Background: >There is a wide variation in the reported rate of acute adrenal insufficiency (AI) related adverse events (sick day episodes and adrenal crises) between centres.
Objective: Evaluate the level of consensus on the criteria that should be considered essential for defining and managing adverse events associated with acute AI in children.
Methods: Three groups of clinicians including active users of the International Congenital Adrenal Hyperplasia & International Disorders of Sex Development (I-CAH/I-DSD) Registries (n = 66), non-active users of I-CAH/I-DSD (n = 35) and the EuRRECa e-Reporting Registry (n = 10) were approached to complete an online survey.
Results: 56 clinicians from 27 countries responded to the survey; the response rates for the three Registry groups were 42 (65%), 11 (31%) and 3 (30%), respectively. Written corticosteroid management plans and one to one patient/parent education were provided by 54 (96%) and 51 (91%) clinicians, respectively; 33 (59%) provided steroid-aware emergency cards. 56 (100%) clinicians advised an increase in glucocorticoid dosing (sick day dosing) in the event of fever; 55 (98%) and 53 (95%) advised sick day dosing in the event of severe infection (eg. pneumonia) and major surgery, respectively. Less common indications for sick day dosing included vaccination and mild afebrile intercurrent illness, recommended by 17 (30%) and 9 (16%) clinicians, respectively. The most frequently reported sick day dosing regimen was tripling the total daily dose of hydrocortisone and administering 3 times daily, reported by 24 (43%) clinicians. 40 (71%) specified the duration of sick day dosing as ≥48 hours for severe infections. Vomiting and diarrhoea were the most common indications for IM hydrocortisone, reported by 34 (61%) and 25 (45%) clinicians, respectively. Over 50% of respondents indicated that essential clinical criteria for an adrenal crisis should include fatigue and nausea or vomiting and over 60% indicated that the criteria should include hypotension, hyponatraemia, hyperkalaemia and clinical improvement following parenteral glucocorticoids. In the event of adrenal crisis, 47 (84%) reported that the majority of patients were admitted to hospital. A bolus parenteral injection of hydrocortisone and glucose infusions were the most frequently administered medications, reported by 50 (89%) and 32 (57%) of clinicians, respectively.
Conclusions: Although there is considerable variation in the definition and management of AI related adverse events in children amongst specialist centres, there is also good evidence of consensus that can be used to develop standardised criteria for developing benchmarks and facilitating care improvement.
22 Sep 2021 - 26 Sep 2021