LONG-TERM FOLLOW-UP OF PERSISTENT HYPERINSULINEMIC HYPOGLYCEMIA OF INFANCY

Twenty six children with hypoglycaemia were diagnosed and followed bet ween 1975 and 1995. Diagnosis was confirmed by a high insulin:glucose ratio, and low free fatty acid and 3-hydroxybutyrate on fasting. All p atients were treated with diazoxide at a maximum dose of 20 mg/kg/day. Requirement of a higher dose was considered as a failure of medical t reatment and an indication for surgery. Sixteen children responded to diazoxide; 10 failed to respond and underwent pancreatic resection. Si x of the latter group started with symptoms in the neonatal period. El even of the 26 children have neurological sequelae. Head growth and ne urological outcome correlated well. Additionally, non-specific electro encephalogram abnormalities (slow waves) appear to he indicative of su bclinical hypoglycaemia during follow up.