Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0011849 (diabetes)
 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pancreatic islet-cell tumors (ICTs) are rare malignancies usually recognized by specific clinical endocrinopathies. The purpose of this study is to evaluate our surgical experience with functioning pancreatic ICT in an academic referral center. Twenty patients (male:female 12:8) with a mean age of 53 years (range 26-82) underwent surgery for a functioning pancreatic ICT [gastrinoma (eight), multiple endocrine neoplasia (three), insulinoma (seven), glucagonoma (four), and VI-Poma (vasoactive intestinal peptide; one)] between June 1975 and March 2001. Signs and symptoms of hormonal excess were present in 95 per cent (19 of 20). One patient (glucagonoma) presented with obstructive jaundice and mild glucose intolerance. Elevated peptide levels were detected preoperatively in 65 per cent, including all patients with an insulinoma. Curative resections were attempted in 80 per cent including three procedures for insulinoma. Palliative procedures were performed in 20 per cent--all gastrinomas. One patient with an insulinoma had diffuse nesidioblastosis. Three patients (with gastrinoma, insulinoma, and glucagonoma) had lymph node-positive disease and three patients with gastrinoma had liver metastasis. The overall 30-day morbidity rate was 30 per cent and mortality rate 0 per cent. Symptomatic improvement was achieved in 90 per cent at a mean follow-up of 44 months. Two patients developed diabetes after a subtotal and a total pancreatectomy, respectively. Sixty-three per cent of patients who underwent an attempted curative resection are alive at a mean follow-up of 47 months (range 3-231) and all patients who underwent a palliative procedure are alive at a mean follow-up of 31 months (range 27-36). Functioning pancreatic ICTs are fascinating tumors that produce distinct clinical syndromes. Symptomatic improvement is accomplished in the majority of patients after surgery and short-term palliation is achieved in patients with nonresectable disease.
 ...
PMID:Surgical experience with functioning pancreatic neuroendocrine tumors. 1220 98

Glucagonomas are rare tumors originating in alpha-cells of the pancreas. The most common clinical presentation is the association of diabetes mellitus, necrolytic erythema, weight loss and anemia. The diagnosis of pancreatic tumor is usually made by abdominal computed tomography and/or endoscopic ultrasonography. Indium-labeled octreotide scanning is useful for the localization of most neuroendocrine tumors and their metastases. Glucagon release can be confirmed by a high concentration of plasma glucagon. We report the case of a 74-year-old patient who had a glucagonoma with particular presentation of neurological impairment and weight loss. The diagnosis was confirmed by usual imaging procedures and plasma glucagon level. Medical treatment was started with long-acting repeatable octreotide (Sandostatin(R) LAR). After a one-year follow-up, the patient remained well. The original presentation and benefit of a new, long-acting somatostatin analog for the treatment of inoperable glucagonoma are discussed.
 ...
PMID:[Clinical response of an atypical glucagonoma treated with a long-acting somatostatin analog]. 1243 3

Glucagonoma of the pancreas is a rare tumor with distinct clinical manifestations, such as necrolytic migratory erythema,weight loss, anemia, diabetes mellitus, and hypoamino-acidemia. We report the case of a 68-year-old Japanese man who underwent curative resection for malignant glucagonoma of the pancreas diagnosed through anemia and diabetes mellitus. The patient had had diabetes mellitus for 20 years. Anemia was diagnosed in 1998. On admission, the hemoglobin level was 8.3g/dl, but the levels of serum iron, vitamin B12, and erythropoietin and, the number of reticulocytes were within normal limits. The levels of carcinoembryonic antigen (CEA), carbohydrate antigen (CA)19-9, and DUPAN-2 were also within normal limits, and exocrine function of the pancreas (PFD, 75%) was normal. Ultrasonography (US) revealed a hypoechoic tumor in the distal pancreas. Computed tomography (CT) demonstrated a high-density area 4 cm in diameter with calcification. The serum glucagon level was very high (2360 pg/ml), but the levels of other hormones such as somatostatin or gastrin were within normal limits, while insulin was low. Glucagonoma of the pancreas was diagnosed, and distal pancreatectomy with splenectomy was performed. Histological examination revealed a malignant endocrine tumor,which was immunohistochemically positive for chromogranin A and glucagon. Two months after the operation, the serum glucagon level had decreased to within normal limits and the hemoglobin level had increased to 10.4 g/dl. The case of glucagonoma reported here was found through diagnostic examinations of anemia and treated by surgical resection, by which the patient's anemia was largely alleviated. Therefore, we recommend checking patients who have diabetes mellitus and anemia in order to diagnose and treat glucagonoma in its early stage.
 ...
PMID:Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus. 1291 65

