1/54. hyperinsulinism: molecular aetiology of focal disease.Persistent hypoglycaemia in infancy is most commonly caused by hyperinsulinism. A case is reported of the somatic loss of the maternal 11p in an insulin secreting focal adenoma in association with a germline SUR-1 mutation on the paternal allele in a baby boy with hyperinsulinism diagnosed at 49 days old. A reduction to homozygosity of an SUR-1 mutation is proposed as a critical part of the cause of focal hyperinsulinism.- - - - - - - - - - ranking = 1keywords = hyperinsulinism, hypoglycaemia, infancy (Clic here for more details about this article) |
2/54. Mixed duct-acinar-islet cell tumor of the pancreas: report of a case.A 72-year-old Japanese woman presented at our hospital complaining of altered consciousness on arising every morning. The laboratory findings showed hypoglycemia and hyperinsulinemia. Abdominal ultrasonography revealed a tumor in the body of the pancreas. With a diagnosis of insulinoma, a surgical excision of the tumor was performed. A light microscopic examination and an immunohistochemical study revealed the tumor to consist of duct, acinar, and islet cell components. Mixed tumors of the pancreas are rare, and their clinical features and pathogenesis remain unclear. A further accumulation of clinical cases as well as a large number of histopathological studies on these rare mixed tumors is needed.- - - - - - - - - - ranking = 0.11950101627589keywords = hyperinsulinemia (Clic here for more details about this article) |
3/54. Uncontrolled insulin secretion from a childhood pancreatic beta-cell adenoma is not due to the functional loss of ATP-sensitive potassium channels.We report the case of an 8-year-old child who presented with severe hyperinsulinaemic hypoglycaemia due to a pancreatic islet cell adenoma. In vivo, there was no beneficial response to the hyperglycaemia-inducing agent diazoxide and as a consequence the child underwent a subtotal pancreatectomy. in vitro studies of adenomatous beta-cells revealed no operational defects in ATP-sensitive potassium channel activity and appropriate responses to diazoxide. In comparison with patients with focal adenomatous hyperplasia, genetic analysis of the isolated adenoma showed no loss of heterozygosity for chromosome 11p15 and expression of the cyclin-dependent kinase inhibitor p57(kip2). This case illustrates that the excess insulin secretion from an infantile adenoma has an aetiology different from that observed in hyperinsulinism in infancy.- - - - - - - - - - ranking = 0.37847121538095keywords = hyperinsulinism, hypoglycaemia, infancy (Clic here for more details about this article) |
4/54. Familial hyperinsulinism presenting in adults.Two adult siblings presented with recurrent syncope due to severe hyperinsulinemic hypoglycemia. Exploratory laparotomy in the elder sibling showed a grossly normal pancreas, but histologic examination revealed islet cell hyperplasia. Neither sibling has any evidence of the multiple endocrine neoplasia type 1 syndrome, nor is there any other family history to suggest this diagnosis. To our knowledge, this is the first report of adult-onset familial hyperinsulinism without other manifestations of multiple endocrine neoplasia type 1 syndrome. A simple provocative test for hyperinsulinism was also suggested by these cases. Because the initial patient related his symptoms to exercise, we used treadmill exercise in both patients to diagnose hyperinsulinism and observe its response to therapy.- - - - - - - - - - ranking = 2.1753507461667keywords = hyperinsulinism (Clic here for more details about this article) |
5/54. Malignant islet cell tumor of the pancreas with multiple hormone production and expression of CEA and CA19-9. Report of an autopsy case.An autopsy case of malignant islet cell tumor of the pancreas is presented. The patient, a 64-year-old woman showed severe hypoglycemia as the initial symptom, and hyperinsulinemia was demonstrated by laboratory examinations. Metastatic tumors in the liver were found by abdominal computed tomography. autopsy revealed a tumor measuring 6.5 x 3 x 2 cm occupying the pancreas from the body to the tail. From the results of histological and immunohistochemical studies, this was diagnosed as a malignant islet cell tumor producing multiple hormones such as insulin, glucagon, somatostatin and pancreatic polypeptide, as well as expressing the tumor-related antigens CEA and CA19-9. These findings suggested that the tumor cells showed differentiation to both endocrine cells and pancreatic duct cells.- - - - - - - - - - ranking = 0.11950101627589keywords = hyperinsulinemia (Clic here for more details about this article) |
