Diffuse nesidioblastosis causing hyperinsulinemic hypoglycemia: the importance of pancreatic sampling on EUS - 23/08/11
Doi : 10.1016/j.gie.2008.02.071
Guruprasad P. Aithal, PhD, FRCP
Department of Hepatology, Wolfson Digestive Diseases Centre, Nottingham University Hospitals, Queen’s Medical Centre, Nottingham
Dileep N. Lobo, DM, FRCS
Division of Gastrointestinal Surgery, Wolfson Digestive Diseases Centre, Nottingham University Hospitals, Queen’s Medical Centre, Nottingham, United Kingdom
| Commentary The term nesidioblastosis (G. nesidion, dim. of nesos, island + blastos, germ + oma, tumor) was coined in 1938 by Laidlaw, to describe the neodifferentiation of islets of Langerhans from pancreatic ductal epithelium in infancy; hyperplasia of the beta cells results in hyperinsulinemic hypoglycemia and is estimated to account for 3% to 4% of such cases in adults. Nesidioblastosis can be focal or diffuse throughout the pancreas and may be familial, sporadic, or, as recently reported, associated with Roux-en-Y gastric bypass. Presenting symptoms are confusion, headaches, dizziness, and syncope, which may be exacerbated by fasting and improve after eating. Diagnostic testing includes measurement of glucose, insulin, and C-peptide levels, and perhaps catheterization with venous sampling of the portal and pancreatic veins or an intra-arterial calcium stimulation test to help identify focal lesions in patients in whom findings on conventional imaging are inconclusive. The focal lesion often is too small to be identified on imaging studies, and the surrounding pancreas appears normal. Most patients with focal nesidioblastosis have a solitary lesion; about 25% of cases are multifocal, usually involving the body and tail of the pancreas. In this case, the obvious focal lesions were granulomas, and nesidioblastosis was found only incidentally when “normal” pancreas was sampled. EUS with FNA is a relatively new technique not yet completely worked into treatment algorithms for nesidioblastosis. Its role in this case was clearly important and suggests it be done in all cases of hyperinsulinemic hypoglycemia. Lawrence J. Brandt, MD Associate Editor for Focal Points |
© 2008
American Society for Gastrointestinal Endoscopy. Pubblicato da Elsevier Masson SAS. Tutti i diritti riservati.
Vol 68 - N° 3
P. 571-572 - settembre 2008 Ritorno al numero
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