Familial Glucocorticoid Deficiency Medication

Updated: Feb 16, 2019
  • Author: Andrea M Haqq, MD, MHS, FRCPC, FAAP; Chief Editor: Sasigarn A Bowden, MD, FAAP  more...
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Medication Summary

For hypoglycemia in neonates, administer intravenous (IV) 10% dextrose at 2.5 mL/kg as a rapid IV bolus followed by a continuous IV infusion of 3-5 mL/kg/h (5-8 mg glucose per kg/min). In children, administer 50% dextrose diluted to 25% in water at an initial dose of 1 mL/kg IV followed by an IV infusion of 10% dextrose at 2-3 mL/kg/h (3-5 mg glucose per kg/min).

If any difficulty in establishing IV access occurs, intramuscularly administer glucagon at 0.03 mg/kg (not to exceed 1 mg). Glucagon therapy has a transient effect and must be followed by an intravenous dextrose infusion as above.

For familial glucocorticoid deficiency, treatment includes the replacement of glucocorticoids in order to avoid not only adrenal crisis but to allow normal growth in these children. Glucocorticoid replacement is achieved with hydrocortisone (12-16 mg/m2/24h PO divided into 3 doses). An equivalent dose of prednisone or dexamethasone may be administered to adults and the occasional patient who has difficulty with compliance. However, the potential for growth suppression with either prednisone or dexamethasone is greater than hydrocortisone; and, therefore, these agents should be used with caution. Administer the lowest dosage necessary to prevent symptoms of adrenal failure in order to avoid suppression of growth.



Class Summary

These are used for physiologic replacement of glucocorticoid deficiency. They elicit anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Hydrocortisone (Hydrocortone, Cortef)

DOC because of mineralocorticoid activity and glucocorticoid effects.

Prednisone (Deltasone, Orasone, Sterapred, Pediapred)

Not first-line drug in children because of concerns of growth suppression. Can be used in cases of severe noncompliance. Replace physiologic dose of hydrocortisone with an equipotent dose of prednisone. Prednisone is 4 times more potent than hydrocortisone.