Official reprint from UpToDate®
www.uptodate.com ©2015 UpToDate®

Treatment of adrenal insufficiency in children

Patricia A Donohoue, MD
Section Editor
Mitchell Geffner, MD
Deputy Editor
Alison G Hoppin, MD


Adrenal insufficiency is defined by the impaired synthesis and release of adrenocortical hormones. It is classified based upon the mechanism:

  • Primary adrenal insufficiency, also known as Addison's disease, results from disease intrinsic to the adrenal cortex (table 1).
  • Central adrenal insufficiency is caused by impaired production or action of adrenocorticotropic hormone (ACTH). It can be caused by several mechanisms:

  • Secondary adrenal insufficiency results from pituitary disease impairing release of adrenocorticotropic hormone (ACTH). In addition, unresponsiveness of end-organs to adrenocortical hormones is classified as secondary adrenal insufficiency because this presents in a similar manner as diseases caused by adrenocorticotropic hormone (ACTH) deficiencies (table 2).
  • Tertiary adrenal insufficiency results from the impaired release or effect of corticotropin releasing hormone (CRH) from the hypothalamus (table 3).

Clinical findings of children with adrenal insufficiency include fatigue and gastrointestinal complaints of nausea and vomiting. Additional symptoms can be categorized based upon the specific hormones affected (table 4). Signs of mineralocorticoid deficiency (hypotension, dehydration, and electrolyte abnormalities) are often observed in patients with primary adrenal insufficiency.

The most common cause of adrenal insufficiency in infants is classic congenital adrenal hyperplasia, which is discussed separately. (See "Treatment of classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency in infants and children".)


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: Jul 2015. | This topic last updated: Oct 4, 2013.
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 ©2015 UpToDate, Inc.
  1. Kenny FM, Preeyasombat C, Migeon CJ. Cortisol production rate. II. Normal infants, children, and adults. Pediatrics 1966; 37:34.
  2. Linder BL, Esteban NV, Yergey AL, et al. Cortisol production rate in childhood and adolescence. J Pediatr 1990; 117:892.
  3. Oyama T. Influence of general anesthesia and surgical stress on endocrine function. In: Anesthesia and the patient with endocrine disease, Brown BR (Ed), F.A. Davis Company, Philadelphia 1980. p.173.
  4. Joint LWPES/ESPE CAH Working Group.. Consensus statement on 21-hydroxylase deficiency from the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology. J Clin Endocrinol Metab 2002; 87:4048.
  5. Donohoue PA. Adrenal disorders. In: Pediatric Practice Endocrinology, Kappy MS, Allen DB, Geffner ME. (Eds), McGraw Hill Medical, New York 2010. p.132-190.