Persistent Hyperinsulinemic Hypoglycemia

摘要: Hyperinsulinism can occur throughout childhood but is most common in infancy. Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) the important cause early The excessive secretion insulin responsible for profound and requires aggressive treatment to prevent severe irreversible brain damage. Onset be neonatal period or later, with severity decreasing age. PHHI a heterogeneous disorder two histopathological lesions, diffuse (DiPHHI) focal (FoPHHI), which are clinically indistinguishable. FoPHHI sporadic characterized by somatic islet-cell hyperplasia. DiPHHI corresponds functional abnormality whole pancreas often recessive although rare dominant forms occur, usually outside newborn period. Differentiation between lesions because therapeutic approach genetic counselling differ radically. PET scanning 18-fluoro-dopa distinguish PHHI. A combination glucose glucagon started as an emergency soon tentative diagnosis made. This followed diazoxide other medication. Patients who resistant medical require pancreatectomy; definitively cured limited pancreatectomy, subtotal following there high risk diabetes mellitus. hyperinsulinism older children commonly caused pancreatic adenoma.