Hyperinsulinism in infants and children.
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.
Stanley
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Division of Endocrinology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, USA.
D002648:Child; D003131:Combined Modality Therapy; D003981:Diazoxide / Q000627:therapeutic use; D006801:Humans; D006946:Hyperinsulinism / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism / Q000628:therapy*; D007223:Infant; D015282:Octreotide / Q000627:therapeutic use; D010180:Pancreatectomy; D011379:Prognosis; D012307:Risk Factors
D003981:Diazoxide; D015282:Octreotide