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. 2021 Oct 13:12:757566.
doi: 10.3389/fendo.2021.757566. eCollection 2021.

Acute Illness and Death in Children With Adrenal Insufficiency

Chris Worth  1 , Avni Vyas  2 , Indraneel Banerjee  1   2 , Wei Lin  1 , Julie Jones  1 , Helen Stokes  1 , Nicci Komlosy  3 , Steven Ball  2   3 , Peter Clayton  1   2
Affiliations

Acute Illness and Death in Children With Adrenal Insufficiency

Chris Worth et al. Front Endocrinol (Lausanne). .

Abstract

Background: Adrenal Insufficiency (AI) can lead to life-threatening Adrenal Crisis (AC) and Adrenal Death (AD). Parents are trained to prevent, recognise and react to AC but there is little available information on what parents are actually doing at home to manage symptomatic AI.

Methods: Three approaches were taken: (A) A retrospective analysis of patient characteristics in children and young people with AD over a 13-year period, (B) An interview-aided questionnaire to assess the circumstances around AC in children currently in our adrenal clinic, and (C) a separate study of parent perceptions of the administration of parenteral hydrocortisone.

Results: Thirteen patients died (median age 10 years) over a thirteen-year period resulting in an estimated incidence of one AD per 300 patient years. Those with unspecified adrenal insufficiency were overrepresented (P = 0.004). Of the 127 patients contacted, thirty-eight (30%) were identified with hospital attendance with AC. Responses from twenty patients (median age 7.5 years) with AC reported nausea/vomiting (75%) and drowsiness (70%) as common symptoms preceding AC. All patients received an increase in oral hydrocortisone prior to admission but only two received intramuscular hydrocortisone. Questionnaires revealed that 79% of parents reported confidence in the administration of intramuscular hydrocortisone and only 20% identified a missed opportunity for injection.

Conclusions: In children experiencing AC, parents followed 'sick day' guidance for oral hydrocortisone, but rarely administered intramuscular hydrocortisone. This finding is discrepant from the 79% of parents who reported confidence in this task. Local training programmes for management of AC are comprehensive, but insufficient to prevent the most serious crises. New strategies to encourage use of parenteral hydrocortisone need to be devised.

Keywords: adrenal crisis; adrenal insufficiency; hydrocortisone; outcomes; socioeconomic.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Flowchart demonstrating numbers of patients identified at each stage of the study. Patient groups were compared at every stage to assess for differences. Results are provided in the manuscript and Tables 1 and 2 .
Figure 2
Figure 2
Venn diagram of parent-reported symptoms. Most patients presented with overlapping symptoms and very few patients presented to hospital with an isolated symptom. Nausea/vomiting, pyrexia and drowsiness were the most common parent-reported symptoms.
See this image and copyright information in PMC

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