Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Diagnosis of adrenal insufficiency in children

Patricia A Donohoue, MD
Section Editor
Joseph I Wolfsdorf, MB, BCh
Deputy Editor
Alison G Hoppin, MD


Adrenal insufficiency is defined by the impaired synthesis and release of adrenocortical hormones. It is classified based upon whether the etiology is primary, secondary, or tertiary:

Primary adrenal insufficiency, also known as Addison's disease, results from disease intrinsic to the adrenal cortex (table 1).

Secondary adrenal insufficiency is caused by either impaired release or effect of adrenocorticotropic hormone (ACTH) from the pituitary gland (table 2).

Tertiary adrenal insufficiency results from the impaired release or effect of corticotropin releasing factor (CRH) from the hypothalamus (table 3).

In addition, there are disorders of end-organ unresponsiveness to adrenocortical hormones that present in a similar manner as diseases caused by adrenocortical hormone deficiencies. These include glucocorticoid (cortisol) resistance and aldosterone resistance. (See "Causes and clinical manifestations of primary adrenal insufficiency in children", section on 'End-organ unresponsiveness'.)


Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Sep 2016. | This topic last updated: Apr 25, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
  1. Oelkers W. Adrenal insufficiency. N Engl J Med 1996; 335:1206.
  2. Bornstein SR, Allolio B, Arlt W, et al. Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 2016; 101:364.
  3. Donohoue PA. The adrenal gland and its disorders. In: Principles and Practice of Pediatric Endocrinology, Kappy MS, Allen DB, Geffner ME (Eds), Charles C. Thomas Publisher, LTD, Springfield 2005. p.357.
  4. Pacák K, Palkovits M. Stressor specificity of central neuroendocrine responses: implications for stress-related disorders. Endocr Rev 2001; 22:502.
  5. Cetinkaya S, Ozon A, Yordam N. Diagnostic value of salivary cortisol in children with abnormal adrenal cortex functions. Horm Res 2007; 67:301.
  6. Müller J. Aldosterone: the minority hormone of the adrenal cortex. Steroids 1995; 60:2.
  7. Fibiger J. Beitrage zur kenntniss des weiblichen schweinzwittertums. Virchow Arch Path and Anat 1905; 181:1.
  8. Haber E, Koerner T, Page LB, et al. Application of a radioimmunoassay for angiotensin I to the physiologic measurements of plasma renin activity in normal human subjects. J Clin Endocrinol Metab 1969; 29:1349.
  9. Nye EJ, Grice JE, Hockings GI, et al. Comparison of adrenocorticotropin (ACTH) stimulation tests and insulin hypoglycemia in normal humans: low dose, standard high dose, and 8-hour ACTH-(1-24) infusion tests. J Clin Endocrinol Metab 1999; 84:3648.
  10. Streeten DH, Anderson GH Jr, Dalakos TG, et al. Normal and abnormal function of the hypothalamic-pituitary-adrenocortical system in man. Endocr Rev 1984; 5:371.
  11. Vanderschueren-Lodeweyckx M, Wolter R, Malvaux P, et al. The glucagon stimulation test: effect of plasma growth hormone and on immunoreactive insulin, cortisol, and glucose in children. J Pediatr 1974; 85:182.