Non-insulinoma pancreatogenous hypoglycemia syndrome

Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS) is a rare disorder characterized by episodes of hypoglycemia (low blood sugar) caused by excessive insulin secretion from the pancreas in the absence of an insulin-producing tumor (insulinoma). The condition is also sometimes referred to as nesidioblastosis in adults or non-insulinoma hyperinsulinemic hypoglycemia. In NIPHS, the beta cells of the pancreas — the cells responsible for producing insulin — are diffusely enlarged or abnormally functioning, leading to inappropriate insulin release, particularly after meals (postprandial hypoglycemia). This distinguishes it from insulinoma, where a discrete tumor is the source of excess insulin. The primary body system affected is the endocrine/metabolic system, though the neurological system is also significantly impacted due to recurrent low blood sugar episodes. Key symptoms include neuroglycopenic manifestations such as confusion, dizziness, visual disturbances, difficulty concentrating, weakness, tremor, sweating, palpitations, and in severe cases, seizures or loss of consciousness. Symptoms typically occur two to four hours after eating. The condition predominantly affects adults and has gained increased recognition in patients who have undergone gastric bypass surgery, though it can also occur in individuals without prior surgical history. Diagnosis of NIPHS can be challenging and often requires selective arterial calcium stimulation testing (SACST) to confirm diffuse pancreatic beta-cell dysfunction rather than a focal lesion. Treatment options include dietary modifications (such as frequent small meals low in simple carbohydrates), medications to suppress insulin secretion (such as diazoxide, octreotide, or acarbose), and in refractory cases, partial pancreatectomy. However, surgical outcomes can be variable, and hypoglycemia may recur even after surgery. Management requires a multidisciplinary approach involving endocrinologists and specialized surgeons.

Common questions about Non-insulinoma pancreatogenous hypoglycemia syndrome