Persistent hyperinsulinemic hypoglycemia of the neonate

Persistent hyperinsulinemic hypoglicemia of the neonate is a rare heterogenous disease (clinically, histologically, metabolically and genetically), which is characterized by inadequatly high insuline rates in the presence of severe hypoglicemia. Hyperinsulinism, rather a syndrome than a disease, of which the main metabolic feature is hypoglicemia and decreased concentration of free fatty acids and ketones in serum (insulin inhibits lypolisis and synthesizes ketonic bodies), presents a major diagnostic and therapeutic chalenge. The disease is often followed by brain atrophy contributed by the attacks of hypoglicemia. It is inherited as an autosomally recessive and autosomally dominant disease. The genetic defects is located on the short arm of the chromosome 11. The authors report a successfully applied conservative treatment in a neonate with persistent hyperinsulinemic hypoglicemia.