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Allgrove (AAA) Syndrome Medication

  • Author: Robert J Ferry, Jr, MD; Chief Editor: Stephen Kemp, MD, PhD  more...
 
Updated: Dec 18, 2015
 
 

Corticosteroids

Class Summary

Careful replacement of glucocorticoids in patients with known adrenal insufficiency is critical to avoid adrenal crisis and to allow for normal growth in children. Growth must be monitored closely, as overtreatment with glucocorticoids impairs linear growth.

Providing stress doses of corticosteroids during illness or injury is another important feature of medical management. Typically, a doubling or tripling of the oral dose is sufficient for routine illnesses. A larger increase in dose (provided IV if necessary) is required for severe illness and major trauma (see Adrenal Insufficiency).

Hydrocortisone (Hydrocortone, Cortef)

 

Hydrocortisone is preferred owing to its balanced (1:1) mineralocorticoid and glucocorticoid effects. It is useful in the management of inflammation caused by an immune response. The patient may still require daily supplementation with fludrocortisone to provide adequate mineralocorticoid activity.

Prednisone (Deltasone)

 

Prednisone is not preferred in children because of its potential for growth-suppressive effects with greater potency and a longer duration of action compared with hydrocortisone. It is an immunosuppressant for the treatment of autoimmune disorders; it may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Prednisone stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.

In patients who have difficulty complying, it is acceptable to replace hydrocortisone with an equipotent dose of prednisone (prednisone is 4-5 times as potent as hydrocortisone). Doses can be adjusted based on symptoms and the monitoring linear growth and weight gain.

Dexamethasone (Decadron)

 

Dexamethasone is not preferred in children because of its potential for growth-suppressive effects with greater potency and a longer duration of action compared with hydrocortisone. It decreases inflammation by suppressing the migration of PMN leukocytes and reducing capillary permeability. Dexamethasone is the least preferred for maintenance or stress dosing because of its lack of mineralocorticoid activity.

Fludrocortisone (Florinef)

 

Fludrocortisone provides physiologic replacement of mineralocorticoid deficiency. The dose must be sufficient to lower plasma renin activity to normal without inducing hypertension.

 
 
Contributor Information and Disclosures
Author

Robert J Ferry, Jr, MD Professor, Division of Pediatric Endocrinology, Department of Pediatrics, University of Tennessee Health Science Center

Robert J Ferry, Jr, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research

Disclosure: Received research funds for: Eli Lilly & Co.

Specialty Editor Board

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 Medical Association, American Pediatric Society, Association of Clinical Scientists, Endocrine Society, Florida Medical Association, Pediatric Endocrine Society, Society for Pediatric Research, Southern Society for Pediatric Research, Society for the Study of Reproduction, American Federation for Clinical Research, Pituitary Society

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD Former Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas for Medical Sciences College of Medicine, 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, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

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, Pediatric Endocrine Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

Jacalyn Bishop, MD Pediatric Endocrinologist, Private Practice

Disclosure: Nothing to disclose.

Bruce A Boston, MD Chief, Division of Pediatric Endocrinology, Director, Pediatric Endocrine Training Program, Doernbecher Children's Hospital; Professor, Department of Pediatrics, Division of Pediatric Endocrinology, Oregon Health and Science University School of Medicine

Bruce A Boston, MD is a member of the following medical societies: Alpha Omega Alpha, American Diabetes Association, Endocrine Society, and Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Daniel L Marks, MD, PhD Assistant Professor, Department of Pediatrics, Division of Pediatric Endocrinology, Oregon Health Sciences University and Doerenbecher Children's Hospital

Daniel L Marks, MD, PhD is a member of the following medical societies: American Academy of Pediatrics and Oregon Medical Association

Disclosure: Nothing to disclose.

References
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