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Treatment of adrenal insufficiency in children

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

INTRODUCTION

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). These patients may present with glucocorticoid deficiency, with or without deficiencies of mineralocorticoids and adrenal androgens.

Central adrenal insufficiency is caused by impaired production of adrenocorticotropic hormone (ACTH). It can be caused by either pituitary disease that impairs release of adrenocorticotropic hormone (ACTH; secondary adrenal insufficiency (table 2A)); or by interference with corticotropin-releasing hormone (CRH) release from the hypothalamus (tertiary adrenal insufficiency (table 2B)). These patients have only glucocorticoid deficiency and not mineralocorticoid deficiency. In addition, they may have other pituitary hormone deficiencies, depending on the underlying central nervous system disease.  

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 3). 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".)

               
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Literature review current through: Nov 2017. | This topic last updated: Nov 30, 2017.
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