Etiology, diagnosis and management of adrenal insufficiency in children

Abstract

Adrenal insufficiency(AI) is a disorder that can result from primary adrenal failure(primary type) or secondary adrenal disease due to impairment of the hypothalamic–pituitary axis(central type). It is characterized by glucocorticoid deficiency, with or without inappropriate mineralocorticoid or androgen production.The diagnosis and management of children with AI remain challenging as clinical characteristics of AI in children maybe non-specific and acute adrenal crisis is life-threatening, needing life-long glucocorticoid replacement treatment and education of the patient and the family.New formulations of hydrocortisone which should be able to simulate the circadian rhythm of steroid secretion are supposed to improve outcome in patients with adrenal insufficiency in the near future. Key words: Adrenal insufficiency; Adrenal crisis; Glucocorticoid; Mineralocorticoid