Table 15 Recommendations for the management of AS in children[91]

1. Stress steroids during periods of physiological stress

Adrenal crisis/critical illness*:

Hydrocortisone injection (Solu-Cortef) 100 mg/m² (max. 100 mg) IV/IM stat with saline volume expansion, followed by 25 mg/m² q 6 hours (max. 25 mg q 6 hours); call endocrinologist on call

Surgery*:

Hydrocortisone injection (Solu-Cortef) 50–100 mg/m² IV (max 100 mg) pre-operatively, then 25 mg/m² q 6 hours (max 25 mg q 6 hours); call endocrinologist on call

Illness or fever:

20 mg/m²/day hydrocortisone equivalent, divided BID or TID

Fever >38.5°C or vomiting:

30 mg/m²/day hydrocortisone equivalent, divided TID

Unable to tolerate orally:

Hydrocortisone must be administered parenterally as Solu-Cortef, 25 mg/m²/dose q 6 hours IV or q 8 hours IM

2. ± Daily physiologic dose of hydrocortisone (8–10 mg/m ² /day)

3. Patient/family education

 – Stress steroid dosing

 – Emergency medical contact information in case of illness

4. Information card*

5. Consider medical identification tag