From the Research
Women with Congenital Adrenal Hyperplasia (CAH) during pregnancy require glucocorticoid and sometimes mineralocorticoid replacement therapy to prevent adrenal crisis, maintain maternal health, and ensure optimal pregnancy outcomes. The recommended regimen includes hydrocortisone (15-25 mg daily divided into 2-3 doses) as the preferred glucocorticoid because it does not cross the placenta as readily as dexamethasone or prednisone, minimizing fetal exposure 1. For mineralocorticoid replacement, fludrocortisone (0.05-0.2 mg daily) is typically continued if the woman was taking it pre-pregnancy.
Key Considerations
- During pregnancy, hormone requirements often increase, particularly in the third trimester, necessitating dose adjustments based on clinical symptoms and laboratory monitoring 2.
- Regular assessment of blood pressure, electrolytes, plasma renin activity, and 17-hydroxyprogesterone levels is essential to guide therapy 3.
- Stress dosing (2-3 times maintenance dose) is required during labor and delivery to prevent adrenal crisis.
- The therapy addresses the underlying cortisol deficiency in CAH, which results from enzymatic defects in adrenal steroid biosynthesis, most commonly 21-hydroxylase deficiency 4.
Management Approach
- Preconception genetic testing and counseling with a high-risk obstetrics specialist is recommended for women with CAH who desire to become pregnant 1.
- Women with classic CAH should first optimize glucocorticoid treatment, followed by ovulation induction medications and gonadotropins if needed 1.
- Prenatal screening and diagnosis options during pregnancy include maternal serum cell free-DNA for sex of the baby, and chorionic villus sampling and amniocentesis for diagnosis of CAH 1.
Outcome Improvement
- The goal of hormone replacement therapy in CAH during pregnancy is to improve morbidity, mortality, and quality of life outcomes for both the mother and the fetus 5.
- By preventing adrenal crisis and maintaining maternal health, hormone replacement therapy can help ensure optimal pregnancy outcomes and reduce the risk of complications 2.