PMON191 Current Management of Acute Adrenal Insufficiency Related Adverse Events in Children with Congenital Adrenal Hyperplasia- Results of an International Survey of Specialist Centres

Abstract

Abstract Background There is wide variation in the reported rate of acute adrenal insufficiency (AI) related adverse events (sick day episodes and adrenal crises) between centres and the definition of these events as well as their management may vary from one centre to another. A greater standardisation of the definition and management of these events would facilitate the benchmarking and comparison of care across centres. Objective Evaluate the level of consensus on the criteria that should be considered 'essential' for defining and managing acute AI related adverse events in children with Congenital Adrenal Hyperplasia. Methods 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; 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%) and 55 (98%) clinicians advised an increase in glucocorticoid dosing (sick day dosing) in the event of fever or severe infection (eg. pneumonia). 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 intramuscular hydrocortisone, reported by 34 (61%) and 25 (45%) clinicians, respectively. Over 50% of respondents indicated that essential clinical criteria for 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. 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 on specific aspects that can be used to develop standardised criteria and lead to greater benchmarking of care. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.