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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).


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Literature review current through: Oct 2015. | This topic last updated: Oct 4, 2013.
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  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.