6/54. octreotide scintigraphy localizes somatostatin receptor-positive islet cell carcinomas.Tyr-3-octreotide is a synthetic derivative of somatostatin and a somatostatin-receptor analogue. The iodine-123-labelled compound localizes somatostatin-receptor-positive tumours. In this paper two patients are reported in whom somatostatin receptors were demonstrated in vitro. In a 60-year-old female with an islet cell carcinoma of the pancreas, multiple liver metastases and previously unrecognized bone metastases in the right acetabulum could be diagnosed as the reason for a persistent hypoglycaemia. In a 60-year-old male an islet cell carcinoma of the pancreas was localized with 123I-Tyr-3-octreotide. The somatostatin receptors were demonstrated in vitro and the tumour was successfully treated with somatostatin. These studies demonstrate that 123I-Tyr-3-octreotide offers the possibility of localizing somatostatin-receptor-positive tumours and their metastases. Moreover the method makes it possible to determine the receptor status of a tumour in vivo.- - - - - - - - - - ranking = 0.067689154147435keywords = hypoglycaemia (Clic here for more details about this article) |
7/54. Immunocytochemical staining for human chorionic gonadotropin subunits does not predict malignancy in insulinomas.Insulinomas, the most common pancreatic endocrine tumors, produce a clinical syndrome of hyperinsulinism and hypoglycemia. Although the majority of insulinomas are benign, a significant proportion (4-16%) behave aggressively. Because malignant potential cannot be assessed adequately by histopathologic criteria, reliable serum and immunocytochemical markers for malignancy have been sought. Recent reports suggest that subunits of human chorionic gonadotropin (hCG) are of prognostic value in pancreatic endocrine tumors. Elevated serum levels of either the alpha-subunit or beta-subunit of hCG have been reported to be associated with malignancy in pancreatic endocrine tumors. Both hCG and its alpha-subunit have been demonstrated in malignant pancreatic endocrine tumors by immunohistochemistry. In this study, 17 insulinomas have been analyzed by immunohistochemistry using monospecific antibodies to both the alpha-subunit and beta-subunit of hCG. Clinical follow-up was obtained in all cases, and four of the tumors proved to be malignant. Alpha-subunit immunoreactivity was found in only two tumors, both of which were benign. Immunoreactivity for the beta-subunit was not found in any tumor. The authors' results indicate that for this subset of pancreatic endocrine tumors, staining for hCG subunits is of no value in predicting malignant behavior.- - - - - - - - - - ranking = 0.31076439230952keywords = hyperinsulinism (Clic here for more details about this article) |
8/54. Prolonged control of hypoglycaemia by L-asparaginase in islet cell carcinoma producing insulin and gastrin.L-asparaginase (140,000 units) infused into the hepatic artery resulted in a remission from disabling hypoglycaemia for nine months in a man with islet cell carcinoma of the pancreas and hepatic metastases. The tumour produced insulin and gastrin with resulting hypoglycaemia and recurrent peptic ulceration which were unresponsive to other drugs. Following L-asparaginase there was a fall in both plasma and insulin and gastrin.- - - - - - - - - - ranking = 0.40613492488461keywords = hypoglycaemia (Clic here for more details about this article) |
9/54. Giant insulinoma.A patient with an extremely large insulinoma but short duration of symptoms is reported on. fasting hypoglycemia, hyperinsulinism, and angiography were the modalities used to diagnose and locate the tumor.- - - - - - - - - - ranking = 0.31076439230952keywords = hyperinsulinism (Clic here for more details about this article) |
10/54. Muscle wasting after prolonged hypoglycaemic coma: case report with electrophysiological data.A case of distal muscle wasting associated with hypoglycaemia is described. Motor and sensory conduction studies are reported that provide little evidence of a hypoglycaemic peripheral neuropathy. Damage to anterior horn cells or motor roots appears to be a more likely explanation for the amyotrophy. Recovery occurred clinically with evidence of reinnervation by axonal collateral sprouting.- - - - - - - - - - ranking = 0.067689154147435keywords = hypoglycaemia (Clic here for more details about this article) |
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