PRENATAL TREATMENT OF CONGENITAL ADRENAL-HYPERPLASIA DUE TO 21-HYDROXYLASE DEFICIENCY

CONGENITAL adrenal hyperplasia due to 21-hydroxylase deficiency is the most common cause of female pseudohermaphroditism. The severe classic disorder results in excess adrenal secretion of androgen from early fetal life, thereby causing the virilization of the external genitalia of affected female fetuses from the time of sexual differentiation onward. In human fetuses, sexual differentiation is believed to occur between 9 and 13 weeks of gestation.12 We can thus presume that the increased secretion of androgen by the adrenals in fetuses with congenital adrenal hyperplasia begins during or even before this critical period. Congenital adrenal hyperplasia is an HLA-linked autosomal recessive … © 1990, Massachusetts Medical Society. All rights reserved.