Immediate Treatment for Congenital Adrenal Hyperplasia (CAH) in Crisis

Emergency Management Protocol

The American College of Internal Medicine recommends administering 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, as this dose provides both glucocorticoid and mineralocorticoid effects by saturating 11β-hydroxysteroid dehydrogenase type 2 1, 2, 3
Administer hydrocortisone 100 mg IV bolus immediately without waiting for laboratory confirmation, as high-dose hydrocortisone provides adequate mineralocorticoid activity 1, 4, 5
Initiate rapid fluid resuscitation with 0.9% isotonic saline at 1 L/hour in the first hour to address severe dehydration and hypotension 1, 2, 5
Draw blood samples for cortisol, ACTH, electrolytes, creatinine, urea, and glucose before treatment if possible, but never delay hydrocortisone administration waiting for results 1, 3, 6

Never delay treatment for diagnostic confirmation—mortality increases with delayed intervention, and diagnosis can be established later even after treatment has commenced 1, 3, 6
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 6

Taper parenteral glucocorticoids over 1-3 days to oral therapy once the patient is stabilized and can tolerate oral medications 5, 6
Transition to maintenance hydrocortisone 15-25 mg daily divided into 2-3 doses (children: 6-10 mg/m² body surface area) 1, 2, 6
Add fludrocortisone 50-200 µg daily as a single morning dose for mineralocorticoid replacement once oral intake is established 1, 2

Ensure patient has emergency supplies of injectable hydrocortisone for self-administration during severe illness 1, 4, 5
Provide medical alert identification jewelry and steroid emergency card to trigger appropriate treatment by emergency personnel 1, 4, 5
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, 5, 6