Management of Congenital Adrenal Hyperplasia

Diagnostic Approach

For bilateral adrenal incidentalomas, guidelines recommend collecting 17-hydroxyprogesterone levels to rule out congenital adrenal hyperplasia, as suggested by The Journal of Urology 1, 2

The Endocrine Society recommends hydrocortisone dosing of 15-25 mg/day in divided doses for patients with Congenital Adrenal Hyperplasia 3
Standard regimen options for hydrocortisone include three daily doses (7:00 AM, 12:00 PM, 4:00 PM): 10+5+2.5 mg or 15+5+5 mg or 10+5+5 mg, or two daily doses (7:00 AM, 12:00 PM): 15+5 mg or 10+10 mg, as recommended by the Journal of Internal Medicine 3
Alternative glucocorticoids, such as prednisolone, can be used at a dose of 4-5 mg/day, typically as a single morning dose (7:00 AM) or divided (3 mg at 7:00 AM + 1-2 mg at 2:00 PM), as suggested by the Journal of Internal Medicine 3
Dexamethasone should be avoided for routine treatment of CAH, according to the Journal of Internal Medicine 3
Cortisone acetate can be used at a dose of 25-37.5 mg/day in divided doses, as recommended by the Journal of Internal Medicine 3

Fludrocortisone can be used at a dose of 50-200 μg daily, usually taken as a single dose upon awakening, as suggested by the Journal of Internal Medicine 3
Higher doses (up to 500 μg daily) of fludrocortisone may be needed in children, younger adults, or during pregnancy, according to the Journal of Internal Medicine 3

Assessment parameters for monitoring treatment include clinical symptoms (salt cravings, lightheadedness, blood pressure), as recommended by the Journal of Internal Medicine 3

Medication interactions, such as increased dose with anti-epilepsy drugs, barbiturates, antituberculosis drugs, etomidate, topiramate, and decreased dose with grapefruit juice, licorice, should be considered when treating patients with CAH, as suggested by the Journal of Internal Medicine 3
Patients should eat sodium salt and salty foods without restriction and avoid potassium-containing salts, according to the Journal of Internal Medicine 3