Hyperinsulinism in infants and children

Pediatr Clin North Am. 1997 Apr;44(2):363-74. doi: 10.1016/s0031-3955(05)70481-8.


Hyperinsulinism is the most common cause of hypoglycemia in early infancy. Congenital hyperinsulinism, formerly termed nesidioblastosis, is usually caused by genetic defects in beta-cell regulation, including a severe recessive disorder of the sulfonylurea receptor, a milder dominant form of hyperinsulinism, and a syndrome of hyperinsulinism plus hyperammonemia. Transient neonatal hyperinsulinism may be associated with perinatal asphyxia or small-for-dates birthweight and maternal diabetes. To prevent permanent brain damage from hypoglycemia, the treatment of infants with hyperinsulinism must be prompt and aggressive. A combination of medical therapy with diazoxide or octreotide, a long-acting somatostatin analog, and surgical 95% subtotal pancreatectomy may be required.

Publication types

  • Review

MeSH terms

  • Child
  • Combined Modality Therapy
  • Diazoxide / therapeutic use
  • Humans
  • Hyperinsulinism / diagnosis*
  • Hyperinsulinism / etiology
  • Hyperinsulinism / metabolism
  • Hyperinsulinism / therapy*
  • Infant
  • Octreotide / therapeutic use
  • Pancreatectomy
  • Prognosis
  • Risk Factors


  • Diazoxide
  • Octreotide