1/130. 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) |
2/130. Giant insulinoma in a patient with multiple endocrine neoplasia-type I: a case report.We report a case of giant cystic insulinoma constituting part of multiple endocrine neoplasia (MEN) type I. A 29-year-old Japanese man presented with a history of recurrent hypoglycemic attacks. Endocrine examination showed hyperinsulinemia discordant with hypoglycemia, and a giant cystic insulinoma (11 x 10 cm) located in the pancreatic tail was detected radiologically. hyperprolactinemia due to pituitary adenoma and hyperparathyroidism due to parathyroid hyperplasia were also present. The insulinoma, prolactinoma and hyperplastic parathyroid gland were surgically removed. Fluorescent microsatellite analysis detected loss of heterozygosity (LOH) in chromosome 11q13 in dna samples from all resected tissues but not from white blood cells. This is a rare case of MEN type I because of the giant cystic insulinoma and the evidence of common LOH detected in all MEN type I tissues.- - - - - - - - - - ranking = 0.18172036436286keywords = hyperinsulinemia (Clic here for more details about this article) |
3/130. Clinical presentation of PCOS following development of an insulinoma: case report.A 24 year old woman presented with a prolonged clinical history of fasting and exertional hypoglycaemia, and was subsequently diagnosed with an insulinoma. Concurrent symptoms of oligomenorrhoea and hyperandrogenism of similar duration were noted. Biochemically, hyperinsulinaemia was observed in association with a raised serum luteinizing hormone (LH), raised testosterone and androstendione concentrations. Surgical removal of the insulinoma resulted in resolution of the clinical and biochemical features of the polycystic ovarian syndrome (PCOS) but minimal change was observed in the ovarian ultrasound appearances. This case demonstrates the role of insulin in mediating the hypersecretion of both LH and androgens in women with polycystic ovaries. We suggest that hyperinsulinaemia converted occult 'polycystic ovaries' to become clinically manifest as 'polycystic ovary syndrome'. This paradigm has clear implications for women with insulin dependent diabetes mellitus who presumably have systemic hyperinsulinaemia.- - - - - - - - - - ranking = 0.080753506435888keywords = hypoglycaemia (Clic here for more details about this article) |
4/130. adult hyperinsulinemic hypoglycemia not caused by an insulinoma: a report of two cases.nesidioblastosis is rare in adults and accounts for 0.5-5% of cases of organic hyperinsulinemia. The diagnosis of nesidioblastosis should be considered when peroperative imaging modalities fail to localize a lesion in patients with hyperinsulinism. Two female patients, aged 55 and 16 years, with hyperinsulinemic hypoglycemia are reported. somatostatin receptor scintigraphy showed slight focal activity in both patients. The first patient underwent a Whipple procedure and became diabetic. The second patient underwent a distal hemi-pancreatectomy and suffered from recurrent hypoglycemic episodes 3 months after surgery, for which she is presently being treated with octreotide. Histological examination of the resected pancreata revealed focally increased islet tissue and a number of slightly hypertrophic beta cells. Such histological abnormalities have been related to functional changes of beta-cells. In infantile nesidioblastosis, a proportion of cases has been associated with mutations in one of several genes. Whether such mutations, leading to hyperinsulinism, also play a role in adult nesidioblastosis is presently unknown.- - - - - - - - - - ranking = 0.7945463317345keywords = hyperinsulinism, hyperinsulinemia (Clic here for more details about this article) |
5/130. Combining the selective arterial calcium injection test and intraoperative blood glucose monitoring for multiple insulinomas: report of two cases.To date, no precise methods of preoperatively localizing multiple insulinomas or intraoperatively confirming their complete removal have been established. We describe herein the effectiveness of combining the selective arterial calcium injection (SACI) test to locate tumors preoperatively, with intraoperative continuous blood glucose monitoring (IOBGM) to confirm their complete removal. During the 8-year period from 1990 to 1997, we experienced two patients with multiple insulinomas. The first patient required resection of a residual tumor in the remnant pancreas for persistent hyperinsulinemia after a distal pancreatectomy performed under conventional