Abstract
It has been reported that prenatal dexamethasone (DEX) treatment of women carrying female CAH fetuses suppresses fetal adrenal function and prevents virilization of external genitalia. In 2 CAH families, the mothers were treated in their 2nd pregnancies with DEX (1mg/d) from week 10.Dex was stopped 5 d before amniocentesis (wk 17), then resumed until delivery. Both fetuses (46, XX) had high amniotic fluid 17OHP levels (10.5, 10.4;normal range:1,2-3.8 ng/ml) suggestive of homozygous 21-OHase def.Pregnancies and deliveries (term) were normal. Both newborns were virilized (Prader IV). Plasma levels of progesterone (P), 17-hydroxyprogesterone (17OHP), aldosterone (Aldo), cortisol, estriol and DEX were measured by specific RIAs in Pat.1 from wk 17, in Pat. 2 from wk 25 until delivery. Results (Pat.I/Pat.2) in ng/ml.
Whereas P, 17OHP and cortisol levels were generally suppressed comparison with normal pregnancies, estriol levels were not sufficiently suppressed throughout pregnancy, despite appropriate DEX levels. This suggests that fetal adrenal function was not adequately suppressed, possibly due to insufficient DEX dose and/or increased metabolic clearance rate.
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Dörr, H., Sippell, W., Haack, D. et al. PITFALLS OF PRENATAL TREATMENT OF CONGENITAL ADRENAL HYPERPLASIA (CAH) DUE TO 21-HYDROXYLASE DEFICIENCY. Pediatr Res 20, 1182 (1986). https://doi.org/10.1203/00006450-198611000-00052
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DOI: https://doi.org/10.1203/00006450-198611000-00052
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