How is pediatric adrenal insufficiency (Addison disease) diagnosed?

Updated: Mar 10, 2020
  • Author: Kimberly Tafuri, DO; Chief Editor: Sasigarn A Bowden, MD  more...
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Clinical suspicion is important because the presentation of patients with adrenal insufficiency may be insidious and subtle.

A diagnosis of adrenal insufficiency is confirmed if the serum cortisol level is less than 18 mcg/dL in the presence of a markedly elevated serum ACTH concentration and plasma renin activity. Based on normative data of children of various ages, adrenal insufficiency is likely if the serum cortisol concentration is less than 18 mcg/dL 30-60 minutes after administration of 250 mcg of cosyntropin (synthetic ACTH 1-24). [2, 3] These criteria may not apply to premature or low-birth-weight infants, who have low cortisol secretion and, most likely, decreased cortisol binding to carrier proteins. [4] Therefore, the diagnosis of adrenal insufficiency in premature infants remains problematic.

When a patient's serum cortisol response to cosyntropin is subnormal but his or her serum ACTH level is not elevated, the possibility of central adrenal insufficiency should be considered.

Because the adrenal glands may not have had sufficient time to atrophy in the absence of adrenocorticotropic hormone (ACTH) stimulation, the relatively cumbersome and risky insulin-tolerance test or metyrapone stimulation test may be preferable to a cosyntropin challenge if the patient has recent-onset (ie, < 10 d) central adrenal insufficiency (eg, a patient who recently underwent surgery of the hypothalamus or pituitary regions). The insulin-tolerance test is still considered the criterion standard.

Computed tomography (CT) scanning is the imaging study of choice in the evaluation of adrenal insufficiency and helps to identify adrenal hemorrhage, calcifications, and infiltrative disease.

Histologic findings in adrenal insufficiency depend on the underlying cause. CT scan–guided fine-needle aspiration sometimes helps in diagnosing the etiology of infiltrative adrenal disease.

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