ESPE Abstracts

Background: Adrenal Insufficiency (AI) is a heterogeneous diagnosis comprising multiple central and peripheral causes. The unifying feature is the requirement for glucocorticoid replacement and subsequent risk of life-threatening Adrenal Crisis (AC) and Adrenal Death (AD). Very few studies have investigated rates of AD in children. Multiple studies have reported symptoms of children with AC as interpreted by medical staff but no studies have looked at parentally reported symptoms prior to hospital admission. We investigated preceding symptoms and parental management of AC and incidence of AD and associated factors.

Methods: Data from children and young people with AC and AD over a 13-year period, were reviewed. Medical notes and coroner’s reports were reviewed for those with AD. Separately, data were collected through interview-aided questionnaires from children with AI who had AC in the previous eight years. A final study investigated parent perceptions 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 and one AD per year in our patient cohort. Those with unspecified adrenal insufficiency were overrepresented (P = 0.004). Thirty-eight patients 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 from parents. Questionnaires revealed that 79% of parents reported confidence in the administration of intramuscular hydrocortisone and only 20% identified a missed opportunity for injection.

Conclusions: The rate of AD in our cohort was higher than published figures for children with T1DM, a figure already significantly elevated from the population mean. This highlights the importance of medical professionals understanding the severity of this disease. In children experiencing AC, parents followed ‘sick day’ guidance for oral hydrocortisone in all cases. However, these same patients rarely administered intramuscular hydrocortisone. This is a contrasting finding from the 79% of parents who reported confidence in this exact task, highlighting a significant discrepancy between perceived confidence with a task and performance of that at the time of greatest clinical need. Local training programmes for management of AC are comprehensive, but apparently insufficient to prevent the most serious crises and deaths. New strategies to encourage use of parenteral hydrocortisone must be devised.