Treatment of adrenal insufficiency in children
- Patricia A Donohoue, MD
Patricia A Donohoue, MD
- Professor of Pediatrics
- Medical College of Wisconsin
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).
Subscribers log in hereLiterature review current through: Sep 2017. | This topic last updated: Mar 29, 2016.References
- Kenny FM, Preeyasombat C, Migeon CJ. Cortisol production rate. II. Normal infants, children, and adults. Pediatrics 1966; 37:34.
- Linder BL, Esteban NV, Yergey AL, et al. Cortisol production rate in childhood and adolescence. J Pediatr 1990; 117:892.
- 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.
- Rivkees SA, Crawford JD. Dexamethasone treatment of virilizing congenital adrenal hyperplasia: the ability to achieve normal growth. Pediatrics 2000; 106:767.
- Charmandari E, Johnston A, Brook CG, Hindmarsh PC. Bioavailability of oral hydrocortisone in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J Endocrinol 2001; 169:65.
- 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.
- 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.
- Kim MS, Donohoue PA. Adrenal disorders. In: Pediatric Practice: Endocrinology, 2nd ed, Kappy MS, Allen DB, Geffner ME (Eds), McGraw-Hill Medical, New York 2014. p.169.