How is mineralocorticoid deficiency managed in congenital adrenal hyperplasia (CAH)?

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

Administer fludrocortisone (0.05-0.2 mg/d PO) to patients with mineralocorticoid deficiency. Administer NaCl (2-5 g/d) to infants to counteract salt wasting. Older children can usually scavenge adequate salt to provide for their needs and may lose their salt-wasting tendencies as they mature. The dose of glucocorticoid is adjusted by clinically evaluating the patient (for an absence of symptoms of glucocorticoid deficiency and normal growth) and by periodically measuring the concentrations of precursor hormones. For example, in 21-hydroxylase deficiency, keeping plasma concentrations of 17-hydroxyprogesterone in the 200- to 500-ng/dL range and keeping androstenedione in the normal physiologic range is desirable.

Plasma ACTH concentrations are of little help in adjusting doses of glucocorticoid in patients with primary adrenal insufficiency. Monitoring symptoms of salt craving and blood pressure, PRA, and electrolyte levels are helpful in adjusting the dose of fludrocortisone. High blood pressure with suppressed PRA should prompt a reduction in fludrocortisone dose.


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