Noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS)

The most common endogenous hyperinsulinemic hypoglycemia in non-diabetic adult is insulinoma. Non-insu-linoma functional p cell disorders are less common in adult. Endogenous hyperinsulinemic hypoglycemia predominantly presented as postprandial hypoglycemia is termed noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS). NIPHS was easily delayed diagnosed despite repeated episodes of neuroglycopenia due to negative 72hour fast test.The pathologic features are islet cell hypertrophy, enlarged and hyperchromatic β cell nuclei, sometimes are coexistent with increased islets number or size. Diagnosis is often made when endogenous hyperinsulinemic hypoglycemia is provoked by mixed meal test. Selective intraarterial calcium stimulation test (IACS) often confirms the presence of endogenous hyperinsulinism and localizes the area of hyperfunctioning β-cells. There is no standard treatment for NIPHS to date. The most acceptable strategy is subtotal pancreatectomy guided by IACS. Application of rapid insulin assay with IACS performed intra-operatively may be more precise in determining the extent and completeness of subtotal pancreatectomy.