1/20. 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 = hypoglycaemia (Clic here for more details about this article) |
2/20. 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 = 0.66666666666667keywords = hypoglycaemia (Clic here for more details about this article) |
3/20. 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 = 0.66666666666667keywords = hypoglycaemia (Clic here for more details about this article) |
4/20. Anaesthetic management of a case of nesidioblastosis for subtotal pancreatectomy.hyperinsulinism is a rare cause of severe persistent hypoglycaemia in the neonatal period. It is associated with a high incidence of brain damage and mental retardation as a consequence of repeated episodes of hypoglycaemia. Subtotal to near total pancreatectomy is indicated as a matter of urgency to decrease the amount of circulating insulin. The perioperative management of a 45-day-old, 5 kg male infant with hyperinsulinaemia (nesidioblastosis) is described. He had a history of generalized tonic clonic seizures 4 h after birth. The blood sugar at that time was 0.66 mmol x l(-1) (12 mg x dl(-1)) and serum calcium was 2.4 mmol x l(-1) (9.82 mg x dl(-1)). The insulin : glucose ratio was 1.6 (normal < 0.4). Occasional episodes of hypoglycaemia persisted in spite of medical line of management with intravenous dextrose 12%, 2 h gastric tube feeds, hydrocortisone (5 mg x kg(-1) x day(-1) i.v.) and oral diazoxide 10 mg x kg(-1), 8 h for 3 weeks. A CT scan and USG did not reveal any abnormality of the pancreas. However, the EEG varied from one of abnormally low amplitude to an isoelectric record. Renal, liver function tests and coagulation profile were normal. The patient was scheduled for elective subtotal pancreatectomy. The anaesthetic management with emphasis on glucose homeostasis and fluid balance is discussed.- - - - - - - - - - ranking = 1keywords = hypoglycaemia (Clic here for more details about this article) |
5/20. A case of familial nesidioblastosis: prenatal diagnosis of foetal hyperinsulinism.Persistent neonatal hyperinsulinaemic hypoglycaemia due to nesidioblastosis is a rare condition probably transmitted by an autosomal recessive inheritance. Recurrent hypoglycaemic episodes become evident after birth and cause severe neurological damage without intensive treatment. The intrauterine detection of hypoglycaemia and hyperinsulinism in newborns subsequently diagnosed as affected by nesidioblastosis has not yet been reported. We describe a case of familial nesidioblastosis in which an intrauterine diagnosis could be suggested by high levels of insulin and c-peptide and low values of glucose in the amniotic fluid.- - - - - - - - - - ranking = 0.66666666666667keywords = hypoglycaemia (Clic here for more details about this article) |
6/20. Tolerance to somatostatin analogue in a preterm infant with islet cell dysregulation syndrome.An infant of 31 weeks' gestation presented with refractory neonatal hypoglycaemia secondary to islet cell dysregulation. Treatment was started with somatostatin analogue and his glycaemic control improved initially. Tolerance developed, however, in that the dose required to maintain control increased by a factor of 40. The infant subsequently underwent pancreatectomy. It is safe to use somatostatin analogue in a preterm infant, but tolerance to the drug rapidly develops.- - - - - - - - - - ranking = 0.33333333333333keywords = hypoglycaemia (Clic here for more details about this article) |
7/20. Long-term use of somatostatin analogue SMS 201-995 in the treatment of hypoglycaemia due to nesidioblastosis.An infant presented with hypoglycaemia secondary to nesidioblastosis. Subtotal pancreatectomy failed to prevent recurrent post-operative hypoglycaemia. Subcutaneous somatostatin analogue (SMS 201-995) was shown to increase the blood concentrations of glucose and B-hydroxybutyrate while lowering serum insulin levels. Regular use of somatostatin analogue was helpful in long term management without causing significant side effects.- - - - - - - - - - ranking = 2keywords = hypoglycaemia (Clic here for more details about this article) |
8/20. Complications of diazoxide in the treatment of nesidioblastosis.Two children with hypoglycaemia secondary to hyperinsulinaemia were treated with diazoxide. They suffered serious side effects of cardiac failure and truncal ataxia.- - - - - - - - - - ranking = 0.33333333333333keywords = hypoglycaemia (Clic here for more details about this article) |
9/20. A case of multiple endocrine adenomatosis (Type 1) with nesidioblastosis, terminating with an exocrine pancreatic carcinoma.We describe a patient with multiple endocrine adenomatosis Type I, characterized by pituitary-dependent Cushing's syndrome, marked hyperprolactinaemia, primary hyperparathyroidism and hyperinsulinism leading to hypoglycaemia. The patient subsequently developed an exocrine pancreatic carcinoma at the age of 32 years from which she died. An additional finding was the demonstration by immunocytochemistry of nesidioblastosis in the pancreas.- - - - - - - - - - ranking = 0.33333333333333keywords = hypoglycaemia (Clic here for more details about this article) |
10/20. The surgical treatment of hyperinsulinism in infancy and childhood.Hyperinsulinaemic hypoglycaemia in infancy may be transient or persistent. Persistent hypoglycaemia is caused by nesidioblastosis of the pancreas, a diffuse pancreatic endocrine abnormality involving all the endocrine tissue, or by an islet cell adenoma. The cause of nesidioblastosis is not known but unless controlled its hypoglycaemic effects lead to convulsions, mental retardation or death. patients with transient hyperinsulinaemic hypoglycaemia, and some with persistent hypoglycaemia, respond to medical therapy but those with an adenoma and many of those with nesidioblastosis will require partial or total pancreatectomy. The surgical treatment of six infants and a child is discussed. Five infants had nesidioblastosis; one infant and one nine-year-old girl had an islet cell adenoma. It is suggested that in the very young when there is no evidence of an insulinoma, resection at the initial operation should be greater--up to 90 per cent--than the previously recommended 75 per cent.- - - - - - - - - - ranking = 1.3333333333333keywords = hypoglycaemia (Clic here for more details about this article) |
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