Emergency and perioperative management of adrenal insufficiency in children and young people: BSPED consensus guidelines

Abstract

Adrenal insufficiency (AI) is characterised by a deficiency in adrenal cortisol production. It can arise due to a primary adrenal disorder (eg, autoimmune Addison’s disease or congenital adrenal hyperplasia) which is commonly associated with glucocorticoid and mineralocorticoid deficiency. Secondary AI occurs when a pathology in the pituitary gland causes a lack of adrenocorticotropic hormone leading to a cortisol deficiency (eg, hypopituitarism) or as a result of suppression of the hypothalamic–pituitary–adrenal axis from exogenous glucocorticoids.1 The glucocorticoid deficiency in AI is managed by replacing hydrocortisone. During times of physiological stress, additional glucocorticoids are required. Diagnosis is often delayed due to the non-specific symptoms. Failure to recognise AI and initiate treatment can result in an adrenal crisis.1 ### Information about the current guideline The Paediatric Adrenal Insufficiency Group comprising a multidisciplinary team of paediatric endocrinologists, endocrine nurse specialists and a pharmacist was established with the support of the British Society of Paediatric Endocrinology and Diabetes (BSPED) to standardise the management of paediatric AI.2 This guideline published in November 2022 focuses on management of AI in children during an acute illness with oral sick day dosing, intramuscular and intravenous regimens in emergencies and management during the perioperative period. ### Previous guideline and current practice Prior to this guideline, there were no unified guidelines for managing paediatric AI leading to substantial variations in treatment. National Institute for Health and Care Excellence (NICE) 2020 guidance on Addison’s disease recommends the paediatric endocrinology team provide emergency management for adrenal crisis with the same age-based …