Jl. Nivelon et al., PRENATAL TREATMENT OF CONGENITAL ADRENAL- HYPERPLASIA DUE TO 21-HYDROXYLASE DEFICIENCY - A REVIEW OF 9 CASES, Annales de pediatrie, 40(7), 1993, pp. 421-425
Prenatal treatment based on administration of dexamethasone to the mot
her during pregnancy was initiated early during nine pregnancies with
a high risk of congenital adrenal hyperplasia due to 2 1 -hydroxylase
deficiency. The purpose of this treatment was to prevent fetal viriliz
ation by reducing production of androgens by the adrenal glands. Prena
tal diagnosis was achieved by comparing amniotic fluid cell HLA genoty
pes and more recently by subjecting trophoblasts to molecular genetic
studies. Together with prenatal determination of fetal sex, this allow
ed to determine that only two female fetuses were affected. Efficacy o
f continued prenatal treatment in these two cases was good in one case
and mediocre in the other. The treatment was well tolerated by the mo
thers and fetuses.