Background/Aims: Continuous intravenous glucagon is frequently used in the management of severe congenital hyperinsulinism (HI), but its efficacy in these patients has not been systematically evaluated. The aim of this study was to describe the use of continuous intravenous glucagon and to evaluate its effect on the glucose infusion rate (GIR) requirement in infants with HI.
Methods: Retrospective chart review of children with HI who received continuous intravenous glucagon for prevention of hypoglycemia at the Children’s Hospital of Philadelphia between 2003 and 2013.
Results: Forty (22 male) infants were included, and median (IQR) age at glucagon treatment was 29 (23, 54) days. Median glucagon dose was 205 (178, 235) mcg/kg/day and duration of treatment was 5 (3, 9) days. GIR reduced from 18.5 (12.9, 22.8) to 11 (6.6, 17.5) mg/kg/min 24 h after starting glucagon (p < 0.001), and hypoglycemia frequency reduced from 1.9 (1.3, 2.9) to 0.7 (0.3, 1.2) episodes per day. Vomiting (n = 11, 13%), rash (n = 2, 2%), and respiratory distress (n = 15, 19%) were seen during glucagon treatment.
Conclusion: An intravenous glucagon infusion reduces the required GIR to maintain euglycemia, decreasing the risks associated with the administration of high fluid volume or fluids with high-glucose concentrations.
Glucose Hypoglycemia Insulin Neonate Pancreas
Glucose infusion rate
ATP-sensitive potassium channel
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Barrington KJ (2000) Umbilical artery catheters in the newborn: effects of position of the catheter tip. Cochrane Database Syst Rev. CD000505Google Scholar
Belik J, Musey J, Trussell RA (2001) Continuous infusion of glucagon induces severe hyponatremia and thrombocytopenia in a premature neonate. Pediatrics 107:595–597CrossRefGoogle Scholar
Carter PE, Lloyd DJ, Duffty P (1988) Glucagon for hypoglycaemia in infants small for gestational age. Arch Dis Child 63:1264–1266CrossRefGoogle Scholar
Charsha DS, McKinley PS, Whitfield JM (2003) Glucagon infusion for treatment of hypoglycemia: efficacy and safety in sick, preterm infants. Pediatrics 111:220–221CrossRefGoogle Scholar
Ferrara C, Patel P, Becker S, Stanley CA, Kelly A (2016) Biomarkers of insulin for the diagnosis of hyperinsulinemic hypoglycemia in infants and children. J Pediatr 168:212–219CrossRefGoogle Scholar
Hawkes CP, Adzick NS, Palladino AA, De Leon DD (2016) Late presentation of fulminant necrotizing enterocolitis in a child with hyperinsulinism on octreotide therapy. Horm Res Paediatr 86(2):131–136CrossRefGoogle Scholar
Hussain K, Bryan J, Christesen HT, Brusgaard K, Aguilar-Bryan L (2005) Serum glucagon counterregulatory hormonal response to hypoglycemia is blunted in congenital hyperinsulinism. Diabetes 54:2946–2951CrossRefGoogle Scholar
Lord K, De Leon DD (2013) Monogenic hyperinsulinemic hypoglycemia: current insights into the pathogenesis and management. Int J Pediatr Endocrinol 2013:3CrossRefGoogle Scholar
Lord K, Dzata E, Snider KE, Gallagher PR, De Leon DD (2013) Clinical presentation and management of children with diffuse and focal hyperinsulinism: a review of 223 cases. J Clin Endocrinol Metab 98:E1786–E1789CrossRefGoogle Scholar
Lord K, Radcliffe J, Gallagher PR, Adzick NS, Stanley CA, De Leon DD (2015) High risk of diabetes and neurobehavioral deficits in individuals with surgically treated hyperinsulinism. J Clin Endocrinol Metab 100(11):4133–4139CrossRefGoogle Scholar
Meissner T, Wendel U, Burgard P, Schaetzle S, Mayatepek E (2003) Long-term follow-up of 114 patients with congenital hyperinsulinism. Eur J Endocrinol 149:43–51CrossRefGoogle Scholar
Miralles RE, Lodha A, Perlman M, Moore AM (2002) Experience with intravenous glucagon infusions as a treatment for resistant neonatal hypoglycemia. Arch Pediatr Adolesc Med 156:999–1004CrossRefGoogle Scholar
Palladino AA, Stanley CA (2011) A specialized team approach to diagnosis and medical versus surgical treatment of infants with congenital hyperinsulinism. Semin Pediatr Surg 20:32–37CrossRefGoogle Scholar
Quesada I, Tuduri E, Ripoll C, Nadal A (2008) Physiology of the pancreatic alpha-cell and glucagon secretion: role in glucose homeostasis and diabetes. J Endocrinol 199:5–19CrossRefGoogle Scholar
Steinkrauss L, Lipman TH, Hendell CD, Gerdes M, Thornton PS, Stanley CA (2005) Effects of hypoglycemia on developmental outcome in children with congenital hyperinsulinism. J Pediatr Nurs 20:109–118CrossRefGoogle Scholar
Unger RH, Cherrington AD (2012) Glucagonocentric restructuring of diabetes: a pathophysiologic and therapeutic makeover. J Clin Invest 122:4–12CrossRefGoogle Scholar
Vajravelu ME, De Leon DD (2018) Genetic characteristics of patients with congenital hyperinsulinism. Curr Opin Pediatr 30(4):568–575PubMedGoogle Scholar