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

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JIMD Reports, Volume 45

Part of the book series: JIMD Reports ((JIMD,volume 45))

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.

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Abbreviations

GIR:

Glucose infusion rate

HI:

Hyperinsulinism

KATP :

ATP-sensitive potassium channel

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Author information

Author notes

  1. Colin P. Hawkes and Juan J. Lado contributed equally to this work.

Authors and Affiliations

  1. Division of Endocrinology and Diabetes, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Colin P. Hawkes, Stephanie Givler & Diva D. De Leon

  2. Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Colin P. Hawkes & Diva D. De Leon

  3. Division of Pediatric Endocrinology, Ann & Robert H Lurie Children’s Hospital of Chicago, Chicago, IL, USA

    Juan J. Lado

Authors
  1. Colin P. Hawkes
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  2. Juan J. Lado
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  3. Stephanie Givler
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  4. Diva D. De Leon
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Corresponding author

Correspondence to Diva D. De Leon .

Editor information

Editors and Affiliations

  1. Tulane University Medical School, New Orleans, LA, USA

    Eva Morava

  2. Division of Metabolism & Children’s Research Centre, University Children’s Hospital Zürich, Zürich, Switzerland

    Matthias Baumgartner

  3. Division of Child and Adolescent Neurology, Mayo Clinic, Rochester, MN, USA

    Marc Patterson

  4. Clinical and Molecular Genetics Unit, UCL Institute of Child Health, London, UK

    Shamima Rahman

  5. Division of Human Genetics, Medical University Innsbruck, Innsbruck, Austria

    Johannes Zschocke

  6. Center for Child and Adolescent Medicine, Heidelberg University Hospital, Heidelberg, Germany

    Verena Peters

Additional information

Communicated by: Gerard T. Berry, M.D.

Appendices

Funding Sources

This study was supported by training grant T32DK063688-13S to J.J.L. and grant 5R01DK098517 to D.D.D.L.

Compliance with Ethics Guidelines

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2000 (5).

Conflict of Interest

Colin Hawkes, Juan Lado, Stephanie Givler, and Diva D. De Leon declare that they have no conflict of interest.

Author Contributions

Colin Hawkes analyzed data and wrote the manuscript, Juan Lado collected and analyzed data and wrote the manuscript, Stephanie Givler collected data, and Diva D. De Leon designed study and edited the manuscript.

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

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Hawkes, C.P., Lado, J.J., Givler, S., De Leon, D.D. (2018). The Effect of Continuous Intravenous Glucagon on Glucose Requirements in Infants with Congenital Hyperinsulinism. In: Morava, E., Baumgartner, M., Patterson, M., Rahman, S., Zschocke, J., Peters, V. (eds) JIMD Reports, Volume 45. JIMD Reports, vol 45. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2018_140

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  • DOI: https://doi.org/10.1007/8904_2018_140

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-58646-4

  • Online ISBN: 978-3-662-58647-1

  • eBook Packages: Biomedical and Life SciencesBiomedical and Life Sciences (R0)

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