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Background
Adrenal insufficiency is a rare disease caused by either primary adrenal failure (Addison disease) or by impairment of the hypothalamic-pituitary-adrenal axis. Steroid replacement therapy normalises quality of life, however, adherence can be problematic.
Objective
This article provides information on adrenal insufficiency focusing on awareness of initial symptoms and on risk scenarios, emergency management and baseline investigations, complete investigations and long term management.
Discussion
Early recognition of adrenal insufficiency is essential to avoid associated morbidity and mortality. Initial diagnosis and decision to treat are based on history and physical examination. Appropriate management includes emergency resuscitation and steroid administration. Initial investigations can include sodium, potassium and blood glucose levels. However, complete investigations can be deferred. Specialist advice should be obtained and long term management includes a Team Care Arrangement. For patients, an emergency plan and emergency identification are essential.
Keywords: Addison disease; diagnosis, differential; fludrocortisone; hydrocortisone; patient care planning
Primary adrenal insufficiency was first described by the English physician Thomas Addison in 1855.1 Addison disease is a rare condition with an estimated prevalence of 4-11 per 100 000 and an incidence of 0.8 per 100 000 population/year.2,3 In children, boys constitute approximately 75% of patients in contrast to adults, where the majority (70%) are women.4 The underlying pathology is a destruction of the cortex of the adrenal gland.5 Secretion of cortisol is reduced or absent, with or without associated reduction or absence of aldosterone. Adrenocorticotrophic hormone (ACTH) levels are increased.2
Historically, tuberculosis was the most common cause of Addison disease.4,5 Today however, 70-80% of all cases of Addison disease in the Western world are due to autoimmune adrenalitis;4,6 whereas worldwide infectious diseases - such as tuberculosis,7 fungal infections (histoplasmosis, cryptococcus), and cytomegalovirus - are still important causes. Acute haemorrhage (ie. in meningococcal septicaemia) although uncommon, is also a cause of primary adrenal insufficiency.5
Overall, Addison disease in the paediatric population is most commonly attributed to primary adrenal insufficiency, which occurs in approximately one in 15 000 births and usually presents in the neonatal period or early childhood.8 Ninety-five percent of cases are due to deficiency of an enzyme (21 hydroxylase) involved in the steroidogenesis pathway, which also results in impaired aldosterone synthesis. In contrast to Addison disease, the adrenal cortex is not destroyed and hence...