Treatment of Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency in Pregnancy

Clinical Scenario

This protocol addresses the management of congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency in women who are pregnant. Pregnancy introduces specific clinical considerations that shape the treatment approach and require careful monitoring throughout gestation and at delivery.

Treatment Approach — Overview

Management centers on glucocorticoid therapy, with the approach guided by monitoring for signs of insufficiency and specific considerations that apply during pregnancy — including different requirements across trimesters and around the time of labor and delivery. Certain glucocorticoid agents are specifically contraindicated in this setting.

Full regimen, agent selection, and trimester-specific adjustments available in the structured protocol →
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References
DOI: 10.1210/jc.2018-01865
  1. In women with CAH who become pregnant we recommend continued prepregnancy doses of HC/prednisolone and fludrocortisone therapy, with dosage adjustments if symptoms and signs of GC insufficiency occur.
  2. Clinicians should evaluate the need for an increase in GC during the second or third trimester and administer stress doses of GCs during labor and delivery.
  3. In such cases, a GC dosage increase of 20% to 40% from the 24th week onward is often beneficial (215).
  4. In women with CAH who are pregnant, or trying to become pregnant, we recommend against using GCs that traverse the placenta, such as Dex.
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