How is congenital adrenal hyperplasia (CAH) managed?

Updated: Oct 06, 2020
  • Author: Thomas A Wilson, MD; Chief Editor: Sasigarn A Bowden, MD  more...
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Answer

Management is as follows:

  • Patients with dehydration, hyponatremia, or hyperkalemia and a possible salt-wasting form of CAH should receive an IV bolus of isotonic sodium chloride solution (20 mL/kg or 450 mL/m2) over the first hour, as needed, to restore intravascular volume and blood pressure; this may be repeated if the blood pressure remains low

  • Dextrose must be administered if the patient is hypoglycemic and must be included in the rehydration fluid after the bolus dose to prevent hypoglycemia

  • After samples are obtained to measure electrolyte, blood sugar, cortisol, aldosterone, and 17-hydroxyprogesterone concentrations, the patient should be treated with glucocorticoids; treatment should not be withheld while confirmatory results are awaited

  • After the patient's condition is stabilized, treat all patients who have adrenal hyperplasia with long-term glucocorticoid or aldosterone replacement (or both), depending on which enzyme is involved and on whether cortisol and/or aldosterone synthesis is affected

  • Patients who are sick and have signs of adrenal insufficiency should receive stress dosages of hydrocortisone (50-100 mg/m2 or 1-2 mg/kg IV administered as an initial dose), followed by 50-100 mg/m2/day IV divided every 6 hours


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