Symptoms and early diagnostic possibilities of pancreatic endocrine cells hyperplasia (nesidioblastosis)]

Nesidioplastosis in adults is one of a rare causes of hyperinsulinemic hypoglycemia. Symptoms include chronic or recurrent hypoglycemias, often with neurological signs. Due to the looses of consciousness with coexisting seizures, in many cases patients are treated on epilepsy. Right diagnosis is usually late established, when the damages in the central nervous system (CNS) are irreversible. Early diagnosis of the disease and appropriate treatment might help to avoid serious disability in these patients. The aim of the study was to asses modern diagnostics of the nesidioblastosis with an emphasis on the biochemical and hormonal tests and imaging modalities. Patients enrolled to the study were aged between 18 and 72 years of age, and had chronic or recurrent hypoglycemia caused by hyperinsulinemia. In all patients fasting glucose and fasting insulinemia tests were performed, as well as the fasting blood test or in the 24-hour profile tests. Several techniques were used including ultrasound (US), abdominal computer tomography (CT), in two patients magnetic resonance imaging, scintigraphy of somatostatin receptors in seven patients, and in two patients scintigraphy with glucagone-like peptide-1 (GLP-1) analogue-labeled marker was done. In the performed tests low values of the blood glucose were found, whereas insulin levels, however not adequate to the blood glucose, were nearly always within the normal range. In the standard imaging only in one patient tumor lesion in the pancreatic tail was revealed, though not confirmed in the intraoperative histology. In the scintigraphy examination with the somatostatin analogue in one patient slightly increased collection of the marker in whole pancreas was reported and in the other patient focal collection in the pancreatic tail was observed. Scintigraphy with GLP-1 analogue revealed focal collection of the marker in one case. Five patients were underwent surgical treatment. In the histopathology in all operated patients hyperplasia of the endocrine pancreatic cells with positive immuno. histochemic reaction on the insulin was found. In the three cases despite hyperplasia of pancreatic islets, small sizes insulinomas were detected as well. 1. The diagnosis of nesidioblastosis should be taken into consideration in all patients with unclear-cause hypoglycemias, in whom simultaneously insulin blood level is inadequate to the level of glucose. 2. Widely available imaging examinations: US, CT, MRI are useless in the diagnosis of nesidioblastosis. 3. Among the imaging methods in preoperative diagnostics of hypoglycemia with concomitant hyperinsulinemia somatostatin receptor scintigraphy seems to have specific, though limited role - it is valuable only in the severe, diffused lesions. 4. Recurrent hypoglycemias after 70% excision of the pancreas may indicate the possibility of coexistence of pancreatic islets hyperplasia and insulin secreting insulinoma.