No abstract available.
Hyperplasia* ; Islets of Langerhans* ; Nesidioblastosis*

Nesidioblastosis, also known as persistent hyperinsulinemic hypoglycemia of infancy(PHHI) or familial hyperinsulinsm, is the most common cause of recurrent severe hypoglycemia in infancy. It is an autosomal recessive disorder characterized by irregular insulin secretion leading to inappropriately raised plasma insulin concentration compared to blood glucose levels. Recently, mutations in the sulfonylurea receptor(SUR) have been described in association with PHHI. The mainstay of medical treatment is glucose infusion and diazoxide or long acting somatostatin. If medical treatment fails in preventing hypoglycemia, near total pancreatectomy is recommended. We report one case of nesidioblastosis cured by near total pancreatectomy with brief review of literatures.
Blood Glucose ; Congenital Hyperinsulinism* ; Diazoxide ; Glucose ; Humans ; Hypoglycemia ; Infant, Newborn* ; Insulin ; Nesidioblastosis* ; Pancreatectomy ; Plasma ; Somatostatin

Nesidioblastosis is a term that describes the prolonged differentiation of islets of Langerhan's cells from the epithelium of the pancreatic duct. Nesidioblastosis is a primarily childhood disease, which is often associated with hyperinsulinemic hypoglycemia, but rarely found in adults. A 62-year-old man with hyperinsulinemic hypoglycemia complained of general weakness and mental change while fasting. Abdominopelvic CT, celiac angiography, intra-arterial calcium stimulation test and endoscopic ultrasonography did not reveal any tumor in the pancreas. A subtotal pancreatectomy (nearly 80% of pancreas was removed) was then carried out. He was diagnosed with nesidioblastosis inform the pathology after the operation. Herein, our experience of a 62-year-old Nesidioblastosis patient is reported.
Adult* ; Angiography ; Calcium ; Endosonography ; Epithelium ; Fasting ; Humans ; Hypoglycemia* ; Middle Aged ; Nesidioblastosis* ; Pancreas ; Pancreatectomy ; Pancreatic Ducts ; Pathology

Nesidioblastosis is characterized by a diffuse proliferation of islet cells arising from pancreatic ducts and is the most common cause of hyperinsulinemic hypoglycemia in newborns and infantile. It is exceedingly rare in adults and no concensus regarding its diagnosis and management is available. We herein describe an elderly man with fasting hypoglycemia, inappropriate insulin hypersecretion. And pathologic examination of his pancreas revealed the characteristic finding of nesidioblastosis confirmed by immunohistochemical stain.
Adult ; Aged* ; Diagnosis ; Humans ; Hypoglycemia* ; Infant, Newborn ; Insulin ; Islets of Langerhans ; Nesidioblastosis* ; Pancreas ; Pancreatic Ducts

No abstract available.
Adenoma, Islet Cell* ; Adult* ; Diabetes Mellitus, Type 2* ; Humans ; Islets of Langerhans* ; Nesidioblastosis*

Focal nesidioblastosis is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults. Because it is difficult to localize and detect with current imaging modalities, nesidioblastosis is challenging for biliary-pancreatic surgeons. ⁶⁸Gallium-DOTA-D-Phe¹-Tyr³-octreotide PET scanning and ¹¹¹indium-pentetreotide diethylene triamine pentaacetic acid octreotide scanning may be superior to conventional imaging modalities in determining the localization of nesidioblastosis. We report the successful surgical treatment of a 54-year-old woman with focal hyperplasia of the islets of Langerhans, who experienced frequent hypoglycemic symptoms and underwent various diagnostic examinations with different results.
Adult ; Diagnosis* ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Islets of Langerhans ; Middle Aged ; Nesidioblastosis* ; Octreotide ; Positron-Emission Tomography* ; Surgeons

