How should stress be treated in patients with congenital adrenal hyperplasia (CAH)?

Updated: Oct 06, 2020
  • Author: Thomas A Wilson, MD; Chief Editor: Sasigarn A Bowden, MD  more...
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Answer

One of the important physiologic responses to stress is an increase in the cortisol production that ACTH mediates. Patients with adrenal insufficiency of any etiology cannot mount this response and must be given stress doses of glucocorticoid. In the patient with a minor illness (temperature of < 38°C), the dosage of hydrocortisone should be at least doubled. For patients with relatively severe illness (temperature of >38°C), the dosage of glucocorticoid should be tripled. If the patient is vomiting or listless, administer parenteral glucocorticoid (50-75 mg/m2 of hydrocortisone IM or IV or an equivalent dosage of methylprednisolone or dexamethasone). Because hydrocortisone succinate has a short duration of action, the dose must be repeated every 6-8 hours at a dosage of 50-100 mg/m2/d until the patient is well.

All patients with adrenal insufficiency must have injectable glucocorticoid available, and the caretaker must be instructed in its use and importance. Glucocorticoid or mineralocorticoid replacement has no contraindications when it is needed, and it has few drug-drug interactions.


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