Allgrove (AAA) Syndrome Medication
- Author: Robert J Ferry, Jr, MD; Chief Editor: Stephen Kemp, MD, PhD more...
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 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 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 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 provides physiologic replacement of mineralocorticoid deficiency. The dose must be sufficient to lower plasma renin activity to normal without inducing hypertension.
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