Focal nesidioblastosis is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults. Because it is difficult to localize and detect with current imaging modalities, nesidioblastosis is challenging for biliary-pancreatic surgeons. ⁶⁸Gallium-DOTA-D-Phe¹-Tyr³-octreotide PET scanning and ¹¹¹indium-pentetreotide diethylene triamine pentaacetic acid octreotide scanning may be superior to conventional imaging modalities in determining the localization of nesidioblastosis. We report the successful surgical treatment of a 54-year-old woman with focal hyperplasia of the islets of Langerhans, who experienced frequent hypoglycemic symptoms and underwent various diagnostic examinations with different results.
Adult ; Diagnosis* ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Islets of Langerhans ; Middle Aged ; Nesidioblastosis* ; Octreotide ; Positron-Emission Tomography* ; Surgeons

Nesidioblastosis is a rare disease characterized clinically by persistent hypoglycemia with inappropriately elevated circulating insulin concentration. Adequate early diagnosis should be established and subtotal pancreatectomy performed before itreversible cerebral damage caused by glucose deficit. The authors got a chance to anesthetize 56-day-old male patient for subtotal pancreatectomy because of nesidioblastosis, Following an induction of anesthesia with intravenous thiapental sodium 5 mg/kg and succinylchohne 1 mg/kg, endotracheal intubation was performed and anesthesia was maintained with pancuronium bromide and O2-N2O-enflurane. Intravenous fluid was maintained with 1-2-3 solution 30 ml and 15% D/W 40 ml mixed with 20 ml of 20 mEq/L NaCI.
Anesthesia* ; Early Diagnosis ; Glucose ; Humans ; Hypoglycemia ; Insulin ; Intubation, Intratracheal ; Male ; Nesidioblastosis* ; Pancreatectomy* ; Pancuronium ; Rare Diseases ; Sodium

Nesidioblastosis is a rare disorder, and it usually considered as a cause of neonatal hyperinsulinemic hypoglycemia. A 35 year-old-woman with hyperinsulinemic hypoglycemia was admitted in an unconscious condition. Abdominal CT, pancreas MRI and celiac angiography with an intra-arterial calcium stimulation test revealed a suspicious insulin-producing tumorous lesion in the head of pancreas. The patient underwent enucleation of the pancreas head tumor under the initial diagnosis of insulinoma. However, the tumor was confirmed histologically as nesidioblastosis that showed ductoendocrine proliferations and numerous small endocrine cell groups. Nesidioblastosis is classified into a focal type and a diffuse type, which are characterized by different clinical outcomes. The patient in our case showed a normal blood glucose level after operation, which is often the case for the focal type. Herein, we report this very rare case of adult nesiodioblastosis that was successfully treated by surgical resection.
Adult ; Angiography ; Blood Glucose ; Calcium ; Endocrine Cells ; Head ; Humans ; Hyperinsulinism ; Hypoglycemia ; Insulinoma ; Nesidioblastosis ; Pancreas ; Unconscious (Psychology)

Hyperinsulinemic hypoglycemia in the absence of exogenous insulin use is caused by disorders such as insulinoma, diffuse beta-cell hyperplasia/nesidioblastosis, and autoimmune hypoglycemia. Nesidioblastosis is a rare cause of hypoglycemia in adults, accounting for 0.5~7.0% of organic hyperinsulinemia cases. Although pancreatic resection is considered the best treatment modality for curing nesidioblastosis, there is no consensus regarding the indications for and extent of the surgery due to its high risk and complication rate. A 75-year-old woman presented with an altered mental state, a mass suspected of being an insulinoma, and insulin receptor antibodies. The patient underwent surgery because of recurrent life-threatening hypoglycemia. Postoperative pathology of her pancreas revealed nesidioblastosis.
Accounting ; Adult ; Aged ; Antibodies ; Consensus ; Female ; Humans ; Hyperinsulinism ; Hypoglycemia ; Insulin ; Insulinoma ; Nesidioblastosis ; Pancreas ; Receptor, Insulin