Pediatric Adrenal Insufficiency (Addison Disease) Medication

Updated: Feb 16, 2017
  • Author: Phyllis W Speiser, MD; Chief Editor: Sasigarn A Bowden, MD  more...
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Medication

Medication Summary

Glucocorticoid replacement is required in all forms of adrenal insufficiency (Addison disease). Mineralocorticoid replacement is required only in primary adrenal insufficiency, because aldosterone secretion is reduced in primary adrenal insufficiency but not in secondary (central) adrenal insufficiency. In acute adrenal crisis (eg, hypotension, hypoglycemia) use pharmacologic doses of glucocorticoids, which can be in the form of hydrocortisone, methylprednisolone, or dexamethasone.

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Mineralocorticoids

Class Summary

Mineralocorticoids are used as replacement therapy in aldosterone deficiency and as prophylaxis against hyponatremia and hyperkalemia in patients with primary adrenal insufficiency (Addison disease).

Fludrocortisone

Fludrocortisone is the drug of choice (DOC) for mineralocorticoid replacement therapy if the zona glomerulosa of the adrenal cortex does not produce aldosterone. This agent allows patients to achieve normal sodium homeostasis.

Fludrocortisone is available only in an oral (PO) formulation. If patient cannot tolerate PO, parenteral hydrocortisone can provide a mineralocorticoid effect. Infants may require sodium chloride supplements, because their diets often provide insufficient sodium.

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Glucocorticoids

Class Summary

Glucocorticoid agents give patients with adrenal insufficiency (Addison disease) the equivalent of the body's missing cortisol produced by the adrenal cortex under normal conditions and under stress. Dexamethasone and betamethasone cross the placenta to an appreciable degree; therefore, both agents should not be used in pregnant women unless they are specifically indicated (ie, to aid maturation of the fetal lung or to suppress fetal adrenal function).

Hydrocortisone (Cortef, Solu-Cortef, A-Hydrocort)

Hydrocortisone is the glucocorticoid drug of choice (DOC) because of its mineralocorticoid activity and glucocorticoid effects and its equivalency to the adrenal product (ie, cortisol). This agent has a short half-life; therefore, hydrocortisone does not inhibit growth in children to same degree as more potent, longer-acting synthetic glucocorticoid agents (eg, prednisone, methylprednisolone, dexamethasone). Because of short action, hydrocortisone must be administered orally (PO) twice or thrice daily (bid/tid). When administered intravenously, hydrocortisone is usually given every 6 hours.

In healthy person, mean cortisol secretion is about 5-10 mg/m2/d. The aim of replacement therapy is to supply only as much as needed. The target level is best judged subjectively on basis of patient's own sense of well-being.

Dose requirements are greater PO than parenterally because some hydrocortisone is inactivated as it passes through liver. Equivalent low doses can be derived for prednisone, methylprednisolone, and dexamethasone (which have a minimum of about 4, 5, and 40-50 times the potency of hydrocortisone, respectively).

Dexamethasone (Baycadron)

Dexamethasone provides glucocorticoid activity. At pharmacologic doses, this agent decreases inflammation by suppressing migration of polymorphonuclear leukocytes and by reducing capillary permeability. Dexamethasone and prednisone may be used for allergic and inflammatory conditions.

Methylprednisolone (Medrol, Solu-Medrol, Depo-Medrol)

Methylprednisolone also provides glucocorticoid activity. At pharmacologic doses, this agent decreases inflammation by suppressing migration of polymorphonuclear leukocytes and by reversing increased capillary permeability.

Methylprednisolone is available in a liquid formulation, unlike hydrocortisone.

Prednisone

Prednisone provides glucocorticoid activity. At pharmacologic doses, this agent decreases inflammation by suppressing migration of polymorphonuclear leukocytes and by reversing increased capillary permeability. Like methylprednisolone, prednisone is available in a liquid formulation.

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