From the FDA Drug Label
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From the Research
Gestational mothers with Congenital Adrenal Hyperplasia (CAH) should be treated with glucocorticoid therapy, typically with hydrocortisone at doses of 15-25 mg daily divided into 2-3 doses, adjusted based on clinical symptoms and hormone levels, as recommended by the most recent study 1. The primary goal of treatment is to maintain hormone levels within the normal pregnancy range while avoiding overtreatment, which can lead to maternal Cushingoid symptoms and fetal growth restriction. Some key points to consider in the management of CAH during pregnancy include:
- Medication doses often need to be increased by 20-40% in the second and third trimesters due to increased metabolism and the rising levels of cortisol-binding globulin.
- Regular monitoring is essential, with clinical assessment every 4-6 weeks and measurement of serum 17-hydroxyprogesterone, androstenedione, and renin levels to guide dose adjustments.
- Stress-dose steroids (typically triple the usual dose) should be administered during labor and delivery.
- Patients should be managed by a multidisciplinary team including an endocrinologist and high-risk obstetrician. This approach helps prevent adrenal crisis during pregnancy, protects the fetus from excess androgens, and ensures optimal maternal and fetal outcomes, as supported by recent studies 1, 2. It is also important to note that the use of modified-release hydrocortisone (MR-HC) has been shown to improve biochemical disease control in adults with CAH, with reduction in steroid dose over time and patient-reported benefit 3. However, the choice of glucocorticoid replacement regimen and the safety and benefits of dexamethasone therapy are still debated issues, and more research is needed to determine the most effective form of glucocorticoid replacement therapy in CAH for children and adults 4, 5.