09). It is concluded that none of these functional tests will be of diagnostic value in cases suspected of glucagonomas. The results also show that glucose homeostasis is remarkably unaffected by the extreme hyperglucagonaemia of these patients and that hypoaminoacidaemia is an important consequence of chronic hyperglucagonaemia.
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PMID:Functional studies in patients with the glucagonoma syndrome. 51 Aug 30

Two patients with clinical and histologic findings consistent with necrolytic migratory erythema are presented. Unlike previously described patients with this disorder, neither patient had substantially elevated glucagon levels nor an associated pancreatic islet cell tumor. The cause of the skin disease in these patients remains unknown but may be related to the underlying small-bowel disorder present in both.
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PMID:Necrolytic migratory erythema without glucagonoma. 53 89

A newly recognized disease in dogs, ulcerative dermatosis associated with diabetes mellitus (diabetic dermatopathy), was diagnosed in 2 dogs with pancreatic endocrine tumors that had immunohistologic evidence of glucagon production. Dogs developed diabetes mellitus in the later stages of the illness, months after the skin disease was first observed. Liver disease was identified and characterized by high serum alkaline phosphatase and alanine transaminase activities. Clinically, erythema and crusting involved the footpads, the face, perioral and genital skin, and ventrum. Histologically, skin lesions were intercellular and intracellular edema and necrosis of the upper half of the epidermis and diffuse parakeratosis. Clinically and histologically, skin lesions closely resembled necrolytic migratory erythema of people, a skin disease that usually is associated with a glucagon-secreting pancreatic endocrine tumor and diabetes mellitus (glucagonoma syndrome): The morphologically descriptive term, superficial necrolytic dermatitis, was preferred over the previously proposed names hepatocutaneous syndrome and diabetic dermatopathy, which each connote only a single feature of the disease.
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PMID:Glucagon-producing pancreatic endocrine tumors in two dogs with superficial necrolytic dermatitis. 227 59

Necrolytic migratory erythema has been considered to be a paraneoplastic dermatosis. Although some individuals have had no associated neoplasm, a pancreatic glucagonoma is typically found in the vast majority. We describe a 71-year-old woman with findings strongly suggestive of the glucagonoma syndrome. The plasma glucagon level was modestly elevated. Special studies of the pancreas, including computerized axial tomography and selective angiography, did not demonstrate a tumor. Surgery, however, revealed a 5-cm tumor in the head of the pancreas. Although immunocytochemical stains showed only rare, weakly reactive cells, the electron microscopic study confirmed the diagnosis of an islet cell tumor and revealed characteristic morphologic features of glucagonoma.
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PMID:Glucagonoma syndrome. A clinicopathologic, immunocytochemical, and ultrastructural study. 298 44

A 63-year-old white woman with perioral dermatitis, a sore tongue, and an erythematous dermatosis in the inframammary and perineal regions underwent surgical removal of a pancreatic glucagonoma. The patient's plasma and pooled normal human plasma containing Sigma glucagon were fed to human keratinocyte cultures and increased arachidonic acid levels by 300% and 200%, respectively, when compared to pooled normal human plasma with no added commercial glucagon. These experiments suggest that glucagon may increase inflammatory mediators such as arachidonic acid and its metabolites in the epidermis, causing the skin lesions seen in the glucagonoma syndrome.
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PMID:Glucagonoma syndrome: in vitro evidence that glucagon increases epidermal arachidonic acid. 609 May 15

We report on a 76-year-old female patient with clinical findings consistent with necrolytic migratory erythema. Plasma glucagon levels were slightly elevated, but advanced radiologic techniques revealed no evidence of an underlying pancreatic tumour. The dermatosis responded excellently to oral therapy with dapsone. The possible aetiology and pathogenesis of the "pseudoglucagonoma syndrome" are discussed.
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PMID:[Pseudoglucagonoma syndrome. Case report, therapeutic possibilities and hypotheses on the etiology]. 775 43