diagnostic procedures. In the second patient, a SACI test was performed in addition to other diagnostic imaging procedures, and while computed tomography scan, endoscopic ultrasonography, and arteriography demonstrated multiple tumors in the body and tail of pancreas, the SACI test suggested that there were also tumors in the head of pancreas. Intraoperative ultrasonography showed a tumor measuring 5 mm in diameter on the surface of the head of the pancreas, apart from the multiple tumors in the body and tail of the pancreas. Complete removal of all tumors was assessed by the hyperglycemic rebound after resection of the tumors, determined by IOBGM. Based upon our experience, we believe that the SACI test and IOBGM are helpful for establishing precise localization and achieving complete removal of multiple insulinomas.- - - - - - - - - - ranking = 0.18172036436286keywords = hyperinsulinemia (Clic here for more details about this article) |
6/130. The effectiveness of administering a minimal dose of octreotide long-term prior to surgery for insulinoma: report of a case.We report herein the case of an 80-year-old woman with insulinoma who was regarded as an unsuitable candidate for immediate surgery due to her advanced age and obesity, for whom octreotide, a long-acting analogue of somatostatin, was used to improve her hypoglycemia and hyperinsulinemia without hyperalimentation. Administering a minimal dose of octreotide for a long period resulted in the improvement of leg edema, weight control, and cardiopulmonary function, and resection of the pancreatic tumor was safely carried out without any complications.- - - - - - - - - - ranking = 0.18172036436286keywords = hyperinsulinemia (Clic here for more details about this article) |
7/130. Incidental adult nesidioblastosis after distal pancreatectomy for endocrine microadenoma.Persistent hyperinsulinaemic hypoglycaemia (nesidioblastosis) presenting with hypoglycaemia is extremely rare in adults. The features are suggestive of an insulinoma with a vague presentation and delayed diagnosis. We describe a report of adult nesidioblastosis in association with a pancreatic endocrine microadenoma.- - - - - - - - - - ranking = 0.16150701287178keywords = hypoglycaemia (Clic here for more details about this article) |
8/130. insulinoma in childhood.A 9-year-old boy with convulsions is herein described. He was diagnosed and treated for epilepsy and insufficient adrenal function for four years with no response. hypoglycemia from hyperinsulinism was found and the source of the hyperinsulinism was a tumor of the tail of the pancreas--located by computerized tomographic scan and magnetic resonance imaging. Distal pancreatectomy was performed with good results. histology of the tumor showed islet cell tumors with capsular invasion. For this type of patient, long-term follow-up should include: prevention of metastasis or recurrence, and testing for multiple endocrine neoplasia type 1.- - - - - - - - - - ranking = 0.61282596737164keywords = hyperinsulinism (Clic here for more details about this article) |
9/130. 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 = 0.18172036436286keywords = hyperinsulinemia (Clic here for more details about this article) |
10/130. Lessons to be learned: a case study approach insulinoma presenting as a change in personality.A 43-year-old man presented with attacks of altered behaviour over a short period of time; they were associated with episodes of hypoglycaemia. The clinical suspicion of insulinoma prompted investigations that quickly established serum insulin and c-peptide levels to be elevated at the times when blood glucose values were low. A physical lesion was found in the head of the pancreas by means of computerised tomography and endo-duodenal ultrasound scan; an octreotide scan was negative. The patient underwent laparotomy and enucleation of a benign tumour, measuring 2.6 cm in diameter, lying within the head of the pancreas; histological examination confirmed it to be an insulinoma. Postoperatively, the patient's personality gradually became more normal and his fasting blood glucose concentrations returned to within normal limits. The diagnosis and management of insulinoma are discussed in the context of this clinical case; there is also reference to the protean clinical manifestations that may occur in this condition- and its differential diagnosis.- - - - - - - - - - ranking = 0.080753506435888keywords = hypoglycaemia (Clic here for more details about this article) |
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