From the Guidelines
Women with Congenital Adrenal Hyperplasia (CAH) in pregnancy require an increased hydrocortisone dose by 2.5 or 10 mg daily due to the physiologic increase in corticosteroid-binding globulin (CBG) and total serum cortisol during the third trimester. This adjustment is crucial to prevent adrenal crisis and ensure optimal maternal and fetal outcomes. According to the consensus statement on the diagnosis, treatment, and follow-up of patients with primary adrenal insufficiency 1, the increased requirement for hydrocortisone is necessary to compensate for the increased metabolism of cortisol by the growing placenta.
Key considerations for hydrocortisone dose regulation in CAH women during pregnancy include:
- Monitoring of serum electrolytes, blood pressure, and clinical symptoms to guide dose adjustments
- Evaluation of salt cravings, blood pressure, and serum electrolytes to adjust fludrocortisone dose, as plasma renin activity (PRA) levels may not be a reliable parameter during pregnancy 1
- Administration of a bolus parenteral dose of 100 mg of hydrocortisone during delivery, repeated if necessary every 6 hours, and doubling the oral dose for 24-48 hours postpartum 1
Regular follow-up with an endocrinologist experienced in CAH management during pregnancy is essential to ensure optimal maternal and fetal outcomes. The goal is to maintain serum electrolytes, plasma renin activity, and 17-hydroxyprogesterone levels within the upper normal range for pregnancy, while minimizing the risk of maternal hypertension, gestational diabetes, and fetal growth restriction. Close monitoring and individualized dose adjustments are critical to achieve the best possible outcomes for CAH women during pregnancy.
From the Research
Hydrocortisol Dose Regulation in Congenital Adrenal Hyperplasia Women During Pregnancy
- The management of congenital adrenal hyperplasia (CAH) during pregnancy is crucial to prevent complications and ensure the health of both the mother and the fetus 2.
- Women with classic CAH require glucocorticoid therapy to be adjusted during pregnancy, at the time of delivery, and postpartum, and should be monitored for adrenal crisis 2, 3.
- The choice of glucocorticoid and the dosage may vary depending on the individual case, and the safety and benefits of dexamethasone therapy aimed at preventing genital virilization are still debated issues 3.
- A study found that flutamide decreases cortisol clearance in patients with CAH, and therefore, glucocorticoid replacement doses should be reduced when flutamide is added to the treatment regimen 4.
- In general, the management of CAH during pregnancy involves a multidisciplinary approach, including endocrinologists, obstetricians, and genetic counselors, to ensure the best possible outcomes for both the mother and the fetus 2, 5, 6.
- A multicenter survey found that hydrocortisone was the most commonly used glucocorticoid in pregnant women with adrenal insufficiency, including those with CAH, and that glucocorticoid dosage was increased at any time during pregnancy in 57% of cases 6.
Key Considerations
- Pregnant women with CAH should be closely monitored for adrenal crisis and other complications, and their glucocorticoid therapy should be adjusted as needed 2, 3, 6.
- The use of flutamide and other medications should be carefully considered and monitored to avoid adverse effects and ensure the best possible outcomes for both the mother and the fetus 4.
- A multidisciplinary approach to care is essential to ensure that pregnant women with CAH receive the best possible management and support during pregnancy, delivery, and postpartum 2, 5, 6.