Patients with Addison disease usually present with features of both glucocorticoid and mineralocorticoid deficiency. The predominant symptoms vary depending on the duration of disease. Patients may present with clinical features of chronic Addison disease or in acute addisonian crisis precipitated by stress factors, such as infection, trauma, surgery, vomiting, diarrhea, or noncompliance with replacement steroids.
Idiopathic autoimmune Addison disease tends to be more common in women and children. The most common age at presentation in adults with Addison disease is 30-50 years; however, the disease may present earlier in patients with any of the polyglandular autoimmune syndromes or congenital adrenal hyperplasia, or if onset is due to a disorder of long-chain fatty acid metabolism.
The diagnosis of adrenocortical insufficiency rests on assessment of the functional capacity of the adrenal cortex to synthesize cortisol. This is accomplished primarily by use of the rapid ACTH stimulation test. The 30-minute cortisol value is more sensitive than the 60-minute value because cortisol levels have been shown to decrease in the 60-minute sample. Measurement of aldosterone is optional. However, its measurement may help in distinguishing primary adrenal failure from secondary adrenal failure.
In patients in acute adrenal crisis, intravenous (IV) access should be established urgently, and an infusion of isotonic sodium chloride solution should be begun to restore volume deficit and correct hypotension. Some patients may require glucose supplementation. The precipitating cause should be sought and corrected where possible. Clinical improvement, especially blood pressure response, should be evident within 4-6 hours of hydrocortisone infusion.
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Cite this: Romesh Khardori. Fast Five Quiz: Adrenal Gland Disorders - Medscape - Dec 11, 2019.