Table 1.
Disease | Pre-conception | First trimester | Second and third trimesters | Delivery | Post-partum |
---|---|---|---|---|---|
Adrenal insufficiency | 1. Hydrocortisone 10–12 mg/m2/day (usually 15–20 mg) in two or three divided oral doses;2. Cortisone acetate (once daily 25–37.5 mg/day), prednisone or prednisolone (3–5 mg/day) can also be used.3. Avoid dexamethasone as it is not inactivated by 11β-HSD2.Note: (a) Prednisone and cortisone both require activation by maternal 11β-HSD1 (to form active prednisolone and cortisol respectively);(b) Women on other GCs should be switched to hydrocortisone. | Maintain preconception doses. No need to adjust GC replacement dose unless there is evidence of intercurrent illness. Parenteral GCs if intractable vomiting. | Adjust according to clinical course, often a 20–40% increase in the third trimester is needed. | 100 mg of hydrocortisone intramuscularly (or intravenously) at the onset of active labour (cervix dilation >4 cm or contractions every 5 min for 1 h, or both), followed by hydrocortisone 200 mg every 24 h either via continuous intravenous infusion or 50 mg every 6 h is recommended. | Adjust according to clinical condition or intercurrent illness. For the first two to four days a double oral dose should be maintained, provided there are no complications and pre-conception dose can be restored thereafter. |
Congenital adrenal hyperplasia | 1. Use prednisone, prednisolone or hydrocortisone, all of which are inactivated by placental 11β-HSD2;2. Consider steroid treatment in women with NCCAH who are infertile or have history of miscarriage;3. Pre-conception counselling and genetic testing in both the women and partners;4. Dexamethasone treatment should still be regarded as experimental and not routine. | Maintain preconception doses. No need to adjust GC replacement dose unless there is evidence of intercurrent illness.Parenteral GCs if intractable vomiting | Adjust according to clinical course, often a 20–40% increase in the third trimester is needed. | 100 mg hydrocortisone intramuscularly (or intravenously) at the onset of active labour (cervix dilation >4 cm or contractions every 5 min for 1 h, or both), followed by hydrocortisone 200 mg every 24 h either via continuous intravenous infusion or 50 mg every 6 h is recommended. | Adjust according to clinical condition or intercurrent illness. For the first two to four days a double oral dose should be maintained, provided there are no complications and pre-conception dose can be restored thereafter. |
Antenatal treatment for lung maturation | 4 × 6 mg IM doses of dexamethasone given 12 h apartor2 × 12 mg IM doses of betamethasone 24 h apart. Note: IV administration can be considered in anti coagulated women, Consider the possibility of tertiary adrenal insufficiency. | If adrenal insufficiency has been diagnosed, follow treatment above. | If adrenal insufficiency has been diagnosed, follow treatment above. | ||
All above | Women should be advised of possible adrenal crises; GC dose adjustment in case of stressful situations (a double dose for the period of illness and parenteral glucocorticoid in case of vomiting or emergency situations); Education for the woman and the partner regarding self-administration of parenteral hydrocortisone, and the wearing of an alert bracelet or equivalent is essential. |
GC: glucocorticoid; 11β-HSD: 11: β-hydroxysteroid dehydrogenase; NCCAH: non-classic congenital adrenal hyperplasia.