Adrenal insufficiency: causes and prevention of adrenal crisis

Adrenal insufficiency (AI) is defined as clinically deficient production or action of glucocorticoids (Bornstein, 2009). Cortisol is the major glucocorticoid hormone and is produced by the adrenal cortex. Inability to produce sufficient amounts of cortisol is associated with increased morbidity and mortality (Zaloga &Marik, 2001). AI is a lifethreatening disorder resulting from disruption of the hypothalamic–pituitary–adrenal (HPA) axis. Depending on which part of this pathway is affected, the disorder is classified as primary or secondary. AI can be congenital or acquired and a lifelong or temporary diagnosis. Appropriate care must be taken to assess for signs and symptoms of acute AI and prevent acute adrenal crisis that can be fatal. Health care providers must educate patients with known AI to prevent untimely deaths from adrenal crisis. Both cortisol and aldosterone are produced in the adrenal cortex and regulate normal physiologic functions, such as blood pressure, glucose levels, and sodium and fluid balance. Symptoms of adrenocortical insufficiency include weakness, fatigue, anorexia, abdominal pain, orthostatic hypotension, salt cravings, and darkening of the skin (Bornstein, 2009). Secondary AI results when the hypothalamus or pituitary gland is affected, disrupting the HPA axis. Primary adrenal insufficiency (PAI) is rare in the western population and estimated to be around 90–140 per million (Arlt & Allolio, 2003). The most common cause of PAI is the endocrine disorder congenital adrenal hyperplasia (CAH). This disease is an autosomal recessive disorder of cortisol biosynthesis with or without aldosterone deficiency and has an estimated incidence of 1 in 15,000 (Merke & Bornstein