Endocrine Abstracts

The most common reason for the rare condition of hyperinsulinemia-related hypoglycaemia is insulinoma, a tumor of pancreatic islet cells. However, nesidioblastosis characterized by diffuse or focal hyperplasia of the pancreatic islet cells is the most common cause of hyperinsulinemic hypoglycemia in newborns. Nesidioblastosis seen in newborns is now called ‘persistent hyperinsulinemic hypoglycemia of infancy’ (PHHI) while the condition in adults is called ‘noninsulinoma pancreatogenous hypoglycemia syndrome’ (NIPHS) as a separate entity. It is impossible to clinically differentiate insulinomas from NIPHS.

A 38-year-old female presented with neuroglycopenic symptoms in the form of drowsiness, inability to speak, numbness in the mouth, and nausea in the last 3–4 months. Endogenous hyperinsulinemia was found with recurrent neuroglycopenic symptoms (the glucose level was 25 mg/dl, insulin 43.9 μ/ml, and C-peptide 5.54 ng/ml). No lesion was found on imaging tests including enhanced computed tomography (CT) methods performed with a preliminary diagnosis of insulinoma. A suspicious hyperperfusion was present in the pancreatic tail on the perfusion CT examination performed after obtaining approval. The selective arterial calcium stimulation test (SACST) result was consistent with a diffuse disease in the body and tail. The patient underwent partial (75%) pancreatectomy and is now followed up as a diabetes patient on intensive insulin treatment at the postoperative 38th month.

The NIPHS is rarely seen in the adult age group. SACST seems to be the most suitable test to differentiate diffuse or multiple disease from insulinoma and to guide the surgery when advanced radiological imaging methods are inadequate to detect the presence of insulinoma. Regarding perfusion CT, it would be more appropriate to wait for comparative data to be put forward in a more consistent manner. When no response to medical treatment, partial/total pancreatectomy is appropriate treatment option as it enables recovery from the hypoglycemic episodes despite leading to diabetes.