Original articleClinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism
Section snippets
Methods
Between 1999 and 2002, data were prospectively collected from all neonates admitted to the Neonatal Intensive Care Unit at Children’s Hospital of Philadelphia who were referred to the Division of Endocrinology for hypoglycemia due to suspected hyperinsulinism. Those neonates with hyperinsulinism that subsequently spontaneously resolved were diagnosed with prolonged neonatal hyperinsulinism. After excluding infants of diabetic mothers and neonates with permanent genetic forms of hyperinsulinism
Clinical characteristics
Table I summarizes the clinical features of the 26 neonates with prolonged neonatal hyperinsulinism. All were born within 100 miles of Philadelphia. Compared with the reference newborn population, there was a significant excess of males, SGA newborns, and cesarean deliveries. Thirty-five percent of the neonates had signs of perinatal stress (including metabolic acidosis and/or abnormal fetal tracing) suggesting birth asphyxia, although none had a 5-minute APGAR score below 6. Prematurity and
Discussion
The results of the present study define a group of neonates who have hypoglycemia persisting for several weeks to months after birth as the result of a prolonged form of hyperinsulinism that eventually resolves completely. This prolonged neonatal hyperinsulinism syndrome is frequently associated with male sex, low birth weight, perinatal stress, and cesarean delivery. In contrast to children with severe neonatal-onset hyperinsulinism associated with KATP mutations, neonates with prolonged
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Supported in part by a research fellowship award from the Juvenile Diabetes Research Foundation (F.M.H.), National Institutes of Health grant R01 DK56268 (C.A.S.), and General Clinical Research Center grant M01 RR00240.