Affiliations: Department of Pediatrics, Division of Neonatology, University of Minnesota Amplatz Children's Hospital, University of Minnesota, Minneapolis, MN, USA | Department of Pediatrics, Division of Endocrinology, University of Minnesota Amplatz Children's Hospital, University of Minnesota, Minneapolis, MN, USA | Center for Neurobehavioral Development, University of Minnesota, Minneapolis, MN, USA
Note:  Current address: Division of Neonatology and Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
Note:  Corresponding author: Raghavendra Rao, Department of Pediatrics, Mayo Mail Code 39, 420 Delaware Street, SE, Minneapolis, MN 55455, USA. Tel.: +1 612 626 0644; Fax: +1 612 624 8176; E-mail: [email protected]
Abstract: This patient series reports on prolonged hyperinsulinism in four newborn infants without characteristic perinatal risk factors. The condition presented with asymptomatic hypoglycemia soon after birth and was accompanied by inappropriately elevated plasma insulin levels and suppressed ketogenesis and lipolysis. Glucose infusion rates between 11 and 20 mg/kg/min were necessary for correction of hypoglycemia. Sustained euglycemia could be achieved only after starting diazoxide. In all infants, diazoxide could be weaned off within weeks without the recurrence of hypoglycemia. This case series illustrates the varied perinatal history and clinical course of prolonged neonatal-onset hyperinsulinism and highlights the importance of considering this condition in infants without typical predisposing factors.