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Primary Adrenal Insufficiency

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2 Key Points Î Primary adrenal insufficiency (PAI) was first described in 1855 by Thomas Addison and is therefore commonly termed Addison's disease. Î PAI is defined by the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and/or mineralocorticoids. Î PAI is a severe and potentially life-threatening condition due to the central role of these hormones in energy, salt, and fluid homeostasis. Î Cortisol deficiency results in a decrease in feedback to the hypothalamic-pituitary axis and subsequent enhanced stimulation of the adrenal cortex by elevated levels of plasma ACTH. Consequent to disruption of adrenal mineralocorticoid synthesis, renin release by the juxtaglomerular cells of the kidneys increases. This is of clinical, diagnostic, and therapeutic relevance because PAI needs to be distinguished from secondary adrenocortical insufficiency due to insufficient production of ACTH and without impact on the renin- angiotensin-aldosterone system. Who Should Be Tested and How Î The Endocrine Society (ES) recommends diagnostic testing to exclude PAI in acutely ill patients with otherwise unexplained symptoms or signs suggestive of PAI (volume depletion, hypotension, hyponatremia, hyperkalemia, fever, abdominal pain, hyperpigmentation or, especially in children, hypoglycemia). (1|⊕⊕⊕ ) Î ES recommends confirmatory testing with the corticotropin stimulation test in patients with clinical symptoms or signs suggesting PAI when the patient's condition and circumstance allow. (1|⊕⊕⊕⊕) Optimal Diagnostic Tests Î ES suggests the standard dose (250 μg for adults and children ≥2 y of age, 15 μg/kg for infants, and 125 μg for children <2 y of age) IV corticotropin stimulation (30 or 60 min) test over other existing diagnostics tests to establish the diagnosis of adrenal insufficiency. Peak cortisol levels below 500 nmol/L (18 μg/dL) (assay dependent) at 30 or 60 minutes indicate adrenal insufficiency. (2|⊕⊕ ) Î ES suggests the low-dose (1 μg) corticotropin test for diagnosis of PAI only when the substance itself is in short supply. (2|⊕⊕ ) Î If a corticotropin stimulation test is not feasible, ES suggests using a morning cortisol <140 nmol/L (5 μg/dL) in combination with ACTH as a preliminary test suggestive of adrenal insufficiency (until confirmatory testing with corticotropin stimulation is available). (2|⊕ ) Diagnosis

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