Pediatric Adrenal Insufficiency (Addison Disease) Medication

  • Author: Phyllis W Speiser, MD; Chief Editor: Stephen Kemp, MD, PhD   more...
 
Updated: Jun 10, 2011
 

Medication Summary

Glucocorticoid replacement is required in all forms of adrenal insufficiency (Addison disease). Mineralocorticoid replacement is required only in primary adrenal insufficiency (Addison disease), 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).

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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).

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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).

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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.

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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.

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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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Contributor Information and Disclosures
Author

Phyllis W Speiser, MD  Chief, Division of Pediatric Endocrinology, Steven and Alexandra Cohen Children's Medical Center of New York; Professor of Pediatrics, Hofstra-North Shore LIJ School of Medicine at Hofstra University

Phyllis W Speiser, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Thomas A Wilson, MD  Professor of Clinical Pediatrics, Chief and Program Director, Division of Pediatric Endocrinology, Department of Pediatrics, The School of Medicine at Stony Brook University Medical Center

Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Karl S Roth, MD  Professor and Chair, Department of Pediatrics, Creighton University School of Medicine

Karl S Roth, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Nutrition, American Pediatric Society, American Society for Clinical Nutrition, American Society of Nephrology, Association of American Medical Colleges, Medical Society of Virginia, New York Academy of Sciences, Sigma Xi, Society for Pediatric Research, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Barry B Bercu, MD  Professor, Departments of Pediatrics, Molecular Pharmacology and Physiology, University of South Florida College of Medicine, All Children's Hospital

Barry B Bercu, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Federation for Clinical Research, American Medical Association, American Pediatric Society, Association of Clinical Scientists, Endocrine Society, Florida Medical Association, Lawson-Wilkins Pediatric Endocrine Society, Pituitary Society, Society for Pediatric Research, Society for the Study of Reproduction, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD  Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas College of Medicine and Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

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Regulation of the adrenal cortex. ACTH = adrenocorticotropic hormone; CRF = corticotropin-releasing factor; neg. = negative.
Left photograph shows hyperpigmentation on the dorsum of a patient's hand before the treatment of primary adrenal insufficiency. Right photograph shows normal pigmentation after treatment.
Left photograph shows a patient with Addison disease who has prominent pigmentation in areas not exposed to the sun, such as the palmar creases. Right photograph shows normal pigmentation after treatment.
Left photograph shows vitiligo in a patient with autoimmune adrenalitis. Right photograph shows an area of hyperpigmentation surrounding the vitiligo.
Left photomicrograph shows autoimmune adrenalitis. Right photomicrograph shows tuberculous adrenalitis. Note the caseous granuloma.
Computed tomography scan shows enlarged adrenal glands in a patient with early active autoimmune adrenalitis. Patients with chronic disease present with the opposite picture of hypotrophic adrenals.
 
 
 
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