Necrolytic migratory erythema is characterized by waves of irregular erythema in which a central bulla develops, and subsequently erodes and becomes crusted. It usually occurs in patients with an alpha-islet cell tumor of the pancreas. However, necrolytic migratory erythema has also been observed in patients without an associated glucagonoma. We describe a woman with iatrogenic necrolytic migratory erythema. She received intravenous glucagon for hypoglycemia associated with an insulin-like growth factor II-secreting hemangiopericytoma. After chemotherapy, she developed necrolytic migratory erythema. The characteristics of the previously reported patients with nonglucagonoma-associated necrolytic migratory erythema are reviewed. In patients with nonglucagonoma-associated necrolytic migratory erythema, the dermatosis-related conditions most commonly observed were celiac disease or malabsorption, cirrhosis, malignancy, and pancreatitis; less common conditions included hepatitis, inflammatory bowel disease, heroin abuse, and odontogenic abscess. Although the pathogenesis of necrolytic migratory erythema remains unknown, hyperglucagonemia appears to have had a causative role in the development of this dermatosis in our patient. Patients who develop necrolytic migratory erythema should be evaluated for the presence of a glucagonoma; if a glucagonoma is ruled out, evaluation for other conditions known to occur with necrolytic migratory erythema, such as liver disease, malabsorptive disorders, and nonislet-cell tumors is warranted.
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PMID:Iatrogenic necrolytic migratory erythema: a case report and review of nonglucagonoma-associated necrolytic migratory erythema. 959 6

The glucagonoma syndrome is a rare disease in which a typical skin disorder, necrolytic migratory erythema, is often one of the first presenting symptoms. Weight loss and diabetes mellitus are two other prevalent characteristics of this syndrome. Necrolytic migratory erythema belongs to the recently recognized family of deficiency dermatoses of which zinc deficiency, necrolytic acral erythema and pellagra are also members. It is typically characterized on skin biopsies by necrolysis of the upper epidermis with vacuolated keratinocytes. In persistent hyperglucagonemia, excessive stimulation of basic metabolic pathways results in diabetes mellitus at the expense of tissue glycogen stores, and muscle and fat mass. Multiple (essential) nutrient and vitamin B deficiencies develop, which contribute to the dermatosis. In addition, glucagonomas may produce various other products, like pancreatic polypeptide, that add to the catabolic effects of glucagon.
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PMID:The glucagonoma syndrome and necrolytic migratory erythema: a clinical review. 1553 29

Necrolytic migratory erythema (NME) is an uncommon inflammatory dermatosis with a distinctive clinical and histological appearance. It shows irregular erythema, bullae, erosion, crusts and pigmentation. While it is typically associated with glucagonoma, some cases of NME without glucagonoma have been reported. Herein, we report a case of necrolytic migratory erythema associated with malabsorption 30 years after ileocolectomy. She presented erosive erythema with scale or partly flaccid bullae on her intergluteal cleft, buttock and extremities. Her laboratory data revealed essential amino acid deficiency and a slightly decreased serum zinc level, while her plasma glucagon level was low. With diagnosis of non-glucagonoma-associated NME with malabsorption due to short-bowel syndrome, she was treated and improved by i.v. amino acid supplement. Histological findings of NME include necrotic changes of keratinocytes in the upper epidermis, proliferation of those in the lower epidermis and inflammatory cell infiltration of upper dermis. We also examined the expression pattern of epidermal keratins (K6, K10) and Ki-67, one of the markers of proliferative activity, to assess the proliferation and differentiation of keratinocytes in a NME lesion by immunostaining. The findings with these immunostainings support the characteristics of HE-staining, and suggest hyponutrition may induce changing differentiation/proliferation of keratinocytes.
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PMID:Necrolytic migratory erythema without glucagonoma in a patient with short bowel syndrome. 1692 38


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