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The Effect of Continuous Intravenous Glucagon on Glucose Requirements in Infants with Congenital Hyperinsulinism

  • Colin P. Hawkes
  • Juan J. Lado
  • Stephanie Givler
  • Diva D. De Leon
Research Report
Part of the JIMD Reports book series

Abstract

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.

Keywords

Glucose Hypoglycemia Insulin Neonate Pancreas 

Abbreviations

GIR

Glucose infusion rate

HI

Hyperinsulinism

KATP

ATP-sensitive potassium channel

References

  1. Barrington KJ (2000) Umbilical artery catheters in the newborn: effects of position of the catheter tip. Cochrane Database Syst Rev. CD000505Google Scholar
  2. Belik J, Musey J, Trussell RA (2001) Continuous infusion of glucagon induces severe hyponatremia and thrombocytopenia in a premature neonate. Pediatrics 107:595–597Google Scholar
  3. Carter PE, Lloyd DJ, Duffty P (1988) Glucagon for hypoglycaemia in infants small for gestational age. Arch Dis Child 63:1264–1266Google Scholar
  4. Charsha DS, McKinley PS, Whitfield JM (2003) Glucagon infusion for treatment of hypoglycemia: efficacy and safety in sick, preterm infants. Pediatrics 111:220–221Google Scholar
  5. 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 168:212–219Google Scholar
  6. Food and Drug Administration (n.d.) Glucagon for injection (rDNA origin). https://www.accessdata.fda.gov/drugsatfda_docs/label/2015/201849s002lbl.pdf
  7. 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):131–136Google Scholar
  8. 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 54:2946–2951Google Scholar
  9. Lord K, De Leon DD (2013) Monogenic hyperinsulinemic hypoglycemia: current insights into the pathogenesis and management. Int J Pediatr Endocrinol 2013:3Google Scholar
  10. 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–E1789Google Scholar
  11. 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):4133–4139Google Scholar
  12. Meissner T, Wendel U, Burgard P, Schaetzle S, Mayatepek E (2003) Long-term follow-up of 114 patients with congenital hyperinsulinism. Eur J Endocrinol 149:43–51Google Scholar
  13. 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 156:999–1004Google Scholar
  14. Palladino AA, Stanley CA (2011) A specialized team approach to diagnosis and medical versus surgical treatment of infants with congenital hyperinsulinism. Semin Pediatr Surg 20:32–37Google Scholar
  15. 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 199:5–19Google Scholar
  16. 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 20:109–118Google Scholar
  17. Unger RH, Cherrington AD (2012) Glucagonocentric restructuring of diabetes: a pathophysiologic and therapeutic makeover. J Clin Invest 122:4–12Google Scholar
  18. Vajravelu ME, De Leon DD (2018) Genetic characteristics of patients with congenital hyperinsulinism. Curr Opin Pediatr 30(4):568–575Google Scholar

Copyright information

© Society for the Study of Inborn Errors of Metabolism (SSIEM) 2018

Authors and Affiliations

  • Colin P. Hawkes
    • 1
    • 2
  • Juan J. Lado
    • 3
  • Stephanie Givler
    • 1
  • Diva D. De Leon
    • 1
    • 2
  1. 1.Division of Endocrinology and DiabetesThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  2. 2.Department of Pediatrics, Perelman School of MedicineUniversity of PennsylvaniaPhiladelphiaUSA
  3. 3.Division of Pediatric EndocrinologyAnn & Robert H Lurie Children’s Hospital of ChicagoChicagoUSA

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