Glucagonoma is a rare islet alpha-cell pancreatic tumor. Patients usually present with necrolytic migratory erythema, diabetes mellitus, thromboembolism, and weight loss. Diagnosis is based on the presence of a pancreatic tumor in association with hyperglucagonemia. Tumor characterization is made by computed tomography and/or pancreatic endoscopic ultrasonic and indium-labeled octreo-scan. Surgery is the main component of the treatment, in some cases in association with chemotherapy. We report the case of a 72-year-old patient who developed a recurrent glucagonoma, 20 years after surgical resection.
 ...
PMID:[Recurrent glucagonoma 20 years after surgical resection]. 1477 Jan 22

We report a case of glucagonoma syndrome, revealed by a necrolytic migratory erythema that had developed for four Years, associated with anorexia, severe weight loss, anemia, hypoprotidemia, and hypoaminoacidemia. The fasting blood glucose level tended paradoxically to be low (0.6 g/l). Elevated plasma glucagon levels confirmed our diagnosis. The absence of diabetes was explained by an independent insulin secretion derived from this composite pancreatic tumor, authenticated by the histological analysis and the proinsulin level. This level was similar to those typically observed in insulinomas. Six Months after a complete surgical exeresis, symptoms disappeared and biological results returned to normal values.
 ...
PMID:[Necrolytic migratory erythema revealing glucagonoma without diabetes]. 1503 34

Migratory necrotizing dermatitis is one of the most distressing presenting symptoms of glucagonomas. This rare functioning pancreatic endocrine tumor is third in incidence after insulinomas and gastrinomas and is often malignant at the time of diagnosis. Elevated serum glucagon levels cause decreased amino acid levels which is believed to be the principal cause of the dermatitis. Other symptoms include anemia, visual scotomata and mild diabetes mellitus. Medical treatment alone including octreotide and amino acid supplementation has been reported to eliminate the dermatitis. Nonetheless, surgical resection or debulking remains the definitive treatment when possible. Because of its rarity, diagnosis may be delayed by years accounting for the high rate of metastasis at presentation. Reported here is the case of a 77-year-old man who presented with a migratory necrotizing dermatitis after antibiotic treatment and whose diagnosis of a glucagonoma was then delayed for over 1 year.
 ...
PMID:Migratory necrolytic dermatitis presenting after cephalosporin administration in a patient with a pancreatic head mass. 1516 86

Glucagonoma is a rare neuroendocrine neoplasm. Characteristics are clinical (necrolytic migratory erythema, weight loss), biological (diabetes) and radiological (pancreatic tumor). The Authors report a case of glucagonoma that also secreted insulin.
 ...
PMID:[Necrolytic cutaneous erythema and pancreatic tumour: the glucagonoma]. 1519 27

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.
 ...
PMID:The glucagonoma syndrome and necrolytic migratory erythema: a clinical review. 1553 29

A 44-year-old woman was diagnosed with type II diabetes in 1998 and 1 year later she developed necrolytic migratory erythema, which is a specific skin lesion of glucagonoma. During the clinical investigation, a nodular 6 cm mass in the distal pancreatic region and multiple cystic liver metastases were found. She was operated on, and glucagonoma was detected and the long-acting, repeatable, octreotide treatment was started. 3 years after resection of a pancreatic glucagonoma she presented to a hospital emergency department with diabetic ketoacidosis. Hepatic multiple cystic metastases were visualized by computed tomography. During hospitalization she developed severe pulmonary embolism and deep-venous thrombosis of the lower extremities. Indium-labeled octeotide scintigraphy showed multiple cystic lesions in the liver with additional lesions in the iliocecal region, which had not been visualized by computed tomography. Despite somatostatin therapy the tumor had expanded in the liver. Arterial chemoembolization was performed but 6 months later she died.
 ...
PMID:Malign cystic glucagonoma presented with diabetic ketoacidosis: case report with an update. 1594 15

Glucagonoma is a very rare endocrine pancreatic tumor. At diagnosis, most glucagonomas are malignant and often metastatic. Suspicion of glucagonoma is based on characteristic presentations known as "glucagonoma syndrome". Glucagonoma is often found in the pancreatic body and/or tail and is usually large enough to be localized by computed tomography. We report a case of diffuse glucagonoma necrolytic migratory erythema (NME) in a 45-year-old man with mild diabetes mellitus, mild anemia, and weight loss over 1.5 years. Diffused enlarged pancreas was noted on abdominal ultrasonography incidentally during a routine health check-up. The levels of carcinoembryonic antigen and carbohydrate antigen 19-9 were within normal limits. No enlarged lymph node or extrapancreatic tumor mass was found by several imaging studies. Total pancreatectomy was performed, and the pathology revealed glucagon-producing islet cells and intrapancreatic vascular emboli of tumor cells. He died due to internal bleeding and sepsis after surgery. Presentation of diffuse malignant glucagonoma with tumor emboli but no metastasis or NME is unusual.
 ...
PMID:Rare presentation of endocrine pancreatic tumor: a case of diffuse glucagonoma without metastasis and necrolytic migratory erythema. 1595 5


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>