1/42. nesidioblastosis, myelodysplastic syndrome and nodular diabetic glomerulosclerosis in an elderly nondiabetic woman: an autopsy report.nesidioblastosis as the cause of hyperinsulinaemic hypoglycaemia in an adult is rare. We report here an additional case of nesidioblastosis, which resulted in fatal hyperinsulinaemic hypoglycaemia in a 72-year-old woman with an underlying myelodysplastic syndrome. The diagnosis of nesidioblastosis was established only after post-mortem examination with a careful exclusion of minute insulinoma. To our surprise, the renal pathology disclosed typical diabetic nodular glomerulosclerosis in the same patient who had no previous history of diabetes mellitus (DM). nesidioblastosis has been reported to cause 'reversal' of Type 1 DM and insulinoma causing 'reversal' of Type 2 disease. We therefore hypothesize that our patient might have had an undiagnosed DM in the past, which resulted in the typical diabetic nodular glomerulosclerosis. The nesidioblastosis caused a 'reversal' of DM and even the ultimate development of hyperinsulinaemic hypoglycaemia.- - - - - - - - - - ranking = 1keywords = diabetes (Clic here for more details about this article) |
2/42. 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 = 1keywords = diabetes (Clic here for more details about this article) |
3/42. Deliberate sulphonylurea poisoning mimicking hyperinsulinaemia of infancy.A 6 month old child presenting with seizures was found to be hypoglycaemic secondary to hyperinsulinism. A family history of type II diabetes prompted estimation of sulphonylurea in the baby's blood, which was found to be high. A multidisciplinary case conference concluded that the sulphonylurea ingestion was likely to be the result of munchausen syndrome by proxy. When investigating hypoglycaemia of infancy this possibility should be considered.- - - - - - - - - - ranking = 1keywords = diabetes (Clic here for more details about this article) |
4/42. Hypoglycaemia from islet cell hyperplasia and nesidioblastosis in a patient with type 2 diabetes mellitus--a case report.INTRODUCTION: We report the first case of hypoglycaemia from beta cell hyperplasia with nesidioblastosis in an Asian adult with pre-existing type 2 diabetes. CLINICAL PICTURE: A 57-year-old Chinese woman presented with hyperinsulinaemic hypoglycaemia despite discontinuation of oral hypoglycaemic agents 4 months after diagnosis of type 2 diabetes. Preoperative portal venous sampling suggested regionalisation to the neck of the pancreas. Intraoperative ultrasound and palpation of the fully mobilised pancreas were non-localising. TREATMENT: A subtotal 85% pancreatectomy was performed with success. OUTCOME: histology showed no evidence of tumour, but revealed islet hyperplasia and nesidioblastosis. Her diabetes was subsequently well controlled on metformin therapy. CONCLUSION: Endogenous hyperinsulinism from beta cell hyperplasia with nesidioblastosis may rarely occur in type 2 diabetics. However, this remains a diagnosis of exclusion that is confirmed only on surgical pathology. In affected individuals, preoperative portal venous sampling may be falsely localising, especially if selective sampling of the smaller peri-pancreatic veins is omitted. Definite treatment involves pancreatectomy, although the extent of surgical resection is not well established.- - - - - - - - - - ranking = 7keywords = diabetes (Clic here for more details about this article) |
5/42. adult-onset nesidioblastosis causing hypoglycemia: an important clinical entity and continuing treatment dilemma.HYPOTHESIS: nesidioblastosis is an important cause of adult hyperinsulinemic hypoglycemia, and control of this disorder can often be obtained with a 70% distal pancreatectomy. DESIGN: The records of all adult patients operated on for hypoglycemia between 1974 and 1999 were reviewed retrospectively. patients with the pathologic diagnosis of nesidioblastosis were contacted for follow-up (1.5-21 years) and are presented. patients' results were compared with those of 36 other individuals with this disorder who were previously reported in the literature. SETTING: The University of chicago Medical Center (chicago, Ill), a tertiary care facility. patients: A consecutive sample of all patients operated on for hypoglycemia. INTERVENTIONS: Seventy percent distal pancreatectomy for all patients with nesidioblastosis, and maintenance therapy with verapamil hydrochloride for 2 patients. MAIN OUTCOME MEASURES: achievement of normoglycemia with and without medication, development of insulin-dependent diabetes mellitus, pancreatic exocrine insufficiency, and need for reoperation. RESULTS: Of 32 adult patients who underwent surgical exploration for hyperinsulinemic hypoglycemia at our institution, 27 (84%) were found to have 1 or more insulinomas, and 5 (16%) were diagnosed with nesidioblastosis. Each patient with nesidioblastosis underwent a 70% distal pancreatectomy. Follow-up duration for the 5 patients ranged from 1.5 to 21 years, with 3 patients (60%) asymptomatic and taking no medications, and 2 patients (40%) experiencing some recurrences of hypoglycemia. The 2 patients with recurrences are now successfully treated with a calcium channel blocker, an approach, to our knowledge, never before reported for adult-onset nesidioblastosis. CONCLUSIONS: nesidioblastosis is an uncommon but clinically important cause of hypoglycemia in the adult population, and must always be considered in a patient with a presumptive preoperative diagnosis of insulinoma. This study indicates that a 70% distal pancreatectomy is often successful in controlling hypoglycemia, and rarely results in diabetes mellitus. However, the optimal treatment of this disorder remains to be determined.- - - - - - - - - - ranking = 2keywords = diabetes (Clic here for more details about this article) |
