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/m2/day)
3. Patient/family education
– Stress steroid dosing
– Emergency medical contact information in case of illness
4. Information card*
5. Consider medical identification tag

IV: intravenous; IM: intramuscular; BID: twice daily; TID: three times daily; QID: four times daily; q: every.

*At a minimum, symptomatic patients require an information card and stress dosing during critical illness and surgery.

Reproduced from Ahmet et al., 2011 [91].