Jc. Cresto et al., LONG-TERM FOLLOW-UP OF PERSISTENT HYPERINSULINEMIC HYPOGLYCEMIA OF INFANCY, Archives of Disease in Childhood, 79(5), 1998, pp. 440-444
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.