Immediate Treatment for Congenital Adrenal Hyperplasia (CAH) in Crisis
Administer hydrocortisone 100 mg IV or IM immediately upon suspicion of adrenal crisis, followed by aggressive fluid resuscitation with 0.9% saline at 1 liter over the first hour—treatment must never be delayed for diagnostic procedures. 1
Emergency Management Protocol
Immediate Actions (First Hour)
Administer hydrocortisone 100 mg IV bolus immediately without waiting for laboratory confirmation, as this dose provides both glucocorticoid and mineralocorticoid effects by saturating 11β-hydroxysteroid dehydrogenase type 2 1, 2, 3
Initiate rapid fluid resuscitation with 0.9% isotonic saline at 1 L/hour in the first hour to address severe dehydration and hypotension 1, 3
Draw blood samples for cortisol, ACTH, electrolytes, creatinine, urea, and glucose before treatment if possible, but never delay hydrocortisone administration waiting for results 1, 4
Continuing Management (Hours 1-24)
Continue hydrocortisone 100 mg IV or IM every 6-8 hours (total 100-300 mg/day) until clinical recovery 1, 2
- Alternative: continuous IV infusion of 200 mg hydrocortisone over 24 hours preceded by 50-100 mg bolus may better maintain physiologic cortisol levels 5
Administer 3-4 liters of 0.9% saline over 24-48 hours with frequent hemodynamic monitoring to avoid fluid overload 2, 3, 4
Do NOT add separate mineralocorticoid (fludrocortisone) during acute crisis, as high-dose hydrocortisone provides adequate mineralocorticoid activity 2, 4
Pediatric Modifications
Administer initial normal saline bolus of 10-20 mL/kg (maximum 1,000 mL) in children with hypotension 4
Use hydrocortisone 100 mg IV/IM (same dose as adults) for stress-dose coverage in children with vasopressor-resistant hypotension 4
Critical Clinical Pitfalls
Common Errors to Avoid
Never delay treatment for diagnostic confirmation—mortality increases with delayed intervention, and diagnosis can be established later even after treatment has commenced 1, 4
Do not rely on hyperkalemia to confirm diagnosis—it is present in only 50% of cases, while hyponatremia occurs in 90% but its absence should not prevent treatment 4
Recognize that even mild gastrointestinal upset can precipitate crisis in CAH patients who cannot absorb oral medications when they need them most 4
Precipitating Factors to Address
Identify and treat the underlying trigger (most commonly gastrointestinal illness with vomiting/diarrhea, infections, surgical procedures without adequate steroid coverage, or physical trauma) 3, 4
Provide antibiotic therapy if infection is suspected as part of treating the precipitating cause 3
Transition to Maintenance Therapy
Recovery Phase (Days 1-3)
Taper parenteral glucocorticoids over 1-3 days to oral therapy once the patient is stabilized and can tolerate oral medications 3, 4
Transition to maintenance hydrocortisone 15-25 mg daily divided into 2-3 doses (children: 6-10 mg/m² body surface area) 1, 4
Add fludrocortisone 50-200 µg daily as a single morning dose for mineralocorticoid replacement once oral intake is established 1
Supportive Care Considerations
Consider ICU or high-dependency unit admission for severe cases with persistent hypotension or end-organ dysfunction 3, 4
Provide gastric stress ulcer prophylaxis and consider low-dose heparin depending on severity of intercurrent illness 3, 4
Monitor serum electrolytes frequently to guide fluid management and avoid complications 2, 4
Prevention of Future Crises
Ensure patient has emergency supplies of injectable hydrocortisone for self-administration during severe illness 1, 2, 3
Provide medical alert identification jewelry and steroid emergency card to trigger appropriate treatment by emergency personnel 1, 2, 3
Educate on stress dosing: double or triple oral glucocorticoid dose during minor illness with fever; use parenteral hydrocortisone during severe illness or inability to take oral medications 1, 3, 4
Arrange early endocrinology consultation for ongoing management and education on emergency protocols 4