6/42. insulinoma induced hypoglycemia in a type 2 diabetic patient.insulinoma in a patient with pre-existing diabetes mellitus is extremely rare. We report a case of an insulinoma in a patient with type 2 diabetes mellitus who after 14 years of sulfonylurea treatment experienced recurrent episodes of hypoglycemia. Endogenous hyperinsulinism was confirmed and endoscopic ultrasonography identified a pancreatic tumor, which was positive for insulin by immuno-histological staining. After surgical excision of the tumor, no further hypoglycemic attacks occurred. Loss of body weight after removal of the tumor correlated with a dramatic reduction of insulin resistance to such a degree that diet alone proved sufficient for satisfactory glycemic control.- - - - - - - - - - ranking = 2keywords = diabetes (Clic here for more details about this article) |
7/42. Two hyperandrogenic adolescent girls with congenital portosystemic shunt.We describe two adolescent girls with a congenital portosystemic shunt who exhibited hyperandrogenism in addition to insulin resistant hyperinsulinaemia. Case 1 was referred to our clinic to undergo a routine clinical work-up prior to tonsillectomy at 14 years of age. Mild liver dysfunction was identified and hypogenesis of the portal vein with a congenital portosystemic shunt diagnosed. Primary amenorrhoea and virilization were evident and an endocrinological evaluation revealed hyperandrogenism and insulin resistant hyperinsulinaemia. Case 2 was referred at 15 years of age because of cardiomegaly. Mild liver dysfunction and hyperbilirubinaemia led to a diagnosis of agenesis of the portal vein with a congenital portosystemic shunt. Virilization was evident and an endocrinological evaluation revealed hyperandrogenism and insulin resistant hyperinsulinaemia. The haemodynamics of these patients were similar to those of secondary portosystemic shunt due to liver cirrhosis, which is often associated with hyperinsulinaemia and/or non-insulin dependent diabetes mellitus. On the other hand, hyperandrogenism is associated with certain insulin-resistant conditions with hyperinsulinaemia, including the polycystic ovary syndrome (PCO). Hyperinsulinaemia is believed to cause hyperandrogenism in patients with PCO by stimulating androgen production in both the ovary and adrenal gland. Therefore, in congenital portosystemic shunts, hyperinsulinaemia is also thought to cause hyperandrogenism due to the same mechanism. CONCLUSION: A certain percentage of female patients with hyperandrogenism, likely including those with polycystic ovary syndrome may also have congenital portosystemic shunts. Our results indicate that serum levels of total bile acids and ammonia are prognostic indicators of this hepatic vascular anomaly.- - - - - - - - - - ranking = 1keywords = diabetes (Clic here for more details about this article) |
8/42. Enhanced intra-abdominal visceral fat accumulation in patients with Werner's syndrome.OBJECTIVE: Studies were made on the abnormality of glucose and lipid metabolism and its cause in four patients with Werner's syndrome to infer the reason for accelerated atherogenesis in this syndrome. RESULTS: Of these four patients, hypercholesterolemia was found in three, hypertriglyceridemia in four, hypoalphalipoproteinemia in two and hypertension in two. All the patients had insulin-resistant diabetes mellitus and three of them had apparent hyperinsulinemia. Abdominal computed tomography revealed that all of them had visceral fat obesity, namely augumented intra-abdominal adipose tissue. CONCLUSION: The clinical features of these patients resemble those recently designated as insulin resistant syndrome (syndrome X) or visceral fat syndrome. The metabolic abnormality may be one of important factors in the accelerated atherogenesis in this syndrome.- - - - - - - - - - ranking = 1keywords = diabetes (Clic here for more details about this article) |
9/42. Pancreatic beta-cell stimulation tests in transient and persistent congenital hyperinsulinism.In congenital hyperinsulinism (HI). the in vivo pancreatic beta-cell function is poorly described. Among 14 neonates with severe hyperinsulinaemic hypoglycaemia, 2 patients had very prolonged or persistent hypoglycaemia and mutation in the sulphonylurea receptor SURI gene. Patient 1 had transient HI and was treated medically for 3.5 mo before clinical remission was seen. He had initially very high basal and stimulated c-peptide and insulin levels, followed by a state of normal preprandial values, but blunted beta-cell glucose sensitivity, before complete beta-cell normalization occurred. A single. paternal SURI mutation, G1382S, was found suggesting focal type HI. Patient 2 had persistent HI and underwent 3 pancreas resections up to the age of 2 y, 7 mo, followed by a state of mild diabetes. On biopsy, diffuse-type beta-cell hypertrophy was seen. The beta-cell response to glucose and glucagon stimulation was blunted before, as well as after, pancreas resections. Compound heterozygosity for the SUR1 mutations 3992-3c to g and N188S was found. CONCLUSION: Transient, possibly focal, HI with paternal SUR1 mutation was associated with a gradual, but complete normalization of the in vivo beta-cell function; in the diffuse type HI, a blunted beta-cell response to glucose and glucagon stimulation persisted. In vivo beta-cell stimulation tests may contribute to the characterization of the HI subtypes.- - - - - - - - - - ranking = 1keywords = diabetes (Clic here for more details about this article) |
10/42. polycystic ovary syndrome: a case presentation.polycystic ovary syndrome (PCOS) is a disorder of excessive adrenal hormone production that results in characteristic features. It is associated with hyperinsulinemia and, if left untreated, carries a risk for developing such complications as diabetes and heart disease. The clinical features are described here by means of a case presentation. Using the Ferriman-Gallwey score, the degree of hirsutism is identified. Differential diagnoses and recommended treatments are discussed. The role of the advanced practice nurse in the diagnosis and care of patients with PCOS is included.- - - - - - - - - - ranking = 1keywords = diabetes (Clic here for more details about this article) |
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