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Causes and clinical manifestations of central 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 results from disease intrinsic to the adrenal cortex. These patients may present with deficiencies of one or more of the classes of hormones produced in the adrenal cortex (glucocorticoids, mineralocorticoids, and androgens).

Central adrenal insufficiency can be 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 1)); or by interference with corticotropin-releasing hormone (CRH) production from the hypothalamus (tertiary adrenal insufficiency (table 2)). 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.  

The causes and clinical manifestations of central adrenal insufficiency in children will be reviewed here. The causes of primary adrenal insufficiency and its manifestations, diagnosis, and treatment in children are discussed elsewhere. (See "Causes and clinical manifestations of primary adrenal insufficiency in children" and "Diagnosis of adrenal insufficiency in children" and "Treatment of adrenal insufficiency in children".)

CLINICAL MANIFESTATIONS

The signs and symptoms of central adrenal insufficiency are caused by glucocorticoid deficiency, often combined with symptoms caused by underlying central nervous system (CNS) disease including other pituitary hormone deficiencies:

               
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Literature review current through: Oct 2017. | This topic last updated: Nov 01, 2017.
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