Adrenal Hypoplasia Treatment & Management

Updated: Nov 04, 2016
  • Author: Thomas A Wilson, MD; Chief Editor: Robert P Hoffman, MD  more...
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Treatment

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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