(Pediatrics in Review. 1997;18:59-62.)
© 1997 American Academy of Pediatrics
Consultation with the Specialist: Treatment of Adrenocortical Insufficiency
Gilbert P. August, MD*
*
Chairman, Department of Endocrinology &
Metabolism, Children's National Medical
Center; Professor of Pediatrics, George
Washington University School of Medicine,
Washington, DC.
The adrenal cortex secretes three types
of hormones: mineralocorticoids,
glucocorticoids, and androgens. Acute or
chronic deficiencies of aldosterone, the
primary mineralocorticoid, or of
cortisol (hydrocortisone), the primary
glucocorticoid, can rapidly become
life-threatening.
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Etiology
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Adrenocortical insufficiency can be
primary or secondary (Table 1
).
The most common causes of adrenocortical
insufficiency today are congenital
adrenal hyperplasia in the newborn
and failure to provide adequate stress
doses of glucocorticoids to patients
receiving long-term treatment with
these agents.
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Table 1. Etiology of Adrenocortical Insufficiency
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Symptoms
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The symptoms of adrenocortical
insufficiency depend on whether cortisol or
both cortisol and aldosterone are
deficient (Table 2
).
Pure aldosterone
deficiency is rare, but does occur in
children who have a defect in the enzyme
complex corticosterone methyl
oxidase. Pseudohypoaldosteronism
produces similar symptoms but
presumably is due to a defect in the
aldosterone receptor. Hyporeninemic
hypoaldosteronism is even rarer.
Patients who have acquired
immunodeficiency syndrome (AIDS) can
develop a mineralocorticoid deficiency
without concurrent cortisol deficiency.
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Table 2. Signs and Symptoms of Adrenocortical Insufficiency
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Hyperpigmentation, a sign of
cortisol deficiency, develops over time and
is seen most easily on the backs of the
hands, elbows, and knees; in the
creases of the hands; and on . . . [Full Text of this Article]
Copyright © 1997 by the American Academy of Pediatrics.
