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Emergency management of adrenal insufficiency in children: advocating for treatment options in outpatient and field settings
  1. Bradley S Miller1,
  2. Sandra P Spencer2,
  3. Mitchell E Geffner3,
  4. Evgenia Gourgari4,
  5. Amit Lahoti5,
  6. Manmohan K Kamboj2,
  7. Takara L Stanley6,
  8. Naveen K Uli7,
  9. Brandy A Wicklow8,
  10. Kyriakie Sarafoglou1
  1. 1 Department of Pediatrics, University of Minnesota Masonic Children’s Hospital, Minneapolis, Minnesota, USA
  2. 2 Department of Pediatrics, Nationwide Children’s Hospital, Columbus, Ohio, USA
  3. 3 Department of Pediatrics, Children’s Hospital of Los Angeles, Los Angeles, California, USA
  4. 4 Department of Pediatrics, MedStar Georgetown University Hospital, Washington, DC, USA
  5. 5 Department of Pediatrics, Le Bonheur Children’s Hospital, Memphis, Tennessee, USA
  6. 6 Department of Pediatrics, Massachusetts General Hospital, Boston, Massachusetts, USA
  7. 7 Department of Pediatrics, UH Rainbow Babies and Children’s Hospital, Cleveland, Ohio, USA
  8. 8 Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Manitoba, Canada
  1. Correspondence to Dr Bradley S Miller, Department of Pediatrics, University of Minnesota Masonic Children’s Hospital, 2450 Riverside Ave, Minneapolis, MN 55454, USA; mille685{at}umn.edu

Abstract

Adrenal insufficiency (AI) remains a significant cause of morbidity and mortality in children with 1 in 200 episodes of adrenal crisis resulting in death. The goal of this working group of the Pediatric Endocrine Society Drug and Therapeutics Committee was to raise awareness on the importance of early recognition of AI, to advocate for the availability of hydrocortisone sodium succinate (HSS) on emergency medical service (EMS) ambulances or allow EMS personnel to administer patient’s HSS home supply to avoid delay in administration of life-saving stress dosing, and to provide guidance on the emergency management of children in adrenal crisis. Currently, hydrocortisone, or an equivalent synthetic glucocorticoid, is not available on most ambulances for emergency stress dose administration by EMS personnel to a child in adrenal crisis. At the same time, many States have regulations preventing the use of patient’s home HSS supply to be used to treat acute adrenal crisis. In children with known AI, parents and care providers must be made familiar with the administration of maintenance and stress dose glucocorticoid therapy to prevent adrenal crises. Patients with known AI and their families should be provided an Adrenal Insufficiency Action Plan, including stress hydrocortisone dose (both oral and intramuscular/intravenous) to be provided immediately to EMS providers and triage personnel in urgent care and emergency departments. Advocacy efforts to increase the availability of stress dose HSS during EMS transport care and add HSS to weight-based dosing tapes are highly encouraged.

  • adrenal insufficiency
  • pituitary-adrenal system
  • glucocorticoids
  • endocrinology

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Footnotes

  • Contributors All the authors have participated in the concept and design, analysis and interpretation of data, drafting or revising of the manuscript, have approved the manuscript as submitted, and have agreed to be accountable for all aspects of the work.

  • Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests BSM is a consultant for AbbVie, Ascendis, Ferring, Novo Nordisk, Pfizer, Sandoz, Soleno and Tolmar and has received research support from Alexion, Ascendis, BioMarin, Endo Pharmaceuticals, Genentech, Genzyme, Novo Nordisk, Opko, Sandoz, Sangamo, Shire, Tolmar and Versartis. MK received grant support from T1D Exchange Quality Improvement Collaborative. MG is a consultant for Spruce Biosciences, Millendo, Pfizer, and BridgeBio. KS receives research support from the DHHS Federal Food and Drug Administration, NIH National Cancer Institute, March of Dimes, National Science Foundation, Spruce Biosciences, Alexion and Neurocrine.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Presented at The work has been presented at Pediatric Academic Society Meeting 2018, ’Year In Review: Emergency Management of Adrenal Insufficiency in Children: A Clinical Practice Guideline', Toronto, Ontario, Canada, May 2018.

  • Patient consent for publication Not required.

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