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Adrenal Hypoplasia Treatment & Management

  • Author: Thomas A Wilson, MD; Chief Editor: Stephen Kemp, MD, PhD  more...
 
Updated: Feb 11, 2013
 

Medical Care

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  • Patients with adrenal hypoplasia are generally hypovolemic and may be hypoglycemic; therefore, initial therapy should consist of intravenous normal saline and dextrose.
  • If hypotensive, a bolus dose of 20 mL/kg of isotonic intravenous fluid over the first hour may be necessary to restore blood pressure. This can be repeated if the blood pressure remains low.
  • Once samples for serum electrolytes, blood sugar, cortisol, 17-hydroxyprogesterone, and adrenocorticotropic hormone (ACTH) concentrations are obtained, treat the patient with glucocorticoids. This therapy is based on suspicion of adrenal insufficiency because it may be life preserving.
  • A cosyntropin stimulation test confirms the diagnosis of adrenocortical insufficiency.
  • Dexamethasone may be given prior to the cosyntropin without interfering with the results of the test because acute administration of dexamethasone does not interfere with the cortisol response or with the cortisol assay. Otherwise, hydrocortisone is preferable because of its mineralocorticoid activity.
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Surgical Care

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  • Surgery is not necessary in the management of congenital adrenal hypoplasia; however, a patient requiring surgery must be covered with stress doses of glucocorticoids during the perioperative period.
  • The following recommendations are empiric rather than evidence-based:
    • Administer 50-75 mg/m2 hydrocortisone intramuscularly or intravenously on call prior to surgery.
    • During the procedure, treat the patient with additional hydrocortisone. This may be accomplished with either a hydrocortisone drip of 2-4 mg/m2/h, or as an additional bolus of 10-25 mg/m2 intravenously every 6 hours throughout the procedure.
    • Continue hydrocortisone in the immediate postoperative period.
    • On the second and third postoperative day, the dose of hydrocortisone can be decreased by 50% each day, to a minimum of the patient's usual daily requirement, provided no complications exist and the patient is recovering well.
    • By the fourth postoperative day, the usual daily dose of steroids may be resumed if the patient is recovering well. If complications occur, stress doses of glucocorticoids must be continued.
    • Fludrocortisone may be held on the day of surgery and while the patient is receiving stress doses of hydrocortisone because this high dose should provide ample mineralocorticoid effect.
    • If the patient is unable to take fludrocortisone by mouth in the postoperative period, stress doses of hydrocortisone may be continued for a longer period to provide adequate mineralocorticoid activity.
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Consultations

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  • Endocrinologist when adrenal insufficiency is suspected
  • Geneticist for genetic diagnosis and counseling
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Diet

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  • Patients should not be on a sodium-restricted or fluid-restricted diet.
  • Patients should have ample access to salt since patients are deficient in aldosterone secretion and, therefore, are generally salt wasters.
  • Monitor and restrict caloric intake if excess weight gain occurs on therapy because glucocorticoids stimulate appetite and weight gain.
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Activity

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  • After appropriate glucocorticoid and mineralocorticoid therapy is instituted, no restrictions on activity are necessary.
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Contributor Information and Disclosures
Author

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, Pediatric Endocrine Society, Phi Beta Kappa

Disclosure: Nothing to disclose.

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.

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