Management of Acute Adrenal Crisis in Congenital Adrenal Hyperplasia
The correct answer is B: IV hydrocortisone and isotonic normal saline. This baby is in acute adrenal crisis requiring immediate emergency resuscitation with intravenous therapy, not oral medications.
Immediate Emergency Treatment Protocol
Administer IV hydrocortisone 100 mg immediately as a bolus, followed by isotonic normal saline (0.9% NaCl) infusion to correct hypovolemia and electrolyte disturbances. 1, 2
Why IV Hydrocortisone Plus Saline is Critical
The high-dose IV hydrocortisone serves dual purposes: it provides glucocorticoid replacement to correct hypoglycemia AND saturates type 2 hydroxysteroid dehydrogenase to achieve mineralocorticoid effects, which addresses both the hyperkalemia and hyponatremia simultaneously 1
Isotonic saline (0.9% NaCl) should be infused rapidly (1 liter over the first hour in older children, weight-adjusted for infants) to correct hypovolemia and hyponatremia 1
Never delay treatment for diagnostic procedures—draw blood for cortisol and ACTH levels, then treat immediately without waiting for results 1, 2
Why the Other Options Are Incorrect
Option A (Oral Hydrocortisone and Fludrocortisone) is Wrong
Oral medications are inappropriate during acute adrenal crisis because the infant may have impaired gastrointestinal absorption due to shock, vomiting, or hemodynamic instability 1
Oral therapy is reserved for maintenance treatment AFTER stabilization, typically initiated 1-3 days after the acute crisis resolves when the infant can tolerate enteral intake 1, 3
Fludrocortisone is not needed acutely because high-dose IV hydrocortisone provides sufficient mineralocorticoid activity during the crisis phase 1
Option C (Sodium Bicarbonate and Insulin) is Wrong
This treats hyperkalemia symptomatically without addressing the underlying adrenal insufficiency, which is the root cause of all three electrolyte abnormalities 1, 2
Treating hyperkalemia with insulin could worsen the hypoglycemia, creating a dangerous situation in an already critically ill infant 1
Sodium bicarbonate does not address the hypovolemia or cortisol deficiency that are life-threatening in this scenario 2
Monitoring During Acute Phase
Check serum electrolytes (sodium, potassium) every 4-6 hours until normalized to guide ongoing fluid and electrolyte management 1
Monitor blood glucose frequently to ensure hypoglycemia resolves with hydrocortisone and dextrose-containing fluids if needed 1
Perform hemodynamic monitoring with frequent blood pressure checks to assess response to volume resuscitation 1
Avoid rapid correction of hyponatremia (>10-15 mmol/L per 24 hours) to prevent cerebral edema, even though the infant is hyponatremic 1
Transition to Maintenance Therapy
Once stabilized (typically 1-3 days), taper to oral hydrocortisone 6-10 mg/m² body surface area daily in divided doses 1, 3
Initiate fludrocortisone 50-200 µg daily as a single morning dose for long-term mineralocorticoid replacement 1, 3
All infants with classical CAH require lifelong glucocorticoid and mineralocorticoid replacement with regular monitoring of growth, blood pressure, and electrolytes 1, 4
Critical Pitfall to Avoid
The most dangerous error is choosing oral medications (Option A) when the infant is in acute crisis. This delay in appropriate IV therapy can result in cardiovascular collapse and death. The triad of hypoglycemia, hyponatremia, and hyperkalemia in a baby with CAH represents a medical emergency requiring immediate intravenous intervention 1, 2