Nesidioblastosis a rare cause of hipoglycemia in adults

Lopez Alaminos Maria Elena; Fernandez Mikel Gonzalez; Alvarado Rosas Karol Almendra; Urzaiz Leticia Serrano; Diego Garcia Patricia de; Pablo Trincado; Perez Javier Acha

doi:10.1530/endoabs.70.AEP481

Introduction: Noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS) is an entity with low prevalence (4%), more frequent in neonates and infants and with a male preponderance, characterized by endogenous hyper insulinemic hypoglycemia, not caused by an insulinoma. Pancreatic histology usually reveals diffuse nesidioblastosis.

Case report: A 21-years-old man without a remarkable personal or family history was referred describing a 15 kg weight gain and late-afternoon asthenia and drowsines for the last two years that improved eating carbohydrates. He also described several episodes of loss of consciousness and neuroglycopenic symptoms cooccurring with glycemia of 32 mg/dl despite nutritional modifications and diazoxide. Results of a fasting test showed: glycemia 42 mg/dl, plasma insulin 11.5 mcrUI/ml, C-peptide 2.35 ng/ml, proinsulin 1.5 pmol/l, low beta-hydroxybutyrate, a negative sulfonylurea/meglitinide screen and negative insulin antibodies and insulin-receptor antibodies. As an insulinoma was suspected, transabdominal ultrasonography, abdominal computed tomography (CT), magnetic resonance imaging (MRI), and a 99 m Tc somatostatin receptor scintigraphywere performed, with negative results. A selective arterial calcium stimulation test (SACST) with hepatic venous sampling after calcium gluconate infusion did not show any gradient.


	Superior mesenteric artery	Gastrodudenal artery	Splenic artery
Basal Insulin mcr UI/ml	19.50	21.30	19.10
Insulin 30 s mcrUI/ml	22.20	24.40	33.20
Insulin 60 s mcrUI/ml	35.90	35.30	48.30
Insulin 120 s mcrUI/ml	71.60	71.60	67.10

Due to persistent symptoms refractory to medical management and localization results a partial pancreatectomy was performed in which histopathology descriptions showed patchy nesidioblastosis changes, islet cell hypertrophy and redifferentiation of islet of Langerhans cells from pancreatic duct epithelium. After surgery patient´s symptoms greatly improved.

Discussion: The predominant clinical feature of noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS) is usually postprandial hypoglycemia but also fasting hypoglycemia can occur, with biochemical findings similar to insulinoma. For patients with NIPHS and refractory postprandial hypoglycemia, surgery remains the mainstay of therapy.

Endocrine Abstracts

AEP481