Endocrine Abstracts (2006) 11 P130

Adult-onset nesidioblastosis- a rare clinical case

T Martins, L Gomes, J Guimarães, M Melo & M Carvalheiro


University Hospitals of Coimbra, Coimbra, Portugal.


Introduction: Nesidioblastosis is a rare but well recognized disorder of persistent hyperinsulinemic hypoglycemia in infancy, but adult-onset nesidioblastosis associated with hyperinsulinemic hypoglycemia, termed noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS) has also been reported. These patients experience predominantly postprandial hypoglycaemia instead of fasting hypoglycaemia that characterizes insulinoma. Histological findings are islet hypertrophy islet-cells budding off exocrine ducts.

Clinical case: We describe an extremely rare case of NIPHS in a 23 year-old girl. She experienced predominantly postprandial hypoglycaemia with several lost consciousness episodes. Low plasma glucose level (19 mg/dl) and high insulin level (35.4 micraUI/ml) with a ratio insulin/glucose of 1.86 were consistent with the possible presence of insulinoma. Factitious causes were excluded.

Imaging studies: CT, endoscopy with ultrasonography, selective arteriography, octreotide scanning and surgical exploration with intraoperative ultrasonography were negative. It was performed a selective arterial calcium stimulation with hepatic venous sampling (ASVS) that suggested the presence of insulinoma in the body and tail of the pancreas. It was performed a subtotal pancreatectomy (body and tail). Postoperatively, her hypoglycaemic episodes completely disappeared. Histological examination of the ressected pancreas revealed hyperplasia and hypertrophy of beta cells islets- adult nesidioblastosis.

Discussion: Nesidioblastosis is a rare cause of hyperinsulinemic hypoglycaemia. Diagnosis and management continue to be a challenging experience of the endocrinologist and surgeon. When imaging studies are inconclusive, the ASVS is a powerful method for the localisation of insulinomas and can also identify diffuse forms of the disease.

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