1/24. 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 = hypoglycaemia (Clic here for more details about this article) |
2/24. 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 = 1keywords = hypoglycaemia (Clic here for more details about this article) |
3/24. 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 = 1keywords = hypoglycaemia (Clic here for more details about this article) |
4/24. 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 = 6keywords = hypoglycaemia (Clic here for more details about this article) |
5/24. 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 = 1keywords = hypoglycaemia (Clic here for more details about this article) |
6/24. Idiopathic hypoglycaemia in sibs with morphological evidence of nesidioblastosis of the pancreas.Two sibs who sustained severe hypoglycaemia in the neonatal period are reported. In spite of treatment with frequent feeds intravenous glucose, glucagon, hydrocortisone, and diazoxide, hypoglycaemia persisted, and both infants eventually required subtotal pancreatectomy. Tests for leucine toleranct were normal though the second case showed some protein sensitivity. Histological and immunohistochemical studies indicated nesidioblastosis in both specimens of pancreata. The children are presently performing at mildly retarded levels, and required diazoxide and anticonvulsant medication for some time postoperatively. Because both sexes are represetned, an autosomal recessive inheritance pattern is suggested. The theory of a gut hormone stimulating insulin production is briefly discussed.- - - - - - - - - - ranking = 6keywords = hypoglycaemia (Clic here for more details about this article) |
7/24. Clinical correlations of serum proinsulin-like material in islet cell tumours.To examine the possibility that the concentration of circulating proinsulin-like material (PLM) might be helpful in evaluating the therapeutic response of patients with islet cell tumours, serum levels of PLM in three patients with islet cell tumours were correlated with hypoglycaemic symptoms and plasma glucose concentrations before and after treatment. In two patients ranges of fasting PLM concentration were 0.21-0.29 and 0.91-0.93 ng/ml, respectively, before treatment. After surgical excision of their islet cell adenomas, PLM concentrations decreased to 0.06-0.09 and 0.03-0.05 ng/ml. Insulin concentrations were low preoperatively in both patients and were unchanged postoperatively. The resulting relief from hypoglycaemia was paralleled by a reduction of PLM, with no significant change in insulin. In a third patient, treatment with streptozotocin resulted in marked symptomatic improvement, a 65% reduction in PLM concentration, but no significant change in insulin levels. Relapse was associated with increasing frequency of hypoglycaemic symptoms and increasing PLM concentrations. These findings suggest that changes in the levels of serum PLM may prove to be a sensitive indicator of the response of islet cell tumours to therapy.- - - - - - - - - - ranking = 1keywords = hypoglycaemia (Clic here for more details about this article) |
8/24. pregnancy complicated by insulinoma. Case report.An insulinoma was excised during pregnancy. The tumor was located by radiography and severe hypoglycaemia made it necessary to administer diazoxide to the mother. The out come in the pregnancy was satisfactory. The rationale and risks of giving diazoxide in pregnancy are discussed.- - - - - - - - - - ranking = 1keywords = hypoglycaemia (Clic here for more details about this article) |
9/24. insulinoma: poor recognition of clinical features is the major problem in diagnosis.Traditionally it is taught that hypoglycaemia may cause a clinical picture which mimics a variety of neurological and psychiatric disorders. Yet patients with insulinoma continue to baffle many medical specialists, who presumably are not sufficiently aware of the clinical features of hypoglycaemia. After examining medical records of seventeen patients, diagnosed as suffering from "insulinoma" in major Melbourne hospitals from 1971 to 1976, it was evident that these patients frequently undergo extensive investigations for supposed neurological disorders, the correct diagnosis being missed until they develop catastrophic symptoms. Of these seventeen patients, the diagnosis was made with reasonable speed in only six cases, while eight patients were initially discharged from hospital with a completely erroneous diagnosis. It seems likely that a number of patients with insulinoma, whose symptoms are less dramatic than those reported here, are being mistakenly treated as having epileptiform or psychiatric disorders.- - - - - - - - - - ranking = 2keywords = hypoglycaemia (Clic here for more details about this article) |
10/24. insulinoma in a black South African. A case report.A 69-year-old black woman with an insulinoma presented with recurrent episodes of sweating and confusion culminating in two episodes of hypoglycaemic coma. The diagnosis was confirmed by finding an inappropriately elevated serum insulin level in the presence of hypoglycaemia after a fast of 14 hours. Computed tomography revealed a large tumour in the head of the pancreas. Removal of the tumour necessitated partial resection of the head and body of the pancreas, which in turn necessitated certain repair and drainage procedures. postoperative complications, while not insignificant, were acceptable. At 1-year follow-up the patient is well.- - - - - - - - - - ranking = 1keywords = hypoglycaemia (Clic here for more details about this article) |
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