Effects of hypoglycemia on developmental outcome in children with congenital hyperinsulinism.

Children with congenital hyperinsulinism are at risk for recurring, severe episodes of hypoglycemia that can cause seizures, brain damage, and developmental delay. To assess the frequency of permanent brain damage in this disorder, we carried out a telephone survey of 68 children who presented to The Children's Hospital of Philadelphia between 1980 and 2000. One third of the group had some degree of developmental delay. Those presenting in the first week of life and those with medically unresponsive hyperinsulinism were more likely to have delays. Early recognition and control of hypoglycemia are essential for preventing developmental delay in these children.