Prenatal treatment of congenital adrenal hyperplasia resulting from 21-hydroxylase deficiency

J Pediatr. 1984 Nov;105(5):799-803. doi: 10.1016/s0022-3476(84)80310-8.

Abstract

In an attempt to prevent in utero virilization of female fetuses with 21-hydroxylase deficiency, six mothers at risk were treated with either hydrocortisone (n = 1) or dexamethasone (n = 5) in early pregnancy. Treatment was continued to term in the two pregnancies in which the diagnosis of an affected female fetus was confirmed. In patient 1 (hydrocortisone treatment) fetal adrenal suppression was only partial but the external genitalia were only slightly abnormal. In patient 2 (dexamethasone treatment) fetal adrenal suppression was achieved and the external genitalia were normal at birth. These encouraging results open a new prospect for treating congenital adrenal hyperplasia in utero.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • 17-alpha-Hydroxyprogesterone
  • Adrenal Hyperplasia, Congenital* / drug therapy*
  • Adult
  • Amniotic Fluid / analysis
  • Dexamethasone / therapeutic use
  • Female
  • Humans
  • Hydrocortisone / therapeutic use
  • Hydroxyprogesterones / blood
  • Infant, Newborn
  • Pregnancy
  • Prenatal Care*
  • Steroid Hydroxylases / deficiency*

Substances

  • Hydroxyprogesterones
  • 17-alpha-Hydroxyprogesterone
  • Dexamethasone
  • Steroid Hydroxylases
  • Hydrocortisone