Stress Dosing of Steroids in Congenital Adrenal Hyperplasia
For patients with congenital adrenal hyperplasia requiring stress dosing, administer hydrocortisone 100 mg IV immediately for adults (or 50-100 mg/m² for children) during major physiological stress, followed by 200 mg/24h continuous infusion or divided doses every 6 hours, then taper to double the maintenance dose for 24-48 hours once stable. 1, 2
Acute Crisis Management
When adrenal crisis is suspected or confirmed:
Adults:
- Immediate hydrocortisone 100 mg IV bolus
- Follow with continuous IV infusion of 200 mg/24h OR 50 mg IV/IM every 6 hours
- Aggressive fluid resuscitation with 1000 mL isotonic saline in first hour
- Continue stress dosing until patient is stable and tolerating oral intake 1, 2
Children (weight-based):
- <15 kg (0-2 years): 25 mg hydrocortisone IV/IM
- 15-25 kg (2-6 years): 50 mg hydrocortisone IV/IM
25 kg (>6 years): 100 mg hydrocortisone IV/IM
- Maintenance: 50-100 mg/m²/24h divided every 6 hours OR continuous infusion at 2-3 mg/m²/h
- Fluid bolus: 10-20 mL/kg 0.9% saline, repeat as needed 2
Critical point: Fludrocortisone is NOT required acutely when high-dose hydrocortisone is given, as hydrocortisone provides sufficient mineralocorticoid activity at stress doses 2.
Surgical Stress Dosing
The approach varies by surgical magnitude:
Major Surgery (general or regional anesthesia):
- Preoperative: Hydrocortisone 100 mg IV at induction
- Intraoperative/Postoperative: Continuous infusion 200 mg/24h OR 50 mg IV/IM every 6 hours
- Once tolerating oral intake: Double maintenance dose for 48 hours to 1 week depending on recovery
- Example: If usual dose is 10-5-5 mg, give 20-10-10 mg for up to one week postoperatively 3
Minor Surgery/Dental Procedures:
- Extra morning dose 1 hour before (typically 10 mg hydrocortisone)
- Double oral dose for 24 hours post-procedure
- Return to normal maintenance dose 2, 4
Labor and Vaginal Delivery:
- Hydrocortisone 100 mg IV at onset of labor
- Repeat every 6 hours if needed during prolonged labor
- Double oral dose for 24-48 hours postpartum 2, 4
Illness-Related Stress Dosing
The 2010 Endocrine Society guideline provides clear thresholds 5:
Triple the daily dose when:
- Fever ≥39°C (102.2°F)
- Gastroenteritis with vomiting/diarrhea preventing oral intake
- Major trauma
Double the daily dose when:
- Fever 38-38.9°C (100.4-102°F)
- Moderate illness requiring bed rest
Continue for 2-3 days or until recovery
Do NOT increase doses for:
- Mental/emotional stress
- Anxiety or examinations
- Minor illness without fever
- Physical exercise (even strenuous) 5
Critical Pitfalls to Avoid
Underdosing is more dangerous than overdosing - The evidence shows adrenal crises occur in 6-8 per 100 patient-years, with mortality risk. Short-term high-dose glucocorticoids cause no long-term harm 3.
Vomiting requires immediate parenteral administration - If the patient cannot retain oral medication, give IM hydrocortisone immediately (100 mg adults, 50 mg/m² children) and seek medical attention 1, 5.
Hypoglycemia risk in children - Children with CAH are at particular risk for hypoglycemia during illness. Check glucose hourly; if <3.5 mmol/L, give 2-5 mL/kg of 10% dextrose 2. Recent data shows adrenal crisis with probable hypoglycemia occurred in children ages 1.1-11.3 years 6.
Medication errors during hospitalization - 8.6% of patients report previous adrenal crisis due to insufficient glucocorticoid medication during hospital stays, often from dosing errors or omissions 3. Ensure stress dosing is clearly documented and not interrupted.
Patient education is paramount - Studies show only 34.8% of adults perform stress dosing according to recommendations, compared to 72.3% of children, highlighting the critical need for repeated education especially during transition to adult care 7.
Monitoring During Stress Dosing
- Blood pressure and cardiac function continuously
- Sodium and potassium levels (expect hyponatremia and hyperkalemia to correct)
- Blood glucose, especially in children
- Clinical improvement should be evident within hours of appropriate dosing 2, 1, 2
Tapering Back to Maintenance
Once the patient is clinically stable, eating/drinking normally, and the acute stressor has resolved:
- Reduce from stress dose to double maintenance for 24-48 hours
- Then return to regular maintenance dosing
- For major surgery, may need doubled doses for up to one week 3
All patients should carry emergency hydrocortisone injection kits and wear medical alert identification 1, 5.