Acute Illness and Death in Children With Adrenal Insufficiency

Abstract

BackgroundAdrenal 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.MethodsThree 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.ResultsThirteen 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.ConclusionsIn 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.

Adrenal insufficiency in pediatric kidney transplantation recipients

“…Although this has not been previously reported in PKT recipients, Worth et al. reported that 30% of their cohort (children with AI, n = 127) had a recorded emergency department visit due to acute illness, 49 and Eyal et al. reported recurrent hospital admissions were significantly associated with adrenal crisis among children with AI 50 .…”

Cortisol Levels During Acute Illnesses in Children and Adolescents

“…According to a recent study by Worth et al, the rate of death due to an adrenal crisis among children with adrenal insufficiency has remained high. The correct GC stress dose for different acute illnesses has the potential to prevent death .…”

Emergency and perioperative management of adrenal insufficiency in children and young people: British Society for Paediatric Endocrinology and Diabetes consensus guidance

“…Symptoms of AI may initially be nonspecific and include growth faltering, lethargy, poor feeding, abdominal pain, vomiting and weight loss. 3 Infants may develop hypoglycaemia irrespective of the cause of AI. A recent review provides a comprehensive summary of causes of AI in children and adults.…”

“…Suppression of ACTH leading to AI may also result from exogenous high dose glucocorticoid use. Symptoms of AI may initially be non-specific and include growth faltering, lethargy, poor feeding, abdominal pain, vomiting and weight loss 3. Infants may develop hypoglycaemia irrespective of the cause of AI.…”